Melorheostosis in children. Clinical features and natural history

Abstract

Experience in the management of fourteen children with melorheostosis has been reviewed. The principal and presenting clinical features were unilateral soft-tissue contractures associated with inequality of limb length. In contrast to the disease in adults, pain occurred infrequently and was never intense. The average interval between the discovery of the clinical features and the correct diagnosis was six years. The distinctive radiographic feature in the child was an endosteal pattern of hyperostosis marked by streakiness of the long bones and spotting of the small. This differs from the usual subperiosteal or extracortical pattern of hyperostosis seen in adults. The surgical treatment of the contractures proved difficult and recurrence of the deformity was the rule. Distal ischaemia occurred when the chronically contracted and flexed joint was rapidly extended.

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“…The successful rates of these procedures are high [20][21][22][23]. In people with melorheostosis and severe contracture, traditional methods such as manipulations, casts, open extensive soft tissue release, open capsulotomies, osteotomies, arthrodesis and correction with Ilizarov frame, often have uncertain results, low successful rates or high recurrence rates [17,[24][25][26].…”

Melorheostosis of the hand affecting the c6 sclerotome and presenting with carpal tunnel syndrome

“…Deformity of the affected limb and limb length discrepancy are caused by premature closure of the growth plate; fibrotic changes in the skin and soft tissue results in continued tethering as they do not stretch with the growth of the limb. (8)(9)(10) Due to loss of thumb apposition, involvement of the radial aspect of the hand is disabling. (6) In our patient, we offered a further operation to fuse her thumb in an abducted position for better functionality.…”

Osteosclerotic bone dysplasia (melorheostosis) of the mandible

“…In the early stages, the hyperostosis can be found either as a linear endosteal apposition in the long bones or as a focal sclerosis in small bones 22 . In adults subperiosteal or extracortical bone apposition is more frequent 5 .…”