Repair Of Atresia Research Articles

Factors Associated With Adverse Outcomes Following Duodenal Atresia Surgery in Neonates: A Retrospective Study.

ObjectivesThere is limited evidence on the infants' postoperative complications who have undergone surgical repair of duodenal atresia and stenosis. This study aimed to identify the factors associated with poor surgical outcomes after the initial repair.MethodsWe retrospectively reviewed the data of 82 patients who underwent surgery for duodenal atresia and stenosis between January 1994 and December 2013 at our institution. Gestational age, birth weight, fetal growth, and other associated anomalies were recorded. Multivariate regression analyses were used to identify the factors associated with surgical outcomes, including postoperative complications and time to full oral intake.ResultsThe median gestational age was 37.6 weeks, with 30 (37%) preterm (<37 weeks) and 11 (13%) early preterm (<34 weeks) infants. The median birth weight was 2531 g, with 27 (33%) patients < 2000 g and 10 (12%) patients < 1500 g. Postoperative surgical complications were identified in 18 (22%) cases, of which 12 (15%) required additional operations. Multivariate regression analysis revealed that a combination of very low birth weight (<1500 g) and early preterm was significantly associated with both surgical and non-surgical postoperative complications (p = 0.0028 and 0.021, respectively) and a prolonged time to full oral intake postoperatively (p = 0.013).ConclusionVery low birth weight and early preterm were significantly associated with postoperative complications and a prolonged time to full oral intake.

Acid suppression duration does not alter anastomotic stricture rates after esophageal atresia with distal tracheoesophageal fistula repair: A prospective multi-institutional cohort study

IntroductionAnastomotic stricture is the most common complication after esophageal atresia (EA) repair. We sought to determine if postoperative acid suppression is associated with reduced stricture formation. MethodsA prospective, multi-institutional cohort study of infants undergoing primary EA repair from 2016 to 2020 was performed. Landmark analysis and multivariate Cox regression were used to explore if initial duration of acid suppression was associated with stricture formation at hospital discharge (DC), 3-, 6-, and 9-months postoperatively. ResultsOf 156 patients, 79 (51%) developed strictures and 60 (76%) strictures occurred within three months following repair. Acid suppression was used in 141 patients (90%). Landmark analysis showed acid suppression was not associated with reduction in initial stricture formation at DC, 3-, 6- and 9-months, respectively (p = 0.19–0.95). Multivariate regression demonstrated use of a transanastomotic tube was significantly associated with stricture formation at DC (Hazard Ratio (HR) = 2.21 (95% CI 1.24–3.95, p<0.01) and 3-months (HR 5.31, 95% CI 1.65–17.16, p<0.01). There was no association between acid suppression duration and stricture formation. ConclusionNo association between the duration of postoperative acid suppression and anastomotic stricture was observed. Transanastomotic tube use increased the risk of anastomotic strictures at hospital discharge and 3 months after repair. Level of Evidence2

A case of esophageal atresia complicated by a right-sided aortic arch with right ductus arteriosus and inferior vena cava interruption with hemiazygos continuation

During the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF), cardiovascular malformations occasionally create a technical challenge. We report a novel case of a 4-day-old girl with EA/TEF and multiple cardiovascular malformations, including right-sided aortic arch (RAA), right ductus arteriosus (RDA), single ventricle with single atrium, common atrioventricular valve, pulmonary atresia, bilateral superior vena cava, and interruption of the inferior vena cava (IVC) with hemiazygos continuation. In this case, a right-sided approach would require the mobilization of the RAA and RDA, which were supplying the pulmonary blood flow due to pulmonary atresia. Alternatively, the left-sided approach would require the mobilization of the hemiazygos vein, which was essential for venous return from the lower body due to IVC interruption. We performed the less intrusive left-sided approach, and the postoperative course was uneventful. Right-sided EA/TEF repair should be avoided because RDA spasm or injury caused by RAA mobilization would be fatal. In cases of interrupted IVC with azygos or hemiazygos vein continuation, care must be taken not to ligate these vessels or block the venous return. Preoperative evaluation is important to prevent complications in such complicated cases. If sufficient information is not available, the left-sided approach may be preferred. • In case of RAA with RDA, the EA/TEF repair should be approached from the left side. •Detection of cardiovascular malformations is necessary in case of EA/TEF with RAA. •This is the first case report of EA/TEF with RAA that clearly records RDA.

