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Myelomeningocele is the most serious central nervous system birth defect compatible with life. Described from times of hippocrates and aristotle, who even recommended infanticide for these children Progress in treatment rapid since introduction of valved Many aspects still remain undiscovered. Associated in most cases with Arnold Chiari type II malformation Possible Aetiology Cytotoxic agents - Vinblastine and cyto chalasins Calcium channel blockers Phospholipase
Myelomeningocele is the most serious central nervous system birth defect compatible with life. Described from times of hippocrates and aristotle, who even recommended infanticide for these children Progress in treatment rapid since introduction of valved Many aspects still remain undiscovered. Associated in most cases with Arnold Chiari type II malformation Possible Aetiology Cytotoxic agents - Vinblastine and cyto chalasins Calcium channel blockers Phospholipase
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Myelomeningocele is the most serious central nervous system birth defect compatible with life. Described from times of hippocrates and aristotle, who even recommended infanticide for these children Progress in treatment rapid since introduction of valved Many aspects still remain undiscovered. Associated in most cases with Arnold Chiari type II malformation Possible Aetiology Cytotoxic agents - Vinblastine and cyto chalasins Calcium channel blockers Phospholipase
Copyright:
Attribution Non-Commercial (BY-NC)
Verfügbare Formate
Als PPT, PDF, TXT herunterladen oder online auf Scribd lesen
Associated in most cases with Arnold Chiari type II malformation Possible Aetiology Cytotoxic agents – Vinblastine & cyto chalasins Calcium channel blockers Phospholipase – C or concanavalin A Retinoic acid, Hydroxyurea, Mitomycin C
Deficiency of folate
Misexpression of Pax 3 gene product causes neural
tube defects Epidemiology Incidence 0.7 – 0.8 per 1000 live births
Recurrence risk for parents with
1 previously affected child rises to 1-2% 2 previously affected children raises risk to as high as 10 %
For a parent with myelomeningocele the risk of
child with the same is 3% Prenatal diagnosis
Maternal AFP at 16 to 18 wks detects open neural
tube defects with sensitivity of 75% ( not specific)
Fetal USG has a sensitivity of nearly 100%
( Banana sign & Lemon sign)
Amniotic fluid AFP & AChE done together final
method of identification Initial neurosurgical care
Includes 2. Stabilizing the infant, identifying associated abnormalities, closing myelomeningocele
4. Treating associated hydrocephalus when
present Initial clinical assessment Spinal level & size of lesion, redundancy and quality of surrounding skin, presence of kyphosis can affect difficulty of repair
Sensorimotor level determined by observing movements of
lower limbs & response to sensation
Initial evaluation for hydrocephalous i.e. head circumference,
palpation of anterior fontanelle, sutural diastasis
Assessment of lower cranial nerves and brainstem function
Initial clinical assessment
Poor prognostic signs
Poor feeding/nasal regurgitation, Weak cry, stridor, central apneoa, Hypotonia, poor head control, weak upper extremities
General paediatric examination to identify
associated congenital anomalies Stabilisation & pre operative care Placode protected since birth by covering with sterile saline soaked gauzes (not with povidone iodine) since it contains partially functioning neural tissue
Systemic antibiotics for 1st 3 post natal days to
prevent CSF / UT infections Operative treatment Operative treatment Placode closed in 1st 48 – 72 hrs to avoid neurologic deterioration & meningitis
Care taken to avoid cutting dorsal nerve roots along ventral
persistent tethering sectioned or treated simutaneously
Dural sac made capacious to prevent tethering
Operative treatment Significant kyphosis interferes with closure, compromises spinal stability hence kyphectomy performed simultaneously
Skin subcutaneous tissues undermined and closed
primarily
For large skin defects transposition flaps or
musculocutaneous flaps used Treatment of Hydrocephalous
