Cleft Lip and Palate

What the Anesthesiologist Should Know before the Operative Procedure

Cleft lip and cleft palate, either alone or in combination, are among the most common congenital anomalies, occurring in approximately 1 in 500 births. Orofacial clefts can lead to significant physiologic disturbance, disrupting the mechanisms of swallowing, breathing, hearing and speech. Feeding is difficult affected neonates because the aberrant communication between oral and nasal cavities prevent the generation of negative pressure required for suckling. The lack of nasal septation leads to the nonphysiologic mixing of food and air in the nasopharynx, putting affected children at risk for aspiration. Abnormal anatomy in the nasopharynx also leads to eustachian tube dysfunction, which can result in chronic middle ear effusion and conductive hearing loss. Secondary defects of velopharyngeal function can occur, resulting in high nasal speech and pronunciation difficulty.

The physiologic consequences of non-repair of orofacial cleft are significant, but surgical repair must be postponed long enough to allow for adequate growth of the infant, and a complete workup for comorbidities. Orofacial clefts are associated with 300 known syndromes, including several that may lead to difficult airway. Clefts can also be associated with nonsydrome abnormalities like clubfoot or other limb and/or ear deformities. Preoperative evaluation by a geneticist is recommended.

1. What is the urgency of the surgery?

What is the risk of delay in order to obtain additional preoperative information?

Repair of cleft lip and cleft palate is performed on an elective basis. The goal is to complete the surgery early enough to prevent disruption of speech and hearing, but late enough that growth of midfacial tissue is not compromised. The typical sequence is cleft lip repair at 2-3 months of age, and cleft palate repair at 6-12 months of age.

2. Preoperative evaluation

Preoperative evaluation for orofacial cleft repair must include investigation for other associated abnormalities. Some of the most common syndromes associated with cleft lip and cleft palate include velocardiofacial syndrome, Goldenhar syndrome, Treacher Collins syndrome, and trisomy 21. Cleft palate can also be found in the Pierre-Robin sequence, as micrognathia causes superior displacement of the tongue during fetal development, preventing palatal fusion. Pre-anesthetic evaluation should include a thorough history, as well as cardiac and airway examination. A hemoglobin level should also be obtained.

Medically unstable conditions warranting further evaluation include congenital cardiac defects and airway obstruction. Delaying surgery may be indicated if: physical exam or history is concerning for undiagnosed associated abnormalities, or if child is underweight or anemic.

3. What are the implications of co-existing disease on perioperative care?

b. Cardiovascular system:

Congenital heart disease

Orofacial clefts are associated with a wide variety of cardiac defects, including: ventricular and atrial septal defects, arteriovenous canal abnormalities, tetralogy of Fallot, patent ductus arteriosus, and coarctation of the aorta.

Perioperative evaluation

A complete evaluation begins with a thorough history looking for cyanosis, diaphoresis with feeds, tachypnea, or syncope. Physical examination should include auscultation for a murmur and palpation of peripheral pulses. Any concerns should be further investigated by electrocardiogram and echocardiogram.

Perioperative risk reduction strategies

Depending on the cardiac defect, congenital heart disease can lead to desaturation, hypotension, arrhythmia, congestive heart failure, or sudden death. A thorough understanding of the patient’s particular diagnosis is required. If congenital heart disease is severe, cleft surgery should be postponed until after cardiac repair. If proceeding with cleft surgery, consideration should be given to bacterial endocarditis prophylaxis. Care should be taken to avoid introduction of venous air in a child with a shunting lesion.

c. Pulmonary:

Aspiration

Children with cleft palate may be prone to chronic aspiration due to the non-physiologic mixing of food and air in the nasopharynx. An active or recent pneumonia may necessitate delay of surgery.

Perioperative evaluation

Preoperative history should rule out cyanosis, tachypnea, cough, or other signs of lower respiratory tract infection. Examination should include thorough auscultation of the lungs and measurement of room air oxygen saturation and temperature.

