Apert Syndrome

Apert syndrome, also referred to as acrocephalosyndactyly, occurs at a frequency of 1 in 160,000 live births (Cohen et al., 1992). Acrocephalosyndactyly, a defect involving the cranium and the extremities, occurs in Apert, Pfeiffer, Carpenter’s, and Saethre-Chotzen syndromes. The characteristic features of Apert syndrome include turribrachycephaly (high steep flat forehead and occiput), mid-face hypoplasia, and orbital hypertelorism (Fig. 25-2). Cleft palate occurs in approximately 30% of these patients. Choanal atresia and occasionally tracheal stenosis have been reported and can cause airway obstruction. Congenital cardiac disease is one of the more common associated visceral anomalies, occurring in approximately 10% of patients. Genitourinary anomalies (hydronephrosis, cryptorchidism) also occur in 10% of patients with Apert syndrome (Cohen and Kreiborg, 1993). Severe synostosis can result in increased ICP and, if uncorrected, developmental delay. Syndactyly of the hands and feet with the fusion of digits two to four can occur and can make intravenous (IV) access difficult. Kreiborg and coworkers (1992) have reported cervical spine fusion, and they have suggested that cervical spine films prior to anesthesia may help predict difficult intubation. Although infants and children with Apert syndrome are often difficult to intubate, many have been intubated uneventfully. In some cases, suboptimal laryngoscopic views secondary to abnormal anatomy may require flexible fiberoptic intubation. The laryngeal mask airway (LMA) may be a reasonable adjunct in those patients who are difficult to ventilate or intubate. However, to date there are no reported instances of their use in infants and children with Apert syndrome. The clinical features and anesthetic implications of Apert syndrome and the other acrocephalosyndactylies are outlined in Table 25-1. Unlike Apert syndrome, the other acrocephalosyndactylies are not typically associated with difficult airways. However, midface hypoplasia is common in these infants and may cause significant upper airway obstruction intraoperatively and postoperatively (Perkins, 1997).

FIGURE 25-2 Apert syndrome.

Pfeiffer Syndrome

Pfeiffer syndrome is another example of an acrocephalosyndactyly. The incidence of Pfeiffer syndrome is approximately 1 in 100,000 live births. This syndrome is characterized by bicoronal synostosis, proptosis, midface hypoplasia, and broad thumbs and great toes (Moore et al., 1995). Patients with Pfeiffer syndrome can also present with hydrocephalus, which may contribute to increased intracranial pressure. There are three types of Pfeiffer syndrome, and typically the clinical features, degree of airway obstruction, and mortality rates increase with types 2 and 3 (Moore et al., 1995).

Saethre-Chotzen and Carpenter’s Syndromes

The clinical features of Saethre-Chotzen include brachycephaly, facial asymmetry, low hairline, proptosis, beaked nose, large halluces (great toes), and pectus excavatum. Some patients may have renal anomalies, cryptorchism, developmental delay, and epilepsy. It can be difficult to differentiate it from the other acrocephalosyndactylies because there can be significant clinical variability (Nascimento et al., 2004). Carpenter’s syndrome is the most rare of the syndromic craniosynostosis, with only 45 reported cases since the mid 1990s. As with all of the syndromic craniosynostosis, patients with Carpenter’s syndrome have synostosis, midface hypoplasia, and musculoskeletal deformities. They may also have hypogonadism, developmental delay, and obesity (Idestrand et al., 2009).

Crouzon Syndrome

Crouzon syndrome, also known as craniofacial dysostosis, is one of the syndromic craniosynostoses. These infants present with craniosynostosis, proptosis, and midface hypoplasia but without visceral or extremity involvement (Fig. 25-3). As in other patients with midface hypoplasia, significant airway obstruction can occur and may require early tracheostomy (Sirotnak et al., 1995). Table 25-1 outlines the main clinical features and anesthetic issues as they relate to patients with Crouzon syndrome. During infancy, patients with Crouzon syndrome may come to the operating room for tracheostomy or cranial vault remodeling.

FIGURE 25-3 Crouzon syndrome.

Cranial Vault

Surgical correction for craniosynostosis can be performed with an open or an endoscopic procedure. The calvarial vault reconstruction typically involves both plastic surgery (by craniofacial surgeons) and neurosurgery. The surgical approach for an open cranial vault reconstruction is through a bicoronal incision (Fig. 25-4, A). A blocking stitch or clips may be applied to the skin flaps to minimize blood loss. The clips may be more effective in preventing bleeding, but some surgeons have expressed concern about the risk for ischemia of the underlying hair follicles (Ray Harschberger, personal communication, 2009). The scalp flap is dissected off the forehead and mobilized down to expose the superior orbital rim (see Fig. 25-4, B). The calvarium is typically removed by the neurosurgeons in one or several pieces. A bandeau osteotomy is then performed along the lateral temporal bones and the nasion to mobilize the superior orbital rim (see Fig. 25-4, C). Once the osteotomies are complete, the surgical field is protected with moist gauze. The calvarium and the orbital bandeau are sectioned and the pieces are reshaped and replaced in a manner that replicates a normal head shape. The bone is secured with a craniofacial plating system, the scalp flaps are replaced, and the coronal incision is closed.

FIGURE 25-4 Bicoronal skin incision. A, A bicoronal incision is made for access to the calvarium. Note the skin clips on the scalp flap to minimize bleeding. B, The skin flap is mobilized off the forehead to expose the orbits. C, The calvarium and the supraorbital rim are seen in approximation.

