A randomised controlled trial of amnioexchange for fetal gastroschisis.

OBJECTIVE: Morbidity in fetuses affected by gastroschisis is mainly the result of bowel ischaemic and inflammatory processes. Experimental studies on animal models show that clearing amniotic fluid from the digestive secretions by amnioexchange procedures reduces the inflammatory process. We evaluated the benefit of the amnioexchange procedure for fetal gastroschisis in humans. DESIGN: Prospective, interventional, randomised study. SETTING: Eight referral centres for fetal medicine. POPULATION: Pregnant women carrying a fetus with gastroschisis. METHODS: We compared, in utero, amnioexchange with a sham procedure. The protocol included, in both arms, steroid injections at 30 weeks of gestation and the use of postnatal minimal enteral feeding. MAIN OUTCOME MEASURES: The primary outcome was a composite variable based on the duration of ventilation and parenteral nutrition. Secondary outcomes were the effectiveness and safety of the amnioexchange procedure, including the rate of perinatal death, time to full enteral feeding, primary closure, and late feeding disorders. RESULTS: Sixty-four patients were randomised. There was no difference in the composite criteria between the amnioexchange and control groups. Based on an intention-to-treat analysis, there were no significant between-group differences in pregnancy outcome or complications. When studying the relationship between digestive compounds and amniotic fluid inflammatory markers, a clear correlation was found between bile acid and both ferritin and interleukin 1ß (IL1ß). CONCLUSIONS: In humans, amnioexchange, as described in our protocol, is not an option for fetal care; however, we provide supplementary proof of the involvement of inflammation in the pathogenicity of gastroschisis and suggest that future research should aim at reducing inflammation. ClinicalTrials.gov: NCT00127946. TWEETABLE ABSTRACT: A prospective, interventional, randomised study shows no benefit of amnioexchange for fetal gastroschisis in humans.

[Recurrent tracheoesophageal fistula in type III esophageal atresia. Diagnosis and treatment are not easy]. / Reperméation de fistule œso-trachéale dans l'atrésie de l'œsophage de type III : un diagnostic et une prise en charge difficile.

Recurrent tracheoesophageal fistula (TEF) is possible after repair of congenital esophageal atresia. The frequency of recurrent TEF is observed in about 10% of the cases. Within a cohort of 67 children with type III esophageal atresia repaired between 1998 and 2009, we aimed to identify the number of children with recurrent TEF, the risk factors for this condition, and the treatment proposed. The sex ratio was 1.7. Surgery was performed between 4 and 36 hours of life. Five children (7.5%) had a recurrent TEF, usually during the first 3 months, revealed by respiratory symptoms related to feeding in some cases. We noted that recurrent TEF was more frequent with anastomotic leakage (P=0.09) or postsurgical pneumothorax (P<0.01). The diagnosis was made in four cases out of five by a methylene blue test performed during a tracheobronchial endoscopy. Surgical treatment was noted in four children, with three postsurgical secondary effects. One child was treated by endoscopy and an esophageal clip. With a median follow-up of 52 months, no recurrence was noted. The recurrence of TEF may be linked to postsurgical events. Diagnosis is not easy and treatment is not clearly codified. Endoscopic treatment may be an advantageous option to surgery, likely with less morbidity.

Oesophagogastric reconnection is possible.

INTRODUCTION: The first oesophagogastric dissociation was described by Bianchi in 1997 for the treatment of severe gastro-oesophageal reflux (GOR) in neurological patients to avoid serious respiratory complications. The dissociation leads to malabsorption and growth problems. We describe the first two cases of oesophagogastric reconnection long after lung growth. PATIENTS: Case no. 1 was a 12.7-year-old child with a history of type I oesophageal atresia. He required oesophagocoloplasty and gastric dissociation by the Bianchi procedure at the age of 3 months. He has oesophagogastric reconnection at 11 years old because he had a several macrocytic anaemia. Case no. 2 was also a 12-year-old boy, with an unlabelled multiple malformation syndrome with type I oesophageal atresia. He had an oesophagocoloplasty with gastric dissociation at the age of 3 months to protect his lungs. The reconnection was proposed at 10 years old because he developed multiple nutritional deficiencies and growth retardation. RESULTS: The two cases have no clinical symptom of reflux and their post-operative digestive contrasts are normal. Reconnection appears possible with or without preserving the Roux-en-Y loop. It improves absorption and corrects any vitamin deficiencies, allows the reconnected stomach to be monitored in terms of reflux and malignancy and enables enteral nutrition supplementation to be stopped. The possibility of reconnection after the Bianchi procedure therefore extends its indications, mostly in the case of oesophageal atresia complicated by severe GOR with pulmonary repercussions or tracheoesophageal cleft.

