MedWorm: Congenital Diaphragmatic Hernia Repair

A review of patch options in the repair of congenital diaphragm defects

Pediatric Surgery International — Wed, 01 Feb 2012 17:10:05 +0100

Abstract Repair of congenital diaphragmatic hernia (CDH) continues to pose a dilemma for the pediatric surgeon. While the management of CDH has evolved from surgical urgency to delayed repair after medical optimization with substantial improvements in mortality, morbidity continues to perplex clinicians. Significant morbidity occurs with recurrence, re-recurrence and complications such as obstructions, principally with mesh repair. When primary closure is not possible, mesh repair is indicated. While there are several non-absorbable prosthetic, absorbable biosynthetic and composite mesh types available, the ideal mesh remains elusive. In this article, we reviewed the current materials, reported advantages, and animal and clinical studies of non-absorbable prosthetic, absor...

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An Evaluation of Medium-term Outcomes of Thoracoscopic Congenital Diaphragmatic Hernia Repair in Neonates and Infants

Journal of Surgical Research — Sat, 14 Jan 2012 22:29:59 +0100

Introduction: Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has become more widespread. Our previous study demonstrated improved short term outcomes with minimally invasive surgical (MIS) repair of CDH, including quicker return to enteral nutrition, shorter duration of narcotic use, and fewer major complications. the current study evaluates medium term outcomes of neonatal thoracoscopic CDH repair compared to open repair. Methods: Institutional review board approval was obtained to analyze data from our neonatal surgical registry to study patients under six months of age who underwent CDH repair from January 2003 to July 2011. Patients with Morgagni's defects, inadequate records, or traumatic diaphragmatic hernias were excluded. Demographic, operative details and follow-up ...

Pharmacokinetics and End Organ Effect of PDE5 Inhibitors in the Fetal Lamb Model of Congenital Diaphragmatic Hernia

Journal of Surgical Research — Sat, 14 Jan 2012 22:28:58 +0100

(Source: Journal of Surgical Research)

Fetal surgery for severe congenital diaphragmatic hernia?

The Ultrasound Review of Obstetrics and Gynecology — Sun, 01 Jan 2012 05:00:00 +0100

Authors: Jani JC, Nicolaides KH PMID: 22213616 [PubMed - in process] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Breastfeeding the infant with congenital diaphragmatic hernia post extracorporeal membrane oxygenation.

Neonatal Network — Sun, 01 Jan 2012 05:00:00 +0100

Authors: Spatz D, Raphael L, Froh EB Abstract Infants born with congenital diaphragmatic hernia (CDH) often require extracorporeal membrane oxygenation (ECMO). Infants on ECMO may experience a long period of being nothing by mouth (NPO) while receiving parenteral nutrition. Once the infant with CDH is repaired and off ECMO, human milk should be used to initiate enteral feedings. Human milk provides immunologic, developmental, and nutritional protection for these highrisk infants and may be crucial in decreasing morbidities commonly associated with post-ECMO survivors. These mother-infant dyads require extensive lactation support to ensure maintenance of milk supply and successful transition to direct breastfeeding. Three case studies are presented as exemplars to demonstrate how br...

The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital diaphragmatic hernia

Journal of Pediatric Surgery — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions: The CDH-CPI accurately stratifies survival in left-sided congenital diaphragmatic hernia. The amalgamation of 10 prenatal parameters of the CDH-CPI may be a better prenatal predictor than any single prognostic variable currently used. (Source: Journal of Pediatric Surgery)

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Split abdominal wall muscle flap repair vs patch repair of large congenital diaphragmatic hernias

Journal of Pediatric Surgery — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions: The split abdominal wall muscle flap allows for closure of large congenital diaphragmatic hernia defects with autologous tissue. This approach is associated with significantly fewer recurrences than patch repairs. (Source: Journal of Pediatric Surgery)

Simplified technique for minimally invasive repair of congenital diaphragmatic hernia using hollow-needle snare and transthoracic traction stitches

Journal of Pediatric Surgery — Sun, 01 Jan 2012 05:00:00 +0100

Conclusion: Hollow-needle snare and TTS are simple and available tools that can facilitate MI repair of CDH. This initial experience demonstrates the technique's effectiveness and its excellent cosmetic outcomes. (Source: Journal of Pediatric Surgery)

Fetal surgery for severe congenital diaphragmatic hernia?

Ultrasound in Obstetrics and Gynecology — Thu, 29 Dec 2011 00:55:52 +0100

(Source: Ultrasound in Obstetrics and Gynecology)

Fetal counselling for surgical conditions

Early Human Development — Mon, 26 Dec 2011 10:48:08 +0100

Abstract: Foetal counselling is best achieved by a multidisciplinary team that can favourably influence the perinatal management of prenatally diagnosed anomalies and provide this information to prospective parents. Prenatal diagnosis has remarkably improved our understanding of surgically correctable congenital malformations. It has allowed us to influence the delivery of the baby, offer prenatal surgical management and discuss the options of termination of pregnancy for seriously handicapping or lethal conditions. Antenatal diagnosis has also defined an in utero mortality for some lesions such as diaphragmatic hernia and sacrococcygeal teratoma so that true outcomes can be measured. The limitation of in-utero diagnosis cannot be ignored. The aim of prenatal counselling is to provide info...

Outcome of fetuses with congenital diaphragmatic hernia and associated intrafetal fluid effusions managed in the era of fetal surgery

Ultrasound in Obstetrics and Gynecology — Sun, 25 Dec 2011 20:40:51 +0100

ConclusionsOur observations do not support the view that intrafetal effusions are an adverse prognostic factor in fetuses with CDH. In CDH fetuses with effusions and severe pulmonary hypoplasia treated with FETO, neonatal survival is similar to that in isolated cases undergoing the intervention. Whether pleural effusions should be addressed by thoracic drainage procedures remains unproven. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

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Use of Endoscopic Surgical Spacer to Improve Safety During Thoracoscopic Repair of Congenital Diaphragmatic Hernia

Journal of Laparoendoscopic — Tue, 13 Dec 2011 20:04:02 +0100

Journal of Laparoendoscopic & Advanced Surgical Techniques , Vol. 0, No. 0. (Source: Journal of Laparoendoscopic)

Perinatal outcome of congenital diaphragmatic hernia in an Australian tertiary hospital

The Australian and New Zealand Journal of Obstetrics and Gynaecology — Tue, 06 Dec 2011 05:00:00 +0100

Conclusions: Although the overall mortality rate for this condition remains high, fetuses with isolated CDH born at term have relatively high survival rates. This study provides data for counselling parents in tertiary centres with advanced neonatal care but where antenatal intervention for this condition has not been introduced. (Source: The Australian and New Zealand Journal of Obstetrics and Gynaecology)

Psychosocial and cognitive consequences of major neonatal surgery

Journal of Pediatric Surgery — Thu, 01 Dec 2011 05:00:00 +0100

Conclusions: To ensure true quality of life after neonatal surgical stress, pediatric surgeons must consider not only physical assessments but also cognitive, emotional, and psychosocial assessments. (Source: Journal of Pediatric Surgery)

Increase in fetal pulmonary artery diameters during late gestation is a predictor of outcome in congenital diaphragmatic hernia with liver herniation

Journal of Pediatric Surgery — Thu, 01 Dec 2011 05:00:00 +0100

Conclusion: PA diameter appears to be correlated with prognosis in infants with CDH + LH. (Source: Journal of Pediatric Surgery)

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Synchronous ipsilateral Bochdalek and Morgagni diaphragmatic hernias: a case report

Journal of Pediatric Surgery — Thu, 01 Dec 2011 05:00:00 +0100

We report a case of a patient who was diagnosed with CDH postnatally and was found on imaging to have simultaneous Bochdalek and Morgagni hernias on the right side. During the operative repair of these defects, an additional left-sided Morgagni-type defect was also found. To the best of our knowledge, this form of CDH has not been previously reported. (Source: Journal of Pediatric Surgery)

Causes of death and autopsy findings in a large study cohort of individuals with Cornelia de Lange syndrome and review of the literature

American Journal of Medical Genetics Part A — Tue, 08 Nov 2011 05:00:00 +0100

AbstractTo identify causes of death (COD) in propositi with Cornelia de Lange syndrome (CdLS) at various ages, and to develop guidelines to improve management and avoid morbidity and mortality, we retrospectively reviewed a total of 426 propositi with confirmed clinical diagnoses of CdLS in our database who died in a 41‐year period between 1966 and 2007. Of these, 295 had an identifiable COD reported to us. Clinical, laboratory, and complete autopsy data were completed on 41, of which 38 were obtainable, an additional 19 had autopsies that only documented the COD, and 45 propositi had surgical, imaging, or terminal event clinical documentation of their COD. Proband ages ranged from fetuses (21–40 weeks gestation) to 61 years. A literature review was undertaken to identify all reported ...

Three-Component Intraperitoneal Mesh Fixation for Laparoscopic Repair of Anterior Parasternal Costodiaphragmatic Hernias

Journal of the American College of Surgeons — Mon, 07 Nov 2011 05:00:00 +0100

Anterior costodiaphragmatic hernias are frequently a consequence of thoracic drain placement via the substernal route after cardiothoracic surgery or sternum resection. These types of hernias are basically incisional hernias. Costodiaphragmatic hernias can also be congenital. One rare form is Morgagni hernia, accounting for 1% to 6% of all congenital diaphragmatic hernias. The localization is para- and retrosternal and it is thought to be due to the incomplete union among the ribs, transverse septum, and sternum. The initial description is ascribed to Giovanni Battista Morgagni (1682−1771), a famous Italian anatomist at the renowned University of Padua, who described this type of diaphragmatic hernia as early as 1761. Some decades later, Dominique Jean Larrey (1766−1842), first medical...

Pulmonary development considerations in the surgical management of congenital diaphragmatic hernia

Early Human Development — Tue, 01 Nov 2011 04:00:00 +0100

This article reviews the evolution of the current treatment strategies in congenital diaphragmatic hernia and its future directions. (Source: Early Human Development)

Postoperative regional distribution of pulmonary ventilation and perfusion in infants with congenital diaphragmatic hernia

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions: The grade of clinical disease severity in infants following CDH repair is closely related to the ventilation-perfusion abnormality as seen using SPECT. The persistence of pulmonary artery hypertension into the postoperative neonatal period appears to be an important pathophysiological factor related to ventilation-perfusion abnormalities. Single photon emission computed tomography provides valuable clinical information for patient follow-up. (Source: Journal of Pediatric Surgery)

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The first male with thoracoschisis: case report and review of the literature

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

Abstract: A male infant presented at birth with intestine and liver herniated through a defect 3 cm below the left nipple on the anterior thoracic wall. Riedel lobe, attached to the left liver lobe, and the transverse colon were seen protruding through the defect at the region of the left eighth intercostal space at surgery. A fibrous band extending from the lower defect border to the bladder was present. The hernia content was reduced inside the abdomen and the fibrous band, and Riedel lobe and necrotic-appearing omentum were excised. Thoracoschisis is a very rare congenital anomaly with only 4 cases reported. This is the first isolated thoracoschisis case without an accompanying diaphragmatic hernia. (Source: Journal of Pediatric Surgery)

Brave new world or the unfortunate natural history of “lethal” disease: when to push the envelope?

