Long-gap Esophageal Atresia Research Articles

Nutrition delivery and growth outcomes in infants with long-gap esophageal atresia who undergo the Foker process

BackgroundPredictors of growth outcomes in patients with long-gap esophageal atresia (LGEA) are not known. We examined nutrition and growth in-hospital and post-discharge in LGEA patients who underwent the Foker Process (FP). MethodsSingle-center, retrospective cohort study of infants with LGEA undergoing primary (non-rescue) FP from 2014 to 2020. Weight-for-age z scores (WAZ, 0 = average), macronutrient prescription, anthropometry, and clinical variables were collected. Longitudinal median regression evaluated differences in WAZ over time. Multivariable median regression examined variables associated with change in WAZ at 1 year. Results45 patients met criteria, with median (IQR) age at repair of 4 (2, 5.8) months and WAZ of -0.96 (-1.55, -0.40). On admission, 11% were moderately (WAZ < -2) and 9% were severely (WAZ < -3) malnourished. Lower admission WAZ was significantly associated with improvement in WAZ at 1-year follow-up (p = 0.002); EA type (59% type A), esophageal leak (16%), median days paralyzed (13), ventilated (21), on parenteral nutrition (35), or to full enteral nutrition (35) were not associated with change in WAZ. Median WAZ remained stable while in-hospital, and patients maintained their growth curves through 3-year follow-up. ConclusionThroughout infancy, most primary FP LGEA patients have weight for age that is below average. Using targeted nutritional intervention, those who present with malnutrition can still achieve adequate growth despite prolonged and complicated hospital courses.

Favorable Outcome of Electively Delayed Elongation Procedure in Long-Gap Esophageal Atresia.

The ideal approach to long gap esophageal atresia is still controversial. On one hand, preserving a patient's native esophagus may require several steps and can be fraught with complications. On the other hand, most replacement procedures are irreversible and disrupt gastrointestinal physiology. The purpose of this study was to evaluate the short- and medium-term outcome of electively delayed esophageal elongation procedures before esophageal reconstruction in patients with long-gap esophageal atresia. Since the neonatal esophagus grows over-proportionally and can increase its wall thickness in the first few months of life, we hypothesized that postponing the elongation steps until 3 months of age would lead to a lower complication rate. We thus retrospectively recorded complications such as mediastinitis, anastomotic leakage, stricture formation, or gastroesophageal reflux requiring surgery, and compared it to reported outcomes. In our treatment protocol, patients born with long-gap esophageal atresia underwent gastrostomy placement and were sham fed until 3 months of age. We then assessed the gap between the esophageal ends and started serial elongation procedures. We only proceeded to the reconstruction of the esophagus when its length allowed a tension-free anastomosis. From April 2013 to April 2019, we treated 13 Patients with long-gap esophageal atresia. Nine patients without prior surgical procedures underwent Foker procedures. Four patients arrived with a pre-existing cervical esophagostomy and thus underwent Kimura's procedure, two of them with a concomitant Foker elongation of the lower pouch. Esophageal reconstruction was feasible in all patients, while none of them developed mediastinitis at any point in their treatment. We managed the only anastomotic leak conservatively. Almost half of the patients did not require any further intervention following reconstruction, while three patients required multiple (≥5) anastomotic dilatations. All but one patient achieved full oral nutrition. Only one child required a fundoplication to manage gastroesophageal reflux symptoms. Electively delayed esophageal elongation procedures in patients with long-gap esophageal atresia allowed preservation of the native esophagus in all patients. The approach had low peri-procedural morbidity, and patients enjoy favorable functional outcomes. Therefore, we suggest considering this method in the management of patients with long-gap esophageal atresia.

Childhood outcome after correction of long-gap esophageal atresia by thoracoscopic external traction technique

BackgroundThoracoscopic external traction technique (TTT) is a relatively new surgical intervention for patients with long-gap esophageal atresia (LGEA) that preserves the native esophagus. The major accomplishment with TTT is that esophageal repair can be achieved within days after birth.This study evaluates the childhood outcome in LGEA patients treated with TTT, including gastrointestinal outcome, nutritional status and Health-Related Quality of Life (HRQoL). MethodsA cohort study including all LGEA patients that underwent TTT between 2006-2017 was conducted. Patients and/or their parents were invited to fill out questionnaires regarding reflux symptoms and HRQoL. ResultsTTT was successful in 11/13 patients (85%). Esophageal anastomosis was accomplished at a median age of 12 days (range 7-138), first oral feeding was started at a median of 16 days postoperatively (range 5-37). All patients required multiple dilatations and 10 patients required anti-reflux surgery.At median follow-up of seven years, five patients reported mild and one moderate reflux complaints. All patients but one reached age-appropriate oral diet. Most patients (80%) were within normal growth range. Overall HRQoL was comparable to healthy controls. ConclusionTTT provides acceptable results in childhood. Oral feeding can be started as soon as two weeks postoperatively. Almost all patients are able to eat an age-appropriate oral diet. Overall HRQoL was comparable to healthy controls.

