Macrodystrophia Lipomatosa of the foot. Extensile dorsal approach for Reductive Surgery. Report of 4 cases

Abstract

Macrodystrophia lipomatosa (ML) is a rare cause of local gigantism affecting hands or feet of congenital non-hereditary origin and unknown etiology. The main characteristic of this disease is the overgrowth of the mesenchymal structures as bone, tendons, vessels, nerves and, predominantly, the fibroadipose tissue. The low frequency of this pathology implies a difficulty to establish management guidelines. The most recommended treatment for this condition is the reductive surgery as an alternative to amputation of the affected segment. Our objective is to report the clinical results of the reductive surgery in four patients with ML in the forefoot. Methods: Four cases of ML surgically treated in our center between 2008 and 2016 were retrospectively analyzed after approval from our institutional review board. For each case, clinical history at admission, pre and post-operative radiographs and pre and post-operative clinical images were obtained. Results: Patients were adults between 28 and 38 years old and followed between 1 and 4 years. The toes involved were: 1 hallux, 2 second toes and 1 fourth toe. All had failed conservative treatment prior to surgery. SURGICAL TECHNIQUE: An extensile dorsal approach preserving the neurovascular bundles was performed. Bone was resected until a harmonic appearance of the toe related to the rest of the foot was obtained. In one case, removal of the distal phalanx was necessary to achieve adequate reduction. The remnant soft tissue was resected from dorsal and distal. Skin was closed using non-absorbable sutures. Wound dehiscence was observed in 2 patients and managed conservatively. No major complications were observed. All the patients were satisfied and able to wear regular shoes postoperatively. Conclusion: The reductive surgery for adults with symptomatic ML of the foot offers good functional results. The extensile dorsal approach allows an excellent surgical exposure, preservation of neurovascular supply and adequate tissue resection. Based on our clinical results and the high satisfaction observed in our 4 patients, we suggest reductive surgery as a good alternative to amputation in selected patients with ML.