Strip Craniectomy with Helmet

The endoscopic approach is less invasive and has been reported to cause less blood loss (Jimenez et al., 2002). This procedure is more commonly used for sagittal synostosis, although it has been described for the repair of other single suture and even multiple-suture synostosis. The surgical approach is through smaller incisions. As with open approaches, significant blood loss can occur if the sagittal sinus is entered. Jiminez reported a small percentage of patients who required transfusions, and most were discharged on the first postoperative day. Unlike those who had an open calvarial reconstruction, these patients do require helmet therapy after this repair.

Midface Advancement

Midface advancements are performed to correct midface hypo-plasia. The different types of advancements include the Le Fort I maxillary advancement, the Le Fort III maxillary and upper face advancement (Fig. 25-5), and the monobloc advancement, which includes the maxilla, upper face, orbits, and forehead. The Le Fort I osteotomy is typically performed for patients with midface hypoplasia secondary to cleft lip and palate. The Le Fort III and monobloc osteotomies are typically performed for patients with midface hypoplasia secondary to syndromic craniosynostosis (Apert, Pfeiffer, Crouzon syndromes). The surgical approach for the Le Fort III and monobloc osteotomies are through bicoronal, intraoral, and often eyelid incisions. Significant blood loss can occur during the surgical dissection and osteotomies. Once the midface is mobilized, the advancement can be performed immediately with rigid fixation or gradually with internal or external distraction osteogenesis. Distraction osteogenesis became a common in craniofacial surgery in 1997, and the advantages include less morbidity, more long-term stability, and improved aesthetic outcome (Fearon, 2001; Shetye et al., 2007). The external distraction device has a frame that is anchored to the skull, with a distraction bar positioned perpendicular to the face. The distraction bar is anchored to the newly mobilized midface with wires (Fig. 25-6). Once in place, the midface may be distracted at a rate of 1 to 2 mm a day.

FIGURE 25-5 Le Fort osteotomies. Le Fort I mobilizes the maxillae. Le Fort II mobilizes the nose. Le Fort III mobilizes the entire midface.

FIGURE 25-6 Midface distraction. An adolescent boy with Apert syndrome, in a midface distractor.

Airway

Airway management in these patients can be very challenging during attempts at ventilation, intubation, or both. Fortunately, difficult airways are not common. However, the incidence is higher in those patients with syndromes and in those patients who have had previous reconstruction. Successful techniques described for infants and children include using the Bullard laryngoscope, laryngeal mask airway, flexible fiberoptic scope, and retrograde intubation (Cooper and Murray-Wilson, 1987; Blanco et al., 2001; Brown et al., 1993, 2004) (see Chapter 10, Equipment, and Chapter 12, Airway Management). A combination of techniques may be required to secure the airway. For example, the LMA has been used to facilitate the passage of the fiberoptic scope and endotracheal tube (Inada et al., 1995) (Fig. 25-7). Although those authors report this technique in a patient with Treacher Collins syndrome, it could be used for any craniofacial patient with a difficult airway. An endotracheal tube and LMA size chart helps in choosing the appropriate equipment (Table 25-2, and see also Table 10-3 in Chapter 10, Equipment). Cuffed endotracheal tubes may be more challenging, because the cuff on the endotracheal tube may not fit through the smaller LMAs. However, the air-Q (Trudell Medical Marketing, London, Ontario, Canada) has its short stalk and detachable adapter that make it easy to pass a cuffed endotracheal tube with the pilot balloon. If an uncuffed endotracheal tube is used, it can be secured while removing the LMA by telescoping a smaller endotracheal tube through the top of the larger tube (see Fig. 25-7, B). When passing an endotracheal tube through an LMA that is a size 3 or smaller, an uncuffed endotracheal tube is easier to use, but it runs the risk for creating a large air leak.

FIGURE 25-7 A, Once the laryngeal mask airway (LMA) is positioned in the mouth, the fiberoptic scope is placed through it, and the endotracheal tube is fed over the fiberoptic scope into the trachea. B, Another uncuffed endotracheal (one-half size smaller) is used as a pusher to facilitate LMA removal without extubation.

TABLE 25-2 Equipment Sizes for Airway Management

The airway for patients having craniosynostosis surgery can be secured with a standard oral endotracheal tube. For these patients, a nasal intubation may be easier to perform when using the flexible fiberoptic scope. A standard endotracheal tube may be preferred to a nasal RAE (Ring, Adaire, Ellwyn) tube because of the surgical approach, which involves a bicoronal incision.

Patients having Le Fort osteotomies require a nasal or an oral intubation depending on the type of Le Fort. Those having a Le Fort I osteotomy (for clefts) will need a nasal endotracheal tube so the maxillae and the mandible can be aligned properly. Nasal endotracheal tubes have been damaged during Le Fort osteotomies, resulting in difficulty with ventilation (Bidgoli et al., 1999), so care must be taken by the surgeons when mobilizing the maxillae. Patients having a Le Fort III osteotomy for midface distraction can be intubated orally with an oral RAE tube.

Some infants with craniofacial anomalies require tracheostomy because of significant upper airway obstruction (Perkins, 1997; Sculerati et al., 1998). Adequate preparation entails having all the necessary equipment available and having personnel who are trained and experienced in the use these airway instruments. It may also mean having a pediatric otorhinolaryngologist immediately available.

