Morita Keiichi, Fukuzawa Hiroaki, Maeda Kosaku
Department of Pediatric surgery, Kobe Children's Hospital, Kobe, Japan.
Pediatr Int. 2015 Dec;57(6):1187-9. doi: 10.1111/ped.12711. Epub 2015 Sep 10.
The first-choice therapy for biliary atresia (BA) is Kasai hepatoportoenterostomy, which has been shown to greatly improve outcome. Various long-term complications, however, such as portal hypertension and hepatopulmonary syndrome (HPS), can occur in patients with native liver. A rare case of brain abscess in an 11-year-old girl with HPS associated with BA is reported. The patient underwent hepatoportoenterostomy for BA at 53 days of age, with resolution of hyperbilirubinemia. At 10 years of age, she was diagnosed with severe HPS with right-to-left shunting, and preparations for liver transplantation proceeded. Three months after the diagnosis, she had a right parietal brain abscess. Given that the brain abscess enlarged in size, surgical drainage of the brain abscess was performed. The postoperative course was uneventful, but a slight left hemiplegia remained at discharge. The presumed mechanism of abscess formation in HPS may be right-to-left bacterial transit through intrapulmonary vascular dilatations and/or arteriovenous fistulae.
胆道闭锁（BA）的首选治疗方法是Kasai肝门肠吻合术，该手术已被证明能显著改善治疗效果。然而，患有天然肝脏的患者可能会出现各种长期并发症，如门静脉高压和肝肺综合征（HPS）。本文报道了一例11岁患有与BA相关的HPS的女孩发生脑脓肿的罕见病例。该患者在53日龄时因BA接受了肝门肠吻合术，高胆红素血症得以缓解。10岁时，她被诊断为伴有右向左分流的严重HPS，并开始准备肝移植。诊断后三个月，她出现了右顶叶脑脓肿。鉴于脑脓肿体积增大，遂对其进行了手术引流。术后病程平稳，但出院时仍遗留轻度左侧偏瘫。HPS中脓肿形成的推测机制可能是细菌通过肺内血管扩张和/或动静脉瘘发生右向左转移。
