Median sinirin lipofibromatöz hamartomu

Sinirin lipofibromatöz hamartomu, çok ender rastlanan, en çok median siniri tutan, genellikle genç insanların ekstremitelerinde görülen, selim, konjenital bir periferik sinir tümörüdür. Sinir dokusu, diffüz olarak fibroadipöz doku ile infiltre olur ve sinir fasiküllerini invaze etmeden birbirinden ayırır. Median sinirin ve dallarının lipofibromatöz hamartomu, genellikle karpal tünel sendromu belirtileri ile birlikte görülür ve makrodaktili belirgindir. On sekiz yaşında bir kadın hastada median siniri sağ el bileği düzeyinde tutan, ikinci parmakta makrodaktiliye neden olan ve karpal tünel sendromu belirtilerine yol açan lipofibromatöz hamartom görüldü. Ameliyatta kitle boyunca median sinire gevşetme ve sinirin çevresindeki yağlı dokunun bir kısmına eksizyon yapıldı. Ameliyattan 14 ay sonra yapılan kontrolde hastanın yakınmalarının kaybolduğu, kitlenin oldukça küçüldüğü ve duyusal ya da motor fonksiyonlar açısından bir kayıp olmadığı saptandı.

Anahtar Kelimeler:

Erişkin, Manyetik rezonans görüntüleme, Median sinir, Cerrahi işlemler, operatif, El bileği, Tedavi sonucu, Lipom, Hamartom, Neoplazmlar, sinir dokusu, Karpal tünel sendromu

Lipofibromatous hamartoma of the median nerve

Lipofibromatous hamartoma of the nerve is a very uncommon, congenital, benign, peripheral nerve tumor. It is mostly encountered in the extremities of young adults, involving the median nerve in the majority of cases. The nerve tissue is infiltrated by diffuse fibroadipose tissue which dissociates the fasciculi without invasion. Patients with lipofibromatous hamartoma of the median nerve usually present with symptoms of carpal tunnel syndrome, usually accompanied by marked macrodactyly. Lipofibromatous hamartoma of the median nerve was encountered in an 18-year-old female patient, involving the wrist, causing macrodactyly of the index finger, and resulting in symptoms of carpal tunnel syndrome. Median nerve release and partial excision of the adipose tissue along the mass were performed. Fourteen months postoperatively, the patient had no complaints and the mass decreased in size without any motor or sensory functional losses.

Keywords:

Adult, Magnetic Resonance Imaging, Median Nerve, Surgical Procedures, Operative, Wrist, Treatment Outcome, Lipoma, Hamartoma, Neoplasms, Nerve Tissue, Carpal Tunnel Syndrome,

PDF

___

1.Amadio PC, Reiman HM, Dobyns JH. Lipofibromatous hamartoma of nerve. J Hand Surg [Am] 1988;13:67-75.
2. Guthikonda M, Rengachary SS, Balko MG, van Loveren H. Lipofibromatous hamartoma of the median nerve: case report with magnetic resonance imaging correlation. Neurosurgery 1994;35:127-32.
3. Paletta FX, Senay LC Jr. Lipofibromatous hamartoma of median nerve and ulnar nerve: surgical treatment. Plast Reconstr Surg 1981;68:915-21.
4. Warhold LG, Urban MA, Bora FW Jr, Brooks JS, Peters SB. Lipofibromatous hamartomas of the median nerve. J Hand Surg [Am] 1993;18:1032-7.
5. Gouldesbrough DR, Kinny SJ. Lipofibromatous hamartoma of the ulnar nerve at the elbow: brief report. J Bone Joint Surg [Br] 1989;71:331-2.
6. Hız M, Kasabalıgil A, Dervişoğlu S, Kasabalıgil A. Ulnar sinirin intranšral lipomu. Turkish Journal of Neoplasia 1992;1:49-52.
7. Herrick RT, Godsil RD Jr, Widener JH. Lipofibromatous hamartoma of the radial nerve: a case report. J Hand Surg [Am] 1980;5:211-3.
8. Jacob RA, Buchino JJ. Lipofibroma of the superficial branch of the radial nerve. J Hand Surg [Am] 1989;14:704-6.
9. Silverman TA, Enzinger FM. Fibrolipomatous hamartoma of nerve. A clinicopathologic analysis of 26 cases. Am J Surg Pathol 1985;9:7-14.
10. Price AJ, Compson JP, Calonje E. Fibrolipomatous hamartoma of nerve arising in the brachial plexus. J Hand Surg [Br] 1995;20:16-8.
11. Patel ME, Silver JW, Lipton DE, Pearlman HS. Lipofibroma of the median nerve in the palm and digits of the hand. J Bone Joint Surg [Am] 1979;61:393-7.
12. Sobel E, Giorgini RJ, Potter GK, Schwartz RD, Chieco TM. Progressive pedal macrodactyly: surgical history with 15 year follow-up. Foot Ankle Int 2000;21:45-50.
13 Davis TR. Diagnostic criteria for upper limb disorders in epidemiological studies. J Hand Surg [Br] 1998;23:567-9.
14.Aulisa L, Tamburrelli F, Padua R, Romanini E, Lo Monaco M, Padua L. Carpal tunnel syndrome: indication for surgical treatment based on electrophysiologic study. J Hand Surg [Am] 1998;23:687-91.
15. Jablecki CK, Andary MT, So YT, Wilkins DE, Williams FH. Literature review of the usefulness of nerve conduction studies and electromyography for the evaluation of patients with carpal tunnel syndrome. AAEM Quality Assurance Committee. Muscle Nerve 1993;16:1392-414.
16. Louis DS, Hankin FM, Greene TL, Dick HM. Lipofibromas of the median nerve: long-term follow-up of four cases. J Hand Surg [Am] 1985;10:403-8.
17. Terzis JK, Daniel RK, Williams HB, Spencer PS. Benign fatty tumors of the peripheral nerves. Ann Plast Surg 1978;1:193-216.
18. De Maeseneer M, Jaovisidha S, Lenchik L, Witte D, Schweitzer ME, Sartoris DJ, et al. Fibrolipomatous hamartoma: MR imaging findings. Skeletal Radiol 1997;26:155-60.
19. Hems TE, Burge PD, Wilson DJ. The role of magnetic resonance imaging in the management of peripheral nerve tumours. J Hand Surg [Br] 1997;22:57-60.
20.Marom EM, Helms CA. Fibrolipomatous hamartoma: pathognomonic on MR imaging. Skeletal Radiol 1999;28:260-4.
21. Callison JR, Thoms OJ, White WL. Fibro-fatty proliferation of the median nerve. Plast Reconstr Surg 1968;42:403-13.
22. Rowland SA. Case report: ten year follow-up of lipofibroma of the median nerve in the palm. J Hand Surg [Am] 1977;2:316-7.
23. Bergman FO, Blom SE, Stenstrom SJ. Radical excision of a fibro-fatty proliferation of the median nerve, with no neurological loss symptoms. Plast Reconstr Surg 1970;46:375-80.