[Medium-term outcome, follow-up, and quality of life in children treated for type III esophageal atresia]. / État de santé, suivi et qualité de vie à moyen terme d'enfants opérés d'une atrésie de l'œsophage de type III.

The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good.

[Post-traumatic small-bowel obstruction: two case studies]. / Sténoses post-traumatiques du grêle chez l'enfant : à propos de 2 cas.

Post-traumatic small-bowel obstructions are rare and late complications following blunt abdominal trauma. Timely diagnosis is frequently impeded because of nonspecific associated symptoms, and furthermore, the degree of urgency is frequently underestimated due to the accidental cause. During the last 6 years (2005-2011), we have observed only 2 cases (4 and 10 years old) with post-traumatic small-bowel obstruction. On admission the patients had a contusion on the abdominal wall, duodenal hematoma and a Chance fracture of the lumbar spine on the computed tomography (CT) scan. Although early clinical improvement was noted, progression of the disorder was observed for the following 15 days like an occlusive syndrome (abdominal distension, nausea, emesis). Abdominal ultrasound and computed tomography scan revealed small-bowel obstruction. At laparotomy, jejunal stenosis was found associated with mesenteric tears, which was resected with end-to-end anastomosis. Six to 8 months of follow-up ascertained the therapeutic efficacy of the procedure. Post-traumatic small-bowel obstructions are rare; the clinical presentation is unspecific and appears with a time lag following the trauma. Echography and computed tomography scan can establish both the diagnosis and therapeutic choice.

[Congenital diaphragmatic hernia: respiratory and vascular outcomes]. / Hernie de coupole diaphragmatique : devenir respiratoire et vasculaire pulmonaire.

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care, and new postnatal treatment strategies, the rates of mortality and morbidity in the newborn with CDH remain high as the result of severe respiratory failure secondary to PH and PPH. Later, lung function assessments show obstructive and restrictive impairments due to altered lung structure and lung damage due to prolonged ventilatory support. The long-term consequences of pulmonary hypertension are unknown. Other problems include chronic pulmonary aspiration caused by gastro-oesophageal reflux and respiratory manifestations of allergy such as asthma or rhinitis. Finally, failure to thrive may be caused by increased caloric requirements due to pulmonary morbidity. Follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.

[Congenital diaphragmatic hernia: antenatal care]. / Hernie congénitale du diaphragme : prise en charge anténatale.

The prenatal evaluation of the postnatal prognosis of fetuses displaying congenital diaphragmatic hernia (CDH) has improved over the past five years. Although the accuracy of these outcome predictions remains a matter of debate, it seems important that all teams in charge of those fetuses use the same prognostic factors in order to be able to improve and compare their practice. Prediction will be based on Lung over Head Ratio (LHR) between 22 and 28 weeks or the LHR observed/expected whatever the gestational age, (the measurement of which relies on very strict criteria), the position of the liver and lung volumes measured by MRI. These factors allow the identification of a group of fetuses likely to have a poor outcome. In the group with LHR less than 1 or LHR o/e less than 25% and where the liver is in the thorax, survival is less than 20%. In utero treatment could be offered to these fetuses. A balloon can be placed in the trachea, under the vocal cords, by foetoscopy between 28 and 30 weeks of pregnancy. The balloon is retrieved at 34 weeks. The preliminary results show that survival in this group increases from 20% to up to 50%. The morbidity does not seem to be increased but is currently under evaluation.

Pulmonary sequestration of the left upper lobe associated with a bronchogenic cyst: case report of an exceptional association.

Intralobar pulmonary sequestration (ILPS) and bronchogenic cyst are rare congenital diseases. We present the first case of the association between an ILPS located in the upper lobe and a bronchogenic cyst. This association has been discovered antenatally in a girl. The cystic lesion has been shown by a fetal MRI and confirmed by a CT scan at 3 weeks of life. She underwent a left upper lobectomy by thoracoscopy at the age of 5 months. Postoperative course was uneventfull. This case confirms the congenital origin of pulmonary sequestration and shows the interest of an early surgery.

Frantz's tumor: is mutilating surgery always justified in young patients?

BACKGROUND: Solid pseudopapillary tumor (Frantz's tumor) of the pancreas is a rare lesion. It is of low-grade malignancy but can cause extensive local invasion. The aim of this study was to assess the outcome of Frantz's tumors after incomplete resection. METHODS: We contacted all authors who published case reports describing incomplete resection of Frantz's tumor between 1985 and 2008 to request follow-up information. RESULTS: Follow-up information was obtained for 11 out 18 patients who underwent incomplete resection. Estimated median survival rate was 5.7 years (69.5 months). CONCLUSION: Since Frantz's tumor typically develops mainly in children and young women, a 5.7 year survival rate is unacceptable. Thus complete resection of locally invasive solid-pseudopapillary tumor of the pancreas is always justified, even at the price of difficult, mutilating surgery.

