NEUROFIBROMATOSIS PPT
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DR. DIYAR A. SALIHPLASTIC SURGERY RESIDENT
KURDISTAN – SLEMANI2014
Neurofibromatosis
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Neurofibromatosis • Multiple NF• Café-au-lait spots• Other findings
Neurofibroma • Nerve sheath tumor
Definitions
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• Autosomal dominant disorder1. NF-1 : chromosome 172. NF-2 : chromosome 22
Etiopathogenesis
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• Caf’e-au-lait spots (≥6)1. >5 mm prepubertal2. >15 mm postpubertal
• Neurofibroma (≥2 any type or 1 PFN)
• Eyes: 1. Lisch nodules (≥2) - iris hamartomas 2. Optic glioma
• Freckling – Axilla or Inguinal area
• Bones: distinctive osseous lesions;1. Sphenoid wing dysplasia2. Long bone cortex thinning +/- pseudoarthrosis
• First degree relative: with NF-1 with above criteria
Diagnostic criteriaNF-1 (Two or more):
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• Bilateral 8th CN mass
• A first-degree relative with NF-2 and either:1. Unilateral eighth nerve mass, or
2. Two or more of the following:• Neurofibroma• Meningioma• Glioma• Schwannoma• Juvenile posterior subcapsular lenticular opacity
NF-2
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• Variation
• Age: 1. Begins in childhood, 2. Progress to adulthood.
• Sites1. Bones2. Spintal root3. Peripheral nerves4. Pheochromocytoma 10%5. Penile shaft (enlargement)6. Digits (enlargement)
• Plastic surgeon NF-1
Clinical features
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Caf’e-au-lait spots
1. Hyperpigmented areas
2. Differentiated from congenital nevi (by Punch biopsy)
3. Birth- up to 1 yr
4. ↑ Number & size
5. 20-30 mm
NF-1
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1. The clinical hallmark
2. Multiple cutaneous & subcutaneous nodular tumors.
Nodular tumor
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• Before 5 yr
• 80%
Axillary freckling
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• Slit-lamp exam
• Pigmented, dome-shaped nodules
• Onset by 10 yr
• All 20 yr
Lisch nodules
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1. Optic glioma 15%
2. Brainstem glioma
3. Benign or malignant astrocytomas
4. Meningiomas
5. Medulloblastomas
6. Malignant schwannomas
Intracranial tumors
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1. Sphenoid wing aplasia/dysplasia
• 5-7%
• Communication
• Proptosis
• Pulsatile exophthalmos
1. Macrocephaly
2. Scoliosis
3. Anterior tibial bowing
Skeletal abnormalities
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a. Craniofacial
1. Sphenoid wing dysplasia
2. 1-10%
3. Unknown etiology
Neurofibroma
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b. Plexiform
1. Eyes • Ptosis• Thickening• Visual obstruction• Glaucoma • Ectropion• Epiphora
2. Cheek• Grossly involved / Ptosis
3. Nose• Hypertrophy• Distortion of soft tissue &
cartilages
4. Teeth• Maxillary & mandibular
plane distortion
5. Speech problems• Mandibular division of
TGN
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• Incidence: 13% in NF-1
• Origin: Neurosarcoma – Malignant Schwannomas
• Risk factors:1. NF-1 / 50%2. Only plexiform 3. Medium & large nerves
• Features: pain (most reliable indicator)
• Treatment: medical attention, biopsy, excision
• Prognosis: metastasis
Malignant degeneration
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• Bleeding1. Packing
2. Returning in 48 hr
3. Monitoring
4. i.v. access
5. Hypotensive technique
• Recurrence 1. Decreased tensile strength
Treatment – Planning
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A. Upper eyelid approach
1. Levator aponeurosis shortening
2. Temporal lobe repositioning
3. Sphenoid bone graft
4. Eyelid skin invagination
5. Orbital prosthesis
Cranio-orbital NFa. Skeletal reconstruction
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B. Coronal approach
1. Forehead lift
2. Supraorbital bar repositioning
3. Dural separation
4. Ophthalmic nerve & vessels visualization
5. Middle cranial fossa-orbit separation with bone graft
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1. Medial & lateral canthal management
2. Ptosis correction
b. Soft tissue reconstruction
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Indications for treatment
1. Pain
2. Considerable enlargement
3. Mandible and maxilla deformity
Facial plexiform NF
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Forehead
Forehead lift
1. Coronal approach
2. Hairline frontal approach
Facial plexiform NF
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Cheek
1. Skeletal reconstruction• Performed first• Occlusal plane leveling• Osseous structure reduction• Orthognathic surgery
2. Soft tissue reconstruction:• Facelift: Permanent suture
anchoring to bony skeleton or• Tensor fascia lata sling• Direct full-thickness excision of
redundant tissue.
Facial plexiform NF
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Nose & Lips
1. Skeletal reconstruction first
2. Soft tissue redundancy:• Direct excision (both
horizontal & vertical)
Facial plexiform NF
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Thank you