J Korean Dent Soc Anesthesiol.  2002 Jun;2(1):33-37. 10.17245/jkdsa.2002.2.1.33.

An Anesthetic Management in a Pedodontic Patient with Lowe Syndrome: A Case Report

• Choi YK
• Oh JY
• Kim DO
• Shin OY
• Lee KH

• KMID: 2345191
• DOI: http://doi.org/10.17245/jkdsa.2002.2.1.33

Abstract

The oculo-cerebro-renal syndrome of Lowe (Lowe syndrome) is an X-linked recessive disorder involving the eyes, nervous systems, and kidneys. The clinical manifestation of this syndrome is characterized by congenital cataracts, glaucoma, seizure disorder, psychomotor growth retardation, hypotonia, renal tubular acidosis, aminoaciduria, rickets, and osteoporosis. We report a 5-year old boy underwent general anesthesia for the treatment of multiple dental carries. During intraoperative period, marked metabolic acidosis was noted and such acidosis was partially corrected by hyperventilation. We suggest that patients with Lowe's syndrome should be attention and treated to possible anesthetic hazards such as metabolic acidosis due to renal tubular dysfunction, rise of intraocular pressure in patient with glaucoma, the fragility of the bone structures due to rickets and osteoporosis.

Keyword

Oculo-cerebro-renal syndrome of Lowe

MeSH Terms

Acidosis
Acidosis, Renal Tubular
Anesthesia, General
Cataract
Epilepsy
Glaucoma
Humans
Hyperventilation
Intraocular Pressure
Intraoperative Period
Kidney
Male
Muscle Hypotonia
Nervous System
Oculocerebrorenal Syndrome*
Osteoporosis
Rickets