Test 3 part 3 Flashcards
_________________ is due to the degeneration of nervous tissue in the spinal cord, particullary with sensory neurons connected with cerebellum. this is often associated with kyphoscoliosis
freidreichs ataxia
what laboratory tests may be done for pt with kyphoscoliosis presenting for surgery?
CBC, BMP, UA
type and screen
for patients with severe scoliosis presenting for major surgery it is reasonable to consider ___________ and _________ tests
PFTs; ABG
anesthesia considerations for pts with kyphoscoliosis
1.postop pulmonary complications are common
2. plan for induction
3. monitoring
4. blood optimization (iron, epo)
5. postop care
what monitoring should be considered for pt presenting with kyphoscoliosis
- a line
- neurologic
- wake up test
_________________ is a rare autosomal dominate inherited disease of connective tissue that affects bone, sclera, and the inner ear
Osteogenesis imperfecta
2 types of osteogenesis imperfecta
OI congenita
OI tarda
OI congenita
fractures will occur in utero, death occurs during the perinatal period
OI tarda
childhood or early adolescence with presence of blue sclera, fractures from trivial trauma, kyphoscoliosis, bowing of femur/tibia, otosclerosis –> deafness
with osteogenesis imperfecta, aside from primary sx, what else may be present?
impaired plt fx
hyperthermia with hyperhidrosis
increased serum thyroxine
Tx of OI
biphosphonates (strengthen bone)
anesthesia mangement of pt with OI
- intubation: decreased ROM C-spine, possible mandibular fx
- kyphoscoliosis and pectus excavatum –> VQ mismatch
- regional anesthesia is acceptable (check plt)
- mild intraoperative hyperthermia
in a pt with OI, using _____________, an common induction agent, may lead to fasiculations which may produce fractures
succ
_______________ is an inherited autosomal dominant connective tissue d/o that is due to a defect in glyocprotein fibrillin-1
marfan syndrome
Clinical features of Marfan syndrome
- tall stature, long tubular bones (“abe lincoln”)
- high arched palate
- pectus excavatum or carinatum
- kyphoscoliosis
- hyperextensibility of the joints
- early development of emphysema
- high incidence of spontaneous pneumo
- ocular d/o
- risk of aortic dissection
if have a patient with marfans syndrome, what is the one prudent thing to avoid during surgery?
any sustained increase in systemic blood pressure d/t risk of aortic dissection
CV complications in pt with marfan
aortic dilation/dissection
prolapsed valves
bacterial endocarditis
for pt with marfan syndrome, they often have what cardiac conduction abnormality
BBB
if pt with marfans has a dilated thoracic aorta, what should they be put on prophylactically
Beta blocker
_______________ poses a unique risk of aortic rupture/dissection in pts with marfans syndrome
pregnancy
anesthesia implications for marfans syndrome
- focus on CV abnormalities w/ high suspicion for pneumothorax
- will have high arched palate –> crowded teeth and TMJ dislocation = airway concern
- aortic dissection prevention
__________________ is a group of inherited connective tissue d/o that are caused by abnormal production of procollagen and collagen
Ehlers-Danlos syndrome
what type of Ehlers Danlos syndrome may be associated with increased risk of death
vascular type
characteristics of Ehlers-Danlos Syndrome
- joint hypermobility
- skin fragility or hyperelasticity
- bruising and scarring
- musculoskeletal pain - susceptible to OA
- GI, uterus, and vasculature have increased type III collagen –> spontaneous rupture