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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 1: Sagittal T1W MRI image reveals proliferation of fatty tissue (arrows) on the volar aspect of the index finger with signal intensity similar to that of subcutaneous fat; there is dorsal angulation of the affected digit
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Figure 0003: Case 1: Sagittal T1W MRI image reveals proliferation of fatty tissue (arrows) on the volar aspect of the index finger with signal intensity similar to that of subcutaneous fat; there is dorsal angulation of the affected digit

Mentions: A 7-year-old, right-hand-dominant male child presented with a progressive disproportionate enlargement of the thumb and index finger of his right hand since 1 year of age. The patient denied having any pain or neurovascular symptoms and there was no family history of extremity gigantism. On physical examination, a nontender, soft tissue mass was palpable on the volar aspect of the enlarged fingers and there was dorsal angulation of the index finger [Figure 1]. There were no overlying cutaneous changes, pitting edema, or bruit. The patient was able to use the hand well and had an adequate grip. A plain radiograph demonstrated soft tissue swelling along the volar aspect of the thumb and index finger, an enlarged distal phalanx of the index finger, and osteoarthritic changes in the distal interphalangeal joint [Figure 2]. An MRI with a 1.5-T unit demonstrated increased fatty tissue along the palmar aspect of the right index finger and thumb, extending up to the thenar eminence. It was seen as a hyperintense area on T1W images with intermediate signal on T2W images [Figure 3]. The underlying bones revealed normal signal intensity and intact periosteum.


Case Report: Macrodystrophia lipomatosa - Illustration of two cases.

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

Case 1: Sagittal T1W MRI image reveals proliferation of fatty tissue (arrows) on the volar aspect of the index finger with signal intensity similar to that of subcutaneous fat; there is dorsal angulation of the affected digit
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 0003: Case 1: Sagittal T1W MRI image reveals proliferation of fatty tissue (arrows) on the volar aspect of the index finger with signal intensity similar to that of subcutaneous fat; there is dorsal angulation of the affected digit
Mentions: A 7-year-old, right-hand-dominant male child presented with a progressive disproportionate enlargement of the thumb and index finger of his right hand since 1 year of age. The patient denied having any pain or neurovascular symptoms and there was no family history of extremity gigantism. On physical examination, a nontender, soft tissue mass was palpable on the volar aspect of the enlarged fingers and there was dorsal angulation of the index finger [Figure 1]. There were no overlying cutaneous changes, pitting edema, or bruit. The patient was able to use the hand well and had an adequate grip. A plain radiograph demonstrated soft tissue swelling along the volar aspect of the thumb and index finger, an enlarged distal phalanx of the index finger, and osteoarthritic changes in the distal interphalangeal joint [Figure 2]. An MRI with a 1.5-T unit demonstrated increased fatty tissue along the palmar aspect of the right index finger and thumb, extending up to the thenar eminence. It was seen as a hyperintense area on T1W images with intermediate signal on T2W images [Figure 3]. The underlying bones revealed normal signal intensity and intact periosteum.

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