Perioperative risk reduction strategies

If pneumonia is suspected or confirmed, it may be prudent to postpone airway surgery until 4-6 weeks after recovery.

However, in a child with chronic aspiration, a disease-free interval may not exist.

Recent upper respiratory tract infection (URI)

The average child has a URI every 2 months. Airway surgery in the setting of current or recent URI will increase the risk of postoperative pulmonary complications, including: prolonged intubation and/or prolonged admission, coughing, breath-holding, desaturation, atelectasis, croup, bronchospasm, and laryngospasm.

Perioperative evaluation

Preoperative history should focus on report of cough, runny nose, fever, lethargy, or decreased appetite. The time course of symptoms must be elucidated. Physical examination should include auscultation of the lungs and inspection for nasal secretions.

Perioperative risk reduction strategies

In the setting of current or recent URI, the most conservative approach would be to postpone airway surgery until 4-6 weeks after recovery. If proceeding with surgery in a symptom-free child who is less than 4 weeks out from a URI, the anesthesiologist should be prepared for a potential increase in pulmonary complications.

Reactive airway disease (asthma)

Reactive airway disease is a common pediatric concern, and the incidence may be increased in a child with chronic aspiration from cleft palate. Anesthesia for asthmatic children is particularly high risk in the setting of recent URI, so this combination of comorbidities may require postponement of surgery.

Perioperative evaluation

A thorough asthma history includes frequency and severity of attacks, inciting factors, and history of intubation or emergency room visits. Preoperative use of bronchodilators or steroids should be determined.

Perioperative risk reduction strategies

Children with asthma should undergo medical optimization before presenting for surgery. A preoperative albuterol nebulizer treatment should also be considered. Orofacial cleft surgery requires endotracheal intubation. Thus, it is important to ensure adequate depth of anesthesia before intubation to avoid bronchospasm. Awake extubation may be preferred in an infant with bloody secretions after airway surgery, but in the asthmatic, this approach may increase the risk of bronchospasm on emergence. Inhaled albuterol or intravenous lidocaine may be helpful in preparation for extubation.

d. Renal-GI:

Genitourinary or gastrointestinal anomalies may be present as part of a genetic syndrome. If suspected, appropriate radiographic studies should be obtained preoperatively for diagnosis.

Preoperative fasting guidelines are the same as for most other pediatric surgeries – 2 hours for clear liquids, 4 hours for breast milk, and 6 hours for formula.

HEMATOLOGIC

Anemia

The timing of cleft lip repair often coincides with an infant’s physiologic nadir of hemoglobin. Many surgeons will choose to delay cleft repair until the hemoglobin level is greater than 10 g/dL.

Perioperative evaluation: Hematologic history and exam should evaluate for pallor, tachypnea, or tachycardia. Laboratory determination of hemoglobin should be obtained before orofacial cleft surgery.

Perioperative risk reduction strategies: A preoperative hemoglobin level of 10 g/dL is preferred before proceeding with surgery. Allowable blood loss should be calculated, and a unit of cross-matched blood should be available for cleft palate surgery.

AIRWAY

Difficult intubation

Cleft lip and palate are associated with several difficult airway syndromes, all of which require thorough preoperative airway evaluation. Even isolated cleft palate can increase the difficulty of airway manipulation if the laryngoscope blade becomes lodged in the cleft. Bilateral cleft lip, combined cleft lip and cleft palate, retrognathia, and age <6 months have all been associated with poor glottic visualization.

Perioperative evaluation: Any history of airway obstruction should be ruled out, asking about choking, gasping, or cyanotic episodes. Airway examination should include inspection of the oral cavity, determination of mandibular size, and confirmation of full range of neck motion. Preoperative radiographs of the mandible may be helpful to determine the severity of retrognathia or micrognathia.

