title: Development of the hip in diastrophic dysplasia.
abstract: We examined 50 patients with diastrophic dysplasia both clinically and radiologically. Two legally aborted fetuses were dissected. The mean age of the patients was 16.2 years (newborn to 38) and the mean follow-up was 11.4 years (3 months to 34 years). The fetal hips and MRI of newborn infants showed congruity and no significant joint deformity. Flexion contracture of the hip became evident later in 93% and was progressive. The radiological appearance of the proximal femoral ossific nuclei was delayed and in 17% of males and 28% of females the ossific nuclei had not appeared by the age of 12 years. Radiological measurements differed considerably from reference values and were related to the rapid and progressive restriction of rotational movement and the increase in flexion contracture. The typical findings were flattening and inferomedial bulking of the femoral head and a double-hump deformation. The changes in the hip led to secondary osteoarthritis before early middle age. We describe the clinical and radiological measurements which define the early degeneration of the joint.
authors.affiliation: Department of Paediatric Orthopaedics, Hospital for Children and Adolescents, University of Helsinki, Finland.
authors.forename: P
authors.initials: P
authors.lastname: Vaara
authors.forename: J
authors.initials: J
authors.lastname: Peltonen
authors.forename: M
authors.initials: M
authors.lastname: Poussa
authors.forename: J
authors.initials: J
authors.lastname: Merikanto
authors.forename: M
authors.initials: M
authors.lastname: Nurminen
authors.forename: I
authors.initials: I
authors.lastname: Kaitila
authors.forename: S
authors.initials: S
authors.lastname: Ryöppy
mesh_terms: D000029:Abortion, Legal; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D003286:Contracture / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005269:Femur / Q000000981:diagnostic imaging / Q000254:growth & development / Q000473:pathology; D005270:Femur Head / Q000000981:diagnostic imaging / Q000254:growth & development / Q000473:pathology; D005333:Fetus; D005500:Follow-Up Studies; D006618:Hip Dislocation, Congenital / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D006621:Hip Joint / Q000000981:diagnostic imaging / Q000196:embryology / Q000254:growth & development* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D010003:Osteoarthritis / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D010012:Osteogenesis / Q000502:physiology; D011247:Pregnancy; D011859:Radiography; D016059:Range of Motion, Articular / Q000502:physiology; D012016:Reference Values; D012399:Rotation