High-risk Infant Part 2 Flashcards
cleft lip and cleft palate is also known as
orofacial cleft
a group of conditions that includes cleft lip, cleft palate, and both together.
cleft lip and cleft palate, known as orofacial palate
CL/CP: the development of the face is coordinated by complex morphogenetic events and rapid proliferative expansion, and is thus highly susceptible to _, rationalizing the high incidence of facial malformations.
environmental and genetic factors
cause of cleft lip and palate
environmental and genetic factors
during the first _ - _ weeks of pregnancy, the shape of the embryo’s head is formed. and during this, five primitive tissue lobes grow.
6-8 weeks
during the first 6-8 weeks of pregnancy, the shape of the embryo’s head is formed. and during this, five primitive tissue lobes grow (5)
frontonasal prominence (1)
maxillar prominence (2)
mandibular prominence (2)
primitive tissue lobe, from the top of the head down towards the future upper lip.
frontonasal prominence (1)
primitive tissue lobe, from the cheeks, which meet the first lobe to form the upper lip.
maxillar prominence (2)
primitive tissue lobe, just below, additional lobes grow from each side, which form the chin and lower lip.
mandibular prominence (2)
genetic factors contributing to cleft lip and palate formation have been identified for some syndrome cases (6), but knowledge about genetic factors that contribute to the more common isolated cases of CLP is still patchy.
van der woude syndrome
siderius x
stickler’s syndrome
loeys-dietz syndrome
hardikar syndrome
patau syndrome (trisomy 21)
syndrome connected to CLP that increases the occurrence of these deformities threefold.
van der woude syndrome
syndrome connected to CLP that linked intellectual disability in addition to cleft lip or palate, symptoms include facial dysmorphism and mild mental retardation.
siderius x
syndrome connected to CLP that cause cleft lip and palate, joint pain, and myopia.
stickler’s syndrome
syndrome connected to CLP that cause cleft palate or bifid uvula, hypertelorism, and aortic aneurysm.
loeys-dietz syndrome
syndrome connected to CLP that can cause cleft lip and palate, hydronephrosis, intestinal obstruction, and other symptoms.
hardikar syndrome
cleft lip and palate may be present in many different chromosome disorders including _.
patau syndrome (trisomy 21)
diagnosis of CLP made at the time of birth
physical examination
diagnosis of CLP in utero
ultrasound
type of cleft that is either a small gap or indentation
partial or incomplete palate
type of cleft that continues into the nose
complete cleft
cleft lip/palate is an “umbrella term” for collection of orofacial clefts which include the (3).
upper lip
maxillary alveolus (dental arch)
hard or soft palate
clefts that does not affect the palate structure of the mouth (3)
upper lip
maxillary alveolus (dental arch)
hard or soft palate
cleft can occur one-sided
unilateral
cleft can occur two-sided
bilateral
CLP can occur as one-sided or two-sided, it is due to the failure of fusion of the _ and _ (formation of the primary palate.
maxillary
medial nasal processes
is a condition in which the two plates of the skull that form the hard palate (roof of the mouth) are not completely joined. the soft palate is in these cases cleft as well. in most cases, cleft lip is also present.
cleft palate
cleft palate can occur as soft and hard palate, possibly including a gap in the jaw
complete
cleft palate can occur as a hole in the roof of the mouth, usually as a cleft soft palate
incomplete
when cleft palate occurs, the _ is split. it occurs due to the failure of fusion of the (3) (formation of the secondary palate)
uvula
lateral palatine processes
nasal septum
median palatine processes
within the first - months after birth, surgery is performed to close the cleft lip.
2-3 months
the rules of ten in cleft lip was coined by _ and _ in _.
wilhelmmesen
musgrave
1969
the 3 rules of 10 condition for surgery of cleft lip
is at least 10 weeks of age
weighs at least 10 pounds
has at least 10g of hemoglobin
two main categories of ARM
require protective colostomy
does not require
t/f: if the cleft lip is bilateral and extensive, two surgeries may be required to close the cleft, one side first, and the second side a few weeks later.
t
birth defects in which the rectum is malformed. it is a spectrum of different congenital anomalies in males and females which vary from fairly minor lesions to complex anomalies.
anorectal malformations (ARM)
cause of ARM
in 8% of patients, genetic factors
form of ARM where colon remains close to the skin. in this case, there may be a stenosis (narrowing) of the anus, or the anus may be missing altogether, with the rectum ending in a blind pouch.
low lesion
form of ARM where the colon is higher up in the pelvis and there is a fistula conencting the rectum and the bladder, urethra, or the vagina.
high lesion
form of ARM, (from the term, an analogous orifice in reptiles and amphibians), in which the rectum, vagina, and urinary tract are joined into a single channel.
persistent cloaca
associated anomalies of imperforated anus
Vertebral anomalies
Anal atresia
Cardiovascular anomalies
Tracheoesophageal fistula
Esophageal atresia
Renal or radial anomalies
Limb defects
the decision to open a colostomy during ARM is usually taken within the first _ hours of birth.
24 hours
can be used to determine the type of imperforate anus
ultrasound
depending on the severity of the imperforate, it is treated either with a _ or _.
perineal anoplasty
colostomy
most popular surgical technique for definitive repair of anorectal malformations
posterior sagittal approach (PSARP)
involves the dissection of the perineum without entry into the abdomen and 90% of ARM in boys can be repaired this way.
posterior sagittal approach (PSARP)
