Spina Bifida Flashcards
S3Q3
SPINA:
pathology - main shit, when, methionine, gene, reductase, primary neurolization when + forms what (2) where
epidemiology- sex, family, factor (2)
PATHOLOGY
- neural tube defect
- spine not close by 3rd week gestation
- primary neurolization (day 22): brain & spine in ectoderm
- methionine: protein & other compounds
- gene: convert folate, process amino acids, make reductase
- reductase: make vitamin folate (B9)
EPIDEMIOLOGY
- F>M
- 1st degree (sibling, child)
- multifactorial; polygenic inheritance, environmental
SPINA: Dx & Prog
prenatal dx - US when, check (2) where (2), condition + when
prognosis - prenatal dx = what, tx + when = what, neurogenic in who, children today lifespan, folic acid dosage, factors (3)
PRENATAL DX
- fetal US: 16-24w
- alpha fetoprotein & acetylcholinesterase in maternal serum & amniotic fluid
- inc AFP in amniotic = amniocentesis (16-18w)
PROGNOSIS
- prenatal dx myelomeningocele = less ventriculomegaly
- aggressive tx + closure in neonatal = better survival
- children today = N lifespan
- folic acid: 4g/day 1st trimester
- factors: hydrocephalus, arnold chiari, level of lesion
SPINA: Subtypes
spina bifida occulta - other name, structure, sx (2), level
spina bifida cystica - cystica level, manifesta (3), myeloschisis (1.2), meningocele (1.2), myelo (4=1)
which mild, most severe
SPINA BIFIDA OCCULTA
- closed spine dysraphism
- mild
- only pos vertebral structures (arch), no sac/cyst
- usually lumbar/sacral
- sx: nodosa (black skin), hair patch
SPINA BIFIDA CYSTICA
- open spine dysraphism
- has sac & bulging
- cystica: lumbar
- manifesta: myeloschisis, hydrocephalus, tethered cord
- myeloschisis: skin exposed s sac & meninges; most severe
- meningocele: vertebral arch + meninges + CSF
- myelomeningocele: arch + meninges + CSF + neural/spinal cord = more functional limit below lesion
SPINA: Ax
impairments - direct (4), foot (3), other (4)
neuro complications (5.1)
IMPAIRMENTS
- spasticity, flaccid, weak, contracture
- foot: equinovarus, calcaneus, rocker bottom
- cognitive, seizure, neurogenic, early puberty
NEURO COMPLICATIONS
- diastematomyelia, tethered, arnold
- forebrain affectation, ventricles
- non-neuro: latex allergy
SPINA: Prognosis
ambulation (3G)
community amb s AD
- gr. 4-5 glutes & TA
not complete w/c
- gr. 4-5 iliopsoas & quads
partial or complete w/c
- gr. 0-3 iliopsoas
CONGENITAL: Congenital Hip Dislocation
teratogenic/antenatal - what, when, severity
congenital hip - what
acetabular dysplasia - what, when, lead to (1=1)
congenital sublux - what (2)
unstable hip - what, severity
pathology - position, acetabulum (3), femoral head (2.1), adductors
- teratogenic/antenatal: hip fixed during gestation; severe
- congenital: may or may not be reduced
- acetabular: shallow acetabulum (3-4m in x-ray) = femoral head may slip out = hip dislocation
- congenital subluxation: femoral head not in acetabulum or dislocated
- unstable hip: most mild
pathology
- hip in ExAdEr
- acetabulum: roof more sloped, shallow
- femoral head: sup & pos, flatten
- adductors: contracted
CONGENITAL: Congenital Hip Dislocation
ax (neonatal) - barlow, ortolani, pistoning other term (2) + how + result
ax (when) - galleazi, hart, trendelenburg other term (2)
AX NEONATAL
- barlow: for dislocatable, ADD
- ortolani: for relocatable, ABD
- pistoning/telescoping/dupuytren: up down force on femur in 90 hip & knee = inc skin fold
- barlow & ortolani til 2y since hip fixed on acetabulum
AX INFANCY-2Y
- galleazi: one knee higher
- hart/ABD test: move hip = inc ROM/ABD
- trendelenburg/waddling/abd lurch: tight ADD
CONGENITAL: Congenital Hip Dislocation
measurement - hilgenreiner, perkin, shenton, acetabular index + normal value
