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NEUROFIBROMATOSIS TYPE I /prosthodontic courses

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NEUROFIBROMATOSIS TYPE I /prosthodontic courses

The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.

The Indian Dental Academy is the Leader in continuing dental education , training dentists in all aspects of dentistry and
offering a wide range of dental certified courses in different formats.

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NEUROFIBROMATOSIS TYPE I /prosthodontic courses

  1. 1. NEUROFIBROMATOSIS TYPE I– A CASE REPORT • INDIAN DENTAL ACADEMY • Leader in continuing Dental Education www.indiandentalacademy. com
  2. 2. INTRODUCTION  Neurofibromatosis is a group of genetic disorders that primarily affect the cell growth of neural tissues.  NF-I is estimated to occur in one of every 3000 births.  Autosomal dominant disease Franz M. Enzinger : Soft Tissue Tumors 3rd Etd. 851-863 pg. www.indiandentalacademy. com
  3. 3. 22 yr old male patient c/o swelling on the left middle one third of the face since birth. CASE REPORT www.indiandentalacademy. com
  4. 4. HOPI:  Swelling in the left middle one third of face & nose was noticed since birth and has gradually increased to the present size.  Associated with intermittent pricking type of pain, aggravated by touching the swelling & regress within 1 ½ hrs.  Associated with intermittent burning sensation in the left eye since birth when exposed to the sunlight.  No H/O paresthesia, difficulty in breathing.  H/O of similar kind of nodular growths present all over the body since birth which were asymptomatic & no increase in size of nodular growths were reported. www.indiandentalacademy. com
  5. 5. MEDICAL HISTORY: Patient had visited general physician 15 yrs back DRUG HISTORY: No H/O any drug allergies DENTAL HISTORY: First visit to dentist FAMILY HISTORY: No members in family with similar findings www.indiandentalacademy. com
  6. 6.  On inspection,  Site - chest, back & both the arms  Size - 0.5-1.5cms  Number - multiple nodular growths  Shape - well defined, round  Borders - smooth  Surface changes – pigmented  On palpation,  soft  compressible  non- tender  Surface skin was pinchable GENERAL EXAMINATION www.indiandentalacademy. com
  7. 7.  Site - chest, back & axillae  Size - 1x1cms  Number - multiple  Shape - irregularly shaped  Borders - smooth  Surface changes - brown colored macules resembling CAFÉ- AU- LAIT spots  Examination of the axillae bilaterally revealed Freckling. www.indiandentalacademy. com
  8. 8.  Gross facial asymmetry on middle one third of left side of the face was noticed.  On examination the swelling was diffuse and measuring approx 3x3cms in dimensions.  Extensions:  Superiorly, base of the nose  Inferiorly, 1cm below line joining tragus and ala of nose  Laterally, vertical line drawn from the pupil of eye  Medially, crossing the midline of the nose  No other surface changes noticed HEAD AND NECK EXAMINATION www.indiandentalacademy. com
  9. 9.  Another nodule measuring about 1x 0.5cm in size noted distal to the enlarged left ala of nose with same features but was tender on palpation.  Another round nodule noticed 1cm away corner of the mouth  The malar region appeared to be enlarged & hair growth was noticed. • Bridge of the nose was widened • Nasal tip - deviated • Left nostril was enlarged • Lip on left side appeared to be pulled down. www.indiandentalacademy. com
  10. 10. ۩ EYE: A round, diffuse, poorly defined nodule was noted below the infraorbital margin, measuring 1x1cm in size. Vision & eye movements were normal. ۩ SCALP, EARS, TMJ: No clinically detectable abnormality ۩ LYMPH NODES: Non- palpable www.indiandentalacademy. com
  11. 11. INTRA ORAL EXAMINATION On hard tissue examination, Teeth present: 87654321 12345678 87654321 12345678 Decayed teeth: 15, 16www.indiandentalacademy. com
  12. 12. PROVISIONAL DIAGNOSISPROVISIONAL DIAGNOSIS NEUROFIBROMATOSIS TYPE I www.indiandentalacademy. com
  13. 13. DIFFERENTIAL DIAGNOSIS • Multiple endocrine neoplasia type 2B (MEN) syndrome • Klippel Trenaunay Weber Syndrome www.indiandentalacademy. com
  14. 14. REFERRALS & INVESTIGATIONS: • Opthalmologic consultation INVESTIGATIONS: • Radiographs • Routine blood Investigations • Ultrasound • Cranial Angiography www.indiandentalacademy. com
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  19. 19. • Immediate treatment: Debulking procedure was done for the lesion over the left nose. www.indiandentalacademy. com
  20. 20. HISTOLOGICAL FEATURES www.indiandentalacademy. com
  21. 21. CONFIRMED CLINICAL DIAGNOSIS NEUROFIBROMATOSIS TYPE 1 www.indiandentalacademy. com
  22. 22. DISCUSSION TYPES OF NEUROFIBROMATOSIS • NF I Von Recklinghausen type • NF II Acoustic type • NF III Mixed type • NF IV Variant type • NF V Segmental type • NF VI Café au- lait type • NF VII Late onset type • NF VIII Gastrointestinal type • NF IX Neurofibromatosis/ Noonan type Gorlin J.R : Syndromes of the Head and Neck, 3rd Etd.www.indiandentalacademy. com
  23. 23. ☻ Schwann cells have angiogenic potential. Immunohistochemical staining & reverse transcribed polymerase chain reaction (RT-PCR) vascular endothelial growth factor & basic fibroblast growth factor angiogenesis HYPERVASCULARITY OF NEUROFIBROMA. Sharma Sheela, Vincent M. Riccard and Nancy Ratner; Angiogenic and Invasive Properties of Neurofibroma Schwann Cells; The Journal of Cell Biology. Volume 111, 1990 www.indiandentalacademy. com
  24. 24.  Six or more café-au-lait macules over 5 mm in greatest diameter in prepubertal persons and over 15 mm in greatest diameter in post-pubertal persons.  Two or more neurofibromas of any type or one plexiform neurofibroma.  Freckling in the axillary or inguinal regions (Crowe's sign)  Optic glioma.  Two or more Lisch nodules (Iris hamartomas).  A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudoarthrosis.  A first-degree relative (parent, sibling or offspring) with neurofibromatosis type-I, based on the above criteria. Proposed by the National Institutes of Health Consensus Development Conference in 1988. DIAGNOSTIC CRITERIA www.indiandentalacademy. com
  25. 25. ORAL MANIFESTATIONS ♠ Enlargement of fungiform papillae - 50% affected patient ♠ Macroglossia. ♠ Gum involvement in NF-I - 5%. www.indiandentalacademy. com
  26. 26. RADIOGRAPHIC FEATURES Oral radiographic findings may include  Enlargement of the mandibular foramen & branching of the mandibular canal  Increased bone density  Concavity of the medial surface of the ramus  Increased dimension of the coronoid notch Other findings:  Macrocranium  Macroencephaly  Cervical kyphosis www.indiandentalacademy. com
  27. 27.  MALIGNANT TRANSFORMATION: 3 to 5 % of neurofibromatosis type 1 (NF1) patients may develop malignant peripheral nerve sheath tumors.  TREATMENT:  The treatment is often directed towards prevention or management of complications.  Facial neurofibromas can be removed for cosmetic or functional purpose.  Long term follow up of such cases is mandatory.  PROGNOSIS: variable depending upon the clinical presentation - Fair Justus-Martijn Brinkman et al :The correlation between clinical, nuclear and histologic findings in a patient with Von Recklinghausen's disease. World Journal of Surgical Oncology 2007, 5:130. www.indiandentalacademy. com
  28. 28. CONCLUSION: • Most of the cases of neurofibromatosis are diagnosed but left untreated. • Our case demonstrated an uncommon presentation of hypervascular neurofibromatosis in which the neurofibroma had been excised for esthetic purpose. www.indiandentalacademy. com
  29. 29. THANK YOU www.indiandentalacademy. com

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