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Macrodystrophia lipomatosa: radiographic observations.
S K Gupta1, O P Sharma, S V Sharma
1Department of Diagnostic Radiology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.
The British Journal of Radiology
|September 1, 1992
Summary
Macrodystrophia lipomatosa (MDL) involves overgrowth of fat and bone in digits, presenting diverse radiographic features. This study details 23 cases, highlighting typical and uncommon skeletal and soft tissue changes in MDL.
Area of Science:
- Radiology
- Orthopedics
- Medical Imaging
Background:
- Macrodystrophia lipomatosa (MDL) is a rare congenital condition characterized by progressive, localized overgrowth of adipose and mesenchymal tissues.
- The radiographic manifestations of MDL can be varied, making diagnosis challenging.
Purpose of the Study:
- To comprehensively describe the spectrum of radiographic findings in 23 cases of macrodystrophia lipomatosa.
- To identify typical and uncommon radiological features associated with MDL.
Main Methods:
- Retrospective review of radiographic imaging (X-rays, angiography) from 23 patients diagnosed with MDL.
- Detailed analysis of bone morphology, soft tissue hypertrophy, and associated anomalies.
Main Results:
- Typical findings included mesodermal tissue hypertrophy, particularly fat overgrowth, and enlarged phalanges and metatarsals.
- Uncommon features observed were elongated and thinned bones, small phalanges/metatarsals, slanting articular surfaces, syndactyly, polydactyly, brachydactyly, and symphalangism.
- Palmar, plantar, forearm, and leg involvement occurred in a significant number of cases; angiography was non-specific.
Conclusions:
- Macrodystrophia lipomatosa exhibits a wide range of radiographic presentations, with digital involvement being most common.
- Radiographic evaluation is crucial for characterizing MDL, though findings can be diverse and include rare anomalies.