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Anesthetic Management of an Infant With Postnatally Diagnosed Tracheal Agenesis Undergoing Tracheal Reconstruction: A Case Report

Willer, Brittany L. MD*; Bryan, Kayla G. MD; Parakininkas, Daiva E. MD; Uhing, Michael R. MD§; Staudt, Susan R. MD; Dominguez, Kathleen M. MD; McCormick, Michael E. MD#; Mitchell, Michael E. MD**; Densmore, John C. MD††; Oldham, Keith T. MD††; Berens, Richard J. MD‡‖

doi: 10.1213/XAA.0000000000000603
Case Reports: Case Report
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A term infant born cyanotic failed multiple intubation attempts and tracheostomy placement. After esophageal intubation resulted in the ability to ventilate, he was presumed to have tracheal agenesis and distal bronchoesophageal fistula. He was transferred to our institution where he was diagnosed with Floyd Type II tracheal agenesis. He underwent staged tracheal reconstruction. He was discharged to home at 4 months of age with a tracheostomy collar, cervical spit fistula, and gastrostomy tube. He represents the sole survivor-to-discharge of tracheal agenesis in the United States. We describe the anesthetic considerations for a patient with tracheal agenesis undergoing reconstruction.

From the *Department of Anesthesiology, University of Iowa Stead Family Children’s Hospital, Iowa City, Iowa; Department of Anesthesiology, Le Bonheur Children’s Hospital, Memphis, Tennessee; Department of Critical Care, §Department of Neonatology, and Department of Anesthesiology, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin; Marshfield Clinic, Marshfield, Wisconsin; and #Department of Otolaryngology, **Department of Cardiothoracic Surgery, and ††Department of Pediatric Surgery, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin.

Accepted for publication May 22, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Brittany L. Willer, MD, 895 Grouse Lane, North Liberty, IA 52317. Address e-mail to blwiller@gmail.com.

The patient was a 37-week gestational age 3.1 kg male born at an outside hospital via spontaneous vaginal delivery with Apgar scores of 1, 3, and 4 at 1, 5, and 10 minutes of life. Written consent for this case report was obtained from the parents. The pregnancy was complicated only by polyhydramnios. At birth, he was limp and cyanotic with minimal improvement after initial bag-mask ventilation. Aggressive bag-mask ventilation interrupted by repeated attempts at intubation resulted in abdominal distension and persistent desaturation prompting transfer to the operating room. Figure 1 represents the airway management and resultant heart rate and saturations in the first hour of life. In the operating room, the baby was induced with sevoflurane by mask. Atropine 0.1 mg was given at the beginning of the case. An otolaryngologist evaluated the airway with rigid bronchoscope, finding a firm tissue mass. The rigid scope was advanced down the esophagus confirming the tracheoesophageal fistula (TEF). Per the surgeon’s operation note, “The scope was passed into the esophagus where a tracheoesophageal fistula was visualized fairly low in the esophagus. The scope was removed and a 3.0-mm endotracheal tube (ETT) was placed in the esophagus and the patient ventilated through the esophagus to the lungs through the tracheoesophageal fistula.” With improvement in saturations and presence of Etco2, the ETT was stabilized, a planned tracheostomy was performed that was also unsuccessful, because no identifiable trachea was found in the neck. With the evolving clinical picture of a surgically absent trachea and improved ventilation with esophageal intubation to the distal airway, tracheal agenesis was presumed. With progressive gastric distension, a pediatric surgical consult was requested. After telephone conference with the surgical team at the Children’s Hospital of Wisconsin (CHW), a quaternary pediatric center in Wisconsin, a laparotomy was performed. A preemptive gastrostomy (G) tube followed by a temporary clip at the gastroesophageal (GE) junction was performed in an effort to avoid further compromise of ventilation from increasing gastric distension through a distal fistula. The infant’s saturations improved from <30% the hour before esophageal intubation, to the 70s to 80s with ventilation through the distal fistula, and finally to the 90s with clipping of the GE junction. He was then mechanically ventilated with conventional ventilation using 100% fraction inspired oxygen, peak inspiratory pressures of 35, and positive end-expiratory pressure (PEEP) of 5. Further workup was deferred pending transfer to CHW Neonatal Intensive Care Unit. On arrival at CHW, a computed tomography angiogram confirmed long-segment tracheal atresia with an esophageal fistula at the level of the carina and distal bronchomalacia (Figure 2). A chest x-ray evaluation confirmed rib fusion anomalies, and an echocardiogram revealed a large patent ductus arteriosus (PDA). Baseline head ultrasound and electroencephalogram were normal.

Figure 1.

Figure 1.

Figure 2.

Figure 2.

A description of the sequential operative cases, preoperative/postoperative ventilator management, and subsequent airway changes are provided in the Table. The general surgical approach to the patient was to intervene only when necessary and to keep the surgical interventions as simple as possible. Therefore, for the first few days of life, his G was kept to water seal. Only when the temporary clip proved insufficient, as evidenced by bubbling in the water seal from the G and progressively inadequate ventilation, was he taken to the operating room (OR) for intervention. On day of life 4, he presented to the OR for video laryngoscopy and definitive division of the esophagus, distal to the fistula. Umbilical artery and umbilical vein catheters were in place. Anesthesia was induced and maintained with low-dose sevoflurane and fentanyl infusion. Once manual ventilation through the esophagotracheal tube was confirmed as feasible, neuromuscular blockade was given, with ventilation provided with peak pressures of 24, PEEP of 5 yielded tidal volumes 25 to 30 mL. Video laryngoscopy (Figure 3) revealed laryngeal structures including epiglottis and vocal cords, with a blind end in the immediate subglottic airway. Flexible esophagoscopy again identified the esophagocarinal fistula (Figure 4). While the fistula was identified, the specific components were not investigated because of the concern for tissue injury or disruption with further instrumentation. He then underwent definitive stapling of the GE junction below the level of the esophageal fistula and PDA ligation.

Table.

Table.

Figure 3.

Figure 3.

Figure 4.

Figure 4.

Subsequent to the first operative event at CHW, he developed abnormal tonic posturing. Magnetic resonance imaging showed bilateral grade 2 intraventricular hemorrhages; however, no seizure activity was identified on a follow-up electroencephalogram. With recovery from his initial intervention, it was determined he would benefit from a more stable airway. He returned to the OR on day of life 12 for creation of a “pseudo” tracheostomy. Anesthesia was induced with sevoflurane and fentanyl. He was maintained with fentanyl, dexmedetomidine, and sevoflurane. He was initially spontaneously ventilated, with pressure-controlled ventilation of peak inspiratory pressure of 24 and 8 of PEEP. With the patient’s spontaneous negative inspiratory effort, he had intermittent obstruction as noted by loss of end-tidal CO2, thought to be due to dynamic collapse of the compliant esophagus around the ETT. Once manual ventilation was confirmed as feasible, neuromuscular blockade was administered, resulting in more reliable Etco2 tracings and the ability to decrease PEEP to 4, yielding tidal volumes of 40 to 50 mL.

Considerations for this procedure were 2-fold. First, deciding the best approach to dividing the floppy esophagus that would allow for rapid reintubation in the event of lost airway or if the compliant esophagus proved to be too distensible to allow adequate ventilation once opened. Next, careful control of the distal esophagus was imperative because inadvertent retraction into the thorax could make it near-impossible to regain control of the airway in a timely fashion. With these considerations in mind, neck exploration commenced and the esophagus was opened partially, as distal as feasible to minimize dead space, but still above the tip of the ETT. A second ETT (3.5 cuffed) was placed on the field into the distal esophagus in a side-by-side fashion relative to the original oroesophageal ETT. The ventilation circuit was exchanged to the new ETT and once adequate ventilation was confirmed both by Etco2 tracing and chest wall movement, the original ETT was withdrawn, the esophagus was divided, and the airway stoma was matured to the sternal notch. The endoesophageal tube was secured to the patient’s neck as a “pseudo” tracheostomy, and placement just above the level of the esophagocarinal fistula was confirmed with flexible bronchoscopy. The proximal esophagus was then brought out to the skin and a cervical esophagostomy was created, serving as a “spit fistula” (Figure 5). The patient was returned to the neonatal intensive care unit under continuous neuromuscular blockade.

Figure 5.

Figure 5.

