STUDY 0F OPHTHALMOLOGIC PLEXIFORM NEUROFIBROMATOSIS TYPE-1 IN AN ADULT MALE

B. Sheikh, S. Fatima, M. A. Memon, K.D. Pitafi

Abstract


Neurofibromatosis type one in its primitive age was known as von Recklighausens  disease.Neurofibromatosis type 1 is an autosomal dominant disorder of chromosomal 17q that appear in 4000 to 5000 births. The patients who  show mild symptoms in early age may develop more severe problems. The patient here shows plexiform neurofibromatosis on the left side of the eye, and was gone through surgery. The eye plexiform restarted growing gradually after few months of surgery, and covered almost half of the face, then the patient undergone second surgery. In plexiform of eye the proliferation of Schwann cells occur inside the nerve sheath, producing an irregularly thickened, distorted tortuous structure. The tumors develop when cells of peripheral nerves multiply out of control within the nerve sheath; the eye plexiform tumor was benign, confirmed by histopathology of tumors. The patient shows large size café-au- lait macules on the chest, freckling, tumors on both the palms are very well prominent. The case was sporadic. Keywords: Plexiform, von Recklinghausen's, café-au- lait macules. 1.


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