Revista chilena de pediatría
versión impresa ISSN 0370-4106
CAMMARATA-SCALISI, FRANCISCO; CAMPAGNARO GEREMIA, JOSÉ GREGORIO; DA SILVA, GLORIA y OLIVO CUESTA, CARLA. Macrodystrophia Lipomatosa: A Case Report. Rev. chil. pediatr. [online]. 2010, vol.81, n.6, pp. 531-535. ISSN 0370-4106. http://dx.doi.org/10.4067/S0370-41062010000600007.
Macrodystrophia lipomatosa (ML), is a Rare cause of non-hereditary localized gigantism, due to the progressi-ve proliferation of mesenchymal tissue, with a disproportionate increase of fibroadipose tissue in one or more digits of the affected extremity. This congenital abnormality occurs more frequently in the distribution of the median nerve (arm) and plantar nerve (leg). Etiology is uncertain, and it is frequently misdiagnosed as another cause of macrodactylia. This paperöreports a case of a 6 y.o. boy who presented overgrowth of the lst and 2nd digit of theöright foot, and slight increase of the 3rd digit in the other foot. A multidisciplinary evaluation was performed, corresponding to the second case reported in Venezuela.
Palabras clave : Macrodystrophia lipomatosa; localized gigantism; clinic presentation; etiology.