[Appendicovesicostomy (Mitrofanoff procedure) in children: Long-term follow-up and specific complications]. / Dérivations urinaires continentes trans-conduit selon Mitrofanoff chez l'enfant : suivi à long terme et complications spécifiques.

OBJECTIVE: In children, intermittent catheterization by appendicovesicostomy according to Mitrofanoff is an interesting alternative to the urethral approach. Objective of the study was to evaluate the rate of appendicovesicostomy's specific complications. METHOD: From 1997 to 2017, data on children treated and followed for an appendicovesicostomy in an academic institution were collected retrospectively. Rates of surgical complications specifically encountered on appendicovesicostomy, time of onset, frequency, and necessity of surgical reinterventions have been reported. RESULTS: Thirty-four patients were operated on and followed for a median of 6.2 years [0.3-24]. Fifty percent had a complication, occurring after a median of 8 months [2-90], and 38% required at least one surgical revision. If complication occurred, adjustment of medical treatment and intermittent catheterization was effective in 12% of patients, endoscopic or over-fascial surgery was necessary in 17% of cases, and under-fascial revision in 21% of cases. Median time to complication was 4 months [1-90] after creation or revision of appendicovesicostomy. Thirty-height percent of difficult channel catheterization were reported, of which 46% were over or under-fascial stenosis. Inaugural urinary incontinence was 18%, and only 9% if using the appendix. At the end, 97% of appendicovesicostomy were continent. CONCLUSIONS: Appendicovesicostomy is a high risk of complications and postoperative revisions surgery, in order to have a functional continent channel. LEVEL OF EVIDENCE: 4.

[Tissue expansion in management of giant omphalocele parietal sequelae]. / Place de l'expansion cutanée dans le traitement des séquelles pariétales des omphalocèles géantes.

OBJECTIVE: According to major difficulty for the giant omphalocele management in the visceral reintegration and the parietal closure, many teams use currently conservative treatment by topical application. These techniques are suppliers of a covered eventration and a scar sequela requiring a complementary treatment. We report the place of the tissue expansion as complementary treatment. PATIENTS AND METHODS: Two patients with a giant omphalocele benefited from a protocol of cutaneous expansion for the correction of their abdominal scar±of their residual eventration. RESULTS: An eventration closure was possible thanks to this protocol. The skin expansion allowed the complete excision of the abdominal scar and the defect cover. An additional skin graft was necessary in the first case. CONCLUSION: The cutaneous expansion in the parietal sequela management of the giant omphaloceles seems to be an interesting alternative. This technique should be realized remotely and except any septic context.

Long-term outcome of severe hypospadias.

OBJECTIVE: To evaluate, from the patient's point of view, long-term urinary and sexual outcome of surgery for severe hypospadias. PATIENTS AND METHOD: Patients treated for severe posterior hypospadias during childhood and now aged 20-35 years were included. Outcome was evaluated using the Hypospadias Objective Scoring Evaluation and our own quality of life questionnaire. RESULTS: Of 48 patients, 13 were lost to follow up. The questionnaire was thus sent to 35 patients of whom 27 agreed to answer (77% response rate). Fifteen presented proximal and 12 scrotal hypospadias. Average age at surgery was 3.3 years and the children underwent an average of 3.7 procedures. There were 40% early complications, and 59% late complications, including 33% fistulae and 26% stenosis. At present, 70% of patients have an apical meatus, 82% have a straight penis, 37% present difficulty initiating voiding, and one patient has a persistent fistula; 74% say their penis looks abnormal, either because of the absence of foreskin, penis size or scarring. Sexual and global satisfaction rates, determined using a visual analogue scale, are respectively 68% and 63%. Satisfaction is statistically correlated to the presence of early complications or stenosis, but not fistula. It decreases if the patient thinks his penis is not normal, even if the anatomical result is good. CONCLUSION: Urinary and sexual outcome is good for most patients though the results are variable. Satisfaction seems more affected by subjective feelings than the anatomical result.

Should we perform orchidopexy for cryptorchidism in children with severe encephalopathy?

OBJECTIVE: To conduct a survey of practices in France concerning cryptorchidism in patients presenting with severe encephalopathy. MATERIAL AND METHOD: We conducted an e-mail survey of current practices in 27 pediatric surgery departments in 27 French university or general hospitals. The study concerned children with severe mental deficiency. RESULTS: The survey showed a disparity in attitudes towards cryptorchidism in patients with severe encephalopathy, ranging from no treatment to orchidopexy in all cases. In most cases, the indication for surgery was the psychological aspect for the parents or accessibility to clinical examination. CONCLUSIONS: This survey illustrates the unease concerning the pertinence of performing orchidopexy in these children. As improved fertility is not really an issue in children with severe encephalopathy, psychological aspects apply only to the family, and, as the benefit of orchidopexy for prevention or early diagnosis of testicular cancer is marginal, the risk/benefit ratio of orchidopexy for children with severe encephalopathy and cryptorchidism is unclear.

One-step management of apple-peel atresia.

PURPOSE: Apple-peel atresia is a rare form of bowel atresia associating proximal jejunal atresia with a typical coil-shaped distal small bowel. Many of the children suffering from this condition are still managed with initial enterostomy followed by delayed anastomosis. PATIENTS AND METHOD: Between 2000 and 2007, we managed four patients with apple-peel atresia. Three by primary anastomosis and one by initial enterostomy with delayed anastomosis. RESULTS: Total duration of total parenteral nutrition (TPN) was an average 109 days. Survival rate was 100% with an average follow-up of 4.3 years. CONCLUSION: Nowadays, the prognosis of apple-peel atresia depends mainly on prolonged TPN-related morbidity rather than immediate postoperative complications. We therefore recommend that those infants affected be managed by primary anastomosis in order to reduce the duration of parenteral nutrition, followed by careful nutrition by teams experienced in neonatal TPN and short bowel syndrome.

Successful management of a retroperitoneal kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using alpha-interferon.

It has been shown recently that Kasabach-Merritt phenomenon, the association of a vascular tumour and consumption coagulopathy, does not--as previously thought--complicate "classical" infantile hemangiomas but distinctive entities called kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), both tumours on the same neoplastic spectrum. These tumours have been found in the neck, face, thorax, abdomen, retroperitoneum and limbs and are associated with a mortality rate of as high as 30 %. Several therapeutic modalities, including alpha-interferon, vincristine, radiotherapy and surgery have been reported in the literature. We report a case of retroperitoneal kaposiform hemangioendothelioma regression using alpha-interferon and discuss the current knowledge of this entity and its treatment.

Peritoneal needle suction for intestinal perforation in the preterm neonate.

UNLABELLED: A few years ago, most intestinal perforations in the premature newborn appeared within the clinical context of necrotising enterocolitis (NEC). Since then, we have observed an increase in the number of isolated perforations appearing outside typical NEC. The fact that the perforations are more often isolated, and the healing capabilities of the premature intestine, led us to propose peritoneal needle suction (PNS) alone as first treatment for intestinal perforations in the premature neonate. MATERIALS AND METHOD: The charts of 6 consecutive premature infants presenting with intestinal perforations treated initially by PNS alone were reviewed. RESULTS: The patients' median birth weight was 1030 g, with a median gestational age of 27 weeks. In 5 out of 6 infants (83 %), PNS achieved complete exsufflation without recurrence of the pneumoperitoneum and complete intestinal healing, allowing complete enteral feeding 30 to 71 days after perforation. One infant with recurrent pneumoperitoneum after 3 PNS and peritoneal drainage was operated. All infants survived. CONCLUSION: We believe that for early perforations of the premature neonate, the poor diffusion of the infection and the frequent capacity of the perforation to close and subsequently heal without scars, favour a minimally invasive management using PNS.

Interest of laparoscopy in polysplenia syndrome.

