| Popis: |
Melorheostosis of Leri is a non-familial condition of hyperostosis of the cortical bone that usually presents unilaterally in long bones of the upper and lower limbs, but may also present in vertebra, ribs, skull and jaw. The incidence of this disease is quite rare, only about 300 cases have been reported worldwide. We present a case, which may be the first documented case in sub-Saharan Africa.S.K. is a 14 year old male student who presented to the hospital with an 18 month history of persistent pain in the joints of the right upper limb and a limb length discrepancy since birth which has worsened with growth. Examination revealed generalised hypoplasia of the right upper limb with shortening of the limb and atrophy of the muscles, also hypoplasia and contracture of the thumb was observed. The radiographs of the limb showed multiple areas of dense hyperostosis and scleroderma, which showed a linear distribution along the radial half of the bones.In children presentation of melorheostosis, is more likely be as limb length discrepancy, deformity or joint contractures which may be seen before radiographic evidence of any bony changes. Improvement in imaging techniques will therefore result in early diagnosis and greater success with conservative management. Also the increased frequency of tumours necessitates long-term follow up.melorheostosis, scleroderma. |
