Esophageal Atresia of Newborns

M. Messina, Francesco Molinaro, Alfredo Garzi, and RossellaAngotti

Contents

Salient Points . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Background . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Associated Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Genetic Counseling . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5

Clinical Description and Diagnosis . . . . . . . . . . . . . . . . . 5

Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6Preoperative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6Risk Categorization and Prognosis (Outcome) . . . . . . . 6Selection for Nontreatment . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

Emergency Ligation of the Distal TracheoesophagealFistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7

Operative Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7Thoracoscopic Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8Postoperative Management . . . . . . . . . . . . . . . . . . . . . . . . . . . 10Complications and Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . 10

Esophageal Replacement . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13

M. Messina (*)Department of Pediatrics, Obstetrics and ReproductiveMedicine, Section of Pediatric Surgery, University ofSiena, Siena, Italy

Department of Pediatrics, Obstetrics and ReproductiveMedicine, Section of Pediatric Surgery, Policlinico “LeScotte”, Siena, Italye-mail: mario.messina@unisi.it

F. Molinaro • R. AngottiDepartment of Pediatrics, Obstetrics and ReproductiveMedicine, Section of Pediatric Surgery, University ofSiena, Siena, Italy

A. GarziDepartment of Pediatrics, Obstetrics and ReproductiveMedicine, Section of Pediatric Surgery, University ofSiena, Siena, Italy

Università degli Studi di Salerno, Fisciano, Italy

# Springer International Publishing Switzerland 2016G. Buonocore et al. (eds.), Neonatology,DOI 10.1007/978-3-319-18159-2_228-1

1

AbstractEsophageal atresia includes a group of congen-ital anomalies characterized as interruption ofthe continuity of the esophagus combined withor without a persistent communication with thetrachea. These congenital malformations occurin 1:2,500–3,000 live births.

Over the last 40 years, there has been asteady improvement in the overall survivalrate due to early diagnosis and prompt referral,improvements in preoperative care and diag-nosis and treatment of associated anomalies,advances in anesthetic techniques, and sophis-ticated neonatal intensive care.

There is increasing experience with thethoracoscopic approach to repair esophagealatresia (Brandigi et al., J Siena Acad Sci5, 2013; Bax and van Der Zee, J Pediatr Surg37: 192–196, 2002; Rothenberg, J Pediatr Surg37: 869–872, 2002; Nguyen et al., JLaparoendosc Adv Surg Tech A 16: 174–178,2006; Krosnar and Baxter, Paediatr Anaesth15: 541–546, 2005; Rothenberg, Semin PediatrSurg 14: 2–7, 2005a; Rothenberg, Esophagealatresia and tracheoesophageal fistula. In:Najmaldin et al. (eds) Operative endoscopyand endoscopic surgery in infants and children.Hodder Arnold, London, pp 89–97, 2005b),but this requires advanced skills in minimalinvasive surgery.

Salient Points

1. Prenatal diagnosis is an important step to planan early management of patients with esopha-geal atresia.

2. Bronchoscopy is a preoperative obliged step.3. The thoracoscopic approach is a good option

for patients with esophageal atresia (Type III).

Background

Esophageal atresia includes a group of congenitalanomalies characterized as interruption of thecontinuity of the esophagus combined with or

without a persistent communication with the tra-chea (Chittmittrapap et al. 1980). These congeni-tal malformations occur in 1:2,500–3,000 livebirths. The overwhelming majority of cases ofesophageal atresia are sporadic/non-syndromic,although a small number within this nonfamilialgroup are associated with chromosomal abnor-malities (Chittmittrapap et al. 1980). Familial/syndromic cases of esophageal atresia areextremely rare, representing less than 1 % of thetotal. Esophageal atresia is two to three timesmore common in twins (Merci et al. 1998). Theetiology of esophageal atresia is likely multifac-torial and remains unknown (Crisera et al. 1999;Zhou et al. 1999).

Embryology

The mechanism that causes tracheoesophagealmalformations is still unclear; however, the devel-opment of reproducible animal models of theseanomalies has allowed detailed analysis of thevarious stages of faulty organogenesis (Zhouet al. 1999). By contrasting these stages withnormal development, it has been possible to iden-tify key developmental processes that may bedisturbed during embryogenesis (Roessleret al. 1996). It is generally accepted that the respi-ratory primordium appears as a ventral evagina-tion on the floor of the postpharyngeal foregut atthe beginning of the fourth week of gestation andthat the primitive lung buds are located at thecaudal end of this evagination. During a periodof rapid growth, the ventrally placed tracheabecomes separated from the dorsally placedesophagus (Roessler et al. 1996; Digilioet al. 1999). The presence of associatedmalformations could provide clues as to the pos-sible etiology of esophageal atresia(Chittmittrapap et al. 1980; Zhou et al. 1999;Marsh et al. 2000). Such malformations are pre-sent in 50 % of cases and can occur in distinctpatterns. These are nonrandom associations ratherthan syndromes because the presence of anoma-lies in one systemmakes it more likely that defectsexist in another. One of the best-described associ-ations is the VACTERL association, which

2 M. Messina et al.

comprises vertebral, anorectal, cardiac, tracheoe-sophageal, renal, and limb abnormalities. The pat-tern of these associations is likely to be dictated bythe timing of a possible insult that affects multiplemorphogenetic events (Chittmittrapap et al. 1980;Marsh et al. 2000; Mee et al. 1992).

