Postoperative hyperammonemic encephalopathy due to unexpected constipation in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: a case report.
JA Clin Rep
; 10(1): 42, 2024 Jun 21.
Article
en En
| MEDLINE
| ID: mdl-38904738
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.
Texto completo:
1
Colección:
01-internacional
Idioma:
En
Revista:
JA Clin Rep
Año:
2024
Tipo del documento:
Article
País de afiliación:
Japón