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Sequential Liver-Kidney Transplant for Cranioectodermal Dysplasia
Rebecca Berger1, Min Kim1, Leyat Tal1,2
1Baylor College of Medicine, Houston, Texas, USA.
Insights
Sequential liver-kidney transplants can be a viable option for children with Cranioectodermal dysplasia (CED), a rare genetic disorder causing organ failure. This case study highlights successful outcomes, offering hope for managing CED complications.
Area of Science:
- Pediatric Nephrology
- Hepatology
- Transplantation Immunology
Background:
- Cranioectodermal dysplasia (CED) is a rare ciliopathy associated with significant morbidity and mortality, primarily due to liver and kidney dysfunction.
- Management of CED-related organ failure remains challenging, with limited treatment options and a single prior report of successful combined kidney-liver transplantation in a pediatric patient.
Observation:
- A pediatric patient diagnosed with CED presented with progressive liver and kidney dysfunction.
- The patient underwent a sequential liver transplant at age 7, followed by a kidney transplant 5 years later.
Findings:
- The liver transplant experienced complications, including a portal vein stricture requiring revision and subsequent portal hypertension.
- Despite comorbidities like thrombocytopenia, the patient experienced an uncomplicated kidney transplant.
- A 3-year follow-up post-kidney transplantation demonstrated positive outcomes.
Implications:
- Sequential liver-kidney transplantation is a viable therapeutic strategy for pediatric patients with CED experiencing progressive, sequential organ failure.
- This approach may improve long-term survival and quality of life for CED patients.
- Further research and case studies are warranted to establish best practices for managing CED-related organ failure.
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.
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