Classification

In 1976 Kluth (1976) published an extensive Atlasof Esophageal Atresiawhich comprised ten majortypes, each with numerous subtypes which isbased on the original Vogt classification. Itwould appear to be more valuable to describe theanatomical anomaly rather than assign a label,which may not be widely recognizable (Fig. 1)(Driver et al. 2001; Poenaru et al. 1993).

Esophageal Atresia with DistalTracheoesophageal Fistula (86 %, VogtIIIb, Gross C)This is the most common variety in which theproximal esophagus, which is dilated, and themuscular wall thickened ends blindly in the supe-rior mediastinum at about the level of the third orfourth thoracic vertebra. The distal esophagus,which is thinner and narrower, enters the posteriorwall of the trachea at the carina or more com-monly 1–2 cm more proximally in the trachea.The distance between the blind proximal esopha-gus and the distal tracheoesophageal fistula variesfrom overlapping segments to a wide gap. Veryrarely the distal fistula may be occluded or oblit-erated leading to the misdiagnosis preoperatively

of an isolated atresia (Driver et al. 2001; Poenaruet al. 1993).

Isolated Esophageal Atresia WithoutFistula (7 %, Vogt II, Gross A)The proximal and distal esophagus ends blindlywithout any connection to the trachea. The prox-imal esophageal segment is dilated and thick-walled and usually ends higher in the posteriormediastinum at around the second thoracic verte-bra. The distal esophagus is short and ends avariable distance above the diaphragm. The dis-tance between the two ends will determinewhether a primary repair is feasible (rarely) orwhether a delayed primary anastomosis or anesophageal replacement should be performed. Itis important to exclude a proximal tracheoe-sophageal fistula in these cases (Driveret al. 2001; Poenaru et al. 1993).

Tracheoesophageal Fistula WithoutAtresia (4 %, Gross E)There is a fistulous connection between an ana-tomically intact esophagus and trachea. The fistu-lous tract may be very narrow or 3–5 mm indiameter and is commonly located in the lowercervical region. They are usually single, but twoand even three fistulas have been described(Driver et al. 2001; Poenaru et al. 1993).

Esophageal Atresia with ProximalTracheoesophageal Fistula (2 %, Vogt III,Gross B)This rare anomaly needs to be distinguished fromthe isolated variety. The fistula is not at the distalend of the upper pouch but is sited 1–2 cm above

Fig. 1 Anatomical types ofesophageal atresia

Esophageal Atresia of Newborns 3

the end on the anterior wall of the esophagus(Driver et al. 2001; Poenaru et al. 1993).

Esophageal Atresia with Proximaland Distal Tracheoesophageal Fistula(<1 %, Vogt IIIa, Gross D)In many of these infants, the anomaly wasmisdiagnosed managed as proximal atresia anddistal fistula as a result of recurrent respiratoryinfections; investigations carried out revealed atracheoesophageal fistula, previously mistakenfor a recurrent fistula. With the increasing useof preoperative endoscopy (bronchoscopy and/oresophagoscopy), early recognition of the doublefistula is made and total repair performed at theinitial procedure. If the proximal fistula is notidentified preoperatively, the diagnosis shouldbe suspected by a large gas leak emanatingfrom the upper pouch during the fashioning ofthe anastomosis (Driver et al. 2001; Poenaruet al. 1993).

Pathophysiology

The motility of the esophagus is alwaysaffected in esophageal atresia. The disorderedperistalsis more commonly involves the distalesophageal segment. Whether the motility dis-order is primarily due to abnormal innervationas evidenced by an abnormality in neuropeptidedistribution or secondary to vagal nerve dam-age occurring during the surgical repair remainsuncertain. The resting pressure in the wholeesophagus is significantly higher than in normalpatients, and the closing pressure of the loweresophageal sphincter is reduced. The trachea isalso abnormal in esophageal atresia. The abnor-mality consists of an absolute deficiency oftracheal cartilage and an increase in the lengthof the transverse muscle in the posterior tra-cheal wall. When severe, these abnormalitiesresult in tracheomalacia with collapse of thetrachea over a 1–2-cm segment in the vicinityof the fistula (Spitz et al. 1994; Lopezet al. 2006).