Intracranial Pressure

Patients with craniosynostosis may develop intracranial hypertension. Typically this is diagnosed by the presence of papilledema during an ophthalmologic examination. Clinically, increased ICP may range from being asymptomatic to complaints of visual changes, headaches, somnolence, and vomiting. Children with chronically elevated ICP may be relatively asymptomatic and active. Chronically elevated ICP can have anatomic and physiologic consequences that may affect the surgical and anesthetic management. Computed tomography scans and magnetic resonance imaging (MRI) will show a beaten-copper-pot appearance of the skull in patients with chronically elevated ICP. The close proximity of the brain to the skull may make removal of the calvarium difficult. Mannitol (0.25 to 0.75 g/kg) and mild hyperventilation may facilitate this process.

Hemodynamic instability can occur in patients with chronically elevated ICP after the calvarium is removed. The probable physiologic cause is the sudden reduction in blood pressure required to maintain cerebral perfusion pressure once the calvarium is removed and the ICP equilibrates with atmospheric pressure. Patients may require transient hemodynamic support with vasoactive medication (e.g., epinephrine or phenylephrine).

Blood Conservation and Transfusion Medicine

Craniofacial procedures are often long, exposing infants to the risks of hypovolemia, hypothermia, blood loss, and venous air emboli. The craniofacial procedures typically performed during the first year of life include cranial vault remodeling (including fronto-orbital, posterior, and total vault advancement) and strip craniectomy. One of the most pressing concerns related to anesthesia care in craniofacial surgery is the management of intraoperative bleeding. The cranial procedures can involve significant blood loss because of the duration of the procedure, the many exposed skin and bone surfaces, and the rare complication of entering large vessels such as the sagittal sinus. Blood loss during these procedures has remained an issue since Whitaker and colleagues’ description of perioperative blood loss in 1979. Blood loss is often as high as half to one blood volume, and nearly 90% to 100% of the infants undergoing these procedures may require a blood transfusion (Faberowski et al., 1999; Tuncbilek et al., 2005; Stricker et al., 2010). Even the strip craniectomy, which is typically performed to correct nonsyndromic isolated sagittal synostosis and results in less blood loss, can produce significant hemorrhage. In a recent study by White and others (2009), predictors of blood loss during craniofacial surgery included surgery time greater than 5 hours, age less than 18 months, the presence of multiple-suture craniosynostosis, and syndromic craniosynostosis. Infants undergoing strip craniectomy usually experience less blood loss, but this population may be at increased risk because they come to the operating room at the nadir of their physiologic anemia (i.e., at 2 to 3 months of age). Preparation for these procedures requires a baseline hematocrit, and blood typing and cross-match. Some centers also obtain coagulation studies. Adequate IV access needs to be obtained for resuscitation. In an infant, at least two large-bore (22- to 20-gauge) peripheral IV catheters should be placed to provide adequate access. Arterial pressure monitoring is recommended for beat-to-beat analysis of blood pressure and intravascular volume status, as well as for blood gas monitoring. A central venous catheter may be placed for central venous pressure monitoring, or in patients with difficult IV access.

Recently, some centers have reported significant success with minimizing blood loss and exposure to allogeneic blood products during craniofacial surgery. Surgical techniques that minimize blood loss include infiltrating subcutaneous epinephrine, needle tip cautery, scalp clips for the scalp flap, and bone wax for the osteotomy edges. Several medical and pharmacologic blood conservation techniques have been described in the older pediatric patient, but less information is available for the infant having cranial vault reconstruction.

Preoperative blood donation has been described in children as small as 8 kg (Mayer et al., 1996), but this technique has significant limitations in the infant. Their young age, small size, and lower hematocrit may make blood collection more challenging and less feasible. Acute normovolemic hemodilution has been described in older children and adolescents and may be effective. Hemodilution is relatively contraindicated in the infant less than 6 months old. The normal physiologic advantage with hemodilution (increased preload, increased stroke volume, and decreased systemic vascular resistance, increased tissue oxygenation) may be lost in the infant because of fetal hemoglobin, which more avidly binds oxygen, and a naturally less compliant myocardium.

Because most infants presenting for cranial vault reconstruction have a normal physiologic anemia, erythropoietin was proposed as a therapy to address this concern. Preoperatively, recombinant erythropoietin decreases the transfusion requirements in infants having craniosynostosis repair. Fearon and Weinthal (2002) reported the dosage of erythropoietin as 600 units/kg given subcutaneously once per week along with oral iron supplementation. Erythropoietin was started 3 to 4 weeks before surgery, and the incidence of blood transfusions in infants having craniosynostosis repair decreased from 93% to 57%.

In March of 2007, the U.S. Food and Drug Administration placed a black-box warning on synthetic erythropoietin because of the concern of increased death, deep venous thrombosis, and cancer spread in adult patients receiving synthetic Epogen (Singh et al., 2006; see also www.fda.gov/for consumers consumers). These concerns have occurred only in the adult population, but some centers have stopped using synthetic erythropoietin for craniofacial surgery. A multicenter retrospective review of 396 pediatric craniofacial patients receiving erythropoietin did not reveal an increase in perioperative complications, specifically death or deep venous thrombosis (Naran et al., 2010).

