The perioperative management of a patient with lowe syndrome for general anesthesia: A case report.
10.4097/kjae.2009.56.1.112
- Author:
Jin Yong CHUNG
1
;
Jae hyoun KWON
;
Kwi Chu SEO
;
Seok Young SONG
;
Woon Seok ROH
;
Bong Il KIM
Author Information
1. Department of Anesthesiology and Pain Medicine, School of Medicine, Catholic University, Daegu, Korea. anessy73@cu.ac.kr
- Publication Type:Case Report
- Keywords:
General anesthesia;
Hypotonia;
Lowe syndrome;
Metabolic acidosis
- MeSH:
Acidosis;
Anesthesia, General;
Cataract;
Eye;
Humans;
Intellectual Disability;
Intraocular Pressure;
Kidney;
Muscle Hypotonia;
Nervous System;
Oculocerebrorenal Syndrome;
Perioperative Period;
Prevalence;
Scalp;
Seizures
- From:Korean Journal of Anesthesiology
2009;56(1):112-115
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Lowe syndrome (the oculo-cerebro-renal syndrome of Lowe, OCRL) is a multi-system disorder that affects the eyes, nervous system, and kidney. OCRL is a rare X-linked recessive disease with a prevalence of approximately 1 : 500,000. The clinical features of OCRL include congenital cataracts, growth and mental retardation, areflexia, hypotonia, and renal tubular dysfunction (Fanconi-type). Chronic metabolic acidosis and hypotonia may be the most important component affecting management of the peri-anesthetic period during general anesthesia. However, problems such as electrolyte imbalance, seizure, fragility of the bone structures, and increased intraocular pressure should also be considered during the perioperative period. We report here the perioperative management of a patient with Lowe syndrome during the removal of multiple scalp cysts under general anesthesia.
