Abstract:

BACKGROUND: Hepatic hereditary hemorrhagic telangiectasia (HHHT) is a rare disease, which can lead to life-threatening complications. Liver transplantation has been an only curative option; however, the therapeutic effect of liver transplantation in HHHT is rarely reported.
OBJECTIVE: To explore the therapeutic effect of liver transplantation in patients with HHHT.
METHODS: The clinical data of one 59-year-old female patient with HHHT, who had undergone orthotropic liver transplantation at the Institute of Liver Transplantation of General Hospital of Chinese People’s Armed Police Forces, China was analyzed retrospectively. The period of follow-up was 8 months. Abdominal ultrasonography and liver function were observed after operation.
RESULTS AND CONCLUSION: This female patient had been in good health during 8-month follow-up after liver transplantation. She resumed family daily life. Liver transplantation can offer an effective therapy for patients with HHHT, and can provide satisfactory postoperative long-term results. Liver transplantation should be proposed earlier in the course of symptomatic HHHT presenting with life-threatening conditions. Palliative interventions, especially on the hepatic artery, should be avoided in view of their high (infectious) complication rate. However, the sample size was small with relatively short time follow-up. Thus, the long-term therapeutic effect still needs to be explored.