Antifibrinolytic therapy has been described in pediatric surgery, but the data for craniofacial surgery are limited. Aprotinin, a serine protease inhibitor, decreases perioperative blood loss and transfusion requirements during craniofacial procedures. In a prospective, randomized, and blinded placebo-controlled study evaluating the effect of aprotinin in infants and children having cranial vault remodeling and frontal orbital advancements, D’Errico and colleagues (2003) noted a reduction in the amount of packed red blood cells being transfused intraoperatively and postoperatively in those receiving aprotinin. However, aprotinin did not reduce the number of patients requiring transfusion. Although no adverse events were reported, the option of aprotinin has been curtailed with its removal from the market. Tranexamic acid and aminocaproic acid are two antifibrinolytics that remain available for potential pediatric use. The data to guide the use of tranexamic acid and aminocaproic acid in this population are limited, but a small study by Durán de la Fuente and colleagues (2003) demonstrated a reduction in estimated blood loss and transfusion requirements. The tranexamic acid was not infused continuously but instead was administered every 8 hours at a dosage of 15 mg/kg. Table 25-3 shows standard dosage regimens for tranexamic acid and aminocaproic acid.

TABLE 25-3 Dosage Guidelines for Antifibrinolytic Therapy

In the past, the use of a cell saver was reported as being impractical for small pediatric patients because of the size of the collection reservoir (De Ville, 1997). Recently, the cell-saver reservoirs are available in sizes as small as 25 mL. This technology may reduce the rate of autologous blood transfusion in infants having craniofacial surgery. In fact, the most significant benefit may occur when cell-saver technology is combined with erythropoietin pretreatment. In a prospective analysis evaluating the use of cell saver with a 55-mL pediatric bowl for patients pretreated with erythropoietin, only 30% of those infants having cranial vault remodeling required allogeneic blood (Fearon, 2005). Krajewski and coworkers (2008) in a prospective randomized trial describe a 5% transfusion rate in infants having elective craniosynostosis surgery when both cell saver (25-mL collection reservoir) and erythropoietin were used, compared with a 100% transfusion rate for those not receiving cell-saver techniques or erythropoietin.

Temperature and Positioning

Craniofacial procedures can be very long—sometimes several hours. Complications resulting from long surgical procedures include skin breakdown, neuropathic injury, and hypothermia. Attention must be paid to the initial setup to ensure adequate positioning and padding to minimize these intraoperative injuries. Infants having cranial vault remodeling may be positioned prone, and attention to protecting the face and eyes is important. Patients with midface hypoplasia and proptosis may present a challenge when placed prone, because adequately protecting the face and eyes may be more difficult. The infant is placed on a full-access forced-hot-air blanket to minimize hypothermia, and the surgical site (head) is then isolated from the body using plastic drapes (Fig. 25-8). This not only minimizes convective and radiant heat losses but also prevents conductive heat loss to a bed wet from irrigation and blood. Blood products should be warmed through a fluid warmer before administration (except for platelets). Intravenous fluid warmers may be used, particularly if large volumes of cold allogeneic blood are being administered.

FIGURE 25-8 The head is isolated from the torso with plastic drapes. The drapes are placed in opposite directions and sealed to minimize moisture and thus hypothermia from blood and irrigation fluids.

Venous Air Embolism

Venous air embolism (VAE) is a potential complication of craniofacial and neurosurgical procedures (see Chapter 22, Anesthesia for Neurosurgery). VAE can present with hemodynamic instability and can result in death. VAE occurs commonly in pediatric patients having cranial procedures. A prospective study using precordial Doppler in infants and children having craniosynostosis repair detected VAE in 82% of the patients; 31% percent developed hypotension secondary to VAE, but none developed cardiovascular collapse (Faberowski et al., 2000). This is higher than the previously reported incidence of 66% (Harris et al., 1987). Infants may be at increased risk for VAE, because significant hemorrhage during cranial vault remodeling can result in low central venous pressures. In addition, the relatively large size of the infant head may raise the surgical site above the level of the heart, thereby increasing the pressure gradient for air entrainment. Some advocate the placement of central venous catheters to monitor the trend of central venous pressures and minimize the risk for air embolism. However, no data suggest that central venous pressure monitoring decreases the risk for VAE.

Management of VAE begins with preventing hypovolemic states by providing adequate volume resuscitation and using a precordial Doppler for early detection of VAE. The precordial Doppler should be placed over the left or right parasternal border between the third and sixth intercostal spaces (Schubert et al., 2006). Lowering the head of the bed, left lateral positioning, flooding the surgical field with saline, applying bone wax, discontinuing nitrous oxide, and providing inotropic support are all measures that have been used to acutely manage VAE. Pediatric advanced life support with chest compressions and epinephrine is required for patients who develop symptomatic low blood pressure, bradycardia (heart rate < 60 bpm), or pulseless electrical activity.

Anesthesia Management

Airway management in the infant with Pierre Robin sequence can be very challenging because of difficulty with mask ventilation and intubation. The laryngeal mask has been successfully used to ventilate and to assist in the intubation of these patients (Markakis et al., 1992). The LMA has even been placed in the awake patient with PRS after regional nerve blockade (Stricker et al., 2008). Nasal intubation with the flexible fiberoptic scope has also been described (Blanco et al., 2001). Other airway devices used for patients with PRS and difficult intubation include the Storz video laryngoscope (KARL STORZ Endoscopy-America, Inc.) and the Airtraq optical laryngoscope (PRODOL MEDITEC S.A.). In addition, when infants have significant difficulty with ventilation or intubation, aside from oral pharyngeal and nasal airways, a suture (0-silk) can be placed at the base of the tongue to displace the tongue anteriorly to assist with ventilation or intubation.

