The Journal of Pediatric Surgery: Its First 50 Years

This article is based on the Journal of Pediatric Surgery Lecture at the 2017 meeting of the British Association of Pediatric Surgeons. This lecture was renamed The Jay L. Grosfeld /Journal of Pediatric Surgery Lecture in honor of Dr. Jay L. Grosfeld who was Editor-in-Chief of the Journal of Pediatric Surgery (JPS) from 1994 until his passing in 2016. The first part of this paper recounts the first 50 years of JPS starting with the efforts of Drs. Koop and Gans to start a Journal devoted to Pediatric Surgery. The second part of the paper reviews the top ten citations in the first 50 years of JPS. Finally, the last part of the article discusses what the future may hold for JPS.     

Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium

Purpose

Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF.

Esophageal atresia, duodenal atresia, and unilateral lung agenesis: a case report

The association of pure esophageal atresia, duodenal atresia, and unilateral lung agenesis has not been reported previously. Here the authors present a case of a newborn with this constellation of anomalies that underwent staged repair. The primary principle guiding treatment was the avoidance of iatrogenic injury to the single lung. Therefore, the order of operations proceeded as follows: (1) placement of a decompressing gastrostomy tube, (2) repair of the duodenal atresia, and (3) repair of the esophageal atresia. The congenital closed loop obstruction caused by the esophageal and duodenal atresias was beneficial in that it resulted in growth by stretching of the distal esophagus, allowing a tension-free primary repair of the esophageal atresia.     

Esophageal atresia and tracheoesophageal fistula with distal esophageal stenosis: preoperative diagnosis

This is a presentation of a case of preoperative diagnosis of apparent esophageal atresia and an apparent distal and tracheoesophageal fistula from a peripheral hospital. After surgery, histopathologic findings showed the absence of cartilage and bronchial glands, suggesting the etiology as a fibromuscular stenosis.     

Esophageal atresia without distal tracheoesophageal fistula: high incidence of proximal fistula

Background

This retrospective study was performed to test our suspicion that the incidence of esophageal atresia with proximal fistula in our institution is much higher than is generally reported.

Methods

The charts of all patients with esophageal atresia and/or tracheoesophageal fistula admitted in the period 1982 to 2000 were analyzed. The type of atresia and/or tracheoesophageal fistula was noted, and the relative incidence was calculated and compared with the relative incidence in a cumulative series of 3492 patients taken from 9 published studies.

Results

In the period under study, 123 patients with esophageal atresia and/or tracheoesophageal fistula were identified. The relative incidence of esophageal atresia without distal fistula was statistically not different (10.6% in the present series against 8.49% in the reference group). A statistically significant difference in the relative incidence of esophageal atresia with proximal fistula, however, was found: 5.69% in the present series against 1.05% in the reference group (P < .0001). Looking at the subgroup of patients without a distal fistula, more than half of the patients did have a proximal fistula.

Conclusions

The relative incidence of esophageal atresia with proximal fistula in this series of children with esophageal atresia and/or tracheoesophageal fistula is significantly higher than reported in the literature. This is on the account of the subgroup of patients without a distal fistula in which the incidence of a proximal fistula is more than 50%. Especially in this subgroup, the existence of a proximal fistula should be ruled out preoperatively.     

Management of patients with combined tracheoesophageal fistula,esophageal atresia,and duodenal atresia

INTRODUCTION

Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge.

PRESENTATION OF CASE

Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms.

DISCUSSION

While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3–7 days under controlled circumstances.

CONCLUSION

A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes.     

Esophageal diverticulum in an infant with Down's syndrome and type III esophageal atresia

An 18-month-old infant with Down's syndrome presented with a symptomatic esophageal diverticulum (ED) located at the cervical esophagus. He had been operated on successfully for an esophageal atresia and distal tracheoesophageal fistula in the newborn period. Neither surgical maneuvers nor esophageal trauma could explain the ED, which was resected through a cervical approach.     

Triad of tracheoesophageal fistula-esophageal atresia, pulmonary hypoplasia, and duodenal atresia

We describe 3 cases of tracheoesophageal fistula with esophageal atresia who also had right pulmonary hypoplasia-aplasia and duodenal atresia. We review the current literature and propose that this may be a distinct association.     

Challenging surgical dogma in the management of proximal esophageal atresia with distal tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium

Purpose

Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF.

Transpleural repair of esophageal atresia without a primary gastrostomy: 240 patients treated between 1951 and 1983

Esophageal atresia with distal tracheoesophageal fistula has been treated at one institution between 1951 and 1983 primarily by prompt transpleural repair without gastrostomy. Two hundred and seventy one patients are assigned to three time periods: (1) 1951 to 1963, prior to modern intensive care; (2) 1964 to 1973, the initial era of mechanical ventilation; and 1974 to 1983, the era of readily available ventilators and TPN. During these time periods mean birthweight decreased from 2,780 g to 2,670 g to 2,500 g, while the incidence of associated anomalies increased from 41% to 44% to 55%. Eleven patients had no operation or gastrostomy only and died. Twenty (predominantly in the early years) had staged repairs with initial gastrostomy and fistula ligation. Primary anastomosis was done in 240 patients regardless of birth weight, 229 of which were by the transpleural approach. Gastrostomy was performed primarily in 28 patients for varied indications. Operative mortality (definitive as well as staged repairs) fell over the three time periods from 44% to 15% to 7%. Anastomotic leaks occurred in 20% of patients who underwent primary repair regardless of the time period. The mortality associated with a leak, however, decreased from 88% to 47% to 0%. Anastomotic strictures requiring more than two dilatations occurred in 15% in all periods. The overall incidence of recurrent fistula was 5%. Of 200 patients surviving the initial hospitalization, follow-up longer than 2 months is available for 156 patients with a median follow-up of 30 months. There were 15 late deaths.(ABSTRACT TRUNCATED AT 250 WORDS)     

Repair of esophageal atresia with tracheoesophageal fistula via thoracotomy: a contemporary series

Background

A recent series detailing thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) reported lower complication rates compared with historic controls. This study provides a contemporary cohort of patients repaired via thoracotomy for comparison with the recent large multi-institutional thoracoscopic series.

