Cleft Palate Surgery – Procedures

What the Anesthesiologist Should Know before the Operative Procedure

Cleft palate is the fourth most common birth defect. It can occur as an isolated defect or as part of a syndrome along with other congenital anomalies (Pierre Robin syndrome, Goldenhar syndrome). Isolated cleft palate occurs in 1/2500 births. Its incidence is highest in Native Americans (3.74/1000) and lowest in Africans (0.18/1000). Cleft palate occurs more in girls, whereas cleft lip with or without cleft palate is seen more in boys. The causes for a cleft palate include genetic and environmental factors. Drugs taken during pregnancy as well as exposure to toxins or viral agents have been associated with cleft palates. Cleft palate may be complete (involving both soft and hard palates) or incomplete as well as unilateral or bilateral.

1. What is the urgency of the surgery?

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

Cleft palate, depending on the severity of the defect and its association with cleft lip, could result in the following conditions:

• feeding problems

• failure to thrive

• recurrent ear infections

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• malalignment of teeth

• speech impediments

• airway/respiratory problems

The timing of surgery is surgeon dependent but the goal of surgery is to correct the defect before the development of speech (1 year) occurs. Temporary closure of the defect is carried out by using a palatal obturator in order to assist feeding and promote growth. The Latham appliance is a device inserted surgically with pins at 4 to 5 months of age, and the parents are taught to turn the screw daily so as to narrow the defect. In general, cleft lip repair is usually performed at 3 months of age and the cleft palate is corrected at 7 to 18 months of age. There are no data to support repair of palate earlier than 7 months. Cleft palate and cleft lip repair are elective procedures.

2. Preoperative evaluation

Cleft palate may be part of a syndrome and, if so, is associated with other congenital anomalies. Children with cleft palate have feeding problems and so are prone to chronic aspiration and pulmonary compromise. Their growth may lag behind that of other children.

It is important to ascertain the following in the history:

• Birth: Prematurity, any airway intervention required, other congenital anomalies

• Airway issues: Presence of micrognathia, glossoptosis, and cleft palate (Pierre Robin sequence) can cause profound airway obstruction. In most cases, patency can be obtained by placing the baby prone. In some cases temporary relief is obtained by using a nasopharyngeal airway or by stitching the tongue to the lower lip (tongue-lip adhesion). In severe cases, intubation, mandibular distraction or tracheostomy is required. Retrospective analysis showed the incidence of airway compromise in isolated cleft palate with no medical issues to be 18%.

• Feeding issues: Prone for aspiration due to feeding problems. To reduce the risks of aspiration, the infant is fed in an upright position using special nipples (premie nipple, Habermann nipple), which requires minimal suction for the fluid to flow.

• Respiratory compromise: History of pneumonia and wheezing secondary to aspiration. Recent upper respiratory infections.

• Cardiac issues: History of blue spells, tachypnea, fatigue during feeding (although may be difficult to distinguish clinically from that caused by the cleft palate itself). There is an association of cardio-velo abnormalities.

• Neurologic issues: Seizures, hypotonia.

• Family history of any issues related to anesthesia

Cleft palate is an elective procedure. If patient has an acute respiratory infection it should be treated prior to surgery. Some have chronic sinus issues and surgery need not be postponed in these patients. Major cardiac issues should be resolved prior to surgery.

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

Coexisting diseases that have implications for perioperative care include:

• Congenital cardiac disease

• Respiratory compromise

• Difficult airway

• Failure to thrive, nutritional deficiencies

b. Cardiovascular system:

Congenital heart disease, if present, should be fully evaluated. In general cardiac disease should be addressed prior to cleft palate repair, but the cardiology and plastic surgery teams should work together to make an appropriate clinical plan for each patient depending on the specific lesion.

c. Pulmonary:

Chronic aspiration may predispose to pneumonia or reactive airway disease. Ideally elective surgery would be scheduled at least 4 weeks after an acute respiratory infection, but it may be impossible to have these children completely clear. Underlying respiratory disease may increase postoperative oxygen needs or predispose to bronchospasm in the perioperative period.

d. Renal-GI:

N/A

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)

Feeding difficulties may result in failure to thrive and nutritional deficiencies. Nutritional status should be optimized as much as possible before surgery.