Associated Anomalies

Over 50 % of infants with esophageal atresia haveone or more additional anomalies. The systemsaffected are as follows:

• Cardiovascular 29 %• Anorectal anomalies 14 %• Genitourinary 14 %• Gastrointestinal 13 %• Vertebral/skeletal 10 %• Respiratory 6 %• Genetic 4 %• Other 11 %

There is an increased incidence of associatedanomalies in pure atresia (65 %) and a lowerincidence in H-type fistula (10 %). The VATERassociation first described by Quan and Smith in1973 (Quan and Smith 1972) consists of a com-bination of anomalies including vertebral,anorectal, tracheoesophageal, and renal or radialabnormalities. This association was laterexpanded as the VACTERL association to includecardiac and limb defects (Chittmittrapapet al. 1980; Merci et al. 1998; Criseraet al. 1999; Zhou et al. 1999; Roessleret al. 1996; Digilio et al. 1999; Marshet al. 2000). Other associations, which mayinclude esophageal atresia, are the CHARGEassociation (coloboma, heart defects, choanalatresia, retarded growth and development, genitalhypoplasia, and ear deformities), Potter’s syn-drome (renal agenesis, pulmonary hypoplasia,typical dysmorphic facies), and Schisis associa-tion (omphalocele, cleft lip and/or palate, genitalhypoplasia). Genetic defects associated withesophageal atresia include trisomies 21 and18 and 13q deletion. Of the cardiac anomalies,the most common are ventricular septal defectand tetralogy of Fallot. Major cardiacmalformations are one of the main causes of mor-tality in infants with esophageal atresia(Chittmittrapap et al. 1980; Marsh et al. 2000;Babu et al. 2000). The vertebral anomalies inesophageal atresia are mainly confined to the tho-racic region and are responsible for later

4 M. Messina et al.

development of scoliosis. The claim that the pres-ence of 13 ribs is associated with long-gap atresiahas not been substantiated. Of the gastrointestinalanomalies, the most frequently encountered areduodenal atresia and malrotation, while there isan increased incidence of pyloric stenosis. Mis-cellaneous anomalies include cleft lip and palate,omphalocele, lung abnormalities, choanal atresia,and hypospadias (Babu et al. 2000; Filstonet al. 1982a).

Genetic Counseling

The majority of cases of esophageal atresia aresporadic/non-syndromic, although a small num-ber within this non-familiar group are associated

with chromosomal abnormalities. Familial/syndromic cases of esophageal atresia areextremely rare. Esophageal atresia is two to threetimes more common in twins. The overall risk ofesophageal atresia recurrence in a sibling of anaffected child is about l % (Digilio et al. 1999;Marsh et al. 2000).

Clinical Description and Diagnosis

The diagnosis of esophageal atresia may besuspected prenatally by the finding of a smallor absent fetal stomach bubble on ultrasoundscan performed after the 18th week of gesta-tion. Overall the sensitivity of ultrasonographyis 42 %, but in combination withpolyhydramnios, the positive predictive valueis 56 % (Stringer et al. 1995; Shulmanet al. 2002). Available methods of improvingthe prenatal diagnostic rate include ultrasoundexamination of the fetal neck to view the blind-ending upper pouch and to observe fetalswallowing and magnetic resonance imaging(Langer et al. 2001; Litingtung et al. 1998;Cloud 1968). The newborn infant of a motherwith polyhydramnios should always have anasogastric tube passed soon after delivery toexclude esophageal atresia. Infants with esoph-ageal atresia are unable to swallow saliva andare noted to have excessive salivation requiringrepeated suctioning. A plain X-ray of the chestand abdomen will show the tip of the catheterarrested in the superior mediastinum (T2–T4),while gas in the stomach and intestine signifiesthe presence of a distal tracheoesophageal fis-tula (Fig. 2). The absence of gastrointestinalgas is indicative of an isolated atresia. A finebore catheter may curl up in the upper pouchgiving the false impression of an intact esoph-agus, or rarely it may pass through the tracheaand proceed distally into the esophagusthrough the fistula (Stringer et al. 1995; Langeret al. 2001).

Fig. 2 Plain X-ray (chest and abdomen) showing theradiopaque tube in the blind upper esophageal pouch. Airin the stomach indicates the presence of a distal tracheoe-sophageal fistula

Esophageal Atresia of Newborns 5

Management

Preoperative

Once the diagnosis of esophageal atresia hasbeen established, the infant will need to be trans-ferred to a pediatric surgical center. A suctioncatheter, preferably of the double lumen type, isplaced in the upper esophageal pouch to suctionsecretions and prevent aspiration. The infant isplaced on its side in the portable incubator whilemonitoring the usual vital signs. Vascular accessshould be provided as a precautionary measure,but intravenous fluid administration is not usu-ally necessary (Filston et al. 1982a). The preterminfant with respiratory distress requires specialattention. Clearly there is a need for endotrachealintubation and mechanical ventilation. In addi-tion there is the added risk of gastric over disten-tion and rupture of the stomach due to escape ofrespiratory gases down through the distal fistulainto the stomach due to the increased pulmonaryresistance. This sequence of events can be mini-mized by positioning the end of the endotrachealtube distal to entry of the tracheoesophageal fis-tula and by applying gentle low pressure venti-lation (Lopez et al. 2006; Filston et al. 1982a;Stringer et al. 1995). All infants with esophagealatresia should have an echocardiogram prior tosurgery. The ECHO and/or MR will define anystructural anomaly of the heart or great bloodvessels and occasionally may indicate a right-sided aortic arch, which occurs in 2.5 % ofcases. These diagnostic tests determine the sideof approach for the operative repair (Babuet al. 2000).

Risk Categorization and Prognosis(Outcome)

In 1962, Waterston proposed a classification ofinfants born with esophageal atresia into threegroups “with different chances of survival.” Theclassification based on birth weight, associatedanomalies, and pneumonia comprised:

• Group A: Over 5 1/2 lb (2,500 g) birth weightand well

• Group B:1. Birth weight 4–5 1/2 lb (1,800–2,500 g) and

well2. Higher birth weight, moderate pneumonia,

and congenital anomaly• Group C

1. Birth weight under 4 lb (1,800 g)2. Higher birth weight and severe pneumonia

and severe congenital anomaly

Over the last 40 years, there has been a steadyimprovement in the overall survival rate due toearly diagnosis and prompt referral, improve-ments in preoperative care and diagnosis andtreatment of associated anomalies, advances inanesthetic techniques, and sophisticated neonatalintensive care (Chittmittrapap et al. 1980). TheSpitz classification for survival in esophageal atre-sia comprises:

• Group I: Birth weight over 1,500 g with nomajor cardiac anomaly

• Group II: Birth weight less than 1,500 g ormajor cardiac anomaly

• Group III: Birth weight less than 1,500 g plusmajor cardiac anomaly

Major cardiac anomaly was defined as eithercyanotic congenital heart disease that requiredpalliative or corrective surgery or non-cyanoticheart anomaly that required medical or surgicaltreatment for cardiac failure (Spitz et al. 1994).

Selection for Nontreatment

Infants with Potter’s disease (bilateral renal agen-esis) syndrome and trisomy 18 which is fatal inthe first year of life in over 90 % of affectedinfants should be offered by opinion of no activetreatment. Similarly, infants with totallyuncorrectable major cardiac defects or withgrade IV intraventricular hemorrhage should beconsidered for nonoperative management.

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Emergency Ligation of the DistalTracheoesophageal Fistula

Generally, the operative correction of an esopha-geal atresia is not regarded as an emergency pro-cedure. The one exception is the preterm infantwith severe respiratory distress syndrome requir-ing ventilatory support. Ventilatory gases escap-ing down the distal fistula result in gastricdistension, which further impedes respiratoryfunction. With progressively increasing gastricdistension, the stomach may eventually rupturecausing a tension pneumoperitoneum, which ren-ders ventilatory support even more difficult (Spitzet al. 1994; Filston et al. 1982a; Stringeret al. 1995). Many maneuvers have been advo-cated to alleviate the problem including position-ing of the endotracheal tube distal to the fistula.However, if the fistula is sited at the level of thecarina, this maneuver is impossible to achieve.Others have advocated blocking of the fistula bya Fogarty catheter passed at bronchoscopy. Theaffected infants are usually preterm and in criticalrespiratory status. The smallest-caliber broncho-scope will not permit ventilation, while maneu-vering a Fogarty catheter into the distal esophagusin a cyanotic infant will aggravate the conditionand further exacerbate the hypoxia (Cloud 1968;Holmes et al. 1987).

Operative Approach

The operation is performed under general endo-tracheal anesthesia with dependable vascularaccess and employing gentle ventilatory pressureso as not to produce gastric distension (Holmeset al. 1987; Hagberg et al. 1986).

Intraoperative endoscopy: Preliminary bron-choscopy may be carried out to define the site ofentry of the distal tracheoesophageal fistula and toassess the presence of tracheomalacia. An alterna-tive is to carry out an esophagoscopy to dame thelength of the upper esophagus and to exclude anupper pouch fistula, which is more common withisolated esophageal atresia (Spitz et al. 1993;Spitz 1973; Yeung et al. 1992).

Position: The infant is placed on the left sidewith the right arm across the front of the chest for aright posterolateral thoracotomy.

Incision: A “conventional” incision centeredl cm below the inferior angle of the scapulaapproximately 5–6 cm long is made. The muscleof the chest wall may either be split or dividedwith electrocautery taking care to preserve thelong thoracic nerve to serratus anterior. The thoraxis opened through the fourth or fifth intercostalspace by dividing the intercostal muscles or byentry through the bed of the unresected rib.

An alternative incision is an axillary incision.This incision has been proposed by Bianchi andhas been used for most thoracic operations overthe years. The skin incision can be vertical orhorizontal although a horizontal incision is cos-metically more pleasing. The subcutaneous tissueand fascia are divided to expose the serratus ante-rior. Tissue planes are developed between thesuperficial fascia and the muscle layers. Careshould be taken to avoid the intercostobrachialand long thoracic nerves. Latissimus dorsi isretracted posteriorly, and the serratus anteriormuscle is elevated to expose the desired ribspace for thoracotomy (Bianchi et al. 1998).

Extrapleural approach: It has the advantage ofconferring protection of the pleural space in theevent of an anastomotic leak. Commencing pos-teriorly, the pleura is gently freed off the chestwall using blunt dissection. The dissection pro-ceeds into the mediastinum to provide goodaccess to the esophagus. The extrapleuralapproach is slightly more time-consuming andhas theoretically advantages over the transpleuralapproach still used by many surgeons (Cloud1968; Agrawal et al. 1999).

