MedWorm.com provides a medical RSS filtering service. Over 6000 RSS medical sources are combined and output via different filters. This feed contains the latest items from the 'Seminars in Pediatric Surgery' source. Thu, 09 Feb 2012 01:09:42 +0100 http://www.medworm.com/index.php?rid=5599943&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000977%2Fabstract%3Frss%3Dyes Pediatric surgeons play a critical role in diagnosing, staging, and treating malignant solid tumors in children. Over the years, the surgical management of the primary tumor site has evolved from an aggressive en-bloc resection at diagnosis to a more tailored surgical approach, often affecting definitive local control after the delivery of neoadjuvant therapy, as currently directed by many solid tumor protocols. In fact, inappropriate upfront resection can lead to unnecessary short- and long-term morbidity, an incomplete resection, and may be associated with a delay in the initiation of the systemic chemotherapy that is critical to the treatment of gross or occult metastatic disease. Therefore, it is important for the pediatric surgeon, as a member of the multidisciplinary team involved in... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599943 Wed, 18 Jan 2012 06:36:29 +0100 5599943 http://www.medworm.com/index.php?rid=5599942&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000953%2Fabstract%3Frss%3Dyes Distant metastases regularly occur in children with solid tumors. The most affected organ is the lung. Nearly in all extracranial pediatric solid tumors, the presence of lung metastases is associated with an adverse prognosis for the children. Therefore, the correct treatment of lung metastases is essential and influences the outcome. Despite different national and international trials for pediatric tumor entities, specific surgical aspects or guidelines for lung metastases are usually not addressed thoroughly in these protocols. The aim of this article is to present the diagnostic challenges and principles of surgical treatment by focusing on the influence of surgery on the outcome of children. Special points of interest are discussed that emphasize sarcomas, nephroblastomas, hepatoblasto... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599942 Wed, 18 Jan 2012 06:36:29 +0100 5599942 http://www.medworm.com/index.php?rid=5599941&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000941%2Fabstract%3Frss%3Dyes Rhabdomyosarcoma (RMS) is a malignant childhood tumor of mesenchymal origin that currently has a greater than 70% overall 5-year survival. Multimodality treatment is determined by risk stratification according to pretreatment stage, postoperative group, histology, and site of the primary tumor. Pretreatment staging is dependent on primary tumor site, size, regional lymph node status, and presence of metastases. Unique to RMS is the concept of postoperative clinical grouping that assesses the completeness of disease resection and takes into account lymph node evaluation. At all tumor sites, the clinical grouping, and therefore completeness of resection, is an independent predictor of outcome. Overall, the prognosis for RMS is dependent on primary tumor site, patient age, completeness of res... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599941 Wed, 18 Jan 2012 06:36:29 +0100 5599941 http://www.medworm.com/index.php?rid=5599940&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861100093X%2Fabstract%3Frss%3Dyes Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTS) represent a subgroup of sarcomas that encompass more than 50 distinct histologies. All are rare, but some are more common in patients younger than 20 years of age. The management of patients with many histologies overlap. However, this review will focus on issues unique to a select few NRSTS that are most common in pediatric and adolescent patients. Here, we will discuss the recent advances in the diagnosis, surgical management, and treatment of NRSTS. Adequate surgical local control of the primary tumor is a critical component of the treatment strategy will be emphasized in this review because it determines local and distant recurrence. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599940 Wed, 18 Jan 2012 06:36:29 +0100 5599940 http://www.medworm.com/index.php?rid=5599939&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000928%2Fabstract%3Frss%3Dyes represent a diverse group of tumors that present from in utero through adolescence at many nongonadal locations, from the neck to the sacrococcygeal region. Surgical resection remains the central element of management, and accurate surgical staging is essential to properly ascertain the correct risk-based treatment. The management for all benign tumors (mature and immature teratomas) and select completely resectable malignant tumors is surgery alone. Modern-day chemotherapy is extremely effective in infants and children with unresectable and metastatic disease and these children have a very high survival rate. The use of neoadjuvant chemotherapy allows vital organ preservation and there is no role for resection of vital structures at the time of initial presentation. (Source: Seminars in ... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599939 Wed, 18 Jan 2012 06:36:29 +0100 5599939 http://www.medworm.com/index.php?rid=5599938&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000916%2Fabstract%3Frss%3Dyes Thyroid cancer is an uncommon childhood malignancy that presents primarily in young children or adolescent females and may be related to radiation exposure or genetic predisposition. Gene alterations, such as RET mutation or RET/PTC rearrangement, are not uncommon. Recent studies have lead to an increased understanding of the role of these particular gene alterations in the diagnosis, prognosis, and treatment of thyroid cancer. Surgery remains the mainstay of treatment for thyroid cancer followed by radioactive iodine when appropriate. In patients with MEN2, prophylactic thyroidectomy is recommended, although a delay in the initial diagnosis is common. With early aggressive treatment and long-term follow-up, these patients generally have excellent outcomes. Recent research suggests potenti... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599938 Wed, 18 Jan 2012 06:36:29 +0100 5599938 http://www.medworm.com/index.php?rid=5599937&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000904%2Fabstract%3Frss%3Dyes This report provides a comprehensive review on the diagnosis and management of this tumor in children and adolescents, including its oncogenesis and associated syndromes. Surgery remains a mainstay of treatment, but there are no standard guidelines available at this time regarding the best practice for multimodality therapy as our understanding of the biology of GIST is still in evolution. Therefore, pediatric patients with GIST should be ideally treated in the context of clinical trials at specialized, multidisciplinary centers throughout the course of their disease, especially because these patients may live for years after diagnosis. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599937 Wed, 18 Jan 2012 06:36:29 +0100 5599937 http://www.medworm.com/index.php?rid=5599936&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000989%2Fabstract%3Frss%3Dyes Hepatoblastoma is the most common liver tumor of early childhood. According to recent studies its incidence seems to be increasing in North America and Europe. Since new histological variants have been described recently the formerly clear-cut distinction of hepatoblastoma and hepatocellular carcinoma may not be valid anymore and a new histological classification will be inaugurated by an international working group. Recent research identified prognostically relevant gene signatures as well as potential molecular targets for therapy of hepatoblastoma. The multicentric study groups in the USA, Europe and Japan recommend cisplatin based chemotherapy for neoadjuvant and adjuvant treatment. However, their risk stratification systems and general treatment strategies differ substantially. Theref... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599936 Wed, 18 Jan 2012 06:36:29 +0100 5599936 http://www.medworm.com/index.php?rid=5599935&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000898%2Fabstract%3Frss%3Dyes This article will summarize advances in our knowledge of the biology of Wilms tumor and describe the impact on clinical treatment of Wilms tumor. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599935 Wed, 18 Jan 2012 06:36:29 +0100 5599935 http://www.medworm.com/index.php?rid=5599934&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000965%2Fabstract%3Frss%3Dyes is a heterogeneous disease; tumors can spontaneously regress or mature, or display an aggressive, therapy-resistant phenotype. Increasing evidence indicates that the biological and molecular features of neuroblastoma significantly influence and are highly predictive of clinical behavior. Because of this, neuroblastoma has served as a paradigm for biological risk assessment and treatment assignment. Most current clinical studies of neuroblastoma base therapy and its intensity on a risk stratification that takes into account both clinical and biological variables predictive of relapse. For example, surgery alone offers definitive therapy with excellent outcome for patients with low-risk disease, whereas patients at high risk for disease relapse are treated with intensive multimodality thera... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599934 Wed, 18 Jan 2012 06:36:29 +0100 5599934 http://www.medworm.com/index.php?rid=5599933&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000886%2Fabstract%3Frss%3Dyes Major advances have occurred in the management of pediatric solid tumors of infants and children in the last several years. New information is available regarding their diagnosis, and in some cases new drug therapy is available that is directed at recently identified genetic alterations. Recent cooperative group trials have also clarified appropriate treatment in several tumor systems, and risk-based therapy is used increasingly to minimize the long-term morbidity of treatment. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599933 Wed, 18 Jan 2012 06:36:29 +0100 5599933 http://www.medworm.com/index.php?rid=5599932&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611001053%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5599932 Wed, 18 Jan 2012 06:36:29 +0100 5599932 http://www.medworm.com/index.php?rid=5276772&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000333%2Fabstract%3Frss%3Dyes This article seeks to review the history of laparoscopic surgery, apply lessons learned during the past 10 years to the evolution of single incision laparoscopic surgery, and urge for sound prospective evaluation for the use of laparoscopic surgery using a single umbilical incision. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276772 Mon, 03 Oct 2011 17:22:54 +0100 5276772 http://www.medworm.com/index.php?rid=5276771&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000321%2Fabstract%3Frss%3Dyes This study is a retrospective analysis of all magnet-assisted laparoscopic operations performed at the Fundacion Hospitalaria Private Children's Hospital from September 2009 to January 2011. Outcomes include demographics, diagnosis, operative time, intraoperative complications, and conversion rates. Forty-four magnet-assisted laparoscopic operations were performed. The operations included 23 appendectomies, 8 cholecystectomies, 3 Nissen fundoplications, 2 gastrojejunostomies, 2 splenectomies, 2 ovarian tumor/cyst resections, 1 retroperitoneal lymphangioma resection, 1 left adrenalectomy, 1 total abdominal colectomy and 1 pulmonary wedge resection. The mean operative times for the most commonly performed operations were 61 minutes for appendectomy and 93 minutes for cholecystectomy. The ope... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276771 Mon, 03 Oct 2011 17:22:54 +0100 5276771 http://www.medworm.com/index.php?rid=5276770&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861100031X%2Fabstract%3Frss%3Dyes The single-site umbilical laparoscopic approach to abdominal surgery has become an evolving trend over the past few years. These operations have some technical limitations attributable to the loss of instrument triangulation, crowding of instruments, loss of ergonomic movements, and the fact that the view of the telescope/camera is in-line with the working instruments. However, because the size of the umbilical incision is determined by the size of the lesion or segment of bowel being resected during a traditional laparoscopic bowel resection, these operations are perhaps the most suited procedures for this approach. In this review, the techniques and applications for single-site umbilical laparoscopic bowel resection in children will be discussed. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276770 Mon, 03 Oct 2011 17:22:54 +0100 5276770 http://www.medworm.com/index.php?rid=5276769&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000308%2Fabstract%3Frss%3Dyes This article will describe the reasons that the single-site approach might be useful for splenectomy and also the technique used at the author's institution. Moreover, a brief review of the current literature in children will be presented. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276769 Mon, 03 Oct 2011 17:22:54 +0100 5276769 http://www.medworm.com/index.php?rid=5276768&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000345%2Fabstract%3Frss%3Dyes This article will describe the single-site approach used at the Children's Hospital of Alabama and the early outcomes from its use in a relatively small group of infants. An improvement in the cosmetic appearance of the abdominal wall with the single-site approach appears to be the primary reason for its use. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276768 Mon, 03 Oct 2011 17:22:54 +0100 5276768 http://www.medworm.com/index.php?rid=5276767&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000291%2Fabstract%3Frss%3Dyes This article will describe the single-site umbilical laparoscopic cholecystectomy, which has been performed at Children's Mercy Hospital since 2009. Also, the literature concerning this approach in adults and in children will be discussed. Finally, a prospective randomized trial comparing the single-site cholecystectomy with the traditional 4-port laparoscopic cholecystectomy will also be described. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276767 Mon, 03 Oct 2011 17:22:54 +0100 5276767 http://www.medworm.com/index.php?rid=5276766&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861100028X%2Fabstract%3Frss%3Dyes This article will review the background of laparoscopy for appendectomy, the introduction of the single-incision approach, available data and outcomes from current literature, and a description of our technique. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276766 Mon, 03 Oct 2011 17:22:54 +0100 5276766 http://www.medworm.com/index.php?rid=5276765&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000278%2Fabstract%3Frss%3Dyes The minimally invasive approach has become the preferred technique for many pediatric operations. Advances in instrumentation and equipment have allowed for the introduction of a new type of minimally invasive surgery: single-site umbilical laparoscopic surgery (SSULS). The ultimate goal of SSULS is to leave the patient with no visible scar. In this article, we describe an array of instruments and equipment that are used for SSULS and discuss some of the related controversies of SSULS in children. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276765 Mon, 03 Oct 2011 17:22:54 +0100 5276765 http://www.medworm.com/index.php?rid=5276764&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000266%2Fabstract%3Frss%3Dyes The laparoscopic revolution in adults began in Europe in the mid-1980s and in the United States a few years later. After the early descriptions of the use of laparoscopy in adults, a few pediatric surgeons began to adopt this approach and use it commonly in the first part of the 1990s. However, in the pediatric surgical arena, it was slow to be adopted by most pediatric surgeons until the second half of the 1990s. The primary reason for this slow adoption was a lack of appreciation of the benefits of the laparoscopic approach, including a reduction in postoperative hospitalization. Moreover, there was slow appreciation of the reduced pain after a laparoscopic operation. In addition, the laparoscopic technique was a novel one, and many pediatric surgeons found the approach difficult. Finall... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276764 Mon, 03 Oct 2011 17:22:54 +0100 5276764 http://www.medworm.com/index.php?rid=5276763&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000618%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=5276763 Mon, 03 Oct 2011 17:22:54 +0100 5276763 http://www.medworm.com/index.php?rid=4972118&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000199%2Fabstract%3Frss%3Dyes Faulty ventral openings of the urethra constitute a broad spectrum of malformations that are subsumed under the term “hypospadia.” The normal development of the urethra and the genitals critically depends on the following events: (a) formation of the external genitalia, (b) fate of the cloacal membrane, and (c) formation of the distal urethra. The purpose of this study was to demonstrate these events using microsurgical techniques and scanning electron microscopy in staged rat embryos. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972118 Tue, 28 Jun 2011 01:41:37 +0100 4972118 http://www.medworm.com/index.php?rid=4972117&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000230%2Fabstract%3Frss%3Dyes Numerous researchers studied the morphology of the testicular descent, including the possible function of the gubernaculum. However, a clear illustration of this process is still missing. The aim of this paper was to illustrate the embryology of the testicular descent in the rat by scanning electron microscopy. In a first phase of the intra-abdominal testicular descent, the testis moves actively from the lower pole of the kidney towards the bladder neck. In a second inguinal phase the testis enters groin and moves in the developing processus vaginalis peritonei caused by the disappearance of the bulb of the gubernaculums testis. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972117 Tue, 28 Jun 2011 01:41:37 +0100 4972117 http://www.medworm.com/index.php?rid=4972116&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000229%2Fabstract%3Frss%3Dyes Despite the progress in prenatal diagnosis and intervention as well as postnatal therapeutic strategies, congenital diaphragmatic hernia (CDH) is still associated with a meaningful mortality because of the induced pulmonary hypoplasia. An essential key in understanding the pathogenesis of CDH is the underlying embryology, which has been neglected during the last decades. Likewise, the development of the normal diaphragm is still poorly understood. Obsolescent perceptions, mainly formed from histologic sections, are still propagated. In this review we present an atlas of scanning electron microscopy images that depict the normal and defective development of the diaphragm in the nitrofen rat model for CDH. Our findings suggest that the normal diaphragm mainly develops from the posthepatic me... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972116 Tue, 28 Jun 2011 01:41:37 +0100 4972116 http://www.medworm.com/index.php?rid=4972115&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000187%2Fabstract%3Frss%3Dyes In this study, scanning electron microscopy of staged rat embryos between the gestational days 10-15 was performed to show the normal development of the hindgut and the abnormal development in Danforth's short tail (SD) mice. Our studies in normal and abnormal development indicate that the embryonic cloaca never passes through a stage that is similar to any form of anorectal malformation in neonates, including the so-called “cloacas” in females. To explain the abnormal development in anorectal malformations, further studies are mandatory. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972115 Tue, 28 Jun 2011 01:41:37 +0100 4972115 http://www.medworm.com/index.php?rid=4972114&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000217%2Fabstract%3Frss%3Dyes In most textbooks of embryology and pediatric surgery, the puzzling spectrum of midgut “malrotations” is explained by an “impaired” process of rotation of the midgut. However, this “process of rotation” is explained in a rather schematic way and aims more to explain pathologic findings whereas detailed embryologic investigations are still rare in this field. Good animal models which would allow the comparison of normal and abnormal midgut development are missing. In this paper we describe the development of the midgut in form of an atlas. Scanning electron microscopy is used in rat embryos to illustrate the crucial embryologic processes of midgut development. The main result shown in these illustrations is that clear signs of a process of rotation are missing. (Source: Seminars... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972114 Tue, 28 Jun 2011 01:41:36 +0100 4972114 http://www.medworm.com/index.php?rid=4972113&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000205%2Fabstract%3Frss%3Dyes This study was performed in chicken embryos in accordance to the developmental stages described. As the main results from these illustrations show, we found no evidence for lateral foregut ridges inside the undivided foregut chamber and no fusion of lateral foregut components to form a trachea-esophageal septum. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972113 Tue, 28 Jun 2011 01:41:36 +0100 4972113 http://www.medworm.com/index.php?rid=4972112&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000175%2Fabstract%3Frss%3Dyes In the presented series of papers, the main focus lies in the illustrations of the development of embryos and embryonic organs. In our opinion, the form of an “atlas” is an appropriate way to illustrate step by step the normal development of organ systems that are of interest for pediatric surgeons. Whenever possible, illustrations of abnormal development are added. We believe that appropriate and illustrative findings in various fields of embryology are still lacking. This explains why today many typical malformations are still not explained satisfactorily. Thus, in this series of papers, scanning electron microscopy was used to illustrate embryonic development. Scanning electron microscopy was used because of the following advantages: (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972112 Tue, 28 Jun 2011 01:41:36 +0100 4972112 http://www.medworm.com/index.php?rid=4972111&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000382%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972111 Tue, 28 Jun 2011 01:41:36 +0100 4972111 http://www.medworm.com/index.php?rid=4972110&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000370%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4972110 Tue, 28 Jun 2011 01:41:36 +0100 4972110 http://www.medworm.com/index.php?rid=4655316&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001150%2Fabstract%3Frss%3Dyes Developmental psychopathology is common in children with exstrophy. It may be mild or severe, and it may persist or transform as the child grows. The pediatric urologist is ideally situated to identify signs or symptoms of early developmental psychopathology in these children. Presented in this article are techniques for identifying the child requiring full assessment and for establishing referral-consultants. Screening instruments are suggested, as well as how to use these to educate the parents and the child. Methods are provided to identify, as well as to educate, selected consultants in child psychology and psychiatry about the clinical realities of exstrophy. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655316 Wed, 30 Mar 2011 21:27:53 +0100 4655316 http://www.medworm.com/index.php?rid=4655315&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001149%2Fabstract%3Frss%3Dyes This article summarizes some of the challenges and reconstructive methods to permit eventual continence in children with cloacal exstrophy. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655315 Wed, 30 Mar 2011 21:27:53 +0100 4655315 http://www.medworm.com/index.php?rid=4655314&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001137%2Fabstract%3Frss%3Dyes The OEIS complex (ie, omphalocele-exstrophy-imperforate anus-spinal defects) is a rare and complex anomaly requiring collaboration among multiple pediatric surgical subspecialties throughout the early childhood period. Initial gastrointestinal management involves identification of all intestinal components with reconstruction of the entire length of intestines in-line leading to an end colostomy. Candidacy for an abdominoperineal intestinal pull-through procedure is dependent upon the patient's ability to form solid stools, degree of pelvic neuromuscular development, and ability to comply with a bowel management program. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655314 Wed, 30 Mar 2011 21:27:53 +0100 4655314 http://www.medworm.com/index.php?rid=4655313&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000035%2Fabstract%3Frss%3Dyes This article reviews the different options available for pelvic osteotomy and stabilization of the pubic symphysis in patients with cloacal exstrophy. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655313 Wed, 30 Mar 2011 21:27:53 +0100 4655313 http://www.medworm.com/index.php?rid=4655312&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001125%2Fabstract%3Frss%3Dyes Cloacal exstrophy, one of the most severe congenital anomalies compatible with life, occurs in up to 1 in 200,000 lives births. The condition affects nearly every major organ system with severe neurologic, skeletal, gastrointestinal, and genitourinary ramifications. With increased understanding of the anatomy and embryology combined with refinements in prenatal diagnosis and postnatal care, there is now near-universal survival of patients with cloacal exstrophy. Functional and cosmetic outcomes have improved with modifications in surgical technique. However, debate continues regarding the issue of gender identity, and long-term data are still accruing with respect to the best strategy for management. Despite the extensive malformations noted, many patients have gone on to live fruitful liv... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655312 Wed, 30 Mar 2011 21:27:52 +0100 4655312 http://www.medworm.com/index.php?rid=4655311&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001113%2Fabstract%3Frss%3Dyes The bladder exstrophy-epispadias-cloacal exstrophy complex is a spectrum of genitourinary malformations requiring multiple major reconstructive operations on each affected child. The need for surgical correction in this condition often continues through adolescence and into adulthood. Experience in caring for individuals with exstrophy-epispadias has taught us a great deal about the long-term functional, psychological, and social outcomes involved. Children undergoing repeated hospital admissions and extensive multiple operations have the potential for long-term adjustment problems with incontinence, ambulatory difficulties, psychological disturbance, sexual dysfunction, and issues surrounding self-esteem and social integration. By examining relevant published works from the world literatu... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655311 Wed, 30 Mar 2011 21:27:52 +0100 4655311 http://www.medworm.com/index.php?rid=4655310&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001101%2Fabstract%3Frss%3Dyes Despite improvements in modern surgical reconstructive techniques, many patients with epispadias-exstrophy continue to experience urinary incontinence. Continent diversion is commonly performed to achieve urinary continence and improve quality of life. In this work we describe the population that can be considered for continent urinary diversion, consider the benefits and implications of concurrent augmentation and bladder neck closure, and review recent literature regarding continence outcomes and common complications. Even in this complex patient population, urinary continence can be reliably achieved by bladder augmentation and the use of intermittent catheterization via a catheterizable cutaneous stoma with or without closure of the bladder neck. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655310 Wed, 30 Mar 2011 21:27:52 +0100 4655310 http://www.medworm.com/index.php?rid=4655309&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001095%2Fabstract%3Frss%3Dyes Children with bladder exstrophy present a formidable surgical challenge. Like all major reconstructive surgeries, the best hope for a favorable outcome lies in achieving success in the first operative attempt. Regardless of the surgical approach, however, complications do occur. A failed exstrophy closure is a major complication with significant implications on the long-term surgical outcome and ultimate fate of the urinary tract. Successful repeat exstrophy closure can be accomplished in most cases when performed in conjunction with pelvic osteotomy and proper postoperative immobilization. Modern staged repair of exstrophy, complete primary repair of exstrophy, and immediate continent urinary diversion have been advocated by different groups in the management of a failed exstrophy closure... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655309 Wed, 30 Mar 2011 21:27:52 +0100 4655309 http://www.medworm.com/index.php?rid=4655308&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001083%2Fabstract%3Frss%3Dyes A major goal of bladder exstrophy (BE) management is achieving urinary continence, most commonly with surgical bladder neck reconstruction (BNR). This is a report of outcome of BNR after complete primary repair of exstrophy (CPRE). At our institution, patient history, ultrasound, cystogram (VCUG) and urodynamic study (UDS) were performed during a prospective evaluation of patients with BE. Dry interval of >3 hours was used as the definition of continence and dry interval (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655308 Wed, 30 Mar 2011 21:27:52 +0100 4655308 http://www.medworm.com/index.php?rid=4655307&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000047%2Fabstract%3Frss%3Dyes Epispadias is a rare diagnosis and most commonly described as a part of the bladder exstrophy complex. Epispadias is characterized by failure of the urethral plate to tubularize on the dorsum with the defect ranging from a glandular to a penopubic location. In addition, male patients demonstrate a dorsal chordee whereas female patients exhibit a bifid clitoris. The goal of surgical correction is the placement of the meatus in its anatomical position and the creation of functional genitalia with good cosmetic outcomes. Two different major reconstruction principals, the modified Cantwell-Ransley and the Mitchell Bagli repair, are used in specialized centers worldwide. Both procedures comprise the major principles of epispadias surgery but differ in timing and the extent of urethral mobilizat... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655307 Wed, 30 Mar 2011 21:27:51 +0100 4655307 http://www.medworm.com/index.php?rid=4655306&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000023%2Fabstract%3Frss%3Dyes Until the 1970s, bladder exstrophy was a frequently fatal birth defect that, at the very least, conferred a shortened life span with devastating consequences for afflicted patients. Beginning with the modern era of surgical management, survival has become routine, and now the focus is on optimizing a near-normal return to function and cosmesis while limiting the frequency and morbidity of reconstructive procedures. Here we discuss the most frequently used strategies, their principles, and most recent outcomes for the management of exstrophy worldwide. Advantages and disadvantages of each approach are discussed in broad terms with the understanding that one universally accepted technique for managing bladder exstrophy has yet to be developed. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655306 Wed, 30 Mar 2011 21:27:51 +0100 4655306 http://www.medworm.com/index.php?rid=4655305&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001071%2Fabstract%3Frss%3Dyes Classic bladder exstrophy (CBE) patients are born with a pubic diastasis that increases steadily with age from a mean value of 4 cm at birth to a mean of 8 cm at age 10, compared with a mean normal width of the pubic symphysis of 0.6 cm at all ages. The width of the sacrum and length of the posterior (iliac) segment of the pelvis in CBE patients are normal; however, the anterior (ischiopubic) segment of the pelvis is a mean 30% shorter and both the anterior and posterior segments are externally rotated compared to controls. The main role of osteotomy in treatment of CBE appears to be to relax tension on the bladder and repaired abdominal wall during wound-healing. Anterior innominate osteotomy with optional posterior vertical iliac osteotomy presents several advantages over the prior conve... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655305 Wed, 30 Mar 2011 21:27:51 +0100 4655305 http://www.medworm.com/index.php?rid=4655304&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610001174%2Fabstract%3Frss%3Dyes This article covers the embryologic theories of this congenital defect and the subsequent bony pelvic and pelvic floor muscular defects characteristic of exstrophy. Primarily, the anatomic focus will be on classic bladder exstrophy because it is the most common and well studied to date. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655304 Wed, 30 Mar 2011 21:27:50 +0100 4655304 http://www.medworm.com/index.php?rid=4655303&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000059%2Fabstract%3Frss%3Dyes The first description of bladder exstrophy was noted on Assyrian tablets nearly 4000 years ago. Since then various papers has been published with an increasing rate. According to the available historical data, almost all operative techniques had been described during the last two centuries. We believe, the pioneers put a lot of work in this field and passed on their theoretic knowledge and surgical experience to the current era. Our duty is keep this treasure and add the benefits of recent new technological developments for the future care of our bladder exstrophy patients. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655303 Wed, 30 Mar 2011 21:27:50 +0100 4655303 http://www.medworm.com/index.php?rid=4655302&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861000106X%2Fabstract%3Frss%3Dyes Great strides have been made in the treatment of the bladder exstrophy spectrum during the last 10 years, and the results have improved markedly. Basic science discoveries, such as the nature of the collagen, smooth muscle, and neural innervations at the cellular level; the nature of the bony pelvis at the cellular level; and better definition of the pelvic floor musculature have better defined the true nature of this major birth defect. In addition, clinical discoveries, such as the improved role of osteotomy in both bladder and cloacal exstrophy, better immobilization and pain control after primary closure in the newborn, and larger series showing that most all patients will require some sort of outlet procedure regardless of the initial repair, have increased success rates and outcomes.... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655302 Wed, 30 Mar 2011 21:27:49 +0100 4655302 http://www.medworm.com/index.php?rid=4655301&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000102%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655301 Wed, 30 Mar 2011 21:27:49 +0100 4655301 http://www.medworm.com/index.php?rid=4655300&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000096%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655300 Wed, 30 Mar 2011 21:27:49 +0100 4655300 http://www.medworm.com/index.php?rid=4655299&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858611000072%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4655299 Wed, 30 Mar 2011 21:27:49 +0100 4655299 http://www.medworm.com/index.php?rid=4290802&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000739%2Fabstract%3Frss%3Dyes Each form of congenital hyperinsulinism (CHI)—focal, diffuse, atypical—requires its own surgical strategy and technique. Focal CHI is treated by a positron emission tomography/computed tomography-guided, local resection which is confined only to the lesion. As much healthy pancreatic tissue as possible is preserved. On the contrary, the therapeutic mainstay of diffuse CHI must be conservative nowadays. Only in the exceptional cases in which medical treatment fails surgical therapy is warranted to prevent hypoglycemia. However, the extension of resection that is able to cure hyperinsulinism while avoiding diabetes is not known today. The outcome, therefore, is unpredictable. In the rare atypical cases it is important to stop the resection at the right time in order not to finish unneces... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290802 Tue, 28 Dec 2010 22:38:44 +0100 4290802 http://www.medworm.com/index.php?rid=4290801&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000740%2Fabstract%3Frss%3Dyes Beyond the 2 classical forms of congenital hyperinsulinism, focal and diffuse, we report our experience on the surgical treatment of atypical forms. We define 2 subtypes among these atypical forms of hyperinsulinism: in case of a giant focal form the surgical strategy is the same as in focal forms. In case of hyperinsulinism caused by a mosaic, our experience suggests the benefit of a limited resection from the tail to the body of the pancreas. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290801 Tue, 28 Dec 2010 22:38:44 +0100 4290801 http://www.medworm.com/index.php?rid=4290800&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000806%2Fabstract%3Frss%3Dyes We describe both methods of pancreatectomy. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290800 Tue, 28 Dec 2010 22:38:44 +0100 4290800 http://www.medworm.com/index.php?rid=4290799&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000764%2Fabstract%3Frss%3Dyes Advances in imaging and surgical techniques allow a complete cure for children with focal-type congenital hyperinsulinism (CHI). In contrast, management of diffuse-type CHI remains a matter of controversy. To prevent hypoglycemic brain damage, extensive surgery has been recommended in the past, resulting in diabetes. On the basis of 2 data sets of patients with congenital hyperinsulinism, the German registry for CHI with 235 patients (ages 1 day to 19 years) and the diabetes treatment register (Diabetes Patienten-Verlaufsdokumentationssystem initiative), a follow-up study was initiated for diabetes mellitus and the intellectual and physical development as well as motor function. In our ongoing study, we investigated 20 patients with CHI (12 male, mean ages 9.9 years). Six of 20 patients ha... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290799 Tue, 28 Dec 2010 22:38:44 +0100 4290799 http://www.medworm.com/index.php?rid=4290798&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000776%2Fabstract%3Frss%3Dyes (CHI) are caused by mutations in GLUD1 (encoding glutamate dehydrogenase), GCK (encoding glucokinase), HADH (encoding for L-3-hydroxyacyl-CoA dehydrogenase), SLC16A1 (encoding the monocarboxylat transporter 1), HNF4A (encoding hepatocyte nuclear factor 4α) or UCP2 (encoding mitochondrial uncoupling protein 2). The clinical presentation is very heterogeneous in regards to age of onset, severity, and manner of symptoms, as well as the response to medical treatment. Special individual characteristics have to be accounted in diagnosis and treatment. Diazoxide is the first-line drug for the rare forms of CHI for long-term treatment but is not entirely effective in some of these rarer defects (GCK, MCT1). The use of diazoxide is often limited by side effects and the use of octreotide as second... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290798 Tue, 28 Dec 2010 22:38:44 +0100 4290798 http://www.medworm.com/index.php?rid=4290797&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861000079X%2Fabstract%3Frss%3Dyes This report describes a center specializing in HI with a team of experts consisting of endocrinologists, nurse practitioners, geneticists, radiologists, pathologists, and a surgeon. It describes the center's paradigm for managing severe HI on the basis of more than 250 cases of HI in the past 10 years, including the diagnosis of HI, medical options, genetics of HI, imaging in HI, the surgical approach to HI, and outcomes. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290797 Tue, 28 Dec 2010 22:38:44 +0100 4290797 http://www.medworm.com/index.php?rid=4290796&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861000082X%2Fabstract%3Frss%3Dyes In surgery for focal congenital hyperinsulinism (CHI), the identification and complete resection of the focus without collateral damage is of utmost importance. In a pilot study we applied intra-abdominal high-frequency sonography during surgery for focal CHI in 2 infants. The focus could be identified, its relation to the pancreatic and common bile duct could be shown, and the typical octopus-like tentacles could be demonstrated. In one case the resection was successful; in the other it was not. These preliminary results suggest that intraoperative sonography could be a valuable tool in the surgical therapy of focal CHI and warrants further evaluation in a clinical study. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290796 Tue, 28 Dec 2010 22:38:44 +0100 4290796 http://www.medworm.com/index.php?rid=4290795&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000788%2Fabstract%3Frss%3Dyes The unfavorable prognosis of congenital hyperinsulinism (CHI) can be avoided if the patients are treated with high-dose glucose infusions and timely surgical intervention. Circumscribed foci used to be identified by selective percutaneous pancreatic vein catheterization and determination of the insulin level. Fluorine-18-L-dihydroxyphenylalanine-positron emission tomography (PET) was developed as a milder alternative for diagnostic localization of focal disease. The uptake of fluorine-18-L-dihydroxyphenylalanine is considerably increased in foci with high insulin synthesis rates. In Berlin, diagnosis was achieved by high definition PET/computed tomography with multiphase contrast media protocols that provided all necessary data with one investigation. We have investigated 135 patients with... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290795 Tue, 28 Dec 2010 22:38:44 +0100 4290795 http://www.medworm.com/index.php?rid=4290794&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000831%2Fabstract%3Frss%3Dyes Adenosine triphosphate (ATP)-sensitive potassium channels (KATP channels) have a central role in the regulation of insulin secretion in pancreatic β cells. They are octameric complexes organized around the central core constituted by the Kir6.2 subunits. The regulation of the channel itself takes place on the sulfonylurea receptor-1 subunit. The channel opens and closes according to the balance between adenine nucleotide ATP and adenosine diphosphate. Hyperinsulinemic hypoglycemia (also named congenital hyperinsulinism, or CHI) is associated with loss-of-function KATP channel mutations. Their frequency depends on the histopathological form and the responsiveness of CHI patients to diazoxide. ABCC8/KCNJ11 defects are identified in approximately 80% of patients with CHI refractory to diazox... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290794 Tue, 28 Dec 2010 22:38:44 +0100 4290794 http://www.medworm.com/index.php?rid=4290793&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000752%2Fabstract%3Frss%3Dyes A genetic diagnosis is now possible for approximately 45%-55% of patients with hyperinsulinemic hypoglycemia. Understanding the genetic etiology of the disease in these patients is clinically important because a genetic diagnosis will provide information on prognosis, recurrence risk, and importantly may also guide clinical management. The aim of this review is to provide an outline of the 7 different molecular mechanisms underlying this heterogeneous disease and to demonstrate that the clinical phenotype can act as a useful guide when prioritizing the order of genetic testing. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290793 Tue, 28 Dec 2010 22:38:44 +0100 4290793 http://www.medworm.com/index.php?rid=4290792&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000818%2Fabstract%3Frss%3Dyes Congenital hyperinsulinism is clinically characterized by an inappropriate insulin secretion resulting in recurrent severe hypoglycemia. Nesidioblastosis, the proliferation of islet cells budding off from ducts, has been considered for years as the histologic lesion responsible for the syndrome. In our morphologic studies, we demonstrate that nesidioblastosis is not specific of the disease, which is actually not a single entity. Indeed, we recognize the existence of 2 different forms—a diffuse form and a focal form—and demonstrate that they can be differentiated by morphologic criteria, even on frozen sections during surgery. This histologic distinction directs the therapeutic approach because the patients experiencing the focal form of the syndrome can be completely cured by a very li... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290792 Tue, 28 Dec 2010 22:38:44 +0100 4290792 http://www.medworm.com/index.php?rid=4290791&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000727%2Fabstract%3Frss%3Dyes Congenital hyperinsulinism (CHI) is usually not a disease that is first on the list as a diagnosis in the newborn. If a newborn infant presents with macrosomia, seizures, tremulousness, hypotonia, cyanosis, or hypothermia, it is challenging to think of a disease with an incidence of 1 in 50,000 live births. CHI is characterized by inappropriate secretion of insulin by the β cells of the islets of Langerhans. It is an enigmatic disease with many facets. Hypoglycemia of the newborn can be transient. If CHI occurs later in infancy or childhood, hypoglycemia usually is less severe, and the diagnosis is often delayed. CHI exhibits unique symptomatology but can have many causes. The clinical course cannot be predicted because some instances are self-limiting with spontaneous cure, some result i... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290791 Tue, 28 Dec 2010 22:38:44 +0100 4290791 http://www.medworm.com/index.php?rid=4290790&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000879%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290790 Tue, 28 Dec 2010 22:38:44 +0100 4290790 http://www.medworm.com/index.php?rid=4290789&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000867%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4290789 Tue, 28 Dec 2010 22:38:44 +0100 4290789 http://www.medworm.com/index.php?rid=4029280&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000466%2Fabstract%3Frss%3Dyes Minimally invasive techniques have now become standard for the treatment of many surgical conditions in children. During the past decade, there has been increasing interest in the use of this technology for the management of injured children. Laparoscopy has become an important adjunct in the evaluation of both blunt and penetrating intra-abdominal trauma and frequently is both diagnostic and therapeutic. Laparoscopic techniques have been used to repair injuries involving the gastrointestinal tract, solid organs, and the diaphragm. These procedures have been performed successfully, and avoid the complications associated with formal laparotomy. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029280 Tue, 05 Oct 2010 05:24:08 +0100 4029280 http://www.medworm.com/index.php?rid=4029279&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000429%2Fabstract%3Frss%3Dyes Recent research has uncovered the prevalence of posttraumatic stress disorder (PTSD) after traumatic experiences in the pediatric population at an alarming rate. This review discusses new challenges in identifying children at risk for the development of PTSD, as well as understanding the unique aspects of this disorder found in the pediatric population. In addition to the characterization of pediatric PTSD, this review addresses current screening tools and treatment strategies used in childhood stress disorders. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029279 Tue, 05 Oct 2010 05:24:08 +0100 4029279 http://www.medworm.com/index.php?rid=4029278&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000454%2Fabstract%3Frss%3Dyes Hemorrhagic shock in the pediatric trauma patient is an uncommon but fundamental problem for the treating clinician. Current management of hemorrhagic shock involves initial resuscitation with crystalloid fluids followed by infusion of blood components as necessary. In management of the adult trauma patient, many institutions have implemented massive transfusion protocols to guide transfusion in situations requiring or anticipating the use of greater than 10 U of packed red blood cells. In the pediatric population, guidelines for massive transfusion are vague or nonexistent. Adult trauma transfusion protocols can be applied to children until a pediatric protocol is validated. Here, we attempt to identify certain principles of transfusion therapy specific to pediatric trauma and outline a s... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029278 Tue, 05 Oct 2010 05:24:08 +0100 4029278 http://www.medworm.com/index.php?rid=4029277&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000442%2Fabstract%3Frss%3Dyes Brain injuries represent the most common cause of mortality and long-term morbidity from trauma in children. The management of closed head injuries focuses on prevention of secondary injury by optimizing the delivery of oxygen and nutrients to the injured brain while minimizing neuronal metabolic demand. Despite the known differences between the immature and mature brain, treatments used in head-injured children are mainly extrapolated from those employed in adults due to the paucity of class one and two studies focused on the pediatric age group. Therapies intended to minimize secondary brain injury, such as cerebrospinal fluid drainage, hypertonic saline infusion, barbiturate coma induction, brain cooling, and decompressive craniectomy, vary widely in their clinical application among pra... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029277 Tue, 05 Oct 2010 05:24:08 +0100 4029277 http://www.medworm.com/index.php?rid=4029276&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000417%2Fabstract%3Frss%3Dyes This article reviews the current literature to discuss the initial evaluation, management, and long-term outcomes in children sustaining MTBI. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029276 Tue, 05 Oct 2010 05:24:08 +0100 4029276 http://www.medworm.com/index.php?rid=4029275&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000399%2Fabstract%3Frss%3Dyes Recent disasters involving pediatric victims have highlighted the need for pediatric hospital disaster preparedness. Although children represent 25% of the U.S. population, there are significant gaps in pediatric disaster preparedness across the country. Disaster planners and others tend to overlook pediatric needs, and therefore plans are often inadequate. To establish an effective hospital and community-based pediatric disaster management system, administrative and hospital leadership are key. Disaster planners and hospital leadership should establish and improve their management of pediatric victims in the event of a disaster through staff training, family reunification planning, and use of available pediatric disaster management tools. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029275 Tue, 05 Oct 2010 05:24:08 +0100 4029275 http://www.medworm.com/index.php?rid=4029274&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000387%2Fabstract%3Frss%3Dyes Cervical spine injury in pediatric trauma occurs rarely; however, there is significant potential for considerable morbidity when it does occur. Screening for cervical spine injuries has been shown to be most sensitive in adult trauma centers when combined with reliable physical examination findings. Because pediatric trauma patients suffer from a different range of injuries than adults, and often are not reliable due to age limitations or associated head injury, the same strategies employed in adult trauma do not always hold true in children. We look at the differences in adult and pediatric cervical spine anatomy and traumatic mechanisms, as well as the differences between cervical spine injury in infants/children and adolescents/teens. In addition, we examine the literature currently ava... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029274 Tue, 05 Oct 2010 05:24:08 +0100 4029274 http://www.medworm.com/index.php?rid=4029273&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000405%2Fabstract%3Frss%3Dyes This article reviews the data involving the risk of medical radiation exposure and discusses strategies for managing the risk. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029273 Tue, 05 Oct 2010 05:24:08 +0100 4029273 http://www.medworm.com/index.php?rid=4029272&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000375%2Fabstract%3Frss%3Dyes Of all the interventions available to aid recovery of the injured child, few have the power of proper nutritional support. Healing after trauma depends not only on restoration of oxygen delivery, but on “substrate delivery,” or provision of calories to support metabolic power and specific nutrients to allow rebuilding of injured tissue. Failure to deliver adequate substrate to the cells is revealed as another form of shock. Nutritional interventions after trauma are most effective when informed by the specific ways that children diverge physiologically (metabolic rate, biomechanics, physiological response to trauma) from adults. This review describes these responses and outlines a general strategy for safely delivering energy and specific substrates to protect and heal injured children... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029272 Tue, 05 Oct 2010 05:24:08 +0100 4029272 http://www.medworm.com/index.php?rid=4029271&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000430%2Fabstract%3Frss%3Dyes Since mortality statistics were first systematically recorded in the United States, trauma has remained the leading cause of death in the pediatric population. For children younger than 19 years of age, trauma is responsible for more than one-third of all deaths. We have seen the evolution in the accreditation of pediatric trauma centers, the establishment of the Emergency Medical Services for Children Act, and the development of computed tomography; yet, the rate of mortality is largely unchanged. Gene therapy has emerged as a reality, fetuses have successfully undergone surgery in utero, and vaccinations for diarrheal illnesses have cured hundreds of thousands, and trauma remains the leading cause of death in the pediatric population. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029271 Tue, 05 Oct 2010 05:24:07 +0100 4029271 http://www.medworm.com/index.php?rid=4029270&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000491%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=4029270 Tue, 05 Oct 2010 05:24:07 +0100 4029270 http://www.medworm.com/index.php?rid=3697897&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000211%2Fabstract%3Frss%3Dyes For many years karyotyping has been a successful tool to identify chromosome aberrations in congenital malformations. It has proven to be a highly reliable technique for identifying numerical chromosome changes and structural chromosomal rearrangements, such as deletions, duplications, translocations, or inversions. However, karyotyping has limited resolution of 5-10 Mb and depends on the availability of metaphases. In the last decade, we have experienced the implementation of molecular cytogenetic techniques, resulting in the identification of chromosomal aberrations smaller than 5 Mbp. Because DNA is used as starting material also analysis of paraffin or frozen biopsies is possible. Several genes and loci have been identified, and careful genotype phenotype correlation has lead to the re... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697897 Sat, 26 Jun 2010 06:25:06 +0100 3697897 http://www.medworm.com/index.php?rid=3697896&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861000020X%2Fabstract%3Frss%3Dyes Cancer is a disease whose progression is driven by a series of accumulating genetic and epigenetic changes influenced by hereditary factors and the somatic environment. These changes result in individual cells acquiring a phenotype that provides those cells with a survival advantage over surrounding normal cells. Our understanding of the processes that occur in malignant transformation is increasing, with many discoveries in cancer cell biology having been made through the study of childhood tumors. The processes involved in oncogenesis and cancer progression will be discussed in this review. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697896 Sat, 26 Jun 2010 06:25:06 +0100 3697896 http://www.medworm.com/index.php?rid=3697895&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000235%2Fabstract%3Frss%3Dyes is a very common anomaly of the male genitalia, affecting 2%-4% of male infants and is more common in premature infants. There are two separate stages of testicular descent. The first stage occurs at 8-15 weeks' gestation in the human fetus and is characterized by enlargement of the genito-inguinal ligament, or gubernaculum, and regression of the cranial suspensory ligament. The testis remains close to the future inguinal region as the fetal abdomen grows. Leydig cells in the testis produce insulin-like hormone 3, which stimulates the caudal gubernaculum to grow and become thicker. Mullerian inhibiting substance may have a role in the first phase of descent by stimulating the swelling reaction in the gubernaculum. The second phase of testicular descent requires migration of the gubernacul... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697895 Sat, 26 Jun 2010 06:25:05 +0100 3697895 http://www.medworm.com/index.php?rid=3697894&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000193%2Fabstract%3Frss%3Dyes Ventral body wall defects include ectopia cordis, bladder exstrophy, and the abdominal wall malformations gastroschisis and omphalocele. The etiology of ectopia cordis, gastroschisis, and bladder exstrophy is not known, but they may be linked to abnormalities in the lateral body wall folds responsible for closing the thoracic, abdominal, and pelvic portions of the ventral body wall. These folds form in the fourth week (postfertilization) of development as a combination of the parietal layer of lateral plate mesoderm and overlying ectoderm and must move ventrally to meet in the midline. There are differential rates of cell proliferation in the folds and asymmetries in their movement that may be involved in teratogenic effects of toxic factors. Also, the fusion process between the folds is c... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697894 Sat, 26 Jun 2010 06:25:05 +0100 3697894 http://www.medworm.com/index.php?rid=3697893&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000181%2Fabstract%3Frss%3Dyes Today, the normal and abnormal development of the hindgut is still a matter of speculation. However, as the result of recent studies in appropriate animal models, most embryologic events that finally lead to abnormal hindgut development are better known than in the past: (1) the process of maldevelopment starts in early embryonic stages; (2) the cloacal membrane is always too short in its dorsal part, thus, the dorsal cloaca is missing; and (3) as a result, the hindgut remains attached to the sinus urogenitalis, forming the recto-urethral fistula. In the past, an impaired process of septation was believed to be the main cause of abnormal hindgut development. In contrast to this, our results indicate that the development of the septum is more passive than active. Furthermore, the results of... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697893 Sat, 26 Jun 2010 06:25:05 +0100 3697893 http://www.medworm.com/index.php?rid=3697892&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585861000017X%2Fabstract%3Frss%3Dyes (HSCR) is characterized by absence of the enteric nervous system in a variable portion of the distal gut. Affected infants usually present in the days after birth with bowel obstruction. Despite surgical advances, long-term outcomes remain variable. In the last 2 decades, great advances have been made in understanding the genes and molecular biological mechanisms that underlie the disease. In addition, our understanding of normal enteric nervous system development and how motility develops in the developing fetus and infant has also increased. This review aims to draw these strands together to explain the developmental and biological basis of HSCR, and how this knowledge may be used in the future to aid children with HSCR. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697892 Sat, 26 Jun 2010 06:25:05 +0100 3697892 http://www.medworm.com/index.php?rid=3697891&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000223%2Fabstract%3Frss%3Dyes Congenital malformations of the esophagus are frequently encountered by pediatric surgeons, especially esophageal atresia with or without tracheo-esophageal fistula. However, the molecular mechanisms underlying the development of these various anomalies are not clear. Here we present a brief overview of the embryologic development of the tracheoesophageal tube, along with some of the genetic controls which, when defective can lead to abnormal separation of this tube. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697891 Sat, 26 Jun 2010 06:25:05 +0100 3697891 http://www.medworm.com/index.php?rid=3697890&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000144%2Fabstract%3Frss%3Dyes (CDH) is a congenital anomaly consisting of a posterolateral defect in the diaphragm also known as a Bochdalek hernia. It occurs in 1 in 2000 to 3000 newborns and is associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care and the new postnatal treatment strategies, many newborns with CDH continue to have high rates of mortality and morbidity as the result of severe respiratory failure secondary to PH and PPH. The pathogenesis of CDH and associated PH and PPH is poorly understood. Herein, we aim to review diaphragm and pulmonary development and correlate this to the abnormalities found in CDH. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697890 Sat, 26 Jun 2010 06:25:05 +0100 3697890 http://www.medworm.com/index.php?rid=3697889&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000168%2Fabstract%3Frss%3Dyes The objective of this review is to briefly review normal lung development and to analyze the known molecular mechanisms underlying those diseases. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697889 Sat, 26 Jun 2010 06:25:05 +0100 3697889 http://www.medworm.com/index.php?rid=3697888&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000156%2Fabstract%3Frss%3Dyes Even in ancient cultures, such as in China and Egypt, major congenital anomalies have always intrigued people. With the evolution of anatomy and later on embryology, scientists and more recently pediatric surgeons have tried to “comprehend” major congenital anomalies as experiments of nature in need for clinical care. This attitude brings pediatric surgery in a pivotal role for understanding these anomalies together with developmental biology, teratology, and clinical and molecular genetics. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697888 Sat, 26 Jun 2010 06:25:05 +0100 3697888 http://www.medworm.com/index.php?rid=3697887&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000296%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697887 Sat, 26 Jun 2010 06:25:05 +0100 3697887 http://www.medworm.com/index.php?rid=3697886&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000284%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3697886 Sat, 26 Jun 2010 06:25:05 +0100 3697886 http://www.medworm.com/index.php?rid=3388074&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585860900081X%2Fabstract%3Frss%3Dyes Anorectal malformations (ARMs) and Hirschsprung disease (HD) are the most common congenital colorectal defects in the newborn. The outcomes of HD and ARMs have improved significantly because of improved understanding of the pathologic anatomy and physiology of these defects and of the modern surgical techniques. Still, many patients suffer from defective bowel control even as adults. Some of these also have problems with urinary control and sexual functions. The functional problems are more pronounced in patients with ARMs. Compared with healthy people, both patients with ARMs and those with HD have limitations in their quality of life. Inferior quality of life is more common in patients with ARMs. There are very few published data on long-term outcome of adults with ARMs and HD. The effec... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388074 Mon, 22 Mar 2010 15:21:00 +0100 3388074 http://www.medworm.com/index.php?rid=3388073&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000808%2Fabstract%3Frss%3Dyes We describe the rationale and key aspects for success of a bowel management program for the treatment of pediatric fecal incontinence. A retrospective review was done of the medical strategies used for the treatment of over a 1000 patients with fecal incontinent, combining the experience of the authors' previously published reports (Peña A, Guardino K, Tovilla JM, et al. J Pediatr Surg 1998; 33:133-137 and Bischoff A, Levitt MA, Bauer C, et al. J Pediatr Surg 2009; 44:1278-1284) and additional treated patients after those publications. Emphasis was placed on the review of the key factors needed to achieve success. Through the years, we have learned important lessons that resulted in our current strategy which allows us to obtain better results than earlier in our series. At present, the k... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388073 Mon, 22 Mar 2010 15:21:00 +0100 3388073 http://www.medworm.com/index.php?rid=3388072&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000730%2Fabstract%3Frss%3Dyes Ideally, after operative management of Hirschsprung disease, a child should thrive, avoid recurrent episodes of abdominal distention and enterocolitis, and be fecally continent. However, there is a small group of patients that do not do well after their pull-through procedure. The purpose of this article is to describe our algorithm for the work-up and management of the post pull-through patient with Hirschsprung disease who is not doing well. These children can be categorized into 2 distinct groups: (1) those who are soiling, and (2) those who suffer from distention and enterocolitis. Both of these patient types can be systematically treated with a combination of bowel management, dietary changes, and laxatives, and, potentially, a redo operation, with the goal of having a clean, and happ... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388072 Mon, 22 Mar 2010 15:21:00 +0100 3388072 http://www.medworm.com/index.php?rid=3388071&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000791%2Fabstract%3Frss%3Dyes Children with anorectal malformations (ARMs) constitute a significant group within a pediatric surgery practice. In females, the most common ARM is an imperforate anus with a rectovestibular fistula, followed by an imperforate anus with a rectoperineal fistula and then cloacal anomalies. Some malformations, such as an imperforate anus with a rectovestibular fistula, may seem straightforward, treated with a posterior sagittal anorectoplasty; however, it is vital to recognize the association with gynecologic anomalies. Girls with the most complex anorectal defect, cloacal malformation, have significant associated urological and gynecologic anomalies, which should be recognized and treated. An opportunity to diagnose and treat such anomalies may be missed in the newborn period or at the defin... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388071 Mon, 22 Mar 2010 15:20:59 +0100 3388071 http://www.medworm.com/index.php?rid=3388070&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000729%2Fabstract%3Frss%3Dyes In this review we describe lessons learned from the authors' series of patients born with the most complex of congenital anorectal problems, cloacal malformations, with the hope to convey the improved understanding and surgical treatment of the condition's wide spectrum of complexity learned from patients cared for over the last 25 years. This includes a series of 490 patients, 397 of whom underwent primary operations, and 93 who underwent reoperations after attempted repairs at other institutions. With regard to the newborn, we have learned that the clinician must make an accurate neonatal diagnosis, drain a hydrocolpos when present, and create an adequate, totally diverting colostomy, leaving enough distal colon available for the pull-through, and a vaginal replacement if needed. A corre... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388070 Mon, 22 Mar 2010 15:20:59 +0100 3388070 http://www.medworm.com/index.php?rid=3388069&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000778%2Fabstract%3Frss%3Dyes The management of patients with colorectal disease in the pediatric population is challenging. Such management is all the more challenging when facing the constraints imposed by an environment with limited clinical resources. Three types of colorectal problems are highlighted in this article: anorectal malformations, Hirschsprung's disease, and acquired rectovaginal fistula in the human immunodeficiency virus–positive population. Through the use of illustrative cases, the authors discuss the pitfalls and challenges encountered in the diagnosis, treatment, and appropriate disposition of these patients. The bulk of the experience used to write this article was acquired in low- and middle-income countries in Africa. The authors hope that the lessons learned will help others manage such pati... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388069 Mon, 22 Mar 2010 15:20:59 +0100 3388069 http://www.medworm.com/index.php?rid=3388068&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000754%2Fabstract%3Frss%3Dyes Hirschsprung disease (HD) and anorectal malformations (ARMs) result from alterations in hindgut development. It has long been recognized that both recur in families and thus result, at least in part, from genetic factors. Progress in the understanding of the genetic basis of HD has been made by the application of findings from genetic animal models of altered enteric nervous system development to human beings. Several genes have been shown to be important for human enteric nervous system development, and current work is progressing to identify genetic interactions that may explain the variable phenotype of HD. By contrast, understanding of the genetic factors underlying ARMs is much less developed. We and others have shown that genetic factors play an important role in the pathogenesis of ... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388068 Mon, 22 Mar 2010 15:20:58 +0100 3388068 http://www.medworm.com/index.php?rid=3388067&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000766%2Fabstract%3Frss%3Dyes This article will review the technical principles of the transanal endorectal pull-through, and will address ongoing controversies in the application of this technique. We will also discuss an organized approach to the problem of obstructive symptoms that may affect a subgroup of patients after the transanal pull-through. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388067 Mon, 22 Mar 2010 15:20:58 +0100 3388067 http://www.medworm.com/index.php?rid=3388066&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585860900078X%2Fabstract%3Frss%3Dyes The operative management of pediatric colorectal diseases has improved significantly in recent years through the development of innovative approaches for operative exposure and a better understanding of colorectal anatomy. Advances in transanal and minimal access techniques have formed the cornerstone of this innovation, leading to improved functional outcomes, earlier recovery, and superior cosmetic results for a number of colorectal diseases. In this regard, we have witnessed a significant evolution in the way that many of these conditions are managed, particularly in the areas of anorectal malformations and Hirschsprung disease. Furthermore, a more thorough understanding of the pathophysiology underlying encopresis and true fecal continence has led to novel and less invasive approaches ... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388066 Mon, 22 Mar 2010 15:20:58 +0100 3388066 http://www.medworm.com/index.php?rid=3388065&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000742%2Fabstract%3Frss%3Dyes This article reviews recent advances made in the investigation of children with colorectal motility disorders, including the role of transit studies (marker studies and scintigraphy), options for assessing anatomy (ultrasound, contrast enema, and sectional imaging) and the use of manometry, both anorectal and colonic. Current concepts in microscopic evaluation are outlined. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388065 Mon, 22 Mar 2010 15:20:58 +0100 3388065 http://www.medworm.com/index.php?rid=3388064&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000717%2Fabstract%3Frss%3Dyes In Marcus Pfister's children's book, The Rainbow Fish, the main character is a fish whose scales incorporate all colors of the rainbow. The fish is bright and beautiful, and admired by all other fish in the ocean whose scales have no color at all. One day, the rainbow fish is approached by one of those fish who asks him for a rainbow-colored scale. Willingly, the rainbow fish gives one of his colors away. Word spreads among the other fish in the ocean, and they each approach the rainbow fish and ask to receive one of his scales. The rainbow fish obliges. Eventually, he gives away all of his colored scales, and the ocean is full of beautiful fish of all colors and shades. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388064 Mon, 22 Mar 2010 15:20:58 +0100 3388064 http://www.medworm.com/index.php?rid=3388063&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000120%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388063 Mon, 22 Mar 2010 15:20:58 +0100 3388063 http://www.medworm.com/index.php?rid=3388062&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858610000053%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3388062 Mon, 22 Mar 2010 15:20:58 +0100 3388062 http://www.medworm.com/index.php?rid=3131933&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000699%2Fabstract%3Frss%3Dyes The multivisceral liver-intestine-pancreas-stomach allograft was first described by Starzl nearly 50 years ago. Since then, over 1000 children have received small bowel transplantation (SBTx), alone or with the liver and other organs, for refractory short gut syndrome (SGS) because of a variety of congenital conditions. In 2001, SBTx was approved as definitive therapy for SGS by Medicare. Currently, 1- and 5-year graft survival routinely exceeds 90% and 80%, respectively. The expected outcomes also include freedom from parenteral nutrition, normalization of growth parameters, and quality of life. However, recurrent rejection, complications of high-dose immunosuppression, or chronic rejection, which is more likely to occur after SBTx without a liver graft, account for differences between ea... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131933 Thu, 31 Dec 2009 14:54:02 +0100 3131933 http://www.medworm.com/index.php?rid=3131932&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000675%2Fabstract%3Frss%3Dyes Multidisciplinary management of intestinal failure has progressed over the last 30 years, facilitating the transition to enteral autonomy in many pediatric patients. However, there remains a select group of patients who reach a plateau in advancement of their enteral nutrition. Numerous surgical options have been pursued to attempt to slow intestinal transit, taper dilated bowel, and promote intestinal adaptation. The purpose of this chapter is to review the current literature on autologous intestinal reconstruction surgery, including a brief historical perspective, descriptions of procedures, and reported surgical outcomes. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131932 Thu, 31 Dec 2009 14:54:02 +0100 3131932 http://www.medworm.com/index.php?rid=3131931&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000663%2Fabstract%3Frss%3Dyes Gastrointestinal motility disorders in their most severe forms may directly lead to intestinal failure. Abnormal motor function may also contribute to the overall gut dysfunction of children who have other underlying gastrointestinal diseases, such as Hirschsprung disease or gastroschisis. Understanding the extent and the severity of the dysmotile segments has direct therapeutic and prognostic implications. Our ability to study gastrointestinal motility has greatly improved in the past few years, with the development of less-invasive diagnostic tests. Optimal treatment of children with intestinal motility disorders relies on a multidisciplinary approach, which focuses on optimizing nutrition, improving gastrointestinal motility, and reducing psychosocial disability. Patient education is im... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131931 Thu, 31 Dec 2009 14:54:02 +0100 3131931 http://www.medworm.com/index.php?rid=3131930&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000651%2Fabstract%3Frss%3Dyes This article reviews our evolving understanding of the role of glucagon-like peptide 2 (GLP-2) in controlling the adaptive process. The ability of the remnant intestine to produce GLP-2 appears to be predictive of the adaptive process; exogenous GLP-2 may be a therapy to augment adaptation. Strategies for monitoring patients, including conventional means, such as anthropomorphic measurements, plasma levels of specific nutrients, and vitamins and radiological contrast studies are reviewed. Investigational methods, such as nutrient balance studies, plasma citrulline levels, and the absorption of inert sugars (3-0 methyl glucose, mannitol, and lactulose) are discussed with the evidence to support their use. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131930 Thu, 31 Dec 2009 14:54:02 +0100 3131930 http://www.medworm.com/index.php?rid=3131929&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000705%2Fabstract%3Frss%3Dyes The structural and functional changes during intestinal adaptation are necessary to compensate for the sudden loss of digestive and absorptive capacity after massive intestinal resection. When the adaptive response is inadequate, short bowel syndrome (SBS) ensues and patients are left with the requirement for parenteral nutrition and its associated morbidities. Several hormones have been studied as potential enhancers of the adaptation process. The effects of growth hormone, insulin-like growth factor-1, epidermal growth factor, and glucagon-like peptide 2 on adaptation have been studied extensively in animal models. In addition, growth hormone and glucagon-like peptide 2 have shown promise for the treatment of SBS in clinical trials in human beings. Several lesser studied hormones, includ... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131929 Thu, 31 Dec 2009 14:54:02 +0100 3131929 http://www.medworm.com/index.php?rid=3131928&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585860900064X%2Fabstract%3Frss%3Dyes Children with intestinal failure (IF) suffer from insufficient intestinal length or function, making them dependent on parenteral nutrition (PN) for growth and survival. PN and its components are associated with many complications ranging from simple electrolyte abnormalities to life-threatening PN-associated liver disease, which is also called intestinal failure-associated liver disease (IFALD). From a nutrition perspective, the ultimate goal is to provide adequate caloric requirements and make the transition from PN to full enteral nutrition (EN) successful. Upon review of the literature, we have summarized the most effective and innovative PN and EN therapies for this patient population. Antibiotic-coated catheters and antibiotic or ethanol locks can be implemented, as they appear effec... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131928 Thu, 31 Dec 2009 14:54:02 +0100 3131928 http://www.medworm.com/index.php?rid=3131927&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000638%2Fabstract%3Frss%3Dyes The outcome for children with congenital enteropathies or massive surgical resections has improved significantly over the past two decades. Advances in understanding of the pathophysiology of intractable diarrhea and of the mutations causing many of the congenital enteropathies have enabled initiation of preventive measures for intractable diarrhea, and have enabled clinicians to provide focused treatment of immune-mediated congenital diarrheal illnesses. Children with surgical short bowel syndrome also face an improved outcome because of improvements in the composition of parenteral nutrition (TPN) and in enteral alimentation strategies. It is now recognized that, through adaptation, small intestinal surface area and absorptive function may improve over time to facilitate emancipation fro... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131927 Thu, 31 Dec 2009 14:54:01 +0100 3131927 http://www.medworm.com/index.php?rid=3131926&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000626%2Fabstract%3Frss%3Dyes The management of the child with intestinal failure is complex, and it is developing into a multispecialty field of its own led by expert teams of both transplant and nontransplant surgeons, gastroenterologists, and dieticians. Patients are at risk for medical, surgical, and nutritional complications that should be anticipated so that they can be prevented or managed appropriately. Catheter associated infections and intestinal failure associated liver diseases are important complications that impact the likelihood of bowel adaptation and long-term survival. The clinical assessment of a pediatric intestinal failure patient should include evaluation of the child within the context of recognized prognostic factors. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131926 Thu, 31 Dec 2009 14:54:01 +0100 3131926 http://www.medworm.com/index.php?rid=3131925&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000614%2Fabstract%3Frss%3Dyes Pediatric short bowel syndrome (SBS) is most commonly caused by congenital or acquired conditions of the newborn. SBS is associated with an inability of the bowel to adequately absorb water and nutrients in sufficient quantities to meet caloric, fluid, and electrolyte demands, thus necessitating dependence on parenteral nutrition (PN). It is this dependence on PN, that is responsible for the majority of morbidity and mortality associated with SBS, including central venous catheter infections and PN-induced cholestatic liver dysfunction. There are very few estimates of SBS incidence and mortality in the literature. The epidemiology of SBS is reviewed and the limitations of the published literature are discussed. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131925 Thu, 31 Dec 2009 14:54:01 +0100 3131925 http://www.medworm.com/index.php?rid=3131924&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000687%2Fabstract%3Frss%3Dyes The understanding and management of pediatric intestinal failure is undergoing an exciting transformation. Recent innovations in medical and surgical therapy, coupled with the evolution of specialized multidisciplinary intestinal failure treatment centers, have been associated with significant improvements in patient survival. Although intestinal failure remains a major cause of pediatric morbidity and mortality, current research offers reason for continued optimism. This issue of the Seminars in Pediatric Surgery attempts to summarize recent progress in a logical manner, with contributions from groups that are leaders in the treatment and investigation of intestinal failure. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131924 Thu, 31 Dec 2009 14:54:01 +0100 3131924 http://www.medworm.com/index.php?rid=3131923&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000845%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=3131923 Thu, 31 Dec 2009 14:54:01 +0100 3131923 http://www.medworm.com/index.php?rid=2837738&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585860900047X%2Fabstract%3Frss%3Dyes Gastrointestinal motility disorders can develop in neurologically impaired children and those with congenital malformations of the gut. It is characterized by moderate to severe abdominal pain, vomiting, and failure to thrive. Antral dysmotility after fundoplication and increased sympathetic over activity are 2 factors associated with this condition that make it difficult to treat. This paper proposes a management strategy using metoclopramide, celiac plexus blockade, and thoracic splanchnectomy. It reviews our experience with 11 patients. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837738 Mon, 28 Sep 2009 17:21:50 +0100 2837738 http://www.medworm.com/index.php?rid=2837737&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000468%2Fabstract%3Frss%3Dyes Gut motility disorders represent a significant challenge in clinical management with current palliative approaches failing to overcome disease and treatment-related morbidity. The recent progress with stem cells to restore missing or defective elements of the gut neuromusculature offers new hope for potential cure. Focusing on enteric neuropathies such as Hirschsprung's disease, the review discusses the progress that has been made in the sourcing of putative stem cells and the studies into their biology and therapeutic potential. It also explores the practical challenges that must be overcome before stem cell-based therapies can be applied in the clinical arena. Although many obstacles remain, the speed of advancement of the enteric stem cell field suggests that such therapies are on the h... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837737 Mon, 28 Sep 2009 17:21:50 +0100 2837737 http://www.medworm.com/index.php?rid=2837736&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000456%2Fabstract%3Frss%3Dyes Intestinal transplantation (ITx) has now become an accepted form of replacement therapy for intestinal failure (IF) and its complications. Disorders of bowel motility may represent up to 25% of patients on waiting lists for ITx. Some patients with short bowel as a cause for IF may also have dysmotile bowel. Underlying pathology of the intestine is heterogenous and may be due to abnormalities in the nerve supply, intestinal smooth muscle, or may be regarded as idiopathic (chronic intestinal pseudo-obstruction). Outcome after ITx for dysmotility has previously been guarded, but providing the appropriate graft is used and taking into account the functional motility of residual bowel, it should not be different from other indications. The subject is reviewed emphasizing the potential pitfalls ... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837736 Mon, 28 Sep 2009 17:21:50 +0100 2837736 http://www.medworm.com/index.php?rid=2837735&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000481%2Fabstract%3Frss%3Dyes The gastrointestinal tract responds to significant mechanical or functional obstruction by dilatation and hypertrophy of the segment proximal to the obstruction. Excessive dilatation compromises motility, and absorption and is associated with considerable morbidity (intraluminal stasis, sepsis) such that bowel dilatation represents a major liability that predisposes the patient to intestinal failure. The dilated bowel proximal to an obstruction provides valuable autologous material for reconstruction with “tissue appropriate to the part.” Bowel elongation and dilatation are integral to the natural intestinal adaptation response to loss of small bowel and can also be induced through a structured “Bowel Expansion” program. The additional absorptive tissue that is progressively genera... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837735 Mon, 28 Sep 2009 17:21:50 +0100 2837735 http://www.medworm.com/index.php?rid=2837734&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000444%2Fabstract%3Frss%3Dyes Internal anal sphincter (IAS) achalasia is a clinical condition with presentation similar to Hirschsprung's disease, but with the presence of ganglion cells on rectal suction biopsy. The diagnosis is made by anorectal manometry, which demonstrates the absence of the rectosphincteric reflex on rectal balloon inflation. The IAS is regulated by several neurogenic mechanisms, and so its pathogenesis is thought to be multifactorial, including the absence of nitrergic innervations, defective innervation of the neuromuscular junction, and altered distribution of interstitial cells of Cajal. The recommended treatment of choice is posterior IAS myectomy. Recently, however, the use of intrasphincteric botulinum toxin has been used to treat this condition, but further long-term studies are needed to ... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837734 Mon, 28 Sep 2009 17:21:49 +0100 2837734 http://www.medworm.com/index.php?rid=2837733&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000432%2Fabstract%3Frss%3Dyes Intestinal failure associated liver disease (IFALD) is one of the most common and devastating complications in infants with intestinal failure. Although multifactorial, its pathophysiology is clearly related to the administration of parenteral nutrition (PN), with a recent focus on the role of PN lipid emulsions. This paper will review the evidence for the use of omega-3 fatty acid PN lipid emulsions, which are proposed to have efficacy in the treatment of IFALD. Mechanisms explaining their effects will be considered as will future research directions. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837733 Mon, 28 Sep 2009 17:21:49 +0100 2837733 http://www.medworm.com/index.php?rid=2837732&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000420%2Fabstract%3Frss%3Dyes We review the current clinical evaluation and management of the most common esophageal and gastrointestinal motility disorders in children based on the literature and our experience in a pediatric motility center in the United States. The disorders discussed include esophageal achalasia, pre- and post-fundoplication motility disorders, gastroparesis, motility disorders occurring after repair of congenital atresias, motility disorders associated with gastroschisis, chronic intestinal pseudo-obstruction, motility after intestinal transplantation, motility disorders after colonic resection for Hirschsprung's disease, chronic functional constipation, and motility disorders associated with imperforate anus. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837732 Mon, 28 Sep 2009 17:21:49 +0100 2837732 http://www.medworm.com/index.php?rid=2837731&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000419%2Fabstract%3Frss%3Dyes Diagnosis and management of Hirschsprung's disease (HSCR) requires understanding of the malformation's anatomic features and multigenic nature. Rectal biopsies, intraoperative frozen sections, and resection specimens provide invaluable information. Extraction of these data requires thoughtful biopsy technique, adequate histologic sections, histochemistry, and collaboration of surgeon and pathologist. Critical consideration of transition zone anatomy and published studies of “transition zone pull through” indicate that more research is needed to determine how much ganglionic bowel should be resected from HSCR patients. Many HSCR-susceptibility genes have been identified, but mutational analysis has limited practical value unless family history or clinical findings suggest syndromic HSCR... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837731 Mon, 28 Sep 2009 17:21:49 +0100 2837731 http://www.medworm.com/index.php?rid=2837730&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000407%2Fabstract%3Frss%3Dyes Gastrointestinal motility disorders and chronic constipation are common pediatric problems. Symptoms of abdominal discomfort are frequently encountered in the daily practice of pediatricians and pediatric surgeons. Normal peristalsis depends on the interaction between muscles, nerve cells, and tendinous connective tissue of muscularis propria. Malfunction of any of these components results in a motility disorder. Aganglionosis, typically of the left distal colon, is the cause of Hirschsprung disease. Hypoganglionosis constitutes another gastrointestinal motility disorder. In hypoplastic hypoganglionosis, the number of nerve cells and the size of ganglia of the enteric nervous system are reduced, resulting in symptoms similar to aganglionosis. In intestinal neuronal dysplasia type B, submuc... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837730 Mon, 28 Sep 2009 17:21:49 +0100 2837730 http://www.medworm.com/index.php?rid=2837729&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000390%2Fabstract%3Frss%3Dyes Motility patterns in the mature intestine require the coordinated interaction of enteric neurons, gastrointestinal smooth muscle, and interstitial cells of Cajal. In Hirschsprung's disease, the aganglionic segment causes functional obstruction, and thus the enteric nervous system (ENS) is essential for gastrointestinal motility after birth. Here we review the development of the ENS. We then focus on motility patterns in the small intestine and colon of fetal mice and larval zebrafish, where recent studies have shown that the first intestinal motility patterns are not neurally mediated. Finally, we review the development of gastrointestinal motility in humans. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837729 Mon, 28 Sep 2009 17:21:49 +0100 2837729 http://www.medworm.com/index.php?rid=2837728&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000493%2Fabstract%3Frss%3Dyes Two years ago, I corresponded with Professor Jay Grosfeld about the lack of evidence-based medicine concerning gastrointestinal motility disorders. In a prompt reply, he described it as one of the “black box” areas in surgery and how little is known about this spectrum of disorders. A lot of experimental work has unravelled the mysteries of the “enteric nervous system (ENS),” and there is a growing interest in the management of these disorders by pediatricians, gastroenterologists, and neurogastroenterologists that seems very far removed from the realms of the general pediatric surgeon. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837728 Mon, 28 Sep 2009 17:21:49 +0100 2837728 http://www.medworm.com/index.php?rid=2837727&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000535%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837727 Mon, 28 Sep 2009 17:21:49 +0100 2837727 http://www.medworm.com/index.php?rid=2837726&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000523%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2837726 Mon, 28 Sep 2009 17:21:49 +0100 2837726 http://www.medworm.com/index.php?rid=2624086&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585860900033X%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2624086 Wed, 22 Jul 2009 11:28:45 +0100 2624086 http://www.medworm.com/index.php?rid=2558566&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000298%2Fabstract%3Frss%3Dyes Performance of bariatric surgery in pediatric patients carries profound ethical burdens for all stakeholders: morbidly obese children and adolescents, their parents and families, pediatric physicians and surgeons, pediatric health care institutions, and society. The decision to proceed with a bariatric intervention should be made only after it is established that the patient's comorbidities could not be treated with less invasive means, the patient has a favorable risk/benefit profile, the patient and her/his family have received extensive preoperative counseling and given informed consent, and the pediatric bariatric team has a comprehensive system of short- and long-term care. The patient and her/his family should be counseled about the innovative aspects of the bariatric intervention, i... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558566 Wed, 01 Jul 2009 15:51:32 +0100 2558566 http://www.medworm.com/index.php?rid=2558565&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000286%2Fabstract%3Frss%3Dyes This article discusses the problems obesity presents to children and their families, highlights the unique aspects of treating obesity in children, reviews the currently utilized bariatric surgical procedures, and introduces those bariatric procedures that are under development. When considering whether to use bariatric surgical procedures in a multidisciplinary weight management program for children, the special needs and characteristics of children with a severe weight problem must be considered. Development of bariatric surgical techniques and devices and implementation of these tools in multidisciplinary weight management programs need greater attention. This will require the combined efforts of the pediatric health care providers from many specialties and partnerships with industry to... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558565 Wed, 01 Jul 2009 15:51:32 +0100 2558565 http://www.medworm.com/index.php?rid=2558564&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000274%2Fabstract%3Frss%3Dyes The global epidemic of obesity has not spared children. Although prevention of obesity is commendable, we cannot hide from the pressing need to identify, assess, and actively manage children seriously afflicted by obesity and its associated conditions. Sustained weight loss (or, for children, lowering of body mass index standard deviation score) delivers major health benefit, but in children has been difficult to achieve. In adults, the success of the diabetes prevention programs using practical lifestyle interventions is indisputable. Medical therapy, although currently limited in it scope, provides some promise for older children. There is now accumulating evidence, generally of poor quality that surgical interventions (laparoscopic adjustable gastric banding and Roux-en-Y gastric bypass... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558564 Wed, 01 Jul 2009 15:51:32 +0100 2558564 http://www.medworm.com/index.php?rid=2558563&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000262%2Fabstract%3Frss%3Dyes This article reviews recommendations on assessment of BMI, nutrition and activity, and obesity-related comorbidities. Principles of obesity prevention and treatment are discussed and high-risk eating and activity behaviors are addressed. Prevention and treatment of obesity are reviewed as set in the context of the Medical Home using the principles of the chronic disease model. The stepwise approach to obesity treatment and prevention is reviewed along with specific evidence-based/informed strategies. The skills needed to implement the recommendations, such as integration of the care team, roles and training, links to the health care system, connection to the community, and the role of the subspecialist, are discussed. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558563 Wed, 01 Jul 2009 15:51:31 +0100 2558563 http://www.medworm.com/index.php?rid=2558562&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000250%2Fabstract%3Frss%3Dyes The prevalent surge in childhood and adolescent obesity within the past 3 decades poses a significant challenge for many pediatric clinicians who are charged with treating this condition. Comprehensive, research-based pediatric obesity treatment programs are essential to addressing this problem and preventing the transition of obesity and its many comorbidities into adulthood. This paper will identify dietary, physical activity, and behavioral approaches to lifestyle change and describe how they are incorporated as part of multidisciplinary treatment interventions in youth. Specific tailoring of treatment programs to address age and varying degrees of overweight and obesity will also be presented along with recommendations for future research. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558562 Wed, 01 Jul 2009 15:51:31 +0100 2558562 http://www.medworm.com/index.php?rid=2558561&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000249%2Fabstract%3Frss%3Dyes This review focuses on the diagnosis, risk factors, prevalence, pathogenesis and treatment of pediatric nonalcoholic steatohepatitis (NASH). NASH is a progressive form of nonalcoholic fatty liver disease (NAFLD), the most common cause of chronic liver disease in children. The factors that account for differences between children with NASH and children with milder forms of NAFLD are unclear. The diagnosis of NASH requires interpretation of liver histology because no noninvasive markers predict the presence or severity of NASH. There is no proven treatment for NASH. Several clinical trials for NAFLD are in progress; however, clinical trials focusing on NASH are needed. Heightened physician awareness of NAFLD, NASH, and associated risk factors is important to identify and treat affected child... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558561 Wed, 01 Jul 2009 15:51:31 +0100 2558561 http://www.medworm.com/index.php?rid=2558560&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000237%2Fabstract%3Frss%3Dyes The clustering of traditional cardiovascular disease risk factors is known as the metabolic syndrome. The metabolic syndrome was first characterized as a distinct entity by Dr. Gerald Reaven in 1988. The intent was to identify individuals at greatest risk for cardiovascular disease mortality and those in urgent need of lifestyle intervention. Since then the metabolic syndrome has evolved into a diagnosable entity recognized by the National Cholesterol Education Program, Adult Treatment Panel III, World Health Organization, and the International Diabetes Foundation. However, the metabolic syndrome as a diagnosis faces considerable controversy, particularly when applied to the pediatric population. Due to the changes in growth and development, the adult criteria for the metabolic syndrome ca... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558560 Wed, 01 Jul 2009 15:51:31 +0100 2558560 http://www.medworm.com/index.php?rid=2558559&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000225%2Fabstract%3Frss%3Dyes Obesity has reached epidemic proportions in the USA with a nearly fourfold rise in the prevalence of childhood obesity. There are many possible etiologies of obesity as the adipose tissue plays a significant, complex role in the physiology of fuel metabolism and hormone regulation. The development of obesity represents a pathophysiologic increase in fat mass in which multiple metabolic pathways are deranged. The consequences of these metabolic derangements, including insulin resistance and inflammation, are reflected in obesity-related comorbidities and can be seen in the setting of pediatric obesity. Obese adolescents demonstrate increased rates of early maturation, orthopedic growth abnormalities, diabetes mellitus, obstructive sleep apnea, hypertension, steatosis, and polycystic ovarian... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558559 Wed, 01 Jul 2009 15:51:31 +0100 2558559 http://www.medworm.com/index.php?rid=2558558&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000213%2Fabstract%3Frss%3Dyes Although the overall health status of the pediatric population in the United States has improved, the last quarter century has witnessed a dramatic rise in the prevalence of childhood obesity and its associated comorbid conditions. Specifically, the medical community is witnessing a steady and alarming rise in the prevalence of chronic diseases among obese children, including diabetes, hypertension, obstructive sleep apnea, hyperlipidemia, and cardiovascular disease. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558558 Wed, 01 Jul 2009 15:51:31 +0100 2558558 http://www.medworm.com/index.php?rid=2558557&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000328%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2558557 Wed, 01 Jul 2009 15:51:31 +0100 2558557 http://www.medworm.com/index.php?rid=2481750&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000146%2Fabstract%3Frss%3Dyes Adverse medical events occurring in the context of care of children undergoing elective surgical procedures are usually truly unexpected occurrences with the potential for long-term consequences. Such events, when they occur, are obviously devastating to the patient and family, but frequently also to the pediatric surgeon and his/her team. When such events occur, it is important to ensure uninterrupted care of the patient, a full disclosure of events leading to harm, and an ongoing accessibility to the family for disclosure of new information as it becomes available. Finally, it is important that the adverse event be systematically reviewed as a “critical incident,” so that opportunities for practice improvement leading to enhanced patient safety can be realized and compliance with new... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481750 Fri, 01 May 2009 04:00:00 +0100 2481750 http://www.medworm.com/index.php?rid=2481749&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000134%2Fabstract%3Frss%3Dyes We present a newborn infant who was admitted with sepsis and respiratory failure after home delivery. Ultrasound done on day 8 to check for umbilical venous line placement incidentally showed a simple cyst measuring 3.6 × 5.9 × 6.9 cm that was presumed to be of ovarian origin. Percutaneous needle aspiration was atraumatic and revealed serous fluid, with a high estradiol level. Four days later, surgery was indicated for clinical deterioration with suspected hemorrhage into the cyst. We found a midgut volvulus with extensive necrosis secondary to a jejunal duplication cyst. Ovaries were normal and there was no evidence of malrotation. Postoperatively, after discussion with the parents, support was withdrawn and the child died. We should not rely solely on ultrasonographic features and flui... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481749 Fri, 01 May 2009 04:00:00 +0100 2481749 http://www.medworm.com/index.php?rid=2481748&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000122%2Fabstract%3Frss%3Dyes The intraoperative management of a neonate with esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) is a true anesthetic challenge. Pediatric anesthesia textbooks recommend a distal tracheal intubation beyond the fistula and spontaneous ventilation, if possible, until surgical control of the fistula is achieved to minimize gastric distention. A full-term neonate with Trisomy 21 presented with an EATEF and was transferred to the operating theater for repair after appropriate evaluation. After induction of anesthesia, a size 3.0 endotracheal tube was inserted orally with confirmation of its position by good air entry and chest movement bilaterally. After positioning for thoracotomy, the patient desaturated and became bradycardic with abdominal distention. Despite reintubation,... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481748 Fri, 01 May 2009 04:00:00 +0100 2481748 http://www.medworm.com/index.php?rid=2481747&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000110%2Fabstract%3Frss%3Dyes We present a patient who became paraplegic after resection of a chest wall tumor. After neoadjuvant radio- and chemotherapy, a 10-year-old girl with primitive neuroectodermal tumor (PNET) of the right chest underwent a right chest wall resection. Ribs #3, 4, 5, and 6 were resected en masse with a pulmonary wedge resection of right upper and middle lobes. To obtain clear margins, the ribs were disarticulated from the vertebral column. Significant bleeding arose when the fourth rib was detached. Presuming an intercostal vessel bleed, the area was packed with surgicel with resolution of the bleeding. The patient was kept sedated and ventilated in the PICU. The next day, she complained of paresis of her lower extremities. MRI revealed compression of the spinal cord at the T4 level. Emergency d... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481747 Fri, 01 May 2009 04:00:00 +0100 2481747 http://www.medworm.com/index.php?rid=2481746&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000109%2Fabstract%3Frss%3Dyes Use of a spring-loaded silastic silo has been advocated as a means of gentle reduction of the herniated bowel, while avoiding the possible complications of primary closure of gastroschisis. We recently encountered intestinal venous congestion during elective silo reduction of gastroschisis. Two babies with gastroschisis were treated postdelivery with a spring-loaded silo placed under the fascial defect and the eviscerated bowel suspended within the silo. Patient #1 had no bowel matting. On day of life 2, the bowel within the silo was noted to be dusky. The silo was removed, and the bowel was indeed congested, but viable. Complete reduction with a modified Bianchi closure was performed at the bedside. Patient #2 had severe matting of the bowel and did not require intubation for silo placeme... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481746 Fri, 01 May 2009 04:00:00 +0100 2481746 http://www.medworm.com/index.php?rid=2481745&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000092%2Fabstract%3Frss%3Dyes We present two patients who developed delayed fistulisation following esophageal replacement surgery. The first is a 13-year-old child who, at the age of 3 years, underwent a trans-mediastinal colonic esophageal replacement for a refractory corrosive injury followed by a retrosternal reverse gastric tube after an early catastrophic leak. Ten years later, he presented with a history of intermittent chest pain for 6 months. He developed a tension pneumopericardial tamponade caused by a fistula between gastric tube and pericardium. He recovered after sternotomy. The second was born prematurely with type C esophageal atresia and other malformations. After esophageal anastomosis, he developed a refractory stricture that was resected at 10 months. Despite a fundoplication at 4 years, the recurre... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481745 Fri, 01 May 2009 04:00:00 +0100 2481745 http://www.medworm.com/index.php?rid=2481744&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000080%2Fabstract%3Frss%3Dyes Gastric volvulus in infants, children, and adolescents is a rare event. The purpose of this study is to examine this congenital abnormality and its consequences, using cases with significant adverse outcomes. We will specifically discuss issues of diagnosis and treatment of acute gastric volvulus in association with diaphragmatic anomalies. In addition, the different types of gastric volvulus are compared and contrasted, highlighting areas that may allow for early recognition and prevention of complications related to this foregut obstructive process. Three clinical cases are reviewed, all with significant morbidity and/or mortality. These cases are combined with larger case series to arrive at a list of relatively specific clinical and radiologic findings that can alert the clinician to t... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481744 Fri, 01 May 2009 04:00:00 +0100 2481744 http://www.medworm.com/index.php?rid=2481743&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000079%2Fabstract%3Frss%3Dyes Percutaneous endoscopic gastrostomy (PEG) provides for enteral nutrition in children with feeding problems. PEG, however, is not without complications. The present study has a twofold aim: (1) comparing our incidence of major complications after PEG with the incidence in other centers, and (2) identifying risk factors for major complications. All patients receiving a PEG or laparoscopic-assisted PEG (lap PEG) in the period 1992-2008 were reviewed. Primary outcome was the occurrence of major complications, defined as the need for surgery, nonprophylactic antibiotics, or blood transfusion, and procedure-related death. Potential risk factors, eg, age under 1 year, mental retardation, scoliosis, constipation, hepatomegaly, upper abdominal surgery, ventriculoperitoneal shunt, peritoneal dialysi... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481743 Fri, 01 May 2009 04:00:00 +0100 2481743 http://www.medworm.com/index.php?rid=2481742&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000067%2Fabstract%3Frss%3Dyes Gastrostomy tubes are used in the pediatric population when long-term enteral feeding is needed. A common method of placement is percutaneously with endoscopy (PEG, percutaneous endoscopic gastrostomy). Although PEG placement is a straightforward procedure most of the time, it can be associated with a significant rate of minor complications and a smaller but significantly important rate of major complications. Some of these complications may also occur after any type of gastrostomy. We will present representative case studies outlining major complications and discuss how we may be able to prevent them at the time of PEG insertion or during PEG to low-profile button gastrostomy exchange. The proposed guidelines apply to all types of gastrostomies. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481742 Fri, 01 May 2009 04:00:00 +0100 2481742 http://www.medworm.com/index.php?rid=2481741&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000055%2Fabstract%3Frss%3Dyes Erosion of the skin over a totally implanted vascular access device (TIVAD) is a rare event that may lead to life-threatening sequelae. From 1994 to 2007, we reviewed the medical records and central line database of 960 central line insertions for the complication of skin erosion over the TIVAD. Outcome measures included age, gender, and nutritional status, number of days until complication, insertion site, and attending surgeon. A total of 540 of the 960 central lines were TIVAD. Skin erosion occurred in 9 patients for an incidence of 1.67%. Average age at insertion was 51 months (range 25-116.5 months). The average catheter duration use in days was 335 with a range of 39-1575 days. Malnutrition defined as BMI (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481741 Fri, 01 May 2009 04:00:00 +0100 2481741 http://www.medworm.com/index.php?rid=2481740&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000043%2Fabstract%3Frss%3Dyes Discussions on the complications of central venous catheterization in children typically focus on infectious and the more common mechanical complications of pneumothorax, hemothorax, or thrombosis. Rare complications are often more life-threatening, and inexperience may compound the problem. Central venous catheter complications can be broken down into early or late, depending on when they occur. The more serious complications are typically mechanical and occur early, but delayed presentations of pericardial effusions, cardiac tamponade, and pleural effusions may be of equal severity, and delay in diagnosis can be catastrophic. Careful insertion techniques, as well as continued vigilance in the correct position and function of central venous catheters, are imperative to help prevent seriou... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481740 Fri, 01 May 2009 04:00:00 +0100 2481740 http://www.medworm.com/index.php?rid=2481739&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000031%2Fabstract%3Frss%3Dyes This paper presents four severe cardiac injuries that occurred in patients who underwent the minimally invasive repair of pectus excavatum (MIRPE). These complications occurred in different clinical settings, namely in a patient with an extremely severe form of pectus, in a patient who had previously undergone an open repair, after a previous open heart surgery, and at the time of bar removal. The purpose of this article is to review the circumstances leading to these cardiac injuries, share what we have learned from these patients, and hopefully help avoid these complications in the future. (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481739 Fri, 01 May 2009 04:00:00 +0100 2481739 http://www.medworm.com/index.php?rid=2481738&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585860900002X%2Fabstract%3Frss%3Dyes Complications are part of the life as a surgeon, hence the popular saying, “The only surgeons who don't have surgical complications are those who don't operate.” Many complications are unavoidable. For example, a small percentage of wound infections will always occur in patients with perforated appendicitis, despite meticulous technique and the use of properly timed preoperative antibiotics. Those who leave all contaminated wounds open may never have to report a wound infection, but is it worth submitting all children to the discomfort of an open wound and dressing changes to avoid a 5-10% rate of wound infection? Personally, I don't think so, and I accept the fact that I will have to report a certain number of these complications. However, other than these predictable and, to a certai... Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481738 Fri, 01 May 2009 04:00:00 +0100 2481738 http://www.medworm.com/index.php?rid=2481737&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000183%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481737 Fri, 01 May 2009 04:00:00 +0100 2481737 http://www.medworm.com/index.php?rid=2481736&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS1055858609000171%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481736 Fri, 01 May 2009 04:00:00 +0100 2481736 http://www.medworm.com/index.php?rid=2481735&cid=s_33252_33_f&fid=33252&url=http%3A%2F%2Fwww.sempedsurg.org%2Farticle%2FPIIS105585860900016X%2Fabstract%3Frss%3Dyes (Source: Seminars in Pediatric Surgery) Seminars in Pediatric Surgery journals http://www.medworm.com/rss/comments.php?id=2481735 Fri, 01 May 2009 04:00:00 +0100 2481735