Cleft

Question 1

Infant female with cleft lip and palate

The parent Is asking how common is this?

Answer 1

Cleft lip with or without cleft palate occurs in 1/940 live births

Any particular population ?
*Highest incidence in Asians and Native Americans (1:500)
*African Americans lowest incidence(1:2000)
*Isolated Cleft Palate (1:1500-2000)
CL common in male
CP common in female

Question 2

Why its common on the left side?

Answer 2

it has been observed that the right palatal shelf reaches the horizontal position faster than the left during palatal shelf fusion.

Question 3

Describe the typical nasal deformities associated with the cleft lip at this age (ie: how are the columella, septum, medial & lateral crura and alar base distorted?)

Answer 3

short columella, collapsed ala on side of cleft, tip droop, short/ deviated septum, widened alar base

Question 4

Parents want to know, what’s the chance of our next baby having a cleft lip?

Answer 4

If the Cleft is Isolated (Non-Syndromic): 2-5%
If either parent or another close family member also has a cleft, the risk may be slightly higher, potentially increasing to 5-10%.

If the cleft is part of a genetic syndrome (such as Van der Woude Syndrome or 22q11.2 deletion syndrome), the recurrence risk depends on the specific syndrome’s inheritance pattern. For example, an autosomal dominant syndrome carries a 50% risk if one parent is a carrier.

Question 5

What are the most common skeletal jaw deformities in cleft lip/palate pt?

Answer 5

maxillary retrusive, anterior crossbite, posterior crossbite with lesser segment, transverse deficient, alveolar clefting, missing teeth

Question 6

Van der Woude Syndrome (VWS)

Most common syndrome with cleft lip

Answer 6

Genetic disorder characterized primarily by cleft lip and/or cleft palate and small indentations or pits on the lower lip.

AD mutations in the IRF6 gene.

1:35,000 to 1:100,000

Have missing teeth

Cleft lip and/or cleft palate.

Lower lip pits (characteristic small depressions or fistulae on the lower lip).

Hypodontia

Question 7

What is the difference between the primary palate and secondary palate?

Answer 7

Primary palate (pre-maxilla) contains the incisive canal and the 4 anterior teeth(incisors).
The secondary palate is everything behind the pre-maxilla and forward of the SP

Question 8

SYNDROME ASSOCIATED CLEFT PALATE.

Velocardiofacial Syndrome (DiGeorge)

Answer 8

• Velocardiofacial Syndrome (DiGeorge)
• Autosomal dominant 22q chromosomal abnormality
• Palatal deformity can range from weakness to complete cleft of the palate
• 1:4,000
• Facial manifestations can include small ears, flattened midface.
• Cardiac defects – TOF, VSD
• Learning disability

Question 9

SYNDROME ASSOCIATED CLEFT PALATE
Stickler Syndrome

Answer 9

SYNDROME ASSOCIATED CLEFT PALATE
* Stickler Syndrome
* Autosomal dominant, affects type II collagen
* 1:7,500-10,000
* Cleft palate with eye abnormalities
* Can include retinal detachment, cataracts
* Typically have flattened midface, can have hearing loss and
spondyloepiphyseal dysplasia
* Children with cleft and myopia should be referred to ophthalmologist.

Question 10

Pierre Robin Sequence (PRS)

Answer 10

Pierre Robin Sequence (PRS) is a condition present at birth that includes a combination of three main features:
Micrognathia
Glossoptosis
Recurrent airway obstruction

Question 11

What causes cleft lip?

Answer 11

6 weeks gestation median nasal processes fail to fuse with maxillary process

Question 12

What causes cleft palate?

Answer 12

At 8-12 weeks palatine shelves of max process merge in midline and fuse with nasal septum/vomer 🡪 failure leads to cleft palate (closes anterior to posterior)

Question 13

Mom asks – why can’t my baby drink out of a regular bottle with a cleft palate

Answer 13

(use specialty bottle like Haberman or Dr. Brown’s - 1 way valve, CP can’t form adequate seal)

Question 14

Is cleft lip more common in males or females?

Answer 14

Male and usually left side more common

Is isolated cleft palate more common in males or females – F

What % of isolated cleft palate associated with specific syndromes- 50%

Question 15

Any syndromes that are most commonly associated with isolated CP

Answer 15

Sticklers, van der woude, PRS, 22q deletion syndrome)

Question 16

What are factors associated with clefting?

Answer 16

family hx, low folic acid, smoking and alcohol, meds (retinoids, anticonvulsants)

Question 17

What comprises a cleft team

Answer 17

Plastic and or OMFS, lactation, ENT, neurosurg, psychologist, pediatric dentist, orthodontist, speech + language pathologist , audiologist, genetics, pediatrician

Question 18

How is cleft lip classified?

Answer 18

microform, unilateral or bilateral, incomplete vs complete (complete goes through nares)

Question 19

Tx timeline – after discharge & feeding/weight gain

Answer 19

Cleft lip ~ 3 months old with or without primary rhinoplasty

Cleft palate 9-18 months old
(speech development)

Speech surgery 3-5 yrs

Alveolar Bone graft (6-9 yrs old based on dental development)

Ortho surgery at maturity (14-16 yrs in females, 16-18 yrs in males)

Rhinoplasty last with revisions

Question 20

Goal for cleft palate repair

Answer 20

Reorient muscles (levator, palatoglossys, palatopharyngeal) and repair uvula

Question 21

What is the rational for rule of 10 in cleft pts?

Answer 21

• 10 weeks: optimal tissue healing, minimize psychological impact on the child
• 10 pounds: Minimize the risk of anesthesia.
• 10 Hgb: minimize the risk of intra operative bleeding.

Question 22

How does cleft lip occur?

Answer 22

• At 6 weeks gestation: Median nasal process fuses with maxillary process to form upper lip, philtrum of the lip, base of nose, and primary palate. Failure of fusion results in a clefting of the lip and/or alveolus.

Question 23

How does cleft palate occur?

Answer 23

• 8–12 weeks gestation: Palatine shelves of maxillary processes merge in the midline to fuse with nasal septum/vomer to form secondary palate. This fuses from anterior to posterior. The degree of clefting is dependent on timing of disruption.

Question 24

Miller rotational advancement flap

Answer 24

• Excision of hypoplastic tissue and three layer closure, skin, orbicularis and mucosa.
• Adv: allow columellar lengthening.
• The reason the technique is called a “rotational-advancement” flap is because the non-cleft side is cut in such a way that the tissue rotates to create a longer vertical width and the cleft side advances horizontally. This allows the scar to be hidden in a normal philtral line.

Question 25

Describe Bardach palatoplasty

Answer 25

I make two parallel incisions along the cleft margins and add relaxing incisions near the molars for flap mobilization. I then elevate bilateral mucoperiosteal flaps, ensuring I preserve the greater palatine artery. I close the nasal layer first with absorbable sutures from the hard palate to the uvula. Next, I reposition and suture the oral layer in the midline without tension. Finally, I reconstruct the uvula by reapproximating the uvular muscles to restore soft palate function

Question 26

the Miller rotational advancement flap for cleft lip repair

Answer 26

I begin by designing a triangular advancement flap from the non-cleft side and a rotational flap from the cleft side.

After making the incisions, I elevate the flaps carefully.

Next, I rotate the cleft-side flap downward to align the philtral column, and advance the non-cleft side flap medially to complete the closure.

The muscle layers are meticulously reapproximated to restore function and form, followed by precise skin closure to create a natural-looking lip contour.