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Sturge-Weber Syndrome Clinical Presentation

Timothy Lukavsky D.D.S. Assistant Director, Special Care Dentistry Advanced Care Clinic Arizona School of Dentistry & Oral Health A.T . Still University, Mesa, AZ. Sturge-Weber Syndrome Clinical Presentation. Sturge-Weber Syndrome. What is it? Encephalotrigeminal Angiomatosis

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Sturge-Weber Syndrome Clinical Presentation

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  1. Timothy Lukavsky D.D.S. Assistant Director, Special Care Dentistry Advanced Care Clinic Arizona School of Dentistry & Oral Health A.T. Still University, Mesa, AZ Sturge-Weber SyndromeClinical Presentation

  2. Sturge-Weber Syndrome What is it? Encephalotrigeminal Angiomatosis • Neurological Skin Disorder • Non-familial congenital disorder of unknown cause

  3. Sturge-Weber Syndrome Common Symptoms • Facial Birthmark (Port Wine Stain) • Glaucoma, Seizures, Ipsilateral Leptomeningeal Angioma • Eye, Endocrine, and Organ Irregularities • Developmental Disabilities • Intellectual Disabilities

  4. Sturge-Weber Syndrome Port Wine Stain/Hemangioma Cause • Overabundance of capillaries surrounding the trigeminal nerve Clinical Relevance • Bleeding as result of injury or rupture of capillary bed

  5. Sturge-Weber Syndrome Neurological Symptoms Common Cause • Malformation of blood vessels in the pia mater of brain • Meningeal Concretions Results in • Seizures (Worsens with age) • Muscle weakness (Opposite Side)

  6. ASDOH Sturge-Weber Case Report

  7. ASDOH Sturge-Weber Case Report 64 Year old Female Ht: 5'1" Wt. 167 lbs.. BP: 125/75 HR 60 Medical History • Sturge-Weber syndrome (encephelotrigeminal angiomatosis) • Tuberous Sclerosis (TSC): • Multiple Sclerosis • Multiple Angiomas on Brain • Seizure disorder-tonic clonic(most recent seizure was 8/2/13) • Renal insufficiency Allergies • Sulfa, Dilantin, TB skin test (annual chest x-ray in lieu of skin test)

  8. ASDOH Sturge-Weber Case Report 64 Year old Female Medications • Phenobarbitol: 1/2gr (30mg) 2 in the morning and 1 at night • Divalproex SOD DR (Depakote) 500mg 2 tablets a day • Divalproex SOD DR (Depakote) 250mg 2xday • Levetiracetam (Keppra) 1000 mg 2 in the morning 1/2 at night • Levothyroxine 75mcg 1x/day • Potassium chloride 20 meq 1x/day • Triamterene/HCZT 37.5/25mg 1xday (HTN/water retention in the body) • Furosemide tab 40 mg 2 a day (for water retention) • Aspirin 81 mg 1x/day (for heart)

  9. ASDOH Sturge-Weber Case Report First Visit April 14, 2008 (59 Year old Female) S: Pt. presents for new patient exam. Ht: 5'1" Wt. 167 lbs. BP: 125/75 HR 60 Developmental Dx: • Pt. has hx of Sturge-Weber syndrome (encephelotrigeminal angiomatosis): Congenital disorder characterized by facial birthmark and neurological abnormalities. Caregiver reports pt. has been diagnosed with multiple angiomas on her brain but is un-aware of how many and where. Pt. also has dx of tuberous sclerosis (TSC): genetic disorder causing benign tumors to grow in the brain and other vital organs. Pt. has seizure disorder and moderate ID as a result of multiple brain angiomas and tumors associated with TSC. By caregiver's description, pt. has generalized tonic-clonic seizures 2-4 times per day that last in duration from 15 to 90 seconds. Caregiver reports pt usually has aura of a blank stare prior to having seizure.

  10. ASDOH Sturge-Weber Case Report First Visit April 14, 2008 (59 Year old Female) S: Pt. presents for new patient exam. Ht: 5'1" Wt. 167 lbs. BP: 125/75 HR 60 Medication • Phenobarbital: 129.6 mg/day: anticonvulsant that alters sensory cortex, cerebellar, and motor activities. • Depakote: 1500mg/day: Anticonvulsant: exact mechanism of action unknown. • Keppra: 4000mg/day: Anticonvulsant: exact mechanism of action unknown. • Levothyroxine: 25 mcg/day: Synthetic T4; increases metabolism • Potassium Chloride: 10 MEQ/day major intracellular cation participating in multiple physiologic processes incl. nerve impulse conduction, cardiac, skeletal, and smooth muscle contraction, and normal renal fxn maintenance Known Allergies • Sulfa, Dilantin, TB skin test (Pt. has annual chest x-ray in lieu of skin test.

  11. ASDOH Sturge-Weber Case Report First Visit April 14, 2008 (59 Year old Female) T: P: Perform exam and any necessary radiographs A: No alternatives, exam necessary to establish pt.'s baseline needs. R: Little risk for today. General consent form for routine received from legal guardian. Q: Caregiver has no questions at this time.

  12. ASDOH Sturge-Weber Case Report First Visit April 14, 2008 (59 Year old Female) O: Performed IOE/EOE, and OCS. OCS negative. Caregiver reports pt. has smoked 1 pack of cigarettes per day for 35 years. Pt. has generalized occlusal wear on all occlusal surfaces. Apparent caries charted. Pt. has heavy calculus build up and FMD will be required to identify all carious lesions. PANO and posterior PAs taken. All posterior teeth appear to have class II mobility or greater. All anterior teeth have class I mobility. Charting of conditions will be completed at time of FMD. • 8 PA @ 63kv, 8ma, .08sec • Pano 70kv, 8ma, 16sec

  13. ASDOH Sturge-Weber Case Report First Visit April 14, 2008 (59 Year old Female) P: Instructed caregiver to call dental clinic should any apparent emergencies arise before next visit. Pt. will return for FMD and completion of charting. NV: 05/14/08 Behavior: Pt. is non-verbal. Pt. can nod yes or no and may speak on occasion but cannot hold a conversation. Pt. cooperative so far.

