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Successful Thoracoscopic Repair of Esophageal Atresia with Tracheoesophageal Fistula in a Newborn with Single Ventricle Physiology

Mariano, Edward R., MD*; Chu, Larry F., MD, MS; Albanese, Craig T., MD; Ramamoorthy, Chandra, MBBS§

doi: 10.1213/01.ANE.0000175778.96374.4F
Pediatric Anesthesia: Case Report

A neonate with VACTERL association including tricuspid atresia was scheduled for thoracoscopic esophageal atresia with tracheoesophageal fistula (EA/TEF) repair and laparoscopic gastrostomy tube placement. In addition to standard noninvasive monitoring, arterial blood pressure, central venous pressure, and cerebral oxygen saturation were monitored. Gastric distension resulting from positive pressure ventilation prevented laparoscopic gastrostomy tube placement. Thoracoscopy with a CO2 insufflation pressure of 6 mm Hg at low flow (1 L/min) was well tolerated hemodynamically despite hypercarbia and cerebral oxygen saturation was maintained. Careful monitoring and good communication were critical to the safe management of this single ventricle patient during thoracoscopic EA/TEF repair.

IMPLICATIONS: Esophageal atresia and tracheoesophageal fistula in conjunction with single ventricle physiology carries a significant risk of mortality. We present the anesthetic management of a neonate with unpalliated tricuspid atresia who underwent a thoracoscopic tracheoesophageal fistula repair.

*Department of Anesthesia, University of California at San Diego School of Medicine, San Diego, California; †Department of Anesthesia, ‡Department of Surgery, Division of Pediatric Surgery, §Department of Anesthesia, Division of Pediatric Anesthesia, Stanford University School of Medicine, Stanford, California

Accepted for publication April 13, 2005.

Address correspondence and reprint requests to Edward R. Mariano, MD, Department of Anesthesia, University of California at San Diego Medical Center, 200 West Arbor Drive, San Diego, CA 92103–8770. Address e-mail to

Esophageal atresia with tracheoesophageal fistula (EA/TEF) associated with congenital heart disease (CHD) carries a high mortality rate (1). Improved survival in patients with EA/TEF has been attributed to early definitive surgery and advances in neonatal intensive care (2,3). Thoracoscopic EA/TEF repair has been accomplished in recent years (4,5). Although intrathoracic CO2 insufflation may have significant hemodynamic effects, particularly in neonates with congenital heart disease (CHD), there is little information regarding the safe anesthetic management of these patients. We present the successful perioperative management of a neonate with unpalliated tricuspid atresia (TA) undergoing thoracoscopic EA/TEF repair.

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Case Report

A full-term, 2.7-kg, neonate presented with EA/TEF and was scheduled for urgent repair on the first day of life. Echocardiography demonstrated TA with right ventricular hypoplasia, a 5-mm unrestricted ventricular septal defect, normally related great vessels without pulmonic stenosis, and a large patent ductus arteriosus (PDA). Other anomalies (hemi-vertebrae, imperforate anus, and unilateral renal agenesis) completed the VACTERL association. The patient was tracheally intubated in the neonatal intensive care unit (NICU) for respiratory distress, sedated with morphine, and ventilated in SIMV mode on room air. Umbilical vessels were cannulated, and dopamine was infused at 5–10 μg/kg/min for inotropic support with IV prostaglandin E1 (PGE1) to maintain ductal patency.

In the operating room, monitoring included cerebral oxygen saturation (rSO2i) using the Invos 5100 (Somanetics, Troy, MI) plus standard noninvasive monitors. General anesthesia was induced with fentanyl (7 μg/kg total for the case) and vecuronium and maintained with isoflurane (0.4%–0.6% expired) in oxygen/air (Fio2 0.21 – 0.45) and pressure-control ventilation (set peak inspiratory pressure 13 cm H2O). Dopamine and PGE1 were continued intraoperatively. A urinary catheter was placed for bladder decompression. Gastrostomy tube placement for postoperative feeding was attempted laparoscopically with a maximum CO2 insufflation pressure of 8 mm Hg, but visualization was difficult as a result of tense stomach distension. Impaired ventilation resulted in hypercarbia (Paco2 = 72 mm Hg) despite hyperventilation (Table 1). Laparoscopy was therefore abandoned in favor of a mini-laparotomy.

Table 1

Table 1

After laparotomy closure and before thoracoscopy, rigid bronchoscopy demonstrated the TEF 1 cm above the carina. The patient was then positioned prone with 30° right-sided elevation. CO2 insufflation alone (6 mm Hg at 1 L/min) achieved right lung collapse with excellent mediastinal exposure. During thoracoscopy, hypercarbia and acidosis developed but were well-tolerated hemodynamically (Table 1). The rSO2i was stable above 50% throughout the 257-min procedure. Packed red blood cells 30 mL/kg were required to maintain hematocrit over 40% and preload during intrathoracic insufflation in addition to 10 mL/kg of 5% albumin despite minimal blood loss and urine output more than 0.5 mL · kg−1 · h−1.

The patient was transferred to the NICU postoperatively, tracheally extubated 1 wk later, and subsequently underwent a modified Blalock-Taussig shunt for palliation of TA at 1 mo of age.

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Neonatal thoracoscopy introduces significant physiologic changes. One-lung ventilation (OLV) increases pulmonary vascular resistance (PVR) through hypoxic pulmonary vasoconstriction. Although pulmonary blood flow (Qp) was decreased, mixed venous O2 saturation and cardiac index (CI) were not affected by OLV in a neonatal animal model (6). Increases in PVR can reopen the foramen ovale or prevent PDA closure, causing a return to fetal circulation even in the absence of CHD.

PVR is dependent on Paco2, Pao2, pH, and lung volumes. Peritoneal insufflation increases PVR and decreases CI, which some patients with CHD may not tolerate (7–9). Right-sided thoracoscopy reduces venous return, CI, and mean arterial blood pressure even at low insufflation pressure as a result of direct compression of the vena cavae and right atrium (10,11). CO2 absorption results in hypercarbia and acidosis (12–14). Because end-tidal CO2 is a poor measure of Paco2 in patients with cyanotic CHD (15), arterial blood gas monitoring is essential.

Increased PVR may be better tolerated in patients with pulmonary overcirculation compared to neonates with normal cardiac physiology. In single-ventricle physiology (SVP), the balance between Qp and systemic blood flow (Qs) depends on the ratio of vascular resistance for each circulation (16). TA may or may not be associated with pulmonary outflow tract obstruction. In patients with unobstructed Qp, cardiac output often prefers the low-resistance pulmonary circuit and Qp restriction is required to limit overcirculation and maintain systemic perfusion. Inotropic drugs augment CI in the volume-loaded single ventricle, improving oxygen delivery (16).

We elected to continue dopamine intraoperatively and increase preload during thoracoscopy to maintain CI. We transfuse patients with cyanotic CHD to a goal hematocrit of 40%. Because cerebral oximetry aids management during pediatric cardiac surgery and can detect the effects of hypercarbia on cerebral blood flow (17,18), we monitored rSO2i in our patient. Trend monitoring of rSO2i is potentially beneficial during noncardiac surgery in patients with SVP as cerebral oxygenation may be impaired during periods of hemodynamic instability.

In conclusion, although the long-term benefit of thoracoscopic EA/TEF repair remains to be determined, this minimally invasive approach may be safely performed in neonates with complex CHD.

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