title: Anesthesia for gingivectomy and dental extractions in a child with I-cell disease--a case report.
abstract: I-cell disease (mucolipidosis II) is a rare metabolic disorder resulting from the deficiency of a specific lysosomal enzyme, N-acetylglucosamine-1-phosphotransferease. Developmental delay and growth failure are common presentations of I-cell disease. Psychomotor deterioration is rapid and progressive. Some physical signs, such as hip dislocations, inguinal hernia, hepatomegaly, joint limitation, and skin changes may be present at birth. Coarse facial features and skeletal abnormalities become more conspicuous with time. The life expectancy of children with this condition is poor, with death usually occurring around the fifth year. A case report of the anesthetic management of gingivectomy with multiple dental extractions in a 5 years old Omani female, with I-cell disease, is presented. The problems faced and their management during anesthesia are described.
authors: Anaesthesia Department, Al Nahdha Hospital, Muscat, Sultanate of Oman, P.O. Box: 937, PC 112 Ruwi. abdulkaderm2000@hotmail.com
authors: Abdul Kader M
authors: AK
authors: Mahfouz
authors: G
authors: G
authors: George
mesh_terms: D000758:Anesthesia / Q000379:methods*; D002675:Child, Preschool; D005260:Female; D005890:Gingivectomy; D006801:Humans; D017214:Laryngeal Masks; D009081:Mucolipidoses / Q000150:complications*; D014081:Tooth Extraction