J Korean Surg Soc.  2011 Oct;81(4):271-275. 10.4174/jkss.2011.81.4.271.

Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy

• Jung E
• Park WH
• Choi SO

• KMID: 1445748
• DOI: http://doi.org/10.4174/jkss.2011.81.4.271

Abstract

PURPOSE
Whereas the Kasai portoenterostomy (KPE) is an accepted first line of surgery for bile drainage in infants with biliary atresia, its long-term effectiveness is not clear because its etiology and pathogenesis remains unknown. This study was aimed to investigate the late complications occurring in long-term survivors and the current status of living patients who survived over 10 years after KPE.
METHODS
A retrospective analysis of the medical records of 32 patients who underwent KPE from 1990 to 2000 was done. We analyzed 10-year survival rates with the Kaplan-Meier method and the current status of the long-term survivors.
RESULTS
The overall 10-year survival rate by Kaplan-Meier method after KPE was 76.2%. Eight (25%) patients had died, including 4 who were transplanted. Nineteen (59.4%) patients survived over 10 years. Among them, 6 (31.6%) patients had portal hypertension, and 5 (26.3%) had episodes of cholangitis. Two had intrahepatic cyst and 2 had intestinal obstruction. Six (31.6%) patients have been well without any complications.
CONCLUSION
The long-term survival rate of biliary atresia is slightly improving. However, two thirds of patients suffer from various complications. One-third of survivors go on without any complication. As biliary atresia is known as a progressive inflammatory disease, careful life-long follow- up is needed in long-term survivals after KPE.

Keyword

Biliary atresia; Hepatic portoenterostomy; Survivors; Portal hypertension; Cholangitis

MeSH Terms

Bile
Biliary Atresia
Cholangitis
Drainage
Humans
Hypertension, Portal
Infant
Intestinal Obstruction
Medical Records
Portoenterostomy, Hepatic
Retrospective Studies
Survival Rate
Survivors
Transplants

Figure

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  Fig. 1 Cumulative survival rate with native liver after Kasai operation over 10 years.

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