Escuela de Medicina Macrodystrophia Lipomatosa of the foot. Extensile dorsal approach for Reductive Surgery. Report of 4 cases Authors: Jorge Briceño F, Mario Abarca M, Jorge Filippi N, Andres Villa M, Pablo Mery P, Joaquin Palma M. Foot and Ankle Unit Department of Orthopedic Surgery Pontificia Universidad Catolica de Chile medicina.uc.cl
Escuela de Medicina DISCLOSURE Macrodystrophia Lipomatosa of the foot. Extensile dorsal approach for Reductive Surgery. Report of 4 cases The authors have no conflicts to disclose. medicina.uc.cl
INTRODUCTION. - Macrodystrophia lipomatosa (MDL) is a rare cause of local gigantism affecting hands or feet. - Non-hereditary and congenital. - Unknown etiology. - The main characteristic of this gigantism is the overgrowth of all the mesenchematic structures like bone, tendons, vessels, nerves and predominantly the fibroadipose tissue. Feriz H. Macrodystrophia lipomatosa progressiva. Virchows Arch Pathol Anat Physiol Clin Med 1925;260:308-68. - The low incidence of this pathology leads to scarce treatment recommendations including cytoreduction and amputation Prasetyono T, et al. Arch Plast Surg 2015;42:391-406
OBJECTIVE Describe the surgical technique and clinical results of extended dorsal approach in the management of 4 cases of MDL of the foot.
METHODS - Retrospective study of case series. - Collection of pre-operative radiological and clinical images; Intraoperative data and postoperative clinical and radiological images. - Review of clinical record of patients.
SURGICAL TECHNIQUE - An extensile dorsal approach was performed in all the patients preserving the neurovascular bundles. - Bone resection was performed until the toe got an harmonic appearance related to the rest of the foot. - In one case removal of the distal phalanx was performed to achieve adequate reduction - Then all the remnant soft tissues were removed from dorsal and distal, keeping always the plantar neurovascular supply. - Skin closure was done with non-absorbable sutures.
CLINICAL CASE EXAMPLE 1 A 38 year old male patient, with a disproportionate increase in the size of the left hallux. Difficulty with shoe wear, pain and paresthesias. No history of neurofibromatosis or other relevant family diseases. Physical examination: preserved sensitivity, restricted mobility in MTP and IP joints. No signs of active infection X-rays: shows a diffuse increase in size of the 1st metatarsal and hallux phalanges with severe involvement of their respective joints. Surgical plan: Partial amputation and cytoreduction of hallux.
CLINICAL CASE EXAMPLE 1 Results and follow up: - Biopsy showed abundant fibrous tissue and confirmed MDL. - Patient evolved with superficial wound complication but no need for further surgeries - At the last follow up with no sensory deficit, previous mobility preserved and with an excellent patient satisfaction.
CLINICAL CASE EXAMPLE 2 Male patient of 41 year old, with no family history of neurofibromatosis or other relevant diseases. No previous interventions. Physical examination: - Disproportionate increase of 4th left toe. - Conservative sensitivity, no wounds. - Decreased motion in MTP and IP joints. Radiographs confirmed increased size of phalanges.
CLINICAL CASE EXAMPLE 2 Clinical follow up: - No wound complications and preserved sensitivity. - Prior mobility preserved without pain. - Patient satisfaction was excellent in terms of functionality and cosmesis.
RESULTS CASE AGE GENDER TOE COMPLICATIONS SATISFACTION 1 38 M HALLUX YES, Superficial wound dehiscence SATISFACTION WITH NO RESERVE 2 41 M 4TH NO SATISFACTION WITH NO RESERVE 3 15 F 2ND YES, Superficial wound dehiscence SATISFACTION WITH MINOR RESERVE 4 45 F 2ND NO SATISFACTION WITH NO RESERVE
CONCLUSION - The reductive surgery for adults with symptomatic MDL of the foot offers good results, in terms of relief of pain and cosmesis. - The use of extended dorsal approach and cytoreduction provides excellent surgical exposure, preserving neurovascular bundles and allows an appropiate reduction of excessive tissue. - Based on our clinical results and the high satisfaction of our patients, we suggest cytoreduction surgery as an alternative to amputation in selected patients with MDL diagnosis.
REFERENCES 1. Feriz H. Macrodystrophia lipomatosa progressiva. Virchows Arch Pathol Anat Physiol Clin Med 1925;260:308-68. 2. Prasetyono T, Hanafi E., Astriana W.. A Review of Macrodystrophia Lipomatosa: Revisitation. Arch Plast Surg 2015;42:391-406. 3. Khan RA, Wahab S, Ahmad I, et al. Macrodystrophia lipomatosa: four case reports. Ital J Pediatr 2010;36:69. 4. Pandey AK. Magnetic resonance imaging of a case of monomelic macrodystrophia lipomatosa. Australas Radiol 2007; 51 Suppl:B227-30. 5. Wang YC, Jeng CM, Marcantonio DR, et al. Macrodystrophia lipomatosa. MR imaging in three patients. Clin Imaging 1997;21:323-7. 6. Van Breuseghem I, Sciot R, Pans S, Geusens E, Brys P, De Wever I. Fibrolipomatous hamartoma in the foot: atypical MR imaging findings. Skeletal Radiol. 2003;32:651 5. 7. Tuzuner T, Parlak AH, Kavak A, et al. A neglected case of macrodystrophia lipomatosa of the foot in an elderly man. J Am Podiatr Med Assoc 2005;95:486-90 8. Ozturk A, Baktiroglu L, Ozturk E, et al. Macrodystrophia lipomatosa: a case report. Acta Orthop Traumatol Turc 2004; 38:220-3. 9. Turkington JR, Grey AC. MR imaging of macrodystrophia lipomatosa. Ulster Med J 2005;74:47-50. 10. Upadhyay D, Parashari UC, Khanduri S, et al. Macrodystrophia lipomatosa: radiologic-pathologic correlation. J Clin Imaging Sci 2011;1:18. 11. Soler R, Rodriguez E, Bargiela A, et al. MR findings of macrodystrophia lipomatosa. Clin Imaging 1997;21:135-7. 12. S. Valverde, J. Llauger y J. Palmer. Macrodistrofia lipomatosa del pie asociada a lipomatosis del nervio plantar. Caso Clínico. Radiología. 2010;52(1):89 91 13. Van der Meer S, Nicolai JP, Schut SM, et al. Bilateral macrodystrophia lipomatosa of the upper extremities with syndactyly and multiple lipomas. J Plast Surg Hand Surg 2011;45: 303-6. 14. Watt AJ, Chung KC. Macrodystrophia lipomatosa: a reconstructive approach to gigantism of the foot. J Foot Ankle Surg 2004;43:51-5. 15. Ho CA, Herring JA, Ezaki M. Long-term follow-up of progressive macrodystrophia lipomatosa: a report of two cases. J Bone Joint Surg Am 2007;89:1097-102.