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

Most patients are not on any medications expect vitamins. If the child is on H-2 blockers or inhaled beta-agonists, they should be given preoperatively.

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

N/A

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

N/A

j. How to modify care for patients with known allergies

Infants may be sensitive to some of the tapes used, so avoid them and use paper tapes.

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.

Infants with spina bifida or bladder exstrophy are more prone to latex allergy.

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

The common antibiotic used during cleft palate surgery is cefazolin (25-30 mg/kg IV). If an allergy to penicillin or cephalosporin is noted, clindamycin can be used (5-10 mg/kg IV).

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

Malignant hyperthermia (MH)

Documented: Avoid all trigger agents such as succinylcholine and inhalational agents. Follow a proposed general anesthetic plan: total intravenous anesthesia with propofol ± opioid infusion ± nitrous oxide. Ensure that an MH cart is available [MH protocol].

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

Cleft palate, depending on the extent of surgery, could result in significant blood loss. Prior to surgery:

• Complete blood count and coagulation studies should be obtained.

• Chest radiograph if indicated -respiratory issues.

• Echocardiography/electrocardiogram if there are any cardiac issues.

Normal range for 6 to 18 months of age:

• Hemoglobin 10.5 to 14 g/dL

• Platelets 150 to 450 × 10⁹ /L

• PT 10.7 to 13.1 seconds

• aPTT 22 to 34 seconds

The reference ranges vary depending on the laboratory.

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

Cleft palate repair is usually done under general anesthesia. Premedication is avoided in patients with airway issues. If premedication is required, the patient should be monitored after it is given. Midazolam (0.5 mg per kg) orally/rectally has good efficacy with an onset of approximately 20 minutes, and a good margin of safety.

Position for the surgery is supine with extension of the neck. A roll may be placed under the shoulder to achieve this and the head is at the end of the table. The extreme extension can result in accidental extubation. The operating table is turned 90 degrees.

Regional anesthesia

The greater palatine nerve, a sensory branch of the maxillary division of the trigeminal nerve, is responsible for the sensory supply to the hard and soft palate, the lower eyelid, the upper lip, and the skin between them and the roof of the mouth. The greater palatine nerve emerges through the greater palatine foramen and the anterior branch lies in a grove parallel to the molar teeth.

With the mouth open, the greater palatine foramen can be located close to the second molar tooth. A 27-gauge needle is inserted medially and anterior, and 1 mL of 0.25% bupivacaine with 1:200,000 epinephrine is injected after negative aspiration. The block is performed on both sides.

In infants who have no molar teeth, bilateral maxillary block can be performed after induction using the suprazygomatic approach. A 25-gauge, 50-mm needle is used .The needle entry point is at an angle formed by the superior edge of the zygomatic arch below and the posterior orbital rim forward, with the head in a neutral position. The needle is inserted perpendicular to the skin and advanced to reach the greater wing of sphenoid at an approximately 20-mm depth. The needle is then reoriented 20 degrees anterior and 10 degrees caudal toward the philtrum and advanced 35 to 45 mm deep to the pterygopalatine fossa. After negative aspiration, 0.15 mL/kg of 0.2% ropivacaine or 0.25% bupivacaine is injected on each side.

The blocks can be used for postoperative analgesia.

Benefits

• Postoperative analgesia and comfort.

• Decreases the opioid requirements and so may reduce risk of respiratory depression.

• Early resumption of feeding by mouth.

• Simple and safe to perform.

Drawbacks

• Requires sedation or general anesthesia to perform the block in the uncooperative patient.

• Possibility of intravascular injection.

General anesthesia

Induction of anesthesia can be achieved by inhalational technique using a face mask or by intravenous technique if an IV line is in place. Spontaneous respiration should be maintained until the ability to ventilate the patient is confirmed. Airway obstruction during induction can be overcome by head repositioning, use of nasopharyngeal or oral airways or an LMA. Lifting the angle of the jaw toward the ceiling, ensuring that the operator’s hand is not compressing the soft tissue under the chin, and ensuring that the tongue stays off the roof of the mouth are the main techniques used to prevent airway obstruction in infants. After obtaining intravenous access and assessing ability to ventilate, neuromuscular blocking agents can be administered to facilitate intubation. Frequently “stiff-arm” restraints are placed at the end of surgery and should be considered in selecting an IV site.