Prenatal diagnosis and evaluation of defect length in esophageal atresia using direct and indirect (tracheal print) signs.

The prenatal diagnosis of esophageal atresia is challenging. The length of the defect of the esophageal atretic portion is one of the parameters affecting outcome and prenatal evaluation of this length has not, to our knowledge, been described previously. We report on seven fetuses assessed prospectively which were suspected to have esophageal atresia. Targeted ultrasound examination of both fetal cervical and thoracic structures was performed in each case in order to assess prenatally the atretic portion. The length of the defect was assessed both directly, by visualizing the interruption of the hyperechoic lines representing the walls of the esophagus in a mid-sagittal view (n = 4), and indirectly, by means of the 'tracheal print' (n = 5). Both methods were used in three cases. Prenatal results were compared with postnatal or postmortem findings. The prenatal diagnosis of esophageal atresia was made correctly in six of the seven cases and in all of these there was concordance between prenatal and postnatal estimates of the esophageal defect lengths. Direct or indirect sonographic assessment of the esophagus in cases of suspected prenatal esophageal atresia improves the specificity of its diagnosis and aids prenatal evaluation.

[Per and post-natal medical management of congenital diaphragmatic hernia]. / Prise en charge médicale per et post-natale de la hernie congénitale du diaphragme.

Congenital diaphragmatic hernia (CDH) is a 1 out of 3500 live-born malformation with persistent 30-40% mortality rate, related to severe pulmonary hypoplasia and hypertension. Better knowledge on the mechanisms inducing failure of adaptation at birth is a prerequisite for improving CDH prognosis. CDH is also associated with longterm morbidity, including prolonged respiratory failure, failure to growth, oral aversion, and scoliosis. Early prevention starting as soon as the first hours of life are required to reduced long term morbidity. The aims of the management are not only to reduce early mortality, related to persistent pulmonary hypertension, but also to prevent late morbidity.

Association of three different congenital malformations in a same pulmonary lobe in a 5-year-old girl.

We report the case of a 5-year-old girl with persistent chest X-ray abnormalities following an episode of pneumonia who has a complex congenital pulmonary malformation comprising of a congenital pulmonary airway malformation, an intralobar sequestration and two bronchogenic cysts, all present within the same lobe. The observation suggests a common embryological origin of these malformations.

Laparoscopic nephrectomy of a cross-fused ectopic kidney in a child with hypertension.

OBJECTIVE: We report a case of laparoscopic nephrectomy of a cross-fused ectopic kidney in a 4-year-old girl with renal hypertension and Fanconi anemia. MATERIALS AND METHODS: We performed a transperitoneal laparoscopy. Ectopic kidney resection was done after dissection of the pathological kidney and after clamping vessels, using an ultrasonic device. Hospitalization time was 4 days. RESULTS: At 6 months, blood pressure was normalized and the patient showed an adequate growth curve. CONCLUSION: The transperitoneal route is very effective when a nephrectomy is necessary. It offers perfect exposure with limited risk of complications.

Ventilation-induced pulmonary vasodilatation in lambs with congenital diaphragmatic hernia is modulated by nitric oxide.

Endogenous nitric oxide (NO) mediates pulmonary vasodilatation at birth, but inhaled NO fails to reduce pulmonary vascular resistance (PVR) in newborns with congenital diaphragmatic hernia (CDH). This study was designed to investigate the effects of ventilation, and the nature of its endogenous mediator, in fetal lambs with experimental CDH. Investigations at 138 days of gestation showed that ventilation markedly decreased PVR. Inhibition of NO synthesis reduced ventilation-induced pulmonary vasodilatation in vivo and increased in vitro isometric tension of vascular rings. Ventilation therefore reduces PVR at birth in lambs with CDH, and endogenous NO seems to contribute to this reduction.

[Kystic pleuropulmonary blastoma fortuitly discovered in an infant]. / Pleuropneumoblastome kystique de découverte fortuite chez un nourrisson.

Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, associated with a poor outcome. We report the case of a 7 week-old boy with a pleuropulmonary blastoma classified as type I. Disease was discovered at a chest X-ray performed as a work-up for a benign acute viral bronchiolitis. The final diagnosis was brought by pathology: a bronchopulmonary malformation had not been ruled out by clinical, radiological and macroscopic findings. Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, for which the poor outcome and tough diagnosis justify a surgical attitude when an intrathoracic bullous lesion is found in an infant.