Over the next few weeks, a custom 4.0 cuffed bivona tracheal tube was exchanged for the endoesophageal “pseudo” tracheostomy. The patient developed worsening hydrocephalus in the face of bilateral grade 2 intraventricular hemorrhage, with resultant occipital focal seizures. He was taken to the OR for a ventriculoperitoneal shunt and treated with antiepileptic medications. As he convalesced in the pediatric intensive care unit on mechanical ventilation, he required increased PEEP with resultant air trapping. He also demonstrated intermittent inadequate ventilation with near-death spells from hypoxemia, precipitated by breath-holding spells or defecation. These “near-death” episodes were thought to be a result of the malacia inherent to the native esophageal tissue compromising the patency of the esophagocarinal fistula. In between these events, the infant appeared to have normal tone and neurological function.

Because of intermittent, and at times unpredictable, inadequacy of patency and ventilation afforded by the existing fistula with associated “near-death” episodes, the infant returned to the OR at 3 months of age for esophagocarinal fistula resection and external stenting of the “pseudo” trachea on cardiopulmonary bypass. Inhalational induction was performed via “pseudo” tracheostomy and anesthetic was maintained with fentanyl and dexmedetomidine infusions, and low-dose volatile agents. An arterial and central line were placed. Prebypass esophagoscopy performed in the absence of PEEP confirmed the collapse of the distal esophagus, acting as a “pseudotrachea,” with inability to visualize the esophagocarinal fistula. He underwent a median sternotomy and was placed onto cardiopulmonary bypass. The esophagus was extubated followed by division of the esophagocarinal fistula. The surgical team, recognizing both the futility of leaving a floppy esophagus as the conduit for spontaneous respirations and the subsequent need for chronic positive pressure ventilator support if there were no alternatives, created a rigid support lattice for the esophagus using a ringed 20-mm Gore-Tex tube graft, suspending the wall of the esophagus with a series of pledgeted sutures around the graft (Figure 6). The stented esophagus was then widely anastomosed to the carina and suspended anteriorly, resulting in 360° rigid external support along the length of the esophagus and a now widely patent esophagocarinal anastomosis. A repeat esophagoscopy showed this “neo” pseudotrachea to be widely patent without positive pressure and even able to withhold suction without collapse. The patient was weaned successfully from bypass after 193 minutes and returned to the pediatric intensive care unit.

Figure 6.

Figure 6.

Three weeks after his esophageal suspension for “pseudo” trachea reconstruction and esophagocarinal fistula reconstruction, he was maintained with a modified bivona tracheostomy tube through the “pseudo” tracheostomy. He was on room air with saturations of 97%. He has had no further near-death episodes with breath holding or during bowel movement and had normal neurological status. He was discharged home at 16 weeks of age.

At 12 months of age, he continues to thrive, meeting normal developmental milestones. He currently remains G dependent, but has developed oral skills. The spit fistula is covered with a neonatal wound ostomy appliance and is free of skin breakdown. The esophageal mucosa is not optimal for respiratory function and, in the absence of humidification, obstructive casts have formed, prompting heat and moisture exchanger on the end of a custom Flextend Bivona tracheostomy during the day, and, at night, he is placed on a ventilator. Repeat esophagoscopy has shown no significant granulation tissue at the anastomosis. The multidisciplinary surgical team is currently in the planning stages of his future alimentary reconstruction by gastric transposition.

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DISCUSSION

Fortunately, tracheal agenesis is a rare anomaly, occurring in less than 1 in 50,000 live births.1 It was first described in 1900 by Payne,2 with an estimated <200 cases documented since that time. The majority of patients with tracheal agenesis have the Floyd Type II variant, as did our patient, with complete agenesis of the trachea and normal bifurcating main bronchi.3 More than 90% have additional congenital anomalies, with many believing tracheal agenesis belongs to the “VACTERL” association (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies and limb abnormalities) or possibly a “TARCD” association (tracheal agenesis, radial ray defects, complex congenital cardiac abnormalities, and duodenal atresia).4.5 Although our patient did have a simple PDA and a rib fusion abnormality, he was fortunate enough not to have any other major abnormality that would qualify him for one of the preceding associations.

Antenatally, these patients may have hyperechogenic lungs, dilated airways, flattened or inverted diaphragm, massive ascites, or large breathing movements as seen on ultrasound.6 This constitution of findings may prompt further anatomic delineation by fetal magnetic resonance imaging, but commonly there is little to cause suspicion prenatally. Diagnosis is therefore dependent on the rapid recognition of the following in the immediate neonatal period: history of polyhydramnios, absence of cry, cyanosis and respiratory distress, and inability to intubate.7 If recognized expeditiously, the situation may be temporized by endoesophageal intubation and ventilation, with gastric decompression. However, in most cases, tracheal agenesis is either not recognized soon enough or the associated anomalies are too complex to allow survival beyond a few hours after birth.8 Furthermore, even if recognized promptly, the neonate with tracheal agenesis is at substantial risk for anoxic brain injury as a result of the time spent diagnosing the lesion and managing the airway.

