special topics: counseling, medical, cranofacial &geneticsz. medically fragile Flashcards
hard palate fusion
in utero between the developmental ages of 8-9 weeks gestation
williams syndrome
rare genetic disorder impacting 1/20,000,
caused by abnormality on C7,
including gene that makes protein elastic,
williams syndrome characteristics
small for age, elfin-like appearance characterized by: small chin, turned-up nose, puffiness around the eyes, long upper lip, wide mouth, with small, widely-spaced teeth
williams syndrome intervention goal
increasing overall expressive & receptive language
bc 50-70 IQ
submucous or
occult cleft palate
surface tissues of soft or hard palate fuse
but underlying muscle or bone do not
client-centered counseling approach
respond with acceptance & empathetic listening
to both the content & the feeling of what the client is saying
PET scan
(positron emission tomography) uses emission-computed tomography that allows imaging of metabolic activity through measurements of radioactivity in the section of the body being viewed
von Langenbeck surgical method
surgical method of cleft palate repair,
involves raising 2 bipedicled flaps of mucoperiosteum,
bringing them back together,
& attaching them to close the cleft
hurlers syndrome
caused by autosomal recessive deficiency of X-L iduronidase
oral manometry
obtains a ratio by comparing pressures achieved in the nostrils-occluded & the nostrils-open conditions,
ratio below .89 is especially indicative or problems,
needs referral for possible further surgery or pharyngeal flap
eustachian tube dysfunction
related to the lack of contraction of the
tensor veli palatini muscle
in cleft palates
HIV & AIDS
use universal precautions,
almost always attacks nervous system,
almost 100% of manifestations are at the level of the head & neck
adult AIDS
experience auditory dysfunction
which can stem from opportunistic infections
as well as drug treatments that are potentially ototoxic
childhood AIDS
may manifest language delays,
elective mutism,
& hysterical aphonia
moebius syndrome
delayed language, articulation disorder, bilabial paresis & weak tongue control for lateralization, elevation, depression, & protrusion, mask-like face, history of feeding problems, uni/bilateral paralysis of the abductors of the eye