Grand Rounds

Neonatal cholestasis in an infant with Rh incompatibility

Consultant in Pediatric Infectious Diseases and Pediatric Hepatology, Nanavati Hospital and Incharge Pediatric HIV, TB and Liver Clinics, B J Wadia Hospital for Children, Mumbai, India

Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056.

Clinical Problem :
A 1 ½ months old boy was referred for jaundice with clay stools since birth. There was history of Rh incompatibility which led to jaundice in neonatal period that required phototherapy and exchange transfusion in the baby. Subsequently the child was noticed to have direct jaundice. On examination, weight was 4.4 kg, he had hepatomegaly. Other systems were normal. Investigations showed bilirubin 6.2 mg, dl {direct=3.2 mg, dl}, SGOT = 10 IU, L, SGPT = 125 IU, L, total proteins = 5.1 gm, dl, albumin= 3.1 gm, dl, alkaline phosphatase = 626 IU, L, GGTP = 250 IU, L. HIDA scan did not show excretion of tracer into small intestine even after 24 hours. An intraoperative cholangiogram {IOC} showed free flow of dye into intestines. Liver biopsy showed minimal intrahepatic and intra-canalicular cholestasis with no bile duct proliferation and mild to moderate cholangitis. After flushing the system after IOC, and supplementing with Ursodeoxycholic acid the liver function test subsequently normalized. At last follow-up at 2 years of age, liver function tests were normal and there was no portal hypertension on doppler.

What is the likely cause of neonatal cholestasis_?

Discussion :
Since the commonest cause of neonatal cholestasis with clay stools is biliary atresia. In presence of HIDA showing no excretion of tracer even after 24 hours, one should rule out biliary artresia. However, one should also remember that alloimmune Rh hemolytic disease of newborn {HDN} has been reported to be a significant risk factor for cholestasis with the published prevalence of cholestasis in neonates with Rh HDN being 13–60 percent. {1} Because of excessive hemolysis, the excess bilirubin can densify as calcium bilirubinate sludge in bile ducts, leading to cholestasis {inspissated bile duct syndrome}. {2} Features suggestive of inspissated bile duct syndrome include dilated bile ducts with sludge or echogenic material in the lumen, raised levels of liver enzymes including GGT, the non-excretory HIDA scan and the response to UDCA as was seen in our patient. Liver biopsy in our patient was classical of inspissated bile duct syndrome. Other predisposing factors for the development of inspissated bile in neonates include prematurity, parenteral nutrition, sepsis and diuretic therapy. {2}

References :
  1. Smits-Wintjens VE, Rath ME, Lindenburg IT, Oepkes D, van Zwet EW, Walther FJ, Lopriore E. Cholestasis in neonates with red cell alloimmune hemolytic disease: incidence, risk factors and outcome. Neonatology. 2012; 101(4):306-10.
  2. Miloh T, Rosenberg HK, Kochin I, Kerkar N. Inspissated bile syndrome in a neonate treated with cefotaxime: sonographic aid to diagnosis, management, and follow-up. J Ultrasound Med. 2009 Apr; 28(4):541-4.

Correct Answers :  yes 28%
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