Management of Patients with Alagille Syndrome Undergoing Living Donor Liver Transplantation: A Report of 2 Cases

Abstract

Case series

Patients: Male, 3-year-old • Female, 12-year-old

Final Diagnosis: Alagille syndrome

Symptoms: Liver failure

Medication: —

Clinical Procedure: —

Specialty: Anesthesiology

Objective:

Rare disease

Background:

Alagille syndrome (AGS) is a rare genetic disease characterized by 5 typical features: peculiar facial anomaly, posterior embryotoxon, chronic cholestasis, butterfly-like vertebral-arch defects, and cardiovascular malformations. AGS in a liver transplant setting is particularly rare in Saudi Arabia. This case report presents successful anesthetic management in AGS patients during liver transplantation surgery.

Case Reports:

We present here 2 patients with AGS who underwent liver transplantation surgery. Case 1 describes a 3-year-old boy who was diagnosed with AGS at the age of 2 weeks, manifesting as a prominent forehead, deep-set eyes, pointed chin, and butterfly-shaped vertebrae, along with coarctation of the aorta, peripheral branch pulmonary artery stenosis, direct hyperbilirubinemia, cholestasis, and G6PD deficiency. Case 2 describes a 12-year-old girl, known to have AGS, who presented with decompensated liver disease, portal hypertension, splenomegaly, hypersplenism, and portal vein thrombosis, as well as the other dysmorphic features of AGS, such as a prominent forehead, deep-set eyes, pointed chin, and butterfly-shaped vertebrae. The anesthetic management of the 2 patients started from the perioperative period with careful assessment and optimization of the cardiovascular system, intraoperative maintenance of stable hemodynamics, the use of inhaled nitric oxide when clinically indicated (used in case 1), and the use of goal-directed management of fluid as well as blood and blood products. These all played a major role in the successful management of anesthesia for these patients.

Conclusions:

The important features of successful anesthetic management included thorough preoperative cardiovascular system evaluation and intra-operative maintenance of normal temperature, ionized calcium, pH, hemoglobin, and stable hemodynamics.