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

We recently witnessed a neonatal intensive care unit infant prenatally diagnosed with a congenital diaphragmatic hernia () succumb to tracheal agenesis () at birth. At the same time, we noticed in the news headlines of adults being “saved” by “lab-made tracheas” . This stimulated our curiosity to review recent developments in tissue engineering and stem cell research that may challenge prior authors' claims that tracheal agenesis is “uniformly fatal” . Although prior tracheal reconstructive efforts have not had long-term success , what our review found seems to bring science fiction (Aldous Huxley's Brave New World, 1931) into reality and allows the creation of a new trachea using the child's umbilical cord stem cells. (Source: Journal of Pediatric Surgery)

Letter to the Editor

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

The article in the April issue by Raval et al , “Costs of congenital diaphragmatic hernia in the united states-extra corporeal membrane oxygenation foots the bill,” stated that Dr Robert Gross operated on the first diaphragmatic hernia. (Source: Journal of Pediatric Surgery)

Reply

Journal of Pediatric Surgery — Tue, 01 Nov 2011 04:00:00 +0100

We thank Dr Raffensperger for noting our oversight and providing an accurate historical account of congenital diaphragmatic hernia repairs. As we move forward and study the costs of cutting edge interventions and modern day critical care on society, we acknowledge that it is crucial to understand the road traveled to provide us the luxury of these discussions. Although originally credited to Bernard of Chartres, John of Salisbury, the 12th century theologian, perhaps described it best with a quote appropriate to this discussion: “…we are like dwarfs on the shoulders of giants, so that we can see more than they, and things at a greater distance, not by virtue of any sharpness of sight on our part, or any physical distinction, but because we are carried high and raised up by their giant ...

Intravesical pressure: A new prognostic indicator in congenital diaphragmatic hernia

Journal of Indian Association of Pediatric Surgeons — Mon, 31 Oct 2011 04:00:00 +0100

Conclusion: Measurement of intravesical pressure is a reliable prognostic indicator in newborns with congenital diaphragmatic hernia. It also helps in predicting postoperative ventilatory requirement. (Source: Journal of Indian Association of Pediatric Surgeons)

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Sonographic appearance of intrathoracic kidney in a fetus with left diaphragmatic hernia

Journal of Clinical Ultrasound — Thu, 27 Oct 2011 04:00:00 +0100

We present the sonographic findings in such a case. The postnatal outcome after successful respiratory management and surgical repair was good. © 2011 Wiley Periodicals, Inc. J Clin Ultrasound, 2011 (Source: Journal of Clinical Ultrasound)

Nitrofen interferes with trophoblastic expression of retinol-binding protein and transthyretin during lung morphogenesis in the nitrofen-induced congenital diaphragmatic hernia model

Pediatric Surgery International — Thu, 20 Oct 2011 15:52:57 +0100

Conclusions Decreased trophoblast expression of retinol transport proteins suggest that nitrofen may interfere with the fetal retinol transport resulting in reduced pulmonary RBP and TTR levels and causing pulmonary hypoplasia in CDH. Content Type Journal ArticleCategory Original ArticlePages 1-6DOI 10.1007/s00383-011-2995-0Authors Balazs Kutasy, The National Children’s Research Center, Our Lady’s Children’s Hospital, Dublin, IrelandJan H. Gosemann, The National Children’s Research Center, Our Lady’s Children’s Hospital, Dublin, IrelandTakashi Doi, The National Children’s Research Center, Our Lady’s Children’s Hospital, Dublin, IrelandNaho Fujiwara, The National Children’s Research Center, Our Lady’s Children’s Hospital, Dublin, IrelandFlorian...

The utility of muscle sparing axillar skin crease incision for pediatric thoracic surgery

Pediatric Surgery International — Tue, 18 Oct 2011 15:56:03 +0100

Conclusions MSASCI may become the standard approach for the thoracic surgery for small children. Content Type Journal ArticleCategory Original ArticlePages 1-6DOI 10.1007/s00383-011-3013-2Authors Tomoaki Taguchi, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanKouji Nagata, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanYoshiaki Kinoshita, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanSatoshi Ieiri, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanTatsuro Tajiri, Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, F...

Upregulation of fibroblast growth factor receptor 2 and 3 in the late stages of fetal lung development in the nitrofen rat model

Pediatric Surgery International — Thu, 13 Oct 2011 05:48:19 +0100

Conclusion Upregulation of FGFR2 and FGFR3 pulmonary gene expression in the late stages of fetal lung development may disrupt FGFR-mediated alveologenesis resulting in PH in the CDH model. Content Type Journal ArticleCategory Original ArticlePages 1-5DOI 10.1007/s00383-011-2985-2Authors Florian Friedmacher, National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, IrelandTakashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, IrelandJan-Hendrik Gosemann, National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, IrelandNaho Fujiwara, National Children’s Research Centre, Our Lady’s Children’s Hospital, Crumlin, Dublin 12, IrelandBalazs Kut...

Smad1 and WIF1 genes are downregulated during saccular stage of lung development in the nitrofen rat model

Pediatric Surgery International — Tue, 11 Oct 2011 05:49:13 +0100

Conclusion We provide evidence, for the first time, that the pulmonary gene expression of Smad1 and Wif1 is downregulated on D18 and D21 (saccular stage of lung development) in the nitrofen-induced hypoplastic lung. These findings suggest that the downregulation of Smad1/Wif1 gene expression may contribute to pulmonary hypoplasia in the nitrofen CDH model by retardation of lung development during saccular stage. Content Type Journal ArticleCategory Original ArticlePages 1-5DOI 10.1007/s00383-011-2987-0Authors Naho Fujiwara, National Children’s Research Centre, Our Lady’s Children Hospital, Dublin 12, IrelandTakashi Doi, National Children’s Research Centre, Our Lady’s Children Hospital, Dublin 12, IrelandJan-Hendrik Gosemann, National Children’s Research C...

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[Does the regional health care organization impact the outcome of infants born with congenital diaphragmatic hernia?].

Archives de Pediatrie — Sat, 01 Oct 2011 04:00:00 +0100

CONCLUSION: This study brings to light the diversity of care and outcome for infants affected with CDH in two French perinatal health care networks. These results may help improve both centers' practices. In Lorraine for instance, the follow-up of these vulnerable children can be improved. PMID: 21873038 [PubMed - in process] (Source: Archives de Pediatrie)

[Thoracic congenital ectopic right kidney with diaphragmatic hernia: A rare developmental anomaly].

Archives de Pediatrie — Sat, 01 Oct 2011 04:00:00 +0100

We report the case of a female infant admitted for mild respiratory distress at birth in relation with maternofetal infection in whom a right posterior opacity on chest x-ray was diagnosed as intrathoracic kidney by sonography and chest CT scan. Diaphragmatic hernia could not be confirmed at this stage. At 2 months, the presence of air blebs on chest x-ray performed for viral bronchiolitis confirmed an associated right Bochdalek hernia. The infant, although asymptomatic, underwent surgery. The intrathoracic kidney was reduced into the abdominal cavity at the time of surgery. The postoperative course was uneventful and renal function was normal. PMID: 21865018 [PubMed - in process] (Source: Archives de Pediatrie)

Prenatal risk stratification for isolated congenital diaphragmatic hernia: results of a Japanese multicenter study

Journal of Pediatric Surgery — Sat, 01 Oct 2011 04:00:00 +0100

Abstract: Background/Purpose: The aim of this study was to establish a prenatal prognostic classification system for risk-stratified management in fetuses with isolated congenital diaphragmatic hernia (CDH).Methods: A multi-institutional retrospective cohort study of isolated CDH, diagnosed prenatally in fetuses delivered during the 2002 to 2007 period at 5 participating institutions in Japan, was conducted. The risk stratification system was formulated based on the odds ratios of prenatal parameters for mortality at 90 days. The clinical severity in CDH infants were compared among the stratified risk groups.Results: Patients were classified into the 3 risk groups: group A (n = 48) consisted of infants showing liver-down with contralateral lung–to–thorax transverse area ratio (L/T) rat...

Evaluation of esophageal motility and reflux in children treated for congenital diaphragmatic hernia with the use of combined multichannel intraluminal impedance and pH monitoring

Journal of Pediatric Surgery — Sat, 01 Oct 2011 04:00:00 +0100

Conclusions: With the use of pH-multichannel intraluminal impedance, both GER and esophageal motility in patients with congenital malformations can be analyzed. In patients with CDH, impaired motility seems to involve only the distal esophagus. In this group, the specific pattern of reflux is probably caused by the involvement of gastroesophageal junction, without significant intrinsic innervation abnormalities as observed in patients with esophageal atresia. (Source: Journal of Pediatric Surgery)

Large diaphragmatic defect: are skeletal deformities preventable?

Pediatric Surgery International — Tue, 20 Sep 2011 05:46:23 +0100

Conclusion The differences in some anthropometric parameters (weight, BMI, and TI) and in the skeletal deformity suggest that the CDH not only disturbs normal lung growth, but also seems to have implications on some other aspects of somatic development. Whether these changes could be related to the type of diaphragmatic reconstruction or rather to the size of the defect remains uncertain. Content Type Journal ArticleCategory Original ArticlePages 1-7DOI 10.1007/s00383-011-2973-6Authors P. Kuklová, Department of Pediatric Surgery, 2nd Faculty of Medicine and University Hospital Motol, Charles University in Prague, V úvalu 84, 15000 Prague 5, Czech RepublicD. Zemková, Department of Pediatrics, 2nd Faculty of Medicine and University Hospital Motol, Charles Universit...

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Delayed presentation of strangulated congenital diaphragmatic hernia: learning from our experience

Hernia — Thu, 15 Sep 2011 15:51:40 +0100

Conclusion Clinicians should always consider a diagnosis of obstructed Bochdaleck hernia in children, because X-ray findings are not always typical or even normal in complicated CDH. VATS may be considered as both diagnostic and therapeutic. Preventive measures for empyema or early intervention in the evolving stage can significantly reduce morbidity. Content Type Journal ArticleCategory Case ReportPages 1-5DOI 10.1007/s10029-011-0882-1Authors S. Singh, Department of Pediatric Surgery, CSM Medical University (formerly King George’s Medical University), Lucknow, 226003 IndiaA. Wakhlu, Department of Pediatric Surgery, CSM Medical University (formerly King George’s Medical University), Lucknow, 226003 IndiaA. Pandey, Department of Pediatric Surgery, CSM Medical Uni...

