Neural TUBE DEFECTS result from failure of the neural tube to close spontaneously between 3rd and 4th week of gestation. - Folic acid supplementation antenataly reduces incident of NTDS. Major NEURAL TUBE DEFECTS are: SPINA BIFIDA oculta Meningocele Myelomenilgocele Encephalocele Anencephaly Dermal sinus Tethered cord.
Neural TUBE DEFECTS result from failure of the neural tube to close spontaneously between 3rd and 4th week of gestation. - Folic acid supplementation antenataly reduces incident of NTDS. Major NEURAL TUBE DEFECTS are: SPINA BIFIDA oculta Meningocele Myelomenilgocele Encephalocele Anencephaly Dermal sinus Tethered cord.
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Neural TUBE DEFECTS result from failure of the neural tube to close spontaneously between 3rd and 4th week of gestation. - Folic acid supplementation antenataly reduces incident of NTDS. Major NEURAL TUBE DEFECTS are: SPINA BIFIDA oculta Meningocele Myelomenilgocele Encephalocele Anencephaly Dermal sinus Tethered cord.
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- Failure of closure of neural tube allows excretion of fetal
substances (e.g. alpha-fetoprotein acetylcholine stares) into the amniotic fluids which are biochemical marker for NTDS. 1. SPINAL DYSRAPHISM (SPINA BIFIDA)
- There is only bony abnormality usually spina bifida-it is of no
clinical significance. In some cases patches of hair, a lipoma, discoloration of skin or dermal sinus in midline of the low back signifies an underlying malformation of spinal cord.
- A spine roentgenogram shows defect in closure of posterior
- Syringomyelia is cystic cavity within spinal cord that may or
may not communicate with CSF pathways.
- Diastometomyelia is division of spinal cord into two halves by
projection of a fibro cartilaginous or bony spur originating from posterior vertebral body & extending posteriorly.
- Tethered cord results when a thickened ropelike filum
terminale persists & anchors the conus at or below the L2 level.
- Above 3 conditions present with-
Delayed bladder control Gait abnormality sometimes with asymmetric weakness Foot deformity Scoliosis or back pain Recurrent meningitis 2. MENINGOMYELOCELE (MYELOMENINGOCELE)
- Most severe from of dysraphism involving. Vertebral column with
incidence 1/4000 live births.
* CLINICAL FEATURES:-
- It may located anywhere along the neurons but lumbosacral region
accounts for 75% case. - Lesion in low sacral region causes bowel & bladder incontinence associated with anesthesia in perineal area (saddle anesthesia) with no motor impairment. - Newborns with defect in midlumbar region typically have a sac like cystic structure covered by a thin layer of partially epithelized tissue. Remnauts of neural tissue are visible within sac which may rupture leading to leakage of C.S.F. - Examination of infants show flaccid paralysis of lower limbs, absent deep tendon reflexes, absent pain & touch sensation & abnormalities like clubfoot & subluxation of hip. Constant urinary dribbling & relaxed anal sphincter may be evident. - So lesion in midlumbar region produces lower motor neuron signs. - Due to neurogenic bladder they tend to have vesico-ureteric-reflux (UVR) recurrent urinary tract infections. If it persists for prolonged period it may lead to pyelonepharitis, hydronephrosis & renal failure. - Hydrocephalus in association with type 2 chiari defect develops in 80% cases of myelomeningocele. In general lower the lesion in neuraxis (e.g. sacrum) lowers the chance of hydrocephalus. - Ventricular enlargement may be slow or rapid causing bulging anterior fontanel, dilated scalp veins, sunset eye sign, irritability, vomiting & macrocephaly. - Many patients with chiari 2 malformation develop symptoms of brainstem dysfunction like dysphasia, choking, strider, apnea, vocalcord paralysis & may lead to death. MANAGEMENT 1. Councelling of parents:- - Give them time to adjust - explain prognosis & complications 2. Antibodies covering coagulase negative - Staphylococcus (CONS) at birth if sac is rupture & early surgical Intervention. 3. Surgery - If sac is unruptured one can wait for few weeks. Evaluation of Other congenital anomalies & renal function should be done Preoperatively. - After repair of myelomeningocele, most infants require shunting Procedure for hydrocephalus. Ventriculo-peritonial shunt. - If symptoms or signs of hindbrain dysfunction appear, early Surgical decompression of medulla & cervical cord is indicated. - Clubfoot may require casting & dislocated hip may require Operative intervention. 4. Urological management: - teaching parent & ultimately patients to regularly catheterize Neurological bladder to reduce residual urine. This will reduce Reflux & its complication like UTI & hydronephrosis. - Regular physical analysis & renal function tests like- urine Culture, s.creatinine, serum electrolyte, renal scans & USG. - In complicated cases- surgical derivation are only options. 5. Bowel management:- - In continence of bowel is common & socially not acceptable but Not very harm full. - Many children can be bowel trained with regime of Timed enemas or suppositories. - Appendicostomy for ante grade enemas may be helpful. 6. Physiotherapy:- - To help to achieve functional ambulance of child & to reduce & Treat foot deformities.