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Elephant Trunk–Like Teratoma of the Face with Compromised Airway in an Infant with Complex Congenital Cardiac Defects: An Anesthetic Challenge

Maddali, Madan Mohan MD; Al Balushi, Faisal Khalfan Ahmed MD; Waje, Niranjan Dilip MD

doi: 10.1213/XAA.0000000000000264
Case Reports: Clinical Care

Large head and neck teratomas are very rare. Depending on their site of origin, they can produce varying degrees of airway compromise and can interfere with the conduct of general anesthesia. Large space–occupying lesions of the face may even interfere with the simple task of mask ventilation rendering inhaled induction of general anesthesia and maintenance of spontaneous ventilation difficult. If these neoplasms coexist with cardiac lesions necessitating corrective or palliative procedures, the task of oxygenation, ventilation, and securing a definitive airway becomes challenging especially in the presence of underlying unstable hemodynamics. We report on the anesthetic management of a female infant with a facial teratoma and single-ventricle physiology undergoing a cardiac palliative procedure where securing a definitive airway with minimal hemodynamic instability was the immediate requirement.

From the Department of Cardiac Anesthesia, Royal Hospital, Muscat, Sultanate of Oman.

Accepted for publication August 26, 2015.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Madan Mohan Maddali, MD, Department of Cardiac Anesthesia, Royal Hospital, P.O. Box 1331, PC 111, Seeb, Muscat, Sultanate of Oman. Address e-mail to

Teratomas are true neoplasms, and their incidence in the head and neck region is rare (1 in >35,000 live births) compared with relatively more common sacrococcygeal teratomas (1 in 4000 live births).1 Teratomas arising in the face may produce varying degrees of airway compromise depending on the extent to which they encroach into the nasopharynx. Airway control with insertion of a definitive airway with minimal changes in oxygenation, ventilation, and hemodynamics is a challenge for the anesthesiologist especially if these tumors are associated with underlying cardiac abnormalities. In this case report, we describe the anesthetic management of an infant with an elephant trunk-like nasopharyngeal teratoma undergoing a palliative procedure for a single-ventricle physiology. IRB approval (RH/MESRC#60) and written consent from the parents to use the child’s pictures for scientific purposes were obtained.

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A 2-month-old, 2.7-kg, 55-cm female baby with signs of irritability, feeding difficulties, recurrent chest infections, and breathing difficulties that worsened when lying on her back was referred to our tertiary care cardiac center for further cardiac evaluation.

Transthoracic echocardiography showed tricuspid atresia with a 4-mm patent foramen ovale, midmuscular ventricular septal defect (8-mm shunting left to right with a pressure gradient of 45 mm Hg), 2-mm patent ductus arteriosus (PDA). A decision was made to perform first-stage palliation in the single-ventricle repair pathway with PDA closure and banding the pulmonary artery.

The child had dysmorphic features with a large teratoma mimicking an elephant trunk arising from the right nasal ala with a very long philtrum and hypertelorism (Fig. 1). The baby had right-sided unilateral choanal atresia with ipsilateral restricted mouth opening and a receding chin. The parents reported that the child was comfortable lying on one side and snored while asleep. The baby’s room air oxygen saturation was 96%.

Figure 1

Figure 1

The history was that a twin pregnancy was detected when the mother had a fetal scan at 25 weeks of gestation. There were no obvious fetal abnormalities in one of the twins. The fetal scan of the second twin showed a small right ventricular cavity and an abnormal structure above the level of the upper lip. The possibility of airway obstruction during birth in the second twin with the abnormal facial mass was considered, and an ex utero intrapartum treatment procedure was planned at a university hospital that was fully equipped to deal with such procedures. However, the mother was admitted before the scheduled date to a neighborhood hospital in the middle of the night with fetal distress. An emergency cesarean delivery had to be performed in the presence of an attending anesthesiologist, neonatologist, and an ear nose throat surgeon capable of performing rigid bronchoscopy and surgical tracheostomies in children.

The twins were born with Apgar scores of 8 and 9, respectively, at the time of delivery. The plan of action at the time of delivery, in case of an airway disaster in the baby with the facial tumor, was to access the airway and attempt tracheal intubation either by direct laryngoscopy or by rigid bronchscopy. If both direct laryngoscopy and rigid bronchoscope failed, the final option would be surgical tracheostomy.

