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Case Report: Macrodystrophia lipomatosa - Illustration of two cases.

Singla V, Virmani V, Tuli P, Singh P, Khandelwal N - Indian J Radiol Imaging (2008)

Bottom Line: Macrodystrophia lipomatosa is a rare cause of congenital macrodactyly, characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue.This developmental anomaly is reportedly more common in the foot than in the hand, with a predilection for the plantar and median nerve distribution.We present two cases of MDL of the hand, one of which had an unusual nerve territory distribution, making clinical diagnosis difficult.

View Article: PubMed Central - PubMed

ABSTRACT
Macrodystrophia lipomatosa is a rare cause of congenital macrodactyly, characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue. This developmental anomaly is reportedly more common in the foot than in the hand, with a predilection for the plantar and median nerve distribution. We present two cases of MDL of the hand, one of which had an unusual nerve territory distribution, making clinical diagnosis difficult. Preoperative diagnosis was however made on the basis of radiography and MRI and was later confirmed on surgery.

No MeSH data available.


Related in: MedlinePlus

Case 2: A clinical photograph of both hands shows enlarged ring and little fingers (arrows) of the left hand as compared to the normal right hand
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Figure 0004: Case 2: A clinical photograph of both hands shows enlarged ring and little fingers (arrows) of the left hand as compared to the normal right hand

Mentions: A 2-year-old right-hand-dominant male child presented with progressive enlargement and swelling of both dorsal and palmar aspects of the left fourth and fifth digits since birth [Figure 4]. No tenderness, pitting edema, bruit, or skin changes were present and the movements of the digits were normal. A radiograph revealed disproportionately enlarged left ring and little fingers; there was soft tissue swelling, along with dorsal angulation of the fourth finger and lateral angulation of the fifth finger [Figure 5]. MRI depicted fatty tissue proliferation around the left fourth and fifth digits, which was more along the dorsal aspect [Figure 6]. There was no medullary or periosteal abnormality. A debulking procedure was performed and the fingers were reconstructed. Extraction of fat [Figure 7] was done through an incision placed over the dorsum of the ring finger extending to the hypothenar eminence, care being taken to spare the extensor tendons. Pathological examination of the material revealed abundant adipose tissue with some interspersed fibrous tissue.


Case Report: Macrodystrophia lipomatosa - Illustration of two cases.

Singla V, Virmani V, Tuli P, Singh P, Khandelwal N - Indian J Radiol Imaging (2008)

Case 2: A clinical photograph of both hands shows enlarged ring and little fingers (arrows) of the left hand as compared to the normal right hand
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2747455&req=5

Figure 0004: Case 2: A clinical photograph of both hands shows enlarged ring and little fingers (arrows) of the left hand as compared to the normal right hand
Mentions: A 2-year-old right-hand-dominant male child presented with progressive enlargement and swelling of both dorsal and palmar aspects of the left fourth and fifth digits since birth [Figure 4]. No tenderness, pitting edema, bruit, or skin changes were present and the movements of the digits were normal. A radiograph revealed disproportionately enlarged left ring and little fingers; there was soft tissue swelling, along with dorsal angulation of the fourth finger and lateral angulation of the fifth finger [Figure 5]. MRI depicted fatty tissue proliferation around the left fourth and fifth digits, which was more along the dorsal aspect [Figure 6]. There was no medullary or periosteal abnormality. A debulking procedure was performed and the fingers were reconstructed. Extraction of fat [Figure 7] was done through an incision placed over the dorsum of the ring finger extending to the hypothenar eminence, care being taken to spare the extensor tendons. Pathological examination of the material revealed abundant adipose tissue with some interspersed fibrous tissue.

Bottom Line: Macrodystrophia lipomatosa is a rare cause of congenital macrodactyly, characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue.This developmental anomaly is reportedly more common in the foot than in the hand, with a predilection for the plantar and median nerve distribution.We present two cases of MDL of the hand, one of which had an unusual nerve territory distribution, making clinical diagnosis difficult.

View Article: PubMed Central - PubMed

ABSTRACT
Macrodystrophia lipomatosa is a rare cause of congenital macrodactyly, characterized by progressive proliferation of all mesenchymal elements, with a disproportionate increase in fibroadipose tissue. This developmental anomaly is reportedly more common in the foot than in the hand, with a predilection for the plantar and median nerve distribution. We present two cases of MDL of the hand, one of which had an unusual nerve territory distribution, making clinical diagnosis difficult. Preoperative diagnosis was however made on the basis of radiography and MRI and was later confirmed on surgery.

No MeSH data available.


Related in: MedlinePlus