Cleft lip and cleft palate are among the most common congenital anomalies. They may occur alone, as part of a syndrome (there are more than 300 syndromes associated with facial clefting), or as a component of a sequence (for example, with Pierre Robin syndrome). Clefts of the palate may occur through the same mechanisms as cleft lip or be secondary to an anatomic obstruction preventing the medial fusion of the maxillary processes.
Closure of the cleft palate may result in insufficient tissue for development of the normal length or function of the soft palate and therefore may require a posterior pharyngeal flap. Velopharyngeal (VP) insufficiency is the cause of the hypernasal speech, nasal emission, and nasal turbulence.
There are many techniques for repairing a cleft palate, but all of them include a hard palate procedure and a soft palate (velar) procedure. Palatoplasties are performed with the intention of obtaining VP competence and normal speech and are typically performed before the first year of age.
Pharyngoplasties are performed to treat an incompetent VP sphincter that allows inappropriate escape of nasal air during speech, or hypernasality, defined as VP insufficiency. Pharyngoplasties are often performed as secondary speech procedures after a palatoplasty that failed to result in VP competence.
Most patients with a cleft lip or palate can be induced with a standard inhalational induction or IV induction.
Airway obstruction can usually be managed with the insertion of an oropharyngeal airway and continuous positive airway pressure.
For the patient with an isolated cleft lip and palate, difficulty with laryngoscopy is common. Factors predicting a more difficult laryngoscopic view include bilateral clefts and retrognathia. A wide oral opening will help to prevent the laryngoscope blade from slipping into the alveolar ridge defect during laryngoscopy. The view during laryngoscopy is unusual because of the cleft soft and hard palate, but the defect usually allows good visualization of the larynx.
Cleft patients with associated craniofacial anomalies or retrognathia may be difficult to ventilate or intubate. Adequacy of mask ventilation should be determined. The patient should remain spontaneously ventilating until the trachea is secured with an endotracheal tube.
Surgical repair of the cleft palate using a laryngeal mask airway (LMA) has been described. However, disruption of a previous cleft palate repair during the placement of an LMA has also been described, suggesting that care should be taken when placing an LMA in a patient with a history of cleft palate repair.
An oral Ring-Adair-Elwyn preformed tracheal tube is routinely used for the intubation because the preformed bend in the tube facilitates the use of the mouth retractor. Care should be given to securing the tube and protecting it from unintentional extubation.
For patients having palate surgery, keeping the mean arterial pressure at 50-60 mm Hg may prevent excessive bleeding from the surgical site. This can be achieved by deepening the anesthetic with inhalation agents, opioids (remifentanil), or α2-agonists (clonidine or dexmedetomidine).
Throat packs can result in airway obstruction if they are unintentionally left in place, and confirmation of their removal must take place before extubation.
The adjunct use of regional anesthesia provides analgesia that is better than that provided by incisional infiltration or opioids alone. The bilateral infraorbital nerve block may be used as an adjunct or as the sole analgesic technique for cleft lip repair. The infraorbital nerve is a sensory nerve that is derived from the second maxillary division of the trigeminal nerve and exits from the infraorbital foramen to enter the pterygopalatine fossa. There are two approaches to the infraorbital nerve block: extraoral (percutaneous) and intraoral. For the extraoral approach, locate the infraorbital foramen and insert a 27-gauge needle toward, but not into, the foramen in the lateral direction. The intraoral approach is achieved by advancing a 27-gauge needle along the inner surface of the lip and cephalad to the infraorbital foramen parallel to the maxillary premolar. First palpate the infraorbital foramen and pull the upper lip superiorly to allow room for the needle and syringe. Keep a finger on the infraorbital foramen during the needle advancement to provide accurate measurement of the desired area. A total volume of 0.5-1.5 mL of bupivacaine 0.25%, levobupivacaine 0.25%, or ropivacaine 0.2% with 1:200,000 epinephrine is injected after negative aspiration for blood.
The anterior branch of the greater palatine nerve may also be blocked for cleft palate repair. Using a 27-gauge needle, insert the needle approximately 1 cm from the first and second maxillary molars on the hard palate. Palpate with the needle to find the greater palatine foramen, whose depth is usually less than 10 mm. A total volume of 0.3-0.5 mL of local anesthesia is injected after negative aspiration for blood.
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