Case report: severe melorheostosis involving the ipsilateral extremities.
Melorheostosis is a rare, noninheritable bone dysplasia characterized by its classic radiographic feature of flowing hyperostosis resembling dripping candle wax, generally on one side of the long bone. The condition originally was described by Leri and Joanny in 1922. Its etiology remains speculative, and treatment in most instances has been symptomatic. Melorheostosis usually affects one limb, more often the lower extremity, and rarely the axial skeleton. We report a rare case of severe melorheostosis in the ipsilateral upper and lower extremities with normal contralateral extremities. The plain radiographs revealed almost all the bones in the affected extremities, from clavicle and scapula to distal phalanges of the fingers and from femur to distal phalanges of the toes, presented extensive, dense hyperostosis and heterotopic ossification in the periarticular soft tissue. Physical examination showed considerable swelling and deformities of the left limbs, stiffness and distortion of the joints, and anesthesia in the left ulnar regions of the forearm and hand. The examination of the right side was normal. Computed tomography scans showed multiple areas of classic candle wax-like hyperostosis and narrowing or disappearance of the medullary cavity. Histologic analysis confirmed the clinical and imaging diagnosis and revealed extremely dense sclerotic bone of cortical pattern.
Long
Hai-Tao
HT
Department of Orthopaedics, Xiangya Hospital of Central South University, Changsha, Hunan, China. doclong0423@sina.com
Li
Kang-Hua
KH
Zhu
Yong
Y
D000328:Adult; D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D004487:Edema / Q000209:etiology; D005121:Extremities; D005260:Female; D006801:Humans; D008557:Melorheostosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D010146:Pain / Q000209:etiology; D010808:Physical Examination; D016059:Range of Motion, Articular; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome