Macrodystrophia lipomatosa: radiographic observations

Op, Sharma; Sharma, Siddharth; Sood, Bimal; Gupta, S. K.

doi:10.1259/0007-1285-65-777-769

Cited by 55 publications

(40 citation statements)

References 13 publications

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“…The pathogenesis of bony enlargement is thought to be because of endosteal and periosteal deposition of bone. [4] Radiological investigations include X-ray, USG, Doppler, and MRI. X-ray show lucent soft tissue overgrowth as well as hypertrophy of bony structures in the distribution of median and plantar nerve.…”

Section: Discussionmentioning

confidence: 99%

Macrodystrophia Lipomatosa- A Rare Congenital Anomaly: A Case Report and Review of Literature

Tripathi¹,

Nanda²,

Kumar³

et al. 2016

AIMDR

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Macrodystrophia lipomatosa is a rare presentation and only few cases are reported in scientific literature. We report a case of Macrodystrophia lipomatosa in a 7 year young girl from a tertiary care hospital Mumbai. Case presentation: A 7 year old female was presented to outpatient department with progressive enlargement of right lower limb with increase in size of right great toe since birth. On clinical examination there was non tender fluctuant soft tissue swelling with good functional limb. Scannogram showed reduction of the medial compartment of right knee joint and the right lower limb length was slightly longer compared to the left side with presence of soft tissue swelling on medial distal thigh and knee region. MRI showed un-encapsulated subcutaneous fatty tissue involving the entire right lower limb along its medial aspect up to the great toe with fatty infiltration of the vastus medialis muscle suggestive of macrodystrophia lipomatosa. Arterial and venous colour Doppler study of right lower limb was normal. Conclusion: Macrodystrophia lipomatosa is a rare congenital, non hereditary condition with localized macrodactyly and proliferation of mesenchymal element and marked increase in fibroadipose tissue. X-ray and MRI are investigation of choice. Doppler study shows normal vascularity.

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Section: Discussionmentioning

confidence: 99%

Macrodystrophia Lipomatosa- A Rare Congenital Anomaly: A Case Report and Review of Literature

Tripathi¹,

Nanda²,

Kumar³

et al. 2016

AIMDR

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“…In 1925 this condition was first described by Feriz, who used the term macrodystrophia lipomatosa to refer to localised gigantism of the lower limb. Golding extended the term, in 1960, to include involvement of the upper limb 5 .…”

Section: Discussionmentioning

confidence: 99%

A case of macrodystrophia lipomatosa of 5th digit of hand with literature review

Patil¹,

Vijayakumar²,

Vijayakumar³

2013

Int J of Biomed & Adv Res

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“…An enlarged digit will be incorrectly diagnosed as macrodactyly. [24] , so a focused examination is required with consideration of differential diagnoses. History of trauma may be suggestive of hematoma.…”

Section: Differential Diagnosis Of Macrodactylymentioning

confidence: 99%

Macrodactyly of Lower Limbs-an Update

Natarajan¹,

Dhua²,

Garg³

2016

jemds

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Congenital macrodactyly is a rare congenital malformation characterized by progressive enlargement of all mesenchymal elements of a digit. The most characteristic finding noted was excessive overgrowth of fibrofatty tissue with unusually large fatty lobules fixed by a mesh of dense fibrous tissue. Hypertrophy and tortuosity of the digital nerve, a striking feature in macrodactyly of the hand was notably absent in cases affecting the foot. Most of the cases are reported as isolated anomalies. In macrodactyly of the foot, excessive proliferation and accumulation of adipose tissue was the basic lesion whereas involvement of the nerve is the fundamental lesion in gigantism of the hand. The final pathway of genesis of these anomalies must be either the local deficiency of a growth inhibiting factor or local expression of a basic intrinsic factor leading to excessive growth of all elements of the digit.

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Macrodystrophia lipomatosa: radiographic observations

Cited by 55 publications

References 13 publications

Macrodystrophia Lipomatosa- A Rare Congenital Anomaly: A Case Report and Review of Literature

Macrodystrophia Lipomatosa- A Rare Congenital Anomaly: A Case Report and Review of Literature

A case of macrodystrophia lipomatosa of 5th digit of hand with literature review

Macrodactyly of Lower Limbs-an Update

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