The Budd-Chiari syndrome in children: the spectrum of management.
The Budd-Chiari syndrome (BCS), characterized by hepatic venous outflow occlusion, is rare in children. We describe the cases of 3 patients with BCS who presented at different stages of liver dysfunction that required tailored management. A 10-year-old boy who presented with a weeklong history of abdominal discomfort received an early diagnosis. Treatment with thrombolytic therapy and adjunctive percutaneous interventional radiologic management led to a favorable outcome in this patient. A 2-year-old girl referred with a 6-week illness not amenable to thrombolytic therapy was managed with a pericardial patch atriocavoplasty to restore hepatic venous outflow tract patency. The third patient, a 5-year-old girl, presented with established cirrhosis and massive ascites. After we controlled the ascites with an extracorporeal shunt system and nutritional resuscitation, we conducted an orthotopic liver transplantation on this patient. The stage of disease at diagnosis influences the management strategy, and an early diagnosis offers patients the best possible chance of cure without major surgery. However, a later presentation can still be successfully managed by more radical surgical interventions. The aims of this case report were to emphasize the importance of a high index of suspicion in the diagnosis of pediatric BCS and to highlight the importance of early referral to a specialized pediatric liver unit with the necessary facilities to deal with the medical and surgical aspects of management.
Cauchi
John A
JA
The Liver Unit, Birmingham Children's Hospital B4 6NH, Birmingham, UK.
Oliff
Simon
S
Baumann
Ulrich
U
Mirza
Darius
D
Kelly
Deirdre A
DA
Hewitson
John
J
Rode
Heinz
H
McCulloch
Mignon
M
Spearman
Wendy
W
Millar
Alastair J W
AJ
D001201:Ascites / Q000209:etiology / Q000601:surgery; D006502:Budd-Chiari Syndrome / Q000150:complications / Q000628:therapy*; D013504:Cardiovascular Surgical Procedures; D002648:Child; D002675:Child, Preschool; D005260:Female; D005343:Fibrinolytic Agents / Q000008:administration & dosage; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D010496:Pericardium / Q000637:transplantation; D015641:Radiography, Interventional; D010959:Tissue Plasminogen Activator / Q000008:administration & dosage
D005343:Fibrinolytic Agents; D010959:Tissue Plasminogen Activator