Exposure of the esophageal segments: Theazygos vein is the first structure encountered onentering the mediastinum. The azygos vein isgently mobilized and divided between ligaturesto expose the esophagus. The distal esophagususually lies directly deep to the azygos vein andis identified by the vagus nerve coursing over itsanterior aspect (Duranceau et al. 1977; Filleret al. 1992; Corbally et al. 1993).

Esophageal Atresia of Newborns 7

Repair of the defect: The distal esophagus ismobilized. A marking seromuscular suture isplaced in the lateral wall of the distal esophagusto assist with orientation. The distal esophagus isdissected to the level of the fistula, and the upperand lower extent of the fistula is marked with finenonabsorbable sutures before dividing the esoph-agus just distal to the fistula. The tracheal side ofthe fistula is closed with interrupted 5/0 sutures toachieve an airtight closure. The tracheal closuremay be tested by instilling warm saline over thesuture line while the anesthesiologist expands thelungs. An end-to-end anastomosis between theproximal and distal esophagus is fashioned usinginterrupted full-thickness fine sutures (Fig. 3).

If there is a wide gap, the distal esophagus canbe mobilized safely well down toward the dia-phragm. Just prior to the final suture being tied,a transanastomotic fine-caliber nasogastric tubemay be passed. This allows gastric decompressionin the early postoperative course and provides amute for early enteral feeding (Holmes et al. 1987;Spitz et al. 1993; Spitz 1996).

In our experience a very tense anastomosis canbe achieved in most cases, and if the infant issubsequently electively paralyzed and mechani-cally ventilated for approximately 5 days postop-eratively, the anastomosis will heal withoutleakage.

However, many methods have been proposedto overcome a wide gap:

• Intracorporeal bougienage• Extracorporeal stretching (Focker’s technique)• Circular myotomy (by Livatidis) or spiral

myotomy (Kimura’s technique)

Others authors have proposed tubularization ofthe upper pouch after creating a flap (Fig. 4).

Others, finally, prefer abandoning any attemptat initial primary anastomosis awaiting delayedprimary anastomosis 6–12 weeks later (Filstonet al. 1982b; Livitidis 1973). The thoracotomyincision is now closed with or preferably withoutintercostal drainage especially if the procedurehas been totally extrapleural, and a technicallysatisfactory anastomosis has been performed.

Thoracoscopic Approach

There is increasing experience with thethoracoscopic approach to repair esophageal atre-sia (Brandigi et al. 2013; Bax and van Der Zee2002; Rothenberg 2002, 2005a, b; Nguyenet al. 2006; Krosnar and Baxter 2005), but thisrequires advanced skills in minimal invasive sur-gery (Hagberg et al. 1986; Livitidis 1973; Puriet al. 1981). It is clear that there are many potentialadvantages of the thoracoscopic approach, as agood cosmetic appearance (minimal scarring), amagnification and excellent visualization, a lesspostoperative pain, and a uniform collapse of thelung that eliminates retraction trauma. However,there are also many disadvantages that must beconsidered by the surgeon. The main disadvan-tages are the small operative space and the need ofgood lung compliance that thoracoscopicapproach always requires. Also, most surgeonsuse a transpleural approach although anextrapleural approach has also been described(Rothenberg 2005a), and an anastomotic leakoccurs. Finally, single-lung ventilation is notalways achievable in neonates. For all these rea-sons, not all infants are suitable for thoracoscopicrepair. It is contraindicated in unstable infant orextremely small. Congenital heart disease andhyaline membrane disease when the infant isable to tolerate only short periods of single-lungventilation during ligation of the fistula should beconsidered relative contraindications (Rothenberg2005b).

The patient is placed in the left lateraldecubitus position, slightly prone, with the rightside elevated (Fig. 5). Three 5-mm trocars are

Fig. 3 The operative repair of an esophageal atresia anddistal tracheoesophageal fistula. (a) Ligature of the fistula.(b) Anastomosis

8 M. Messina et al.

placed. A 5-mm port is placed in the fifth inter-costal space in the posterior axillary, and the pneu-mothorax is created (0.5 l/min, 6 mmhg). A 30�

scope is introduced. Two working ports are placedin the third and sixth intercostal spaces in themidaxillary line. Sometimes a fourth port isrequired to help retract the lung.

Closure of the Tracheoesophageal FistulaThe chest is insufflated and the lung collapsed.The pleura overlying the posterosuperior medias-tinum is incised (transpleural approach), exposingthe azygos vein. The azygos vein is divided by a5-mm LigaSure. The lower esophageal pouch isidentified and traced in a proximal direction towhere it enters the back wall of the trachea. Thefistula can be ligated using a 4-0 Vicryl suture on

the tracheal side, and subsequently it is dividedwith scissors (Fig. 6).

Mobilization of the Upper PouchDownward pressure applied on the orogastrictube by the anesthetist helps identify the upperpouch. The pleura overlying the pouch isincised and the pouch mobilized toward theneck with a combination of blunt and sharpdissection. Mobilization can be continued intothe thoracic inlet, to gain additional esophageallength, depending on the gap that has to beovercome to achieve an esophageal anastomo-sis. The most dependent part of the upperesophageal pouch is opened.