Outcome of fetuses with congenital diaphragmatic hernia and associated intra‐fetal fluid effusions managed in the era of fetal surgery

Ultrasound in Obstetrics and Gynecology — Fri, 09 Sep 2011 04:00:00 +0100

Conclusions:Our observations do not support the view that intra‐fetal effusions are an adverse prognostic factor in fetuses with CDH. In CDH fetuses with effusions and severe pulmonary hypoplasia treated by FETO, neonatal survival is similar as in isolated cases undergoing the intervention. Whether pleural effusions should be addressed by thoracic drainage procedures remains unproven. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Outcome of fetuses with congenital diaphragmatic hernia and associated intra-fetal fluid effusions managed in the era of fetal surgery.

The Ultrasound Review of Obstetrics and Gynecology — Fri, 09 Sep 2011 04:00:00 +0100

CONCLUSIONS: Our observations do not support the view that intra-fetal effusions are an adverse prognostic factor in fetuses with CDH. In CDH fetuses with effusions and severe pulmonary hypoplasia treated by FETO, neonatal survival is similar as in isolated cases undergoing the intervention. Whether pleural effusions should be addressed by thoracic drainage procedures remains unproven. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 21910146 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Omentopexy for the repair of diaphragmatic defect and adhesion assessment of viscera to propylene mesh- an experimental study in rabbits

Indian Journal of Thoracic and Cardiovascular Surgery — Thu, 08 Sep 2011 05:55:18 +0100

Conclusions Omentopexy appears to reduces visceral adhesion to propylene mesh in the repair of diaphragmatic defects, so in intra-abdominal operations where propylene mesh is used, supplemental omentopexy is recommended. Content Type Journal ArticleCategory Original ArticlePages 1-4DOI 10.1007/s12055-011-0115-3Authors Abdolhossein Davoodabadi, Department of General thoracic Surgery, Faculty of Medicine, Kashan University of Medical Sciences, Kashan, IR, IranMehrdad Hosseinpour, Trauma Research Center, Kashan University of Medical Sciences, Kashan, IR, IranNaser Golestani, Department of General thoracic Surgery, Faculty of Medicine, Kashan University of Medical Sciences, Kashan, IR, Iran Journal Indian Journal of Thoracic and Cardiovascular SurgeryOnline ISSN 0...

Conjoined twins pregnancies: experience with 36 cases from a single center

Prenatal Diagnosis — Thu, 08 Sep 2011 04:00:00 +0100

ConclusionConjoined twin pregnancies should be referred to tertiary centers for detailed fetal anomaly and echocardiographic assessment to evaluate prognosis and determine the possibility of postnatal surgical separation. Copyright © 2011 John Wiley & Sons, Ltd. (Source: Prenatal Diagnosis)

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Outcome after fetal intervention

Early Human Development — Thu, 01 Sep 2011 04:00:00 +0100

Advances in ultrasound techniques and screening programs have resulted in timely detection of an increasing number of fetal diseases, some of which may benefit from fetal surgical intervention. The most commonly performed invasive fetal treatments are intrauterine blood transfusion (IUT) for severe fetal anemia and fetoscopic laser coagulation of vascular anastomoses in the placenta of monochorionic twins with twin-to-twin transfusion syndrome (TTTS) . Ultrasound-guided insertion of a thoraco-amniotic shunt is increasingly advocated as treatment in hydropic fetuses with primary hydrothoraces and/or space-occupying congenital lung lesions, such as congenital cystic adenomatoid malformations (CCAM) , and vesico-amniotic shunting is available as intervention in congenital lower urinary tract ...

Neurodevelopmental and neurofunctional outcomes in children with congenital diaphragmatic hernia

Early Human Development — Thu, 01 Sep 2011 04:00:00 +0100

The objective of this review was to provide a critical overview of our current understanding on the neurocognitive, neuromotor, and neurobehavioral development in congenital diaphragmatic hernia (CDH) patients, focusing on three interrelated clinical issues: (1) comprehensive outcome studies, (2) characterization of important predictors of adverse outcome, and (3) the pathophysiological mechanism contributing to neurodevelopmental disabilities in infants with CDH. Improved survival for CDH has led to an increasing focus on longer-term outcomes. Neurodevelopmental dysfunction has been recognized as the most common and potentially most disabling outcome of CDH and its treatment. While increased neuromotor dysfunction is a common problem during infancy, behavioral problems, hearing impairment...

Free fascia lata repair for a second recurrent congenital diaphragmatic hernia

Journal of Pediatric Surgery — Thu, 01 Sep 2011 04:00:00 +0100

We report the successful use of an autologous free fascia lata graft to repair the diaphragm following a second recurrence of CHD. The fascia lata is one of the strongest fascia in the body and is easy to obtain without extremity functional loss even in children. This procedure is regarded as effective for the repair of recurrent CDH. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Thu, 01 Sep 2011 04:00:00 +0100

The purpose of this study was to clarify the reliability of the lung to thorax transverse area ratio (L/T) as a prenatal predictive parameter for patients with congenital diaphragmatic hernia (CDH). A multicenter retrospective cohort study was conducted on 114 isolated CDH fetuses with a prenatal diagnosis during the period between 2002 and 2007 at 5 participating centers in Japan. The relationship between the gestational age and the L/T was analyzed. The most powerful measurement point and accurate cutoff value of the L/T was determined by an analysis of a receiver operating characteristic curve, which was verified by comparing the patients' severity. There was a negative correlation between the gestational age and the L/T in the nonsurvivors, and no correlation in the survivors. There we...

Imperforate anus, diaphragmatic hernia, horseshoe kidney, and pulmonary sling complex: case description

Journal of Pediatric Surgery — Thu, 01 Sep 2011 04:00:00 +0100

This report describes an infant with imperforate anus, delayed presentation of congenital diaphragmatic hernia, horseshoe kidney, and pulmonary sling complex, a unique combination of anomalies that we recently treated at our facility. (Source: Journal of Pediatric Surgery)

COUP-TFII Gene Expression is Upregulated in Embryonic Pleuroperitoneal Folds in the Nitrofen-Induced Congenital Diaphragmatic Hernia Rat Model

European Journal of Pediatric Surgery — Mon, 29 Aug 2011 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1284358Abstract[...]Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

[Does the regional health care organization impact the outcome of infants born with congenital diaphragmatic hernia?]

Archives de Pediatrie — Thu, 25 Aug 2011 23:00:00 +0100

[Thoracic congenital ectopic right kidney with diaphragmatic hernia: A rare developmental anomaly.]

Archives de Pediatrie — Sun, 21 Aug 2011 23:00:00 +0100

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Assessing health-related quality-of-life changes in informal caregivers: an evaluation in parents of children with major congenital anomalies

Quality of Life Research — Fri, 19 Aug 2011 15:57:48 +0100

Conclusions Significant HRQoL differences exist between parents caring for children with congenital anomalies and the general population. It would be useful to further improve our understanding of the HRQoL impact of informal caregiving, separating ‘caregiving effects’ from ‘family effects’, and distinguishing parent–child relationships from other caregiving situations. This study underlines the importance of considering caregivers, also in the context of economic evaluations. It indicates that general HRQoL measures, as used in patients, may be able to detect HRQoL effects in caregivers, which facilitates the incorporation in common economic evaluations of HRQoL effects in carers. Analysts and policy makers should be aware that if HRQoL improvement is an importa...

Retrosternal (Morgagni) diaphragmatic hernia.

The Canadian Veterinary Journal — Mon, 01 Aug 2011 04:00:00 +0100

This study presents the case of a shih tzu puppy, in which a rare congenital Morgagni diaphragmatic hernia was diagnosed. The diagnosis was based on abdominal and thoracic radiographs, including a contrast study of the gastrointestinal tract, which revealed a co-existing umbilical hernia. Both hernias were repaired by surgery. PMID: 22294795 [PubMed - in process] (Source: The Canadian Veterinary Journal)

Congenital Diaphragmatic Hernia: Updates and Outcomes

NeoReviews recent issues — Sun, 31 Jul 2011 23:00:00 +0100

This article reviews the basic pathophysiology behind CDH, describes recent updates in the field, and outlines the long-term outcomes for these fascinating and complex patients. (Source: NeoReviews recent issues)

Preventive antireflux surgery in neonates with congenital diaphragmatic hernia: a single-blinded prospective study

Journal of Pediatric Surgery — Sun, 31 Jul 2011 23:00:00 +0100

Conclusion: Patients profit from fundoplication at CDH repair only within the first year of life. At the present point of this study, simultaneous fundoplication at the time of primary CDH repair cannot be recommended as a standard procedure in all patients with left-sided CDH. (Source: Journal of Pediatric Surgery)

Nitric oxide activity through guanylate cyclase and phosphodiesterase modulation is impaired in fetal lambs with congenital diaphragmatic hernia

Journal of Pediatric Surgery — Sun, 31 Jul 2011 23:00:00 +0100

Conclusions: Guanylate cyclase and PDE 5 play a role in controlling pulmonary vascular tone in fetal lambs with or without CDH. Both enzymes seem to be impaired in fetal lambs with CDH. (Source: Journal of Pediatric Surgery)

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Journal of Pediatric Surgery — Sun, 31 Jul 2011 23:00:00 +0100

Patch repair of a congenital diaphragmatic hernia (CDH) is associated with a much higher rate of recurrence than after primary repair. The biosynthetic options for the repair materials continue to expand. The authors therefore reviewed their experience to benchmark complication rates as they progress with the use of new materials. A retrospective review was conducted of all patients who underwent repair of CDH. Of the 155 patients included in the study, 101 had primary closure and 54 received a diaphragmatic patch. The rates of recurrence, small bowel obstruction (SBO), and subsequent abdominal operations were all significantly higher in the group of patients requiring patch repair. There were 3 types of patch repair: 37 patients received a small intestinal submucosa (SIS) patch, 12 had a ...

Percutaneous closure of a large iatrogenic fistula between the inferior vena cava and the pulmonary vein in a child

Catheterization and Cardiovascular Interventions — Wed, 20 Jul 2011 23:00:00 +0100

AbstractWe hereby describe a complex late postoperative veno‐venous fistula in a child, successfully treated by an interventional percutaneous procedure. It is a unique complication of diaphragmatic hernia surgery that has never been reported before: a late postoperative iatrogenic fistula, between the inferior vena cava and the right lower pulmonary vein, discovered in a 6‐year‐old boy operated at the age of 4 months for a right diaphragmatic hernia. The right to left shunt was completely and uneventfully suppressed by a percutaneous procedure separating the systemic venous return from the pulmonary venous return with an Amplatzer Vascular Plug II. © 2011 Wiley‐Liss, Inc. (Source: Catheterization and Cardiovascular Interventions)

Congenital diaphramatic hernia

Pediatric Radiology — Fri, 08 Jul 2011 06:33:17 +0100

Abstract Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques ha...

