Esophageal Atresia

Esophageal Atresia

Chapter 107 Tracheoesophageal Fistula/ Esophageal Atresia RICHARD J. HENDRICKSON, MD Tracheoesophageal malformations occur in 1 in 4000 births. Som...

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Chapter 107

Tracheoesophageal Fistula/ Esophageal Atresia

RICHARD J. HENDRICKSON, MD

Tracheoesophageal malformations occur in 1 in 4000 births. Some 25% to 40% of these neonates are premature or of low birth weight. B Fifty percent of infants have associated congenital defects, the most common ones being cardiac malformations (35%), GU malformations (20%), and GI malformations (24%). The acronym VACTERL (vertebral defects, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal anomalies, limb defects) summarizes possible defects. Prenatal diagnosis by maternal ultrasound is possible in the presence of polyhydramnios and a small stomach bubble. C Respiratory distress (e.g., choking, coughing, and regurgitation with the first feeding) is the most characteristic sign of esophageal atresia. There is often gastric distention secondary to air inspired through the tracheoesophageal fistula (TEF). More subtle signs include desaturation with nippling associated with noisy breathing. D The current classification describes the anatomic defect rather than being a complex categorization by number and letter. The most common types of tracheoesophageal malformation are proximal esophageal atresia with distal fistula (85%), isolated esophageal atresia (10%), and H-type TEF without esophageal atresia (5%). E Radiographs of the chest and abdomen may demonstrate pneumonitis; air in the GI tract (which rules out isolated esophageal atresia); and associated anomalies such as duodenal atresia (“double bubble”), vertebral and sacral malformations, and other skeletal anomalies. Preoperative echocardiography allows assessment of cardiac structural anomalies (e.g., atrial or ventricular septal defect, valvular anomalies, and patent ductus arteriosus) that could have an impact on anesthesia management. The position of the aortic arch (5% right-sided) can also be determined. Renal ultrasound can be delayed until after esophageal repair but should be performed

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FREDERICK M. KARRER, MD

to identify possible renal anomalies (obstructive uropathy) that may require further intervention and/or follow-up. F A soft, radiopaque catheter, No. 6 to 10 Fr, passed through the mouth or nose into the esophagus as far as it will go, with lateral x-ray confirmation, determines the level of the proximal esophageal remnant. If repair is going to be delayed, a small amount of barium sulfate (1 to 2 ml) should be instilled into the esophagus to rule out a proximal TEF. G Air in the stomach on an abdominal radiograph distinguishes isolated esophageal atresia from proximal esophageal atresia with distal TEF. A stomach bubble suggests a distal TEF with proximal esophageal atresia. Absence of intestinal air suggests isolated esophageal atresia. H Preoperatively, elevating the head of the bed to 45 degrees and Replogle tube sump suction help to prevent aspiration. H2-blockers minimize gastric acid reflux into the bronchial tree. Primary extrapleural anastomosis of esophageal segments can be performed in the first 24 hours of life in the absence of associated life-threatening malformations or pneumonitis. Traditionally, a right thoracotomy with an extrapleural approach is used. Recently, thoracoscopic repair has been reported to be safe and effective. The goals of operative repair include a tension-free anastomosis with good blood supply. A chest tube or drain is left in the chest until a leak has been ruled out, either clinically or radiographically. I Gastrostomy, sump aspiration of the proximal esophageal segment, and total parenteral nutrition (TPN) feeding until clinical status improves is the optimal approach for low–birth weight infants (less than 1500 gm), infants with associated life-threatening anomalies, and those with significant respiratory compromise. Occasionally, in infants with severely compromised pulmonary status whose TEF prevents adequate oxygenation, the fistula is ligated as

a temporizing measure until delayed repair becomes feasible. Fistula ligation can be performed either via right thoracotomy or thoracoscopy. In addition, a gastrostomy tube may be inserted to allow enteral nutrition. Another approach is Fogarty or Foley catheter occlusion of the fistula via bronchoscopy or gastrostomy. This method stabilizes the patient without thoracotomy, avoiding violation of the chest until definitive repair. J In isolated esophageal atresia, there is commonly a long gap between the two segments of the esophagus. The gap should be evaluated with a contrast study after a gastrostomy tube has been placed. A short distal esophagus makes primary repair impossible. Gastrostomy feedings are then instituted, along with sump aspiration of the proximal pouch. After several weeks of gastrostomy feedings, elongation of the proximal and distal esophageal pouches may permit end-to-end anastomosis. K An H-type fistula commonly occurs in the cervical esophagus. The lesion often escapes detection in infancy and is usually discovered later in infancy or childhood because of repeated episodes of pneumonia. Bronchoscopy can be diagnostic. Passing a No. 4 or 5 Fr catheter across the fistula during bronchoscopy allows easier identification during right transcervical exploration. Thoracotomy is rarely necessary but is indicated for unusual fistulas in the thorax. L When the esophageal gap is still too long for anastomosis, cervical esophagostomy is occasionally necessary. Gastrostomy feedings are continued and the esophagus is replaced with stomach, colon, or jejunum. M Morbidity and mortality rates for immediate primary repair and for staged repair are not substantially different. Waterston’s risk classification helped to predict outcome based on birth weight, pneumonia, and associated congenital anomalies. The modern Spitz classification for prediction of

