Bilateral congenital diaphragmatic hernia associated with brachyesophagus

Objectives: The authors report the case of an infant presenting with a bilateral, postero-internal diaphragmatic hernia associated with a brachyesophagus and vertebral malformation. The surgical care and the follow-up of this malformation complex are discussed. Methods: The antenatal examination identified only a left hernia; complete assessment of the malformation was performed at birth. Repair of the hernias was performed 20 h after birth by suturing the hernial orifices, involving neither prosthetic material nor transposition of the muscle. A Collis gastroplasty procedure was carried out 3 months later with repositioning of the hiatus and Toupet fundoplication. The existence of a thoracic vertebral hemiblock led to the development of scoliosis: the child was submitted to orthopedic treatment at the age of 24 months. Conclusion: This case demonstrates the difficulties involved in precise antenatal diagnosis of certain diaphragmatic malformations, and difficulties concerning surgical repair of wide muscular defects with aplasia of the pillars. This polymalformation syndrome requires multidisciplinary care and long-term follow-up

Surgical treatment of diaphragmatic agenesis by transposition of a muscle flap: Report on 15 cases

Between 1987 and 1996 the transposition of a muscle flap using the anterolateral abdominal wall (the internal oblique and transversus abdominus muscle) has been used for the surgical treatment of fifteen cases of diaphragmatic agenesis. There are several advantages of this technique: a more accurate reconstruction of the diaphragmatic dome, a better adapted muscle flap, good tolerance and evolution as a result of the use of autologous material with thus less risk of recurrence and/or infection. The disadvantages are represented by the nine cases of abdominal herniation at the site of the muscle flap, three of which necessitated secondary surgical treatment. Nine children survived this treatment, six died following severe cardio-pulmonary complications associated with this malformation

Carcinoid tumors of the appendix in children

We report ten cases of carcinoid tumor of the appendix observed in children from 1988 to 1996. The patients included six females and four males with an average age of 13 years at presentation. They were admitted after complaining of pain in the lower abdominal quadrant. In eight children who presented with symptoms of acute appendicitis, the tumor was located at the tip of the appendix. Diagnosis was performed after appendicectomy (AE) and pathologic examination, which revealed a tumor slightly under 1 cm in size. Two other children were admitted with clinical signs of peritonitis due to larger tumors measuring more than 2 cm on the base of the appendix. One patient underwent a cecectomy, the other a right hemicolectomy. For all patients follow-up was 3 years, and all recovered fully. According to these findings and a review of the literature, we suggest conservative surgical procedures in children. More than 70% of these tumors are localized at the tip of the appendix and represent an incidental pathologic finding during AE; AE alone is curative. Patients with a bulky tumor of the appendicular base measuring 2 cm and invading the serosa and mesoappendix without metastases may be treated with a cecectomy; ileocecal resection may be indicated in cases where the tumor has infiltrated tissue beyond the cecum with localized metastases and in patients with incomplete gross resection. Right hemicolectomy is questionable in this age group and restricted to rare conditions

Gastric necrosis in newborns: A report of 11 cases

Eleven neonates ranging in gestational age from 34 to 40 weeks presented with gastric necrosis. The 4 full-term neonates showed sudden-onset hemorrage and 'coffee-ground' vomiting; in the 7 premature babies the initial clinical finding was abdominal distention. The criteria for diagnosis were: perinatal distress in prematures and transient neonatal respiratory distress in full-term babies. Radiographic evidence of gastric distention was typical and preceded clinical signs of hematemesis and gastric perforation. Surgery was performed in 8 patients; 3 received medical treatment. At surgery 1 total and 3 subtotal gastrectomies and 4 segmental gastric resections were performed. Three of these patients died post-operatively as a consequence of multiorgan failure; a second look was necessary in one patient 1 week after surgery because of prepyloric perforation due to ulcers. Biopsy specimens taken from the site of perforation demonstrated extensive necrosis; ulceration was disseminated in the surrounding gastric mucosa; no signs of phlogosis were detected. The diagnosis, treatment, and physiopathologic considerations are reviewed

Is the appendix graft suitable for routine biliary surgery in children?

BACKGROUND/PURPOSE: The appendix graft (AG) is used widely for urinary tract replacement in children. Biliary tract replacement is less common. The purpose of this retrospective multicentric study was to evaluate the safety of appendix grafting for biliary reconstruction. METHODS: The files of 33 patients treated at 7 European pediatric centers were reviewed. Indications included choledochal cyst (CC) in 5 cases, biliary trauma (BT) in 1, and biliary atresia (BA) in 27. In CC and BT patients, the graft was inserted isoperistaltically between the proximal biliary duct and second duodenum. In all but one of the BA patients, the graft was placed antiperistaltically by patching its cecal end onto the porta hepatis. RESULTS: Postoperatively, all CC and BT patients initially became asymptomatic but developed laboratory evidence of anicteric cholestasis within 1 year. The most common manifestation was increased gamma-glutamyl-transpeptidase level (GGT), whereas histologic findings showed liver damage (mainly fibrosis). Reoperation has been carried out in 4 CC and 1 BT patients within a mean period of 19 months after appendix grafting. The graft procedure was converted to hepaticojejunostomy (HJ) in 4 and to choledocoduodenostomy in 1. Surgical exploration showed kinking in 1 patient and stenosis in 1. In the remaining 3 cases, there was no discernible cause of cholestasis, and appendix histology findings were normal. In all 5 reoperated patients, liver function findings returned to normal within 1 month. Reoperation is scheduled for the remaining CC patient who currently requires ursodesoxycholic medication to maintain normal liver function and presents histologic evidence of "de novo" sclerosing cholangitis. Results of appendix grafting also were poor in the 27 BA patients. Procedure-related perioperative complications occurred in 4 (15%) including 1 early death from graft necrosis. Another early death resulted from intestinal hemorrhage. Jaundice cleared in only 8 (28%). CONCLUSIONS: The findings of this study suggest that the AG is unsuitable for routine biliary repair in children. It should be used only as a salvage technique when conventional HJ repair is contraindicated. Because of the high risk of graft dysfunction, we recommend screening tests to detect biochemical or histologic cholestasis in any patient previously treated with appendix grafting

115 PW–850 J compressed beam demonstration using the PETAL facility

International audienceThe Petawatt Aquitaine Laser (PETAL) facility was designed and constructed by the French Commissariat à l'énergie atomique et aux énergies alternatives (CEA) as an additional PW beamline to the Laser MegaJoule (LMJ) facility. PETAL energy is limited to 1kJ at the beginning due to the damage threshold of the final optics. In this paper, we present the commissioning of the PW PETAL beamline. The first kJ shots in the amplifier section with a large spectrum front end, the alignment of the synthetic aperture compression stage and the initial demonstration of the 1.15 PW @ 850 J operations in the compression stage are detailed