Polysplenia syndrome (PS) is usually discovered in symptomatic patients in association with congenital heart disease or biliary atresia. Asymptomatic patients can present associated anomalies of the digestive tract such as intestinal malrotation and gastric or splenic malfixation. We report a case of PS presenting as a left flank mass shown to be an accessory spleen by denatured red blood cell scintigraphy. Upper gastrointestinal tract studies showed intestinal malrotation. Laparoscopic exploration confirmed intestinal malrotation and showed the absence of fixation of the accessory spleen. We performed a laparoscopic Ladd's procedure followed by fixation of the accessory spleen and resection of Meckel's diverticulum through a short left-flank incision.

[Feingold syndrome]. / Syndrome de Feingold.

BACKGROUND: Feingold syndrome is a combination of hand and foot anomalies, microcephaly, tracheo-esophageal fistula, esophageal/duodenal atresia, short palpebral fissures and developmental delay. The most frequent physical findings are clinodactyly of the second and fifth fingers with absent or hypoplastic middle phalanges, and syndactyly of the toes. Inheritance is autosomal dominant (MIM number 164280) with full penetrance concerning hand anomalies, variable expressivity and great intrafamilial variability. CASE REPORT: We report the case of an African boy with duodenal atresia, microcephaly, brachymesophalangy of the second and fifth fingers, unilateral thumb hypoplasia, bilateral syndactyly of toes 2-3 and amesophalangy of the toes. Karyotype was normal. No other member of the family was affected. A fresh mutation is possible. CONCLUSION: Brachymesophalangy affecting mainly the second and fifth fingers and amesophalangy of the lateral toes are cardinal clinical findings in Feingold syndrome. These clinical findings define the syndrome of brachydactyly A4-Temtamy type (MIM 112800), suggesting that brachydactyly A4 would be a partially expressed Feingold syndrome. The incidence of esophageal/duodenal atresia is 29%, including all the known cases. All karyotypes of reported patients were normal.

Highly differentiated teratoma and fetus-in-fetu: a single pathology?

A case of sacrococcygeal teratoma is presented with characteristics of fetus-in-fetu. This pseudo-fetus presented a rudimentary single cavity heart, which beat at a different rate to that of the affected infant. X-ray examination showed no spinal column. This case confirms that fetus in fetu can be a remarkably complex, well-differentiated, highly organized teratoma.

Thrombosis of the arterial cannula during extracorporeal membrane oxygenation in a full-term newborn infant.

The authors report a rare case of cannula thrombosis during extracorporeal membrane oxygenation (ECMO). A full-term newborn infant was successively placed on single-cannula veno-venous extracorporeal lung support and then on veno-arterial ECMO, because of persistent pulmonary hypertension. At 140 hours of ECMO, the infant displayed general cyanosis except in the right arm. Since asymmetric hypoxemia during ECMO may be related either to cannula malposition or to a tip thrombosis, a chest x-ray after contrast injection into the arterial line of the circuit was performed. It showed an opacification of the whole cannula but for the last distal centimeter, and of the vascular bed extending from the right subclavian artery. Cannula thrombosis was suspected and confirmed by removal of the arterial cannula. Demonstration of cannula thrombosis by opacification of the arterial line of the circuit indicates catheter removal.

Pancreaticoduodenectomy in childhood for rare pancreatic ductal anomalies--case reports.

In the past 9 years, 3 children suffering rare pancreatic disorders have undergone cephalic pancreaticoduodenectomy (Whipple procedure) required after the failure of initial conservative treatments. Ductal anomalies such as pancreas divisum or predominant Santorini duct can be managed conservatively (especially by papillotomy of the minor papilla), unless the extent of the fibrotic lesions of the pancreas jeopardizes the final evolution of the pancreatic disease. Intrapancreatic gastric duplications remain extremely rare. Even to-day, diagnosis was made only on pathologic specimens after radical surgery. Better knowledge of this rare pathology, with emphasis on its radiological features, may help better assessment. Hemoductal pancreatitis is evocative of the coexistence of gastric duplication and pancreatic aberrant duct. However, when fibrotic and necrotic changes in the duodenopancreatic region are considerable, assessment of this anomaly becomes difficult. Radical though it is, pancreaticoduodenectomy may be necessary to treat cephalic pancreatic lesions in childhood after the failure of reasonable attempts of conservative treatment.