Life-threatening anoxic spells caused by tracheal compression after repair of esophageal atresia: Correction by surgery

Three infants who developed anoxic spells 2, 5, and 20 mo following repair of esophageal atresia developed apneic spells during or within a few minutes of feeding. These episodes began with stridor and cyanosis; when severe, they progressed to apnea and loss of consciousness. Mouth-to-mouth resuscitation was often necessary. Investigations failed to detect esophageal obstruction and/or a recurrent tracheoesophageal fistula. No neurologic or cardiac abnormalities were found. The cause was compression of a 1- to 3-cm segment of trachea anteriorly by a vascular structure and posteriorly by a dilated esophagus that emptied slowly because of poor motility. Endoscopy confirmed the x-ray findings. The aortic arch and innominate artery were suspended to the sternum anteriorly, which relieved the apneic spells in all patients.     

Early short segment left colon interposition for esophageal atresia

Colon Interposition is frequently used for correction of esophageal atresia. The use of both retrosternal right colon^1–5 and transthoracic left or transverse colon^2,6–10 has been recommended. Retrosternal right colon interposition may be complicated by break downs and/or stricture of the anastomosis between cervical pharyngo-esophagus and the proximal interposed colon;^3–5,9 by vascular compromise and ischemia of the colon segment;^2,5 and by peptic ulceration of the distal interposed colon where it is anastomosed to the stomach.¹¹ Long-segment transthoracic left colon interposition may be attended by the first two complications as well;¹⁰ utilization of the esophageal stump for the distal colo-esophageal anastomosis in this technique may prevent gastroesophgeal reflux.^8–10,12Because of these problems, other methods of treatment have been suggested. The use of a gastric tube as esophageal replacement has been recommended;^13–15 however, peptic ulceration of the gastric esophagus may result.¹⁶ Elongation of the upper and, sometimes, the lower esophageal pouch with delayed primary anastomosis has been advocated.^17–19 Anastomotic leaks and/or stricture frequently accompany this procedure.Short-segment transthoracic left colon interpositions for esophageal stricture or varices have been attended by relatively fewer complications.^6–8 Therefore, a modified procedure was adopted for wide-gap esophageal atresia in 1968. A short segment of left colon was interposed transthoracically between the distal esophageal stump and proximal esophageal pouch in two infants after several weeks of bougienage had stretched the proximal pouch well below the aortic arch, so that a colo-esophageal anastomosis could be accomplished within the thorax without difficulty.     

Portal decompression by interposition mesocaval shunt in patients with biliary atresia

Surgical correction of portal hypertension in infants and children has often been frustrated by limitations imposed by the diminutive vessel size. During the past 18 mo, five patients from 1 to 7 yr of age and weighing between 9 and 19 kg, have required portal decompression for bleeding from esophageal varices. In two patients, a previous dissection in the area of the porta hepatis imposed a further technical obstacle. Both of these patients had biliary atresia and one had undergone a successful portoenterostomy (Kasai).¹ Success of the interposition mesocaval graft in adult patients led us to adopt this procedure in our five patients. Autogenous jugular vein was utilized for construction of the shunt in the smaller patients. Radiographic confirmation of shunt patency has been obtained 1 yr postoperatively.     

Circular Myotomy of the Esophagus: Clinical Application in Esophageal Atresia

Three infants born with esophageal atresia that was repaired by end-to-end anastomosis combined with a circular myotomy on the upper segment are reported. The distance between the free surgical margins of the esophagus ranged between 1.5 and 4.5 cm. The esophageal myotomy was used to reduce the tension on the anastomosis. The suture line healed in each patient without clinical or roentgenographic evidence of breakdown. Followup of these patients ranged between 10 months and 3 years. Roentgenographic evaluation of their esophageal motility showed efficient peristaltic activity in the distal esophageal segment. Two of the patients had a subsequent history of impaction of solid particles in the upper esophageal segment at the age of 13 months and 2 years. The possibility that the circular myotomy contributed to this increased incidence of impaction is raised.     

