Abstract
Background
Lipomatosis of the nerve (LN) is a rare disorder characterized by the massive enlargement of peripheral nerves, frequently accompanied by generalized fibroadipose proliferation and skeletal overgrowth. The treatment of this disorder remains controversial, in part because of the rarity and the variability of presentation. Some authors have advocated total resection of this benign lesion including the functioning nerve, while others recommend symptomatic decompression alone.
Methods
We have been routinely following a 10-year-old boy for lipomatosis of the median nerve at the wrist noted shortly after birth. He underwent median nerve resection accompanied by sural nerve grafting at another institute. We review the literature on LN and the efficacy of nerve grafting.
Results
Clinically, he made a good recovery, with mild loss of thenar function and relatively preserved sensation. Serial magnetic resonance imaging over 5 years has revealed progression of the LN at both coaptation sites, fibrofatty proliferation within the nerve grafts as well as distal digital nerves, and enlargement of a fibrous scar at the coaptation sites. This has never been reported in the 9 decades of study of this disease.
Conclusion
We present the first medium-term follow-up of a patient who underwent nerve sacrifice to attempt to cure the LN alongside a historical review of treatment. We believe that macroscopic gross total resection (i.e., microscopic subtotal resection) is insufficient in stopping the potential progression of this hamartomatous lesion because of the persistent effect of trophic factors.
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References
Amadio PC, Reiman HM, Dobyns JH (1988) Lipofibromatous hamartoma of nerve. J Hand Surg 13:67–75
Bains R, Kotwal A, Saeed W (2006) Recurrent carpal tunnel syndrome in a child due to fibrolipomatous hamartoma of the median nerve successfully treated by limited excision and decompression. J Plast Reconstr Aesthet Surg JPRAS 59:1394–1397
Barsky AJ (1967) Macrodactyly. J Bone Joint Surg Am 49:1255–1266
Bergman FO, Blom SE, Stenstrom SJ (1970) Radical excision of a fibro-fatty proliferation of the median nerve, with no neurological loss symptoms. Plast Reconstr Surg 46:375–380
Callison JR, Thoms OJ, White WL (1968) Fibro-fatty proliferation of the median nerve. Plast Reconstr Surg 42:403–413
Cavallaro MC, Taylor JA, Gorman JD, Haghighi P, Resnick D (1993) Imaging findings in a patient with fibrolipomatous hamartoma of the median nerve. AJR Am J Roentgenol 161:837–838
Cherqui A, Sulaiman WA, Kline DG (2009) Resection and nerve grafting of a lipofibrohamartoma of the median nerve: case report. Neurosurgery 65:A229–A235
Chiao HC, Marks KE, Bauer TW, Pflanze W (1987) Intraneural lipoma of the sciatic nerve. Clinical orthopaedics and related research. Clin Orthop Relat Res 221:267–271
Clavijo-Alvarez JA, Price M, Stofman GM (2010) Preserved neurologic function following intraneural fascicular dissection and nerve graft for digital and median nerve lipofibromatous hamartoma. Plast Reconstr Surg 125:120e–122e
Kim DH, Midha R, JA Murovic, RJ Spinner (2007) Kline and Hudson’s nerve injuries: operative results for major nerve injuries, entrapments and tumors, p 454
De Maeseneer M, Jaovisidha S, Lenchik L, Witte D, Schweitzer ME, Sartoris DJ, Resnick D (1997) Fibrolipomatous hamartoma: MR imaging findings. Skelet Radiol 26:155–160
Emmett AJ (1965) Lipomatous Hamartoma of the Median Nerve in the Palm. Br J Plast Surg 18:208–213