Laryngoscopy is said to be difficult in patients with bilateral cleft lip and cleft palate defect (16.46%), micrognathia (50%), and infants younger than 6 months. Conventional midline laryngoscopic approach has a tendency to cause the laryngoscopic blade to be caught in the prepalatal clefts (cleft alveolus and protruding premaxilla). Video laryngoscopes can help in intubation when conventional laryngoscopy is difficult. LMA’s can be used as a guide to intubation with or without a fiberoptic bronchoscope in cases of difficult airway.

A paraglossal approach (right/left) performed using a straight blade reduces the distance from alveolus to laryngeal inlet, avoids the premaxilla from obstructing the view of the glottis, and prevents iatrogenic trauma to the deformed midline tissues. In this approach, the blade is inserted through the corner of the mouth along the pharyngeal gutter (groove between tongue and tonsil), thereby lifting the epiglottis.

External laryngeal manipulation may be required if there is difficulty in obtaining an optimum view. If the tongue is sutured to the lip or mandible, it must be released prior to laryngoscopy, otherwise the tongue is difficult to push and obstructs the view. Fiberoptic bronchoscopy should be available for any difficult intubation.

An oral RAE (Ring, Adair, Ellwyn) endotracheal tube is commonly used for cleft palate repair . This preformed tube can be straightened at the curve for intubation with a stylette, and then secured to center of the chin with benzoin and tape or in older children can be sutured to the mandibular central incisor. An alternate tube is a reinforced endotracheal tube so as to prevent kinking of the tube. The endotracheal tube used should be fixed at the appropriate length to prevent extubation, with the extension of the neck and endobronchial intubation and with the pressure exerted by the mouth gag and throat pack that is being used.

Maintenance of anesthesia can be with inhalational or intravenous agents supplemented with modest doses of opioid. Intraoperative use of a remifentanil or sufentanil infusion would reduce the intraoperative use morphine. Low dose ketamine infusion (1-2 mcg/kg/min) can also reduce the intraoperative narcotics used. Intravenous use of acetaminophen and NSAID (ketorolac) are the other analgesics that can be used so as to reduce amount of opioids used. Neuromuscular blockers may be used to minimize risk of bucking on the mouth gag. The duration of surgery is usually 60 to 120 minutes.

Fluids are administered to replace any deficit and provide maintenance plus losses. Blood loss rarely indicates a transfusion. Temperature homeostasis is maintained using forced air warming with temperature monitoring. Hyperthermia is not unusual when the patient is covered and the head is under lights, so the air warming device can also be used to lower the temperature if needed.

A throat pack is frequently placed to absorb any blood. A Dingman gag is used to keep the mouth open. The gag has a groove for the endotracheal tube so that it is not compressed. Bilateral air entry should be confirmed on chest auscultation and airway pressures should be noted both before and after placement of the gag and throat pack. Release of the gag every 30 mins may reduce the tongue swelling postoperatively. Epinephrine (1:200,000)-containing solution/local anesthetics may be used for hemostasis and the dose monitored.

Anesthesia monitoring consists of oximetry, electrocardiogram, non-invasive blood pressure, end-tidal CO2, rectal temperature, and ventilatory parameters. Blood loss should be accessed hourly. At the end of surgery, the throat pack is removed and surgeon should suction the stomach and the pharynx to remove any blood or secretions. If muscle relaxants are used, they have to be reversed with neostigmine (0.07 mg/kg) or edrophonium (1 mg/kg) and glycopyrrolate (0.01 mg/kg) or atropine (0.02 mg/kg).

The repair of the cleft reduces the pharyngeal space and airway problems on emergence must be anticipated. A nasopharyngeal airway can be inserted by the surgeon at the end of surgery which facilitates suction and airway patency. A tongue stitch is used in some cases to prevent the tongue from causing obstruction. The table is turned back to its original position at the end of surgery so has to have good access to the airway. Patients should be extubated only when fully awake and protective reflexes are present. Extubation can be carried out in the lateral position. Suctioning is done by insertion of catheter between the dependent cheek and gums so as to avoid injury to the surgical repair. The position also avoids the tongue falling back. The patient is extubated once spontaneous respiration and reflexes have returned.

