Postoperative hyperammonemic encephalopathy due to unexpected constipation in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: a case report.

Pub Date : 2024-06-21 DOI:10.1186/s40981-024-00726-z

Haruka Tachibana, Nobuhiko Ohashi, Gaku Okumura, Ryusuke Tanaka, Satoshi Fuseya, Sayako Gotoh, Takashi Ishida, Sari Shimizu, Mikito Kawamata, Satoshi Tanaka

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Abstract

Background: Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare autosomal recessive urea cycle disorder associated with a high risk of exacerbation of hyperammonemia during the perioperative period. Here, we describe an adult patient with HHH syndrome who developed hyperammonemic encephalopathy secondary to postoperative constipation.

Case presentation: A 52-year-old patient with HHH syndrome underwent intrathecal baclofen pump insertion for lower limb spasticity under general anesthesia. The surgery was uneventful, without any increase in serum ammonia levels. However, after surgery, he was constipated, and on postoperative day (POD) 3, he fell into a coma with an exacerbation of hyperammonemia (894 µg/dL). After administering a glycerin enema, he defecated, leading to a rapid decrease in serum ammonia levels to 165 µg/dL. He regained consciousness, and serum ammonia levels remained stable as long as he defecated.

Conclusions: We suggest strict management of defecation during the perioperative period to prevent hyperammonemia in patients with HHH syndrome.

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高鸟氨酸血症-高氨血症-高瓜氨酸尿症综合征患者因意外便秘导致的术后高氨血症脑病：病例报告。

背景：高鸟氨酸血症-高氨血症-高瓜氨酸尿症（HHH）综合征是一种罕见的常染色体隐性尿素循环障碍性疾病，围手术期高氨血症加重的风险很高。在此，我们描述了一名患有 HHH 综合征的成年患者，该患者因术后便秘而继发高氨血症脑病：一名 52 岁的 HHH 综合征患者因下肢痉挛在全身麻醉下接受了鞘内巴氯芬泵植入术。手术过程顺利，血清氨水平没有升高。然而，术后他出现便秘，术后第 3 天（POD），他因高氨血症（894 µg/dL）加重而陷入昏迷。在进行甘油灌肠后，他排便了，血清氨水平迅速降至 165 µg/dL。他恢复了意识，只要排便，血清氨水平就能保持稳定：我们建议在围手术期严格管理排便，以防止 HHH 综合征患者出现高氨血症。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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