Methods

Records of patients with EA/TEF between 1993 and 2008 were reviewed. Attention was focused on demographics and complications including anastomotic leak, recurrent fistulae, stricture formation, and need for fundoplication.

Results

Seventy-two patients underwent repair of EA/TEF via thoracotomy. Complication rates in the current series compared with the thoracoscopic series were anastomotic leak, 2.7% versus 7.6%; recurrent fistulae, 2.7% versus 1.9%; stricture, 5.5% versus 3.8%; and need for fundoplication, 12% versus 24%. Differences in complication rates did not reach statistical significance. Two children in this cohort developed mild scoliosis attributed to congenital vertebral anomalies, neither of whom required intervention.

Conclusions

Thoracoscopic repair of EA/TEF yielded complication rates similar to this contemporary series; however, trends toward increased anastomotic leaks and greater need for fundoplication were noted. No musculoskeletal sequelae were directly attributable to thoracotomy.     

Noncommunicating tubular duplication of the upper pouch in esophageal atresia without fistula

We report a unique combination of an esophageal atresia without fistula associated with a tubular noncommunicating esophageal duplication. The diagnosis was made at delayed repair and led to a successful outcome.     

电视胸腔镜食管良性病变手术20例

目的探讨电视胸腔镜（video-assisted thoracoscopic surgery,VATS）在食管良性病变手术中的临床应用价值。方法 2005年3月～2011年3月,行胸腔镜手术治疗食管良性病变20例,采用3个1.0 cm切口,一个为进镜口,另外两个为操作孔,必要时将一个操作孔延长至5 cm。行良性肿瘤摘除术10例,贲门失弛缓症肌层切开术（Heller术）6例,膈肌食管裂孔疝修补术3例,外伤致膈肌损伤行膈肌修补术1例。结果 20例手术均获成功,1例贲门失弛缓症因术中损伤食管黏膜将一个操作口延长为5 cm的小切口,1例多发食管平滑肌瘤将操作口延长至3 cm。10例良性肿瘤术后病理证实为食管平滑肌瘤。20例手术时间50～152 min,住院时间4～9 d,平均6 d。无手术死亡及严重并发症发生。全组随访2～62个月,平均31个月,无明显进食哽噎等症状出现或复发。结论 VATS治疗食管良性病变安全、有效、可行,关键是选择好适应证和手术入路,可作为经选择的食管良性病变的首选治疗方法。     

Congenital laryngeal atresia associated with esophageal atresia and tracheoesophageal fistula: a case of long-term survival

Congenital laryngeal atresia (LA) is a life-threatening anomaly in which appropriate perinatal management is essential for survival. The authors report a neonate with LA associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) who was successfully resuscitated by emergent tracheostomy. Before birth, the patient had a diagnosis of EA based on the findings of polyhydramnios and absent stomach bubble. Immediately after birth, severe respiratory distress, cyanosis, and sternal retraction were evident. Because either endotracheal or esophageal intubation was unsuccessful, an emergent tracheostomy was performed. A direct laryngoscope revealed a subglottic atresia with normal appearance of the vocal cords. Repair of EA with TEF was performed on the third day of life, and the postoperative course was uneventful. In 2 years follow-up, the patient has no mental retardation nor central nervous impairment. Because of the presence of TEF, the antenatal ultrasonogram did not demonstrate the characteristic findings of the congenital high airway obstruction syndrome. This case represents one of the very few reported cases of successful resuscitation of a neonate with an unanticipated LA in which emergent airway management is required immediately after birth.     

Esophageal atresia/tracheoesophageal fistula in very low-birth-weight neonates: improved outcomes with staged repair

Introduction

The surgical management of esophageal atresia with distal tracheoesophageal fistula (EA/TEF) involves early division of the TEF and primary esophageal anastomosis. However, in premature infants, the morbidity associated with primary repair remains high, and the optimal surgical approach has not been well defined.

Methods

Medical records of very low-birth-weight infants (<1500 g) with EA/TEF from June 1987 to 2008 were retrospectively reviewed. Patients were separated into 2 groups: (1) primary repair and (2) ligation and division of TEF followed by delayed repair of EA. Demographics, anastomotic, and postoperative complications were compared.

Results

Twenty-five premature infants with EA/TEF were identified. Sixteen patients (64%) underwent primary repair, and 9 (36%) were repaired in a staged manner. The leak rate confirmed by esophagram was significantly higher after primary repair (50%) compared to staged repair (0%) (P = .034). Strictures occurred significantly more often in the primary repair (81%) vs the staged repair (33%) group (P = .036). Postoperative pneumonia and sepsis were significantly higher in patients treated with primary repair (P = .028).

Conclusion

Staged repair of EA/TEF in very low-birth-weight premature infants results in a significantly lower rate of anastomotic complications and overall morbidity and should be considered the preferred surgical approach in this group of patients.     

Thoracoscopic management of non-type C esophageal atresia and tracheoesophageal atresia

Purpose

This study evaluates the results of thoracoscopic management of complex, non-type C, EA and TEF in infants.