Situs inversus totalis and congenital hypoglossia

Hypoglossia is a rare congenital malformation, occurring either as an isola ted malformation or in association with other deformities, particularly lim b defects. We describe a female infant with congenital hypoglossia, microgn athia and situs inversus. The main complications were airway compromise and feeding difficulties requiring tracheostomy and gastrostomy. Situs inversu s and hypoglossia have been reported together on six previous occasions, wi th all cases being sporadic. Situs inversus-hypoglossia falls into a spectr um of aetiologically non-specific developmental field defects that Includes the Aglossia-adactylia spectrum and the Agnathia-holoprosencephaly spectru m. Situs inversus-hypoglossia may represent a mild form of Agnathia-holopro sencephaly. Clin Dysmorphol 10: 47-50 (C) 2001 Lippincott Williams & Wilkin s.