Before performing first-stage palliation surgery, the parents were counseled regarding the possibility of difficulties with initially securing the airway and later during tracheal extubation. The child was not premedicated given the potential risk of airway compromise. The induction room was kept warm with difficult airway equipment ready, which included different sizes of ventilation masks, oropharyngeal airways, laryngoscope blades, endotracheal tubes (3–4 mm sizes), laryngeal mask airway (sizes 1 and 1.5), and stylets. The original plan was to assess the extent of airway compromise under inhaled anesthesia and use a C-MAC Video Laryngoscope (Karl Storz GmbH & Co. KG, Tuttlingen, Germany) as the first option of visualization of the vocal cords and for tracheal intubation. The backup plan was fiberoptic-aided tracheal intubation with a 2.8-mm fiberoptic bronchoscope (BF-XP60, Olympus Canada, Inc., Richmond Hill, ON) keeping the C-MAC blade in situ retracting the tongue. In case these two approaches failed, the plan was to insert a supraglottic airway to manage ventilation. A pediatric surgeon experienced in rigid bronchoscopy and surgical tracheostomies was on standby in the event of an airway disaster such as a “cannot ventilate cannot intubate” scenario.

An antifogging agent was applied to the lenses of both scopes before use. After administration of 100% oxygen until a 90% exhaled oxygen concentration was achieved, anesthesia was induced using 4% sevoflurane in oxygen using standard American Society of Anesthesiologists monitoring guidelines ensuring that the baby’s spontaneous breathing was maintained. For adequate ventilation and for inhaled anesthetic induction, an adult-size mask was chosen to obtain a good seal around the nose and mouth encircling the tumor (Fig. 2). Her eyes were taped and cushioned safely with eye pads to avoid the pressure exerted by the large size facemask. The mask chosen also had adequate cushioning. Once adequate depth of anesthesia was achieved as observed by the absence of toe/body movement to a trapezius muscle squeeze test, airway obstruction to mask ventilation was assessed and excluded. Peripheral venous access was established, and 20 μg/kg glycopyrrolate was administered, followed by 3 mg ketamine and 3 μg fentanyl citrate. A Miller-like laryngoscope blade (size 1) that was compatible with the C-MAC Video Laryngoscope was used for laryngeal visualization. With the application of backward, upward, and rightward pressure, only the very posterior portion of vocal cords could be visualized (Cormack–Lehane laryngeal view grade II b). The trachea was intubated with a 4-mm ID noncuffed tube and the position confirmed by auscultation and capnography. Once tracheal tube position was confirmed, 3 mg IV rocuronium was administered for neuromuscular blockade. Invasive hemodynamic monitoring was achieved by cannulation of the right radial artery and the right internal jugular vein was accessed under ultrasound guidance. The baby tolerated the surgical procedure well with stable hemodynamic and ventilator variables. The surgery lasted 90 minutes and was performed through a left thoracotomy.

Figure 2

Figure 2

After banding pulmonary artery with PDA closure, the systolic pressure in the pulmonary artery distal to the band was 50% of the systemic pressure as measured directly on line, and the arterial oxygen saturation was 75% to 80% with a 50% inhaled concentration of oxygen (FIO2). The child was transferred to the pediatric postcardiac surgical intensive care unit for elective mechanical ventilation and hemodynamic monitoring. The child’s trachea was extubated over an 8F suction catheter after 20 hours of mechanical ventilation with the anesthetic team standing by. The suction catheter was removed after confirming the child had unobstructed breathing, and the rest of the postoperative course was uneventful.

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Teratomas are derived from totipotential cells and are not native to the area where the tumors occur. Head and neck teratomas in children are almost always benign, but locally aggressive.2 Teratomas in this region have dramatic clinical presentations and pose a challenge for airway management. A difficult airway in these children can only be anticipated based on the clinical signs and medical history. Bedside physical assessment in children is difficult because of limited or lack of cooperation on their part. Airway access difficulties in children with underlying cardiac lesion are more challenging to the anesthesiologist because hemodynamic fluctuations during the process of airway control would be detrimental.

Anticipation and preparation are key elements in airway management of these babies. Difficult airways in children can lead to life-threatening situations, when adequate ventilation and oxygenation cannot be achieved. Therefore, oxygen administration to pediatric patients, although difficult, should be attempted. While doing so, one must consider the underlying cardiac status of the baby. Infants with single-ventricle physiology with unrestricted pulmonary blood flow who arrive in the operating room without tracheal intubation have clinically balanced circulation because of an increase in pulmonary vascular resistance (PVR) secondary to low lung volumes, increased interstitial lung water, and hypoxia-induced pulmonary vasoconstriction. After administration of general anesthesia with endotracheal intubation and mechanical ventilation, there may be a precipitous decrease in PVR with compromise of systemic perfusion. In these babies with a single ventricle and unrestricted pulmonary blood flow, manipulation of PVR and pulmonary to systemic blood flow ratio can be achieved through ventilatory interventions. Despite the potential decrease in PVR that could occur with 100% oxygen administration, it was done in this baby to compensate for any delay in oxygenation. Any subsequent hypercarbia or hypoxia associated with delay in airway access would contribute towards an increase in PVR and maintain systemic perfusion. After tracheal intubation and arterial line insertion, manipulation of ventilation and adjustment of FIO2 was done to achieve hypercarbia (PaCO2 > 45 mm Hg), pH of 7.30 to 7.35, and oxygen saturation of approximately 85% to 90%.

