John E. Foker

Development of a true primary repair for the full spectrum of esophageal atresia.

OBJECTIVE To determine whether or not a true primary repair, without myotomies and with the gastroesophageal junction below the diaphragm, can be accomplished across the esophageal atresia (EA) spectrum. Our hypothesis is that the esophageal anastomosis can withstand significant tension. The consequences, particularly for those patients with a very long gap atresia, were assessed. SUMMARY OF BACKGROUND DATA Difficulties arise roughly in proportion to the size of the gap between esophageal segments. Reported surgical complications remain frequent, and particularly at the far end of the EA spectrum, not all children are left with a satisfactorily functioning esophagus or esophageal substitute. METHODS The outcomes of all infants who had a true primary repair of EA from 1976-1997 were determined. Surgically, the methods used to achieve a reliable true primary repair were expanded to accomplish this, even for a very long gap EA. RESULTS From 1976-97, 70 infants with or without associated tracheoesophageal fistula (TEF) had primary repairs performed with no surgery-related deaths and 11% later deaths. No interpositions were performed since 1983. There were no discernible anastomotic leaks and one late recurrent TEF related to the early use of balloon dilation. Ten infants had gaps of 5.0-6.8 cm and, among these, four had gaps of 5.5-6.8 cm that could not be pulled together initially. Traction sutures in the esophageal ends, however, produced sufficient lengthening within 6-10 days for a true primary repair. Very long gap repairs produced more reflux (10 of 10 required a fundoplication versus 24 of 70 overall) and more dilations to relieve strictures. Two infants underwent stricture resection with no recurrence. On follow-up, all patients over 2 years of age were eating well or satisfactorily, and none had a gastrostomy tube. CONCLUSIONS (1) The esophageal anastomosis can withstand considerable tension and allows a reliable true primary repair for the full EA spectrum. (2) Growth is rapid and traction sutures will produce significant esophageal lengthening within days. (3) With increasing tension, gastroesophageal reflux (GER) and strictures are more common; however, both are treatable. Follow-up reveals the benefits of true primary repair over other solutions.

Long-gap esophageal atresia treated by growth induction: the biological potential and early follow-up results

This study had two purposes. The first was to determine whether the growth procedure would allow true primary repairs of the most severe end of the esophageal atresia (EA) spectrum with the longest gaps (LG) and most rudimentary lower esophageal segments. The second goal was to provide the first short- to mid-term (3-12 years) follow-up data on the esophageal function and quality of life (QOL) data on the patients in this series. From our series of 60 LG-EA patients who underwent a growth procedure, 42 had the true primary esophageal repair completed 3 years ago. Among these, 18 had gaps over 6 cm, and for 6, only a rudimentary lower esophagus existed well below the diaphragm. No patient was turned down and all had primary repairs. These results suggest that even the most rudimentary segment has the potential to achieve normal size and that the full EA spectrum can have a primary repair. Our follow-up studies indicated that the esophageal function of these previously grown segments was very good. All contacted (40) were eating normally with only 3 receiving supplemental g-tube feeds because of other significant defects. We have actively treated significant reflux and 41/42 had fundoplication. By endoscopy (N = 15) no esophagitis was visible, but on biopsy, mild inflammation was found in 3. No conditions were found which would suggest that there would be a late deterioration or adverse consequences would arise. Based on these ongoing evaluations, the outlook seems very favorable for a good long-term QOL.

Primary repair of ultra-long-gap esophageal atresia: Results without a lengthening procedure

Ultra-long-gap esophageal atresia, defined as a gap length of 3.5 cm or greater, has proved difficult to repair. When primary repair has been attempted, even with bougienage, circular myotomy, or intraabdominal esophageal mobilization to lessen anastomotic tension, leaks, anastomotic disruptions, and recurrent tracheoesophageal fistulas are frequent. Consequently, interposition grafts are commonly used. For long-term function the intact native esophagus should be preferable to an interposition graft or the consequences of circular myotomy. Therefore, even when an ultra-long gap is present, we have carried out a primary repair using our single-layer technique without myotomies. Since 1979, 8 of 58 infants (14%) with esophageal atresia had gaps ranging from 3.5 to 6 cm. All had a primary repair with follow-up from 1 to 11 years. Despite severe anastomotic tension in all cases, there were no anastomotic leaks, disruptions, recurrent tracheoesophageal fistulas, or deaths. The tension, however, may have led to major gastroesophageal reflux in 5 of 8 patients (62.5%), all treated by a Nissen fundoplication, and a stricture in 4 of 8 infants (50%). Three strictures responded to dilation and one was resected. Now, all children are eating a normal diet for age. In conclusion, this technique has allowed primary repair of ultra-long-gap esophageal atresia. Although the severe tension may contribute to strictures needing dilation and gastroesophageal reflux requiring fundoplication, primary repair resulted in a clinically functional native esophagus.