Early recurrence of congenital diaphragmatic hernia is higher after thoracoscopic than open repair: a single institutional study

Journal of Pediatric Surgery — Thu, 30 Jun 2011 23:00:00 +0100

Conclusions: Early recurrence of hernia is higher in thoracoscopic CDH repairs than in open repairs. Technical factors and a steep learning curve for thoracoscopy may account for the higher recurrence rates, but not patient severity of illness. In an already-tenuous patient population, performing the repair thoracoscopically with a higher risk of recurrence may not be advantageous. (Source: Journal of Pediatric Surgery)

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Long-term neurodevelopmental outcomes of congenital diaphragmatic hernia survivors not treated with extracorporeal membrane oxygenation

Journal of Pediatric Surgery — Thu, 30 Jun 2011 23:00:00 +0100

Conclusions: Non–ECMO-treated CDH survivors are at substantial risk for neurodevelopmental problems in late childhood and adolescence. (Source: Journal of Pediatric Surgery)

Right-sided congenital diaphragmatic hernia, hepatic pulmonary fusion, duodenal atresia, and imperforate anus in an infant

Journal of Pediatric Surgery — Thu, 30 Jun 2011 23:00:00 +0100

We present a case of a neonate with VACTERL-like association, with the VACTERL association defined as the non-random association of vertebral, anal, cardiac, esophageal, renal/kidney, and limb defects, as manifested by a hemivertebra, imperforate anus, and digit anomalies, in rare association with duodenal atresia and right-sided diaphragmatic hernia. This constellation is previously undescribed and may offer insight into the pathogenesis of VACTERL and associated birth defects. (Source: Journal of Pediatric Surgery)

The embryology of the diaphragm

Seminars in Pediatric Surgery — Tue, 28 Jun 2011 01:41:37 +0100

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...

Structure and epitope distribution of heparan sulfate is disrupted in experimental lung hypoplasia: a glycobiological epigenetic cause for malformation?

BMC Developmental Biology - Latest articles — Mon, 13 Jun 2011 23:00:00 +0100

Conclusions: The observed spatiotemporal changes in HS structure during normal lung development will likely reflect altered activities of many HS-binding proteins regulating lung morphogenesis. Abnormalities in HS structure and distribution in hypoplastic lungs can be expected to perturb HS:protein interactions, ECM microenvironments and crucial epithelial-mesenchyme communication, which may contribute to lung dysmorphogenesis. Indeed, a number of epitopes correlate with structures recognised by FGFs, suggesting a functional consequence of the observed changes in HS in these lungs. These results identify a novel, significant molecular defect in hypoplastic lungs and reveals HS as a potential contributor to hypoplastic lung development in CDH. Finally, these results afford the prospect that...

Late-presenting congenital diaphragmatic hernia

Archives of Disease in Childhood — Sat, 11 Jun 2011 23:00:00 +0100

Case report A 4-month-old boy was admitted to our paediatric unit with a 12-hour history of episodic grunting and inconsolable crying, poor intake and a tender abdomen. An abdominal x-ray showed a bowel configuration suggestive of volvulus (figure 1A). The boy was transferred to a tertiary centre, where an emergency laparotomy was performed. Surprisingly, no abnormalities were found at surgery. During postoperative re-evaluation, decreased breath sounds were heard over the left hemithorax. A chest x-ray taken to rule out pneumonia showed the presence of the stomach in the thoracic cavity (figure 1B). A repeat laparotomy was performed and a small lateral diaphragmatic hernia was surgically repaired. The patient recovered uneventfully. Retrospective appraisal of the initial abdominal x-ray s...

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Dynamic tracheal occlusion improves lung morphometrics and function in the fetal lamb model of congenital diaphragmatic hernia

Journal of Pediatric Surgery — Tue, 31 May 2011 23:00:00 +0100

Conclusion: Preliminary results suggest that in the fetal lamb CDH model, dTO restores normal lung morphometrics and function, whereas cTO leads to enlarged but less functional lungs. (Source: Journal of Pediatric Surgery)

Minimally invasive repair of congenital diaphragmatic hernia

Journal of Pediatric Surgery — Tue, 31 May 2011 23:00:00 +0100

Conclusion: Minimally invasive techniques appear to have a significant higher recurrent hernia rate, with thoracoscopy being the highest. Although adjusted for patch repair, other factors with regard to disease severity may contribute to differences in outcomes among centers. This study is limited to short-term recurrence during initial hospitalization. (Source: Journal of Pediatric Surgery)

Late gestation fetal magnetic resonance imaging–derived total lung volume predicts postnatal survival and need for extracorporeal membrane oxygenation support in isolated congenital diaphragmatic hernia

Journal of Pediatric Surgery — Tue, 31 May 2011 23:00:00 +0100

Conclusion: Late gestation fetal MRI–derived TLV significantly correlates with postnatal survival and need for ECMO. Fetal MRI may be useful for the evaluation of patients who present late in gestation with a CDH. (Source: Journal of Pediatric Surgery)

Gastric antrum hypertrophy causing outlet obstruction in an infant with congenital diaphragmatic hernia

Journal of Pediatric Surgery — Tue, 31 May 2011 23:00:00 +0100

We report the first case of gastric antrum hypertrophy causing gastric outlet obstruction in an infant with CDH. (Source: Journal of Pediatric Surgery)

Pulmonary Hypertension in Neonates with High-Risk Congenital Diaphragmatic Hernia Does Not Affect Mid-Term Outcome

European Journal of Pediatric Surgery — Sun, 22 May 2011 23:00:00 +0100

Eur J Pediatr Surg 2011; 21: 154-158DOI: 10.1055/s-0031-1271669AbstractPurpose: Congenital diaphragmatic hernia (CDH) presents with a broad spectrum of severity, depending on the degree of pulmonary hypoplasia and persistent pulmonary hypertension (PPH). It is currently not clear whether pulmonary hypertension may affect late morbidity. Aim of the present study was to evaluate the influence of PPH on mid-term morbidity in high-risk CDH survivors.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

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Patch repair is an independent predictor of morbidity and mortality in congenital diaphragmatic hernia

Pediatric Surgery International — Wed, 18 May 2011 06:12:51 +0100

Conclusions Infants requiring PR have significantly higher mortality and suffer greater morbidity than those undergoing NPR. This association is independent of other known predictors of mortality. Identifying prenatal features associated with this high risk group would be of great clinical value. Content Type Journal ArticlePages 1-6DOI 10.1007/s00383-011-2925-1Authors M. E. Brindle, University of Calgary, Calgary, AB, CanadaM. Brar, University of Calgary, Calgary, AB, CanadaE. D. Skarsgard, University of British Columbia, Vancouver, BC, Canadaand the Canadian Pediatric Surgery Network (CAPSNet) Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

Absorbable Versus Nonabsorbable Mesh Repair of Congenital Diaphragmatic Hernias in a Growing Animal Model

Journal of Laparoendoscopic — Thu, 05 May 2011 03:01:59 +0100

Journal of Laparoendoscopic & Advanced Surgical Techniques , Vol. 0, No. 0. (Source: Journal of Laparoendoscopic)

[Intussusception after congenital diaphragmatic hernia repair.]

Archives de Pediatrie — Wed, 04 May 2011 23:00:00 +0100

CONCLUSION: A postoperative intussusception in a similar case could be explained by atony of the herniated bowel, possibly a functional leading point in the postoperative phase, when the peristalsis is reactivated. PMID: 21550215 [PubMed - as supplied by publisher] (Source: Archives de Pediatrie)

Recent advances in neonatal surgery

Seminars in Fetal and Neonatal Medicine — Mon, 02 May 2011 23:00:00 +0100

Over the past decade, tremendous advances have been made in understanding neonatal surgical conditions and many novel medical and surgical approaches have been developed that appear promising. At the same time, these advances continue to highlight the many unsolved problems that require further study. The distinguished authors are leaders in their respective fields and they provide a comprehensive compilation of the evolution of advances in neonatal surgery including the impact of prenatal diagnosis and therapy, current treatment of abdominal wall defects, advances in the treatment of congenital diaphragmatic hernia, application of minimally invasive surgery, progress in the treatment of neonatal short bowel syndrome and advances in the treatment of necrotizing enterocolitis. (Source: Semi...

Evidence to Inform Decisions About Maternal-Fetal Surgery: Technical Brief.

Obstetrics and Gynecology — Sat, 30 Apr 2011 23:00:00 +0100

CONCLUSION:: Although developing rapidly, maternal-fetal surgery research has yet to achieve the typical quality of studies and aggregate strength of evidence needed to optimally inform care. PMID: 21471854 [PubMed - as supplied by publisher] (Source: Obstetrics and Gynecology)

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Institutional practice and outcome variation in the management of congenital diaphragmatic hernia and gastroschisis in Canada: a report from the Canadian Pediatric Surgery Network

Journal of Pediatric Surgery — Sat, 30 Apr 2011 23:00:00 +0100

Conclusions: The existence of perinatal practice and outcome variation for GS and CDH suggests targets for improved delivery of care and justifies efforts to standardize treatment on a national basis. (Source: Journal of Pediatric Surgery)

Impact of prenatal evaluation and protocol-based perinatal management on congenital diaphragmatic hernia outcomes

Journal of Pediatric Surgery — Sat, 30 Apr 2011 23:00:00 +0100

Conclusions: Patients with CDH diagnosed prenatally are a higher risk group. Prenatal evaluation and multidisciplinary perinatal management allows for improved outcome in these patients. (Source: Journal of Pediatric Surgery)

Influence of location of delivery on outcome in neonates with congenital diaphragmatic hernia

Journal of Pediatric Surgery — Sat, 30 Apr 2011 23:00:00 +0100

Conclusions: Outborn delivery is a significant predictor of mortality for infants with antenatally diagnosed CDH. (Source: Journal of Pediatric Surgery)

Bronchomegaly as a complication of fetal endoscopic tracheal occlusion. A caution and a possible solution

Journal of Pediatric Surgery — Sat, 30 Apr 2011 23:00:00 +0100

We report a case of bronchial dilatation after FETO and suggest an alternative surgical treatment. (Source: Journal of Pediatric Surgery)

Intrapericardial diaphragmatic hernia: a rare type of congenital diaphragmatic hernia

Journal of Pediatric Surgery — Sat, 30 Apr 2011 23:00:00 +0100

Abstract: An extremely rare case of congenital intrapericardial diaphragmatic hernia is presented. Since 1981, only 14 cases have been reported in the literature. A 5-year-old girl presented with dyspnea on exertion and easy fatigability. Computed tomography was suggestive of an anterior diaphragmatic hernia. Laparoscopy followed by successful open repair of hernia was performed. (Source: Journal of Pediatric Surgery)

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Discovery and Management of Diaphragmatic Hernia Related to Abandoned Epicardial Pacemaker Wires in a Pregnant Woman with {S,L,L} Transposition of the Great Arteries

Congenital Heart Disease — Sat, 30 Apr 2011 23:00:00 +0100

We report the case of a 23‐year‐old pregnant woman who presented with left upper quadrant pain at 20 weeks gestation. The patient was born with {S,L,L} transposition of the great arteries and had high‐grade conduction disease in infancy compelling epicardial pacemaker placement. A standard transvenous pacemaker was placed at age 9 years, without removal of the epicardial system. The patient's abdominal pain was attributed to herniation of abdominal contents through a diaphragmatic defect at the site of the abandoned epicardial pacing wire. Her pain improved spontaneously but worsened later in pregnancy leading to repair of the diaphragmatic hernia via anterolateral thoracotomy at 30 weeks gestation. The procedure was well tolerated by mother and fetus. At 38 3/7 weeks gestation, the ...