The role of motion tracking in assessing technical skill acquisition using a neonatal 3D-printed thoracoscopic esophageal atresia/tracheo-esophageal fistula simulator

IntroductionAcquiring the technical skills required for thoracoscopic repair of esophageal atresia with tracheo-esophageal fistula (EA/TEF) is challenging. A high-fidelity 3D-printed pediatric thoracoscopic EA/TEF simulator has been developed to address this issue. This study explored motion-tracking as an assessment tool to distinguish between surgeons of different expertise using the simulator. MethodsParticipants performed a single intracorporeal suture between the esophageal ends in EA with TEF. Total relative path lengths of the right and left surgical instruments were recorded during the task. Each video-recorded attempt was assessed by a blinded pediatric surgeon using a modified Objective Structured Assessment of Technical Skills (OSATS) score. Data recorded as median (range) and statistical significance as p<0.05. ResultsThe task was performed by 17 participants. The median OSATS scores identified a significant difference between experts and novices. A difference between left- and right-hands was only found in the mid-skill level group. Right-hand path length was greatest in novices and lowest in experts. Left-hand path length was greatest in novices and the mid-skill level group compared to experts. ConclusionExperts had the lowest total path length for either hand, suggesting they had the greatest efficiency of movement. The similar high path lengths in both hands for novices indicate their relatively low level of skill with either hand. The difference between right- and left-hand path lengths in the mid-skill level group likely reflects the improved right-handed technical skills in contrast to the still developing left hand. Further focus on the left hand during simulation training may improve left-handed economy of movement.

Pediatric esophageal stenoses: Challenges and new surgical device promoting tension-free esophageal anastomosis.

Esophageal stenoses of childhood have a broad spectrum of underlying causes. Their treatment is usually minimally invasive by endoscopic means, but sometimes surgery is necessary in refractory cases. Techniques employed in the surgical treatment of esophageal strictures include resection of the stenotic esophageal segment or esophageal substitution procedures. Esophageal anastomosis has always been a challenge in pediatric surgery. Anastomosis complications are linked to anatomical, biological and technical aspects. Mechanical tension between esophageal ends is an important cause of complications including anastomotic leaks or dehiscence. Eleven cases of esophageal stenoses, surgically treated in the Pediatric Surgery Department of Emergency Clinical Hospital for Children 'Marie S. Curie' by a single team in 5 years, were included in the present study. The results showed that, the main causes of esophageal stenosis were represented by corrosive esophageal injury in five cases, complications of esophageal atresia repair in three cases, congenital esophageal stenosis in two cases and chemotherapy-induced esophageal necrosis in acute lymphoblastic leukemia treatment in one case. The authors also designed and presented a device facilitating esophageal anastomosis under tension. Its principle involved temporary absorption of tension at secure points of the two esophageal pouches and reallocating it in equal amounts following anastomosis while decreasing any stretch-related tissue trauma. In conclusion, this auxiliary tool is beneficial for esophageal anastomosis; however, the standard steps of the esophageal anastomosis procedure should still be considered when necessary.

Pitfalls in laparoscopic duodenal atresia repair requiring modifications in approach based on literature review

Pitfalls in laparoscopic duodenal atresia repair requiring modifications in approach based on literature review

Predictive factors for fundoplication following esophageal atresia repair

One of the most frequent complications after repair of esophageal atresia (EA) is gastroesophageal reflux disease (GERD). Although GERD-associated EA is known to often require anti-reflux surgery, the predicting factors remain unclear. We retrospectively analyzed EA in our institution. Of 65 children with EA treated in our hospital from 1995 to 2018, 45 with Gross C type EA, followed for over 1 year, were enrolled in this study. The patients were divided into fundoplication and non-fundoplication groups and compared in terms of their clinical features. The fundoplication and non-fundoplication groups included 13 and 32 cases, respectively. On univariate analysis, gestational age, body weight, prenatal diagnosis, polyhydramnios, re-do surgery, and gap length of the esophagus differed significantly between the groups (P < 0.05). Early delivery, low body weight, and a long gap length are, are considered to be risk factors for fundoplication. However, the present study further showed that prenatal diagnosis and polyhydramnios were also significant contributing factors. The presence of a prenatal diagnosis and polyhydramnios may induce preterm delivery, therefore, cases of polyhydramnios due to suspected EA should be managed to prevent early delivery. Better understanding of the postnatal course after surgery is required, especially for prenatal diagnosis cases.

