Spina Bifida Facing the primary level viva Clickonravi

Spina Bifida Facing the primary level viva © Clickonravi. com

Spina Bifida n n n Umbrella term Cranial neural tube defects Spinal neural tube defects n SB cystica n n Meningocele Meningomyelocele Lipo MMC SB occulta n n n Tethered cord syndrome Intraspinal lipoma Diastematomyelia 2

Start with history n Family n n Prenatal n n Anemia, diabetes infection (TORCH), teratogenic influences (radiation, drugs, alcohol), folic acid Intrapartum n n Abortion, miscarriages, consanguinity, mental retardation Fetal movements, premature rupture of membranes, method of delivery , breech, polyhydramnios. Post natal n n n Apgar scores, birth weight, diminished activity and cry, head size, apnea, poor sucking. Urinary leaking, fecal incontinence (perineal excoriation) Symptoms due to raised ICT or menningitis (high grade fever, irritable vomitting child) 3

Examination At the level of lesion n Below the level of lesion n Above the level of lesion n 4

At the level of lesion n n The swelling (describe it under the usual S Das headings) Site n n n n n In relation to the vertebras No of segments involved Skin over the swelling Ulcer CSF leak sac integrity Marginal epithelization Neural placode dimensions Obvious deformity of the spinal column n Kyphosis, scoliosis 5

Below the level of lesion n To see the sensory and motor involvement below the lesion Sensory level difficult in new born and infants more useful in bigger children Limbs – motor involvement n Grading of limb movements n n n No movement on stimulus 0 Some movement on painful stimulus 1 Movement on tickling 3 Passive movements 4 Against gravity (spontaneously flexed limbs) 5 6

Below the level of lesion Symmetry of the paralysis – may be more on one side n Spastic type of paralysis – limbs in constantly extended position n Clonus or focal fasciculations n Muscle bulk , trophic ulcers n Movements at the joints indicates the spinal segment involved n 7

Below the level of lesion Knee L 4 -L 5 Knee Extension L 5 -S 2 knee flexion Hip L 1 -L 3 hip flexion Ankle L 5 -S 2 hip extension Sacral roots S 3 S 5 Plantar flexion 8

Below the level of lesion n Bulbo cavernosus reflex n n Press the glans of penis gently while finger appreciates the wink in the external anal sphincter Anocutaneous reflex n Gentle stroking of the perineal area causes a wink in the external anal sphincter Cremastric reflex – S 2 n Indicates the integrity of the sacral nerve roots and the perineal sensation n 9

Below the level of lesion n Anal canal n n Lax, patulous, anal grip, leaking fecal matter on abdominal pressure Urinary bladder n Palpable with full bladder, bladder emptying on slight abdominal pressure (Credes method ) 10

Above the level of lesion n Hydrocephalus n n n n Head size Fontanelles Neck rigidity Second lesion Abnormal head shape Raised ICT protuberant eyes, irritable vomiting child Others n n n Abnormally protuberant abdomen Hydronephrosis lump Vertebral anomalies 11

General examination Level of alertness n Cry n Cranial nerve examination (I have never done it ) n 12

Possible questions n n n n n What is u r diagnosis Embryological aspects Structural anatomy Why is hydrocephalus present What is Arnold Chiari How will u proceed Timing of surgery Principles of repair Basics of shunt surgery 13

n Diagnosis statement - Lumbosacral MMC with lower limb paresis with bladder bowel involvement 14

Embryological aspects Simple non closure theory n Overgrowth and non closure theory n Reopening theory n 15

Structural anatomy 16

n Why is hydrocephalus present Four reasons for associated hydrocephalus n n Obstruction to the outlet of 4 th ventricle (part of AC malformation) Cork in the bottle phenomenon Secondary aqueductal stenosis Defect in CSF absorptions 17

What is Arnold Chiari Malformation Main pathologic group is chiari malformations n Chiari malformations are of 4 types n Type 2 malformation is also called as Arnold chairi malformation which is commonly associated with spina bifida n It has Caudal displacement of cebellar vermis, 4 th ventricle and lower brain stem below the plane of foramen magnum n 18

How will u proceed n Investigations Spinal x rays ap/lat views – it is difficult to decide the segment involved but tells about the vertebral anomalies above and below n Skull x rays – lacunar deformity, reversal of normal curve of bassi-occiput n 19

n USG n n n CT n n Of spine not very useful but good in demonstrating hydrocephalus MRI Spine n n n Most imp invg Done for swelling as well as the cranium and the abdomen tells the exact anatomy of the defect very usefull in lipo MMC where intraspinal lipoma is anticipated Diagnosis of AC malformations Minimum recommended Invg n n X ray spine AP/LAT Usg of swelling and cranium MRI spine General hematology 20

Timing of surgery n Presenting immediately after birth n n Delayed presentations n n n Good skin cover, no infection - wait till the child is physiologically mature – operate at 4 weeks + Very thin cover, impending rupture – no infection – operate within 7 days Ruptured MMC – antibiotics for 2 days – operate Good skin cover, no infection – elective surgery Leaking MMC, infection – antibiotics for 48 -72 hoursrepair Spina bifida occulta n Operate before 1 year to de tether the cord. 21

Principles of repair n n Preservation of the neural elements Closure of the dural defect 22

Shunt surgery n Timing At the time of MMC repair is hydrocephalus is documented n Close the MMC, watch for Hydrocephalus n n Types Ventriculoperitoneal n Ventriculoatrial n 23

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