Fletcher C, Unni K, Mertens F (2002) World Health Organization Classification of Tumours. Pathology and genetics: Tumors of soft tissue and bone. IARC Press Lyon, France
Forthman C, Blazar P (2011) Surgical treatment of nerve tumors in the distal upper extremity. Wolters Kluwer, Philadelphia
Frykman GK, Wood VE (1978) Peripheral nerve hamartoma with macrodactyly in the hand: Report of three cases and review of the literature. J Hand Surg 3:307–312
Goldman AB, Kaye JJ (1977) Macrodystrophia lipomatosa: radiographic diagnosis. AJR Am J Roentgenol 128:101–105
Hebert-Blouin MN, Scheithauer BW, Amrami KK, Durham SR, Spinner RJ (2012) Fibromatosis: a potential sequela of neuromuscular choristoma. J Neurosurg 116:399–408
Houpt P, Storm van Leeuwen JB, van den Bergen HA (1989) Intraneural lipofibroma of the median nerve. J Hand Surg Am 14:706–709
Ho CA, Herring JA, Ezaki M (2007) Long-term follow-up of progressive macrodystrophia lipomatosa. A report of two cases. J Bone Joint Surg Am 89:1097–1102
Hsu CC, Chen DJ, Chen HC (2005) Lipofibromatous hamartoma of the median nerve with long-term follow-up. Chang Gung Med J 28:111–116
Hueston JT, Millroy P (1968) Macrodactyly associated with hamartoma of major peripheral nerves. Aust N Z J Surg 37:394–397
Inglis K (1950) Local gigantism (a manifestation of neurofibromatosis): its relation to general gigantism and to acromegaly; illustrating the influence of intrinsic factors in disease when development of the body is abnormal. Am J Pathol 26:1059–1083
Ishida O, Ikuta Y (1998) Long-term results of surgical treatment for macrodactyly of the hand. Plast Reconstr Surg 102:1586–1590
Johnson RJ, Bonfiglio M (1969) Lipofibromatous hamartoma of the median nerve. J Bone Joint Surg 51:984–990
Kalen V, Burwell DS, Omer GE (1988) Macrodactyly of the hands and feet. J Pediatr Orthop 8:311–315
Kim DH, Kline DG (1996) Management and results of peroneal nerve lesions. Neurosurgery 39:312–319, discussion 319–320
Kim DH, Murovic JA, Tiel RL, Kline DG (2004) Management and outcomes in 318 operative common peroneal nerve lesions at the Louisiana State University Health Sciences Center. Neurosurgery 54:1421–1428, discussion 1428–1429
Kurek KC, Luks VL, Ayturk UM, Alomari AI, Fishman SJ, Spencer SA, Mulliken JB, Bowen ME, Yamamoto GL, Kozakewich HP, Warman ML (2012) Somatic mosaic activating mutations in PIK3CA cause CLOVES syndrome. Am J Hum Genet 90:1108–1115
Lindhurst MJ, Sapp JC, Teer JK, Johnston JJ, Finn EM, Peters K, Turner J, Cannons JL, Bick D, Blakemore L, Blumhorst C, Brockmann K, Calder P, Cherman N, Deardorff MA, Everman DB, Golas G, Greenstein RM, Kato BM, Keppler-Noreuil KM, Kuznetsov SA, Miyamoto RT, Newman K, Ng D, O’Brien K, Rothenberg S, Schwartzentruber DJ, Singhal V, Tirabosco R, Upton J, Wientroub S, Zackai EH, Hoag K, Whitewood-Neal T, Robey PG, Schwartzberg PL, Darling TN, Tosi LL, Mullikin JC, Biesecker LG (2011) A mosaic activating mutation in AKT1 associated with the Proteus syndrome. N Engl J Med 365:611–619
Marom EM, Helms CA (1999) Fibrolipomatous hamartoma: pathognomonic on MR imaging. Skelet Radiol 28:260–264
Mason M (1953) Presentation of cases. Proceedings of the American Society for Surgery of the Hand. J Bone Joint Surg 273–275
Mikhail IK (1964) Median nerve lipoma in the hand. J Bone Joint Surg Br 46:726–730
Moore BH (1942) Macrodactyly and associated peripheral nerve changes. J Bone Joint Surg Br 24:617–631
Narakas A (1976) The fibrolipomatous hypertrophy of the main nerves of the upper extremity. Handchirurgie 8:89–95