Infants presenting for tongue-lip adhesion or mandibular distraction will require nasal intubation. After inhalation induction with spontaneous ventilation, a flexible fiberoptic nasal intubation can be performed. After the tongue-lip adhesion is completed, infants typically recover in the intensive care unit, where they remain intubated, relaxed, and sedated for several days. Extubation can be performed in the operating room when both the anesthesiologist and pediatric otorhinolaryngologist can be present. When extubation fails, patients may require direct laryngoscopy, bronchoscopy, and tracheostomy. After mandibular distraction, the airway may become even more challenging because once the distractors are in place they may prevent effective mask ventilation.

Anesthesia Management

As with PRS, the most significant anesthetic challenge with hemifacial microsomia is airway management (see Pierre Robin Sequence, Anesthesia Management, earlier). Mask ventilation may be difficult because of facial asymmetry. Intubation may be more challenging because of micrognathia and asymmetric mandibular hypoplasia, and also potentially because of decreased cervical range of motion. This difficulty may decrease with age, but it may increase after surgical reconstruction. Successful ventilation and intubation of the infant have been reported with an LMA and fiberoptic laryngoscopy (Johnson, 1994).

Surgical Management

Pharyngoplasty

Pharyngoplasties are performed to treat an incompetent velopharyngeal sphincter that allows inappropriate nasal air escape during speech, or hypernasality, defined as VP insufficiency. Pharyngoplasties, often performed as secondary speech procedures after a palatoplasty that failed to result in VP competence, alter the anatomy of the posterior pharynx and the VP port. The VP port is the muscular sphincter connecting the posterior nasal cavity and pharynx, and it is made up anteriorly by the posterior free edge of the soft palate (velum), posteriorly by the posterior pharyngeal wall, and laterally by the tonsillar pillars or lateral pharyngeal walls. Pharyngoplasties include the posterior pharyngeal flap and the sphincter pharyngoplasty.

The posterior pharyngeal flap (PPF) is a superiorly based flap of tissue raised off the posterior pharyngeal wall. It includes pharyngeal mucosa and a portion of the superior constrictor muscle. The PPF is superiorly based at the level of C2 or the adenoid pad, and it is inset into the posterior free edge of the velum. The PPF is sewn to the middle of the velum creating VP ports on either side of the PPF. These VP ports function as nasal airways.

FIGURE 25-11 Cleft palate repair complete. The mouth retractor is in place. Cotton rolls are placed on either side of the oral RAE tube to prevent compression from the metal tongue blade of the mouth retractor.

The sphincter pharyngoplasty significantly narrows the VP port by elevating the anterior tonsillar pillar bilaterally with the underlying palatopharyngeus muscle. The bilaterally elevated anterior tonsillar pillars are rotated 90 degrees medially and sewn to a horizontal incision made in the posterior pharyngeal wall. Because the tonsillar pillars are a continuation of the posterior free edge of the velum, this technique creates a much smaller muscular sphincter, thereby greatly narrowing the natural VP port.

Because pharyngoplasties alter the anatomy of the VP port and posterior pharynx, they carry the lifetime risk for overcorrection, hyponasality, and, more importantly OSA.

Anesthesia Management

Most patients with a cleft lip or palate can be induced with a standard inhalational induction. If an IV catheter is in place, an IV induction can also be performed. Induction for patients with orofacial clefting can be achieved with an inhalation agent such as sevoflurane. Airway management for most of these children is straightforward, but difficulty with mask ventilation and intubation can occur in infants with craniofacial anomalies or retrognathia. Airway obstruction can usually be managed with the insertion of an oropharyngeal airway and CPAP.

For the patient with an isolated cleft lip and palate, difficulty with laryngoscopy is common. Some studies have described the incidence of difficult laryngoscopy (laryngoscopic view of Cormack and Lehane laryngoscope view grades 3 and 4) to be as high as 4% to 7% (Xue et al., 2006). Factors predicting a more difficult laryngoscopic view include bilateral clefts and retrognathia. Despite this high percentage of poor laryngoscopic view, only 1% of the patients were difficult to intubate, and only one patient had a failed intubation (Gunawardana, 1996).

Cleft patients with associated craniofacial anomalies or retrognathia may be difficult to ventilate or intubate. In these patients, preparations for alternative airway management need to be made. If general anesthesia is induced, the patient should remain spontaneously ventilating until the trachea is secured with an endotracheal tube. Successful intubation through a laryngeal mask with the aid of a fiberoptic scope has been described for pediatric patients with difficult airways (Inada et al., 1995). Other intubating devices include the Shikani Optical Stylet (Shukry et al., 2005; Clarus Medical). Surgical repair of the cleft palate using an LMA has even been described (Beveridge, 1989). 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 (Somerville et al., 2004).

The oral RAE 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 paid to securing the tube and protecting it from unintentional extubation. Surgical preparation of the field can result in removal of the tape from the tube if it is not secured well (Fig. 25-12). Because of the fixed length of the preformed oral RAE tube, there is a risk of main stem intubation in a smaller infant. Cotton rolls can be placed in the Dingman-Dott retractor to minimize this risk. There is also an increased risk of extubation if an unexpectedly small endotracheal tube is required. An armor-reinforced endotracheal tube (see Fig. 10-11 in Chapter 10, Equipment) can be used to secure the airway in this situation. These reinforced endotracheal tubes do not kink with flexion, making them ideal for infants or children who require a smaller than expected endotracheal tube. Breath sounds should be reevaluated after the mouth gag is in place because of possible compression of the tube, and because advancement may cause single-lung ventilation.