Management of the newborn with gastrointestinal anomalies and tracheoesophageal fistula

A standard approach to the otherwise healthy newborn with esophageal atresia and tracheoesophageal fistula has been well established. A significant number of these infants have additional gastrointestinal anomalies that necessitate alteration of the usual operative management. Over 2 1/2 years, nine such newborns have been managed. There were 17 additional gastrointestinal abnormalities, the most frequent being an imperforate anus (5), malrotation (5), and intestinal atresia (4). Management consisted of initial formal laparotomy, thoracotomy, and central line placement. In four infants these procedures were performed at a single sitting. Eight infants survived (89 percent); the single death resulted from intracerebral hemorrhage. Detection of coexisting gastrointestinal anomalies is mandatory in the management of the newborn with esophageal atresia. Standard local gastrostomy and subsequent thoracotomy may delay recognition of these associated anomalies, increase morbidity, and complicate subsequent correction. Preliminary abdominal exploration with correction, diversion, or both followed by thoracotomy, either immediate or staged, is indicated.     

Influence of birth weight on primary surgical management of newborns with esophageal atresia

BackgroundTo determine if birth-weight (BW) influences primary surgical management of newborns undergoing operation for esophageal atresia and tracheo-esophageal fistula (EA-TEF).MethodsNewborns undergoing repair of esophageal atresia at a single specialist centre between 1999 and 2017 were categorised into three groups based on BW; Group A < 1.5 kg, Group B <2.5 kg and Group C >2.5 kg. Outcome data analysed were (i) technical ability of the surgeon to perform primary esophageal anastomosis, (ii) anastomotic leak, (iii) anastomotic stricture, (iv) esophageal replacement, (v) need for other procedures notably fundoplication, aortopexy, tracheostomy and (vi) mortality. Statistical analysis was performed using a two-tailed Fisher's exact test and logistic regression.Results198 patients underwent surgery for EA-TEF during the study period, Group A (n = 13), Group B (n = 73) and Group C (n = 112). Inability to perform a primary anastomosis was significantly higher in Group A vs Group B (p = 0.003) and Group C (p = 0.004). Birthweight was a significant variable in the ability to perform a primary esophageal anastomosis (OR 1.009, p = 0.004). Mortality rate was significantly higher in Group A vs Group C (P = 0.0158).ConclusionsVery low birth weight infants are less likely to achieve a definitive primary anastomosis during emergent repair of esophageal atresia, and have a higher mortality.     

Lengthening technique for long gap esophageal atresia and early anastomosis

Background/Purpose: The treatment of long gap esophageal atresia remains a major surgical challenge. Whereas many approaches have been used for this problem, none are ideal. The authors used a technique originally described by Dr John E. Foker and accomplished early repair in 3 infants with long gap atresia. Methods: Three infants with esophageal atresia underwent thoracotomy shortly after birth and had a long gap preventing primary anastomosis. External traction sutures were placed on each esophageal pouch and exteriorized through the thoracic wall. The esophageal ends were approximated 1 to 2 mm daily by traction on the sutures. Anastomosis was performed when the 2 ends came together. Results: Three infants were included (31, 34, 37 weeks gestation, weights 1.38 kg, 1.9 kg, and 2.3 kg, respectively). The esophageal gaps were 3, 5, and 4.5 cm, respectively. Definitive anastomosis was performed at 14, 17, and 10 days, respectively. Two patients had anastomotic leaks that were treated conservatively. One patient had an esophageal stenosis that required dilatation. Conclusions: This technique allowed rapid esophageal lengthening in these 3 cases and led to early repair of long gap esophageal atresia, avoiding the need for a prolonged hospitalization or eventual replacement as well as long-term swallowing difficulties. J Pediatr Surg 38:737-739. [copy ] 2003 Elsevier Inc. All rights reserved.     

Atresia of the esophagus. New trends in the management of high-risk neonates

Once the reconstruction of esophageal atresia in infancy was reported, immediate repair became standard practice. High-risk infants carry an operative mortality of 30% to 80%. Staged surgical procedures were introduced to improve survival. "Delayed" reconstruction of esophageal atresia in selected cases has been reported to improve survival and eliminate staged surgical procedures. Between 1982 and 1986, 21 newborns were diagnosed as having esophageal atresia. Eight infants (32%) underwent "immediate" repair. In 13 infants repair was "delayed" for seven to 252 days. Four neonates with "pure" esophageal atresia underwent primary anastomosis, one was awaiting surgery, and another died in the postnatal period. As more high-risk infants survive the perinatal period, surgical reconstruction must be planned to maximize operative survival. The goal of delayed management of esophageal atresia is to restore intrinsic esophageal continuity.     

Atresia of the colon.