Paletta FX, Rybka FJ (1972) Treatment of hamartomas of the median nerve. Ann Surg 176:217–222
Paletta FX, Senay LC Jr (1981) Lipofibromatous hamartoma of median nerve and ulnar nerve: surgical treatment. Plast Reconstr Surg 68:915–921
Patel ME, Silver JW, Lipton DE, Pearlman HS (1979) Lipofibroma of the median nerve in the palm and digits of the hand. J Bone Joint Surg 61:393–397
Pulvertaft RG (1964) Unusual tumours of the median nerve. Report of two cases. J Bone Joint Surg Br Vol 46:731–733
Robbins SL, Kumar V, Cotran RS (2010) Robbins and Cotran pathologic basis of disease. Saunders/Elsevier, Philadelphia
Rohilla S, Jain N, Sharma R, Dhaulakhandi DB (2012) Macrodystrophia lipomatosa involving multiple nerves. J Orthop Traumatol Off J Ital Soc Orthop Traumatol 13:41–45
Rowland SA (1967) Lipofibroma of the median nerve in the palm. J Bone Joint Surg Am 49:1309–1313
Rowland SA (1977) Case report: ten year follow-up of lipofibroma of the median nerve in the palm. J Hand Surg 2:316–317
Silverman TA, Enzinger FM (1985) Fibrolipomatous hamartoma of nerve. A clinicopathologic analysis of 26 cases. Am J Surg Pathol 9:7–14
Spinner RJ, Scheithauer BW, Amrami KK, Wenger DE, Hebert-Blouin MN (2012) Adipose lesions of nerve: the need for a modified classification. J Neurosurg 116:418–431
Terzis JK, Barmpitsioti A (2010) Axillary nerve reconstruction in 176 posttraumatic plexopathy patients. Plast Reconstr Surg 125:233–247
Terzis JK, Kokkalis ZT (2008) Outcomes of secondary reconstruction of ulnar nerve lesions: our experience. Plast Reconstr Surg 122:1100–1110
Terzis JK, Konofaos P (2011) Radial nerve injuries and outcomes: our experience. Plast Reconstr Surg 127:739–751
Tsuge K (1985) Treatment of macrodactyly. J Hand Surg 10:968–969
Tsuge K, Ikuta Y (1973) Macrodactyly and fibro-fatty proliferation of the median nerve. Hiroshima J Med Sci 22:83–101
Ulrich D, Ulrich F, Schroeder M, Pallua N (2009) Lipofibromatous hamartoma of the median nerve in patients with macrodactyly: diagnosis and treatment of a rare disease causing carpal tunnel syndrome. Arch Orthop Trauma Surg 129:1219–1224
van der Meer S, Nicolai JP, Meek MF (2007) Macrodystrophia lipomatosa: macrodactyly related to affected nerves, and a review of the literature. Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Mikrochirurgie der Peripheren Nerven und Gefasse : Organ der Vereinigung der Deutschen Plastischen Chirurgen. Handchir Mikrochir Plast Chir 39(6):414–417
Warhold LG, Urban MA, Bora FW Jr, Brooks JS, Peters SB (1993) Lipofibromatous hamartomas of the median nerve. J Hand Surg 18:1032–1037
Yamauchi T, Yoshii T, Sempuku T (2009) Whether to excise a lipofibroma of the median nerve. Scandinavian journal of plastic and reconstructive surgery and hand surgery/Nordisk plastikkirurgisk forening [and] Nordisk klubb for handkirurgi. Scand J Plast Reconstr Surg Hand Surg 43(6):346–349
Yeoman PM (1964) Fatty infiltration of the median nerve. J Bone Joint Surg Br Vol 46:737–739
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The authors appreciate the assistance of David Factor, Rochester, MN.
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Mahan, M.A., Amrami, K.K., Niederhauser, B.D. et al. Progressive nerve territory overgrowth after subtotal resection of lipomatosis of the median nerve in the palm and wrist: a case, a review and a paradigm. Acta Neurochir 155, 1131–1141 (2013). https://doi.org/10.1007/s00701-013-1707-z
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DOI: https://doi.org/10.1007/s00701-013-1707-z