A Case of Larsen Syndrome with Cleft Palate

Abstract

Larsen syndrome is a rare hereditary disease (autosomal dominant or recessive mode of transmission) characterized by the association of congenital multiple dislocations of large joints, flattened and depressed nasal bridge, and hypertelorism. Cleft palate is observed in 30-50% of this syndrome. In this paper, a case of Larsen syndrome with cleft palate is reported. A one-month-old female infant with cleft palate visited our clinic. As this patient had several severe constitutional symptoms with respect to this syndrome, multidisciplinary care was essential, and the timing of each treatment had to be carefully considered. As the general condition was steady at age 8, long-term oral surgical treatment and speech therapy started. Although this patient had been using a tracheal canula since age 1, she could speak by closing the end of the tracheal canula with her fingers, therefore palatoplasty was performed for treatment of velopharyngeal incompetence. Hypernasality and consonantal nasalization have tended to improve after the operation. This case suggests that careful consideration on the timing of treatment, pre- and postoperative control, and collaboration with other departments is important for the treatment of a patient with severe accessory symptoms such as this case.