Transnasal Endoscopic Approach In Repair of Choanal Atresia

Transnasal Endoscopic Approach In Repair of Choanal Atresia

Reparing a long gap Oesophageal Atresia (LGEA) using the combined procedures of foker and kimura

Primary repair of long gap oesophagus atresia (LGEA) requires interdisciplinary expertise to achieve promising results. We report a 9 month old refugee with a spit fistula and a gastrostomy who achieved primary anastomosis in our hospital using the combined surgical methods of Kimura on the upper pouch and Foker in the lower pouch.

Nissen Fundoplication for the Treatment of Gastroesophageal Reflux After Repair of Esophageal Atresia

Nissen Fundoplication for the Treatment of Gastroesophageal Reflux After Repair of Esophageal Atresia

Technical Innovations to Reduce Complication Rates in Esophageal Atresia with Particular Reference to Long-term Outcomes: A Single Surgeon's Experience of 22 Years.

Following esophageal atresia/tracheoesophageal fistula (EA/TEF) repair, the standard leak rate reported in the literature is 5%-10%, and stricture rate is 40%-72%. There is a global quest for surgical innovations to drive down these complication rates which can cause considerable morbidity. A prospectively maintained database of the senior author's patients who had esophageal atresia repair from 1995 to 2016 was reviewed. Two distinct innovations were implemented: (1) adequate or generous mobilization of the lower esophageal pouch and (2) a 2-5 mm slit in distal esophagus to widen its circumference. Forty-three patients with EA/TEF were reviewed. Of those, 40 underwent primary repair. The median follow-up was 12.5 years (range 4-26 years). There were no anastomotic leaks and only 8 (20%) patients developed anastomotic strictures requiring dilations (1-5 dilations/patients). One patient (2.5%) had a recurrent fistula. One early mortality was recorded. At the latest follow-up, 35 (87.5%) patients had normal oral feeding, while 1 (2.5%) patient had occasional food sticking episodes. Four syndromic patients (10%) were on jejunal or gastrostomy feeding. An adequate or generous mobilization of the distal esophageal pouch, together with a 2-5 mm slit in the distal esophagus, achieves a tension-free and wide anastomosis. All anastomoses eventually narrow, sometimes just a little, and starting on a higher scale with a small slit, helps. These seemingly minor innovations, when used together, contributed to a substantially lower complication rate sustained over a 22-year period - no leaks and only 20% stricture rate.

Surgical repair of choanal atresia: Retropalatal endoscopic versus endonasal endoscopic technique

Surgical repair of choanal atresia: Retropalatal endoscopic versus endonasal endoscopic technique

Dandy-Walker syndrome with bilateral choanal atresia: A case report.

IntroductionDandy-Walker syndrome is a rare congenital brain malformation characterized by cerebellar vermis agenesis or hypoplasia, cystic dilatation of the fourth ventricle and a large posterior fossa causing upward displacement of tentorium and torcula. In this paper, we present a case of bilateral choanal atresia with Dandy-Walker Syndrome in a female newborn.Case presentationWe present a case of a female patient who was born at 38th weeks of gestation via emergency cesarean section due to non-reassuring cardiotocography and abnormal antenatal ultrasounds findings. The imaging revealed the presence of Dandy-walker malformation. The patient presented with cyanosis and respiratory distress. Bedside flexible nasoendoscopy revealed bilateral choanal atresia which is confirmed by computed tomography of sinuses. Endoscopic bilateral choanal atresia repair was performed. On postoperative follow up, nasal endoscopy showed bilateral intact flap and patent neochoana.Discussion and conclusionDandy-Walker syndrome is a congenital disorder that can be diagnosed prenatally. The syndrome is associated with multiple anomalies. However, there are few published reports of bilateral choanal atresia in Dandy-Walker Syndrome. Bilateral choanal atresia is considered a life-threatening condition in newborns that requires early surgical intervention.