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TRACHEOESOPHAGEAL FISTULA / ESOPHAGEAL ATRESIA

History and physical examination Prematurity

A

Congenital defects

B

Respiratory and gastrointestinal signs

C

D TRACHEOESOPHAGEAL

Air in stomach G (esophageal atresia with TEF)

F Nasogastric tube

Primary repair

Gastrostomy Sump aspiration TPN ⫾ Fistula occlusion

No air in stomach (isolated esophageal atresia)

Barium

E

Dilation for stricture

I

Tube does not pass into stomach

J Labs Babygram

M

H

Gastrostomy Sump aspiration G-tube feeding

Delayed repair

L Cervical esophagostomy Esophageal replacement

O U T C O M E

Renal ultrasound

K Tube passes into stomach

TEF without esophageal atresia

Bronchoscopy Fistula division

Closure of fistula for recurrence

372 survival is based upon birth weight and major cardiac disease. Postoperatively, a contrast swallow is performed to identify a leak or stricture. If no leak is identified, then enteral feeds are initiated with chest tube or drain removal. Complications after repair include abnormal motility (100%), esophageal stricture (10% to 25%), gastroesophageal reflux (40% to 70%), anastomotic leak (8% to 12%), and recurrent fistula and significant tracheomalacia (both 3% to 5%). Anastomotic leaks usually seal spontaneously, but they contribute to stricture. In most cases, strictures are managed successfully by dilation during the first postoperative year. Recurrent fistula requires reoperation. Clinical tracheomalacia may require aortopexy in infants with TEF. Gastroesophageal reflux is initially

TRACHEOESOPHAGEAL FISTULA / ESOPHAGEAL ATRESIA

treated with acid reduction and/or prokinetic agents but may require fundoplication in up to 25%. Replacement of the esophagus with stomach, jejunum, or colon is associated with mortality rates of 2% to 9% and with increased morbidity, including leak (10% to 30%), stricture (12% to 15%), and revision after failure (10% to 18%). Late complications include intestinal obstruction (5% to 10%) and dysphagia (20% to 40%). Mortality after repair of H-type fistula is very rare, and morbidity from the neck incision or leak is minor. REFERENCES Brown AK, Tam PKH: Measurement of gap length in esophageal atresia. A simple predictor of outcome. J Am Coll Surg 182:41–45, 1996.

Hirschl RB, Yardeni D, Olkham K et al: Gastric transposition for esophageal replacement in children: Experience with 41 consecutive cases with special emphasis on esophageal atresia. Ann Surg 236:531–539, 2002. Kallen K, Mastroiacovo P, Castilla EE et al: VATER non-random association of congenital malformations: Study based on data from four malformation registers. Am J Med Genet 101:26–32, 2001. Rothenberg SS: Thoracoscopic repair of tracheoesophageal fistula in newborns. J Pediatr Surg 37:869–872, 2002. Spitz L, Kiely EM, Morecroft JA et al: Oesophageal atresia: At-risk groups for the 1990s. J Pediatr Surg 29:723–725, 1994. Spitz L: Esophageal atresia: Past, present, and future. J Pediatr Surg 31:19–25, 1996. Stringer MD, McKenna KM, Goldstein RB et al: Prenatal diagnosis of esophageal atresia. J Pediatr Surg 30:1258–1263, 1995. Waterston DJ, Carter RT, Aberdeen E: Tracheoesophageal fistula: A study of survival in 218 infants. Lancet 1:819–826, 1962.

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TRACHEOESOPHAGEAL FISTULA/ESOPHAGEAL ATRESIA

History and physical examination Prematurity

A

Congenital defects

B

Respiratory and gastrointestinal signs

C

D TRACHEOESOPHAGEAL

Air in stomach G (esophageal atresia with TEF)

F Nasogastric tube

Primary repair

Gastrostomy Sump aspiration TPN ⫾ Fistula occlusion

No air in stomach (isolated esophageal atresia)

Barium

E

Dilation for stricture

I

Tube does not pass into stomach

J Labs Babygram

M

H

Gastrostomy Sump aspiration G-tube feeding

Delayed repair

L Cervical esophagostomy Esophageal replacement

O U T C O M E

Renal ultrasound

K Tube passes into stomach

TEF without esophageal atresia

Bronchoscopy Fistula division

Closure of fistula for recurrence