With advances in recognition and care referral systems, novel surgical techniques for tracheal agenesis have been used, resulting in a handful of survivors worldwide, making what was once thought to be a fatal lesion a neonatal congenital anomaly that the anesthesiologist may encounter and should recognize. To our knowledge, this case represents the sole patient with tracheal agenesis to survive to hospital discharge in the United States. Outside the United States, Soh et al9 managed a patient with type I tracheal agenesis with an ETT inserted into the trachea through a larger ETT (the tube-in-tube technique avoided frequent airway obstruction that would occur from granulation at the tip of the single ETT). The patient lived to the age of almost 7 years and was discharged home briefly without ventilator support.9 A patient in Osaka, Japan has been previously managed with a similar technique to the one used in our patient (external esophageal stenting with radial traction sutures). He was discharged from the hospital at 10 months of age without home oxygen or ventilation requirements.10 A few patients have even survived to the point of enteral reconstruction. A patient in Tokyo with Floyd Type I tracheal agenesis underwent enteral reconstruction with a retrosternal G; at the age of 4, he was undergoing oral rehabilitation.11 Hiyama et al12 reported an esophageal reconstruction with colonic interposition performed in a 3-year-old whose airway patency had been managed with a long T tube in the esophagus instead of a stent.

Given the infancy of these experimental repairs, the long-term consequences are not well delineated. Recurrent respiratory infections, as a result of the esophagus being ill-equipped to clear secretions, are likely. Also, there is a long-term risk of esophagocarinal anastomotic narrowing due to granulation tissue. Furthermore, advances in otolaryngology and regenerative medicine have not allowed for successful tracheal transplant or graft engineering, even in animal models, so vocalization has not yet been accomplished.13

The principles of anesthetic management for this patient began in the labor and delivery suite. While this lesion is extremely rare, prompt recognition of the signs that present with the less rare tracheoesophageal fistula were critical to survival. Once recognized, expedient management of the airway with mask ventilation followed by placement of the endoesophageal tube, surgical G and distal clipping of the lower esophageal segment were paramount to stabilization. This allowed the patient to oxygenate and ventilate, allowing transfer to a quaternary children’s hospital where a definitive diagnosis and staged surgical plan could be developed.

Periodic inadequate ventilation and desaturation was one of the biggest ongoing risks to this infant’s survival. As noted, he had many recurrent episodes, often with a precipitating initial event such as defecation, daily cares, or a preceding period of sustained fussiness. We noted the association of the “spells” with recurrent abdominal valsalvas. We suspected that these responses either caused a loss of ventilator synchronization or increased intrathoracic pressure sufficient to collapse the esophagus. As a result, a cascade of events was likely to occur. Hypoxia and hypoventilation would result in increased respiratory distress with further escalation of ventilatory drive, resulting in increased inspiratory efforts, potentiating pseudotracheal collapse because of the esophagomalacia. A myriad of like episodes are recorded resulting in familiarity by the critical care team (including staff physicians, nurses, respiratory therapists, and housestaff) to this underlying pathophysiologic changes and the strategies to allay such events. Their success in applying early the effective strategies to prevent respiratory arrest is laudable. Factors that may have contributed to this success included (1) assignment of primary caregivers, (2) recurrent teamwide communication regarding suspected pathophysiologic changes, (3) behavioral pattern recognition/anticipation to achieve expedient bedside help for “spell” management, (4) using early recognition physiologic monitors, ie, near-infrared spectroscopy, and (5) just-in-time bedside team training of providers in techniques to “break” the event, including but not exclusive to manual ventilation with adjusted continuous positive airway pressure, concomitant behavioral calming techniques, psychotropic medication administration, small-dose trans-ETT topical lidocaine, and ultimately short-term neuromuscular blockade.

Subsequent anesthetic management required (1) maintenance of adequate perfusion, oxygen delivery, and ventilation, (2) provision of adequate analgesia, amnesia, and a surgically quiet operative site, (3) focus on maintenance of the precarious airway. Inherent in the care of children with these rare and challenging lesions is the need for free communication between all members of the team, beginning with early involvement with preoperative planning. Full understanding of the native airway and desired end points of each staged surgical procedure was imperative. Critical to the success of these operative procedures is agreement on the planned technique for visualization of the existing airway structures, surgical manipulation of airway remnants, and rescue measures to be employed in the event of failed intervention. Per our hospital practice, an anesthesia plan was circulated to all members of the team, followed by a circulated surgical plan put forward by each surgical specialty as needed with discussion returned if desired. These plans were disseminated to surgeons, anesthesiologists, perfusionists, nurses, and techs in the OR and critical care units. Issues identified in these plans included but were not limited to the following issues. The availability of extracorporeal membrane oxygenation was identified and was made available during this patient’s care. Neuromuscular blockade was discussed with its use restricted until adequate manual ventilation was proven. End-tidal CO2 values and capnography may not be reliable so transcutaneous CO2 monitoring was discussed.

Nonairway tissue used to replace the trachea required additional discussion. It is important to realize that this patient was likely to require use of a normal esophagus as an airway conduit, which is by nature a highly collapsible tube not well designed for airway mechanics that rely on negative pressure for function. In addition, esophageal mucosa does not possess cilia responsible for clearance of secretions. The child may also have bronchomalacia. In this child, substantial positive end-expiratory pressures were applied to maintain adequate esophageal stenting, allowing for ventilation and airway patency through the esophagocarinal fistula. Modifying the esophagus utilizing the 360° suspension and elimination of the flow-limiting fistula ultimately provided the rigidity and diameter necessary to maintain the patency of the airway during biphasic respiratory function, eventually allowing the patient to wean from positive pressure ventilation.

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CONCLUSIONS

Tracheal agenesis is a rare congenital anomaly that was previously universally fatal soon after birth. With advances and innovations in recognition, prompt referral, and surgical techniques, these patients may be palliated in a staged approach that includes (1) endoesophageal intubation with distal GE junction ligation, (2) “pseudo” trachea formation with the distal esophagus and “spit” fistula formation with the cervical esophagus, and (3) external esophageal stenting with esophagocarinoplasty. The anesthesiologist may encounter these tenuous patients during these staged repairs and needs to be familiar with the lesion, the operative techniques, and the potential for lost airway. Pre-, intra-, and postoperative communication with the surgical team proved vital to the success of this case.

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ACKNOWLEDGMENTS

We would like to acknowledge the management provided by the delivery and surgical teams from Marshfield Clinic, Marshfield Wisconsin. Under the direction of Drs Mirko Krolo, Nathan Schreiber, and Scott Peterson, this child’s very rare and highly fatal disorder was effectively treated, allowing next steps to occur.

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CONTRIBUTIONS

Name: Brittany L. Willer, MD.

Contribution: This author helped care for the patient, and write and revise the manuscript.

Name: Kayla G. Bryan, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Daiva E. Parakininkas, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Michael R. Uhing, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Susan R. Staudt, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Kathleen M. Dominguez, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Michael E. McCormick, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Michael E. Mitchell, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: John C. Densmore, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Keith T. Oldham, MD.

Contribution: This author helped care for the patient, and revise the mansucript.

Name: Richard J. Berens, MD.

Contribution: This author helped care for the patient, and write and revise the manuscript.

This manuscript was handled by: Raymond C. Roy, MD.

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REFERENCES

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2. Payne WA. Congenital absence of the trachea. Brooklyn Med J. 1900;14:568.
3. Floyd J, Campbell DC Jr, Dominy DE. Agenesis of the trachea. Am Rev Respir Dis. 1962;86:557–560.
4. Evans JA, Greenberg CR, Erdile L. Tracheal agenesis revisited: analysis of associated anomalies. Am J Med Genet. 1999;82:415–422.
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10. Watanabe T, Okuyama H, Kubota A, et al. A case of tracheal agenesis surviving without mechanical ventilation after external esophageal stenting. J Pediatr Surg. 2008;43:1906–1908.
11. Fuchimoto Y, Mori M, Takasato F, et al. A long-term survival case of tracheal agenesis: management for tracheoesophageal fistula and esophageal reconstruction. Pediatr Surg Int. 2011;27:103–106.
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