Eleven infants with colon atresia have been managed by staged procedure with survival and good health in ten. Complications have been relatively few. At the initial operation the proximal atretic segment is exteriorized as an end colostomy. Such a procedure is simple and safe, results in rapid relief of the obstruction, and permits normal feeding by mouth within a few days. At a second procedure several weeks or months later, intestinal continuity is established by an end-to-end or end-to-side anastomosis. Gastroschisis has been an associated anomaly in four of these cases. This association gives additional support to the concept that intrauterine interference to the blood supply to a segment of intestines is the etiologic factor responsible for such atresias.     

Surgical treatment of congenital esophageal atresia

Severe gastroesophageal reflux was found in 9 out of 18 infants with congenital esophageal atresia studied radiographically shortly after primary reconstruction. Pulmonary complications were recorded in 18 out of 32 similar patients in long-term follow-up. Strictures at the level of the anastomosis were detectable in 18 out of 32 patients; eleven strictures were severe enough to require dilation or surgical revision. These findings suggest that early evaluation for gastroesophageal reflux may be useful in management of infants with esophageal atresia. The precautions taken preoperatively to prevent complications of gastroesophageal reflux should be continued in the postoperative interval unless a competent lower esophageal sphincter is demonstrated.     

Thirty-five-year institutional experience with end-to-side repair for esophageal atresia

HYPOTHESIS: End-to-side repair (ES) with ligation of the tracheoesophageal fistula (TEF) reduces the risks of stricture and gastroesophageal reflux disease requiring operation compared with the end-to-end repair of esophageal atresia and distal TEF. DESIGN: Case series with institutional and historical control subjects. SETTING: Referral children's hospital. PATIENTS: One hundred thirty-four infants diagnosed as having esophageal atresia and distal TEF between June 30, 1968, and July 1, 2003. INTERVENTIONS: Ninety-six infants having ES and 38 having end-to-end repair. MAIN OUTCOME MEASURES: Patients were studied for overall survival, surgical complications, and well-being during the first year of life. RESULTS: Survival was 95% vs 90% (patients undergoing ES vs end-to-end repair). Complications included anastomotic leak, 8% vs 13%; recurrent TEF, 7% vs 3%, with only 1 recurrence in the last 28 patients having ES; anastomotic stricture (requiring dilatation), 5% vs 13%; gastroesophageal reflux disease requiring operation, 6% vs 18%; and esophageal dysmotility, which was present following nearly all ES and end-to-end procedures. Tracheomalacia-related respiratory symptoms following ES decreased from 50% to 11% at 1 year of age. Age-appropriate diet following ES was achieved in 93% by 1 year; 5% experienced occasional dysphagia or choking episodes. CONCLUSIONS: The ES operation is accompanied by a reduced rate of stricture and gastroesophageal reflux disease requiring operation compared with end-to-end repair. Earlier concerns regarding an unacceptable risk of recurrent TEF were not substantiated.     

Ongoing cirrhosis after successful porticoenterostomy in infants with biliary atresia.

Thirty-one patients were treated by portoenterostomy procedures for extrahepatic biliary atresia. In 20, there was unequivocal evidence of bile drainage. Eleven of these 20 were restudied from 3 to 23 mo postoperatively by liver biopsy. The clinical condition of most of these infants was satisfactory. Despite absence of clinical jaundice, normal rose bengal scan, and normal liver function studies, eight showed progressive degrees of hepatic fibrosis when compared to the results of the biopsy taken at the time of portoenterostomy. Two patients showed stable histology and one, after progressing on a second biopsy, had normal liver histology on a third study. The implications of progressive hepatic histopathology despite bile drainage are discussed.     

Hypogastrinemia and esophageal atresia.

To investigate extraesophageal anomalies in infants with esophageal atresia, preoperative plasma gastrin was measured in 12 infants. The median plasma gastrin was 32 ng/L (interquartile range, 24 to 44). There was significant correlation with birth weight (rs = .73, P less than .05) and gestational age (rs = .74, P less than .05). Within this group 9 infants of greater than 36 weeks' gestation were matched to a group of 20 control infants without esophageal atresia. Infants with esophageal atresia had a significantly lower median plasma gastrin (38 ng/L v 55 ng/L, P less than .05). This may indicate preexisting vagal abnormalities in esophageal atresia unrelated to surgical intervention.     