Hydrocephalous clinically evident at birth (15%) or
develops days to weeks after closure of placode If untreated CSF may leak from wound chances of meningitis USG or CT measurements of ventricular size done Pulsed Doppler measurements of IC bld flow & resistive index in borderline cases Progressive increase in RI more suggestive Treatment of Hydrocephalous
after shunting seen in these pts due to stenosis / obstruction of foramen of Monro
Shunting contralateral ventricle or fenestrating
septum pellucidum treats these cases Hydrocephalous in Older child By 6yrs 52% children with meningomyelocele have had shunt revision & 20 % multiple revisions
Signs & symptoms of shunt malfunction in meningomyelocele
4. Headache, loss of appetite, nausea , vomiting 5. Behavioral changes 6. New onset / change in freq of seizures 7. Decline in motor performence 8. Worsening urinary function 9. Stridor / Vocal cord palsy 10. Pain in back or legs 11. Worsening scoliosis Hydrocephalous in Older child Fundoscopy a must in such cases
Cranial USG & CT for change in ventricular size
Shunt tap provides valuable info on shunt blockage
Traditionally malfunctions treated by shunt revision but
recently third ventriculostomy may be done in >6mths of age Encephalocele Introduction
Congenital malformation where CNS structures in
communication with CSF pathways herniate through defect in cranium
Incidence varies geographically from 1 in 3000 to
1 in 10000 live births Classification
According to According to location
contents of swelling Posterior Meningocele - occipital Encephalocele Anterior Meningoencephaloce - frontal le - sincipital Hydroencephalomeni ngocele - basal Encephalocystocele Pathophysiology Primary abnormality is mesodermal defect resulting in defect in calvarium and dura with herniation of CSF, brain tissue,meninges through defect
Failure of surface ectoderm to separate from neuroectoderm early in
embryonic development
In the calvarium, induction of bone formation may be defective, or
pressure erosion from an intracranial mass may occur
Defects at skull base may be related to faulty closure of neural tube or
failure of basilar ossification
Frontoethmoidal encephaloceles most common in Asia.
Clinical presentation Swelling present at birth Head size small ( larger the herniation smaller the head size )
Frontoethmoidal – swelling over root or bridge of nose
Nasopharyngeal, sphenoethmoidal, transsphenoidal – no obvious external swelling, CSF rhinorrhea or nasal obstruction may be present Orbital – proptosis Occipital – posterior swelling Associated Pathologies
Cleft lip, palate
Micrognathia Spinovertebral anomalies Renal agenesis Retinal detachment Cardiac anomalies Pulmonary & renal hypoplasia Prenatal Diagnosis Diagnosis thought impossible before skull ossification at 10 weeks' gestation (Earliest reported at 13 weeks' gestation)
US appearances of encephalocele variable in first trimester
Once diagnosed, search for associated anomalies, both intracranial
and extracranial (60-80%) performed
Risk of chromosomal abnormalities 13-44%; karyotyping offered to
mother
USG mainstay of fetal imaging, fetal MRI provides superior detail of
central nervous system (CNS) anomalies Prenatal USG 3D Reconstruction of USG Differential diagnosis
- Branchial cleft cyst
- Hemangioma - Cystic hygroma - Myelomeningocele - Scalp edema - Teratoma - Epidermal scalp cyst - Cloverleaf skull Operative treatment Consists of excision of hernial sac & repair of dural defect after replacing brain into cranial cavity
Not an emergency except when CSF leak present
Extensive surgery better tolerated at 6-8 mths of age
VP shunt can be inserted before corrective surgery to prevent
leak
Surgery best avoided when microcephaly with extensive
neural tissue in sac present Prognosis Size, content and presence of hydrocephalus influence prognosis in posterior encephalocele
In absence of hydrocephalus & meningitis
prognosis of anterior encephalocele is usually good
Absence of brain tissue within herniated sac most
favorable prognostic feature for survival Preop & post op anterior encephalocele Preop & post op posterior encephalocele