Dr. Terencia, Dr. C Jayakumar, Dr Bhanu vikraman Pillai, DrNaveenViswanath ,Dr. Praveena Bhaskaran, Dr Navya George
AIMS Kochi.
Forty five old infant with seccundum ASD presented with yellowish discoloration of sclera, trunk, hands, palms and soles from 2 weeks of life and high coloured urine and pale stools .There is no other relevant history
Clinically child had Yellowish discoloration over sclera, body, trunk and extremities and 2cm firm liver below the right coastal margin
Differentials
Neonatal hepatitis
Biliary atresia
Choledochous cyst
Intrauterine infections
Sepsis
Urinary tract infection
Dubin John son
Rotor syndrome
Labs
WBC-13.15K/Ul, N/L-29.4%/56.2%, HB-9.0g/dl, Platelets-599k/Ul
LFT-TSB/DSB-13.24/9.50, AST/ALT -217/157,
PT-INR-12.60/14.0/0.88
GGT-1038U/L
LFT showed conjugated hyperbilirubinemia with transaminitis and GGT was highly elevated.
USG Abdomen partially distended Prefeed gallbladder with clear Lumen. No wall abnormality. Post feed gallbladder shows limited contractility.
Figure 1,2 and 3-HIDA scan at 5min, 10min and 24 hours
Hepatobiliary scintigraphy showed prompt extraction of iminodiacetic acid acid hepatocytes suggestive of preserved hepatocellular function.
Non progression of tracer into intestinal loops even after 24 hours delayed imaging is suggestive of biliary atresia.
Child was taken up for Kasai portoenterostomy
Intraoperative findings showed coarse liver surface, Atretic gall bladder. Intra operative cholangiogram done showed contrast going to GB, no progression of contrast to CBD CHD and CBD with fibrosed Atretic extra hepatic biliary tree.
Post operative period was uneventful. Biopsy features are consistent with a large duct obstruction and nodule formation and Gall bladder with fibrous cone
Atretic gall bladder Porta hepatic remnants- Gautier and Eliot Type 3.(Altered bile ducts lined columnar epithelium )
He was kept Nil orally for 5 days after the surgery.
Clear sips followed liquid diet and then gradually full feeds were given POD-7. Drain was removed on POD-8. At the time of discharge, he was tolerating full feeds, symptoms have reduced and wound looked healthy.
Biliary atresia(BA) is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period and is the most common indication for liver transplantation in children.
Genetic factors may play an important role in etiology but infectious, toxic or immunologic mechanisms are also probably involved.
Most infants are born at fullterm with normal Birth weight and initially thrive and seem helathy. Scleral icterus and/or generalised jaundice pale stools high colored urine are usually over looked so also the haepato splenomegaly
Labs will reveal elevated conjugated bilirubin(>2mg/dl) and mild or moderate elevations in serum aminotransferases with disproportionately increased GGT.
Diagnosis is made imaging ,laboratory tests and liver biopsy to exclude other causes of cholestasis.
Definitive diagnosis is made cholangiogram performed intraoperatively and if biliary atresia is confirmed surgeon performs hepatoportoenterostomy.
Kasai surgery is recommended in all infants as soon as the diagnosis is made, preferably before 60days of age. Infants should be supplemented with high calorie formula and fat soluble vitamins and should be monitored for fat soluble vitamin deficiencies. They should be treated with Ursodeoxycholic acid after kasai
Treating them with prophylactic antibiotics for at least 1 year after kasai to reduce risk of ascending cholangitis as it will hasten the progression of liver disease. Patients with persistent jaundice 3 months after kasai HPE should be referred for liver transplantation evaluation.
Fate of BA-At least 60 to 80% of patients with BA will eventually require liver transplantation. A minority treated with kasai HPE will survive to 20 years or more. However many of these patients have chronic liver disease with cirrhosis and portal HTN.
Carry home message-Its essential to pick out icterus, pale stools and high cooled urine at the earliest
All mothers must be well informed about this
and bringing a picture of the stool and collected urine is a really worth to rule out obstruction
Golden time for Kasai is before sixty days
Infact even five days of phenobarbitone priming to do IDA Scan may also be a luxury for these children