Incidence:

• cleft lip: 1/700 births but more frequent in Asia and rare in Africa; unilateral in 80 % of cases, most often on the left side. It is associated with other anomalies (syndromic) in 20 % of cases (for example, van der Woude syndrome). A (rare) median cleft lip is always syndromic !
• cleft lip palate: associated with other anomalies (syndromic) in 50 % of cases
• cleft palate: 0.4/1000 births, associated with other anomalies (syndromic) in 50 % of cases (for example CHARGE or Stickler syndrome); bifid uvula (1/80 in the Caucasian population) is a minor form of cleft.

More than 300 syndromes include a cleft lip, palate and lip palate.

Many non-syndromic cleft palates and cleft lips are actually multifactorial genetic diseases in which a genetic susceptibility (less severe mutations or noncoding regulatory genes) is associated with environmental factors (antiepileptic drugs, tobacco, alcohol, maternal age).

1 incomplete cleft lip

2 complete cleft lip

3 velar (velum); submucosal clefts of the velum also exist (not shown here)

4 (median) complete cleft palate

5 unilateral cleft lip and palate

6 bilateral cleft lip and palate 

Anesthetic implications: 

• preoperative : multidisciplinary care and search for associated anomalies: clinical examination,  cardiac and brain ultrasound, etc. Obstructive sleep apnea is frequent in case of cleft lip and  palate or cleft palate
• in case of cleft lip and palate or cleft palate: multiple interventions have to be planned expected, the timing of which depends on the teams: in general

-        cleft lip around 2-3 months (some teams: first week of life)

-        cleft palate between 6 and 18 months (some teams: from the age of 3 months)

-        if necessary, velopharyngoplasty and/or alveolar bone graft in childhood

-        in all cases: oral intubation with a preformed (RAE type) or a flexible reinforced tracheal tube

• in case of cleft lip and palate or cleft palate: risk of difficult intubation especially if micrognathia is present (Pierre-Robin sequence) (see this topic);
• in case of cleft lip and palate, it is often useful to fill the cleft at the level of the lips with wet gauze: this avoids that the laryngoscope blade slips into the cleft (which traumatizes the tissues useful for the surgical repair) and facilitates direct laryngoscopy. The same result can be obtained by using a pediatric size gum shield unless the premaxillary bud is too prominent.  Emergence of anesthesia can be delicate with an increased risk of airway obstruction due to the modification of the oropharyngeal morphology, pain, local swelling. To ensure upper airway patency, it is sometimes necessary (micrognathia, Pierre-Robin sequence) to insert a tongue stitch taped on the chin in place or a nasopharyngeal airway for a few hours.
• in case (rare) of early bleeding after a left palate repair, squeeze the neo-palate cephalad with the palmar face of the thumb covered with a wet swab for about 10 minutes : this is generally sufficient to ensure permanent hemostasis. The dehiscence of the sutures that keep the mucosal flap in place can creates an upper airway obstruction: a simple way to maintain the airway patent before and during induction of anesthesia is to place an oropharyngeal airway in the child's mouth to maintain the flap against the palate.
• postoperative precautions

*        after the repair of a cleft palate or lip and palate: nasal intubation or placement of a nasogastric tube should be performed with extreme caution

*        after a pedicled flap between the lower and the upper lip (Abbe-Estlander flap): release of the flap under sedation or nasal fiberoptic intubation

*        after a velopharyngoplasty: avoid as much as possible a nasotracheal intubation. If it is absolutely necessary, the following sequence is recommended:

$        preoperative ENT examination by nasal fibroscopy to check the permeability of the patency of the nares;

$        after anesthetic induction under spontaneous ventilation, a soft-tipped small suction catheter is inserted into one nostril and directed through the nare(s) while palpating the oronasal membrane with a finger of the other hand;

$        when the exploring catheter exits through the pharyngeal neo-orifice, direct laryngoscopy can be performed using the catheter as a guide to railroad a tracheal tube softened in hot water over it.

$        an alternative technique is to perform an initial oral intubation prior to any  nasopharyngeal manoeuvre : this improves the safety during the nasal intubation procedure

*        after the completion of a temporary flap between the dorsal surface of the tongue and the edges of the fistulous opening of a palatal fistula. The release of the flap raises an obvious problem of access to the airway without damaging the fragile mucosal flap. Options: 1) nasotracheal intubation with a fiberscope. (2) progressive release of the lingual part under sedation (inhalation anesthesia  via a nasopharyngeal airway or intravenous sedation with e.g. ketamine) associated with local anesthesia.

References:

-        Geneviève D, Captier G, Blanchet C. 
Syndromes avec fentes labiopalatines. 
In Syndromes dysmorphiques  coordonné par Lacombe D et Philip N, Collection Progrès en Pédiatrie,  Doin 2013, p 261-82

-         Seto-Salvia N, Stanier P. 
Genetics of cleft lip and/or cleft palate: association with other common anomalies. 
Eur J Med Genet 2014, in press

-         Dadure C, Capdevila X. 
Prise en charge anesthésique pour chirurgie de fentes labiopataines de l’enfant. 
Le Praticien en Anesthésie-Réanimation 2011 ; 15 : 206-10.

-         Mahmoud AAA, Fouad AZ, Mansour MA, Kamal AM. 
A novel intubation technique in bilateral cleft palate pediatric patients : hard gum shield-aided intubation. 
Pediatr Anaesth 2013; 23: 349-54.

-         Jagannathan N, Aveyard C. 
Palatal dehiscence : an unusual cause of acute upper airway obstruction in an infant during induction of anesthesia. 
Pediatr Anesth 2008 ; 18:1129.

-        Eipe N, Pillai AD, Choudhrie R.
The tongue flap: an iatrogenic difficult airway? 
Anest Analg 2006; 102:971-2.

-        Hee HI, Conskunfirat ND, Wong S-Y, Chen C. 
Airway management in a patient with a cleft palate after pharyngoplasty: a case report. 
Can J Anesth 2003; 50: 721-4.

-        Kopp VJ, Rosenfeld MJ, Turvey TA. 
Nasotracheal intubation in case of a nasopharyngeal flap in children and adults. 
Anesthesiology 1995; 82: 1063-4.

Updated: October 2019