Hiatal hernias: a review of the pathophysiologic theories and implication for research

Surgical Endoscopy — Fri, 29 Apr 2011 05:42:03 +0100

Conclusions The pathogenesis of hiatal hernias at the molecular and cellular levels is poorly described. To date, no single theory has proved to be the definitive explanation for hiatal hernia formation, and its pathogenesis appears to be multifactorial. Content Type Journal ArticlePages 1-5DOI 10.1007/s00464-011-1725-yAuthors C. Weber, Department of Surgery, Loyola University Medical Center, Stritch School of Medicine, 2160 South First Avenue, Room 3226, Maywood, IL 60153, USAC. S. Davis, Department of Surgery, Loyola University Medical Center, Stritch School of Medicine, 2160 South First Avenue, Room 3226, Maywood, IL 60153, USAV. Shankaran, Department of Surgery, Loyola University Medical Center, Stritch School of Medicine, 2160 South First Avenue, Room 3226, Mayw...

Impact of prenatal diagnosis and therapy on neonatal surgery

Seminars in Fetal and Neonatal Medicine — Thu, 28 Apr 2011 23:00:00 +0100

Summary: The field of neonatal surgery is now inextricably linked to the field of obstetrics. Neonatologists and surgical specialists experienced with the postnatal management and outcomes of infants with various disorders now look in utero along with obstetricians and fetal medicine specialists to characterize the development and well-being of fetuses with congenital anomalies with a goal to optimize fetal, perinatal and postnatal management. The purpose of this article is to examine how prenatal diagnosis and therapy has influenced neonatal surgery. An overview of prenatal diagnosis and the evolution of fetal therapy are provided. In addition, we review the impact of prenatal diagnosis and therapy on the management and outcomes of infants with specific anomalies, including congenital lun...

Prospective study of antenatally diagnosed congenital cystic adenomatoid malformations

Pediatric Surgery International — Mon, 25 Apr 2011 16:41:44 +0100

Conclusion Antenatally diagnosed CCAMs have an excellent prognosis except in children with a large lesion and associated with lung hypoplasia. Postnatal investigations are required in all cases to confirm the diagnosis. Symptomatic CCAMs require immediate surgery in the neonatal period with a good outcome. Asymptomatic CCAMs can be safely operated between 9 and 12 months of age. Content Type Journal ArticlePages 1-6DOI 10.1007/s00383-011-2909-1Authors P. Raychaudhuri, Department of Paediatric Surgery, John Hunter Children’s Hospital, Locked Bag 1, Hunter Region Mail Centre, Newcastle, NSW 2310, AustraliaA. Pasupati, Department of Paediatric Surgery, John Hunter Children’s Hospital, Locked Bag 1, Hunter Region Mail Centre, Newcastle, NSW 2310, AustraliaA. Ja...

Our patients’ stories: my child’s congenital diaphragmatic hernia

Thrive, Children's Hospital Boston — Mon, 25 Apr 2011 15:26:40 +0100

Jeanne Griffin Vaughn, NP, is a nurse practitioner at Massachusetts General Hospital. When she found out her child would be born with a congenital diaphragmatic hernia, she was thankful for her many connections in medicine, but also felt extra worry because she knew how serious medical conditions could be for children. This is her story. At just one-day-old Jeanne’s baby needed immediate medical attention. As an expectant parent, there’s one sentence that no one wants to hear: “There’s something wrong with the baby.” My husband and I heard those dreaded words during our first child’s ultrasound, when the baby was barely at 18 weeks gestation. When we arrived at the doctor’s office that morning, we decided we weren’t going to ask about the baby’s sex because we wante...

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A Neonate with Left Congenital Diaphragmatic Hernia and Transient Retrograde Flow in the Aortic Arch

Pediatric Cardiology — Thu, 21 Apr 2011 06:09:37 +0100

We present a left congenital diaphragmatic hernia (CDH) infant with retrograde flow in the arch that normalized after CDH repair. We hypothesize transient inadequacy of the left heart; prostaglandins might play a pivotal role. Content Type Journal ArticlePages 1-2DOI 10.1007/s00246-011-9997-5Authors Poonam P. Thankavel, Division of Pediatric Cardiology, UT Southwestern Medical Center, Children’s Medical Center, Dallas, TX 75235, USAClaudio Ramaciotti, Division of Pediatric Cardiology, UT Southwestern Medical Center, Children’s Medical Center, Dallas, TX 75235, USAMatthew S. Lemler, Division of Pediatric Cardiology, UT Southwestern Medical Center, Children’s Medical Center, Dallas, TX 75235, USA Journal Pediatric CardiologyOnline ISSN 1432-1971Print ISSN 0172-0643 (Sou...

Pulmonary artery size has prognostic value in low birth weight infants with congenital diaphragmatic hernia

Pediatric Surgery International — Sat, 16 Apr 2011 15:56:34 +0100

Conclusion We are the first to show that RPA size has prognostic value in low birth weight infants with left-sided CDH. Content Type Journal ArticlePages 1-4DOI 10.1007/s00383-011-2899-zAuthors Toshiaki Takahashi, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421 JapanHiroyuki Koga, Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421 JapanToshitaka Tanaka, Department of Obstetrics and Gynecology, Juntendo University School of Medicine, Tokyo, JapanHiromichi Shoji, Department of Pediatrics, Juntendo University School of Medicine, Tokyo, JapanSatoru Takeda, Department of Obstetrics and Gynecology, Juntend...

When referring physicians and researchers disagree on equipoise: the TOTAL trial experience

Prenatal Diagnosis — Sun, 10 Apr 2011 23:00:00 +0100

ConclusionThe solution adopted for fetoscopic endoluminal tracheal occlusion (FETO) is justified by the extended period of time it has been available to patients before the start of the RCT. Strong patient and referring physician preferences do not entail a right to have FETO, since it is a procedure of yet unproven efficacy and safety. In the future, to avoid the dilemmas posed by the TM and in name of the right of future generations of patients to have access to treatment of proven safety and efficacy, researchers must be able to plan RCT in due time. Copyright © 2011 John Wiley & Sons, Ltd. (Source: Prenatal Diagnosis)

Parafollicular C-Cells of the Thyroid are Decreased in Patients with Congenital Diaphragmatic Hernia

European Journal of Pediatric Surgery — Thu, 07 Apr 2011 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1273778AbstractBackground: Parathyroid and thymic anomalies related to embryonic neural crest dysfunction have been demonstrated in rats with congenital diaphragmatic hernia (CDH). These rats, like infants with CDH, have conotruncal, facial, and other neurocristal defects. The present study examines whether parafollicular C-cells (CC) of the thyroid, whose embryogenesis is related to that of the parathyroids and thymus, might also be abnormal in babies with CDH.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Congenital diaphragmatic hernia: Still a moving target

Seminars in Fetal and Neonatal Medicine — Sun, 03 Apr 2011 23:00:00 +0100

Summary: The primary therapeutic target for congenital diaphragmatic hernia (CDH) patients has shifted from emergency surgical repair towards a non-operative emergency of the newborn treated by interdisciplinary teams. The increased understanding of the epidemiological and pathophysiological aspects of CDH have led to an improved knowledge and application of prenatal diagnosis, postnatal ventilation strategies, treatment of associated pulmonary hypertension and the role of extracorporeal membrane oxygenation therapy. In the surgical field, the perspectives have changed with delayed CDH repair, the introduction of minimally invasive surgery and use of prosthetic material for closure of large defects. With decreased mortality, long term multi-organ morbidity has increased in some survivors. ...

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Anaesthesia for specialist surgery in infancy

Anaesthesia and intensive care medicine — Thu, 31 Mar 2011 23:00:00 +0100

Abstract: Common indications for neonatal surgery include inguinal hernias and hypertrophic pyloric stenosis. Less common conditions, that have major implications for anaesthesia, include tracheo-oesophageal fistula, congenital diaphragmatic hernia, exomphalos, gastroschisis and congenital lobar emphysema. The anaesthetic management of these conditions is outlined in this article. (Source: Anaesthesia and intensive care medicine)

Costs of congenital diaphragmatic hernia repair in the United States—extracorporeal membrane oxygenation foots the bill

Journal of Pediatric Surgery — Thu, 31 Mar 2011 23:00:00 +0100

Conclusions: Extracorporeal membrane oxygenation use is the largest contributing factor to the economic burden in CDH. With limited health care resources, judicious resource utilization in CDH care merits further study. (Source: Journal of Pediatric Surgery)

Perioperative course of pulmonary hypertension in infants with congenital diaphragmatic hernia: impact on outcome following successful repair

Journal of Pediatric Surgery — Thu, 31 Mar 2011 23:00:00 +0100

Conclusions: During the perioperative period, PH severity monitoring via the serial assessment of PSR is beneficial. Better outcomes were observed with a preoperative PSR less than or equal to 0.9, and this association needs to be confirmed by prospective study. (Source: Journal of Pediatric Surgery)

Acute kidney injury in congenital diaphragmatic hernia requiring extracorporeal life support: an insidious problem

Journal of Pediatric Surgery — Thu, 31 Mar 2011 23:00:00 +0100

Conclusions: This is the first report using a systematic definition of AKI in patients with CDH on ECLS. There is a high incidence of AKI in these patients, and when it progresses to failure, it is associated with higher mortality, increased ECLS duration, and increased ventilator days. This highlights the importance of recognizing AKI in patients with CDH requiring ECLS and the potential benefit of preventing progression of AKI or early intervention. (Source: Journal of Pediatric Surgery)

Advantages of Video-assisted Thoracoscopic Surgery for Adult Congenital Hernia with Severe Adhesion: Report of Two Cases.

Annals of Thoracic and Cardiovascular Surgery — Thu, 31 Mar 2011 23:00:00 +0100

Authors: Nakashima S, Watanabe A, Hashimoto M, Mishina T, Obama T, Higami T Adults who have undergone surgical repair of congenital diaphragmatic hernia have a prolonged illness. They usually have severe adhesions around the intrathoracic hernial sac; therefore, the adhesion itself as well as misidentification of the hernial defect can make surgical repair difficult, even in open surgery. Here, we present the successful video-assisted thoracoscopic surgical repairs of Bochdalek and Morgagni hernias in patients with severe adhesions of the hernial sac (peritoneum) to the parietal pleura lying over the thoracic wall and diaphragm. An 18-year-old woman with a Bochdalek hernia and a 28-year-old woman with a Morgagni hernia underwent thoracoscopic division of severe adhesions, proper minith...

