Cleft Lip and Cleft Palate

Jesse Hyde, Zach Rosenthal,

Syphay Vongkhamchanh
Objectives

Define Cleft Lip and Cleft Palate

Explain the Physiology, Pathophysiology, and Embryology of Cleft Lip and Cleft
Palate
Define Coexisting Abnormalities Associated with Cleft Lip and Cleft Palate
Describe the Surgical Management of Cleft Lip and Cleft Palate
Explore the Preoperative, Intraoperative, and Postoperative Considerations for
Patient Undergoing Cleft Lip or Cleft Palate Repair
Review Medical Mission Considerations
Review Key Points With a Series of Questions
Definition

Cleft lip and cleft palate are birth defects that occur when a babys lip or mouth do
not form properly during pregnancy, and are collectively termed orofacial clefts.

Orofacial clefts are the most common craniofacial malformations in newborns

Orofacial clefts can consist of cleft lip with or without cleft palate, or isolated cleft
palate
Definition

Orofacial clefts can occur as isolated malformations or as part of another syndrome,

or as a component of a gene sequence

Orofacial clefting noted in over 300 syndromes

5 with highest prevalence:

Treacher Collins Syndrome
Stickler Syndrome
Pierre Robin Sequence
DiGeorge Syndrome
Amniotic Band Sequence
Types of Clefts

Cleft Lip

Unilateral (80%) or bilateral (20%)

Unilateral left side most common

Cleft Palate

Unilateral or Bilateral

Combination of both

Numerous combinations depending on

involvement of lip and palate
Etiology

Exact cause remains unknown

Underlying genes regulate the cell to cell interactions of embryology
Cell proliferation
Cell differentiation
Cell communication
Cell adhesion

Have identified numerous genes thought to be involved

Defective genes affects midface development clefting
In most cases 2-20 genes are thought to be involved
Cleft Lip Embryology
Complete closure of lip usually accomplished by day 35 post-conception
Lateral nasal, median nasal, and maxillary mesodermal processes merge
Failure of merger at any of these 3 sites leads to cleft lip
Location of cleft depends on which site failed to merge
Degree can be mild which involves just the lip
More severe cases involve the palate, midface, nose, eyes, and brain
Cleft Palate Embryology
Palatal closure is typically complete by day 56-58 post-conception
Isolated cleft palate occurs due to incomplete fusion of the palatal shelves
Mechanism of cleft palate remains debated
Abnormalities in apoptosis are thought to be the cause
Isolated cleft palate can occur because it fuses after the lip
Prevalence and Epidemiology

Race

Lowest in African Americans

Highest in American Indian and Japanese

Sex

Males > Females with exception of isolated cleft palate

Maternal Age 35 years old = prevalence

 Comparison

Total Prevalence of Orofacial Clefts = 14.5/10,000 births or 1/690 births

Cleft Lip Cleft Palate Cleft Lip w/ Cleft Palate

Prevalence 3.1/10,000 live births 5.9/10,000 live births 5.6/10,000 live births

Race/ethnicity African Americans < Whites/Hispanic < American Indian/Alaskan Native/Japanese

Sex M>F M<F M>F

*As Reported by National Birth Defects Prevention Network 2007-2011

Environmental Risk Factors

Medications
Anti-seizure Medications - phenytoin, sodium valproate, and topiramate

Methotrexate - folic acid antagonist

Cigarette Smoking

Alcohol
Linked with high prevalence in Native American newborns

Folate Deficiency
Contributes to a range of birth defects
Surgical Management

Cleft lip repair typically performed between 6 -12 weeks of age

Several methods can be used to surgically fix the cleft lip, depending on the
anatomy, to bring symmetry back to the childs face
Surgical Management
Cleft palate repair typically performed between 9 - 14 months old
Palatoplasty - surgical procedure used to correct/reconstruct the palate
Pharyngoplasty - surgical treatment of an incompetent velopharyngeal sphincter that was allowing
inappropriate nasal air escape during speech (which causes hypernasality)

Surgeon preference will dictate the timing and sequence of repair

Primary repair vs staged repair

Goals of surgery:
Help the patient develop normal speech
Aid in swallowing and breathing
Aid in normal development of associated structures of the mouth
Help to provide psychologic well-being
Surgical Management

Sample Staged Repair Plan

3mo - repair of CL and placement of
ventilation tubes
6mo - presurgical orthodontics; first
speech eval
9mo - speech therapy begins
9-12mo - repair of CP
1-7yr - orthodontic treatment
7-8yr - alveolar bone graft
8+ - orthodontic treatment continues
Coexisting Abnormalities

The facial region has physiologic functions that are interrelated with other patient
factors that may, or may not, require management
Hearing
There may be dysfunction of the Eustachian tubes requiring myringotomy and tubes
Swallowing
Could be due to abnormally shaped mouth or difference in tongue position/size
Malnourishment due to difficulty latching or swallowing
Difficulty could contribute to aspiration and/or reactive airway disease
Psychological wellness
Coexisting Abnormalities

There are more than 300 syndromes associated with facial clefting
Pierre Robin Sequence - 80% associated with cleft palate
micrognathia, glossoptosis
Treacher Collins Syndrome - 28% associated with cleft palate
Micrognathia, maxillary hypoplasia, choanal atresia
Stickler Syndrome
Connective tissue disorder, micrognathia, flat face, congenital cardiac disease
Velocardiofacial Syndrome
Microcephaly, microstomia, immune deficiency, congenital cardiac disease, laryngeal and tracheal
anomalies
Klippel-Feil - 15% associated with cleft palate
Short neck, fused cervical vertebrae, congenital cardiac disease

Somerville & Fenlon, 2005

Preoperative Considerations

Assessment
Consider other body system anomalies

Create your airway plan

Make sure plans A, B, & C are ready to go

Is the antibiotic ordered?