Perioperative risk reduction strategies: Children with truly critical airways may present for cleft repair after tracheostomy. Those who are able to maintain their airways at baseline but obstruct during general anesthesia are the most imminent threat to the anesthesiologist. If difficult mask ventilation and intubation are anticipated, awake intubation may be considered. The more common scenario is the child with easy mask ventilation and difficult intubation; various fiberoptic or blind intubation techniques may be considered here.

e. Neurologic:

N/A

f. Endocrine:

N/A

g. Additional systems/conditions which may be of concern in a patient undergoing this procedure and are relevant for the anesthetic plan (e.g., musculoskeletal in orthopedic procedures, hematologic in a cancer patient)

4. What are the patient’s medications and how should they be managed in the perioperative period?

Chronic medications are uncommon in children with isolated orofacial clefts. If the child is taking a medicine for a comorbidity like asthma or reflux, the need for this medicine on the day of surgery should be assessed on a case-by-case basis. Ideally, any oral medications would be administered at least 2 hours before the time of surgery to preserve NPO status.

h. Are there medications commonly seen in patients undergoing this procedure and for which should there be greater concern?

None.

i. What should be recommended with regard to continuation of medications taken chronically?

See above.

j. How to modify care for patients with known allergies

Medication allergies are less common in this age group, likely due to limited drug exposure. Any known allergens should be avoided.

k. Latex allergy – If the patient has a sensitivity to latex (e.g., rash from gloves, underwear, etc.) versus anaphylactic reaction, prepare the operating room with latex-free products.

If the patient has a known sensitivity or allergy to latex, prepare the operating room with latex-free products.

l. Does the patient have any antibiotic allergies – Common antibiotic allergies and alternative antibiotics

There are currently no established guidelines about the use of prophylactic antibiotics for primary cleft repair, so antibiotic use varies widely by surgeon and center. Ampicillin/sulbactam and cefazolin are among the most common choices. For a penicillin-allergic patient, vancomycin or clindamycin are reasonable alternatives.

m. Does the patient have a history of allergy to anesthesia?

Malignant hyperthermia

Documented

Avoid all trigger agents such as succinylcholine and inhalational agents. To prepare the operating room, first remove or disable vaporizers, then replace the circuit, reservoir bag, and CO2 absorbent on the anesthetic machine. Flush the machine and ventilator with at least 10 liters/min fresh gas flow for 10 minutes.

Proposed general anesthetic plan: To avoid volatile agents, induction must be accomplished by intravenous or intramuscular injection of drugs. General anesthesia is then maintained by total intravenous anesthesia (TIVA). Safe agents include propofol, ketamine, dexmedetomidine, narcotics, and non-depolarizing muscle relaxants. Appropriate monitoring must include pulse oximetry, capnometry, electrocardiogram, blood pressure, and temperature.

Ensure MH cart available: All supplies to treat an MH episode must be immediately available. The MH cart should include dantrolene, sterile water (for mixing dantrolene), sodium bicarbonate, mannitol, furosemide, insulin, 50% dextrose, calcium chloride, and antiarrhythmics. Cooling aids should also be available, including hypothermia blankets and/or crushed ice.

Family history or risk factors for MH

If the patient is at risk for MH based on family history, but has not undergone halothane-caffeine contracture testing, he or she should be treated the same as a patient with confirmed MH. Uneventful exposure to triggering agents in the past does not negate the possibility of an MH diagnosis.

Local anesthetics/ muscle relaxants

Perioperative anaphylaxis occurs in approximately 1:10,000 to 1:20,000 anesthetics. Several studies have shown that muscle relaxants are the most common trigger, followed by antibiotics. All paralytics are capable of causing anaphylaxis, even in the absence of prior exposure. More rare causes of intraoperative anaphylaxis include hypnotic agents (propofol, thiopental), opioids, amide-type local anesthetics, and certain colloid solutions. If a history of allergy is known, the offending agent should be avoided. Consideration should also be given to potential cross-reactivity within drug classes.

5. What laboratory tests should be obtained and has everything been reviewed?

For cleft surgery, preoperative determination of hemoglobin level is indicated. There is no evidence to support additional laboratory workup in otherwise healthy children.

