Term
| What commonality do most kids with chromosomal disorders share? |
|
Definition
|
|
Term
| What considerations should cross my mind if i have a kid with Turner Syndrome |
|
Definition
| potential difficult intubation (short stature, webbed neck, small mandible), HTN due to coarctation of the aorta (avoid sympathomemitics like ketamine, endocrine problems (DM, thyroid conditions) |
|
|
Term
| What odd surgical situation may someone with Klinefelter syndrome put you in? |
|
Definition
| Breast CA correction in a male, they have an extra X chromosome and pronounced female characteristics. The most common symptom is infertility. |
|
|
Term
| What are concerns with cri-du-chat? |
|
Definition
| airway stridor and difficult intubations due to abnormal larynx, cleft lip/palate, hypotonia, laryngomalacia |
|
|
Term
| What response do kids with down's have to volatiles? muscle relaxants? |
|
Definition
| They have a reduction in catecholamine stores thus they have a reduced MAC requirement. the response to muscle relaxants is exaggerated. |
|
|
Term
| Along with difficult intubations, volatiles, and muscle relaxant changes, what two concerns may cross your mind when you prepare to anesthetize an down's kid? |
|
Definition
| Neck instability (may sublux at C1-C2) and congenital heart issues |
|
|
Term
| Pierre robin syndrome, what is my main concern? |
|
Definition
| the tounge falling back and producing breathing problems |
|
|
Term
| I have concerns reguarding difficult intubation, upper airway obstruction, increased ICP, and strabismus, what genetic disorder am I? |
|
Definition
|
|
Term
| My symptoms include blue sclera, hx of multipla fractures, abnormal platelet aggrigation, deafness, and intracranial hemmorhage. What congenital disease process am i and what are your anesthetic concerns? |
|
Definition
| osteogenesis imperfecta, use art line (BP cuff will break bones), fragile teeth, document preop and post op fractures, avoid succs (fasciculations can also break bones), elevated temp with general (but not associated with MH) |
|
|
Term
| what two gastric issues are present with scleroderma and how would you treat? |
|
Definition
| reflux (RSIV), and malabsorbtion (especially of Vit K) check coag labs |
|
|
Term
| scleroderma is a multisystem connective tissue disorder, aside from gastric issues, what other 2 major organ systems are effected? |
|
Definition
| lungs (fibrosis, restrictive disease, pulmonary HTN, effusions, cor pulmonale) and renal (renal disease presents 3 years after diagnosis) |
|
|