Prophylaxis may be needed if cardiac issues

Any contraindications to proceeding with surgery?

An undiagnosed murmur needs to be evaluated prior to surgery
Active illness (URIs) could increase the risk of airway obstruction and impede healing of lip and
palate repairs
Intraoperative Considerations

Surgical duration:
< 2 hours for palate surgery
2 - 4 hours for unilateral cleft
4 - 8 hours for bilateral cleft
Positioning
Supine
Roll under patients shoulders
Rose position commonly used
Supine with head over the edge of the operating table

Holzman, 2016
Intraoperative Considerations

Induction
Can use inhalation agent or IV medications
Airway plan options
Awake intubation used for anticipated difficult airways
Direct Laryngoscopy & oral/nasal RAE ETT
LMA with fiberoptic placement of ETT
Surgical airways may not be an option d/t airway abnormalities (assess beforehand)
Maintenance of Anesthesia
Variance depending on provider and procedure; typically no limitations
Example: balanced anesthesia with volatile anesthetic and opioids
Patients need to be spontaneously breathing at the end of the case
Intraoperative Considerations

Monitoring & Equipment

Standard monitoring procedures: ECG, SpO2, NIBP, EtCO2, temp
Oral RAE tracheal tube work well with Dingman-Dott mouth retractor
Surgeons often use lidocaine with epi to help restrict bleeding; be mindful of LA dose
Throat packs will be utilized to collect fluid accumulation in the pharynx
Emergence & Extubation
Surgeon should place nasopharyngeal airway if known difficult airway or other concerns
Remove throat packs
Preference to extubate patient when awake and breathing
Postoperative Considerations

NPA is an effective way to prevent post-op airway obstruction

Airway obstruction causes:
Laryngospasm
Retained throat packs
Bleeding or blood clot in the airway
Swelling caused by trauma from retractors
Respiratory depression due to opioids

Significant edema should preclude extubation

If re-intubated, may require mechanical ventilation for several days to allow
swelling to subside
Perioperative Considerations

Multimodal Analgesia
Narcotic use should be titrated carefully for preservation of spontaneous respirations
IV or PR acetaminophen
Infraorbital Blocks should be considered
Regional anesthesia affecting sensory of the upper lip and nose
NSAIDs (some surgeons prescribe them; controversy exists over involvement in post-op bleeding)
Safety and wound healing
Logan Bow may be placed over the lip repair for protection from accidental trauma
Welcome sleeves (arm splints) to keep fingers out of mouth, disrupting wound healing

Re-starting intake with clear liquids after child is awake & no bleeding seen
Future Planning

Patients with cleft lip/palate may have multiple surgeries performed during their
childhood. Remember to diligently document your findings and interventions to
help provide a safe continuum of care for them.
References
Childrens Hospital Colorado. (2017). Cleft lip/Cleft palate. Retrieved from https://www.childrenscolorado.org/
conditions-and-advice/conditions-and-symptoms/conditions/cleft-lip-and-palate/?&gclid=COKForiX79IC
FQ0zaQod2TkH4q
Davis, P. J. & Cladis, F. P. (2011). Smiths anesthesia for infants and children. (8th ed.) St. Louis, MO: Elsevier
Saunders.
Hardcastle, T. (2009). Anaesthesia for repair of cleft lip and palate. Journal of Perioperative Practice, 19(1),
20-23.
Hodges, S. C. & Hodges, A. M. (2000). A protocol for safe anaesthesia for cleft lip and palate surgery in
developing countries. Anaesthesia, 55(5), 436-441. doi:10.1046/j.1365-2044.2000.01371.x
Holzman, R. S., Mancuso, T. J., & Polaner, D. M. (2016). A practical approach to pediatric anesthesia (2nd
ed.). Philadelphia, PA: Wolters Kluwer.
References
Patel, P. (2016, Feb 7). Unilateral cleft lip repair treatment & management. Medscape. Retrieved March 24,
2017 from http://emedicine.medscape.com/article/1279641-treatment#showall.
Somerville, N. & Fenlon, S. (2005). Anaesthesia for cleft lip and palate surgery. Continuing Education in
Anaesthesia, Critical Care & Pain, 5(3), 76-79. doi:10.1093/bjaceaccp/mki021.
Stoelting, R. K., Hines, R. L., & Marschall, K. E. (2012). Stoelting's anesthesia and co-existing disease. (6th
ed.) Philadelphia: Saunders/Elsevier.
Wilkins-Haug, L. (2017). Etiology, prenatal diagnosis, obstetrical management, and recurrence of orofacial
clefts. In D. Levine (Ed.), UptoDate. Retrieved March 21, 2017 from https://www.uptodate.com/contents/
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