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.