Temporary Retrograde Occlusion of High-Flow Tracheo-Esophageal Fistula Colin D. Gause, MD, Ian Glenn, MD, Michael Liu, MD, Federico G. Seifarth, MD

This report describes a temporary retrograde occlusion technique for control abstract of a high-flow tracheo-esophageal fistula in a critically ill, premature infant born at 29 weeks’ gestational age, with a diagnosis of type C (Gross) esophageal atresia and tetralogy of Fallot (TOF). This procedure is a useful bridging maneuver before definitive surgical correction for extremely low birth weight, unstable neonates with tracheo-esophageal fistula who are suffering from associated malformations.

Esophageal atresia (EA) with tracheo- a guide in which those with stable esophageal fistula (TEF) represents cardiac and respiratory status a spectrum of foregut abnormalities underwent early repair, whereas characterized by incomplete unstable patients underwent delayed esophageal organogenesis with or repair. Spitz et al10 later proposed without associated abnormal a new classification system, designated communication between the trachea I to III, based on birth weight and the and esophagus, with an incidence of presence of major cardiac disease. Department of Pediatric Surgery, Cleveland Clinic Children’s ∼1 in 3500 births worldwide. Patients Okamoto et al11 refined the Spitz Hospital, Cleveland, Ohio fi fi have additional congenital classi cation into a modi ed class Dr Gause performed patient chart review, drafted abnormalities in ∼50% to 70% of system, designated I to IV, with the initial manuscript, and revised the manuscript; cases. These abnormalities are emphasis on cardiac anomalies, Dr Glenn performed chart review and reviewed and predominantly cardiovascular because they represent the revised the manuscript; Dr Liu reviewed and revised (20%–39%),1 although genitourinary predominant predictor of survival. the manuscript; Dr Seifarth performed the surgical – procedures included herein, managed the patient, (14% 24%), gastrointestinal Neonates with a large TEF are at risk conceptualized the article, and reviewed and revised – (14% 23%), musculoskeletal for shunting excessive amounts of air the manuscript; and all authors approved the final – (17% 56%), and central nervous into the gastrointestinal tract, and manuscript as submitted. system (7%) abnormalities are also positive-pressure mechanical www.pediatrics.org/cgi/doi/10.1542/peds.2015-1234 common.1–5 Cardiovascular anomalies ventilation exacerbates this DOI: 10.1542/peds.2015-1234 contribute to the majority of morbidity phenomenon, contributing to Accepted for publication Jun 2, 2015 and mortality, cited as the cause of subsequent hypoventilation. Extremely death in 78% of cases.6 Address correspondence to Federico G. Seifarth, MD, premature neonates or patients with Department of Pediatric Surgery, Cleveland Clinic Anatomic classification is most severe associated anomalies are often Main Campus, Mail Code A120, 9500 Euclid Avenue, commonly described according to the too unstable to undergo early TEF Cleveland, OH 44195. E-mail: [email protected] Gross system, which classifies the repair. Therefore, operative PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, disease into 6 categories designated intervention to occlude the TEF before 1098-4275). 7 AtoF, the most common of which is definitive repair may be necessary to Copyright © 2015 by the American Academy of type C: proximal EA with distal TEF allow adequate ventilation and Pediatrics (84% of cases). The Waterson prevention of gastrointestinal FINANCIAL DISCLOSURE: The authors have indicated classification stratifies these infants complications. Gastrostomy represents they have no financial relationships relevant to this into survival categories A through C, a potential option, but this method article to disclose. based on birth weight and associated allows air to preferentially flow FUNDING: No external funding. 8 fi fi anomalies. This classi cation system through the stula and exit via POTENTIAL CONFLICT OF INTEREST: The authors have was refined by Randolph et al.9 The gastrostomy at the expense of indicated they have no potential conflicts of interest authors used physiologic status as pulmonary ventilation.12 This to disclose.

