Successful combined liver and kidney transplant for COACH syndrome and 5-yr follow-up

Tadahiro Uemura, Edmund Q. Sanchez, Toru Ikegami, David Watkins, Gomathy Narasimhan, Greg J. McKenna, Srinath Chinnakotla, Sherfield Dawson, Henry B. Randall, Marlon F. Levy, Robert M. Goldstein, Goran B. Klintmalm

Research output: Contribution to journalArticlepeer-review

13 Citations (Scopus)


The COACH syndrome is a very rare disorder with cerebellar vermis hypoplasia, oligophrenia, ataxia, coloboma, and hepatic fibrosis. Nineteen cases with COACH diagnosis have been reported. Neurologic abnormalities are the first symptoms in most cases. Complications of the hepatopathy [portal hypertension, esophageal varices, and gastrointestinal (GI) bleeding] contribute extensively to the morbidity and lethality in the course of the disease. We describe a 28-yr-old female with COACH syndrome resulting in chronic renal and hepatic insufficiency. The patient was found to have significant mental retardation, truncal ataxia, motor abnormality and occulomotor abnormality. She began to develop GI bleeding and encephalopathy because of biopsy-confirmed cirrhosis. We performed combined liver and kidney transplant after challenging discussion. Her postoperative course was uneventful, and she was discharged on the ninth postoperative day (POD). She has not had any problems at 1, 3 and 5-yr follow-up with excellent liver and renal function. This is the first description of successful combined liver and kidney transplant with longterm follow-up. The decision for transplant is challenging because COACH syndrome is rare with only descriptive characterization and patients have non-progressive ataxia and mental retardation. However, our case shows that liver and kidney transplant can be medically successful, and the individuals achieve long-term success if they have a stable neurological condition and an excellent support system.

Original languageEnglish
Pages (from-to)717-720
Number of pages4
JournalClinical Transplantation
Issue number6
Publication statusPublished - Dec 2005
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Transplantation


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