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Diaphragmatic Hernias: A Spectrum of Radiographic Appearances

Current Problems in Diagnostic Radiology — Tue, 29 Mar 2011 22:20:49 +0100

Diaphragmatic hernias are common, and although frequently incidental, recognition of both benign and life-threatening manifestations of diaphragmatic hernias is necessary to guide appropriate management. Congenital fetal diaphragmatic hernias, traumatic diaphragmatic rupture, and large symptomatic Bochdalek, Morgagni, and hiatal hernias are typically repaired surgically, while eventration, diaphragmatic slips, and small diaphragmatic hernias do not require intervention or imaging follow-up but should be recognized to avoid confusion with other diagnoses that require additional attention. This pictorial essay will explore the imaging findings and clinical characteristics of these entities. (Source: Current Problems in Diagnostic Radiology)

Does thoracoscopic congenital diaphragmatic hernia repair cause a significant intraoperative acidosis when compared to an open abdominal approach?

Journal of Pediatric Surgery — Tue, 01 Mar 2011 00:00:00 +0100

Conclusions: We present our early experience of thoracoscopic CDH repair. Our results from thoracoscopic repair appear similar to the open procedure performed over the same period. No clear difference in intraoperative pH or recurrence rate has been demonstrated in our series. There is a need for a multicenter prospective study to establish the longer term outcome of this technique. (Source: Journal of Pediatric Surgery)

In utero incarceration of congenital diaphragmatic hernia

Journal of Pediatric Surgery — Tue, 01 Mar 2011 00:00:00 +0100

Abstract: In utero diagnosis of incarcerated congenital diaphragmatic hernia has never been reported. In our case, congenital diaphragmatic hernia presented at 34 weeks of gestation with dilated bowel loops, pleural effusion, and ascites on fetal ultrasound. Preterm delivery and emergency exploration revealed a tight posterolateral diaphragmatic defect with extensive bowel infarction. (Source: Journal of Pediatric Surgery)

Central diaphragmatic hernia in an adult: a rare presentation

Hernia — Mon, 21 Feb 2011 17:07:22 +0100

Discussion Central diaphragmatic hernia is rarely seen in adults. Only one such case is reported so far. Successful repair was performed with laparoscopy. Patient recovered well without any postoperative complications. Content Type Journal ArticlePages 1-3DOI 10.1007/s10029-011-0800-6Authors R. B. Jagad, N M Virani Wockhardt Hospital, Kalawad Road, Rajkot, Gujarat 360007, IndiaP. Kamani, N M Virani Wockhardt Hospital, Kalawad Road, Rajkot, Gujarat 360007, India Journal HerniaOnline ISSN 1248-9204Print ISSN 1265-4906 (Source: Hernia)

Re‐evaluation of stomach position as a simple prognostic factor in fetal left congenital diaphragmatic hernia: a multicenter survey in Japan

Ultrasound in Obstetrics and Gynecology — Fri, 18 Feb 2011 20:55:23 +0100

ConclusionCurrent status and outcomes of prenatally diagnosed left CDH in Japan were surveyed. Stomach herniation into the right chest was not uncommon and its grade correlated with outcome. The combination of liver and stomach positions was useful to stratify patients into three groups (Group I–III) with different prognoses. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

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Childbearing After Hyperthermic Intraperitoneal Chemotherapy: Results From an International Survey

Annals of Surgical Oncology — Fri, 11 Feb 2011 06:46:09 +0100

Conclusion Childbearing after cytoreductive surgery and heated intraperitoneal chemotherapy is possible in women conserving their genital organs after the procedure. The question of fertility should be considered and discussed in women in reproductive age prior to cytoreductive surgery and heated intraperitoneal chemotherapy. Different options could be offered in this setting. Multidisciplinary decision making involving surgical oncologists and fertility specialists is important. Content Type Journal ArticlePages 1-5DOI 10.1245/s10434-011-1595-5Authors Pablo Ortega-Deballon, INSERM UMR 866, Dijon, FranceOlivier Glehen, University Hospital of Lyon South, Pierre Bénite, FranceEdward Levine, Wake Forest University Baptist Medical Center, Winston-Salem, NC USAPompiliu...

Laparoscopic repair of congenital bilateral Morgagni hernia

Surgical Endoscopy — Mon, 07 Feb 2011 06:46:04 +0100

Abstract Morgagni hernia is a rare type of congenital diaphragmatic hernia found in the anterior aspect of the diaphragm. It typically presents in the pediatric population and rarely is diagnosed in adults. Only 3% of diaphragmatic hernias are the Morgagni type, and only 4% of these are found to present bilaterally. Surgical repair of Morgagni hernia has been performed through various approaches including open, laparoscopic, thoracotomy, and video-assisted thoracoscopic surgery (VATS), all with and without mesh. The optimal method of surgical repair is not known due to the rarity of this condition and the limitations of setting up a prospective, randomized trial to evaluate the different methods. Laparoscopic repair with mesh has been described with good short-term results...

New and/or improved aspects of fetal surgery

Prenatal Diagnosis — Fri, 04 Feb 2011 00:00:00 +0100

Fetal Surgery for Congenital Diaphragmatic Hernia Is Back from Never Gone

Fetal Diagnosis and Therapy — Wed, 02 Feb 2011 23:00:00 +0100

Fetal Diagn Ther 2011;29:6–17 (DOI:10.1159/000322844) (Source: Fetal Diagnosis and Therapy)

Terlipressin as rescue therapy for refractory pulmonary hypertension in a neonate with a congenital diaphragmatic hernia

Journal of Pediatric Surgery — Tue, 01 Feb 2011 00:00:00 +0100

We report the case of a 38-week gestational age neonate, with isolated congenital diaphragmatic hernia presenting with refractory persistent pulmonary hypertension, systemic hypotension, and hypoxemia, resistant to usual therapeutics. Arginine vasopressin is responsible for systemic vasoconstriction and decreases pulmonary hypertension. We theorized that terlipressin, its long-acting analogue, could have the same properties. We used terlipressin as rescue therapy after parental and local ethics committee acceptance. After a bolus of terlipressin 20 μg/kg and continuous infusion at a rate of 5 μg/kg per hour, blood oxygen saturation improved from 75% to 98%, oxygen requirements fell from fraction of inspired oxygen 100% to 40%, and mean arterial pressure rose from 28 to 46 mm Hg, allowing...

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Hernia of morgagni and mediastinal lipoma: a case report.

Annals of Thoracic and Cardiovascular Surgery — Tue, 01 Feb 2011 00:00:00 +0100

We report the first life-threatening case of an association between a hernia of Morgagni and a mediastinal lipoma. We present an adult patient with mediastinal lipoma and a right incarcerated hernia of Morgagni with engagement of the stomach, the duodenum and the transverse colon, successfully treated without complications. To our knowledge, this is the first report of an association between those two rare entities in an acute setting. We discuss the differential diagnosis and physiopathology of the condition, referring to published reports. PMID: 21587136 [PubMed - in process] (Source: Annals of Thoracic and Cardiovascular Surgery)

Early experience with thoracoscopic repair of congenital diaphragmatic hernias in pediatric age group: results and lessons learned

Pediatric Surgery International — Tue, 25 Jan 2011 22:53:15 +0100

Conclusions Thoracoscopic repair is feasible and safe for children with CDH, including selective newborn. The technique causes minimal trauma, results in good respiratory function, and promotes early recovery. Content Type Journal ArticlePages 1-4DOI 10.1007/s00383-010-2827-7Authors Rahul Kumar Gupta, Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra IndiaSandesh V. Parelkar, Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra IndiaSanjay N. Oak, Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra IndiaBeejal Sanghvi, Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra IndiaAdvait Prakash, Department of Pediatri...

Fryns syndrome a presentation of two siblings with congenital diaphragmatic hernia

Pediatric Surgery International — Sat, 22 Jan 2011 20:12:00 +0100

Conclusion In spite of lacking many advanced chromosomal evaluations in our institute we recommended for further investigation into isolated and particularly familial cases may lead to the identification of genetic abnormalities detectable with FISH assay, locus-specific DNA probes, or other new techniques. There remains an ongoing need for careful clinical review and blood banking of cases of CDH to allow better insight into the genetic causes of severe fetal anomalies such as CDH. Content Type Journal ArticlePages 1-5DOI 10.1007/s00383-010-2831-yAuthors Mohammed Joudi Aboud, Pediatrics Surgery Unit, The Maternity and Child Teaching Hospital, Al-Qadisiya, IraqMohammed Mojar Al-Shamsy, Pediatrics Department, Faculty of Medicine, Al-Qadisiya University, Diwania, Ira...

Prenatal administration of retinoic acid upregulates connective tissue growth factor in the nitrofen CDH model

Pediatric Surgery International — Sat, 22 Jan 2011 08:00:07 +0100

Conclusion Downregulation of pulmonary CTGF gene and protein expression during later stages of lung development may interfere with normal alveologenesis in the nitrofen CDH model. Upregulation of CTGF pulmonary gene expression after prenatal RA treatment may promote lung growth by promoting alveologenesis in the nitrofen-induced CDH model. Content Type Journal ArticlePages 1-5DOI 10.1007/s00383-010-2833-9Authors Elke Maria Ruttenstock, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandTakashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJens Dingemann, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children...

The role of primary myogenic regulatory factors in the developing diaphragmatic muscle in the nitrofen-induced diaphragmatic hernia

Pediatric Surgery International — Sat, 22 Jan 2011 08:00:04 +0100

Conclusion This is the first study showing that MyoD expression is selectively decreased in the diaphragm muscle in the nitrofen model of CDH. Content Type Journal ArticlePages 1-4DOI 10.1007/s00383-010-2834-8Authors Jens Dingemann, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandTakashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandElke Ruttenstock, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, Ireland Journal Pediatric Surgery InternationalOnline ISSN 1437-9813Print ISSN 0179-0358 (Source: Pediatric Surgery International)

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Defective Neuronal Innervation and Autonomic Imbalance in Human Congenital Diaphragmatic Hernia (CDH) and a Murine Model of CDH

Journal of Surgical Research — Wed, 19 Jan 2011 20:26:51 +0100

Conclusions: There appear to be primary defects in pulmonary neural developmental in CDH resulting in less complex neural innervation and autonomic imbalance. Altered nerve development may contribute to lung defects through effects on airway or vascular development and may contribute to postnatal physiologic derangement that contributes to pulmonary hypertension. Down regulation of VIP ligand could additionally explain the medial hypertrophy of the pulmonary arterial walls and the resulting pulmonary hypertension seen in CDH patients. (Source: Journal of Surgical Research)

Thoracoscopic Repair for Congenital Diaphragmatic Hernia: Experience with 139 Cases

Journal of Laparoendoscopic — Wed, 05 Jan 2011 04:02:09 +0100

Journal of Laparoendoscopic & Advanced Surgical Techniques , Vol. 0, No. 0. (Source: Journal of Laparoendoscopic)

Does minimal access major surgery in the newborn hurt less? An evaluation of cumulative opioid doses

European Journal of Pain — Mon, 03 Jan 2011 00:00:00 +0100

Conclusions: Minimal access surgery for the repair of esophageal atresia or congenital diaphragmatic hernia is not associated with less cumulative opioid doses. (Source: European Journal of Pain)

Technical aspects of fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia

Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

Abstract: In isolated congenital diaphragmatic hernia, prenatal prediction is made based on measurements of lung size and the presence of liver herniation into the thorax. A subset of fetuses likely to die in the postnatal period is eligible for fetal intervention that can promote lung growth. Rather than anatomical repair, this is now attempted by temporary fetal endoscopic tracheal occlusion (FETO). Herein we describe purpose-designed instruments that were developed thanks to a grant from the European Commission. The feasibility and safety of FETO have now been demonstrated in several active fetal surgery programs. The most frequent complication of the procedure is preterm premature rupture of the membranes, which is probably iatrogenic in nature. It does have an impact on gestational ag...