Benefits

• Cooperation of child not required during surgery

• Immobility during surgery

• Respiration can be controlled

Drawbacks

• Loss of airway during induction

• Drugs used to achieve anesthesia may cause respiratory depression

Airway issues: major problem in cleft palate repair

• Airway obstruction preoperatively

• Obstruction of airway during induction of anesthesia and/or obstruction during the postoperative period

• Difficult laryngoscopy and intubation (difficult airway)

• Accidental extubation and endobronchial intubation

• Laryngospasm, bronchospasm especially in the presence of chronic respiratory problems, secretions, and blood

• Edema of the tongue due to compression by Dingman gag and also of the surgical site.

• Subglottic edema

• Overlooked throat pack

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

Most patients undergoing cleft palate repair are infants, so general anesthesia with a secure airway is preferred. Discuss the patient and the procedure with the surgeon regarding any concerns (airway, bleeding, surgical technique) the team may have. If an intensive care bed is required (e.g., child with high potential for postoperative airway obstruction), make sure one is available prior to starting the operation. Inhalation induction is usually performed, but if an IV line is in place, it may be used. Use of muscle relaxation after confirming ability to ventilate generally improves intubating conditions. Oral RAE tube is most convenient. Maintenance is with inhalational agents and opioid. If a block is being placed, the opioid dose is reduced. The use of acetaminophen may reduce opioid requirement. The use of NSAIDs should be deferred until the postoperative period due to bleeding risk.

At the end of the operation, depending on the preoperative status of the airway, a tongue stitch may be placed to facilitate keeping the airway open. If this is to be done, adequate depth must be maintained for this step. After removal of throat pack and suctioning of the stomach and throat under direct vision by the surgeon, the patient is allowed to emerge from anesthesia. Further suctioning is done by placing the catheter tip in the space between the dependent cheek and gums so as to prevent injury to the surgical repair. Some prefer to place the patient in the lateral position prior to emergence to minimize risk of aspiration or obstruction of the airway by the tongue extent after extubation. Once spontaneous respiration has resumed, small doses of fentanyl or morphine may be titrated with the goal of having a calm child but without respiratory depression.

Extubation is done only when the child has good spontaneous efforts, protective reflexes and is awake. Use of any airway device postoperatively (oropharyngeal or nasopharyngeal) should be discussed with the surgeon to avoid damage to the repair and further blood in the airway.

Main complications

Airway: Obstruction of the airway and laryngospasm at the end of case:

There are several reasons that these obstructions may occur. Obstruction may occur initially because the palatal opening that the child has been accustomed to is now closed; there may also be swelling that can contribute to the obstruction. Bleeding and secretions can contribute to laryngospasm. Having a stable repair with a dry field, tongue stich to protrude the tongue (particularly in syndromic children), and a child who is awake but with adequate analgesia are the best methods to prevent these problems from occurring. The team should have a plan to address obstruction or laryngospasm should they occur. Airway issues can arise in the first 24 hours due to edema, so monitoring and close observation are required.

Bleeding: Blood loss is usually modest and rarely requires transfusion. The anesthesia team should be vigilant regarding blood loss in the field; if the throat pack becomes saturated with blood, blood can continue to run into the stomach, increasing the risk of postoperative emesis and also making it difficult to quantify the bleeding.

a. Neurologic:

Because this procedure is performed in relatively young children, parents may have concerns about neurocognitive effects of general anesthesia. Although animal studies raise concern about neuroapoptosis in very young rat pups, many of those studies involved very high doses and lengthy exposure, and the effect appeared to be lessened in the presence of a painful stimulus.

There is only preliminary and mainly retrospective epidemiologic data in humans, and some of the clinical cases involved pre-date modern anesthesia drugs and monitoring. Several of the retrospective studies do raise concern that multiple anesthetics before the age of 3 to 4 years might be associated with learning disabilities, but one twin study did not show a difference between exposed and un-exposed twins. Prospective human studies are beginning but will take several years to obtain conclusive results. Animal data showed adverse effect from nearly all anesthetic drugs with the exception of opioids. In the absence of further data, remifentanil with a lowered dose of volatile anesthetic may be a reasonable option when anesthesia is required in infants, as long as appropriate amounts of other opioids are administered for postoperative analgesia.