The normal hemoglobin level for a term neonate is approximately 16-18 g/dL, and the level will decrease over the next 6-8 weeks until the infant reaches a physiologic nadir of 9-11 g/dL hemoglobin. Hemoglobin production will then increase to adult levels.

Hemoglobin levels: Should be obtained routinely due to physiologic anemia in this age group, and potential for blood loss during surgery.

Electrolytes: Not routinely obtained.

Coagulation panel: Not routinely obtained.

Imaging: Echocardiogram, renal ultrasound, or other imaging may be indicated if a genetic syndrome is suspected.

Other tests: Not routinely obtained.

Intraoperative Management: What are the options for anesthetic management and how to determine the best technique?

Like many pediatric surgeries, cleft surgery is usually performed under general anesthesia to ensure the child’s comfort and provide a quiet operating field for the surgeon. Regional anesthesia is a useful adjunct for post-operative analgesia. With an older and cooperative child, cleft lip surgery can also be performed solely under local/regional anesthesia.

Regional anesthesia

Regional anesthesia is most commonly employed in addition to general anesthesia in orofacial cleft surgery, providing post-operative analgesia.

For cleft lip surgery, infraorbital nerve blocks are used, usually as an adjunct to general anesthesia, and rarely as a total anesthetic. The infraorbital nerve is a terminal branch of the maxillary division of the trigeminal nerve (V2). It supplies innervation to the lower eyelid, side of the nose, upper lip, and a portion of the cheek. Blocking this nerve bilaterally can provide excellent anesthesia in a V-shape between the lower eyelids and upper lip. Transcutaneous and transoral approaches to this nerve block have been described. Typically 1-2 mL of bupivicaine or lidocaine is deposited on each side.

For cleft palate surgery, nasopalatine and palatine nerve blocks can augment post-operative analgesia. The nasopalatine nerve is a nasal branch of the pterygopalatine nerve, which arises from V2. The nasopalatine nerve supplies innervation to parts of the nasal cavity and to the maxillary anterior palate. Block of this nerve is typically performed by the surgeon, who injects local in the incisive foramen, located just behind the upper incisors.

The greater palatine nerve and lesser palatine nerve are palatine branches of the pterygopalatine nerve. They supply innervation to the posterior hard palate and to the soft palate, respectively. The greater palatine nerve can be blocked, often by the surgeon, by depositing 0.5 cc of local in the greater palatine foramen. This foramen is located medial to the maxillary molars.

Benefits: The benefits of performing peripheral nerve blocks to augment a general anesthetic technique include superior postoperative analgesia, reduction of general anesthetic requirements, and decrease in postoperative narcotic use.

Drawbacks: The risks are minimal, but include bleeding/bruising, infection, and injury to nerves or surrounding structures. If performing a transoral infraorbital nerve block, one finger should be kept on the infraorbital foramen to prevent the needle from entering the eye.

Other issues: Local anesthetic, even if absorbed intravascularly, is well below toxic levels for all these blocks.

General Anesthesia

General anesthesia is the technique of choice for most orofacial cleft surgeries, although it may be supplemented by field blocks and/or peripheral nerve blocks performed after induction.

After appropriate monitors are attached, the child typically undergoes inhalation induction, followed by IV placement and intubation with an oral rae tube. Muscle relaxation may be used to facilitate intubation. Laryngoscopy can be complicated by the blade becoming lodged in the cleft palate. If this occurs, the blade should be removed and the cleft can be filled with gauze temporarily to prevent recurrence. Once the tube is secured and the patient is positioned in neck extension, the surgeon will place a pharyngeal pack. The adequacy of ventilation should be confirmed before proceeding.

Benefits: Performing cleft surgery under general anesthesia maximizes patient comfort and minimizes movement during surgery.

Drawbacks: The risks of general anesthesia for cleft repair include inadvertent extubation, endotracheal tube obstruction, aspiration, and postoperative airway obstruction.

