Facing the primary level viva Spina Bifida Facing the primary level viva © Clickonravi.com

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

Start with history Family Prenatal Intrapartum Post natal Abortion, miscarriages, consanguinity, mental retardation Prenatal Anemia, diabetes infection (TORCH), teratogenic influences (radiation, drugs, alcohol), folic acid Intrapartum Fetal movements, premature rupture of membranes, method of delivery , breech, polyhydramnios. Post natal 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)

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

At the level of lesion The swelling (describe it under the usual S Das headings) Site 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 Kyphosis, scoliosis

Below the level of lesion 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 Grading of limb movements No movement on stimulus 0 Some movement on painful stimulus 1 Movement on tickling 3 Passive movements 4 Against gravity (spontaneously flexed limbs) 5

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

Below the level of lesion Knee L4-L5 Knee Extension L5-S2 knee flexion Hip L1-L3 hip flexion L5-S2 hip extension Ankle Sacral roots S3-S5 Plantar flexion

Below the level of lesion Bulbo cavernosus reflex Press the glans of penis gently while finger appreciates the wink in the external anal sphincter Anocutaneous reflex Gentle stroking of the perineal area causes a wink in the external anal sphincter Cremastric reflex – S2 Indicates the integrity of the sacral nerve roots and the perineal sensation

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

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

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

Possible questions 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

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

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

Structural anatomy

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

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

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

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

Timing of surgery Presenting immediately after birth 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 Delayed presentations Good skin cover, no infection – elective surgery Leaking MMC, infection – antibiotics for 48-72 hours- repair Spina bifida occulta Operate before 1 year to de tether the cord.

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

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