Renal artery stenosis associated with melorheostosis

Abstract

Melorheostosis is a benign, rare, congenital disorder of hyperostosis of one or more bones. In this report, we describe a 5-year-old girl with melorheostosis of the left iliac wing, femur, and tibia who developed severe hypertension secondary to left renal artery stenosis. Numerous soft tissue and vascular anomalies have been reported in patients with melorheostosis. To our knowledge this is the first case where renal artery stenosis has been associated with melorheostosis. Several hypotheses for bone and vascular involvement in melorheostosis are reviewed.

Melorheostotic skin changes

“…Numerous soft tissue and vascular anomalies have been reported in patients with melorheostosis 7 . A case of melorheostosis with minimal change nephrotic syndrome, mesenteric fibromatosis, and capillary hemangiomas was reported by Roger et al 8 .…”

“…Melorheostosis may be associated with many conditions and skin disorders (Table 1). 3–11 In cases of linear sclerodermatous skin changes with hypertrichosis and hyperpigmented macules with hypertrichosis, melorheostosis should be considered.…”

Melorheostosis–an unusual cause of amputation

“…It can coexist with changes in the overlying skin and malformations of blood vessels and lymphatics. This unusual condition can also be associated with nephrotic syndrome, carpal tunnel syndrome and renal artery stenosis (Roger et al 1994; Igiesias et al 1994). There is one report in the very recent literature of the occurrence of osteosarcoma in a melorheostostic femur (Murphy et al 2003).…”

Renal artery stenosis associated with Crohn disease

“…Clinical and serological workup did not support the presence of any other large-vessel or medium-vessel vasculitides in our patient. His physical examination also did not reveal clinical stigmata of neurofibromatosis [21], melorheostosis [22], William syndrome [23], Marfan syndrome [24] or Klippel-Trenaunay-Weber syndrome [25], which have all been reported in association with renal artery stenosis.…”