Macrodystrophia lipomatosa

Case contributed by Dr. Kajal Limbad

Presentation

Painless enlargement of middle finger of right hand

Patient Data

Age: 2 years
Gender: Female
X-ray

Radiograph of both hands shows marked hypertrophy of right second and third digit with enlarged and splaying of phalanges. No evidence of soft tissue calcification or focal osseous mass. The overlying soft tissues are markedly overgrown. There are focal lucent areas representing fat within the soft tissues.

Left hand appears normal.

Case Discussion

Macrodystrophia lipomatosa is a rare form of localized gigantism. It is a non-hereditary isolated developmental anomaly, which results in overgrowth of all mesenchymal elements such as bones, fat, phalanges, tendons, nerves, and vessels. The growth of affected parts usually ceases at puberty.

This condition typically presents with painless enlargement of the 2nd or 3rd digit of the hand or foot.  It is usually unilateral but may involve adjacent digits in the distribution of the median or plantar nerves. Occasionally it involves the entire limb.

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Case information

rID: 73350
Published: 10th Jan 2020
Last edited: 13th Jan 2020
Inclusion in quiz mode: Included
Institution: MEDICAL COLLEGE AND SSG HOSPITAL VADODARA

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