Fig. 4 The operative repairof an esophageal atresia byflap

Fig. 5 Patient’s position during thoracoscopic approach

Fig. 6 TEF riveled after azygos vein dissection

Esophageal Atresia of Newborns 9

Esophageal AnastomosisThe anastomosis is performed using interruptedall-layer sutures (5/0 absorbable) tied. The knotcan be tied extracorporeally using a knot pusher orintracorporeally (Fig. 7). Once the posterior wallof the anastomosis has been completed, a catheter(6-Ch nasogastric tube) is introduced through themouth and advanced into the distal esophagus.This facilitates completion of anterior wall ofanastomosis.

Postoperative Management

In all other instances, regular pharyngeal suctionis necessary for the first few postoperative days.The suction catheter should be clearly marked toprevent the tube being passed to the site of theanastomosis and causing damage.Transanastomotic nasogastric feeds may be com-menced on the second or third postoperative day,and when the infant is swallowing saliva, oralfeeds may be started. A follow-up contrast studyis not regularly performed, but if there is anydoubt as to the integrity of the anastomosis, awater-soluble contrast study is carried out(Holmes et al. 1987). A chest tube is usually left(on the sixth intercostal space in the posterioraxillary line).

Complications and Outcomes

Anastomotic LeaksThese occur in 15–20 % of patients, but in onlyone-third or less is there a major disruption(Fig. 8). Major leaks occur in the early postoper-ative period (<48 h) and present with life-threatening tension pneumothorax. Minor leaksmay be detected on the routine contrast studyusually performed on the fifth to seventh daypostoperatively (Spitz et al. 1994; Cloud 1968).

Anastomotic StricturesAnastomotic stenoses develop in 30–40 % ofcases, most of which will respond to one or twodilatations (Fig. 9). Risk factors that have beenimplicated in stricture formation include anasto-motic tension, anastomotic leakage, and gastro-esophageal reflux. Endoscopic dilatation of thestricture can be carried out either at rigidesophagoscopy using semirigid bougies (Savary-Gilliard) of progressively larger caliber or by bal-loon dilatation introduced either at fluoroscopy orduring flexible endoscopy. Even if dilatations canbe considered a simple mini-invasive surgery, it isno free from risks. The main complication is theperforation of esophagus that it is anelastic andrigid (Fig. 10). For this reason, some surgeons, atthe end of the procedure, introduce contrast into

Fig. 7 (a) Nasogastric tube placed after performing anastomosis of the posterior wall; (b) completed anastomosis

10 M. Messina et al.

the esophagus to ensure that there has been noperforation and to establish the effectiveness ofthe dilatation (Livitidis 1973). Only rarely is itnecessary to resort to resection of an intractableanastomotic stricture.

Recurrent Tracheoesophageal FistulaThe incidence of recurrent tracheoesophageal fis-tula is between 5 % and 14 %.

A recurrent fistula should be suspected if theinfant manifests respiratory symptoms (coughingduring feeds, apneic or cyanotic episodes) or hasrecurrent respiratory infections after “successful”repair of the esophageal atresia (Spitz et al. 1994;Stringer et al. 1995; Filston et al. 1982b). Thediagnosis may be suspected on the plain chestradiograph, which shows an air esophagogram.Bronchoscopic examination will reveal the recur-rent fistula at the site of the original tracheoe-sophageal fistula. It is essential to pass a fine

Fig. 9 Anastomotic stricture in patient in 1 month aftersurgery (primary end-to-end anastomosis)

Fig. 8 Anastomotic leak in patient in tenth postoperativeday

Fig. 10 Perforation of esophagus during endoscopic dila-tation, in patient with originally EA type III long gap, whohad underwent primary anastomosis under tension

Esophageal Atresia of Newborns 11

ureteric catheter across the fistula into the esoph-agus and to view the catheter in the esophagus atendoscopy (Holmes et al. 1987).

Gastroesophageal Reflux (GOR)GOR is common in all infants following repair ofesophageal atresia – significant reflux occurs inaround 40 % of cases, about half of whom willrequire surgical management. GOR is more com-mon following anastomosis under tension andafter delayed primary repair (Parker et al. 1979).Incompetence of the lower esophageal sphinctermechanism may be due to a primary neurogenicdisturbance inherent in the development of theesophageal atresia or to technical factors involvedin the repair or it may be due to shortening of theintra-abdominal esophagus and/or abolishment ofthe gastroesophageal angle of His (Bax and vanDer Zee 2002; Rothenberg 2002; Nguyenet al. 2006).

Symptoms of GOR are similar to those ofrecurrent fistula with acute or chronic respiratoryproblems but also include recurrent vomiting andstricture formation. The diagnosis can beestablished on contrast swallow, pH monitoring,and endoscopy and biopsy of the distal esopha-gus. Anti-reflux medication including gastric acidsuppression is only successful in about half thecases. Surgical treatment is problematic given theinherent dysmotility present in the distal esopha-gus. A fundoplication may in itself produce afunctional obstruction at the gastroesophagealjunction. The failure rate of fundoplication carriedout in the first 3 months of life is excessively high.With regard to the nature of the wrap, there areadvocates for partial (Thal) as well as for a short,floppy wrap (Toupet or Nissen type) (Brandigiet al. 2013; Bax and van Der Zee 2002).

