Abstract
Macrodystrophia Lipomatosis is rare congenital hamartomatous enlargement of soft tissue components leading to gigantism of single, multiple digits or entire limb. Clinical findings are recognised as early as in neonatal period to late adulthood. Patients usually present for either cosmetic corrections or mechanical problems. We report a case of 8 year old female with progressive increase in size of left 2nd and 3rd toes. X-ray findings show congenital focal enlargement of soft tissues and bones of 2nd and 3rd toes and adjacent metatarsals. Clinical and radiological features of our patient points out towards this uncommon condition. DOI: http://dx.doi.org/10.3329/bjms.v12i4.13465 Bangladesh Journal of Medical Science Vol. 12 No. 04 October 13 Page 442-444
Highlights
Macrodystrophia lipomatosis [ML] is distinct clinical entity often misdiagnosed as other forms of macrodactyly
It is a rare congenital form of macrodactyly characterised by increase in mesenchymal elements especially overburdance of fibrofatty tissue on plantar aspect of foot
Local examination of the affected foot revealed there was both increase in length and width of 2nd and 3rd toes with increase in subcutaneous tissue mass which was non tender to touch
Summary
Macrodystrophia lipomatosis [ML] is distinct clinical entity often misdiagnosed as other forms of macrodactyly. It is a rare congenital form of macrodactyly characterised by increase in mesenchymal elements especially overburdance of fibrofatty tissue on plantar aspect of foot. Local examination of the affected foot revealed there was both increase in length and width of 2nd and 3rd toes with increase in subcutaneous tissue mass which was non tender to touch (as shown in figure 1 and 2). Singal K K, Singal N, Mehra V, Singh H, Kaur J, Relan P tissues and bonesof 2nd and 3rd toes depicting Congenital Partial Gigantism (as shown in figure 4). Histological features of ML are characterised by markedly increase in all mesenchymal elements dominated by adipose tissue in fine fibrous network involving periosteum, bone marrow, nerve shealth, muscle and subcutaneous tissue.(6)
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