Echocardiographic versus cardiac catheterization diagnosis of infants with congenital heart disease requiring cardiac surgery

The success of noninvasive preoperative evaluation of infants with congenital heart disease using cardiac ultrasound depends not only on diagnostic accuracy, but also on risk of morbidity and mortality as compared with infants who undergo cardiac catheterization. Fifty-six infants (age 10 weeks or younger) with coarctation of the aorta (n = 16), coarctation with ventricular septal defect (n = 12), valvar aortic stenosis (n = 10) or total anomalous pulmonary venous connection (n = 18) were examined. Thirty-one underwent noninvasive preoperative assessment and 25 underwent evaluation including cardiac catheterization. Age, level and duration of support, pH, renal function, mortality, complications of cardiac catheterization and errors of diagnosis were compared. Significant differences between the 2 groups were more frequent preoperative use of prostaglandin E1 and shorter hospital stay in the noninvasively evaluated coarctation group. Of the infants with coarctation and ventricular septal defect, 1 who had cardiac catheterization required renal transplantation and 1 evaluated noninvasively required surgery at age 3 months for mitral stenosis not discovered on preoperative evaluation. One noninvasively evaluated infant with total anomalous pulmonary venous connection had a stenotic communication between the pulmonary venous confluence and the left atrium not detected by ultrasound. Surgery was successful in the latter 2 infants. Noninvasive preoperative diagnosis of some infants with congenital heart disease can be performed without increasing the risk of operative morbidity and mortality. Eliminating cardiac catheterization reduces hospital costs, decreases total numbers of catheterizations performed and influences the structure of training programs.

Enhanced high energy phosphate recovery with ribose infusion after global myocardial ischemia in a canine model

High energy phosphate levels are depressed following global ischemia and require several days to completely recover. Short-term methods to enhance ATP recovery have included infusion of ATP precursors, inhibition of enzymes that catabolize AMP, and membrane transport stabilization. Several precursors have been used to augment adenine nucleotide synthesis including adenosine, inosine, adenine, and ribose. Because of the short-term nature of previous experiments, recovery had been incomplete and the effects in the intact animal unknown. The purpose of this study was to determine the effects of ribose infusion in a long-term model of global ischemia and attempt to identify the precursor which limits myocardial ATP regeneration in the intact animal. Global myocardial ischemia (20 min, 37 degrees C) was produced in dogs on cardiopulmonary bypass. With reperfusion either ribose (80 mM) in normal saline or normal saline alone was infused at 1 ml/min into the right atrium and the animals were followed for 24 hr. Ventricular biopsies were obtained through an indwelling ventricular cannula prior to ischemia, at the end of ischemia, and 4 and 24 hr postischemia and analyzed for adenine nucleotides and creatine phosphate levels. Radiolabeled microspheres were used to measure myocardial and renal blood flows and no significant difference was found between ribose-treated control groups. In both groups, myocardial ATP levels fell by at least 50% at the end of ischemia. No significant ATP recovery occurred after 24 hr in the control dogs, but in the ribose-treated animals, ATP levels rebounded to 85% of control by 24 hr.(ABSTRACT TRUNCATED AT 250 WORDS)

Esophageal stenting in children: indications, application, effectiveness, and complications

BACKGROUND Use of esophageal stents is uncommon in children, and there are few reports. We report the first experience in predominantly small children and infants with retrievable, flexible stents designed for tracheobronchial use. OBJECTIVE Evaluation of initial experience with placement of esophageal stents for benign esophageal disorders in children. DESIGN A retrospective study. SETTING A pediatric, academic, tertiary-referral center. PATIENTS This study involved 7 pediatric patients. INTERVENTIONS Covered tracheobronchial stents were endoscopically placed in pediatric patients with benign esophageal conditions. Removal involved using forceps to pull the purse-string suture into the endoscope channel and collapsing the top of the stent for easy removal. MAIN OUTCOME MEASUREMENTS To evaluate the safety and feasibility of performing endoscopic stent placement in children and to establish criteria for early stent removal. RESULTS Six of 7 patients benefitted from stenting. There were no complications of placement. Novel techniques were developed for difficult retrievals. One patient did not benefit from esophageal stent placement, because the stent migrated downward from the uppermost part of the esophagus. One patient had some gagging, which led to early removal of the stent. A stent was removed emergently in 1 patient for respiratory distress. LIMITATION Small number of patients. CONCLUSIONS Retrievable, covered stents are easily placed and removed from the esophagus in small children. They should be considered for severe unrelenting strictures, especially when associated with esophageal leaks. A need exists for development of esophageal stents designed for pediatric use.