Thoracoscopic intraoperative esophageal close technique for long-gap esophageal atresia.

Long-gap esophageal atresia (EA) remains a challenging operative procedure. Several techniques have been reported to resolve the problem of distance between the proximal and distal esophagus. We report a thoracoscopic intraoperative esophageal close technique for long-gap EA. A female neonate was born at 39 weeks of gestation with long-gap EA (five vertebrae). The patient underwent thoracoscopic esophageal anastomosis after esophageal elongation. Careful dissection of the proximal and distal esophagus was performed; however, the distance between them was still long. Extra-thoracic traction sutures were placed at the upper esophagus. Then, a Neraton catheter was inserted trans-orally and the upper esophagus was pushed and opened. An internal traction suture was placed between the Neraton catheter and lower esophagus. The gap between the proximal and distal esophagus could be approximated by pulling the Neraton catheter. Esophageal anastomosis was performed successfully. Our thoracoscopic intraoperative esophageal close technique was simple and useful.

A narrative review of esophageal tissue engineering and replacement: where are we?

Long-gap esophageal defects, whether congenital or acquired, are very difficult to manage. Any significant surgical peri-esophageal dissection that is performed to allow for potential stretching of two ends of a defect interrupts the esophageal blood supply and leads to complications such as leak and stricture, even in the youngest, healthiest patients. The term “congenital” applied to these defects refers mainly to long-gap esophageal atresia (LGA). Causes of acquired long-segment esophageal disruption include recurrent leaks and fistulae after primary repair, refractory GERD, caustic ingestions, cancer, and strictures. 5,000–10,000 patients per year in the US require esophageal replacement. Gastric, colonic, and jejunal pull-up surgeries are fraught with high rates of both short and long term complications thus creating a space for a better option. Since the 1970’s many groups around the world have been unsuccessfully attempting esophageal replacement with tissue-engineered grafts in various animal models. But, recent advances in these models are now combining novel technologic advances in materials bioscience, stem-cell therapies, and transplantation and are showing increasing promise to human translational application. Transplantation has been heretofore unsuccessful, but given modern improvements in transplant microsurgery and immunosuppressive medications, pioneering trials in animal models are being undertaken now. These rapidly evolving medical innovations will be reviewed here.

Tracheal compression by the stomach following gastric transposition in a toddler

Gastric transposition is widely employed for esophageal replacement. However, postoperative respiratory failure is one of the most fatal complications of this procedure, and its exact cause remains unclear. A 1-year-old boy with VATER association underwent retrosternal gastric transposition for a long-gap esophageal atresia. The patient experienced respiratory failure immediately after surgery. Bronchoscopy and computed tomography revealed tracheomalacia due to tracheal compression from the gastric pull-up. Extubation became difficult, and tracheostomy was performed. Intermittent decompression using a nasogastric tube, tracheostomy, and high positive end-expiratory pressure ventilation were continued until the tracheal lumen was no longer collapsed. The frequency of dyspnea gradually decreased, and the patient's transition to home medical care was possible at the age of two years. This case highlights the need to consider the effects of tracheomalacia after gastric transposition. Reconstruction through a retrosternal route may make subsequent vascular and tracheal procedures for the correction of tracheomalacia more challenging. If a posterior sternal route must be chosen because of the extensive mediastinal adhesions, it may be necessary to use a relatively small-capacity esophageal reconstruction organ, such as the large intestine, or to perform volume reduction surgery of a lifted stomach.

A new method of primary engineering of esophagus using orthotopic in-body tissue architecture

PurposeTissue engineering of esophagus is required for management of long-gap esophageal atresia (LGEA). Collagenous connective tissue membranes fabricated by in-body tissue architecture (iBTA), called biosheets, can repair esophageal defects and generate tissues similar to native esophagus. However, iBTA requires second-stage surgery because of heterotopic preparation of biosheets. Our aim was to develop orthotopic iBTA for primary engineering of the esophagus by interposing a tubular mold to the esophageal defect. MethodThe cervical esophagus of six rats was transected. An acrylic tube (internal diameter 2.6 mm, length 7.0 mm) was inserted and fixed between the ends of the upper and lower esophagus, and a 3 mm-long esophageal defect was created. Four weeks later, the rats were sacrificed for histological analysis. ResultsPostoperatively the rats could intake liquid food. After four weeks, the esophageal defects were filled with regenerated tissues. Histologically the new esophageal walls stained positive for collagen type I. The inner surfaces were covered with stratified squamous epithelium that expressed pan-cytokeratin. In only one of six rats, regeneration of muscular-like tissue was suggested by positive immunohistochemical staining for desmin. ConclusionOrthotopic iBTA can regenerate a substitute esophagus with esophageal epithelium and collagenous wall. This technique may be a novel treatment for esophageal atresia with gaps of various lengths including LGEA.

Contemporary outcomes of the Foker process and evolution of treatment algorithms for long-gap esophageal atresia

BackgroundEsophageal growth using the Foker process (FP) for long-gap esophageal atresia (LGEA) has evolved over time. MethodsContemporary LGEA patients treated from 2014–2020 were compared to historical controls (2005 to <2014). Results102 contemporary LGEA patients (type A 50%, B 18%, C 32%; 36% prior anastomotic attempt; 20 with esophagostomy) underwent either primary repair (n=23), jejunal interposition (JI; n = 14), or Foker process (FP; n = 65; 49 primary [p], 16 rescue [r]). The contemporary p-FP cohort experienced significantly fewer leaks on traction (4% vs 22%), bone fractures (2% vs 22%), anastomotic leak (12% vs 37%), and Foker failure (FP→JI; 0% vs 15%), when compared to historical p-FP patients (n = 27), all p ≤ 0.01. Patients who underwent a completely (n = 11) or partially (n = 11) minimally invasive FP experienced fewer median days paralyzed (0 vs 8 vs 17) and intubated (9 vs 15 vs 25) compared to open FP patients, respectively (all p ≤ 0.03), with equivalent leak rates (18% vs 9% vs 26%, p = 0.47). At one-year post-FP, most patients (62%) are predominantly orally fed. ConclusionWith continued experience and technical refinements, the Foker process has evolved with improved outcomes, less morbidity and maximal esophageal preservation.

Head circumference in infants undergoing Foker process for long-gap esophageal atresia repair: Call for attention

IntroductionWe extended our pilot study in infants following long-gap esophageal atresia (LGEA) repair to report head circumference, an easily obtainable indirect measure of brain size. Data are presented in the context of previously reported body weight and T2-weighted MRI measures of intracranial and brain volumes. MethodsClinical information and head circumference were obtained for term-born (n = 13) and premature (n = 13) infants following LGEA repair with Foker process, as well as healthy term-born controls (n = 20) <1-year corrected age who underwent non-sedated research MRI. General Linear Model univariate analysis with corrected age at scan as a covariate and Bonferroni adjusted p values assessed group differences. ResultsWe report no difference in head circumference between the three groups. Such findings paralleled trends in body weight and total intracranial volume but not in brain volume as previously reported for the same pilot cohort. DiscussionResults suggest uncompromised somatic and head growth after repair of LGEA. In contrast, a novel finding of discrepancy between head circumference (novel data) and brain size (previously published data) in the same cohort suggests that head circumference might not be the best indirect measure of brain size in selected group of patients.

Usefulness of Gastrojejunostomy Prior to Fundoplication in Severe Gastro-Esophageal Reflux Complicating Long-Gap Esophageal Atresia Repair: A Preliminary Study

Background: Gastro-esophageal reflux disease (GERD), requiring surgical correction, and nutritional problems are reported after long-gap esophageal atresia (LGEA) repair and might jeopardize the postoperative course in some babies. We report an exploratory evaluation of the role of transgastric jejunostomy (TGJ) as a temporary nutritional tool before surgery for GERD in LGEA. Methods: Seven infant patients operated on for LGEA with intra-thoracic gastro-esophageal junction (GEJ) and growth failure, requiring improvement in their nutritional profile in anticipation of surgery, were retrospectively evaluated. Post-surgical follow-up, including growth evolution, complications, and parental quality of life (QoL), were considered. Results: The TGJ was placed at a mean age of 8.6 ± 5.6 months. The procedure was uneventful and well-tolerated in all seven cases. At 6.6 ± 2.0 months after TGJ placement, significant weight gain (weight z-score −2.68 ± 0.8 vs −0.9 ± 0.2, p < 0.001) was recorded, allowing the GERD surgery to proceed. A significant difference in hospital admissions between 3 months before and post-TGJ insertion was noted (4.8 ± 0.75 vs. 1.6 ± 0.52, p < 0.01). A significant amelioration of QoL after TGJ placement was also recorded; in particular, the biggest improvements were related to parents’ perceptions of the general health and emotional state of their babies (p < 0.001). Conclusions: The placement of TGJ as a temporary nutritional tool in selected cases of LGEA could improve nutritional conditions and parental QoL before fundoplication, allowing successful surgical treatment of GERD to be carried out.

A New Approach to Tense End-to-End Anostomosis in Primary Surgical Repair of Esophageal Atresia: Vascularized Pleural Flap.

Background: Esophageal atresia (EA) is the most common congenital malformation of the esophagus. If the distance between the proximal and distal pouches is usually more than 2-3 cm, it is considered as long gap esophageal atresia (LGEA). In our study, it was planned to investigate the effect of the use of vascularized pleural flap (VPF) on postoperative recovery in cases with tense end-to-end anastomosis in the primary repair of LGEA. Methods: The postoperative recovery period data of patients who underwent tense end-to-end anastomosis due to LGEA between 01/01/2016 and 01/12/2020 in our clinic were analyzed retrospectively. Results: Between the specified dates, 37 patients were operated for EA. A tense end-to-end anastomosis was performed in 16 of 37 patients. In 5 of these patients, a VPF was placed on the anastomosis line. In the postoperative follow-up, no anastomotic leakage or anastomotic stenosis was observed in 5 patients who underwent tense anastomosis with VPF. In addition, it was observed that patients who underwent tense anastomosis with VPF were started oral nutrition earlier after surgery compared to patients without VPF. Conclusion: The success of the technique we performed in 5 patients without any complications suggested that this technique could be used as a method in tense anastomoses. It was thought that oral nutrition was initiated early in patients using VPF, since there was no anastomotic leak and the surgeon relied on the presence of the VPF.

The Effect of Botulinum Toxin Type A Injections on Stricture Formation, Leakage Rates, Esophageal Elongation, and Anastomotic Healing Following Primary Anastomosis in a Long- and Short-Gap Esophageal Atresia Model - A Protocol for a Randomized, Controlled, Blinded Trial in Pigs.

Background:Esophageal atresia (EA) is a congenital malformation affecting 1:3000-4500 newborns. Approximately 15% have a long-gap EA (LGEA), in which case a primary anastomosis is often impossible to achieve. To create continuity of the esophagus patients instead have to undergo lengthening procedures or organ interpositions; methods associated with high morbidity and poor functional outcomes. Esophageal injections of Botulinum Toxin Type A (BTX-A) could enable primary anastomosis and mitigate stricture formation through decreased tissue tension.Methods and Analysis:In this randomized controlled blinded animal trial, 24 pigs are divided into a long- or short-gap EA group (LGEA and SGEA, respectively) and randomized to receive BTX-A or isotonic saline injections. In the LGEA group, injections are given endoscopically in the esophageal musculature. After seven days, a 3 cm esophageal resection and primary anastomosis is performed. In the SGEA group, a 1 cm esophageal resection and primary anastomosis is performed, followed by intraoperative injections of BTX-A or isotonic saline. After 14 days, stricture formation, presence of leakage, and esophageal compliance is assessed using endoscopic and manometric techniques, and in vivo and ex vivo contrast radiography. Tissue elongation is evaluated in a stretch-tension test, and the esophagus is assessed histologically to evaluate anastomotic healing.Ethics and Dissemination:The study complies with the ARRIVE guidelines for animal studies and has been approved by the Danish Animal Experimentation Council. Results will be published in peer-reviewed journals and presented at national and international conferences.Highlights:The optimal management of long-gap esophageal atresia remains controversialPrimary anastomosis could improve functional outcomes and reduce complicationsBotulinum Toxin Type A decreases tissue tension and could facilitate anastomosisReduced tension could further abate the risk for anastomotic stricture and leakageWe present a model to evaluate the method in long- and short-gap esophageal atresia

Treatment of long-gap esophageal atresia with use of thoracoscopic internal traction elongation

Treatment of long-gap esophageal atresia with use of thoracoscopic internal traction elongation

Foker technique for esophageal reconstruction after cervical esophagostomy

The approach to patients with complicated esophageal atresia continues to be a challenge for the pediatric surgeon. Patients with long-gap esophageal atresia (LGEA) or who had complications during their first surgery may require cervical esophagostomy and gastrostomy for posterior definitive correction. Usually reconstruction is performed through esophageal replacement with gastric, ileal, or colonic interposition. This paper reports on two cases in which the intraoperative stretching technique - Foker - maintaining the native esophagus was used, for reconstruction of the digestive tract in patients submitted to cervical esophagostomy and gastrostomy at birth, one due to diagnosis of LGEA and the other due to complications in the primary correction.

The left-sided repair: An alternative approach for difficult esophageal atresia repair

Purpose: We describe a left-sided approach for long gap esophageal atresia (LGEA) repair in patients who have a large leftward upper pouch and no significant tracheomalacia, or as a salvage strategy after prior failed right-sided repairs. Methods: Retrospective review of patients who underwent repair via traction induced growth (Foker procedure [FP]) from 2014 to 2019 was performed. Surgical technique and post-operative outcomes were evaluated. Results: Of 47 LGEA patients, 18 (38%) were approached via the left side – 94% had a left aortic arch, and 22% had prior attempts at a right-sided anastomosis. More left-sided patients underwent minimally invasive repair (39% vs 7%, p = 0.007) and internal traction (50% vs 10%, p = 0.002) compared to right-sided patients. On multivariate analysis, internal traction was associated with a decreased length of paralysis (p<0.01); length of intubation and hospital stay were similar between groups. Anastomotic leak (17% vs 20%, p = 0.80) and stricture resection (6% vs 24%, p = 0.12) rates were similar. No left-sided FP patient required additional surgery for tracheomalacia, while six right-sided patients required intervention. Conclusion: Left-sided FP can be considered for LGEA patients with a large leftward upper pouch or as a salvage pathway after a failed right chest approach, with similar outcomes to the right-sided approach.

SURGICAL TACTICS OF SAVING THE NATIVE ESOPHAGUS IN CASE OF LONG GAP ESOPHAGEAL ATRESIA

Introduction. The absence of a single surgical tactic for a long gap esophageal atresia (LGEA) treatment as well as frequent unsatisfactory results of applying existing treatment methods urge searching and implementing of a new surgical approach to remediation of malformation. Objective. To optimize surgical treatment of LGEA and provide preservation of native esophagus by elongating and pouches growth inducting. Materials and methods. 6 clinical cases of patients with LGEA who had been treated in National children specialized hospital “Okhmatdyt” during 2018-2019 were analyzed. All patients received staged malformation remediation by elongating of esophagus pouches with traction sutures with further formation of anastomosis. Results. 6 patients with LGEA were surgically treated by esophageal lengthening using the Foker process. The duration of traction varied from 6 to 19 days, the average daily distance of traction of each segment was from 2,4 to 4,8 mm. Segment growth efficiency was observed by diagnostics of metal clips positioning at the ends of esophagus segments on X-ray, or esophagography. In all 6 cases the application of dozed staged traction resulted in esophagus lengthening with elimination of gap from 0-1,0 sm, so that anastomosis of native esophagus was accomplished with the further oral feeding. Conclusions. The method of growth induction allows to overcome a long gap between segments of the atretic esophagus, create conditions for the formation of anastomosis and preserve own esophagus with the restoration of age-related oral nutrition. The concept of induction of esophageal growth eliminates or reduces the use of intestinal plastic of esophagus. The Foker process is an organ-preserving method and is an alternative to operations with loss of an esophagus.

