Neurofibromatosis
Write short note on neurofibroma.
Answer. Neurofibroma is a benign tumor arising from connective tissue of a nerve containing ectodermal neural and mesodermal connective tissue components.
Types of Neurofiroma
Nodular:
- Single, smooth, fim, tender swelling which moves horizontally or perpendicular to direction of nerve.
“Best Ways To Manage Neurofibroma”
Plexiform:
- Occurs along distribution of trigeminal nerve in skin of face.
- Attain enormous size with thickening of skin which hang downwards.
Von-Recklinghausen’s Disease:
- Inherited disease with multiple neurofiromas in body.
- It can be cranial, spinal or peripheral
- Associated with pigmented spots on skin, i.e. Caféau-lait spots.
“Types Of Neurofibromas Explained”
Elephantiasis:
- Origin is congenital and involve limbs.
- Skin of limb is thickened, dry and coarse.
Cutaneous:
- Small, multiple, firm/hard nodules arising from terminal ends of dermal nerves
- It can be pedunculated or sessile.
“Risk Factors For Developing Neurofibroma”
Neurofibroma Clinical Features
- Mild pain or painless swellings seen in subcutaneous and cutaneous plane with tingling, numbness and paresthesia.
- Most commonly affected sites are trunk, face and extremities
- Sessile or pedunculated elevated small nodules of various sizes
- Majority ofpatients have asymmetric areas ofpigmentation known as Café-au-lait spots. They are smooth-edge dark brown macules.
- Lisch nodules are present which are translucent brown pigmentation on iris.
- Crowe’s sign is present, i.e. axillary freckling, brown spot on skin.
“Symptoms Of Neurofibroma”
Neurofibroma Complications
- Cystic degeneration is present.
- Spinal and cranial neurofiromas can cause neurological defiits.
- Spine dumb-bell tumor lead to compression of spinal cord and paralysis of limb.
- Hemorrhage in tissues.
Neurofibroma Treatment
Excision is done.
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