Comparison of staged repair versus single-stage complete repair for pulmonary atresia with ventricular septal defect: A systematic review and meta-analysis.

To evaluate comparative outcomes of the repair of pulmonary atresia with ventricular septal defect (PAVSD), done as staged repair (SR) or single-stage complete repair (CR). A systematic online search was conducted using the following databases: PubMed, Scopus, Cochrane database, The Virtual Health Library, Clinical trials.gov, and ScienceDirect for studies comparing SR versus CR for treatment of PAVSD. Total mortality rate, operative and early post-operative mortality rate, post-operative ventilation duration, duration of post-operative intensive care unit (ICU) stay, need for post-operative extra-corporeal membrane oxygenation (ECMO) support rate, transcatheter re-intervention rate, unplanned re-operation rate, freedom from right ventricular outflow tract (RVOT) re-intervention, and length of hospital stay (LOS) were the evaluated outcome parameters. Four comparative studies reporting a total of 264 patients who underwent SR (167 patients) or CR (97 patients) were included. Total mortality was higher in the SR group compared to the CR group (odds ratio (OR) 2.58, P = 0.03). Two groups were comparable regarding operative and early post-operative mortality (OR 1.37, P = 0.62), post-operative ventilation duration (mean difference (MD) - 1.58, P = 0.43), need for post-operative ECMO support rate (OR 4.72, P = 0.16), transcatheter re-intervention rate (OR 0.60, P = 0.53), unplanned re-operation rate (OR 0.73, P = 0.57), and LOS (MD - 3.39, P = 0.45). Higher rate of freedom from RVOT re-intervention was observed in the SR group (OR 4.16, P = 0.00001). Single-staged early CR of PAVSD significantly reduced total mortality rate as compared with SR. However, there is life-long risk of frequent need for RVOT re-interventions. Future high-quality randomised studies with robust methodological qualities are encouraged to evaluate the optimal timing and technique for repair of PAVSD, by analysing more outcomes in large patient groups and multi-centre studies.

Preservation of the azygos vein versus ligation of the azygos vein during primary surgical repair of congenital esophageal atresia

Preservation of the azygos vein versus ligation of the azygos vein during primary surgical repair of congenital esophageal atresia

Novel Device for Endoluminal Esophageal Atresia Repair: First-in-Human Experience.

Thoracoscopic esophageal atresia (EA) repair affords many benefits to the patient; however, intracorporeal suturing of the anastomosis is technically challenging. Esophageal magnetic compression anastomosis (EMCA) is a compelling option for endoluminal EA repair, but available EMCA devices have prohibitive rates of recalcitrant stricture. Connect-EA is a new endoluminal EMCA device system that employs 2 magnetic anchors with a unique mating geometry designed to reliably create a robust anastomosis and decrease rates of leak and stricture. We describe our first-in-human experience with this novel endoluminal device for staged EA repair in 3 patients (Gross type A, B, and C) at high risk for conventional surgical repair. First, the esophageal pouches were approximated thoracoscopically. After acute tension subsided, the device anchors were endoscopically placed in the esophageal pouches and mated. Anchors were spontaneously excreted in 2 cases. Endoscopic repositioning and retrieval of the anchors were required in 1 patient because of narrowed esophageal anatomy. There were no perioperative complications. Patients were managed for 14 to 18 months. The strictures that developed in the patients were membranous and responded well to dilation alone, resolving after 4 to 5 outpatient dilations. Gastrostomies were closed between 6 and 11 months and all patients are tolerating full oral nutrition. Early experience with this new endoluminal EMCA device system is highly favorable. The device offers considerable benefit over conventional handsewn esophageal anastomosis and anastomotic outcomes are superior to available EMCA devices.

Value of Routine Bronchoscopy at Time of Esophageal Atresia Repair

Value of Routine Bronchoscopy at Time of Esophageal Atresia Repair

Infant study of hemispheric asymmetry after long-gap esophageal atresia repair.

