Mylomeningocele

ped nsg seminar

1. Meningomyelocoele Presented by- Yogesh Dengale II MSc CON PIMS LONI

2. Meningomyelocoele is the most severe type of SPINA BIFIDA. Spina Bifida means "split spine” 4 types of Spina Bifida are: Spina bifida occulta , Meningocele Spina bifida cystica ( Myelomeningocele ) and Lipomeningocele INTRODUCTION

3. Meningomyelocoele occurs due to Failure of closure of the neural tube during the third week of gestation abnormal differentiation of the embryonic neural tube In Meningomyelocoele A cystic swelling occurs over the site of the spinal defect which contains meninges , nerve roots, and the spinal cord itself which has left the vertebral canal DEFINITION

4. 1-2/1000 live birth Neural tube defects are second most common type of birth defect Myelomeningocele is the most common form of neural tube defect. Slightly higher in females than in males (1.2:1) 85% occur in lumbosacral region INCIDENCE - MENINGOMYELOCOELE

5. Poor nutrition - Folic acid deficiency Genetics (People of Northern European and Hungarian ancestry have the highest rates of the disease) Chromosome abnormalities: Trisomies 13 and 18 Triploidy Single gene mutations ETIOLOGY

6. Maternal obesity, hyperthermia pregnancies complicated by diabetes at conception Drugs: valproate , carbamazepine and drugs to induce ovulation Maternal exposures to fumonisins , electromagnetic fields, hazardous waste sites, disinfection by-products found in drinking water and pesticides

7. Spina bifida is caused by the failure of the neural tube to close during the first month of embryonic development (often before the mother knows she is pregnant). Under normal circumstances, the closure of the neural tube occurs around the 23rd ( rostral closure) and 27th (caudal closure) day after fertilization. EMBRYOLOGY

8. Due to risk factor Failure of NTD to close during 1 st month of embryonic development Increase central nervous system pressure Fails to close properly NTD occur PATHOPHYSIOLOGY

9. Both meninges and spinal cord protrude into the skin of the back Leak of cerebrospinal fluid (CSF) is common Severe neurological deficits are common Risk for bacterial meningitis Paraplegia Diminished control of lower limbs, bladder and bowel Hydrocephalus often accompanies Short stature and precocious puberty Meningomyelocele – Clinical Features

10. Orthopedic abnormalities (i.e., club foot, hip dislocation) Bladder and bowel control problems, including incontinence, urinary tract infections, and poor renal function . Pressure sores and skin irritations Abnormal eye movement 68% of children with spina bifida have an allergy to latex Paralysis

11.  Scoliosis Back pain Partial or complete lack of sensation Weakness of the hips, legs, or feet of a newborn Other symptoms may include: Hair at the back part of the pelvis called the sacral area Dimpling of the sacral area Difficulty swallowing, which can lead to choking. Hoarseness. Breath-holding and problems breathing during sleep. Below-average intelligence.

12. Primary Functional deficits System Congenital Anomalies Lower limb paralysis Sensory loss Bladder and bowel dysfunction Cognitive dysfunction Cranial nerve dysfunction Ocular muscle palsies, Swallowing andeating problems and Abnormal phonation Facial clefts Heart malformations, and Genitourinary tract anomalies Urinary tract anomalies solitary kidney or malformed ureters

13. Ultrasound during the second trimester The diagnosis of myelomeningocele is certain when 3 classic central findings are present in ultrasonography concavity of the frontal bones, ventriculomegaly , and Chiari II malformation. Positive screening for maternal serum alpha-fetoprotein (AFP) Screening of the amniotic fluid for AFP, as well as for the presence of acetylcholinesterase DIAGNOSIS

14. Intrauterine surgery Post-natal surgery:- Closure of the Myelomeningocele is performed immediately after birth if external CSF leakage is present and typically within the first 24-48 hours in the absence of CSF leakage Multidisciplinary interventions needed to prevent progressive deterioration of multiple body systems Treatment team consists of pediatric specialists in Physical medicine and rehabilitation Neurosurgery Urology TREATMENT

15. Orthopedics along with Pediatric nursing Physical therapy occupational and recreational therapy psychology and medical social work

16. Anticholinergics : - to suppress detrusor overactivity eg . Oxybutynin chloride Tricyclic antidepressants, eg . Imipramine hydrochloride Alpha-adrenergic antagonists: - decrease bladder outlet resistance, increase urinary flow rate, and improve bladder emptying eg . Terazosin Medication

17. Place the child in prone position. Cover the affected area with sterile gauze piece dipped in normal saline. Maintain hydration. Monitor for associated defects. IMMEDIATE CARE:

18. Catheters Braces High fiber diet Antibiotics may be used to treat or prevent infections such as meningitis or urinary tract infections. LIFE LONG TREATMENT:

19. Initial nursing management of the child with myelome­ningocele involves preventing trauma to the meningeal sac and preventing infection before surgical repair of the defect. Preventing Infection Promoting Urinary Elimination Assessing urinary function Performing clean intermittent catheterization NURSING MANGMENT

20. Dietary supplementation with folic acid has been shown to be helpful in reducing the incidence of spina bifida. Sources of folic acid include whole grains, fortified breakfast cereals, dried beans, leaf vegetables and fruits. It is recommended that any woman considering becoming pregnant take 0.4 mg of folic acid a day. Pregnant women need 1 mg per day. PREVENTION:

21. Difficult delivery with problems resulting from a traumatic birth, including cerebral palsy and decreased oxygen to the brain Frequent urinary tract infections Hydrocephalus Loss of bowel or bladder control Meningitis Permanent weakness or paralysis of legs COMPLICATIONS:

22. Many individuals with spina bifida have an associated abnormality of the cerebellum, called the Arnold Chiari II malformation. In affected individuals, the back portion of the brain is displaced from the back of the skull down into the upper neck. Neurological complications:

23. Specific areas of difficulty in some individuals include planning, organizing, initiating, and working memory. Problem-solving, abstraction, and visual planning may also be impaired. Children with spina bifida and shunted hydrocephalus have higher rates of ADHD. Executive function:

24. Individuals with spina bifida may struggle academically, especially in the subjects of mathematics and reading. In one study, 60% of children with spina bifida were diagnosed with a learning disability. ACADEMIC SKILLS:

25. Compared to typically developing children, youths with spina bifida may have fewer friends and spend less time with peers.  SOCIAL COMPLICATIONS: