Femoral neck fractures in osteogenesis imperfecta

Osteogenesis imperfecta is a connective tissue disease that is usually characterized by autosomal dominant transmission (the autosomal recessive type leads to death in early childhood). Similar to osteopetrosis a congenital and a later appearing form are known. The congenital form can be recognized by intrauterine fractures and by bone deformities already apparent at birth. Typical for this disease is the absence of periosteal bone formation. Symptoms include a transparent blue sclera, deafness, deformation of the vertebral column and foremost the extreme fragility of bone. Later appearing forms can imitate a juvenile or postmenopausal osteoporosis (Fig. 234).

Osteogenesis Imperfecta

Fig. 234. Femoral neck fracture in osteogenesis imperfecta.

This 13-year-old boy with a known osteogenesis imperfecta is deaf, both limbs are deformed (genu varum), he walks with a cane. During a fall he injured his left hip; a. Base of neck fracture, displaced in varus; b. c. After transfer to our institution we stabilized the fracture in proper position with cancellous bone screws four days after the accident; d. The fracture consolidated but after one year we observed signs of an epiphyseal necrosis, the screws were removed; e. Three months later the necrosis had progressed. No weight bearing was permitted; f. 2.5 years after the injury the head was almost remodeled, the contour spherical, the joint space remained congruent. The boy returned to his normal activities of daily living

Fig. 234. Femoral neck fracture in osteogenesis imperfecta.

This 13-year-old boy with a known osteogenesis imperfecta is deaf, both limbs are deformed (genu varum), he walks with a cane. During a fall he injured his left hip; a. Base of neck fracture, displaced in varus; b. c. After transfer to our institution we stabilized the fracture in proper position with cancellous bone screws four days after the accident; d. The fracture consolidated but after one year we observed signs of an epiphyseal necrosis, the screws were removed; e. Three months later the necrosis had progressed. No weight bearing was permitted; f. 2.5 years after the injury the head was almost remodeled, the contour spherical, the joint space remained congruent. The boy returned to his normal activities of daily living

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