Makrodistrofi lipomatoza: Olgu sunumu

Makrodistrofi lipomatoza, doğuştan sınırlı jigantizmin nadir bir türüdür. Sağ ayak başparmağında şişlik, şekil bo-zukluğu ve ayakkabı giymede zorluk şikayeti olan 40 yaşındaki erkek hastada, konvansiyonel röntgen, ultrasonografi ve bilgisayarlı tomografi incelemeleri yapıldı. Radyolojik incelemelerde ayak başparmağında dorsale ve içe açılanma, kemik yapıda hipertrofi ve dejenerasyon, medüller kemikte yoğunluk artışı, çevre yumuşak dokudaki kaslara infiltrasyon gösteren lipomatöz kitle izlendi. Rekonstrüksiyon amacıyla lipomatöz dokular kısmi olarak çıkarıldı. Örneğin patolojik incelemesinde düzenli yapıda yağ, bağ ve nöral doku elemanları izlendi. Ameliyat sonrası iki yıllık sürede hastanın klinik şikayeti olmadı ve lezyon alanında büyüme saptanmadı.

Macrodystrophia lipomatosa: A case report

Macrodystrophia lipomatosa is a rare form of congenital localized gigantism. A forty year old male patient presented with complaints of swelling and deformity in the right toe and difficulty in wearing shoes. Conventional radiographic examination, ultrasonography, and computed tomography showed dorsal and medial deviations in the right toe, hypertrophy and degeneration in bone structure, increased density in the medullary bone, and a lipomatous mass with infiltration to the adjacent soft tissue muscles. Partial removal of the lipomatous tissues was performed. Microscopic examination of the specimen revealed fat deposits, ligamentous, and neural tissue elements in normal structure. The patient had no complaints and there was no change in the size of the lesion within a two-year follow-up period.

___

  • 1.Wang YC, Jeng CM, Marcantonio DR, Resnick D. Macrodystrophia lipomatosa. MR imaging in three patients. Clin Imaging 1997;21:323-7.
  • 2.Brodwater BK, Major NM, Goldner RD, Layfield LJ. Macrodystrophia lipomatosa with associated fibrolipomatous hamartoma of the median nerve. Pediatr Surg Int 2000;16:216-8.
  • 3.Sone M, Ehara S, Tamakawa Y, Nishida J, Honjoh S. Macrodystrophia lipomatosa: CT and MR findings. Radiat Med 2000;18:129-32.
  • 4.Patnaik S. Radiographic and CT features of macrodystrophia lipomatosa [Letter]. Ind J Radiol Imag 2000;10:113-5.
  • 5.Stern PJ, Nyquist SR. Macrodactyly in ulnar nerve distribution associated with cubital tunnel syndrome. J Hand Surg [Am] 1982;7:569-71.
  • 6.Soler R, Rodriguez E, Bargiela A, Martijnez C. MR findings of macrodystrophia lipomatosa. Clin Imaging 1997; 21:135-7.
  • 7. Gupta SK, Sharma OP, Sharma SV, Sood B, Gupta S. Macrodystrophia lipomatosa: radiographic observations. Br J Radiol 1992;65:769-73.
  • 8. Dayal J, Tyagi AR, Jain P, Ranjan R, Sharma A. Macrodystrophia lipomatosa [Images]. Ind J Radiol Imag 2001: 11: 139-41.
  • 9. Ranawat CS, Arora MM, Singh RG. Macrodystrophia lipomatosa with carpal-tunnel syndrome. A case report. J Bone Joint Surg [Am] 1968;50:1242-4.
  • 10. Goldman AB. Heritable diseases of the connective tissue, epiphyseal dysplasias and related conditions. In: Resnick D, editor. Diagnosis of bone and joint disorders. Vol. 6, 3rd ed. Philadelphia: W.B. Saunders; 1995. p. 4148-62.
  • 11. Silverman TA, Enzinger FM. Fibrolipomatous hamartoma of nerve. A clinicopathologic analysis of 26 cases. Am J Surg Pathol 1985;9:7-14.
Acta Orthopaedica et Traumatologica Turcica-Cover
  • ISSN: 1017-995X
  • Başlangıç: 2015
  • Yayıncı: Türk Ortopedi ve Travmatoloji Derneği