The approach to the difficult airway in this child was the maintenance of spontaneous ventilation under inhaled anesthesia and assessment of the airway once the child was adequately asleep. Although maintenance of spontaneous ventilation would theoretically provide the option of backing out and allowing the child to wake up, this might not always be possible. There might be occasions, after inhaled induction, when severe airway obstruction could develop. In anticipation of this, supraglottic airways were kept ready. To manage a potential cannot ventilate, cannot intubate scenario, a pediatric surgeon was on standby for performing rigid brocnchoscopy and if necessary, a surgical tracheostomy.

Optimization of facemask ventilation is a priority before definitive airway management to provide adequate ventilation.3 Therefore, a large-size mask was selected for inhaled anesthetic induction to allow for a good approximation of nose and face around the protruding tumor.

Depth of anesthesia should be adequate for suppressing reflexes during airway assessment and procuring IV access. For successful visualization of the larynx, we ensured that adequate depth of anesthesia was achieved and external laryngeal pressure was applied appropriately. Selection of an appropriate laryngoscope was also important.4

A C-MAC Video Laryngoscope was selected as our first-line airway access device because a clear picture could be obtained on the monitor screen (Tele pack; Karl Storz). The glottis view is improved with a Storz Video Laryngoscope in the presence of a difficult airway.4 A straight blade was selected (Miller-like blade, size 1), because the view on the screen would be similar to what is seen when looking directly into the mouth. In this baby, the endotracheal tube was coaxially inserted into the groove of the laryngoscope blade and advanced until it came into view on the monitor screen. It was then advanced further into the trachea visually confirming the position of the tube’s entrance into the trachea. It was also important that tracheal intubation was done during inspiration to avoid trauma to the cords, which could result in subsequent subglottic edema, thereby hampering extubation.

The management of such difficult pediatric airways does not end until the plan for extubation has also been established. In this baby, although tracheal extubation was smooth and uneventful, it was important that adequate reintubation equipment was available. Because an 8-French pediatric airway exchange catheter (Cook Critical Care, Bloomington, IN) was not available, an 8F suction catheter was used as a tube exchanger before removing the endotracheal tube. Once it was shown that the baby was comfortable, the catheter was removed.

Congenital high airway obstruction syndrome (CHAOS) is a prenatal diagnosis that occurs because of complete or near-complete upper airway obstruction caused by laryngeal atresia, laryngeal cyst, or tracheal atresia. CHAOS is an antenatal diagnosis based on ultrasound examination characterized by large echogenic lungs in the presence of inverted diaphragms, compressed mediastinal structures, dilated tracheobronchial tree, and signs of nonimmune hydrops.5 This baby’s antenatal scan revealed a large mass protruding above the lip with no intrathoracic features suggestive of CHAOS.

One can usually anticipate difficult airway in children based on the clinical signs and medical history. Crafting an airway management plan should include a strategy in case the primary approach does not succeed. Fiberoptic intubation is often the method of choice in these children with anticipated difficult airway access. There could be other approaches adopted in lieu of the approach described in this child. Awake fiberoptic intubation under sedation giving priority to securing the airway, over hemodynamic consequences, could be one such approach but would necessitate a high level of expertise. In babies with predicted difficult intubation, awake insertion of the laryngeal mask to relieve upper airway obstruction, inhaled induction of anesthesia followed by fiberoptic intubation could be an alternative approach.6

The expertise of the medical team is of vital importance to avoid life-threatening situations in children with difficult airways, and selection of the equipment plays a major role. In emergency situations, optimized facemask ventilation (aided by an oropharyngeal/nasopharyngeal airway) or ventilation using supraglottic airway devices could be life saving.

Large space–occupying lesions of the face, besides producing airway obstruction, can also interfere with mask ventilation rendering inhaled induction of general anesthesia and maintenance of spontaneous ventilation difficult. Difficult airway access with coexistent cardiac lesions makes the tasks of oxygenation, ventilation, and securing a definitive airway challenging. Management of such cases would need a high degree of expertise and should be managed in specialized centers (when possible).

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