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Pediatric peripheral neuropathy in proteus syndrome
1Department of Surgery, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903, USA.
Annals of Plastic Surgery
|May 26, 1998
Summary
Proteus syndrome can cause painful median nerve compression due to hamartomas. Surgical decompression of the nerve, involving hamartoma removal, successfully resolved symptoms in a pediatric patient.
Area of Science:
- Medical Genetics
- Neurology
- Dermatology
Background:
- Proteus syndrome is a rare congenital disorder characterized by various hamartomas and overgrowths.
- Peripheral nerve involvement, specifically median nerve compression neuropathy, is an underreported manifestation.
Observation:
- A 5-year-old girl with Proteus syndrome presented with severe left hand pain due to distal median nerve compression.
- Surgical exploration revealed a lipofibromatous hamartoma directly involving the median nerve.
Findings:
- The patient underwent surgical release of the transverse carpal ligament and epineurectomy to remove the hamartoma.
- Post-operative follow-up at 9 months showed complete resolution of symptoms.
Implications:
- This case is the first to describe a peripheral nerve hamartoma in Proteus syndrome.
- Nerve decompression with hamartoma removal is a viable first-line treatment option, potentially avoiding more extensive nerve resection.