SPINA BIFIDA Presentation and Management of Patients at Texas Scottish Rite Hospital for Children

1. SPINA BIFIDA Presentation and Management of Patients at Texas Scottish Rite Hospital for Children Dallas, TX Susan McGuire, DDS April 19, 2013

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

3. SPINA BIFIDA • Overview of Condition • Texas Scottish Rite Hospital for Children • Medical Management • Dental Management

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

5. Formation of Neural Tube Overview • 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.

6. Etiologies Overview • 95% do not have any family history • Maternal • Age • Obesity • Diabetes • Seizure medications • Folic acid deficiencies • 0.4 mg/day

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

8. 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

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

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

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

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

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

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

15. Treatment of Spina Bifida Medical Management Surgery to close defect within 24 to 48 hours of birth

16. Treatment of Spina Bifida Medical Management • 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

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

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

19. 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

20. Arnold-Chiari II Malformation Normal Malformation Medical Management

21. 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

22. Hydrocephalus • 80-90% develop hydrocephalus • Abnormal accumulation of CSF • Increased intracranial pressure • Progressive enlargement of the head • Convulsion • Mental disability Medical Management

23. Shunts Hydrocephalus Medical Management • 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

24. Seizure Disorders Hydrocephalus • 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

25. 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

26. Hydrocephalus Learning Disabilities • Dysfunctions include: • Short attention span • Decreased arm and hand function • Poor eye-hand coordination • Memory deficits Medical Management

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

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

29. Obesity • 40% of patients • Due to • Impaired mobility • Decreased energy expenditure • High carbohydrate food “rewards” Medical Management

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

31. Latex Allergy/Risk • 70% exhibit symptoms • Predisposition to latex allergy unknown • Increased need for health care shunts other allergies. Medical Management

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

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

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

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

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

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

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

39. Oral Complications • Gingivitis • Increased plaque • Gingival hyperplasia • Anticonvulsant medications • Periodontal disease • Increased calculus Dental Management

40. 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

41. Anticipatory Guidance Dental Management • 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

42. Recall Schedule Dental Management 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

43. 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

44. Oral Hygiene Tips • Horizontal scrub toothbrushing technique recommended. • Electric toothbrush • Customized toothbrush handles • Toothettes • Use of floss holders assists with flossing Dental Management

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

46. Behavior Management • Parental Anxiety • Talk to the parent and find out their expectations • Tour the office • Introduce staff Dental Management

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

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

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

50. 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