title: Fibrous dysplasia, shepherd's crook deformity and an intra-capsular femoral neck fracture.
abstract: Fibrous dysplasia (FD) is a rare bone disorder in which normal medullary bone is replaced by fibro-osseous tissue. It typically presents in childhood with pain, skeletal deformities, gait abnormalities and occasionally, fatigue fractures. The management of FD remains a challenge. Surgical procedures have been developed to provide symptom relief, correct skeletal deformity and offer mechanical support in cases at risk of fracture. However, there is a paucity of data on the management of acute femoral neck fractures in the adult population with FD. We report the case of a 23-year-old man with a shepherd's crook deformity secondary to FD, who sustained an intra-capsular femoral neck fracture whilst playing football. The patient initially underwent closed reduction and internal fixation with cannulated screws. However, during the procedure, a guide wire broke whilst inside the femoral head. The patient was referred to the senior author, who undertook a second operation to remove the metalwork and correct the varus deformity using a closing-wedge femoral osteotomy, whilst achieving osteosynthesis at the fracture site. At 1-year follow-up, the patient is pain-free and demonstrates a full range of movement. These cases can be technically demanding and carry a greater risk of complication. It is important that preoperative planning is undertaken and surgery performed by individuals with experience in managing FD and complex femoral neck fractures. Correction of the skeletal deformity whilst fixing the fracture will help restore the mechanical axis and reduce the risk of a recurrent fracture.
authors.affiliation: ST3 Trauma & Orthopaedics Cheltenham General Hospital, Gloucestershire, UK, almouazzen@yahoo.com.
authors.forename: Louay
authors.initials: L
authors.lastname: Al-Mouazzen
authors.forename: Karthig
authors.initials: K
authors.lastname: Rajakulendran
authors.forename: Nurul
authors.initials: N
authors.lastname: Ahad
references.citation: J Orthop Surg (Hong Kong). 2009 Aug;17(2):245-7
references.pmid: 19721165
references.citation: J Bone Joint Surg Am. 1986 Dec;68(9):1415-22
references.pmid: 3536935
references.citation: J Postgrad Med. 1997 Jul-Sep;43(3):83-4
references.pmid: 10740732
references.citation: J Bone Joint Surg Am. 1998 May;80(5):648-58
references.pmid: 9611025
references.citation: J Bone Joint Surg Am. 1962 Mar;44-A:207-33
references.pmid: 14036060
references.citation: J Pediatr Orthop. 2002 Mar-Apr;22(2):255-60
references.pmid: 11856942
references.citation: J Chin Med Assoc. 2005 Jul;68(7):343-6
references.pmid: 16038377
references.citation: Arch Orthop Trauma Surg. 2010 Apr;130(4):497-502
references.pmid: 19629503
references.citation: Hum Pathol. 2012 Aug;43(8):1234-42
references.pmid: 22245114
references.citation: Cases J. 2009 Jan 08;2(1):26
references.pmid: 19128514
references.citation: J Bone Joint Surg Br. 1962 May;44-B:302-18
references.pmid: 14039519
references.citation: J Bone Joint Surg Am. 1950 Apr;32A(2):321-37
references.pmid: 15412174
references.citation: J Orthop Surg (Hong Kong). 2008 Aug;16(2):254-6
references.pmid: 18725684
references.citation: Clin Orthop Relat Res. 1995 Jan;(310):160-4
references.pmid: 7641433
references.citation: J Bone Joint Surg Am. 1972 Jul;54(5):1047-56
references.pmid: 4506620
references.citation: Acta Orthop. 2006 Jun;77(3):469-73
references.pmid: 16819687
references.citation: J Bone Miner Res. 1997 Oct;12(10):1746-52
references.pmid: 9333137
references.citation: Clin Orthop Relat Res. 2001 Jan;(382):6-12
references.pmid: 11154006
references.citation: Orthopedics. 2009 May;32(5):320
references.pmid: 19472968