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SPINA BIFIDA Presentation and Management of Patients at Texas Scottish Rite Hospital for Children Dallas, TX Susan McGuire, DDS April 19, 2013 Slide 2 Assistant Professor, Department of Pediatric Dentistry, Texas A&M University Baylor College of Dentistry, Dallas, TX Assistant Director, Special Needs Dental Clinic, Texas Scottish Rite Hospital for Children, Dallas, TX Slide 3 SPINA BIFIDA Overview of Condition Texas Scottish Rite Hospital for Children Medical Management Dental Management Slide 4 Spina Bifida One of most common congenital birth defects 1-2 per 1000 births worldwide 1500-2000 births in USA annually Prevalence Hispanics>Caucasians>African Americans Girls>Boys Overview Slide 5 Formation of Neural Tube During the first 3-4 weeks, specific cells form a narrow tube that becomes the foundation of the spinal cord, brain bone and tissues. At 28 days of gestation, neural tube closed. If the tube does not close properly anywhere along its entire length, then a neural tube defect will form at the open location. Overview Slide 6 Etiologies 95% do not have any family history Maternal Age Obesity Diabetes Seizure medications Folic acid deficiencies 0.4 mg/day Overview Slide 7 Diagnosis in utero Measurement of maternal serum alpha- fetoprotein (MSAFP) If elevated at 16-18 weeks of gestation can indicate defects Ultrasound examination 18 weeks Amniocentesis Use amniotic fluid alpha fetoprotein (AFAFP) and acetylcholinesterase (AFAChE) to confirm Overview Slide 8 Neural Tube Defects Affect the brains development and leave the spinal cord vulnerable to damage Paralyze or weaken associated muscles and affect organs Two Types Closed Open Overview Slide 9 Closed Neural Tube Defects Localized and confined to the spine; brain rarely affected Neural tissue not exposed; spinal defect fully covered by skin May remain undetected for years, especially in absence of cutaneous markers Types: Lipomyelomeningocele Lipomeningocele Tethered cord Overview Slide 10 Open Neural Tube Defects Involve the entire CNS Neural tissue exposed with associated CSF leakage Skin covering not intact Visible at birth; majority discovered during pregnancy Overview Slide 11 Spina Bifida Characterized by the incomplete development of the brain, spinal cord and/or meninges Nerves located below malformation are affected, resulting in loss of sensation and paralysis. Complications may be minor physical problems to severe physical and mental disabilities. Types: Anencephaly Occulta Meningocele Myelomeningocele Overview Slide 12 Spina Bifida Occulta Mildest and most common form One or more vertebrae are malformed Opening in spine is covered by a layer of skin. Rarely causes disabilities or symptoms Often undetected unless visible exterior sign is present Tuft of hair, dimple, birthmark, bulge at site of malformation Overview Slide 13 Meningocele Meninges protrude from the spinal opening May or may not be symptomatic Noticeable bulge on back Repaired surgically Usually no resultant paralysis Overview Slide 14 Myelomeningocele Most severe and rarest form Occurs when the spinal cord is exposed through the opening in the spine Results in partial or complete paralysis of the parts of the body below the spinal opening Overview Slide 15 Treatment of Spina Bifida Surgery to close defect within 24 to 48 hours of birth Medical Management Slide 16 Treatment of Spina Bifida Before surgery, the child must handled carefully to reduce further damage to exposed spinal cord Lifelong treatment for other organ system disturbances secondary to damage to spinal cord or spinal nerves Medical Management Slide 17 Team Approach to Management at Texas Scottish Rite Hospital (TSR ) Neurosurgeon Developmental Pediatrician Neurologist Urologist Orthopedic Surgeon Dentist Other services involved: Psychology, Child Life, Orthotics, PT, OT, Dietary, Family Services Medical Management Slide 18 Associated Impairments Arnold-Chiari II malformation Hydrocephalus Seizure disorders Bladder and bowel incontinence Musculoskeletal Deformities Learning disabilities Altered wound healing Latex allergy/risk Medical Management Slide 19 Downward displacement of the cerebellum, cerebral tonsils, brainstem, and 4 th ventricle through the foramen magnum and into cervical space Results in compression and elongation of nerves and tissue responsible for vital functions including respirations and protective reflexes Medical Management Arnold-Chiari II Malformation Slide 20 Medical Management NormalMalformation Arnold-Chiari II Malformation Slide 21 Symptomatic in about 33% of affected persons Difficulty swallowing Inspiratory stridor Stiffness or spasticity of arms or hands Poor balance and coordination Leading cause of death in spina bifida population Arnold-Chiari II Malformation Medical Management Slide 22 Hydrocephalus 80-90% develop hydrocephalus Abnormal accumulation of CSF Increased intracranial pressure Progressive enlargement of the head Convulsion Mental disability Medical Management Slide 23 Shunts Placed to relieve intracranial pressure Types: Ventriculoperitoneal (VP) Ventriculoatrial (VA) Ventriculopleural