Cleft lip and palate

Learning objectives

Describe cleft lip and palate
Summarize the anticipated complications during the intraoperative and postoperative period among patients with cleft lip and palate
Discuss the perioperative management of patients with orofacial clefts

Orofacial clefts, encompassing cleft lip with or without cleft palate and cleft palate alone, are the most common craniofacial abnormalities
They are birth defects and result from tissues of the face not joining properly during development
These disorders affect the patient’s feeding, speech, hearing, dental health, appearance, and psychological health
Cleft lip: Fissure in the upper lip and may be incomplete or complete (through to the nasal cavity), unilateral, or bilateral
Cleft palate: Gap in the soft palate that may or may not involve the hard palate, submucous (mildest form), incomplete or complete, unilateral or bilateral

Common syndromes associated with cleft lip and palate

Chromosomal syndromes	Features
Velocardiofacial (DiGeorge) syndrome	Microcephaly and microstomia Flat nasal bridge, small ears, short stature Immune deficiency, congenital cardiac disease Velopharyngeal incompetence with or without cleft palate Laryngeal and tracheal anomalies 22q11 deletion (FISH test) Cleft palate in 30% of cases
Trisomy 21 (Down's syndrome)	Microstomia and relative macroglossia Epicanthic folds, simian crease Congenital cardiac disease Atlantoaxial subluxation and instability
Monogenic syndromes
Van der Woude syndrome	Lower lip pits Hypodontia Congenital cardiac disease Musculoskeletal issues Most common orofacial clefting syndrome
Treacher-Collins syndrome	Micrognathia and maxillary hypoplasia Choanal atresia Eye and ear malformations Intubation may become more difficult with age Cleft palate in 30% of cases
Hemifacial microsomia (Goldenhar syndrome)	Hemifacial and mandibular hypoplasia Cervical spine abnormalities Ear and eye abnormalities Intubation may become more difficult with age
Stickler syndrome	Progressive connective tissue disorder (autosomal dominant) Midface hypoplasia Micrognathia/PRS Retinal detachment and early cataracts Deafness Hypermobility of joints
Sequence
Pierre-Robin sequence (PRS)	Micrognathia Glossoptosis Underlying syndrome/anomalies Usually easier to intubate with age Cleft palate in 80% of cases

Cleft lip

Cleft does not affect the palate structure of the mouth
Partial or incomplete cleft lip: Formed in the top of the lip as a small gap or indentation of the lip
Complete cleft lip: Cleft continues into the nose
Can occur as a one-sided (unilateral) or two-sided (bilateral) condition
Due to failure of the fusion of the maxillary prominence and medial nasal processes (formation of the primary palate)

Cleft palate

The two plates of the skull that form the roof of the mouth are not completely joined
Cleft lip is also present in most cases
Complete cleft palate: Soft and hard palate, possibly including a gap in the mandible
Incomplete cleft palate: A hole in the roof of the mouth, usually as a cleft soft palate
Submucous cleft palate: Mildest form with no visible cleft but a failure of the palatal muscles to unite
The uvula is usually split
Due to failure of the fusion of the lateral palatine processes, nasal septum, or median palatine processes (formation of the secondary palate)
The hole in the roof of the mouth connects the mouth directly to the inside of the nose, resulting in velopharyngeal insufficiency (VPI) → hypernasal speech, nasal emission, and nasal turbulence

Most clefts are polygenic and multifactorial in origin with many genetic and environmental factors contributing
Genetic factors cause clefts in 20% to 50% of the cases
The remaining clefts are attributable to either environmental factors (e.g., teratogens) or gene-environment interactions

Speech therapy: Improve velopharyngeal function and develop normal speech without compensatory articulations
Dental care
Corrective surgery
- Cleft lip: 8-12 weeks
- Cleft palate: 6-12 months (dependent upon the size of the cleft)

Considerations for anesthesia for cleft lip and palate surgery

Patient-related factors	Usually infants or young pediatric patient Associated comorbidities and syndromes Nutritional status Airway obstruction Potential difficult airway
Anesthetic factors	General anesthesia with tracheal tube Shared airway
Surgical factors	Elective surgery (time to optimize)

Orofacial clefts are the most common craniofacial abnormality and patients with clefts require specialized multidisciplinary care from infancy through adulthood
Assess patients appropriately in the preoperative period as cleft anomalies are associated with numerous congenital abnormalities
Management of the airway may be challenging because of a difficult, shared airway, and postoperative airway obstruction
Monitor patients for signs of airway obstruction throughout their recovery from anesthesia
A multimodal approach to analgesia is important to provide optimal conditions for recovery