SPINA BIFIDA Presentation and Management of Patients at Texas Scottish Rite Hospital for Children Dallas, TX Susan McGuire, DDS April 19, 2013 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 SPINA BIFIDA • Overview of Condition • Texas Scottish Rite Hospital for Children – Medical Management – Dental Management 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 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 Etiologies • 95% do not have any family history • Maternal – Age – Obesity – Diabetes – Seizure medications • Folic acid deficiencies – 0.4 mg/day Overview Diagnosis in utero • Measurement of maternal serum alphafetoprotein (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 Neural Tube Defects • Affect the brain’s development and leave the spinal cord vulnerable to damage • • Paralyze or weaken associated muscles and affect organs • Two Types – Closed – Open Overview 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 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 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 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 Meningocele • • • • • Meninges protrude from the spinal opening May or may not be symptomatic Noticeable bulge on back Repaired surgically Usually no resultant paralysis Overview 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 Treatment of Spina Bifida • Surgery to close defect within 24 to 48 hours of birth Medical Management 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 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 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 Arnold-Chiari II Malformation • Downward displacement of the cerebellum, cerebral tonsils, brainstem, and 4th 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 Normal Malformation Medical Management Arnold-Chiari II Malformation • 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 Medical Management Hydrocephalus • 80-90% develop hydrocephalus • Abnormal accumulation of CSF • Increased intracranial pressure – Progressive enlargement of the head – Convulsion – Mental disability Medical Management Shunts Hydrocephalus • 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 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 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 Learning Disabilities • Dysfunctions include: – Short attention span – Decreased arm and hand function – Poor eye-hand coordination – Memory deficits Medical Management 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 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 Obesity • 40% of patients • Due to – Impaired mobility – Decreased energy expenditure – High carbohydrate food “rewards” Medical Management 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 Latex Allergy/Risk • 70% exhibit symptoms • Predisposition to latex allergy unknown – Increased need for health care shunts other allergies. Medical Management Dental Management As dentists, how do we manage all of these medical complications in providing dental care to the spina bifida patient? Dental Management 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 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 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 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 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 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 Oral Complications • Gingivitis – Increased plaque • Gingival hyperplasia – Anticonvulsant medications • Periodontal disease – Increased calculus Dental Management Treating patients • Wheelchair bound patients – Can be left in wheelchair • Sliding board can help with supporting the patient’s head • Wheelchair transfer to dental chair • 2-person lift – Under arms – Legs Dental Management 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 Recall Schedule • Individualized for patient’s needs • Close observation of patients and regular dental exams are important • 2, 3 or 4 month recall schedule can be beneficial Dental Management Home Care •Common Positions – Child positioned in front of adult. Adult cradles the child’s 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 Oral Hygiene Tips • Horizontal scrub toothbrushing technique recommended. – Electric toothbrush – Customized toothbrush handles – Toothettes • Use of floss holders assists with flossing Dental Management 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 Behavior Management • Parental Anxiety – Talk to the parent and find out their expectations – Tour the office – Introduce staff Dental Management 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 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 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 Sedation • All medication doses determined by weight – Obese patients, use ideal body weight/height • Calculation: Height cm2 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 General Anesthesia • 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 • Complete physical work-up for each patient prior to general anesthesia • Must follow NPO guidelines • Benefits must outweigh risks! Dental Management General Anesthesia • More aggressive treatment – Ext vs. Endo therapy – SSCs vs. Class IIs – Amalgam vs. composite – Limit occlusal anatomy • Plaque accumulation – Sealants Dental Management Summary • Know your patient’s medical history – Surgeries – Medications – Allergies • Obtain necessary consults – Need for ABX • Be aware of patient’s anxiety level – Consider sedation/GA • Emphasize prevention. • MOST OF ALL, be patient, understanding, and compassionate. Thank Questions you References • • • • • • • • • • • • • • ADAM Images. Available at http://adameducation.com/adam_images.aspx. Accessed April 3, 2013. Batshaw ML ed., Children with Disabilities, 4th ed., Paul H. Brookes Publishing Co., Baltimore, MD, 2nd 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. Ekmark EM. Risky Business: Preventing skin Breakdown in Children with Spina Bifida. 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