Other issues: The surgeon will typically inject lidocaine with epinephrine in the field to reduce bleeding. Care should be taken to avoid toxic doses (5 mg/kg lidocaine, 10 mcg/kg epinephrine). The patient will be particularly prone to arrhythmia complications if a halothane anesthetic is used.

Airway concerns: A number of different factors can contribute to postoperative airway obstruction, including: edema from tissue trauma, bleeding, tongue swelling due to mouth gag use, new oropharyngeal crowding due to palatal closure, and residual anesthetic effects. If post-extubation airway obstruction is severe, the anesthesiologist should double check that the throat pack was removed. For cleft palate repairs, the surgeon will commonly place a tongue stitch (a suture placed through the tongue for postoperative use). Gentle traction on this tongue stitch can relieve airway obstruction in the postoperative period.

Monitored anesthesia care

Monitored anesthesia care with nerve block or field block is an option for older children undergoing cleft lip repair or revision. It is not an appropriate technique for cleft palate repair.

Benefits: Eliminates general anesthetic exposure and reduces side effects

Drawbacks: If block is inadequate, may contribute to patient discomfort

Other Issues: Unprotected airway puts patient at risk for aspiration of bloody secretion

6. What is the author’s preferred method of anesthesia technique and why?

General anesthesia supplemented by nerve block provides the best combination of intraoperative patient comfort, airway protection, and postoperative analgesia.

What prophylactic antibiotics should be administered?

There are currently no established guidelines about the use of prophylactic antibiotics for primary cleft repair, so antibiotic use varies widely by surgeon and center. Ampicillin/sulbactam and cefazolin are among the most common choices. For a penicillin-allergic patient, vancomycin or clindamycin are reasonable alternatives. According to SCIP recommendations from 2010, if antibiotics are given, the first dose should be received within 1 hour prior to incision, and antibiotics should be discontinued within 24 hours of surgery.

What do I need to know about the surgical technique to optimize my anesthetic care?

Cleft palate surgery requires hyperextension of the neck, so tube position should be checked in neutral and extended positions. Some bleeding is to be expected, rarely requiring transfusion, so the starting hematocrit level should be determined. Several factors contribute to possible post-operative airway obstruction, and the relative merits of awake vs. deep extubation must be weighed against the risk of aspiration or airway compromise.

What can I do intraoperatively to assist the surgeon and optimize patient care?

If bleeding is excessive, controlled hypotension may reduce blood loss. Common techniques for controlled hypotension include nitroprusside, beta blockers, and deepening of the anesthetic. A remifentanil infusion can be helpful as it will not have prolonged effect after discontinuation.

What are the most common intraoperative complications and how can they be avoided/treated?

• Failure to establish or maintain an adequate airway is the most pressing concern in shared airway surgery. Intubation can be difficult, and even after placement, the endotracheal tube can easily become plugged, kinked or dislodged. Failure to ventilate and protect the airway can result in hypoxemia, hypercapnia, arrhythmia or cardiac arrest. To prevent complications, the tube should be meticulously secured, and the adequacy of ventilation should be continuously monitored.
• Bleeding is a common complication of oral surgery. The severity can be reduced by infiltration of the oral cavity with epinephrine-containing local anesthetic. Controlled hypotension can also be helpful if bleeding is severe. Allowable blood loss should be calculated at the beginning of the case, and the patient should be transfused if necessary.
• Postoperative airway obstruction occurs with relatively high frequency. Edema, bleeding, tongue swelling, and residual anesthetic effects can all contribute to the likelihood of post-extubation obstruction. Laryngospasm is common in the pediatric population, and may be precipitated by contact between bloody secretions and the vocal cords. If obstruction occurs, it should be treated with continuous positive airway pressure, secretion management, and potentially reintubation.
• Aspiration of bloody secretions can occur during or after surgery, even with an appropriately sized endotracheal tube in place during surgery. Aspiration after endotracheal tube removal can potentially be avoided by awaiting the full return of airway reflexes before extubation. If aspiration occurs, care is supportive, with supplemental oxygen administration as needed.