  14. ASDOH Sturge-Weber Case Report Second Visit June 19, 2008 (59 Year old Female) S: Pt. present in pain to upper left and lower left. T: PARQ also discovered #17,15,16 Dx abscessed all with buccal draining fistula, therefore due to accute infection phoned public fiduciary at 1:45 PM by consent was given for Ext.s O:BP 104/70 HR: 68 Respiration 19, 2 carps 2% Lidocaine 1:100,000 epi local infiltration left PSA, left IA, and LB, aspiration negative, luxated and delivered #17, 15 and 16, hemostasis obtained, poi given orally and written, Post-Op BP 110/78. P: Comp exam and perio evaluation and FMX

  15. ASDOH Sturge-Weber Case Report Third Visit September 13, 2012 (63 Year old Female) S: 63 y/o female presents to SNCU for comprehensive exam. T: P: Perform exam and any necessary radiographs A: No alternatives, exam necessary to establish pt's baseline needs. R: Little risk for today. General consent form for routine received from legal guardian Q: Caregiver has no questions at this time.

  16. ASDOH Sturge-Weber Case Report Third Visit September 13, 2012 (63 Year old Female) PARQ O: BP: 98/68 HR: 75 RR: 18 A: Limited evaluation P: Limited probing depths acquired (see perio charting); Limited hard tissue evaluation was done (see hard tissue charting). Comprehensive exam could not be completed due to patient's inability to be compliant with dental treatment. Sedation was recommended and this option was discussed with caretaker. They were given paperwork to begin the process and will schedule an appointment wants funds are confirmed. NV: Sedation/comp exam/restorative treatment/extraction

  17. ASDOH Sturge-Weber Case Report Fourth Visit August 26, 2013 (64 Year old Female) S: 64 yo female presents for sedation, FMX, exam, periodontal maintenance and extraction of teeth #s 1, 2, 3, 4, 13, 14, 19, 29, 30, 31, and 32. • CC: None today • Caregiver reports she has not had food or drink in over 6 hours • Please refer to Anesthesia notes

  18. ASDOH Sturge-Weber Case Report Fourth Visit August 26, 2013 (64 Year old Female) O: Radiographic examination reveals: • Radiographic calculus • Teeth #s 1, 2, 3, 4, 29, 30, 31 and 32 have approximately 50% bone loss • Teeth #s 2, 3, 4, 13, 14, 19 30, 31, 32 have radiolucency's indicative of caries • Teeth's #s 4 and 32 have PA radiolucency's Clinical examination reveals: • Class 2 mobility on teeth #s 1, 2, 3, 4, 14, 29, 30, 31, and 32 • Decay on #s 2, 3, 4, 13, 14, 19, 30, 31, 32

  19. ASDOH Sturge-Weber Case Report Fourth Visit August 26, 2013 (64 Year old Female) A: Dx: Chronic Adult Severe Periodontitis, Caries (1, 2, 3, 4, 13, 4, 19, 30, 31, 32) Non restorable teeth (#s 2, 3, 13, 14, 19, 30, 31, 32) Periapical pathology (#s 4 and 32), P: Informed consent was obtained from the pt/ caregiver. After pt was consciously sedated a throat guard was placed. FMX was obtained and clinical examination was done. 4 quads scaling and root planning was completed with cavitron. 6 carps (216 mg) of 2% lidocaine 1:100,000 epi was administered over a span from 8 am to 2 pm. Simple extractions were accomplished on teeth #s 1, 2, 3, 4, 13, 19, 29, 30, 31, and 32 via the following methods: #9 periosteal elevator was used to loosen the marginal gingiva, elevators and forceps were used to deliver all of the teeth simply, all sockets were curetted and irrigated with CHX. 3.0 chromic gut sutures were placed in all extraction sites and pressure gauze was placed.

  20. ASDOH Sturge-Weber Case Report Discussion • Hemorrhage caused by Sturge-Weber Hemangioma • Dental practitioners and oral surgeons need to be aware of these lesions because they may pose serious bleeding risks.

  21. ASDOH Sturge-Weber Case Report Conclusion • Favorable Outcome Achieved Recommendations for Managing Hemorrhage • Patient is blood typed and cross matched • Provision for blood transfusion • Use of hemostatic agents–topical bovine thrombin • Use of postoperative splints • Injecting sclerosing solutions • Percutaneous transcatheter vascular embolization using gelfoam or polyvinyl alcohol. • Electrosurgery

  22. References Clinical Notes. 2008-2013. Arizona School of Dental & Oral Health. Natarajan Manivannan, Subramanium Gokulanathan, Ramakrishnan Swamy Ahathya,1 Gubernath,2 Rajkumar Daniel 1 and Shanmugasundaram1. Sturge-Weber Syndrome. J Pharm Bioallied Sci. Aug 2012; 4(Suppl 2): S349–S352. National institute of neurological disorders and stroke. (October, 2011 04). Retrieved http://www.ninds.nih.gov/disorders/sturge_weber/sturge_weber.htm Oral Panograph/FMX. Apr. 14, 2008. Arizona School of Dental and Oral Health. Yamashiro Mikiko, Furuya Hideki. Dec. 20, 2005. Anesthetic Management of a Patient with Sturge-Weber Syndrome Undergoing Oral Surgery. The American Dental Society of Anesthesiology 53:17-19 2006.

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