Antti Koivusalo

Objective: Several surgical approaches are being used in the reconstruction of long-gap esophageal atresia. We investigated which methods are being used in the Nordic countries and the postoperative complications that occurred in the first year of life. Methods: This study is a retrospective multicenter study, where medical records on children with esophageal atresia Gross type C or D born in the period from 1 January 2000 to 1 May 2017 were reviewed. Results: Forty-four patients were included in this study, forty-three with Gross type C and one with Gross type D. Thirty-six patients were included in the statistical analysis. Delayed esophageal anastomosis was performed in half of the patients and an esophageal replacement procedure in the other half. Postoperative complications were common, but there was no difference in postoperative complications or weight-gain within the first year of life. There were no differences in hospital stay or duration of parenteral nutrition. Attempted primary esophageal anastomosis was significantly more common in patients that underwent an esophageal replacement procedure compared to those who underwent a delayed esophageal anastomosis. Conclusions: No significant relationship between surgical approach and postoperative complications within the first year of life could be demonstrated. Long-term functional studies are warranted.

Objective Etiology of vocal cord paralysis (VCP) and laryngeal dysfunction may be congenital or surgical trauma of recurrent and superior laryngeal nerves. We assessed the incidence, risk factors, and morbidity of VCP after repair of esophageal atresia (EA). Methods Medical records of 201 EA patients from 2000 to 2022 were reviewed for this retrospective study. Postrepair vocal cord examination (VCE) included awake nasolaryngeal fiberoscopy by otolaryngologist or laryngoscopy under spontaneous breathing anesthesia. Before 2017, postoperative VCE was performed in symptomatic patients only and routinely after 2017. Main Results Overall, VCE was performed on 79 (38%) patients (52 asymptomatic), whereas 122 asymptomatic patients underwent no VCE. VCP was diagnosed in 32 of 79 patients (right 12, left 10, and bilateral 10; symptomatic 25 and asymptomatic unilateral 7) corresponding with extrapolated overall VCP incidence of 16 to 24% among 201 patients including asymptomatic ones. Ten patients (bilateral VCP 8 and left VCP 2) required tracheostomy. Of 10 patients with bilateral VCP, three underwent laryngotracheal expansion surgery (left VC lateralization in one and laryngoplasty in two with acquired subglottic stenosis), three remained tracheostomy dependent, three were off tracheostomy, and one died of complications after redo esophageal reconstruction. All patients with unilateral VCP managed without tracheostomy. Cervical dissection or ostomy formation was a major risk factor of VCP. Conclusion Repair of EA is associated with a considerable risk of VCP and associated morbidity. Cervical EA surgery significantly increased the risk of VCP. Bilateral VCP may eventually require laryngotracheal expansion surgery.

Objective The aim of this study was to assess incidence, risk factors, and outcomes of distal recurrent tracheoesophageal fistula (RTEF) after repair of esophageal atresia (EA). Methods Ethical consent was obtained. Data collection was done by review of hospital records of 286 patients (268 in-house and 18 referred) who underwent repair of type C or D EA from 1980 to 2021. Spitz class, long-gap (tracheoesophageal fistula at carina), fundoplication, leakage, and stricture were assessed as RTEF risk factors. Outcome measures were long-term closure of RTEF, retainment of native esophagus, and survival. Results RTEF occurred in 23 patients (19 in-house) with type C (n = 22) or type D (n = 1) EA with median 4.4 (interquartile range [IQR]: 1.7–13) months after repair. Five patients had late RTEF 3.5 to 16 years after repair. Nineteen (7.3%) in-house patients developed RTEF. Presenting symptoms, age at diagnosis, and presence of anastomotic stricture (AS) are listed. No statistically significant risk factors were found, risk ratio of 0.5 to 2.7 (IQR: 0.1–8.7), p-value of 0.25 to 0.75. Detached closure clip and esophageal foreign body contributed to RTEF in two patients. Sixteen patients underwent rethoracotomy for closure of RTEF, 5 (22%) with AS eventually underwent esophageal reconstruction after a period with cervical esophagostomy. Two patients with late-manifested RTEFs underwent closure with laser cauterization. In 22 patients, treatment of RTEF succeeded, whereas 1 (4%) premature patient died of instant re-RTEF. Conclusion RTEF had an incidence of 7% with diverse patterns of manifestation and predicting factors. Closure rate and patient survival were excellent, but RTEF with AS predicted loss of native esophagus. Received: 01 June 2022 Accepted: 06 September 2022 Article published online: 05 December 2022 © 2022. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

