Children with clinical presentation of Hirschsprung's disease - a clinicopathological experience

Enteric neuronal abnormalities include Hirschsprung's Disease [HD], hypoganglionosis, intestinal neuronal dysplasia [IND Type A and B], and immaturity of ganglion cells. All of these are mostly present with the same clinical features of the HD. A total of 92 patients presented with the clinical features of HD were recruited to this study from two tertiary care Children Hospitals from March 2009 to October 2009. They either had their first presentation or called for definitive surgeries with clinical presentation of HD. After applying exclusion criteria, 84 patients were finally left for the study. Among 84 patients, 13 [15.5% proved to be normally ganglionic on rectal biopsies and 71 [84.5%] showed enteric neuronal abnormalities. In these, 51 [71.8%] children had Hirschsprung's disease, 9 [12.7%] revealed immaturity of ganglion cells [IGC], 9 [12.7%] belonged to isolated hypoganglionic group and 2 [2.8%] showed isolated IND type B. In HD group, M: F ratio was 4.1:1, mean age at diagnosis was 1.9 years and the presenting complaints were in the descending order i.e., abdominal distention, constipation, vomiting and delayed passage of meconium. Among the group that showed IGC, M: F ratio was 2: 1, mean age at diagnosis was 12 days of life. The presenting complaints were same as that in HD in a similar order. In isolated hypoganglionic group, M: F ratio was 3.5.: 1, mean age at diagnosis was 2.3 years. The presenting complaints included constipation [88.9%], abdominal distension [77.8%], vomiting [33.3%] and delayed passage of meconium [33.3%]. In isolated IND type B, both patients were males, they came with constipation, abdominal distension and vomiting whereas one of them had history of delayed passage of meconium. HD was the most common enteric neuronal abnormality. Other neuronal abnormalities included isolated hypoganglionosis, immaturity of ganglion cells and isolated hyperganglionos [IND Type B]. Most common presenting complaints were abdominal distension, constipation, vomiting and delayed passage of meconium in all the groups

Biliary Atresia: Liver histology and its relation to the age of the patient

There are gross and histological changes in liver with increasing age in patients with biliary atresia. These changes have inverse relation to the outcome after surgery. There are geographical differences in the histological changes and severity as well as outcome of patients with biliary atresia. This study determines the histological changes in liver biopsy specimen taken during surgery on patients with biliary atresia from various age groups. No such data on Pakistani population is available. All children, total number 42, with age from one month and above, diagnosed to have biliary atresia, were studied. It is a single center, prospective, interventional study over a period of 5 years. Spearman correlation was used to find a correlation between the age of the patient and severity of histological variables. The only constant histological finding that evolved with increasing age was cirrhosis of liver. It was not present up to the age of 2 months. 57.1% of patients from 2 months to 2.5 months and 62.5% of patients up to the age of 3 months had cirrhosis of liver. It was statistically significant with a p value =0.004. Other statistically significant variables were, portal fibrosis with a p value =0.005, bile duct proliferation with a p value =0.002 and portal cholestasis with a p value =0.024. Onset of cirrhosis impairs the prospects of a good recovery. Significant changes take place in liver histology in patients with biliary atresia with increasing age.Early diagnosis and prompt treatment is necessary for a satisfactory outcome. The findings in our population are very similar to the results by many other workers and the onset of cirrhosis appears to be rather earlier in this small group of patients

Routine bilateral groin exploration in patients with unilateral inguinal hernia

381 of 388 clinically unilateral inguinal herniae in children underwent bilateral groin exploration. 64% of the 203 right and 96% of the 178 left inguinal herniae had a contralateral hernia. The complications encountered were death in 1 [associated multiple congenital abnormalities] wound infection in 5 [1.3%], and recurrence and injury to the vas in 1 case each. Contralateral exploration was declared negative only after a joint decision with a consultant