Ostrum-Furst syndrome
Os·trum-·Furst syn·drome
(os'trŭm fŭrst),Klippel-Feil syndrome
A condition (OMIM:214300) characterised by short neck, low hairline and decreased cervical motion.Associated conditions
Congenital scoliosis (60%, most need treatment); Sprengel's deformity (failure of scapula to descend, remains attached to cervical spine by omovertebral bone or fibrous band) (33%); renal disease, often with aplasia (33%); synkinesis (mirror movements); congenital heart disease; brain stem defects; congenital cervical stenosis; syndactyly and hypoplastic thumb; hearing loss (30%).
Pathogenesis
Klippel-Feil syndrome is characterised by congenital failure of normal segmentation of cervical somites at 3–8 weeks's gestation, resulting in two or more fused cervical segments.