High-risk Infant Part 2

Question 1

cleft lip and cleft palate is also known as

Answer 1

orofacial cleft

Question 2

a group of conditions that includes cleft lip, cleft palate, and both together.

Answer 2

cleft lip and cleft palate, known as orofacial palate

Question 3

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.

Answer 3

environmental and genetic factors

Question 4

cause of cleft lip and palate

Answer 4

environmental and genetic factors

Question 5

during the first _ - _ weeks of pregnancy, the shape of the embryo’s head is formed. and during this, five primitive tissue lobes grow.

Answer 5

6-8 weeks

Question 6

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)

Answer 6

frontonasal prominence (1)
maxillar prominence (2)
mandibular prominence (2)

Question 7

primitive tissue lobe, from the top of the head down towards the future upper lip.

Answer 7

frontonasal prominence (1)

Question 8

primitive tissue lobe, from the cheeks, which meet the first lobe to form the upper lip.

Answer 8

maxillar prominence (2)

Question 9

primitive tissue lobe, just below, additional lobes grow from each side, which form the chin and lower lip.

Answer 9

mandibular prominence (2)

Question 10

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.

Answer 10

van der woude syndrome
siderius x
stickler’s syndrome
loeys-dietz syndrome
hardikar syndrome
patau syndrome (trisomy 21)

Question 11

syndrome connected to CLP that increases the occurrence of these deformities threefold.

Answer 11

van der woude syndrome

Question 12

syndrome connected to CLP that linked intellectual disability in addition to cleft lip or palate, symptoms include facial dysmorphism and mild mental retardation.

Answer 12

siderius x

Question 13

syndrome connected to CLP that cause cleft lip and palate, joint pain, and myopia.

Answer 13

stickler’s syndrome

Question 14

syndrome connected to CLP that cause cleft palate or bifid uvula, hypertelorism, and aortic aneurysm.

Answer 14

loeys-dietz syndrome

Question 15

syndrome connected to CLP that can cause cleft lip and palate, hydronephrosis, intestinal obstruction, and other symptoms.

Answer 15

hardikar syndrome

Question 16

cleft lip and palate may be present in many different chromosome disorders including _.

Answer 16

patau syndrome (trisomy 21)

Question 17

diagnosis of CLP made at the time of birth

Answer 17

physical examination

Question 18

diagnosis of CLP in utero

Answer 18

ultrasound

Question 19

type of cleft that is either a small gap or indentation

Answer 19

partial or incomplete palate

Question 20

type of cleft that continues into the nose

Answer 20

complete cleft

Question 21

cleft lip/palate is an “umbrella term” for collection of orofacial clefts which include the (3).

Answer 21

upper lip
maxillary alveolus (dental arch)
hard or soft palate

Question 22

clefts that does not affect the palate structure of the mouth (3)

Answer 22

upper lip
maxillary alveolus (dental arch)
hard or soft palate

Question 23

cleft can occur one-sided

Answer 23

unilateral

Question 24

cleft can occur two-sided

Answer 24

bilateral

Question 25

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.

Answer 25

maxillary medial nasal processes

Question 26

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.

Answer 26

cleft palate

Question 27

cleft palate can occur as soft and hard palate, possibly including a gap in the jaw

Answer 27

complete

Question 28

cleft palate can occur as a hole in the roof of the mouth, usually as a cleft soft palate

Answer 28

incomplete

Question 29

when cleft palate occurs, the _ is split. it occurs due to the failure of fusion of the (3) (formation of the secondary palate)

Answer 29

uvula lateral palatine processes nasal septum median palatine processes

Question 30

within the first _-_ months after birth, surgery is performed to close the cleft lip.

Answer 30

2-3 months

Question 31

the rules of ten in cleft lip was coined by _ and _ in _.

Answer 31

wilhelmmesen musgrave 1969

Question 32

the 3 rules of 10 condition for surgery of cleft lip

Answer 32

is at least 10 weeks of age weighs at least 10 pounds has at least 10g of hemoglobin

Question 33

two main categories of ARM

Answer 33

require protective colostomy does not require

Question 34

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.

Answer 34

t

Question 35

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.

Answer 35

anorectal malformations (ARM)

Question 36

cause of ARM

Answer 36

in 8% of patients, genetic factors

Question 37

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.

Answer 37

low lesion

Question 38

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.

Answer 38

high lesion

Question 39

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.

Answer 39

persistent cloaca

Question 40

associated anomalies of imperforated anus

Answer 40

**V**ertebral anomalies **A**nal atresia **C**ardiovascular anomalies **T**racheoesophageal fistula **E**sophageal atresia **R**enal or radial anomalies **L**imb defects

Question 41

the decision to open a colostomy during ARM is usually taken within the first _ hours of birth.

Answer 41

24 hours

Question 42

can be used to determine the type of imperforate anus

Answer 42

ultrasound

Question 43

depending on the severity of the imperforate, it is treated either with a _ or _.

Answer 43

perineal anoplasty colostomy

Question 44

most popular surgical technique for definitive repair of anorectal malformations

Answer 44

posterior sagittal approach (PSARP)

Question 45

involves the dissection of the perineum without entry into the abdomen and 90% of ARM in boys can be repaired this way.

Answer 45

posterior sagittal approach (PSARP)