Abstract

Biliary atresia (BA) is a rare disease characterized by biliary obstruction in which the etiology remains obscure. In this report we describe a case of 40 days old female child fully breastfed that had not received vitamin K prophylaxis after birth. The latter presented to paediatric emergency department with severe jaundice, abnormal neurological exam and hepatomegaly, head CT scan was suggestive of massive left temporal intra parenchymatous and sub-Arachnoid hemorrhage related to vitamin k deficiency, neonatal cholestasis has been found to be related to biliary atresia which has led to vitamin k deficiency. After the enfant has recovered neurologically he has been referred to paediatric surgery team where a Kasai Portoenterostomy procedure has been done timely. Thus biliary atresia should be considered in all patients with cholestasis because early diagnosis could prevent severe complications and results in a better prognosis.

Keywords: Biliary Atresia; Intracranial Hemorrhage; Vitamin K Deficiency; Kasai Portoenterostomy; Neonatal Cholestasis

References

1. Liesbeth Claire Faverey and Yvan Vandenplas. “Hemorrhagic Diathesis as the Presenting Symptom of Neonatal Cholestasis”. Pediatric Gastroenterology, Hepatology and Nutrition 3 (2014): 191-195.
2. Petersen C and Davenport M. “Aetiology of biliary atresia: what is actually known?” Orphanet Journal of Rare Diseases 8 (2013): 128.
3. Miyao M., et al. “Subdural hemorrhage: A unique case involving secondary vitamin K deficiency bleeding due to biliary atresia”. Forensic Science International 221 (2012): e25-e29.
4. Ijland MM., et al. “Incidence of late vitamin K deficiency bleeding in newborns in the Netherlands in 2005: evaluation of the current guideline”. European Journal of Pediatrics 167 (2008): 165-169.
5. Chardot C. “Biliary atresia”. Orphanet Journal of Rare Diseases 1 (2006): 28.
6. Hussein A., et al. “Kasai portoenterostomy: new insights from hepatic morphology”. Journal of Pediatric Surgery 40 (2005): 322-326.
7. Tang ST., et al. “Diagnosis and treatment of biliary atresia: a retrospective study”. Hepatobiliary and Pancreatic Diseases International 4 (2005): 108-112.
8. Uchida K., et al. “Predicting factor of quality of life in long-term jaundice-free survivors after the Kasai operation”. Journal of Pediatric Surgery 39 (2004): 1040-1044.
9. Moyer V., et al. “North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition”. Journal of Pediatric Gastroenterology and Nutrition 39 (2004): 115-128.
10. Roberts EA. “Neonatal hepatitis syndrome”. Seminars in Neonatology 8 (2003): 357-374.
11. McKiernan PJ. “Neonatal cholestasis”. Seminars in Neonatology 7 (2002): 153-165.
12. Schlesinger AE., et al. “Hepatobiliary system”. In Kuhn JP, Slovis TL, Haller JO (editions): Pediatric Diagnostic Imaging, edition 10. St. Louis, Mosby (2004): 1445-1517.
13. Dellert S and Balistreri W. “Chapter 52 - Neonatal Cholestasis”. In: Walker W, Durie P, Hamilton J., et al. “Pediatric Gastrointestinal Disease, third edition. Lewiston, NY: B.C. Decker Inc (2000): 880-891.
14. Sato H., et al. “Intracranial hemorrhage due to vitamin K deficiency associated with congenital biliary atresia: a case report”. Neurosurgical Emergencies 5 (2000): 77-80.
15. Fischler B., et al. “The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia”. Journal of Pediatric Gastroenterology and Nutrition 27 (1998): 57-64.
16. Van Den Anker JN and Sinaasappel M. “Bleeding as presenting symptom of cholestasis”. Journal of Perinatology 13 (1993): 322-324.
17. Chang MH., et al. “Polymerase chain reaction to detect human cytomegalovirus in livers of infants with neonatal hepatitis”. Gastroenterology 103 (1992): 1022-1025.
18. Ryan CA and Gayle M. “Vitamin K deficiency, intracranial hemorrhage, and a subgaleal hematoma: a fatal combination”. Pediatric Emergency Care 8 (1992): 143-145.
19. Fukuda T., et al. “Intracranial hematoma accompanying bleeding tendency: therapeutic practice and analysis of literature (in Japanese)”. No Shinkei Geka 18 (1990): 511-520.
20. Chaou WT., et al. “Intracranial hemorrhage and vitamin K deficiency in early infancy”. The Journal of Pediatrics 105 (1984): 880-884.