Two new case reports showed success in managing 2 patients with Alagille syndrome (ALGS) who underwent living donor liver transplantation. The authors noted that a thorough preoperative cardiovascular system evaluation and maintenance of hemodynamics, temperature, calcium levels, and pH were key to the success of the transplantations.
“The management of a multisystem disease like [ALGS] warrants a multidisciplinary approach and may require the involvement of a gastroenterologist, cardiologist, ophthalmologist, nephrologist, hepatologist, liver transplant specialist, geneticist, nutritionist, and child development expert,” the authors wrote in the American Journal of Case Reports.
“The hepatic involvement presents a particularly challenging situation as no form of phenotypic or genotypic features can predict the development of end-stage liver disease. Liver transplant remains the last resort for many of these [ALGS] patients with end-stage liver disease.”
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The patients were a 3-year-old boy and a 12-year-old girl, both with ALGS and with family members who were living donors. Both cases comprised a syndromic form of ALGS, with cholestasis, pulmonary artery stenosis, and unusual facial features.
A thorough preoperative cardiac evaluation was conducted and preoperative and postoperative echocardiography was performed in both cases. One patient needed presurgical correction of pulmonary artery stenosis. During the surgery, the patient’s body temperature was maintained in a normal range by increasing the operating room temperature to 24°C (75°F), warming transfused fluids, and using extra blankets. Hemoglobin levels were maintained via blood transfusion.
Both cases were successful and the patients were discharged from the hospital 2 weeks after the surgery. The authors note that the long-term prognosis for these patients is uncertain because of the potential impact of immunosuppressive therapy on vascular and kidney disease.
Alqahtani OJ, Ahmad RN, Abolkhair AB, et al. Management of patients with Alagille syndrome undergoing living donor liver transplantation: a report of 2 cases. Am J Case Rep. Published online August 3, 2022. doi:10.12659/AJCR.936513