Open AccessDifficult intubation management in a child with I-cell disease
Author(s) -
Abdul Kader Mahfouz,
G George,
Suhaila Al-Bahlani,
Mohamed Z Al Nabhani
Publication year - 2010
Publication title -
saudi journal of anaesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.416
H-Index - 24
eISSN - 1658-354X
pISSN - 0975-3125
DOI - 10.4103/1658-354x.65121
Subject(s) - medicine , disease , psychomotor learning , life expectancy , gingivectomy , pediatrics , osteogenesis imperfecta , intensive care medicine , pathology , dentistry , population , cognition , environmental health , psychiatry
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-year-old Omani female with I-cell disease is presented. The problems faced and their management during anesthesia are described.