FIGURE 25-12 Securing an oral RAE endotracheal tube for cleft lip or palate surgery. Note the normal midline position of the oral RAE tube. The tube is initially secured with a double-sided adhesive sticker or adhesive tape. A Tegaderm dressing is placed over the tube and the double-sided adhesive stickers (or tape) to protect against the liquid preparation.

There is no standard technique for the maintenance of anesthesia. Both inhalation and total IV anesthetics have been described for patients having cleft lip and palate surgery. A balanced anesthetic of an inhalation agent (with or without nitrous oxide), an opioid, and a muscle relaxant (optional) is effective and safe. For patients having palate surgery, keeping the mean arterial pressure at 50 to 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). Some studies (Rolla et al., 2003; Steinmetz et al., 2007) have attempted to determine whether the IV or the inhalation technique is better. However, there does not appear to be a clear benefit of either one.

The surgeon infiltrates the palate with epinephrine to prevent excessive bleeding and to facilitate mucosal dissection. It has been demonstrated that inhaled anesthetics sensitize the myocardium and decrease the arrhythmia threshold of epinephrine. Katz and others (1962), almost 50 years ago, described ventricular dysrhythmias when epinephrine was infiltrated during halothane anesthesia. The safe dosage of epinephrine is different for adults and for children. Dosages as low as 1 mcg/kg were found to be arrhythmogenic in adults under halothane anesthesia (Katz et al., 1962). In contrast, a 1983 study found that 10 mcg/kg was safe in children under halothane anesthesia (Karl, 1983). The children in this study ranged from 3 months to 17 years old and were ventilated to maintain an end-tidal CO₂ of 40 mm Hg. The other inhaled anesthetics appear to be as safe or safer than halothane. In adults, the arrhythmogenic dosage of epinephrine was greater than 5 mcg/kg and did not differ between isoflurane, sevoflurane, or desflurane (Moore et al., 1993; Navarro et al., 1994). In children, 10 mcg/kg appears to be a safe maximal dosage of epinephrine for infiltration (see Box 7-3 in Chapter 7, Pharmacology of Pediatric Anesthesia).

Positioning and padding are critical during long periods of anesthesia. In particular, attention should be paid to protecting the infant’s chest wall and extremities from the breathing circuit and the tight monitoring leads. The patient is often rotated 90 to 180 degrees, and a straight adapter can facilitate the positioning of the circuit.

Precautions with the airway management should be taken before extubation, especially for patients with difficult airways. Airway obstruction has been described after palatoplasties (Anthony and Sloan, 2002). Patients undergoing a palatoplasty who have a history of a difficult airway or have an associated congenital anomaly should have a nasopharyngeal airway placed by the surgeon before emergence. This may help maintain a patent airway and decrease the work of breathing after extubation (Fig. 25-13). Throat packs can result in airway obstruction if unintentionally left in place, and confirmation of their removal must take place before extubation.

FIGURE 25-13 Nasopharyngeal airway for airway patency for emergence from cleft palate repair.

Tongue edema is a particular concern for the anesthesiologist taking care of an extubated patient after cleft palate surgery. Profound tongue, palate, and pharyngeal edema can occur from venous engorgement after use of the Dingman-Dott mouth retractor. It is prudent for surgeons to release the retractor repeatedly throughout the procedure to minimize this risk (Anthony and Sloan, 2002; Oste et al., 2004). Patients experiencing respiratory distress need to be reintubated and mechanically ventilated (Fig. 25-14). It may take several for days for the edema to resolve (Anthony and Sloan, 2002).

FIGURE 25-14 Tongue edema secondary to the Dingman-Dott retractor. Note the tongue stitch placed to help displace the tongue anteriorly and relieve the airway obstruction.

Postoperative pain management for cleft lip and palate repair should provide analgesia without respiratory depression, nausea, or vomiting. The best solution seems to be a combination of different medications, usually acetaminophen with the occasional addition of an opioid. Acetaminophen, when administered rectally, is given at an initial dosage of 20 to 40 mg/kg, followed by 20 mg/kg every 6 hours. The oral dosage is 10 to 15 mg/kg every 4 to 6 hours (Birmingham et al., 1997, 2001). Acetaminophen may have an opioid-sparing effect, and some infants after cleft lip repair require none, or very small amounts, in the postoperative period (Stephens et al., 1997).

Nonsteroidal antiinflammatory agents have been described for analgesia after cleft palate surgery (Sylaidis and O’Neill, 1998). Although some evidence suggests there may be no increased risk for bleeding, many surgeons and anesthesiologists choose not to use them routinely. Dosages of opioids, if administered, should be appropriate for weight, to minimize the risk for respiratory compromise in infants. A safe starting dosage of morphine for infants is 0.02 mg/kg every 3 to 4 hours, as needed. Infants undergoing cleft surgery and being given postoperative parenteral narcotics should be appropriately monitored (e.g., for apnea and bradycardia, electrocardiography, oxygen saturation).

