Peri-operative factors predicting the outcome of hepatic porto-enterostomy in infants with biliary atresia.

INTRODUCTION: Without hepatic transplantation, hepatic porto-enterostomy is the only definitive surgical therapy for infants with biliary atresia. Unfortunately, clearance of jaundice by the procedure is not promising. Pre-operative data that may predict the outcome is of great value in the selection of surgical candidates. Early post-operative determinants of outcome also help in follow-up planning. OBJECTIVE: To determine peri-operative factors influencing jaundice clearance after hepatic porto-enterostomy in infants with biliary atresia. PATIENTS AND METHOD: Clinical and laboratory data of pediatric patients undergoing hepatic porto-enterostomy in Songklanagarind Hospital from 1988 to 2001 were reviewed regarding age at operation, clinical presentation, gross pathology of bile duct atresia, liver function profiles and changes after the procedure, liver pathology and post-operative ascending cholangitis. Univariate comparison followed by multivariate logistic regression analysis was analyzed against the clearance of jaundice. Statistical analysis was aided by the Stata 7.0 program. Statistical significance was set at p-value less than 0.05. RESULTS: There were 62 infants operated on during the thirteen-year period. Four cases of operative death and a case lost to follow-up before the second post-operative month was excluded. The median age at the operation was 78 days (34-326 days). Twenty-four cases (42.1%) presented with signs of portal hypertension. After the operation, 19 cases (33.4%) were jaundice free, 6 cases (10.5%) had fair clearance and 32 cases (56.1%) had a poor result. Univariate analysis revealed an association between age at surgery and jaundice clearance. Post-operative stool color and decline of total bilirubin level at one month after surgery were significantly correlated with the outcome (p < 0.01). Cholangitis within the first post-operative month significantly had an adverse effect on the short-term survival probability. Multivariate analysis showed an independent association of jaundice clearance with age at surgery and type of bile duct atresia. CONCLUSION: Age of the infants younger than 60 days and type I of bile duct atresia were the key determinants of successful hepatic porto-enterostomy. Early cholangitis was an accelerator of progressive cirrhosis. Stool color and bilirubin level at one month after surgery can be used as predictors of jaundice clearance.

Associated genitourinary tract anomalies in anorectal malformations: a thirteen year review.

Congenital anomalies in the genitourinary tract are the leading associated anomalies in infants with anorectal malformations (ARM). Certain anomalies such as vesicoureteric reflux (VUR) may cause permanent renal damage. OBJECTIVE: To review associated genitourinary tract anomalies in-cases of anorectal malformations and evaluate the efficacy of ultrasonography in detecting VUR. MATERIAL AND METHOD: Retrospective review of 183 patients with ARM undergoing anoplasty between 1988-2001. RESULTS: Genital anomalies were found in 14 per cent (26 cases). Urologic anomalies were detected in 25.6 per cent (47 cases), with a higher incidence in supralevator anomalies. The most common upper tract anomaly was hydronephrosis, which resolved spontaneously in most of them. VUR was found in 16 cases (21 refluxing units) or 20 per cent of patients to whom voiding cystourethrogram (VCUG) was done. Sonography detected hydroureter and/or hydronephrosis in 3 of 21 refluxing units, despite 17 of them being grade three or more. Half of the cases with reflux had urinary tract infection at least once in the follow-up period despite normal initial urinalysis. Parenchymal scar was positive in four cases with VUR. CONCLUSION: Thorough evaluation of the urinary tract is necessary in infants with anorectal malformations. Ultrasound is an accurate tool in the examination of the upper tract, but not sensitive enough to detect lower tract anomalies, especially VUR.

Congenital megacolon: diagnosis using size of sub-mucosal nerve trunk as a criteria.

Cholinergic hyperinnervation in submucosa of aganglionic segment of Hirschsprung's disease has been described. However, objective measurement of the nerve fibers was insufficiently reported. OBJECTIVES: To study the amount and size of hypertrophic submucosal nerve trunks in the aganglionic segment of Hirschsprung's disease, semiquantitatively and evaluate the possibility of using the size as objective diagnostic criteria. MATERIAL AND METHOD: Thirteen specimens from aganglionic segments from Hirschsprung's disease and six specimens from the age-matched control subjected without colonic innervation disorders were studied with the S-100 immunohistochemical technic. The submucosal nerve trunks were counted per ten HPF and measured in width, semiquantitatively. Fiber sizes were stratified into small (<20 micrometers), medium (20-40 micrometers), and large (>40 micrometers). Maximum fiber size in each specimen was also recorded. RESULTS: Average density of submucosal nerve trunk in the aganglionic segment and the control were 12.5 and 3.4 fibers per ten HPF, respectively. The large nerve trunks were detected in 12 of 13 aganglionic specimens and none was detected in the controls. The maximum fiber size in aganglionic segments was 78.2 micrometers, compared to 24.1 micrometers in the control group. The sensitivity and specificity to set the presence of hypertrophic nerve trunk, larger than 40 micrometers as a diagnostic criteria for Hirschsprung's disease were 92.3 and 100 per cent, respectively. The data suggest the use of this size as an objective diagnostic criterion, which may be valuable in rectal suction biopsy specimens.