Korean J Anesthesiol.  2009 Jan;56(1):112-115. 10.4097/kjae.2009.56.1.112.

The perioperative management of a patient with lowe syndrome for general anesthesia: A case report

• Chung JY
• Kwon JH
• Seo KC
• Song SY
• Roh WS
• Kim BI

• KMID: 2031873
• DOI: http://doi.org/10.4097/kjae.2009.56.1.112

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.

Keyword

General anesthesia; Hypotonia; Lowe syndrome; Metabolic acidosis

MeSH Terms

Acidosis
Anesthesia, General
Cataract
Eye
Humans
Intellectual Disability
Intraocular Pressure
Kidney
Muscle Hypotonia
Nervous System
Oculocerebrorenal Syndrome
Perioperative Period
Prevalence
Scalp
Seizures