Downloaded from www.aappublications.org/news by guest on September 27, 2021 PEDIATRICS Volume 136, number 4, October 2015 CASE REPORT occurrence is particularly intervention. On day 4 she was taken problematic in neonates with poor to the operating room, where she lung compliance. Bronchoscopic underwent a 2.5-cm transverse mini- placement of a Fogarty balloon to laparotomy, Stamm gastrostomy, and occlude the fistula,13,14 endotracheal placement of a 10-Fr Mallinckrodt (ET) tube placement distal to the catheter. At the gastrostomy site, a fistula,12 esophageal banding,15 6-Fr Fogarty catheter was advanced Nissen fundoplication,16 and gastric under fluoroscopic guidance into the division17 are additional options to TEF (Figs 1 and 2). The balloon was prevent diversion of airflow, with cautiously inflated with 1 mL water- bronchoscopic occlusion used most soluble radiopaque contrast until FIGURE 2 commonly. ventilator control indicated Radiographic confirmation of occlusive balloon placement. This case report describes the resolution of the air leak (Figs 3 and 4). management of an unstable, The Fogarty catheter was secured premature, 810-g infant with a type C to the abdominal wall. The TEF and TOF (Waterson C, Spitz Mallinckrodt was then temporarily thoracotomy and suture ligation of fi group III, Okamoto class IV), in which placed to water seal to detect an air the stula. The distal esophagus was the high-flow fistula was managed leak, of which there was none. tacked to the chest wall under mild fi with creation of a gastrostomy and Despite signi cant improvement of tension and the proximal esophageal fluoroscopically guided placement of ventilator management, the patient pouch remained intubated with a retrograde esophageal fistula was deemed too unstable to undergo a Replogle catheter. On day 145, she occlusion balloon catheter, followed repair at that time. underwent complete repair of TOF, by staged repair of the TEF after Over the course of her hospitalization, closure of her patent foramen ovale, stabilization of the patient’s status. the Fogarty balloon was deflated and ligation of her patent ductus every other day for ∼30 minutes to arteriosus. Repair of TEF was prevent accumulation and aspiration deferred for 6 weeks in accordance with the recommendations of our CASE REPORT of esophageal secretions. Gastric tube feeds were begun. On day 25, she was cardiac surgery colleagues. On day fi The patient is a premature female taken to the operating room for 197, the patient underwent de nitive ’ singleton, born at 29 weeks ligation of the TEF. The fistula was reconstruction of the esophagus via gestational age, with an temporarily occluded with a 6-Fr thoracoscopic esophago- ultrasonographic prenatal diagnosis Fogarty catheter via the trachea, and esophagostomy. She tolerated the of TOF. Before the second minute of the patient underwent uncomplicated procedure well, and there were no life, the patient was intubated, and complications. inhaled surfactant was administered The patient was extubated on for respiratory distress of postoperative day 5. Iohexol upper prematurity. Attempts to insert gastrointestinal series on a Replogle orogastric tube failed, postoperative day 7 revealed a patent because the tube could not be anastomosis with contrast flow into . advanced 8 cm. Subsequent the stomach, with no evidence of abdominal radiograph revealed extravasation or anastomotic leak diffuse gastric, small and large bowel (Fig 5). She was started on breast fi gaseous distention, con rming the milk by mouth on postoperative day diagnosis of type C TEF. The Replogle 15, with supplemental gastrostomy tube was positioned in the distal feeds. She recovered well from her esophageal pouch and kept to low operations and continued to gain continuous suction. Contrast weight appropriately. The patient was radiography was not performed. discharged from the hospital into the fi Echocardiography con rmed TOF. care of her parents on day 221 of The patient remained stable for the life, smiling and active. She was last first 3 days of life. However, despite seen in clinic at 14 months of age delicate ventilator management, she (8 months after repair). At that developed increasing abdominal time, she was tolerating tube distention and needed progressive FIGURE 1 feeding at goal and taking puffed ventilator support, prompting urgent Retrograde TEF balloon occlusion via gastrostomy. baby food and yogurt by mouth, with

Downloaded from www.aappublications.org/news by guest on September 27, 2021 e1052 GAUSE et al subsequent feeding or an overly loose wrap resulting in persistent air leak. ET tube placement distal to the fistula12 maybepossibleonlyinaproximal fistula, because efforts to completely pass the fistula may result in ET obstruction at the carina or incidental mainstem bronchus intubation. Retrograde occlusion is temporary and does not alter gastric anatomy, thus not necessitating a reversal procedure17 or surgical band removal.15 It is important FIGURE 5 to note that although a gastrotomy is Postoperative esophagram confirming esopha- created to introduce the retrograde geal patency and integrity of repair. occlusion catheter, the same site is used for placement of an enteral feeding division. Kadowaki et al19 similarly tube, which may be removed without reported retrograde insertion of an additional operation. FIGURE 3 a Fogarty catheter into the distal 3/4 view detail of fistula balloon occlusion. Retrograde occlusion is certainly not esophagus in 4 low birth weight without risks. Inflating a balloon in neonates, which were subsequently the esophagus or TEF is accomplished adequate weight gain given her loosely ligated with sutures. Both under fluoroscopic, not visual adjusted age. techniques require large laparotomies guidance, and thus understandably to allow gastric and lower esophageal raises concern about overdistention 20 dissection. In 1985, Karl and compromise of the esophageal DISCUSSION demonstrated that neither silastic circulation. This concern was Retrograde occlusion of a TEF via band nor suture placement was addressed intraoperatively via slow gastrostomy was first described by necessary to achieve retrograde inflation of the balloon and Hofmann in 1976.18 Hofmann placed esophageal occlusion, performed in instructions to our anesthesia the Fogarty balloon into the distal a premature 1270-g boy. In contrast colleagues to instill the minimum esophagus via open gastrotomy and to the aforementioned reports, we amount of fluid in the balloon to used a silastic band around the cardia applied this technique in an negate an air leak. Forceful placement to perform a functional gastric extremely low birth weight, of the Fogarty catheter also carries premature patient with TOF via mini- the risk of perforation of the laparotomy. To the best of our esophagus or trachea. knowledge, this case represents the first application of retrograde TEF There are important considerations occlusion in an extremely low birth about balloon placement. The balloon weight (810 g) neonate with severe should be placed proximally enough cardiac comorbidities. to minimize formation of a cul-de-sac in which secretions could accumulate, Retrograde TEF occlusion offers with the risk for aspiration. However, advantages over the more common and too proximal balloon placement invasive methods of occlusion. Unlike carries the risk of obstructing the fi 13,14 bronchoscopic stula occlusion, airway. Although fluoroscopy was this method avoids the resistance to used to aid in balloon positioning, fl air owofalarge-boretubeinrelation flexible bronchoscopy could be used to a neonatal trachea. Additionally, to confirm initial balloon position and fi dif culties with the ET tube and to assess airway compromise from associated adjustment will not interfere proximal placement or overinflation. with balloon positioning. Compared with Nissen fundoplication, retrograde balloon occlusion is less invasive,16 and CONCLUSION it avoids potential complications of the FIGURE 4 Nissen fundoplication, notably an Retrograde esophageal occlusion is Anterior detail of fistula balloon occlusion. excessively tight wrap interfering with a rarely used but highly useful

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Downloaded from www.aappublications.org/news by guest on September 27, 2021 Temporary Retrograde Occlusion of High-Flow Tracheo-Esophageal Fistula Colin D. Gause, Ian Glenn, Michael Liu and Federico G. Seifarth Pediatrics originally published online September 21, 2015;

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