TracheomalaciaTracheomalacia may be defined as a structural andfunction weakness of the trachea resulting in par-tial and occasionally complete respiratoryobstruction. The structural abnormality comprisesa deficiency in the cartilage in the tracheal ringsand an increase in the length of the transverse

muscle. The result is that the airway collapsesduring expiration causing expiratory stridor,which varies in severity from a hoarse barkingcough to recurrent respiratory infection to acutelife-threatening episodes of cyanosis or apnea.The incidence of tracheomalacia is around 10 %,and about half will require surgical correction(Duranceau et al. 1977). The definitive treatmentconsists of aortopexy in which the ascending andarch of the aorta are elevated anteriorly toward thesternum. The result is usually dramatic withimmediate resolution of the obstruction to the airpassages.

DysmotilityThis affects the distal esophagus particularly inrelation to abnormal coordination of contractions,which in fact can be seen on contrast studies of theesophagus. The dysmotility is a major factor in thelong-term swallowing problems encountered inthese children (Agrawal et al. 1999).

Respiratory FunctionDuring infancy and for the first 3 years of life,patients with esophageal atresia suffer increasedfrequency of respiratory infections. The tendencyto respiratory infections has variously been attrib-uted to esophageal dysmotility and/or gastro-esophageal reflux with recurrent aspiration or toa primary respiratory abnormality (Filstonet al. 1982a; Stringer et al. 1995).

Esophageal Replacement

The need to replace the esophagus in esopha-geal atresia is extremely rare and should onlybe considered in very long-gap situations orwhere repeated attempts at retaining the hostesophagus have failed and the infant’s survivalis at risk (Spitz et al. 2004; Bax and van derZee 2007). There are basically three methods ofesophageal replacement currently being prac-ticed in children – gastric transposition, colonicinterposition, and jejunal interposition(Fig. 11).

12 M. Messina et al.

References

Agrawal L, Beardsmore CS, MacFadyen UM (1999)Respiratory function in childhood following repair ofoesophageal atresia and tracheoesophageal fistula.Arch Dis Child 81:404–408

Babu R, Pierro A, Spitz L et al (2000) The management ofesophageal atresia in neonates with right-sided aorticarch. J Pediatr Surg 35:56–58

Bax KM, van Der Zee DC (2002) Feasibility ofthoracoscopic repair of esophageal atresia with distalfistula. J Pediatr Surg 37:192–196

Bax NM, van der Zee DC (2007) Jejunal pedicle grafts forreconstruction of the esophagus in children. J PediatrSurg 42:363–369

Bianchi A, Sowande O, Alizai NK, Rampersad B (1998)Aesthetics and lateral thoracotomy in the neonate. JPediatr Surg 33:1798–1800

Brandigi E, Molinaro F et al (2013) Thoracoscopic repairof esophageal atresia with distal fistula in a new born: atechnical case report. J Siena Acad Sci 5. PublishedSince 1761:85–88

Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ (1980)Esophageal atresia and associated anomalies. Arch DisChild 64:364–368

Cloud DT (1968) Anastomotic technique in esophagealatresia. J Pediatr Surg 3:561–564

Corbally MT, Spitz L, Kiely E et al (1993) Aortopexy fortracheomalacia in oesophageal anomalies. Eur J PediatrSurg 3:264–266

Crisera CA, Connelly PR, Marmureanu AR et al (1999)Esophageal atresia with tracheoesophageal fistula:

suggested mechanism in faulty organogenesis. J PediatrSurg 34:204–208

Digilio MC, Marino B, Bagolan P et al (1999)Microdeletion 22q11 and esophageal atresia. J MedGenet 36:137–139

Driver CP, Shankar KR, Jones MO et al (2001) Phenotypicpresentation and outcome of esophageal atresia in theera of the Spitz classification. J Pediatr Surg36:1419–1421

Duranceau A, Fisher SR, Flye M (1977) Motor function ofthe esophagus after repair of esophageal atresia andtracheoesophageal fistula. Surgery 82:116–123

Filler RM, Messineo A, Vinograd L (1992) Severetracheomalacia associated with esophageal atresia:results of surgical treatment. J Pediatr Surg27:1136–1140

Filston HC, Chitwood WR Jr, Schkolne B, Blackmon LR(1982a) The Fogarty balloon catheter as an aid to man-agement of the infant with esophageal atresia andtracheoesophageal fistula complicated by severe RDSor pneumonia. J Pediatr Surg 17:149–151

Filston HC, Rankin JS, Kirks DR (1982b) The diagnosis ofprimary and recurrent tracheoesophageal fistulas: valueof selective catheterization. J Pediatr Surg 17:144–148

Hagberg S, Rubcnson A, Sillcn U, Werkmaster K (1986)Management of long-gap esophagus: experience withend-to-end anastomosis under maximal tension. ProgPediatr Surg 19:88–92

