Funnel chest repair in a patient with Morquio's syndrome

Abstract

A 6-year-old boy with Morquio's syndrome was referred from the department of pediatrics in our hospital for a sterno-turnover operation to repair his funnel chest.
In Morquio's syndrome, also termed mucopolysaccharidosis IV, there are many skeletal abnormalities and growth retardation because of acid sulfatase deficiency. This syndrome, however, has no neurological abnormalities. Instrumental repair of the skeletal deformities is not effective and even results in the suppression of cardiopulmonary functions or in mental depression. Therefore, the sterno-turnover operation seems to be the best therapy for funnel chest in patients with Morquio's syndrome.