The Oral Health and Anesthetic Management of Patients with Sturge
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Transcript The Oral Health and Anesthetic Management of Patients with Sturge
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
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)
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)
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.
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.
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.
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
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.
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
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
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.
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.
2.
3.
4.
5.
6.
7.
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
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. SturgeWeber 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.