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

Extubation is done after clearing the airway and once the child is awake and has good spontaneous efforts and protective reflexes. After extubation, the child should be able to maintain his airway with no support.

c. Postoperative management

1. Pain management – combinations of modalities

a. Local blocks using a long-acting local anesthetic.

b. Nonopioid analgesics such as acetaminophen rectally (30-40 mg/kg) or orally (10-15 mg/kg). IV acetaminophen is now available and the initial dose is 15 mg/kg.

c. Titrated doses of opioids in the first 24 to 48 hours. The child should be monitored for airway issues while receiving narcotics.

2. Arm restraints to prevent flexion at the elbows prevent the child from inserting fingers into the mouth and disrupting the repair.

3. Monitoring the airway is required in all children who have undergone cleft palate repair. Depending on the severity of airway issues, they are monitored in an ICU/step-down setting at least for 24 hours postoperatively.

4. Common postoperative problems are airway and bleeding.

a. AIRWAY

• Use a tongue stitch or nasopharyngeal airway postoperatively if airway issues are anticipated.

• Place child in the lateral position to optimize the airway and prevent aspiration.

• Place the child in a tent to provide humidified oxygen if he or she does not tolerate a mask.

• Racemic epinephrine may be useful in reducing any swelling.

• Monitor using oximetry and apnea monitor. Close observation of the airway for signs of obstruction.

• Titrate narcotics and watch for airway issues.

• Keep emergency airway appliances close by.

b. BLEEDING

• Observe for more than usual bloody secretions from the mouth.

• Monitor vital signs and color. If any clinical concerns, monitor hemoglobin level.

c. CIRCULATION (HYDRATION)

• Continue intravenous fluids till child is taking adequate fluid by mouth.

• Replace blood loss.

What’s the Evidence?

Coté, CJ. “A practice of anesthesia for infants and children”. 2013. pp. 697-99.

Nargozian, C. “The airway in patients with craniofacial abnormalities”. Paediatr Anaesth. vol. 14. 2004. pp. 53-9.

Manna, F, Pensiero, S, Clarich, G. “Cleft lip and palate: current status from the literature and our experience”. J Craniofac Surg. vol. 20. 2009. pp. 1383-1387.

Campbell, A, Costello, BJ, Ruiz, RL. “Cleft lip and palate surgery: an update of clinical outcomes for primary repair”. Oral Maxillofac Surg Clin North Am. vol. 22. 2010. pp. 43-58.

Liau, JY, Sadove, AM, van Aalst, JA. “An evidence-based approach to cleft palate repair”. Plast Reconstr Surg. vol. 126. 2010. pp. 2216-2221. (Surgical timing, complications, techniques of repair.)

Xue, FS, Zhang, GH, Li, P. “The clinical observation of difficult laryngoscopy and difficult intubation in infants with cleft lip and palate”. Paediatr Anaesth. vol. 16. 2006. pp. 283-289.

Kohjitani, A, Iwase, Y, Sugiyama, K. “Sizes and depths of endotracheal tubes for cleft lip and palate children undergoing primary cheiloplasty and palatoplasty”. Paediatr Anaesth. vol. 18. 2008. pp. 845-851.

Sen, I, Kumar, S, Bhardwaj, N. “A left paraglossal approach for oral intubation in children scheduled for bilateral orofacial cleft reconstruction surgery: a prospective observational study”. Paediatr Anaesth. vol. 19. 2009. pp. 159-163.

Jonnavithula, N, Durga, P, Madduri, V. “Efficacy of palatal block for analgesia following palatoplasty in children with cleft palate”. Paediatr Anaesth. vol. 20. 2010. pp. 727-733.

Mesnil, M, Dadure, C, Captier, G. “A new approach for peri-operative analgesia of cleft palate repair in infants: the bilateral suprazygomatic maxillary nerve block”. Paediatr Anaesth. vol. 20. 2010. pp. 343-349.

Nicola, Somerville, Stephen, Fenlon. “Anesthesia for cleft lip and palate surgery”. Contin Educ Anaesth Crit Care Pain.. vol. 5. 2005. pp. 76 -79.

Chepla, KJ, Gosain, AK. “Evidence–Based Medicine: Cleft Palate”. Plast Reconstr Surg. vol. 132. 2013. pp. 1644-1648.

Reena, Bandyopadhyay, KH, Paul, A. “Post operative analgesia for cleft lip and palate repair in children”. Journal of Anesthesiology Clinical Pharmacology.. vol. 32. 2016. pp. 5-11.

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