Pauwels Osteotomy for Femoral Neck Nonunion in Two Adult Siblings with Osteogenesis Imperfecta

Abstract

This is a retrospective review of two adult siblings with osteogenesis imperfecta (OI) type III (according to Sillence classification), who sustained a spontaneous femoral neck fracture and subsequent nonunion. The diagnosis of OI in these two patients was made based on clinical, radiological and genetic findings. The fracture was most likely caused by femoroacetabular impingement secondary to OI induced acetabular protrusio. A valgus osteotomy according to Pauwels'principles and fixation of the osteotomy and nonunion with a locking plate resulted in healing despite compromised bone quality and limited bone stock. Long-term follow up (4.5 years and 6.5 years, respectively) is provided.When treating this difficult problem, improving the mechanobiological environment and decreasing the femoroacetabular impingement by a Pauwels type osteotomy should be considered.

Patterns of femoral neck fracture and its treatment methods in patients with osteogenesis imperfecta

“…Considering the risk of progression of the angular deformity, we preferred corrective osteotomy to in-situ fixation to promote healing of the fracture site by changing the biomechanical environment. Successful treatment of femoral neck fracture nonunion in adults with osteogenesis imperfecta using Pauwels proximal femoral valgus osteotomy was beautifully demonstrated by Kloen et al [18], and the same principle may apply to the angulated-stable fracture pattern.…”

Osteogenesis Imperfecta: Search for Mutations in Patients from the Republic of Bashkortostan (Russia)

“…This change has been described 11 times, and the authors report a mild course of the disease with type I of OI [ 4 , 12 , 32 , 33 , 34 , 35 ]. The c.2461G>A mutation was registered in the database 31 times and described in patients with types I, II, III, and IV of OI [ 4 , 5 , 12 , 13 , 14 , 17 , 36 , 37 , 38 , 39 , 40 , 41 ]. Kloen and co-authors described in detail a patient with this mutation who had multiple fractures with poor healing as a result of this progressive deformation of the lower and upper extremities and compression fractures of the spine.…”

“…Kloen and co-authors described in detail a patient with this mutation who had multiple fractures with poor healing as a result of this progressive deformation of the lower and upper extremities and compression fractures of the spine. The patient could not move independently [ 40 ]. Our patient also has multiple fractures and deformities of the limbs, which correlates with type III of OI.…”

Идентификация Мутаций У Больных Незавершенным Остеогенезом Из Республики Башкортостан

“…Фенотип: сколиоз, деформация нижних конечностей, голубые склеры. Тип наследования на данный момент невозможно выяснить, так как ДНК членов семьи недоступно для анализа [6,7,8].…”