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  • Surgical Algorithm for Management of Cervical Deformity in Acro-osteolysis (Hadju-Cheney Syndrome)

    Final Number:

    Tobias A. Mattei MD; Carlos R. Goulart; Daniel Robert Fassett MD

    Study Design:

    Subject Category:

    Meeting: Congress of Neurological Surgeons 2013 Annual Meeting

    Introduction: Acro-osteolysis (Hadju-Cheney Syndrome) is a rare disorder of bone metabolism characterized by progressive destruction of the distal phalangeal bones, aplasia of the facial sinuses, persistence of the cranial sutures and severe spine deformities.

    Methods: There is no standard strategy for management of cervical deformity and instability in such patients and, as such syndrome involves severe degrees of osteoporosis, standard algorithms for deformity correction in healthy patients may not be applicable

    Results: In this report we describe the case of a 65-year old female patient with Hadju - Cheney Syndrome who presented with signs of cervical myelopathy and severe cervical kyphoscoliosis. The patient was submitted to 360o decompression and fusion of the cervical spine consisting in a staged anterior corpectomy followed by 24h of halo traction followed by another anterior approach for bone graft implantation and ACDF C2-T2, and finally complemented by a posterior C2-T3 fusion with navigation guidance with intra-operative CT-scan (O-arm).

    Conclusions: In this report the authors highlight the particularities of the surgical management of cervical deformity in patients with Hadju-Cheney Syndrome. According to our experience several nuances should be expected in such patients, such as: substantial carotid dolichoectasia, increased bleeding from osseous structures, and extensive epidural fibrosis. In our experience we recommend the following surgical strategies: no use of Casper pins for interbody distraction, use of intra-operative fluoroscopy for achievement of bicortical purchase of anterior cervical screws, placement of pedicle screws (instead of lateral has screws) during posterior approaches and maintenance of halo-vest in the post-operative period for at least 3 months. It was also verified that it is possible to successfully apply cervical distraction after an isolated anterior approach with corpectomy with a satisfactory improvement in cervical lordosis. The proposed therapeutic algorithm may have broader applications for management of cervical deformity in patients with severe reduction in bone density.

    Patient Care: The authors propose a surgical algorithm for management of cervical deformity in the setting of severe osteoporosis. The reported experience provides a useful report of intra-operative nuances as well as technical challenges of the management of cervcial deformity in patients with severe osteoporosis

    Learning Objectives: - To be able to describe the main features of Hadju - Cheney Syndrome (Acro-osteolysis) - To be able to describe the possible challenges for management of cervical deformity in patients with severe osteoporosis - To understand the possible salvage strategies as well as the intra-operative nuances during surgical treatment of cervical deformities in patients with reduced bone density

    References: Herscovici D Jr, Bowen JR, Scott CI Jr. Cervical instability as an unusual manifestation of Hajdu-Cheney syndrome of acroosteolysis. Clin Orthop Relat Res. 1990 Jun;(255):111-6. Diren HB, Kovanlikaya I, Süller A, Dicle O. The Hajdu-Cheney syndrome: a case report and review of the literature. Pediatr Radiol. 1990;20(7):568-9. Cunha I, Saavedra MJ, Oliveira MA, Salvador MJ, Malcata A. Hajdu-Cheney Syndrome: a case of acroosteolysis. Acta Reumatol Port. 2007 Apr-Jun;32(2):169-74. Mummaneni PV, Dhall SS, Rodts GE, Haid RW. Circumferential fusion for cervical kyphotic deformity. J Neurosurg Spine. 2008 Dec;9(6):515-21. doi: 10.3171/SPI.2008.10.08226.

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