Cleft lip and palate - NYSORA

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Cleft lip and palate

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

Definition and mechanisms

  • 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 syndromesFeatures
Velocardiofacial (DiGeorge) syndromeMicrocephaly 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 syndromeLower lip pits
Hypodontia
Congenital cardiac disease
Musculoskeletal issues
Most common orofacial clefting syndrome
Treacher-Collins syndromeMicrognathia 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 syndromeProgressive 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

Signs and symptoms

  • Opening in the upper lip that may extend into the nose or palate

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

Causes

  • 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

Complications

  • Feeding problems
  • Speech problems
  • Hearing problems
  • Frequent ear infections

Risk factors

Pathophysiology

cleft lip, cleft palate, pathophysiology, dental abnormalities, suckling difficulties, speech difficulties, otitis media, conductive hearing loss, vesopharyngeal incompetence, Eustachian tube

Treatment

  • 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)

Management

Considerations for anesthesia for cleft lip and palate surgery

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

cleft lip and palate, management, preoperative, postoperative, congenital heart disease, upper respiratory tract infection, obstructive sleep apnea, renal disorders, neuromuscular disorders, dehydration, malnutrition, airway, atropine, acetaminophen, ECG, airway obstruction, bleeding, disruption suture lines, laryngospasm, edema, NSAIDs, opioids, morphine, ketamine

cleft lip and palate, intraoperative, management, induction, maintenance, anesthesia, sevoflurane, CPAP, difficult airway, direct laryngoscope, bag mask ventilation, muscle relaxant, preformed RAE tracheal tube, ventilation, TIVA, fentanyl, remifentanil, infraorbital nerve block, nasopalatine block, palatine block

Keep in mind

  • 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 

Suggested reading

  • Denning S, Ng E, Wong Riff KWY. Anaesthesia for cleft lip and palate surgery. BJA Education. 2021; 21(10): 384-389.

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