Complications

Cardiac: Arrhythmia can occur in the setting of hypoxemia, hypercapnia, local anesthetic toxicity, or epinephrine overdose (particularly with halothane use).

Pulmonary: Loss of airway, airway obstruction, and aspiration may occur

Neurologic: Neurologic complications are unusual with cleft surgery

a. Neurologic:

N/A

b. If the patient is intubated, are there any special criteria for extubation?

Several factors contribute to possible post-operative airway obstruction or aspiration, so awake extubation is commonly performed. Criteria for extubation include adequate gas exchange and minute ventilation, the presence of protective airway reflexes, and reversal of muscle relaxation.

c. Postoperative management

What analgesic modalities can I implement?

Both opioid and non-opioid analgesics can be used postoperatively to supplement the analgesia provided by intraoperative regional techniques. Acetaminophen 10-15 mg/kg PO, ibuprofen 10-20 mg/kg PO, and ketorolac 0.5 mg/kg IV are commonly used non-narcotic analgesics. NSAIDs may be avoided if hemostasis is in question. Commonly used narcotic analgesics include morphine 0.05-0.1 mg/kg IV, fentanyl 0.5-1 mcg/kg IV, or various opioid/acetaminophen oral preparations.

What level bed acuity is appropriate?

A floor bed on the general ward is usually appropriate, unless the presence of aspiration pneumonitis or airway obstruction necessitates escalation of care.

What are common postoperative complications, and ways to prevent and treat them?

Postoperative complications can include re-bleeding, infection, and poor wound healing. Airway obstruction can occur after PACU discharge in response to oversedation or continued tissue swelling. If there is concern for these complications, continuous pulse oximetry monitoring may be considered.

What’s the Evidence?

Arosarena, OA. “Cleft lip and palate”. Otolaryngol Clin North Am. vol. 40. 2007. pp. 27-60. (A comprehensive review of the topic, including preoperative assessment, surgical techniques, and postoperative complications.)

Mossey, PA. “Cleft lip and palate”. Lancet. vol. 374. 2009. pp. 1773-85. (A comprehensive review of the epidemiology, pathogenesis, and clinical management of cleft lip and palate.)

Gunawardana, RH. “Difficult laryngoscopy in cleft lip and palate surgery”. Br J Anaesth. vol. 76. 1996. pp. 757-9. (Prospective study investigating the incidence and predictors of difficult laryngoscopic view in cleft surgery.)

Nargozian, C. “The airway in patients with craniofacial abnormalities”. Paediatr Anesth. vol. 14. 2004. pp. 53-9. (Review of how various craniofacial abnormalities and syndromes affect airway management, including cleft lip/palate, Treacher Collins, Goldenhar’s, and Trisomy 21.)

Xue, FS. “The clinical observation of difficult laryngoscopy and difficult intubation in infants with cleft lip and palate”. Paediatr Anaesth. vol. 16. 2006. pp. 283-9. (Identifies risk factors for difficult laryngoscopy and intubation, including age, type of cleft deformity, and micrognathia.)

Merritt, L. “Part 2. Physical assessment of the infant with cleft lip and palate”. Adv Neonatal Care. vol. 5. 2005. pp. 125-34. (Provides suggestions for physical assessment of an infant with cleft deformity, including findings that could suggest associated syndromes.)

Salloum, ML. “Combined use of infraorbital and external nasal nerve blocks for effective perioperative pain control during and after cleft lip repair”. Cleft Palate Craniofac. vol. 46. 2009. pp. 629-35. (Description of use of infraorbital nerve blocks for cleft lip repair, as either primary or adjunctive anesthetic technique.).

Smyth, AG, Knepil, GJ. “Prophylactic antibiotics and surgery for primary clefts”. Br J Oral Maxillofac Surg.. vol. 46. 2008. pp. 107-9. (A survey conducted in the United Kingdom shows that there is no consensus among cleft surgeons about antibiotic prophylaxis for primary cleft repair.)

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