MR diagnosis of fibrolipomatous hamartoma of nerve: association with nerve territory-oriented macrodactyly (macrodystrophia lipomatosa)

✍ Scribed by William L. Boren; Robert E. C. Henry; Kenneth Wintch

• Publisher: Springer
• Year: 1995
• Tongue: English
• Weight: 655 KB
• Volume: 24
• Category: Article
• ISSN: 0364-2348
• DOI: 10.1007/bf00198419

✦ Synopsis

Clinical information

A 42-year-old man presented with enlargement and decreased range of motion of the right thumb. The thumb had been enlarged at birth, with progressively greater relative enlargement through the second decade of life. A decreasing range of motion of the thumb had been noted over the last 5 years. Loss of sensation was also reported, involving portions of the long and index fingers. No additional digital enlargement or neurological symptoms were present anywhere in the body. The patient denied antecedent trauma to the involved areas, pain, skin changes, or a family history suggestive of neurofibromatosis.

On physical examination, there was marked enlargement of the right thumb and thenar eminence, both areas soft and compressible. The thumb metacarpophalangeal joint was extended and abducted, the interphalangeal (IP) joint flexed, with minimal motion at both sites (Fig. 1A). Reduced sensation was noted, involving portions of the index and long fingers, as per history.

Radiographs demonstrated increased length and diameter of the thumb phalanges without cortical thickening or contour disturbance. There were exuberant hypertrophic changes at the MCP and IP joint