Abstract
Hajdu-Cheney syndrome (HCS) is a rare metabolic bone disorder that results in severe osteoporosis and various skeletal deformities. Craniospinal pathology is commonly associated with it, but surgical management is challenging due to the distorted anatomy, reduced bone strength, and fusion failure due to osteolysis. Hence, the surgical difficulty in these patients requires careful consideration. In this study, we systematically review all published operative cases and complications to provide a comprehensive review pertaining to the spine and/or cranium in patients with HCS. By highlighting these cases and their associated complications, we aim to prepare practitioners who treat this difficult pathology.
Highlights
BackgroundAccording to the National Organization of Rare Disorders (NORD), roughly 80 cases of Hajdu-Cheney syndrome (HCS) have been reported in the medical literature
Patients often have severe osteoporosis. This predisposes them to fractures and early-onset progressive spinal deformity leading to local impingement and spinal concerns including radiculopathy, myelopathy, scoliosis, basilar invagination, and brainstem compression
Operation: foramen magnum decompression with C1 laminectomy and duraplasty followed by occipitocervical fixation with iliac bone graft and titanium wires
Summary
According to the National Organization of Rare Disorders (NORD), roughly 80 cases of Hajdu-Cheney syndrome (HCS) have been reported in the medical literature. This predisposes them to fractures and early-onset progressive spinal deformity leading to local impingement and spinal concerns including radiculopathy, myelopathy, scoliosis, basilar invagination, and brainstem compression. We aim to analyze all known operative cases of the spine and/or cranium in patients with HCS, the techniques used in these operations, and their corresponding outcomes and complications. How to cite this article Falls C J, Page P S, Stadler J A (December 18, 2021) Craniospinal Surgery in Hajdu-Cheney Syndrome: A Review of Case Reports. Hydrocephalus, multiple compression fractures, midcervical kyphosis, thoracic. At 4 correction, years, worsening spinal stenosis thoracic kyphosis elimination, and stabilization of noticed; an abrupt decrease spine via T4, in height of the. ACDF: anterior cervical discectomy and fusion; BMP: bone morphogenetic protein; HCS: Hajdu-Cheney syndrome
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