gms | German Medical Science

Objectives/Interrogation: Lipofibromatous Hamartoma (LFH) is a rare condition that involves diffuse infiltration of peripheral nerves by normal appearing fibrous and adipose tissue. The epineurium and perineurium are enlarged and distorted by excess fatty and fibrous tissues that infiltrate between and around nerve boundaries. The cause of LFH remains obscure; the tumor is usually present at birth and may be associated with digital enlargement. In the upper extremity, the median nerve is the most commonly involved but also the radial nerve and branches, the ulnar nerve and branches and even the entire brachial plexus may be interested.

Methods: In the period between 1989 and 2017 twelve patients (7 men and 5 women) were observed. The mean age was 20.4 years (range 35 to 15 years). Three patients had associated macrodactyly. Child with macrodactyly under age of 14 years old were excluded from this study. All patients showed a palpable mass, with sensitive peripheral nerve signs in 10 cases; MR examination was done in all cases.

The median nerve and its branches were involved in 10 cases, the ulnar and radial nerves respectively in one case. Open carpal tunnel release was done in 5 cases with the aim to decrease motor and sensor impairments. Microsurgical intra-neural dissection with partial excision of the fibrofatty tisuue of the neoplastic elements of the median nerve was performed in two cases. The median nerve was resected and grafted in one case.

Two cases involving the radial and ulnar nerves were treated with fascia decompression and epineurolysis. Two cases of LFH of the median nerve are under observation.

Results and Conclusions: Four patients had a regression of neurological symptoms after carpal tunnel decompression. Microsurgical dissection generated disappointing results in both cases treated. Poor sensory recovery was recorded when the entire median nerve was excised and reconstructed with sural nerve grafts. Any improved function was reported in the radial and ulnar nerves after fascia decompression and epineurolysis [1].

MRI is a valid a valid support in the preoperative differential diagnosis. Biopsy is not routinely recommended owing to potential functional deficits; The role of microsurgical debulking procedure remains unclear.On the basis of our experience and after reviewing the literature, the treatment should be limited to decompression of the ligament or fascia over the involved nerve; an epineurolysis may be additionally performed.