A further look at the gastric tube as an esophageal replacement in infants and children

Gastric tubes were constructed for infants with pure esophageal atresia as well as for “impossible” or “failed” repairs of esophageal atresia, and for children with esophageal stricture as well. More than two-thirds of the patients had the gastric tube operation before 2 yr of age (esophageal atresia). The children with esophageal strictures had the operation usually between 2 and 10 yr. The two-stage retrosternal reversed gastric tube was used most frequently. The isoperistaltic tube is an excellent alternate method should a reversed gastric tube be impossible to construct. If the gastric tube is short and used as an esophageal interposition in a patient with peptic esophagitis, there is a good possibility of developing esophagitis above the esophagogastric tube anastomosis. The most common complications that arise are related to the esophagogastric tube anastomosis (leak and stricture). We have found no correlation between the operative technique, the postoperative management as it concerns esophageal suction, and the eventual early or late anastomotic leak. Nor were these factors related to the time of eventual closure of the leak, the presence and severity of any subsequent stricture, and the results of its dilatation. The longest follow-up is $\text{7}\text{1}\text{2}$ yr. Once the esophagogastric tube anastomosis has been healed and asymptomatic for 1 yr, the child is well on the way to a life of normal swallowing.     

Evaluation of gastroesophageal function and mechanisms underlying gastroesophageal reflux in infants and adults born with esophageal atresia

Purpose

To evaluate the mechanisms underlying gastroesophageal reflux (GER) following esophageal atresia (EA) repair and gastroesophageal function in infants and adults born with EA.

Methods

Ten consecutive infants born with EA as well as 10 randomly selected adult EA patients were studied during their first postoperative follow-up visit and a purposely planned visit, respectively. A ¹³C-octanoate breath test and esophageal pH–impedance–manometry study were performed. Mechanisms underlying GER and esophageal function were evaluated.

Results

Transient lower esophageal sphincter relaxation (TLESR) was the most common mechanism underlying GER in infants and adults (66% and 62%, respectively). In 66% of all GER episodes, no clearing mechanism was initiated. On EFT, normal motility patterns were seen in six patients (four infants, two adults). One of these adults had normal motility overall (> 80% of swallows). Most swallows (78.8%) were accompanied by abnormal motility patterns. Despite this observation, impedance showed normal bolus transit in 40.9% of swallows. Gastric emptying was delayed in 57.1% of infants and 22.2% of adults.

Conclusions

TLESR is the main mechanism underlying GER events in patients with EA. Most infants and adults have impaired motility, delayed bolus clearance, and delayed gastric emptying. However, normal motility patterns were seen in a minority of patients.     

Crucial bronchoscopic findings in esophageal atresia and tracheoesophageal fistula

We performed a brief bronchoscopy in 42 newborn infants with esophageal atresia and tracheoesophageal fistula (TEF). The procedure was carried out in the operating room, usually just prior to thoracotomy for repair of the anomaly. Most infants had a gastrostomy tube placed initially. Observations included: (1) level of fistula, (2) presence of unusual variants, eg, double fistula, trifurcation fistula, (3) presence and severity of tracheobronchitis, and (4) position of the aortic arch. The endoscopic findings influenced the operative technique or management of 24 of the 42 infants (57%), including 13 infants (31%) with crucial findings which dictated a change in operative technique or management. Examples of the latter were an unsuspected cervical fistula associated with esophageal atresia, repaired by a cervical approach; other unusual variants of TEF (proximal fistula, trifurcation or quadrifurcation TEF), requiring specific dissection at thoracotomy; congenital stenosis of the right mainstem bronchus, requiring postoperative dilatation; or severe tracheobronchitis, which contraindicated thoracotomy. Observations at bronchoscopy accurately predicted the position of the aortic arch in ten recent infants, although the side of dominant pulsation was indeterminate in three other infants. Only one minor complication was attributable to the bronchoscopy. The procedure appeared to be safe and beneficial, and should be considered for all infants with esophageal atresia and TEF.     

Complete vs partial fundoplication in children with esophageal atresia

Purpose

The purpose of the study was to compare outcomes after partial vs complete fundoplication in patients with prior esophageal atresia repair.

Methods

All patients undergoing fundoplication following esophageal atresia repair at a tertiary care pediatric hospital from 1987 to 2006 were retrospectively reviewed. All children had at least 1 year of follow-up postfundoplication.

