Biliary atresia: 20-40-year follow-up with native liver in an Italian centre.

INTRODUCTION: Biliary atresia (BA) represents the leading indication for liver transplantation in childhood. Only few studies reported the outcome of patients who survived more than 20 years on their native liver, and up to date there are no Italian data available. We reported our 40-year single centre experience with long-term follow-up of BA patients. MATERIALS AND METHODS: All consecutive patients who underwent Kasai portoenterostomy (KPE) for BA managed at our Institution between 1975 and 1996 were retrospectively reviewed. Native liver (NLS) and overall survival (OS) were analyzed with Kaplan-Meyer curves and LogRank test. A p value of <.05 was regarded as significant. Quality of life of patients currently surviving with their native liver was assessed through a quality of life questionnaire. RESULTS: During the 22-year period of the study 174 patients underwent surgery (median age 60 days). Clearance of jaundice at 6 months from surgery was achieved in 90 patients (51.7%). NLS was 41% at 5 years, 32% at 10 years, 17.8% at 20 years and 14.9% at 40 years. Cholangitis was recorded in 32%, hepatocellular carcinoma in 0.5%. Twenty-six patients (14.9%) survived with their liver more than 20 years; 84.6% had normal serum bilirubin level and 23% had esophageal varices. Quality of life was comparable with the healthy Italian population in all but one patient. CONCLUSIONS: Our Italian experience confirms KPE represents the cornerstone of treatment for children with BA. Multidisciplinary and meticulous lifelong post-operative follow-up should be guaranteed for these patients because of the possibility of late-onset cholangitis, portal hypertension, hepatic deterioration and liver malignant tumors. TYPE OF THE STUDY: retrospective case series. LEVEL OF EVIDENCE: IV.

"Trident" preputial plasty for phimosis in childhood.

OBJECTIVE: To evaluate the functional and cosmetic result of modified-triple incision preputial plasty for surgical management of phimosis in children. MATERIALS AND METHODS: This prospective study involved all consecutive children (age 3-15) with phimosis, managed at our Institution in a one-year period. All patients unable to retract the foreskin and symptomatic with episodes of posthitis or balanoposthitis or ballooning of the foreskin with voiding were initially included. After a two-month trial of topic corticosteroids treatment, for patients refractory to conservative ointment treatment, "trident" preputial plasty was offered. This procedure combines two lateral Z-plasty and one middle Y-V plasty. All the procedures were performed as a day care, in general anaesthesia by two senior surgeons. Outpatient follow-up controls were scheduled at 1 and 2 week, 1, 6 and 12 months after surgery, respectively. RESULTS: A total of 41 patient were enrolled. Mean operative time was 24 minutes (range 15-43). At 12-month follow-up, all but one patients (97.6%) was able to retract the prepuce. Cosmetic and functional results were satisfactory. CONCLUSION: In selected group of children with phimosis, the "trident" preputial plasty provided excellent cosmetic and functional result. Adequate retraction of the prepuce by the patients soon after surgery is mandatory.

Colonic stricture mimicking Hirschsprung's disease: a localized cytomegalovirus infection.

The diagnosis of congenital cytomegalovirus (CMV) infection is best established by isolating the virus from urine or saliva within the first 2 weeks of life. Detection beyond this point may reflect perinatal acquisition of CMV. Cytomegalovirus is usually transmitted by direct human-to-human contact through vertical or horizontal routes. Infected persons can excrete CMV in urine, saliva, semen, cervical secretions, or breast milk. Because the virus establishes latent infections, blood products and solid organs can also transmit CMV. In the surgical literature, intestinal CMV-infected cells in infants have prevalently been associated with neonatal necrotizing enterocolitis, and only very few cases of primary CMV infection of the gastrointestinal tract of surgical interest in immunocompetent neonates have been reported. We describe a neonate with congenital or perinatal CMV infection with gastrointestinal involvement who developed a colonic stricture and manifested a clinical picture simulating Hirschsprung's disease. The intestinal lesion was a localized segmental CMV infection of the colon in which inflammation dominated the histopathologic finding. Chorioretinitis was also present.