Holmes SJK, Kiely EM, Spitz L (1987) Tracheoesophagealfistula and the respiratory distress syndrome. PediatrSurg Int 2:16–18

Kluth D (1976) Atlas of esophageal atresia. J Pediatr Surg11:901–919

Fig. 11 Esophageal replacement. (a) Stomach, (b) jejunum, (c) colon

Esophageal Atresia of Newborns 13

Krosnar S, Baxter A (2005) Thoracoscopic repair of esoph-ageal atresia with tracheoesophageal fistula: anestheticand intensive care management of a series of eightneonates. Paediatr Anaesth 15:541–546

Langer JC, Hussain H, Khan A et al (2001) prenatal diag-nosis of esophageal atresia using sonography and mag-netic resonance imaging. J Pediatr Surg 36:804–807

Litingtung Y, Lei L, Westphal H, Chiang C (1998) Sonichedgehog is essential to foregut development. NatGenet 20:58–61

Livitidis A (1973) Esophageal atresia: a method ofoverbrindging large segment gaps. Z Kinderchir13:298–306

Lopez PJ, Keys C, Pierro A et al (2006) Oesophagealatresia: improved outcome in high-risk groups? JPediatr Surg 41:331–334

Marsh AJ, Wellesley D, Burge D et al (2000) Interstitialdeletion of chromosome 17 (del(l7)(q22q23.3)) con-firms a link with oesophageal atresia. J Med Genet37:701–704

Mee RBB, Beasley SW, Auldist AW, Myers NA (1992)Influence of congenital heart disease on management ofoesophageal atresia. Pediatr Surg Int 7:90–93

Merci J, Hasthorpe S, Farmer P, Hutson JM (1998) rela-tionship between esophageal atresia with tracheoe-sophageal fistula and vertebral anomalies inmammalian embryos. J Pediatr Surg 33:58–63

Nguyen T, Zainabadi K, Bui T et al (2006) Thoracoscopicrepair of esophageal atresia and tracheoesophageal fis-tula: lessons learned. J Laparoendosc Adv Surg Tech A16:174–178

Parker AF, Christie DL, Cahill JL (1979) Incidence andsignificance of gastroesophageal reflux followingrepair of esophageal atresia and tracheoesophageal fis-tula and the need for anti-reflux procedures. J PediatrSurg 14:5–8

Poenaru D, Laberge JM, Neilson JR, Guttman FM (1993)A new prognostic classification for esophageal atresia.Surgery 113:426–432

Puri P, Blake N, O’Donnell B, Guiney EJ (1981) Delayedprimary anastomosis following spontaneous growth ofesophageal segments in esophageal atresia. J PediatrSurg 16:180–183

Quan L, Smith DW (1972) The VATER association: ver-tebral defects, anal atresia, tracheoesophageal fistula

with esophageal atresia, radial dysplasia. Birth Defects8:75–78

Roessler E, Belloni E, Gaudenz K et al (1996) Mutations inthe human sonic hedgehog gene causeholoprosencephaly. Nat Genet 14:357–360

Rothenberg SS (2002) Thoracoscopic repair of tracheoe-sophageal fistula in newborns. J Pediatr Surg37:869–872

Rothenberg SS (2005a) Thoracoscopic repair of esopha-geal atresia and tracheoesophageal fistula. SeminPediatr Surg 14:2–7

Rothenberg SS (2005b) Esophageal atresia and tracheoe-sophageal fistula. In: Najmaldin A, Rothenberg S,Crabbe D, Beasley S (eds) Operative endoscopy andendoscopic surgery in infants and children. HodderArnold, London, pp 89–97

Shulman A, Mazkereth R, Zalel Y et al (2002) Prenatalidentification of esophageal atresia: the role of ultraso-nography for evaluation of functional anatomy. PrenatDiagn 22:669–674

Spitz L (1973) Congenital esophageal stenosis distal toassociated esophageal atresia. J Pediatr Surg 8:973–974

Spitz L (1996) Esophageal atresia: past, present, andfuture. J Pediatr Surg 31:19–25

Spitz L, Kiely E, Brereton RJ, Drake D (1993) Manage-ment of esophageal atresia. World J Surg 17:296–300

Spitz L, Kiely EM, Morecrofl JA, Drake DP (1994)Oesophageal atresia: at-risk groups for the 1990s. JPediatr Surg 29:723–725

Spitz L, Kiely E, Pierro A (2004) Gastric transposition inchildren a 21-year experience. J Pediatr Surg39:276–281

Stringer MD, McKenna KM, Goldstein RB et al (1995)Prenatal diagnosis of esophageal atresia. J Pediatr Surg30:l258–l1263

Yeung CK, Spitz L, Brereton RJ (1992) Congenital esoph-ageal stenosis due to tracheobronchial remnants: a rarebut important association with esophageal atresia. JPediatr Surg 27:852–855

Zhou B, Hutson JM, Farmer PJ et al (1999) Apoptosis intracheoesophageal embryogenesis in rat embryos withor without adriamycin treatment. J Pediatr Surg34:872–875

14 M. Messina et al.