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Published on: July 5, 2024
Visceral artery anomalies in patients with Alagille syndrome
Yukihiro Sanada1, Itsuki Naya1, Takumi Katano1
1Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Tochigi, Japan.
Insights
Visceral artery anomalies, including aneurysms and stenosis, affect many pediatric patients undergoing liver transplants for Alagille syndrome (AGS). Early detection through imaging is crucial for managing these complications.
Area of Science:
- Vascular Surgery
- Pediatric Gastroenterology
- Transplant Surgery
Background:
- Alagille syndrome (AGS) commonly presents with intracranial and pulmonary vascular anomalies.
- Visceral artery anomalies are less recognized but significant complications in AGS patients.
Purpose of the Study:
- To analyze the incidence and management of visceral artery anomalies in pediatric patients with AGS undergoing living donor liver transplantation (LDLT).
Main Methods:
- Retrospective analysis of 13 LDLTs performed on 12 pediatric AGS patients between May 2001 and December 2017.
- Classification of visceral artery anomalies into aneurysms and stenosis.
Main Results:
- Visceral aneurysm incidence was 16.7% (2/12), including SMA and IPDA aneurysms.
- Visceral artery stenosis incidence was 58.3% (7/12), with 3 patients experiencing renal dysfunction due to renal artery stenosis.
- Endovascular treatment was used for a progressive IPDA saccular aneurysm.
Conclusions:
- Visceral artery anomalies pose a significant morbidity risk in pediatric AGS patients post-LDLT.
- Regular imaging is essential for early detection, even in infants, due to the challenges in identifying anomalies early.
Background:
Intracranial and pulmonary vascular anomalies are well-known complications and causes of mortality in AGS; however, visceral artery anomalies are less commonly recognized. Herein, we present a retrospective analysis of our experience with pediatric LDLT that focuses on the current problems with and treatments for visceral artery anomalies in AGS after LDLT.
Methods:
Between May 2001 and December 2017, 294 LDLTs were performed for 285 pediatric recipients. Of these, 13 LDLTs (4.4%) for 12 AGS patients were performed. We classified the visceral artery anomalies into aneurysms and stenosis.
Results:
The overall incidence of visceral aneurysm was 2 of 12 recipients (16.7%) and included a SMA aneurysm in one patient and an IPDA aneurysm with a subsequent SPA aneurysm in one patient; the ages of the diagnosis of visceral aneurysm were 16.3, 21.1, and 21.7 y, respectively. An endovascular treatment was performed for a progressive IPDA saccular aneurysm (12.0 × 14.5 × 15.0 mm). The overall incidence of visceral artery stenosis was 7 of 12 recipients (58.3%) and the median age at the diagnosis of visceral artery stenosis was 15.5 y (range 1.7-22.9 y). All 3 AGS patients with RA stenosis suffered from renal dysfunction (eGFR of 51, 78, and 51 mL/min/1.73m2 ).
Conclusion:
The morbidity of visceral artery anomalies is not negligible. The performance of periodic imaging examinations is necessary, even for infants, because it is difficult to detect visceral vascular anomalies in the infant stage.
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