Ventriculo-gall bladder VP shunts empty CSF directly into the abdominal cavity, bypassing the venous circulation. Hypersensitivity to loud noises Medical Management Hydrocephalus Slide 24 Seizure Disorders Occur in approx 15% of patients Usually generalized tonic-clonic type Responds well to anticonvulsant medication Recurrent seizure activity may indicate shunt blockage or infection. Medical Management Hydrocephalus Slide 25 Learning Disabilities At least 80% have normal intelligence Cognitive dysfunctions correlated to: Hydrocephalus and related complications Level of lesion Upper level lesions associated with mental retardation Medical Management Hydrocephalus Slide 26 Learning Disabilities Dysfunctions include: Short attention span Decreased arm and hand function Poor eye-hand coordination Memory deficits Medical Management Hydrocephalus Slide 27 Bladder and Bowel Incontinence Urinary complications -Hydronephrosis, incomplete emptying of the bladder, urinary reflux, infections and incontinence -May lead to renal damage -Patients frequently use catheters and antibiotics. Altered GI motor and sensory function Impairs peristalsis Leads to constipation, impaction and bowel incontinence Patients frequently require added fiber to diet. Medical Management Slide 28 Musculoskeletal Deformities Paralysis may lead to deformities 90% of patients with defect above sacral level develop: Scoliosis Kyphosis Surgery often required to correct spinal curvatures. Loss of muscle strength and inactivity may lead to pathological fractures. Medical Management Slide 29 Obesity 40% of patients Due to Impaired mobility Decreased energy expenditure High carbohydrate food rewards Medical Management Slide 30 Altered Wound Healing Altered skin integrity over spine and cord Altered sensory function below the level of the lesion results in risk of skin breakdown throughout life Common risk factors Reduced mobility Nutritional status Bowel and urinary incontinence Medical Management Slide 31 Latex Allergy/Risk 70% exhibit symptoms Predisposition to latex allergy unknown Increased need for health care shunts other allergies. Medical Management Slide 32 Dental Management As dentists, how do we manage all of these medical complications in providing dental care to the spina bifida patient? Dental Management Slide 33 Obtain Medical History Medical conditions Allergies Latex, Drugs Past surgical history Shunt Spinal instrumentation Medications Antibiotics, anticholinergics, sympathomimetics, anticonvulsants and stool softeners Family history Appropriate consults Dental Management Slide 34 Dental Implications of Spina Bifida Associated Impairments Latex Allergy/Risk Requires latex-free environment Medications Anticonvulsants Make sure patient has taken Rx to minimize risk of seizure Antibiotics May need to switch ABX if required for shunt (VA) or spinal instrumentation Dental Management Slide 35 Dental Implications of Spina Bifida Associated Impairments Shunt Avoid putting pressure on shunt while treating patient May exhibit sensitivity to loud noises Seizures Make sure anticonvulsant medication taken Nausea, drowsiness Gingival hyperplasia Anxiolysis/Sedation medications may need to be reduced Know proper protocol to manage seizure Broken teeth, tongue lacerations Dental Management Slide 36 Dental Implications of Spina Bifida Associated Impairments Bladder and Bowel Incontinence Empty prior to dental treatment Scoliosis/Kyphosis Spinal instrumentation requires ABX Paralysis Postural hypotension likely, best not to treat patient supine If chair-bound, treat in chair, tilt chair back Dental Management Slide 37 Dental Implications of Spina Bifida Associated Impairments Obesity For anxiolysis/sedation, use ideal weight for patient Feeding/Swallowing Issues Gastrostomy tube: Increased calculus formation Tracheostomy: Protect airway if patient unable to respond protectively Minimize use of water Use suction judiciously Dental Management Slide 38 Oral Complications Poor oral hygiene Involuntary movements Lack of motor skills Vomit reflex during brushing Dental caries Poor nutrition Reduced salivary flow Long-term use of medications Dental Management Slide 39 Oral Complications Gingivitis Increased plaque Gingival hyperplasia Anticonvulsant medications Periodontal disease Increased calculus Dental Management Slide 40 Treating patients Wheelchair bound patients Can be left in wheelchair Sliding board can help with supporting the patients head Wheelchair transfer to dental chair 2-person lift Under arms Legs Dental Management Slide 41 Anticipatory Guidance Chlorhexidine gluconate mouthwash for gingival/periodontal issues May use as rinse or brush on tissues Fluoride for decreasing caries incidence Apply fluoride at all prophylactic appointments Preventive Restorations Sealants effective to reduce occlusal caries Dental Management Slide 42 Recall Schedule Individualized for patients needs Close observation of patients and regular dental exams are important 2, 3 or 4 month recall schedule can be beneficial Dental Management Slide 43 Home Care Common Positions Child positioned in front of adult. Adult cradles the