• A Salon, S Guero, and J Glicenstein.
• Service d'Orthopédie, Traumatologie, Hôpital Raymond Poincaré, Garches.
• Ann Chir Main Memb Super. 1995 Jan 1;14(6):284-95.

AbstractLipofibromatous hamartoma of the median nerve and its branches is rare, but is the most frequent lesion associated with digital hypertrophy in the upper limb. We present a pediatric series of 8 patients, two of whom had bilateral involvement. Digital enlargement or true macrodactyly was the presenting complaint. Fatty infiltration of the palm was a constant finding and is considered very suggestive of lipofibromatous hamartoma of the median nerve, as confirmed by MRI in cases of uncertain diagnosis. No causal link between the nerve hamartoma and the enlargement of soft tissues in the territory of the nerve has yet been proven; it appears to be a regional disease related to unknown development factors, and other congenital anomalies may be associated in the same territory. Treatment was mostly symptomatic, with carpal tunnel release and superficial removal of epineural proliferation; no interfascicular dissection was performed and we never made any attempt to completely resect the lipofibromatous constituents; no median nerve resection was performed. Debulking of the palm and enlarged digits was associated with nerve decompression, but true macrodactyly required a separate operative procedure and is not the subject of this study. Mean follow-up is 8 years and 5 operated lipofibromas have been reviewed after 13 years. Two-point discrimination was often abnormal, but sensory impairment was stabilized, as well as soft tissue enlargement. However no objective argument can currently support more radical nerve surgery, and the superiority of nerve resection on the course of digital hypertrophy has not been proven.

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