Sequential Liver-Kidney Transplant for Cranioectodermal Dysplasia.

Background: Cranioectodermal dysplasia (CED) is a rare ciliopathy that causes mortality through its impact on liver and kidney dysfunction. To date, there has only been a single report of a successful kidney-liver transplant in a pediatric patient with CED.

Case presentation: We present a pediatric patient who received a sequential liver-kidney transplant due to progressive organ dysfunction caused by CED. At the age of 7, the patient underwent a liver transplant, followed sequentially by a kidney transplant 5 years later. We provide a 3-year follow-up to the kidney transplantation.

Results: The liver transplant was complicated by a portal vein stricture causing portal hypertension, which required revision. The patient had no complications from the kidney transplant despite comorbidities related to the CED diagnosis, such as thrombocytopenia.

Conclusions: We discuss the viability of the sequential liver-kidney transplant for patients with CED and suggest that physicians consider this sequence if their patients with CED present with corresponding sequential organ failure.