RESUMO
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 followup 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.
RESUMO
Ureterocele is a pseudo-cyst dilation of the terminal submucosal ureter. It is a rare malformative uropathy, in particular associated with simplex ureter. We conducted a retrospective study over a period of 10 years. Twelve medical records of patients whose data were collected at the Department of Paediatric Surgery of Monastir between 2006 and 2016 were examined. The average age of patients was 2.7 years (ranging from 7 days to 11 years) with a sex ratio of 1. Patients´ clinical status was dominated by fever due to upper urinary tract infection. Diagnosis was mainly based on renal bladder ultrasound, intravenous urography (IVU) and retrograde urethrography and cystography. Ureterocele was unilateral in 10 cases and bilateral in 2 cases (on a total of 14 cases). It was associated with simplex ureter in all cases and all patients underwent endoscopic surgery. No perioperative adverse event was reported. The postoperative course was uneventful. Clinical and radiological improvements were reported in all cases. Ureterocele associated with simplex ureter is a very rare urinary abnormality. Early diagnosis is essential to avoid upper urinary tract involvement. Endoscopic treatment is a good alternative leading to satisfactory results.