The bilateral infraorbital nerve block works very well as an adjunct or as the sole analgesic technique for cleft lip repair (Eipe et al., 2006). The block, which can be performed by anesthesiologists or surgeons, provides better analgesia than periincisional infiltration or opioids alone (Pradeep et al., 1999; Rajamani et al., 2007). The infraorbital nerve block has been described for all age groups including neonates (Bosenberg and Kible, 1995).

The maxillary division of the trigeminal nerve exits from the infraorbital foramen. The nerve provides the sensory supply to the upper lip, the choana, the maxillary sinus, and part of the nasal septum. This block can be used to provide analgesia for cleft lip surgery, nasal septal repair, and endoscopic sinus surgery. There are two approaches to the maxillary division of the trigeminal nerve: extraoral (percutaneous) and intraoral. For the extraoral approach, the needle is directed toward the infraorbital foramen externally through the skin. The location of the foramen varies with the age. In children and adults, the foramen is located approximately 5 to 10 mm below the infraorbital rim in a vertical line from the pupil of the eye (Fig. 25-15). The thumb or a finger of the hand not holding the syringe can be placed on the inferior orbital rim as a guide and to protect the eye during the injection. The needle is placed just inferior to finger. Once it makes contact with the bone, it is withdrawn slightly and the local anesthetic is injected.

FIGURE 25-15 Infraorbital nerve block. A, The extraoral route. B, The intraoral route.

The intraoral route is accessed through the subsulcal area in the buccal mucosa. The point of insertion is the upper incisor or the second bicuspid on the side to be blocked. A needle is passed through the subsulcal route toward the location of the infraorbital foramen. As with the transcutaneous approach, it is suggested that the thumb or a finger of the hand not holding the syringe be placed on the infant’s inferior orbital rim and used as a guide and to protect the orbit and eye. Infants scheduled for cleft lip repairs require 0.5 to 1 mL of local anesthetic solution on each side; for older children and adolescents, 1.5 mL to 2 mL can be used. Bupivacaine (0.125%, 0.25%, or 0.5%, with or without epinephrine [5 mcg/mL]) or ropivacaine (0.2% or 0.5%) can be used for the block.

The duration of postoperative analgesia depends on the local anesthetic and its concentration. For children and adults, 0.125% bupivacaine provided analgesia for approximately 4 to 8 hours (Pradeep et al., 1999). Bupivacaine (0.5%) provides analgesia for 6 to 24 hours (Nicodemus et al., 1991; Eipe et al., 2006). In neonates, the block may be shorter (2 to 3 hours) (Bosenberg and Kible, 1995).

Treacher Collins Syndrome

Treacher Collins syndrome (TCS), also know as mandibulofacial dysostosis, is an example of a bilateral 6, 7, and 8 craniofacial cleft (Fig. 25-17). It was first described in 1846 by Thompson and was further elaborated on by Treacher Collins. Inheritance is as an autosomal dominant pattern with variable penetration. The syndrome is characterized by poorly developed supraorbital ridges, aplastic/hypoplastic zygomas, ear deformities, hearing loss, cleft palate (in one third), and mandibular and midface hypoplasia. From birth, the adequacy of the airway is of primary concern. The degree of airway obstruction is related to the degree of maxillary and mandibular hypoplasia, choanal atresia, and glossoptosis. Tracheostomy may be required during infancy for those at highest risk for OSA and sudden infant death syndrome. The morbidity associated with tracheostomy is significant and some have advocated distraction osteogenesis or tongue-lip adhesion for these patients (Genecov et al., 2009; Thompson et al., 2009). Approximately 50% of patients with TCS have hearing loss, which occurs secondary to anomalies of the inner ear. They also suffer from external ear anomalies (microtia) (Marres, 2002). Congenital heart disease has been described in patients with TCS but it is uncommon (Gollin, 1976). Developmental delay is also uncommon in this population. These patients are followed by a multidisciplinary craniofacial team and can come to the operating room for a variety of procedures. Aside from cleft lip and palate repair, the timing of major reconstruction typically occurs during childhood or adolescence when the cranioorbital zygomatic bony development is nearly complete.

FIGURE 25-17 Treacher Collins syndrome.

Anesthesia Management

Anesthesia for patients with vascular tumors or malformations can range from routine and uncomplicated to difficult and life threatening. For many patients with a small vascular malformation in an anatomic location of minimal clinical consequence, anesthesia for radiologic evaluation, radiologic intervention, or surgery can be safe and uneventful. However, anesthesia for vascular malformations can be more complicated because of anatomic location (airway), size and systemic involvement (congestive heart failure, thrombocytopenia, blood loss), and sequelae from therapeutic interventions (ethanol sclerotherapy).

Patients with hemangiomas or capillary malformations such as the port-wine stain may present for repeat laser treatments. For those patients with few or no comorbidities, these treatments can be performed in the outpatient setting. Typically, an inhalational anesthetic via a mask with or without IV access is all that is required. Lesions on the face or back may require an LMA or an endotracheal tube to secure the airway (Isago et al., 2006). Nitrous oxide, although safe for induction, may pose a fire hazard when laser treatments are near the face. Postoperative analgesia can be achieved with acetaminophen.