Protocolized management of infants with congenital diaphragmatic hernia: effect on survival

Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

Conclusions: Our new protocol significantly improved survival to discharge for newborns with CDH. Institution of such a protocol is valuable in improving outcomes for patients with CDH and merits consideration for widespread adoption. (Source: Journal of Pediatric Surgery)

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Decreased cerebral oxygen saturation during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia in infants

Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

Conclusions: This preliminary study suggests that thoracoscopic repair of CDH and EA/TOF may be associated with acidosis and decreased cSO2. The effects of these phenomena on future brain development are unknown. (Source: Journal of Pediatric Surgery)

Long term follow-up in high-risk congenital diaphragmatic hernia survivors: patching the diaphragm affects the outcome

Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

Conclusions: Patch repair correlates with higher pulmonary, auxological, and gastroesophageal morbidity without increasing chest wall deformities at long-term follow-up. (Source: Journal of Pediatric Surgery)

Expression of Iroquois genes is up-regulated during early lung development in the nitrofen-induced pulmonary hypoplasia

Journal of Pediatric Surgery — Sat, 01 Jan 2011 00:00:00 +0100

Conclusion: Overexpression of Irx genes in the early lung development may cause pulmonary hypoplasia in the nitrofen CDH model by inducing lung dysmorphogenesis with thickened mesenchyme and diminished airspaces. (Source: Journal of Pediatric Surgery)

Congenital peritoneopericardial diaphragmatic hernia in an alpaca

Australian Veterinary Journal — Sat, 01 Jan 2011 00:00:00 +0100

An adult alpaca was presented because of abdominal pain and was diagnosed with an intestinal obstruction. The putative diagnosis at surgery was an intestinal obstruction caused by peritonitis and intra‐abdominal adhesions. The cause of the inflammation was not determined at that time. The alpaca died soon after surgery from post‐surgical complications and a peritoneopericardial diaphragmatic hernia that was not diagnosed until necropsy. (Source: Australian Veterinary Journal)

Late administration of antenatal vitamin A promotes pulmonary structural maturation and improves ventilation in the lamb model of congenital diaphragmatic hernia

Pediatric Surgery International — Fri, 10 Dec 2010 18:15:24 +0100

Conclusion This is the first study to demonstrate an improvement in lung function and structural maturation when antenatal vitamin A is given in a surgical model of CDH. Content Type Journal ArticleDOI 10.1007/s00383-010-2790-3Authors Nicola A. Lewis, Department of Surgery, Children’s Hospital of Buffalo, State University of New York at Buffalo School of Medicine and Biomedical Sciences, Buffalo, USABruce A. Holm, Department of Surgery, Children’s Hospital of Buffalo, State University of New York at Buffalo School of Medicine and Biomedical Sciences, Buffalo, USAJon Rossman, Department of Surgery, Children’s Hospital of Buffalo, State University of New York at Buffalo School of Medicine and Biomedical Sciences, Buffalo, USADaniel Swartz, Department of Surgery, ...

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Perinatal management of congenital diaphragmatic hernia: when and how should babies be delivered? Results from the Canadian Pediatric Surgery Network

Journal of Pediatric Surgery — Wed, 01 Dec 2010 00:00:00 +0100

Conclusion: Our data do not support advocacy of any specific delivery plan or route nor optimal gestational age for prenatally diagnosed CDH. (Source: Journal of Pediatric Surgery)

Effects of estrogen on lung development in a rat model of diaphragmatic hernia

Journal of Pediatric Surgery — Wed, 01 Dec 2010 00:00:00 +0100

Conclusions: Estradiol can promote lung development in rats with CDH. The down-regulation of TGF-β1 and its signaling pathway may play a role in this effect. (Source: Journal of Pediatric Surgery)

Physiological and clinical characteristics of gastroesophageal reflux after congenital diaphragmatic hernia repair

Journal of Pediatric Surgery — Wed, 01 Dec 2010 00:00:00 +0100

Conclusion: The motor function of the esophageal body and LES is usually preserved in CDH survivors despite the wide range of esophageal acid exposure in early infancy. Those with symptomatic GER outgrow it, unless associated with advanced respiratory distress or neurological impairment. (Source: Journal of Pediatric Surgery)

Umbilical venous catheter complication in an infant with left-sided congenital diaphragmatic hernia: extravasation owing to hepatic vein catheterization

Journal of Pediatric Surgery — Wed, 01 Dec 2010 00:00:00 +0100

We present a case of hepatic extravasation with line erosion through the liver capsule secondary to UVC placement in an infant with left-sided CDH containing the left lobe of the liver. The patient underwent successful repair of the diaphragmatic defect and is a healthy youngster without complication from CDH or extravasation of the liver capsule. Cannulation of the hepatic vasculature cannot be ruled out with radiograph in infants with left-sided CDH. (Source: Journal of Pediatric Surgery)

The Japanese experience with prenatally diagnosed congenital diaphragmatic hernia based on a multi-institutional review

Pediatric Surgery International — Sat, 27 Nov 2010 17:03:32 +0100

Conclusion This multicenter study demonstrated that the 90-day survival rate of isolated prenatally diagnosed CDH was 79%, and that subsequent morbidity remained high. A new treatment strategy is needed to reduce the mortality and morbidity of severe CDH. Content Type Journal ArticleDOI 10.1007/s00383-010-2805-0Authors Hiroomi Okuyama, Department of Pediatric Surgery, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya Hyogo, 663-8501, JapanYoshihiro Kitano, Division of General Surgery, National Center for Child Health and Development, Tokyo, JapanMari Saito, Division of Clinical Research, National Center for Child Health and Development, Tokyo, JapanNoriaki Usui, Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Osaka, JapanNobuyuki...

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Reevaluation of Stomach Position as a Simple Prognostic Factor in Fetal Left Congenital Diaphragmatic Hernia: A Multicenter Survey in Japan

Ultrasound in Obstetrics and Gynecology — Tue, 23 Nov 2010 00:00:00 +0100

Conclusions:Current status and outcomes of prenatally diagnosed left CDH in Japan were surveyed. Stomach herniation into the right chest was not uncommon and its grade correlated with the outcome. Combination of liver and stomach positions were useful to stratify patients into 3 groups (Group I‐III) with different prognoses. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. (Source: Ultrasound in Obstetrics and Gynecology)

Reevaluation of Stomach Position as a Simple Prognostic Factor in Fetal Left Congenital Diaphragmatic Hernia: A Multicenter Survey in Japan.

The Ultrasound Review of Obstetrics and Gynecology — Tue, 23 Nov 2010 00:00:00 +0100

CONCLUSIONS:: Current status and outcomes of prenatally diagnosed left CDH in Japan were surveyed. Stomach herniation into the right chest was not uncommon and its grade correlated with the outcome. Combination of liver and stomach positions were useful to stratify patients into 3 groups (Group I-III) with different prognoses. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd. PMID: 21105020 [PubMed - as supplied by publisher] (Source: The Ultrasound Review of Obstetrics and Gynecology)

Congenital herniation through the foramen Morgagni — clinical presentation, diagnosis and treatment in pediatric population

Central European Journal of Medicine — Mon, 22 Nov 2010 16:46:47 +0100

Abstract Congenital Morgagni hernia is a rare entity, accounting for less than 1,5% of all types of congenital diaphragmatic hernias. The majority of Morgagni hernias are diagnosed late because patients can be asymptomatic or present with non-specific respiratory and gastrointestinal symptoms and signs. The medical records of all patients diagnosed with CMH and treated in our hospital were retrospectively reviewed for age at diagnosis, sex, site of hernia, clinical symptoms, associated anomalies, operative findings and treatment. Over a 20-year period, 5 cases with CMH diagnoses were hospitalized and operated. The age of diagnosis ranged from nine months to 11 years. Male to female ratio was 3:2. Associated anomalies were seen in 2 patients (40%). Most patients had transab...

Identification of TCTE3 as a gene responsible for congenital diaphragmatic hernia using a high-resolution single-nucleotide polymorphism array

Pediatric Surgery International — Wed, 17 Nov 2010 21:03:27 +0100

Conclusion Because TCTE3 encodes a putative light chain of the outer dynein arm of cilia and human diseases caused by ciliary dysfunction show various phenotypes including skeletal defect, TCTE3 may be a genetic candidate influencing CDH. Content Type Journal ArticleDOI 10.1007/s00383-010-2778-zAuthors Risa Teshiba, Division of Genome Analysis, Research Center for Genetic Information, Medical Institute of Bioregulation, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582 JapanKouji Masumoto, Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, JapanGenshiro Esumi, Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Scienc...

Expression of the Wilm’s tumor gene WT1 during diaphragmatic development in the nitrofen model for congenital diaphragmatic hernia

Pediatric Surgery International — Thu, 11 Nov 2010 17:47:26 +0100

Conclusion Downregulation of diaphragmatic WT1 gene expression may impair diaphragmatic development in the nitrofen CDH model. Content Type Journal ArticleDOI 10.1007/s00383-010-2795-yAuthors Jens Dingemann, National Children’s Research Centre, Our Lady’s Children’s Hospital Dublin, Ireland University College Dublin, Dublin 12, IrelandTakashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital Dublin, Ireland University College Dublin, Dublin 12, IrelandElke Ruttenstock, National Children’s Research Centre, Our Lady’s Children’s Hospital Dublin, Ireland University College Dublin, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital Dublin, Ireland University College Dublin, Dublin 1...

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Abnormal development of the enteric nervous system in rat embryos and fetuses with congenital diaphragmatic hernia

Pediatric Surgery International — Thu, 11 Nov 2010 06:50:38 +0100

Conclusions Development of the enteric nervous system of the esophagus, stomach, and small bowel is deficient in rat embryos and fetuses exposed to nitrofen. These anomalies could account in part for the long-term gastrointestinal morbidity observed in CDH survivors. Content Type Journal ArticleDOI 10.1007/s00383-010-2788-xAuthors Leopoldo Martínez, Department of Pediatric Surgery, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, SpainRosa Aras-López, Research Laboratory Idipaz, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, SpainSara Lancha, Research Laboratory Idipaz, Hospital Universitario La Paz, Paseo de la Castellana, 261, 28046 Madrid, SpainMaría Teresa Vallejo-Cremades, Research Laboratory Idipaz, Hospita...

