• Journal of The Korean Dental Society of Anesthesiology
• /
• v.2 no.1 s.2
• /
• pp.33-37
• /
• 2002

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.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.15 no.1
• /
• pp.35-39
• /
• 2019

Oculo-cerebro-renal syndrome, known as Lowe syndrome, is an X-linked gene disorder characterized by congenital cataracts, brain abnormalities and renal dysfunction. Tooth crowding, taurodontism, delayed eruption in the permanent dentition and over-retained primary teeth with ectopic eruption of the permanent teeth have been reported as dental findings. Because of the high incidence of poor cooperation, patients with Lowe syndrome have difficulties in maintaining good oral hygiene, which may require dental treatment. We present a case of dental treatment for the uncooperative child with Lowe syndrome under general anesthesia. A 4-year-old, 11.2 kg boy with Lowe syndrome visited Seoul National University Dental Hospital for gingival swelling. The patient had multiple caries requiring dental treatment. Because of his past history of malignant hyperthermia during inhalation induction, anesthesia was induced and maintained with total intravenous anesthesia (TIVA) after medical consultation. Dental restorative treatments were successfully performed and no complications were observed during and after the procedure. Safe and effective dental management of the patients with Lowe syndrome could be performed with the help of general anesthesia and careful monitoring.