Vascular malformations involving the airway can lead to respiratory obstruction and OSA (Arneja and Gosain, 2008). In the anesthetized patient, there may be problems with ventilation and intubation (Nargozian, 2004; McLoughlin and McBrien, 2009). Some of the vascular malformations may not initially be obstructing lesions, but after sclerotherapy treatment or dependent positioning may swell dramatically leading to an obstructed airway (Sun et al., 2009). These patients may require alternative techniques to secure the endotracheal tube, such as a fiberoptic scope, the GlideScope, esophageal intubating devices, or intubating through an LMA. A hallmark of the management of the difficult pediatric airway is to secure the airway under general anesthesia while preserving spontaneous ventilation. When preservation of spontaneous ventilation under general anesthesia is not feasible, an awake or sedated intubation or tracheostomy is required. Patients with a consumptive process (Kasabach-Merritt syndrome) may be predisposed to intraoperative bleeding secondary to thrombocytopenia, and they may not be candidates for a regional anesthetic technique. However, regional anesthesia (intrathecal block) has been described in patients with Kasabach-Merritt syndrome when their platelet count is stable (Holak and Pagel, 2010).

Anesthesia Management

Many surgical excisions for small and medium nevi in children and adolescents can be performed under local anesthesia in an office setting. Younger patients, or patients who are uncooperative, may require a general anesthetic to facilitate excision. For healthy patients, excisions can typically be performed in an office setting or an ambulatory surgical center. An inhalational anesthetic will achieve appropriate surgical conditions. Depending on the size of the lesion and its location, an IV and an LMA or endotracheal tube may be required. Larger lesions are more likely to require a general anesthetic because of the degree of tissue resection and undermining, the duration of the procedure, and the location of the lesion. Regional anesthesia can be combined with general anesthesia to ensure a pain-free postoperative course. The regional technique can be a neuraxial (single-shot caudal) or peripheral block, depending on the location and size of the surgical excision.

Anesthesia Management

Anesthesia management for the pediatric facial fracture patients begins with a complete trauma assessment to identify airway pathology and then to identify associated injuries. Patients at risk for a brain injury with an underlying intracranial lesion (mandible, facial fracture with GCS < 15 or loss of consciousness) should have a neurosurgical evaluation prior to anesthesia for their facial fractures.

Facial fractures involving the midface commonly result in airway compromise because of the displacement of the midface posteriorly into the oropharynx. Blood and secretions may also contribute to airway obstruction. One third of adult patients in one study with midface fractures required intubation (Ng et al., 1998). Patients with Le Fort III fractures developed more severe airway obstruction than patients with Le Fort I or II fractures. In fact, up to 40% of Le Fort III fractures required tracheostomy in one study of adults, compared with 9% of Le Fort I and II fractures (Bagheri et al., 2005).

The airway management of pediatric patients with facial fractures can be challenging. Those with mild injuries like an isolated zygoma or orbital floor fracture typically do not present a significant challenge. However, those patients with complicated midface fractures (Le Fort III) or panfacial fractures can present a dilemma. Children not properly fasted require either a rapid-sequence induction or a sedated fiberoptic intubation. All patients with suspected or known cervical injuries require cervical immobilization.

Facial fractures that result in malocclusion (mandible or Le Fort I) require maxillomandibular fixation (MMF). Intubation options that allow MMF include nasal or retromolar intubation. If there is concern for a full stomach and aspiration, the airway can be initially secured with an oral endotracheal tube after a rapid-sequence induction. The oral endotracheal tube can then be changed to a nasal endotracheal tube using direct laryngoscopy or fiberoptic guidance. The nasal endotracheal tube can also be placed initially in the awake patient with sedation and topical local anesthetic.

This technique, however, is not an ideal option for the injured pediatric patient. The retromolar approach has been described for patients requiring MMF. This technique requires placing the endotracheal in the retromolar space, and it is meant for short-term intubation. The endotracheal tube can be sutured or wired in place by the surgeon. A smaller tube is recommended, and the anesthesiologist needs to be aware of the peak airway pressures once the tube is manipulated into this space (Lee et al., 2009).

The airway options for the patient with midface fractures or panfacial fractures include the placement of an oral endotracheal tube, submandibular airway, or tracheostomy. If MMF is not required, an oral intubation can be performed. In the patient who requires either MMF or long-term intubation, a submandibular airway or tracheostomy is recommended. A nasotracheal intubation is relatively contraindicated in patients with significant midface fractures (Le Fort II and III) because of concern for neurologic injury in those with a skull base (cribriform plate) fracture.

Traditionally, a short-term tracheostomy was recommended for patients with panfacial fractures because of the prolonged intubation in a patient with a potentially difficult airway or MMF. However, tracheostomy has an associated morbidity, and some have advocated a submental or submandibular approach to the airway (Amin et al., 2002; Anwer et al., 2007). The technique is performed after the patient is anesthetized and orally intubated. An armor-reinforced tube is recommended to prevent kinking. A small submandibular incision is made by the surgeon and bluntly dissected until it communicates with the floor of the mouth. The endotracheal tube cuff and then the end of the endotracheal tube (without the adaptor for the circuit) can be passed through this opening with the assistance of curved forceps or hemostats. The tube is sutured in place (Fig. 25-21). The submandibular airway can be removed at the end of surgery or can be left in place for several days (Amin et al., 2002; Anwer, 2009). This technique has been described for young adolescents (12 years old) (Eipe et al., 2005) and children as young as 6 years (Amin et al., 2002). Patients that are placed in MMF should have wire cutters at the bedside to release the fixation in case of an airway emergency. Even in skilled hands, this may not be quick. In one study, surgeons were able to remove the fixation in 30 seconds, but nonsurgical hospital staff took more than 2 minutes (Goss et al., 1979).

FIGURE 25-21 Submandibular intubation.