radio findings (5)
MEASUREMENT
- hilgenreiner: triradiate cartilage
- perkin: vertical line
- shenton: coxofemoral & obturator
- acetabular index: between triradiate & medial to outer edge of roof; <30
RADIO FINDINGS
- femoral head: up & out, in suplat quadrant
- delayed growt of capital epiphysis ossification centers
- inc acetabular obliquity (>30 index)
- dec central-edge angle
- disrupted shenton line
CONGENITAL: Congenital Hip Dislocation - Tx
conservative - main shit, age, precaution, van rosen ilfeld pavlik frejka purpose + severity
non-conservative - traction cast (age), surgical, open reduction (age & approach), redirection of what
CONSERVATIVE (SPLINTING)
- 4-6m, ABD <60
- van rosen: malleable splint, severe
- ilfeld: stainless aluminum with swivel joint, severe
- pavlik: straps, mild-mod
- frejka pillow: position into ABD, mild
NON-CONSERVATIVE
- traction & plaster cast: >18m
- surgical resection of ADD
- open reduction: 18m-2y, ant iliofemoral approach
- redirection of acetabular dome
CONGENITAL: Congenital Hip Dislocation - Tx
surgery - age, ferguson (age & 1=1), chiari (1=1 & how), salter (what via what & cuts), pemberton (what & cuts)
> 6y - tx, complications (2)
SURGERY (2-6y)
- ferguson: 18m; tenotomy of iliopsoas tendon = bye contracture
- chiari: lat pelvic displacement = inc acetabular coverage, cut femur & put into acetabulum
- salter: acetabular redirection osteotomy; cut iliac crest
- pemberton: acetabular redirection osteotomy; cut iliac crest, iliac tuberosity, acetabulum
6Y
- conservative
- complications: osteonecrosis of femoral head, OA
CONGENITAL: Dactyly
camptodactyly - epidemiology (finger), sx (1=1), tx (2)
macrodactyly - sx, tx
clinodactyly, brachydactyly, pouce flotant
CAMPTODACTYLY
- epi: little finger >
- sx: flexor digitorum sublimis shortened = FLEX contracture
- tx: early manipulation, retention splint
MACRODACTYLY
- sx: larger
- tx: amputation
CLINODACTYLY
- sx: lateral contracture
BRACHYDACTYLY
- sx: shortened fingers
POUCE FLOTANT
- sx: rudimentary thumb s MCP
CONGENITAL: Dactyly
syndactyly - epidemiology (sex + hand vs. foot + ulna vs. radius + finger), sx (1), types (3), tx + age
polydactyly - epidemiology (side + associated), sx (3)
SYNDACTYLY
- epi: M>F, H>F, U>R, seldom thumb
- sx: webbed fingers or toes
- simple: skin + CT
- complex: + bone
- lobster: severe
- tx: surgery within 18m-5y
POLYDACTYLY
- epi: (B), with syndactyly
- sx: supernumerary, small appendage or fully formed
CONGENITAL: Arthrogryposis Multiplex Congenita
pathology, epidemiology (side + spared), sx (7.2), prognosis
- patho: incomplete ankylosis of joints
- epi: symmetrical, trunk spared
- prog: contractures don’t progress
signs/sx
- shoulder IR, elbow & knee FLEX/EXT, wrist FLEX, hip FLEX ER or dislocated, patella absent or dislocated, clubfoot, elevated scapula
- biceps & brachioradialis absent or inactive
CONGENITAL: Sprengel’s
epidemiology (side)
sx - scapula (5), bone, muscle (1=2)
tx - (2) + age
- epi: (B)
signs/sx
- scapula: smaller, elevated 1-4in, smaller vertical, bigger width
- omovertebral bone: osseous between vertebra & scapula
- shortened cervical muscles = ABD restricted, ROT spared
tx
- surgery within 5y
- postural
CONGENITAL:
congenital synostosis of cervical spine - name, pathology, sx (2), motion, tx (1)
cleidocranial dysostosis - sx (2=1), tx
CONGENITAL SYNOSTOSIS OF CERVICAL
- klippel feil: fused cervical vertebra
- sx: low pos hairline, LAT motion more restricted than FLEX/EXT
- pterygium colli/web neck: traps stretch winglike from mastoid to shoulder
CLEIDOCRANIAL DYSOSTOSIS
- sx: no or partial clavicle, late ossification of fontanel, little disability
- tx: none