childs head with one hand and uses other to brush. Child in wheelchair. Adult sits behind it. Lock the wheels and tilt chair into the lap. Dental Management Slide 44 Oral Hygiene Tips Horizontal scrub toothbrushing technique recommended. Electric toothbrush Customized toothbrush handles Toothettes Use of floss holders assists with flossing Dental Management Slide 45 Behavior Management Behavior management techniques become more important in these patients! Patients and their families generally exhibit anxiety due to frequency of medical interventions. It is important to spend additional time with parent and child to establish rapport. Dental Management Slide 46 Behavior Management Parental Anxiety Talk to the parent and find out their expectations Tour the office Introduce staff Dental Management Slide 47 Behavior Management Patient Anxiety Schedule early in day Actively listen to patient Verbal/Non-verbal Speak at patient IQ level Keep appointments short Gradually progress to more complicated procedures Reward patient after successful completion of procedures Dental Management Slide 48 Delivery of Care Immobilization Effective way to diagnose and deliver dental care Helps protect patient, dentist, and staff Make sure parents have clear understanding of use Make sure you obtain proper informed consent Includes parental assistance, extra personnel, mouth props, papoose board Dental Management Slide 49 Sedation Neurologically handicapped patients may be unable to cope with dental treatment Individualized for each patient Review any changes in medical history Review allergies Review medications Confirm patients have taken regular medications the morning of procedure. Dental Management Slide 50 All medication doses determined by weight Obese patients, use ideal body weight/height Calculation: Height cm 2 X 1.65 / 1000 = Kg Consider decrease in dosage of sedative agent by 1/3 for patients taking benzodiazepines, barbiturates, valproic acid, MAO inhibitors, elective serotonin re-uptake inhibitors Must include reversal agent and doses Dental Management Sedation Slide 51 Sedation is contraindicated for ASA Type III and IV patients, or any other patient not cleared by his/her physicians. Amount of dental work needed would require more than 2 sedation appointments. Failed sedation attempt Dental Management General Anesthesia Slide 52 Complete physical work-up for each patient prior to general anesthesia Must follow NPO guidelines Benefits must outweigh risks! Dental Management Slide 53 General Anesthesia More aggressive treatment Ext vs. Endo therapy SSCs vs. Class IIs Amalgam vs. composite Limit occlusal anatomy Plaque accumulation Sealants Dental Management Slide 54 Summary Know your patients medical history Surgeries Medications Allergies Obtain necessary consults Need for ABX Be aware of patients anxiety level Consider sedation/GA Emphasize prevention. MOST OF ALL, be patient, understanding, and compassionate. Slide 55 QuestionsThank you Slide 56 References ADAM Images. Available at http://adameducation.com/adam_images.aspx. Accessed April 3, 2013.http://adameducation.com/adam_images.aspx Batshaw ML ed., Children with Disabilities, 4 th ed., Paul H. Brookes Publishing Co., Baltimore, MD, 2 nd printing, May 1998, p. 529-552. Centers for Disease Control and Prevention. Spina Bifida. Available at http://www.cdc.gov/ncbddd/spinabifida/data.html. Accessed March 25, 2013. http://www.cdc.gov/ncbddd/spinabifida/data.html Ekmark EM. Risky Business: Preventing skin Breakdown in Children with Spina Bifida. Journal Pediatric Rehabilitation Medicine. 2009; 2:37-50. Foster MR. Spina Bifida. Available at http://emedicine.medscape.com.article/311113. Accessed March 26, 2013.http://emedicine.medscape.com.article/311113 Jaccarino J. The Patient with Special Needs: General Treatment Considerations. Available at www.dentalcare.com. Accessed March 16, 2013. www.dentalcare.com Jackson PL, Vessey JA eds., Primary Care of the Child with a Chronic Condition. Mosby-Year Book, Inc., St. Louis, MO, 1992, p. 373-388. Mayo Clinic. Spina Bifida. Available at http://www.mayoclinic.com. Accessed February15, 2013.http://www.mayoclinic.com National Institute of Neurological Disorders and Stroke. Spina Bifida. Available at www.ninds.nih.gov/disorders/spina_bifida/spina_bifida.htm. Accessed March 15, 2013. www.ninds.nih.gov/disorders/spina_bifida/spina_bifida.htm Practical Oral Care for People with Developmental Disabilities. Available at www.nidcr.nih.gov.www.nidcr.nih.gov Queiroz AM, Saiani RA, Rossi CR, Gomes-Silva JM, Belson-Filho P. Oral Findings and Dental Care in a Patient with Myelomeningocele: Case Report of a 3-Year Old Child. Brazilian Dental Journal, 2009, 20(5), p. 434-438. Spina Bifida Association. Available at www.spinabifidaassociation.org/site/c.evKRI7OxIoJ8H/. Accessed February 15, 2013.www.spinabifidaassociation.org/site/c.evKRI7OxIoJ8H/ Scofield, JC, Campbell, PR. Integrating the Spina Bifida Patient into the General Dental Practice. The Journal of Practical Hygiene, May/June 2001, p. 27-31. Steifel, DJ. Dental care Considerations for Disabled Adults, Special Care Dentistry, 2002, 22(3), p. 265-395.