Volume : VIII, Issue : III, March - 2019

Macrodystrophia Lipomatosa: Ensue as localized gigantism (Two cases)

Kadam Monica Mittal

Abstract :

Background – Macrodystrophia lipomatosa is a rare non –hereditary congenital disease. The exact etiology is unknown. The specific body region; sclerotome is involved followed by mesenchymal overgrowth in that region. Cosmetic disfigurement and mechanical dysfunction are two problems associated with Macrodystrophia lipomatosa. The characteristic histopathology finding in Macrodystrophia lipomatosa is increased deposition of adipose tissue in periosteum, nerve sheath, muscle, subcutaneous tissue as well as bone marrow. Case series – Two cases of macrodystrophia lipomatosa are reported. Cases presented as gradual painless macrodactyly since birth. Involvement of left middle finger observed in case one whilst, in case two involvement of right thumb, index and middle finger noted. On clinical examination the hypertrophied digits were soft, non–tender and non–pulsatile. The radiology investigation revealed increased soft tissue shadow as well as bone hypertrophy in both the cases. The histopathology examination confirmed the excessive deposition of adipose tissue not only in in soft tissue planes but also in inter–trabecular spaces. Conclusion – Macrodystrophia lipomatosa is rare non–hereditary condition leading to macrodactyly. The histopathology examination is the gold standard for diagnosis. The mode of management is surgical but does not ensure a favorable outcome.

Article: Download PDF    DOI : https://www.doi.org/10.36106/paripex  

Cite This Article:

MACRODYSTROPHIA LIPOMATOSA: ENSUE AS LOCALIZED GIGANTISM (TWO CASES), Kadam Monica Mittal PARIPEX - INDIAN JOURNAL OF RESEARCH : Volume-8 | Issue-3 | March-2019

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