X-ray and computed tomography findings in macrodystrophia lipomatosa of the foot with secondary osteoarthritic changes diagnosed in an elderly female: a case report

Sharma, Rajesh; Gupta, Puneet; Mahajan, Manik; Arora, Manjit; Gupta, Akash

doi:10.1590/0100-3984.2013.0017

Cited by 5 publications

(7 citation statements)

References 9 publications

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“…ML occurs because of the proliferation of all mesenchymal elements of an extremity, including the phalanges, nerves, muscles, and vessels, with a disproportionate increase in fibroadipose tissue[ 1 ] and presents as focal or regionalized gigantism without an abnormal tall stature. [ 4 ] The degree of overgrowth is faster than the normal growth pattern and this abnormal growth ceases at puberty.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, there may be elongated, thickened phalanges with splayed distal ends, resembling a “mushroom” shape. [ 1 ] Doppler flow studies of the involved limbs show no increased vascularity. In CT scan, characteristic findings noted are osseous hypertrophy as well as excessive growth of soft-tissue containing abnormal hypodense areas of fat attenuation.…”

Section: Discussionmentioning

confidence: 99%

“…In CT scan, characteristic findings noted are osseous hypertrophy as well as excessive growth of soft-tissue containing abnormal hypodense areas of fat attenuation. [ 1 ] MRI is the investigation of choice for establishing the diagnosis. MRI easily demonstrates the excess fibrofatty tissue, which has signal characteristics as hyperintensity on T1-weighted and T2-weighted images and complete suppression (hypointensity) on STIR sequence.…”

Section: Discussionmentioning

confidence: 99%

“…[ 7 ] The fat is not encapsulated, the fibrous strands within the fatty tissue are seen as low-signal-intensity linear strands on T1-weighted images. [ 1 ] Neural thickening may also be visualized.…”

Section: Discussionmentioning

confidence: 99%

“…It is a rare developmental anomaly, presents with localized gigantism involving one or more extremities, commonly one or few digits will be enlarged and present as macrodactyly or as enlarged limb. [ 1 ] Lower limb involvement is common[ 2 ] and is frequently unilateral. Rarely, bilateral presentation is noted either in both lower or both upper limbs.…”

Section: Introductionmentioning

confidence: 99%

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A child with enlarged extremities – A case of macrodystrophia lipomatosa

2020

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Macrodystrophia lipomatosa (ML) is a rare, non-hereditary, developmental anomaly that occurs because of the progressive proliferation of all mesenchymal elements of single or multiple digits or entire extremity, with a disproportionate increase in fibroadipose tissue. Commonly one or few digits of an extremity will be enlarged and present as macrodactyly or as enlarged limb. Lower limb involvement is more common and frequently unilateral. The diagnosis of ML is made by accurate clinical assessment and imaging modalities, such as plain X-ray, computed tomography scan, magnetic resonance imaging, and confirmed by histopathological study. In this case, we described a 10-year-old child who was brought to us with enlarged upper and lower extremities and was diagnosed as a case of ML with the help of clinico-radiological studies and presented here because of focal gigantism involving all four limbs, which is very rare.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A child with enlarged extremities – A case of macrodystrophia lipomatosa

2020

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Surgical Management of Macrodystrophia Lipomatosa, a rare case report of the left hand middle finger macrodactyly

Sumarwoto

Hadinoto

Idulhaq

et al. 2021

International Journal of Surgery Case Reports

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Introduction Macrodystrophia Lipomatosa is a rare congenital non-hereditary disorder, characterized by the presence of gigantism of a small part of the limb or the whole limb due to an overgrowth and disproportionate growth of fibroadipose tissue, causing macrodactyly. Case presentation One case of the 14-year-old girl presented with an enlarged middle finger of her left hand since birth, discomfort during the last 6 months, and resistance to flex, was underwent surgery by performing debulking procedure and a yellow cord-like mass was obtained from the digital nerve covered and enlarged by fibrofatty tissue to the palm area, could be cut off completely. Imaging and histopathological examination revealed to macrodystrophia lipomatosa. We follow up the patient for the finger's range of motion, the neurological disturbance and re-enlargement of the tumor. Discussion Since there were compression of the nerves, functional impairment due to enlarge fingers and cosmesis problems, the surgery was indicated. Types of surgery may include debulking of soft tissue, especially adipose tissue. The mass size was decrease, motor function and movement of the middle finger were normal post operatively, but sensory deficits persisted according to the distribution of the digital nerves. Three months and six years after surgery, no enlargement of the middle finger, normal motor function, normal finger movements but sensory deficit still persisted according to the distribution of the digital nerves. Conclusion Surgical management in macrodactyly due to macrodystrophia lipomatosa of the finger give the satisfactory result.

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