Maffucci lymphangioma syndrome: an unusual variant of Ollier's disease, a case report and a review of the literature

J Pediatr Orthop B. 2003 Mar;12(2):147-50. doi: 10.1097/01.bpb.0000049563.52224.ef.


A case of Maffucci's syndrome in an 18-month old boy is presented, in which the vascular malformation consists of lymphangioma. This is a rare variant of Maffucci's syndrome. So far only six other cases have been reported in which lymphangiomas featured. We believe that the case described here is only the second case to be reported for which a lymphangioma is the only vascular malformation in the syndrome. The literature of the other six cases was reviewed and various features identified enabling this particular disease to be classified as a distinct variant of Maffucci's syndrome.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Enchondromatosis / complications*
  • Enchondromatosis / diagnosis
  • Enchondromatosis / surgery*
  • Fibula / pathology
  • Fibula / surgery
  • Follow-Up Studies
  • Humans
  • Infant
  • Lower Extremity
  • Lymphangioma / complications*
  • Lymphangioma / diagnostic imaging
  • Lymphangioma / surgery*
  • Magnetic Resonance Imaging / methods
  • Male
  • Osteotomy / methods
  • Radiography
  • Risk Assessment
  • Syndrome
  • Tibia / pathology
  • Tibia / surgery
  • Treatment Outcome