https://www.medigraphic.com/pdfs/hepato/ah-2009/ah092h.pdf

Incidence and prognostic factors associated with biliary atresia in western India.

Rhabdomyosarcomas are the most common soft tissue tumors in children. Head and neck is one of the most frequently affected site. Within the nonparameningeal, nonorbital head and neck region, rhabdomyosarcomas of the tongue are very rare. We present a 2-year-old boy diagnosed with rhabdomyosarcoma of the tongue. The child was treated with complete surgical resection and chemotherapy. He is followed up with no evidence of disease 6 months after termination of therapy. The literature on rhabdomyosarcoma of the tongue is reviewed.

Rhabdomyosarcoma of the Tongue: Report of a Case and Review of the Literature

BACKGROUND Small ductules communicating with the bile ducts have been described at the porta hepatis in extrahepatic biliary atresia (EHBA) and these form the basis for hepatic portoenterostomy. The use of cholagogues like dehydrocholic acid (DHC) and ursodeoxycholic acid (UDCA) to enhance bile flow postoperatively has been reported. AIMS This communication describes our experience with the use of cholagogues following surgery in EHBA and attempts to correlate the outcomes with the diameter of the ductules. MATERIAL AND METHODS Fifty five EHBA patients treated by the Kasai procedure form the basis of this study; 35 patients treated during 1979-1986 and administered DHC (3-5 mg/kg) postoperatively and 20 patients treated during 1999-2002 and administered UDCA (15 mg/kg) postoperatively. The diameter of ductules was measured using an optical micrometer on 5 microm serial sections; the ducts were classified as type I (no demonstrable ducts, n = 14), type II ( 50 microm, n = 19). The clinical outcome was categorized as 1 (jaundice free survival at 5 years follow-up, n = 7), 2 (initial good response but deteriorated after one year, n = 27) and 3 (expired within one year following surgery, n = 21). The response to surgery was monitored using biochemical liver function tests (LFT), hepatobiliary scintigraphy (HIDA scan) and occurrence of cholangitis. RESULTS Age did not affect the size of ducts in both DHC and UDCA groups but patients in the DHC group were older than those treated with UDCA (mean age DHC: 105.22 +/- 33.53 days, UDCA: 74.68 +/- 23.73 days; p = 0.009). There was no statistically significant difference between duct size and postoperative LFT in both groups (DHC p = 0.1, UDCA p = 0.5). Bile excretion on HIDA scan was significantly better with larger ducts (DHC p = 0.003, UDCA p = 0.025); overall UDCA showed significantly better bile excretion (p = 0.003) but this was not reflected in the surgical outcome. There was no significant difference in the surgical outcome of those treated with DHC or UDCA but a significantly higher incidence of cholangitis was seen with smaller ducts in the UDCA group (p = 0.02). CONCLUSIONS There was no correlation between duct diameter and postoperative LFT but type III ducts were associated with better bile flow on HIDA scan. Cholangitis was seen more often with type I and II ducts in both DHC and UDCA groups. UDCA administration seemed to be beneficial in patients with type III ducts in increasing bile flow and reducing cholangitis.

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Surgical outcome in relation to duct size at the porta hepatis and the use of cholagogues in patients with biliary atresia

Fibrous remnants from the porta hepatis and wedge biopsies of the liver were studied in 60 patients with biliary atresia. The patients were divided into three groups on the basis of bile duct diameter — I: no ducts; II: 50 μm. The liver damage was classified into three grades on the basis of ductular and parenchymal histopathologic changes and extent of inflammatory pathology. There was no correlation between severity of hepatic damage and duct diameter, although the levels of serum bilirubin and alkaline phosphatase varied in direct proportion to the degree of hepatic damage. There was an inverse relationship between age and duct diameter, but neither correlated with bile flow at surgery or in the postoperative period. The data suggest that Indian children with biliary atresia follow a different clinical course compared to those from Japan and Western countries.

Bile-duct diameter at the porta hepatis and liver biopsies in Indian children with biliary atresia

An isolated bowel segment (IBS) was created in 10 rats by a two staged procedure: (1) Co-optation of the omentum to the submucosa of the bowel segment and (2) division of the mesentery of this segment 6 weeks later. Fluorescein angiography was done in 5 rats and showed that the IBS was sustained by collateral from the omentum. Histological evaluation in 5 other rats showed no adverse changes in the wall or mucous of the IBS. Stomal stenosis has been shown to be of potential advantage for the use of IBS in bowel elongation procedures.

M. K. Singh

Papers

Bile-duct diameter at the porta hepatis and liver biopsies in Indian children with biliary atresia

Creation of an isolated bowel segment by omento - enteropexy evaluation by fluorescein angiography and histopathology.