Results

Of 47 children, 31 (66%) had a partial fundoplication and 16 (34%) had complete fundoplication. Demographics, presence of tracheoesophageal fistula, early complications of esophageal atresia repair, gastroesophageal reflux symptoms before fundoplication, and operative details of fundoplication were statistically similar between groups, except for the frequency of hiatus repair during fundoplication (23% vs 69%, P = .004). Patients were followed for a median of 4.98 years (range, 1-17.8 years). Postfundoplication symptoms of vomiting (39% vs 31%), dysphagia (45% vs 38%), retching (10% vs 25%), abnormal findings on barium study, and need for reoperation (19% vs 13%) were not statistically different between groups. However, a greater proportion of children undergoing partial fundoplication achieved long-term symptom- and medication-free recovery (52% vs 13%, P = .012).

Conclusions

Our data suggest that partial fundoplication is associated with a greater likelihood of symptom- and medication-free recovery than complete fundoplication in children with previously repaired esophageal atresia.     

Colon interposition for the replacement of the esophagus in children.

Thirty-two new cases of colon interposition for esophageal reconstruction are presented. Only one late death occurred in this series, which was unrelated to the colon interposition. Fifteen severe complication were observed in five patients with preexisting esophageal atresia and previously complicated courses following disastrous primary esophageal repairs. The most common complication was pneumonia which occurred 13 times. Five late strictures responded well to dilatation or revision. Long-term weight gain was compared between a group of colon transplant patients and a group of patients with repaired esophageal atresia. Although the esophageal atresia group followed the 25th percentile and the colon interposition group followed the 3rd percentile on the growth curve, no significant difference existed in weight gain over 12 yr.     

The outcome of conservative treatment for anastomotic leakage after surgical repair of esophageal atresia

ObjectiveThe aim of this study was to evaluate the clinical outcome of conservative management of anastomotic leakage (AL) after surgical repair for esophageal atresia.MethodsData from 85 neonates with esophageal atresia who underwent surgical correction were retrospectively analyzed. Conservative treatment had been adopted for AL. The incidence and severity of postoperative AL as well as its effects were analyzed.ResultsAmong the 85 neonates, postoperative AL occurred in 21 (25%) cases, with major leaks in 15 cases and minor leaks in 6. The stricture index of the 21 neonates with AL (0.615 ± 0.032) was significantly different (P = .008) from that of the 64 neonates without leakage (0.509 ± 0.018). The overall incidence of gastroesophageal reflux (GER) was 36%. Esophageal dysmotility and clinically significant tracheomalacia were observed in 69 and 7 infants, respectively, of the 80 surviving patients. The incidence of GER, dysmotility, and tracheomalacia in patients with or without AL was similar. The severity of GER in patients with different numbers of sessions of dilation was significantly different (P = .0015).ConclusionsPostoperative esophageal AL is effectively treatable by conservative methods in most neonates. The occurrence of AL may aggravate the severity of esophageal stricture but does not affect the incidence of GER, esophageal dysmotility, and tracheomalacia.     

Intracardiac repair of tetralogy of Fallot in patients under 5 years of age

Thirty-six infants and small children less than 5 years of age with severe symptoms underwent intracardiac repair for tetralogy of Fallot between January, 1969, and June, 1973, at the Children's Hospital of Buffalo. Of the 36, 24 had primary correction and 12 had a secondary correction after an initial palliative procedure. Since February, 1970, only 5 infants have undergone palliation, the contraindication for total repair being hypoplasia of the distal pulmonary arteries and anomalies of the right coronary arterial system which precluded outflow patching. Three patients who were first seen with clinical, hemodynamic, and angiographic characteristics of pulmonary atresia with ventricular septal defect (Group B) have been included because of demonstrable but rudimentary anatomical continuity between the right ventricle and pulmonary artery. Of the remaining 33 patients (Group A), 22 underwent primary repair without mortality, and 11 had secondary correction with 1 death. Eight infants under 2 in Group A underwent primary correction; the youngest was 5 weeks old. An outflow patch of pericardium was employed in 13 of the 33 children in Group A and in all 3 in Group B. It was observed that the major determinant of successful repair was an annulus diameter larger than 12 mm. Deep hypothermia and circulatory arrest were employed in 20 children under 15 kg. in weight. Heart block was not encountered, and an outflow tract aneurysm was seen in 1 patient who had no patch. It is concluded that primary intracardiac repair is advisable for patients in those centers prepared for infant intracardiac surgery.