Mega-choledochus - Is it pathologically Similar to Megacolon? (Surgical Hypothesis)

Rajah Shunmugam, Vinodh Mutyala Kumar, Vinodh Suppiah, Ahmad Toha

Department of Pediatric Surgery, Gleneagles Hospital, Kota Kinabalu, Sabah, Malaysia.

Keywords

Aganglionosis

Bile duct pathology

Choledochal cyst

Congenital megacolon

Enteric nervous system

Etiopathogenesis

Abbreviations

CDC - Choledochal cyst

CBD - Common bile duct

CHD - Common hepatic duct

Abstract

The traditional classification and etiopathogenic theory of choledochal cyst (CDC) has recently been challenged. A 16-month-old baby girl presented with jaundice, abdominal distention and clay colored stools for 2 weeks. Investigations revealed a Type IVA CDC. At laparotomy, a hugely distended biliary apparatus with an apparent blind distal end was noted. Excision of the CDC and Roux-en-Y hepaticojejunostomy was performed. Her recovery is marked by a transient ascites for just 2 weeks. Histopathology of the excised distal bile duct showed hypertrophied nerve bundles similar to Hirschsprungs disease. Ultrasonography after 4 mo of surgery revealed complete regression of intra-hepatic duct dilation. From these, it appears that Todani Type I and Type IVa CDC may be just variations of the same disease.