Salaria Amit Kumar, Singh Gaganpreet, Dogra Ekta, Kumar Naveen, Sodavarapu Praveen, Neradi Deepak
Department of Orthopaedics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Orthopaedics, AIIMS Bathinda, Punjab, India.
J Orthop Case Rep. 2020 Sep;10(6):72-75. doi: 10.13107/jocr.2020.v10.i06.1882.
Melorheostosis, on its own, is an extremely uncommon disease. It is non-hereditary, sclerosing bone lesion affecting the adjacent soft tissues. Long bones are commonly involved. About 50% of the cases are evident before 20 years of age. It is gradually progressive and involves the adjacent soft tissues resulting in pain, fibrosis, joint contractures, and limb length discrepancies. Various forms of clinical presentation such as hemimelic, monomelic, mono-ostotic, and polyostotic have been described in the literature. Similarly there are different radiological varients like classical, osteoma , osteopathic striatae , and myositis ossificans.. Treatment is mainly palliative.
We describe a highly unusual and extensive involvement of the lower limb in a Melorheostotic patient of 35 years of age with a deformity and limb length discrepancy. X-rays are like that of myositis ossificans and mixed type of pattern with extensive involvement from the pelvis to the toe. There is also intra-articular extension of melorheostosis mimicking synovial chondromatosis. The patient is in our close follow-up and physiotherapy guidance. Such an extensive involvement, along with para-articular and intra-articular involvement, is very rare.
Melorheostosis can extensively involve the whole of the limb right from the pelvis to the digits. It can also extend into the joints producing intra-articular loose bodies. Para-articular soft-tissue masses are also common. Radiologically, it can have a mixture of patterns described in the literature. Close follow-up of the patient is essential so as to provide palliative treatment, deal with the deformities, and to assess the progression of the diseases.
孤立性骨密质增生症本身是一种极为罕见的疾病。它是非遗传性的，会导致相邻软组织硬化的骨病变。长骨常受累。约50%的病例在20岁前出现症状。病情逐渐进展，累及相邻软组织，导致疼痛、纤维化、关节挛缩和肢体长度差异。文献中描述了各种临床表现形式，如半侧肢体、单肢、单骨型和多骨型。同样，在放射学上也有不同的表现形式，如典型型、骨瘤型、骨病条纹型和骨化性肌炎型。治疗主要是姑息性的。
我们描述了一名35岁骨密质增生症患者下肢出现极为罕见且广泛的病变，伴有畸形和肢体长度差异。X线表现类似骨化性肌炎，呈现混合型模式，从骨盆到脚趾广泛受累。骨密质增生症还出现关节内扩展，类似滑膜软骨瘤病。该患者在我们的密切随访和物理治疗指导下。如此广泛的病变，以及关节周围和关节内受累情况非常罕见。
骨密质增生症可广泛累及整个肢体，从骨盆到手指。它还可延伸至关节内，产生关节内游离体。关节周围软组织肿块也很常见。在放射学上，它可呈现文献中描述的多种表现形式。对患者进行密切随访至关重要，以便提供姑息治疗、处理畸形并评估疾病进展。