Staged repair of interrupted aortic arch and ventricular septal defect in infancy

Staged repair of interrupted aortic arch and ventricular septal defect was carried out in 20 infants from 1979 through 1990. Among the important associated cardiac defects were transposition of the great arteries, truncus arteriosus, and anomalous origin of the right pulmonary artery. The first stage, usually consisting of the placement of an 8- or 10-mm polytetrafluoroethylene graft, pulmonary artery banding, and ligation of the patent ductus arteriosus, resulted in 20 survivors (100%) There were two interim deaths (10%) before the second stage of ventricular septal defect closure and pulmonary artery band removal, which had 15 survivors (83%, 15/18). Because the major morbidity and mortality early in this experience could be traced to leaving the pulmonary artery band on too long, early removal (within 2 to 3 months) was begun. Since 1985, 8 (100%) of 8 infants have survived both stages and are now doing well. Because of the relatively large polytetrafluoroethylene graft, only 1 child (aged 9 years) has experienced substantial late aortic arch obstruction and undergone placement of an 18-mm Dacron graft without difficulty. Of interest is the finding that in only 1 (5%) of the 20 patients has major (greater than or equal to 40-mm Hg gradient) left ventricular outflow tract obstruction developed. In summary, the staged repair of interrupted aortic arch with ventricular septal defect has become very reliable despite the condition of the infant or major associated cardiac anomalies and can be recommended for infants at high risk for primary repair. More long-term information will be needed to determine which approach will be the best choice for the majority of infants.

Aortic arch anomalies associated with long gap esophageal atresia and tracheoesophageal fistula

PURPOSE The purpose of this study was to determine whether aortic arch anomalies are associated with long gap esophageal atresia and tracheoesophageal fistula (EA-TEF). METHODS The authors performed a retrospective review of all infants who had EA-TEF from 1980 to 1996 at two pediatric surgery centers. Two hundred three infants who had EA-TEF were identified. RESULTS Twelve infants were noted to have both long gap EA-TEF defined as a gap length greater than 3 cm and aortic arch anomalies. Of these 12, 7 had aberrant right subclavian arteries originating from the descending aorta. Four of the seven infants who had aberrant right subclavian artery (SCA) had gap lengths greater than 4 cm. All four had their fistulae divided initially through a right thoracotomy with primary repair performed at a later date. The remaining five infants who had long gap EA-TEF had right-sided aortic arch with aberrant left subclavian arteries. All five initially underwent exploration through the right chest. On discovery of the long gap EA and concurrent vascular anomaly, the thoracotomies were closed, and the infants underwent definitive repair of both their EA-TEF and their vascular anomaly through a left thoracotomy. CONCLUSIONS The authors find that aortic arch anomalies are associated with long gap EA-TEF. Patients who have these two anomalies tend to have a long gap. Preoperative diagnosis of these anomalies may alter the timing and technique of surgical intervention. The embryogenesis of these vascular lesions may account for this more severe form of esophageal atresia.

31P NMR measurement of ATP synthesis rate in perfused intact rat hearts

Using 31P NMR and the saturation‐transfer method, the unidirectional rate of ATP synthesis was measured in isolated, Langendorff‐perfused, isovolumic rat hearts operating at a rate pressure product of 25.6 ± 2.5 (SE) × 103 mmHg · min−1 and consuming O2; at a rate of 35 ± 2 μmol O2 · min−1. (g dry wt)−1, at 37°C. This rate was 7.2 ± 0.9 μmol · s−1 · (g dry wt)−1 and was related to the rate of oxygen atom consumption by a ratio of 6.3 ± 0.9. These date show that in the intact heart the unidirectional rate of ATP synthesis exceeds the net rate of ATP synthesis and consumption by approximately a factor of 2.

Surgical approaches to aortopexy for severe tracheomalacia

PURPOSE The purpose of this study was to determine the outcomes among three different surgical approaches for performing an aortopexy to treat severe tracheomalacia (STM). METHODS A retrospective review was performed for all patients who underwent an aortopexy by pediatric surgeons at a single institution during 1997-2012. Data collected included details of the operative approaches and clinical results. The data were analyzed using Chi-square and Fisher exact test. RESULTS Forty-one patients underwent an aortopexy. The operation was chosen by the surgeon and not randomized. Exposure was by partial sternotomy (PS) (20), open thoracotomy (12), or thoracoscopic approach (7). Only the PS approach was done by a single team. All groups showed improvement in work of breathing, prevention of severe respiratory distress, and acute life threatening events. These effects were more dramatic for the PS group, especially regarding oxygen and/or ventilator dependence and the ability to undergo tracheostomy decannulation. Among the sixteen patients with failure-to-thrive before successful aortopexy by any technique, ten demonstrated significant improvement in their growth (p=0.025). The recurrence rate for the thoracoscopic approach was 38%, and there were no recurrences in the partial sternotomy and the thoracotomy groups, 38% vs 0% vs 0%, p=0.005. Simultaneous bronchoscopy was utilized more commonly in the PS group compared to the thoracotomy and thoracoscopic group, 95% vs 62% vs 38%. CONCLUSIONS In this series, the partial sternotomy technique had the most reliable resolution of symptoms and no recurrence requiring reoperation. The PS approach to STM has the technical advantages of an improved exposure with equal access to the vessels over the right and left mainstem bronchi, as well as the trachea and a more specific elevation of the arteries, including suspension of the pulmonary arteries and trachea itself when desirable. Simultaneous bronchoscopy during aortopexy and an experienced team also likely contribute to improved outcomes. The variations in populations, follow-up, and use of continuous intraoperative bronchoscopy, however, make firm conclusions difficult.