J Korean Orthop Assoc.  2017 Dec;52(6):566-571. 10.4055/jkoa.2017.52.6.566.

Surgical Treatment of Macrodystrophia Lipomatosa Involving Superficial Peroneal Nerve in the Foot and Ankle

• Kang SH
• Shin SJ
• Eho YJ

• KMID: 2421352
• DOI: http://doi.org/10.4055/jkoa.2017.52.6.566

Abstract

Macrodystrophia lipomatosa is a congenital disease characterized by gradual proliferation in the mesenchymal cell, such as fibroadipose tissue. Pathologically, fatty tissue is deposited in the nerve sheath, periosteum, bone marrow, and subcutaneous tissue, contributing to the macrodactyly of the foot. To date, there has not been any report on macrodystrophia lipomatosa of the superficial peroneal nerve in the Korean orthopedic literature. Conservative approach, such as decompression or debulking surgery, is recommended due to neurogenic dysfunction. However, we report a 43-year-old male with macrodystrophia lipomatosa involving the superficial peroneal nerve of the right foot and ankle, who underwent a second toe ray amputation as well as soft tissue and nerve resection.

Keyword

macrodystrophia lipomatosa; superficial peroneal nerve

MeSH Terms

Adipose Tissue
Adult
Amputation
Ankle*
Bone Marrow
Cytoreduction Surgical Procedures
Decompression
Foot*
Humans
Limb Deformities, Congenital
Male
Orthopedics
Periosteum
Peroneal Nerve*
Subcutaneous Tissue
Toes

Figure

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  Figure 1 Photographs showing cordlike mass on the right foot and ankle with second toe macrodactyly.

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  Figure 2 Radiographs showing interphalangeal and metatarsophalangeal joint arthritis with hypertrophied phalanx and multiple bony spur.

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  Figure 3 Coronal magnetic resonance imaging of the forefoot showing an enlarged superficial peroneal nerve (white arrows). (A) Nerve fascicles of the superficial peroneal nerve are surrounded by fat tissue with high signal intensity within nerve sheath on T1-weighted imaging. T2-weighted image showing fat tissue with intermediate signal intensity (B) and low signal intensity on T2 fat suppressed image (C).

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  Figure 4 Intraoperative photograph showing medial dorsal cutaneous branch of the superficial peroneal nerve, which was hypertrophied with fatty tissue.

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  Figure 5 (A) Infiltration of the epineurium and perineurium by adipose and fibrous tissue, causing an enlargement of the nerve (H&E, ×12.5). (B) Concentric perineurial fibrous tissue and pseudo-onion bulb formation (H&E, ×100).

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  Figure 6 The last follow-up photograph and radiographs of the foot showing no evidence of recurrence.

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