Thymectomy in Children with Juvenile Myasthenia Gravis: Is It Recommended?.

Objective: Juvenile myasthenia gravis (JMG) is a rare disease with possible severe forms. Thymectomy is supported by many authors particularly in generalised forms with positive AcetylCholine Receptor Antibody (AChR- Ab). The aim of our study was to discuss the indication and the outcome of thymectomy in five children with JMG. Methods: We performed a retrospective study of the medical files of patients with JMG, who were hospitalised in our pediatric surgery department for thymectomy. For all the children we performed Acetylcholine Receptor Autoantibody (ACHRAB) test-system producer, a chest x-ray, a chest Computed Tomography Scan (CT-scan) and a Magnetic Resonance Imaging (MRI). Thymectomy was indicated on the presence of anomalies on CT-Scan or MRI and realised via either thoracotomy or video-assisted thoracoscopy. The study was conducted with the approval of the ethics committee at our institution. Results: Based on our findings, thymectomy in JMG with imaging anomalies seems to be associated with clinical improvement. In our patients complete remission after surgery was seen in one case, a clinical improvement with a decrease of the medical therapeutic dosage was noted in three other cases. One patient could not be evaluated because of non adherence to medical treatment. Surgery by thoracotomy or video assisted thoracoscopy (VAT) was also well tolerated by all the patients. Conclusion: The benefits of thymectomy in children with JMG are still controversial. However, many researchers have reported a sustained improvement of symptoms in the majority of patients after thymectomy. Our findings seem to encourage thymectomy in cases of JMG and particularly when imaging anomalies are found. VAT thymectomy was well tolerated and it is considered by many researchers to be the standard method for thymectomy in children.

[Biliary atresia and polysplenia syndrome]

Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period [3 days] for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors [even anicteric] have biliary cirrhosis, which requires lifelong follow up

Biliary atresia: experience of a nord africain center

Background: Biliary atresia [BA] is a progressive inflammatory destructive process of the bile ducts occurring in about one of every 20.000 live births. If left untreated, biliary atresia can lead to liver failure

Aim: This is the first study on biliary atresia from Africa. The Aim of our study is to describe the clinical and prognostic aspects of biliary atresia in a Tunisian medical centre, where integrated medico-surgical management of children with liver diseases is lacking and liver transplant is not available

Methods: Patients who were diagnosed with BA and underwent portoenterostomy between January 1985 and December 2010 at a tertiary regional hospital in Tunisia were included in this analysis

Results: 74 patients were diagnosed with BA. The patients included 34 boys [45.9%] and 40 girls [54.1%]. All patients received Kasai operation as the primary treatment and the median patient age at Kasai operation was 60 days [range 3-180 days]. The median follow up time for the patients was 72 months [range 2 months-23 years 6 months]. Out of the 74 patients who received Kasai operation, 49 patients are being followed regularly in the outpatient clinic. Eight patients died immediately after Kasai operation by either hepatocellular decompensation or by cholangitis. Seventeen patients were lost to follow-up. Fifteen out of 49 patients who underwent portoenterostomy for BA are alive at median six years following Kasai intervention. Ten patients out of the 49 who are being followed regularly were Jaundice free. Two patients had portal hypertension. All these patients had survived. Five patients survived with signs of liver failure in four cases. Two of them had received a liver transplant abroad. Survival with the native liver was 6.7% at 5 years with Kasai operation alone

Conclusion: BA still has a very severe prognosis in Tunisia Reducing the age at Kasai operation remains the most important target to reduce the need for LT in infancy and childhood. Centralised care will help to build surgical expertise

Gastric perforation in the newborn

Gastric perforation in neonates is an uncommon condition. It could be spontaneous but a contributing cause should be sought. To review our experience of treating 8 neonates with gastric perforation over the past 20 years. We reviewed the records of all newborns admitted to our hospital between 1990 and 2010 with regard to gender, age at admission, contributing factors, associated anomalies, site of perforation, type of operation, and outcome. Of the eight neonates, five were female and three male. The average weight was 2130 g. Four babies were premature. Three infants were ventilated for respiratory difficulty. Five patients had associated anomalies. Perforation occurred in the lesser curvature in 4, at the greater curvature in 3, and at the anterior antrum surface in 1. All patients were treated with gastrorrhaphy. Four neonates required additional gastrostomy. Mortality was 75% [6 infants]. Active perinatal management, early treatment of primary pathologies, and protection of the stomach against distension in neonates at risk are essential in the management of neonatal gastric perforation