Downregulation of Midkine gene expression and its response to retinoic acid treatment in the nitrofen-induced hypoplastic lung

Pediatric Surgery International — Wed, 10 Nov 2010 17:23:18 +0100

Conclusion Downregulation of MK gene on D15 may contribute to primary PH in the nitrofen CDH model by disrupting early lung morphogenesis. Upregulation of MK gene after RA treatment in the nitrofen-induced hypoplastic lung suggests that RA may have a therapeutic potential to rescue PH in CDH through RA-responsive growth factor signaling. Content Type Journal ArticleDOI 10.1007/s00383-010-2773-4Authors Takashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandMika Shintaku, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJens Dingemann, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandElke Ruttenstock, National Children’s Research...

Microscopic magnetic resonance imaging of the thoracic venous system in rats with congenital diaphragmatic hernia

Pediatric Surgery International — Wed, 10 Nov 2010 17:23:14 +0100

Conclusions The cervico-thoracic veins are normally patterned but abnormally narrow (except the internal jugulars) in rats with CDH. The same embryonic NC dysregulation that accounts for cardiovascular malformations could also explain these venous anomalies in CDH. Content Type Journal ArticleDOI 10.1007/s00383-010-2789-9Authors A. L. Luis, Department of Pediatric Surgery, Hospital Universitario “La Paz”, Paseo de la Castellana, 261, 28046 Madrid, SpainM. Bret, Department of Radiology, Hospital Universitario “La Paz”, Madrid, SpainE. Cuesta, Department of Radiology, Hospital Universitario “La Paz”, Madrid, SpainR. M. Aras, Department of Pediatric Surgery, Hospital Universitario “La Paz”, Paseo de la Castellana, 261, 28046 Madrid, SpainLiu Xiaomei, Ce...

Prenatal retinoic acid treatment upregulates late gestation lung protein 1 in the nitrofen-induced hypoplastic lung in late gestation

Pediatric Surgery International — Wed, 10 Nov 2010 17:23:11 +0100

Conclusion Downregulation of pulmonary LGL1 gene expression in the late stage of lung development may interfere with normal alveologenesis. Upregulation of LGL1 pulmonary gene expression after RA treatment may promote lung growth by stimulating alveologenesis in the nitrofen CDH model. Content Type Journal ArticleDOI 10.1007/s00383-010-2783-2Authors Elke Maria Ruttenstock, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandTakashi Doi, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandJens Dingemann, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin 12, IrelandPrem Puri, National Children’s Research Centre, Our Lady’s Children’s Hospital, Dublin...

Multimodality Imaging of the Pediatric Diaphragm: Anatomy and Pathologic Conditions [Education Exhibits]

Radiographics recent issues — Fri, 05 Nov 2010 00:00:00 +0100

Apart from serving as an important landmark for description and staging of pathologic conditions, the diaphragm is also affected by various types of pathologic conditions in children. Congenital abnormalities affecting the diaphragm include aplasia or hypoplasia, accessory diaphragm, eventration, and hernias. Congenital diaphragmatic hernias (CDHs) include Bochdalek, Morgagni, and hiatal hernias. Although survival rates are improving with the advent of new therapies, there is still significant morbidity and mortality associated with CDH. The morbidity and mortality depend on the associated congenital anomalies, the size of the hernia, and the degree of lung hypoplasia. Newer surgical repair techniques for the diaphragm have resulted in new postoperative complications and imaging appearance...

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Outcome of Congenital Diaphragmatic Hernia Repair Depending on Patch Type

European Journal of Pediatric Surgery — Fri, 05 Nov 2010 00:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1268456© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Full text (Source: European Journal of Pediatric Surgery)

Down-Regulation of Lung Kruppel-Like Factor in the Nitrofen-Induced Hypoplastic Lung

European Journal of Pediatric Surgery — Thu, 04 Nov 2010 00:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1262800AbstractIntroduction: Pulmonary hypoplasia is a primary cause of high morbidity and mortality in neonates with Congenital Diaphragmatic Hernia (CDH). However, the precise pathogenesis of PH associated with CDH is still not clearly understood. It has been recently reported that lung Kruppel-like factor (LKLF), a member of the Kruppel-like factor family of transcription factors, is predominantly expressed in lungs and plays an important role in lung morphogenesis and functional maturation. It has been reported that homozygous deletion of LKLF gene in mice results in reduced lung morphogenesis. It is further reported that chimeric mice derived from LKLF-/- embryonic stem cells exhibit delayed lung development especially in the later gestational sta...

Effect of insulin-like growth factors on lung development in a nitrofen-induced CDH rat model

Pediatric Surgery International — Tue, 02 Nov 2010 17:19:39 +0100

Conclusions An increased mRNA expression of the markers related to type 1 and 2 alveolar epithelial cells, and morphological changes in the epithelial cells were observed in the IGF-2 group. The administration of IGF-2 to nitrofen-induced hypoplastic lungs might lead to alveolar maturation, which thus results in their improved development. Content Type Journal ArticleDOI 10.1007/s00383-010-2791-2Authors Genshiro Esumi, Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582 JapanKouji Masumoto, Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8...

Postoperative chylous ascites: increased scrotal volume as "alarm bell".

The Turkish Journal of Pediatrics — Mon, 01 Nov 2010 00:00:00 +0100

Postoperative chylous ascites: increased scrotal volume as "alarm bell". Turk J Pediatr. 2010 Nov-Dec;52(6):645-7 Authors: De Carolis MP, Bersani I, Sindico P, Fusco FP, Costa S, Lacerenza S, Romagnoli C Chylous ascites has been reported only rarely as a possible consequence of congenital diaphragmatic hernia (CDH) surgical treatment. The present report regards a case of chylous ascites that developed after surgical treatment of CDH and was interestingly anticipated by increased scrotal volume. The aim was to alert neonatologists and pediatric surgeons about the potential usefulness of this clinical sign as a precocious "alarm bell" for chylous ascites development. PMID: 21428199 [PubMed - in process] (Source: The Turkish Journal of Pediatrics)

Anaesthesia for urgent and emergency surgery

Early Human Development — Sat, 30 Oct 2010 00:59:47 +0100

Abstract: The general principles that guide a pediatric anesthesiologist in the care of a newborn needing urgent and emergent neonatal surgery are reviewed. These include careful evaluation and assessment of the newborn including a detailed prenatal history. A brief review of neonatal physiology including the development of the autonomic nervous system and the development of nociceptive pathways is discussed and how important it is to suppress the stress response to surgical intervention in order to improve surgical outcomes. Most of the current general anesthetics have been associated with anesthetic neurotoxicity in juvenile mammals and several epidemiologic studies in human infants and toddlers have linked surgery occurring in the first 3 years of life with neurocognitive delays in scho...

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Embryonic Wnt gene expression in the nitrofen-induced hypoplastic lung using 3-dimensional imaging

Journal of Pediatric Surgery — Fri, 29 Oct 2010 22:28:21 +0100

Conclusion: We provide evidence for the first time that Wnt2 expression is downregulated at lung bud stage in the nitrofen model. Optical projection tomography is potentially a useful approach to visualize both gene expression and morphology during very early stages of lung development. (Source: Journal of Pediatric Surgery)

Downregulation of FGFRL1 Contributes to the Development of the Diaphragmatic Defect in the Nitrofen Model of Congenital Diaphragmatic Hernia

European Journal of Pediatric Surgery — Thu, 14 Oct 2010 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1267957© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Full text (Source: European Journal of Pediatric Surgery)

Outcome of Congenital Diaphragmatic Hernia Repair Depending on Patch Type

European Journal of Pediatric Surgery — Thu, 14 Oct 2010 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1261939AbstractIntroduction: Patch repair of a congenital diaphragmatic hernia is associated with a much higher rate of recurrence than when primary repair is feasible. The biosynthetic options for the repair materials continue to expand. We therefore reviewed our experience to benchmark complication rates as we progress with the use of new materials.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: European Journal of Pediatric Surgery)

Downregulation of FGFRL1 Contributes to the Development of the Diaphragmatic Defect in the Nitrofen Model of Congenital Diaphragmatic Hernia

European Journal of Pediatric Surgery — Sun, 10 Oct 2010 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1262853AbstractIntroduction: The nitrofen model of Congenital Diaphragmatic Hernia (CDH) displays a diaphragmatic defect of the Bochdalek-type and has been widely used to investigate the pathogenesis of CDH. However, the exact pathomechanism of the diaphragmatic defect is still poorly understood. Fibroblast growth factor (FGF) receptor-like 1 (FGFRL1), a member of the FGF receptor family, plays a key role in physiological diaphragmatic development. FGFRL1 is expressed in the fetal diaphragm at low levels in early gestation and its expression steadily increases, becoming most pronounced in later gestational stages. It has been reported that FGFRL1 homozygous null mice have thin, partially amuscular diaphragms and die at birth due to respiratory failure....

IGFBP-4 Gene Overexpression in the Nitrofen-Induced Hypoplastic Lung

European Journal of Pediatric Surgery — Sun, 10 Oct 2010 23:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0030-1262851AbstractPurpose: The precise mechanism of pulmonary hypoplasia (HP) associated with congenital diaphragmatic hernia (CDH) remains unclear. Insulin-like growth factors (IGFs) play an essential role in fetal lung development through IGF receptors (IGFRs) by regulating cellular proliferation, differentiation and survival. It has been reported that the expression of genes involved in IGF-IGFR signaling is altered in the nitrofen-induced hypoplastic lung during the later stages of lung development. IGF-binding proteins (IGFBPs) control bioavailability, activity and disruption of IGFs through the high affinity IGFBP/IGF complexes. IGFBP-4 is a key inhibitor of IGF-IGFR signaling-mediated cell proliferation. It has been revealed that cell proliferation...

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Surgical Management of the Newborn with Congenital Diaphragmatic Hernia

Karger Publishers — Wed, 06 Oct 2010 11:59:16 +0100

Fetal Diagn Ther (DOI:10.1159/000320244) (Source: Karger Publishers)

Research interests and funding of members of the American Pediatric Surgical Association: report from 2010 American Pediatric Surgical Association Outcomes and Clinical Trials Committee

Journal of Pediatric Surgery — Thu, 30 Sep 2010 23:00:00 +0100

Conclusions: Research activities are common among APSA members and encompass a wide range of pediatric surgery topics. Strikingly, the overall financial support of these efforts is limited, predominantly supported by the surgeons themselves. Funded respondents attained grants through Public Health Service grants, departmental grants, or private institutions. (Source: Journal of Pediatric Surgery)

Abnormal platelet-derived growth factor signaling accounting for lung hypoplasia in experimental congenital diaphragmatic hernia

Journal of Pediatric Surgery — Thu, 30 Sep 2010 23:00:00 +0100

Conclusions: Increased expression of PDGFA and PDGFRα suggests that pulmonary hypoplasia in the nitrofen CDH model may be owing to PDGF-induced oxidative stress during lung development. (Source: Journal of Pediatric Surgery)

Congenital diaphragmatic hernia repair in neonates: is thoracoscopy feasible?

Paediatric Anaesthesia — Wed, 22 Sep 2010 21:15:23 +0100

Authors: Ellinas H, Seefelder C PMID: 20849510 [PubMed - in process] (Source: Paediatric Anaesthesia)