Characterization of mesenchymal stem cells in patients with esophageal atresia

BackgroundPreclinical studies demonstrate that tissue engineering and patient-derived stem cells can regenerate tissue. The goal of this study was to determine whether stem cells from esophageal atresia patients (EA) could be utilized for this purpose. MethodsAdipose tissue was obtained from control, esophageal atresia (EA) and long gap esophageal atresia (LGEA) patients. Mesenchymal stem cells (MSCs) were isolated, expanded, characterized and seeded onto tubular scaffolds for 6 days. Scaffolds were characterized for viability, gene expression and cytokine production. ResultsThe average weight of tissue from the EA and LGEA patients was 145.8mg compared to 2981 mg in controls. Despite the small amount of tissue obtained from neonatal patients, cells were expanded to cover a scaffold. After incubating 6 days on the scaffold, cells were viable and proliferating with differences in gene expression between groups. VEGFA production in the supernatant was increased in EA and LGEA patients; while IL6 production was significantly increased in the control patients. ConclusionsThis study demonstrates the ability to utilize small amounts of adipose tissue from esophageal atresia patients as a cell source for regenerative medicine. Future studies will focus on use of these cells for tissue regeneration in vivo.

Evaluation of Gastroesophageal Reflux Disease 1 Year after Esophageal Atresia Repair: Paradigms Lost from a Single Snapshot?

To analyze the findings of both multichannel intraluminal impedance with pH (MII-pH) and endoscopy/histopathology in children with esophageal atresia at age 1year, according to current recommendations for the evaluation of gastroesophageal reflux disease (GERD) in esophageal atresia. We retrospectively reviewed both MII-pH and endoscopy/histopathology performed in 1-year-old children with esophageal atresia who were followed up in accordance with international recommendations. Demographic data and clinical characteristics were also reviewed to investigate factors associated with abnormal GERD investigations. In our study cohort of 48 children with esophageal atresia, microscopic esophagitis was found in 33 (69%) and pathological esophageal acid exposure on MII-pH was detected in 12 (25%). Among baseline variables, only the presence of long-gap esophageal atresia was associated with abnormal MII-pH. Distal baseline impedance was significantly lower in patients with microscopic esophagitis, and it showed a very good diagnostic performance in predicting histological changes. Histological esophagitis is highly prevalent at 1year after esophageal atresia repair, but our results do not support a definitive causative role of acid-induced GERD. Instead, they support the hypothesis that chronic stasis in the dysmotile esophagus might lead to histological changes. MII-pH may be a helpful tool in selecting patients who need closer endoscopic surveillance and/or benefit from acid suppression.

Distal esophagostomy tube feedings in the treatment of long gap esophageal atresia: Experience with 10 cases

Abstract There are different strategies for the treatment of long gap esophageal atresia (LEA), but many aspects of its management is still controversial. The best treatment is conserving the native esophagus through the connection of two esophageal pouches. In this study, we shared our experiences in management of LEA treatment and introduced a new approch for the conservative management of this condition. In our experience, ten patients with long gap esophageal atresia were treated, followed up and managed by our own designed method. In our technique, we lengthened the both upper and lower pouches instead of one pouches. On the one hand, with the effect of saliva pressure, the upper pouch became longer without proximal esophagostomy. On the other hand, the lower pouch was mechanically lengthened by the feeding effect of gavage. These reduced the distance between the two ends of the esophagus and provided the conditions for the primary anastomosis and saving the native esophagus. There were no complication in these ten patients but this procedure needs more investigation.

Quality of life after esophageal replacement in children

PurposeAssessing quality of life (QoL) after esophageal replacement (ER) for long gap esophageal atresia (LGEA). MethodsAll patients after ER for LGEA with gastric pull-up (GPU n = 9) or jejunum interposition (JI n = 14) at the University Medical Center Groningen and Utrecht (1985–2007) were included. QoL was assessed with 1) gastrointestinal-related QoL using the Gastrointestinal Quality of Life Index (GIQLI)), 2) general QoL (Child Health questionnaire CHF87-BREF (children)/World Health Organization questionnaire WHOQOL-BREF (adults)), and 3) health-related QoL (HRQoL) (TNO AZL TACQoL/TAAQoL). Association of morbidity (heartburn, dysphagia, dyspnea on exertion, recurrent cough) and (HR)QoL was evaluated. ResultsSix patients after GPU (75%) and eight patients after JI (57%) responded to the questionnaires (mean age 15.7, SD 5.9, 12 male, two female). Mean gastrointestinal, general and health-related QoL total scores of the patients were comparable to healthy controls. However, young adults reported a worse physical functioning (p = 0.02) but better social functioning compared to peers (p = 0.01). Morbidity was not associated with significant differences in (HR)QoL. ConclusionsWith the current validated QoL most patients after ER with GPU and JI for LGEA have normal generic and disease specific QoL scores. Postoperative morbidity does not seem to influence (HR)QoL. Type of StudyPrognosis Study. Level of evidenceIII.