PMID- 15598338
OWN - NLM
STAT- MEDLINE
DCOM- 20050331
LR  - 20041215
IS  - 1397-3142 (Print)
IS  - 1397-3142 (Linking)
VI  - 8
IP  - 6
DP  - 2004 Dec
TI  - Answered and unanswered controversies in the surgical management of extra hepatic 
      biliary atresia.
PG  - 628-31
AB  - Almost a half-century since Kasai described the portoentersotomy for extrahepatic 
      biliary atresia (EHBA), some questions about the management of this condition 
      have been resolved and many are unanswered. The most useful diagnostic steps to 
      aid in the diagnosis are debated. Sonography can be helpful but its sensitivity 
      and selectivity are arguable with strong advocates for its effectiveness. 
      Likewise, the magnetic resonance imaging has forceful advocates but also has not 
      been universalized. The liver biopsy, done commonly before an operation for 
      cholestasis, is often not discriminating. The radionuclide scan hepatobiliary 
      iminodiacetic acid (HIDA) scan after phenobarbital stimulation is helpful if 
      negative, but false positive results are common. It is agreed to proceed 
      expeditiously to the operation in the cholestatic infant after a prompt 
      investigation. The proposal to avoid this step and provide liver transplantation 
      as initial management for EHBA has been suppressed by several clinical findings. 
      The Kasai procedure has not worsened the outcome of eventual liver 
      transplantation. The Kasai, even if it eventually fails, will often buy time and 
      allow the child to grow before transplantation is needed. Multiple reoperations 
      prior to the transplant are discouraged. Revisions to improve bile flow have not 
      gained wide popularity. Use of a stoma to divert the bile has been largely 
      abandoned. The need for frozen section examination of the liver at the site 
      chosen for the portoenterostomy is no longer demanded. The preferred type of 
      intestinal conduit is argued. Unanswered questions about the post-operative 
      management include the role of steroids and of prophylactic antibiotics. The 
      Biliary Atresia Research Consortium, a multi-institutional National Institutes of 
      Health (NIH)-supported project, will address many of the unanswered issues.
FAU - Dolgin, Stephen E
AU  - Dolgin SE
AD  - Pediatric Surgery, Mt. Sinai Medical Center, New York, NY 10029, USA. 
      stephen.dolgin@mountsinai.org
LA  - eng
PT  - Journal Article
PL  - Denmark
TA  - Pediatr Transplant
JT  - Pediatric transplantation
JID - 9802574
SB  - IM
MH  - Biliary Atresia/diagnosis/*surgery
MH  - Child
MH  - Cholangitis/etiology/physiopathology
MH  - Humans
MH  - Liver Transplantation
MH  - *Portoenterostomy, Hepatic/adverse effects
MH  - Prospective Studies
MH  - Treatment Failure
EDAT- 2004/12/16 09:00
MHDA- 2005/04/01 09:00
CRDT- 2004/12/16 09:00
PHST- 2004/12/16 09:00 [pubmed]
PHST- 2005/04/01 09:00 [medline]
PHST- 2004/12/16 09:00 [entrez]
AID - PTR243 [pii]
AID - 10.1111/j.1399-3046.2004.00243.x [doi]
PST - ppublish
SO  - Pediatr Transplant. 2004 Dec;8(6):628-31. doi: 10.1111/j.1399-3046.2004.00243.x.