ObjectivesPrevious studies have demonstrated that infants are typically born with a left‐greater‐than‐right forebrain asymmetry that reverses throughout the first year of life. We hypothesized that critically ill term‐born and premature patients following surgical and critical care for long‐gap esophageal atresia (LGEA) would exhibit alteration in expected forebrain asymmetry.MethodsTerm‐born (n = 13) and premature (n = 13) patients, and term‐born controls (n = 23) <1 year corrected age underwent non‐sedated research MRI following completion of LGEA treatment via Foker process. Structural T1‐ and T2‐weighted images were collected, and ITK‐SNAP was used for forebrain tissue segmentation and volume acquisition. Data were presented as absolute (cm3) and normalized (% total forebrain) volumes of the hemispheres. All measures were checked for normality, and group status was assessed using a general linear model with age at scan as a covariate.ResultsAbsolute volumes of both forebrain hemispheres were smaller in term‐born and premature patients in comparison to controls (p < 0.001). Normalized hemispheric volume group differences were detected by T1‐weighted analysis, with premature patients demonstrating right‐greater‐than‐left hemisphere volumes in comparison to term‐born patients and controls (p < 0.01). While normalized group differences were very subtle (a right hemispheric predominance of roughly 2% of forebrain volume), they represent a deviation from the expected pattern of hemispheric brain asymmetry.InterpretationOur pilot quantitative MRI study of hemispheric volumes suggests that premature patients might be at risk of altered expected left‐greater‐than‐right forebrain asymmetry following repair of LGEA. Future neurobehavioral studies in infants born with LGEA are needed to elucidate the functional significance of presented anatomical findings.

Stenting versus stentless repair for bilateral choanal atresia: A systematic review of the literature

BackgroundBilateral choanal atresia requires prompt surgical intervention. Surgeons have historically used stents in the repair process, however their efficacy has come into question in recent years. We performed a systematic review to investigate, primarily, whether stents enjoy more favourable outcomes compared to stentless repair. We also explored the use of operative adjuncts, such as steroids, antibiotics, mitomycin C and KTP laser. MethodsWe performed a search of the Medline and Embase databases using a search strategy developed with the assistance of an academic librarian. Only full peer reviewed articles were included. Abstracts, posters, case reports and proceedings of academic conferences were excluded. ResultsWe identified 48 unique articles for inclusion, composed of a meta-analysis, two randomised control trials and 45 case series. Pooled analysis of the two randomised control trials yielded no statistically significant difference in choanal patency between stented and stentless repair, but a statistically significant reduction in complications, specifically granulation tissue formation, was found in stentless repair. Data from case series were, overall, of mixed quality, making factors contributing to successful outcomes difficult to elucidate. ConclusionOverall, there is a lack of high quality evidence to support the use of either a stented or stentless approach to bilateral choanal atresia repair, however stentless repair may experience fewer complications. Operative techniques, such as the use of mucosal flaps, are worthy of future study. Authors call for future high quality randomised control trials to investigate this uncommon but important condition.

Functional Luminal Imaging Probe in the Management of Pediatric Esophageal Disorders.

Functional luminal imaging probe (FLIP) measures pressure-geometry relationships of digestive luminal space. When used in esophageal disorders, it provides several luminal parameters that help better understand the pathophysiology. Data about the potential utility of FLIP in pediatrics are scarce and there is no standardized use in children. We aim to describe the use of FLIP in our center, its safety, feasibility, and clinical impact in esophageal disorders in children. Consecutive FLIP recordings performed at the Centre Hospitalier Universitaire-Sainte-Justine, Montréal, Canada between February 2018 and January 2021 were extracted. A chart review was conducted for demographics and medical history. Symptomatology after the procedure was evaluated with validated dysphagia scores. Nineteen patients were included (11 girls, median age 16 years, range 3.2-19.6) with achalasia (n = 5), post-Heller's myotomy dysphagia (n = 3), esophagogastric junction outflow obstruction (n = 3), congenital esophageal stenosis (n = 2); post-esophageal atresia repair stricture (n = 3), and post-fundoplication dysphagia (n = 3). There was no significant correlation between integrated relaxation pressure measured with high resolution manometry and distensibility index (DI). The use of FLIP made it possible to differentiate between dysphagia related to an esophageal obstruction (DI < 2.8 mm2/mmHg) and dysphagia without major motility disorder (DI > 2.8 mm2/mmHg) that guided the indication for dilation. FLIP led to a change in management in 47% of the patients. Forty-seven percent of the patients were symptom free at the time of the evaluation. FLIP provides key esophageal luminal values and therefore can play an important role in pediatric esophageal disorders management.