Sanada Yukihiro, Naya Itsuki, Katano Takumi, Hirata Yuta, Yamada Naoya, Okada Noriki, Ihara Yoshiyuki, Onishi Yasuharu, Mizuta Koichi
Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Tochigi, Japan.
Pediatr Transplant. 2019 Mar;23(2):e13352. doi: 10.1111/petr.13352. Epub 2019 Jan 9.
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.
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.
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/1.73m ).
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.
颅内和肺血管异常是阿拉吉斯综合征（AGS）中众所周知的并发症和死亡原因；然而，内脏动脉异常较少被认识到。在此，我们对小儿活体肝移植（LDLT）经验进行回顾性分析，重点关注LDLT后AGS内脏动脉异常的当前问题及治疗方法。
2001年5月至2017年12月期间，为285名小儿受者进行了294例LDLT。其中，为12例AGS患者进行了13例LDLT（4.4%）。我们将内脏动脉异常分为动脉瘤和狭窄。
12名受者中有2名（16.7%）发生内脏动脉瘤，其中1例为肠系膜上动脉（SMA）动脉瘤，1例为胰十二指肠下动脉（IPDA）动脉瘤并继发脾动脉（SPA）动脉瘤；内脏动脉瘤诊断时的年龄分别为16.3岁、21.1岁和21.7岁。对一个进行性IPDA囊状动脉瘤（12.0×14.5×15.0mm）进行了血管内治疗。12名受者中有7名（58.3%）发生内脏动脉狭窄，内脏动脉狭窄诊断时的中位年龄为15.5岁（范围1.7 - 22.9岁）。所有3例患有肾动脉（RA）狭窄的AGS患者均患有肾功能不全（估算肾小球滤过率分别为51、78和51mL/min/1.73m²）。
内脏动脉异常的发病率不可忽视。即使对于婴儿，定期进行影像学检查也是必要的，因为在婴儿期很难检测到内脏血管异常。
