Case Report: Severe Melorheostosis Involving the Ipsilateral Extremities (original) (raw)

Abstract

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.

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Acknowledgments

We express sincere thanks to all those who have offered us help writing this paper. We also express our appreciation to our teachers and classmates in the Department of Orthopaedics of Xiangya Hospital of Central South University, especially to Dr. Wen-Feng Xiao and Dr. Guo-Jun Li, who offered valuable support and help in the course of collecting data and writing the paper.

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Authors and Affiliations

  1. Department of Orthopaedics, Xiangya Hospital of Central South University, No. 87 Xiangya Road, Changsha, Hunan, 410008, China
    Hai-Tao Long MD, Kang-Hua Li MD & Yong Zhu MD

Authors

  1. Hai-Tao Long MD
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  2. Kang-Hua Li MD
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  3. Yong Zhu MD
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Corresponding author

Correspondence toHai-Tao Long MD.

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Each author certifies that he or she has no commercial associations (eg, consultancies, stock ownership, equity interest, patent/licensing arrangements, etc) that might pose a conflict of interest in connection with the submitted article.

Each author certifies that his or her institution has approved or waived approval for the reporting of this case, that all investigations were conducted in conformity with ethical principles of research, and that informed consent for participation in the study was obtained.

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Long, HT., Li, KH. & Zhu, Y. Case Report: Severe Melorheostosis Involving the Ipsilateral Extremities.Clin Orthop Relat Res 467, 2738–2743 (2009). https://doi.org/10.1007/s11999-009-0890-y

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