Pedi Cleft Lip and Palate

Question 1

What syndromes are commonly associated with

facial clefts?

Answer 1

Apert syndrome, ectodermal dysplasia, orofacial-digital I/II,

and Stickler, Treacher-Collins, Van der Woude, and Waar-
denberg syndromes

Question 2

What makes up the primary palate?

Answer 2

● Premaxilla
● Lip
● Nasal tip
● Columella

Question 3

What bones form the hard palate?

Answer 3

● Palatine process of the maxilla

● Horizontal plate of palatine bone

Question 4

How are cleft lips classified?

Answer 4

Unilateral (right or left) or bilateral

Complete (involves the entire vertical thickness of the upper
lip with extension into the nasal floor, often associated with
an alveolar cleft) or incomplete (extending from a slight
muscular diastasis at the vermilion to a small bridge of
tissue at the nasal sill called the Simonart band)

Question 5

How are cleft palates classified?

Answer 5

● Primary (involvement anterior to the incisive foramen) or
secondary (involvement posterior to the incisive foramen)
palate
● Unilateral (one palatal process is fused with the septum,
resulting in oronasal communication on one side only) or
bilateral (no connection between either palatal process
and the septum)
● Complete (cleft of both primary and secondary palate) or
incomplete (involves the secondary palate only, and has
varying degrees of severity)

Question 6

Discuss the nasal deformity associated with cleft

lips.

Answer 6

● Lateral and inferior displacement of alar base and lateral
crus, causing the dome to be flattened and rotated
downward on the cleft side
● Columella: Short, causing a horizontal orientation to the
nostril on the cleft side
● Septum: Tends to deviate toward the cleft side, with the
cartilaginous base displaced off of the maxillary crest

toward the cleft side. This septum malposition contrib-
utes to nasal tip tilt toward the noncleft side.

Question 7

Discuss the typical timing (i.e., age) for repair

of cleft lip and palate.

Answer 7

6 to 12 weeks: Repair cleft lip

10 to 13 months: Repair cleft palate, consider tympanos-
tomy tubes

2 to 5 years: Manage velopharyngeal insufficiency, consider
lip/nose revision
6 to 11 years: Orthodontic evaluation and treatment,
alveolar bone graft
12 to 21 years: Orthodontics and restorative dentistry,
orthognathic surgery (if needed), rhinoplasty (if needed,
typically the last procedure performed)

Question 8

What criteria did surgeons historically use before

proceeding with cleft lip repair?

Answer 8

"Rule of 10s"
● At least 10 weeks old
● Weighs at least 10 pounds
● Hemoglobin of at least 10 g/dL
In the era of modern pediatric anesthesia, these criteria are
not as relevant.

Question 9

What is a lip adhesion procedure?

Answer 9

A lip adhesion procedure converts a complete cleft lip into
an incomplete cleft lip at between 2 and 4 weeks of age,
potentially allowing the definitive lip repair to be performed
with less tension.

Question 10

What are the criteria for performing a lip adhesion?

Answer 10

● Wide, unilateral, complete cleft lip and palate
● Symmetric, wide bilateral, complete cleft lip with a very
protruding premaxilla
● Introduction of symmetry to an asymmetric bilateral cleft
lip

Question 11

What are three broad classifications of techniques

used to repair a unilateral cleft lip?

Answer 11

● Straight-line repair (Rose-Thompson repair)
● Triangular flap repair (Tennison-Randall repair, Skoog
repair)
● Rotation/advancement repair (Millard technique, most
commonly used; Mohler technique)

Question 12

What cleft lip repair technique entails a downward
and lateral rotation of the medial segment of the
cleft lip combined with the medial advancement of
the lateral cleft segment into the defect, which
places the scar in the position of the natural philtral
column?

Answer 12

Millard rotation-advancement technique for cleft lip repair

Question 13

What are four commonly used techniques for closure

of a cleft palate?

Answer 13

● Wardill-Kilner technique (V-Y pushback)
● von Langenbeck technique (bipedicled mucoperiosteal
flaps)
● Bardach two-flap palatoplasty
● Furlow technique (double opposing Z-plasty)

Question 14

What is the most common complication after

palatoplasty?

Answer 14

Velopharyngeal insufficiency

Question 15

What are the features of a submucous cleft palate

(SMCP)?

Answer 15

● Bifid uvula
● Zona pellucida (bluish midline region representing the
muscle deficiency; abnormal insertion of levator veli
palatini)
● Notch in the posterior hard palate due to loss of posterior
nasal spine

Question 16

What pathologic condition, commonly seen in
velocardiofacial syndrome, results when the triad of
visible signs of classic SMCP palatini muscle inserts
into the hard palate abnormally and there is a loss of
muscularus uvulae muscle tissue in the midline?

Answer 16

Occult submucous cleft palate

Question 17

What are the four velopharyngeal closure patterns?

Answer 17

● Coronal (most common)
● Circular
● Circular with a Passavant ridge
● Sagittal

Question 18

What are the surgical treatment options for velo-

pharyngeal insufficiency?

Answer 18

● Nasopharyngeal augmentation
● Sphincter pharyngoplasty
● Pharyngeal flap
● Palatoplasty

Question 19

Name the pathologic processes that originate from
remnants of the dental lamina, are located on the
alveolar ridge of newborns, and occasionally become
large enough to be clinically noticeable as discrete
white swellings on the alveolar ridges. They are
generally asymptomatic and do not produce any
discomfort for the infant and typically disappear
within 2 weeks to 5 months of postnatal life.

Answer 19

Gingival cysts of newborns (aka dental lamina cysts)

Question 20

What is the difference between Epstein pearls and

Bohn nodules?

Answer 20

● Epstein pearls: Cystic, keratin-filled nodules found along
the midpalatine raphe, likely derived from entrapped
epithelial remnants along the line of fusion
● Bohn nodules: Keratin-filled cysts scattered over the
palate, most numerous along the junction of hard and
soft palate and apparently derived from palatal salivary
gland structure