timothy lukavsky d.d.s. assistant director, special care dentistry advanced care clinic arizona...

Sturge-Weber SyndromeClinical Presentation

Timothy Lukavsky D.D.S.Assistant Director, Special Care DentistryAdvanced Care ClinicArizona School of Dentistry & Oral HealthA.T. Still University, Mesa, AZ

Sturge-Weber Syndrome

What is it? Encephalotrigeminal Angiomatosis Neurological Skin Disorder Non-familial congenital disorder of unknown

cause


Common Symptoms

Facial Birthmark (Port Wine Stain) Glaucoma, Seizures, Ipsilateral

Leptomeningeal Angioma Eye, Endocrine, and Organ Irregularities Developmental Disabilities Intellectual Disabilities


Port Wine Stain/Hemangioma

Cause Overabundance of capillaries surrounding the

trigeminal nerve

Clinical Relevance Bleeding as result of injury or rupture of

capillary bed


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)

ASDOH Sturge-Weber Case Report


64 Year old FemaleHt: 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)


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)


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.



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.



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.



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



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.


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


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.


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


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



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



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.


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.


Conclusion

Favorable Outcome Achieved

Recommendations for Managing Hemorrhage

1. Patient is blood typed and cross matched2. Provision for blood transfusion3. Use of hemostatic agents–topical bovine thrombin4. Use of postoperative splints5. Injecting sclerosing solutions6. Percutaneous transcatheter vascular embolization using gelfoam or

polyvinyl alcohol.7. Electrosurgery

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.

timothy lukavsky d.d.s. assistant director, special care dentistry advanced care clinic arizona...

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