The prevalence of Barrett’s esophagus (BE) in adults born with esophageal atresia (EA) is four times higher than in the general population and presents at a younger age (34 vs. 60 years). This is (partly) a consequence of chronic gastroesophageal reflux. Given the overlap between genes and pathways involved in foregut and BE development, we hypothesized that EA patients have an intrinsic predisposition to develop BE. Transcriptomes of Esophageal biopsies of EA patients with BE (n = 19, EA/BE); EA patients without BE (n = 44, EA-only) and BE patients without EA (n = 10, BE-only) were compared by RNA expression profiling. Subsequently, we simulated a reflux episode by exposing fibroblasts of 3 EA patients and 3 controls to acidic conditions. Transcriptome responses were compared to the differential expressed transcripts in the biopsies. Predisposing single nucleotide polymorphisms, associated with BE, were slightly increased in EA/BE versus BE-only patients. RNA expression profiling and pathway enrichment analysis revealed differences in retinoic acid metabolism and downstream signaling pathways and inflammatory, stress response and oncological processes. There was a similar effect on retinoic acid signaling and immune response in EA patients upon acid exposure. These results indicate that epithelial tissue homeostasis in EA patients is more prone to acidic disturbances.

Aim To assess the risk factors for anastomotic stricture (AS) in end-to-end anastomosis (EEA) in patients with esophageal atresia (EA). Methods With ethical consent, hospital records of 341 EA patients from 1980 to 2020 were reviewed. Patients with less than 3 months survival (n = 30) with Gross type E EA (n = 24) and with primary reconstruction (n = 21) were excluded. Outcome measures were revisional surgery for anastomotic stricture (RSAS) and number of dilatations required for anastomotic patency without RSAS. The factors that were tested for risk of RSAS or dilatations were distal tracheoesophageal fistula (TEF) at the carina in C-type EA (congenital TEF [CTEF]), type A/B EA, antireflux surgery (ARS), anastomotic leakage, recurrent TEF, and Spitz group and congenital heart disease. Main Results A total of 266 patients, Gross type A (n = 17), B (n = 3), C (n = 237), or D (n = 9) underwent EEA (early n = 240, delayed n = 26). Early anastomotic breakdown required secondary reconstruction in five patients. Of the remaining 261 patients, 17 (6.1%) had RSAS, whereas 244 patients with intact end to end required a median of five (interquartile range: 2–8) dilatations for anastomotic patency. Main risk factors for RSAS or (> 8) dilatations were CTEF, type A/B, ARS, and anastomotic leakage that increased the risk of RSAS or dilatations from 4.6- to 11-fold. Conclusion The risk of severe AS is associated with long-gap EA, significant gastroesophageal reflux, and anastomotic leakage. Received: 18 June 2021 Accepted: 01 October 2021 Publication Date: 25 November 2021 (online) © 2021. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Introduction The aim of this study was retrospective assessment of late major reoperations after surgery for necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) in neonates. Materials and Methods Appropriate ethical consent was obtained. Data collection was by review of hospital records of 165 successive prematures who underwent surgery for NEC (n = 99) or SIP (n = 66) from 1986 to 2019. Outcome measures were late major abdominal surgeries or endoscopies that occurred after the primary surgical treatment, closure of enterostomies, and weaning from parenteral nutrition (PN). We assessed also risk factors for late abdominal surgeries. Results Hundred and twenty-six (76%) patients (NEC, n = 70, 71% and in SIP, n = 56, 85%) survived. Median follow-up was 13 (interquartile range: 5.9–23) years. Nineteen (15%) patients underwent late abdominal surgery with 0% mortality. Most common was surgery for small bowel obstruction (SBO) in 12(9.5%) patients (NEC, n = 5; 7.0%, SIP, n = 7; 13%, p = 0.36) with 10-year cumulative risk of 8.7 (95% confidence interval [CI]: 3.5–14). Long duration of PN and development of intestinal failure associated cholestasis (IFAC) increased the risk of SBO surgery, relative risk: 1.0–3.2 (95% CI: 1.0–9.1), p = 0.02–0.03. Other surgeries included serial transverse enteroplasty (NEC, n = 2), incisional hernia repair (n = 3), cholecystectomy (n = 1), and ovarian torsion (n = 1). Twenty (29%) NEC and four (7%) SIP patients required endoscopic assessment of postoperative symptoms (p = 0.003). Conclusion Late abdominal reoperations occurred in 15% of patients with NEC and SIP with nil mortality. SBO was the main indication. Risk of SBO was similar between NEC and SIP. Long PN and IFAC increased the risk of SBO. Received: 10 July 2020 Accepted: 11 November 2020 Publication Date: 13 January 2021 (online) © 2021. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany

Introduction Several surgical techniques are available for pediatric esophageal reconstruction. We started to use pedicled jejunum interposition graft (PJIG) because other techniques had significant long-term complications. In this retrospective study, the indications, surgical complications, and long-term outcomes were assessed in patients with PJIG. Materials and Methods With ethical consent, we reviewed the hospital records of 14 patients (7 females) who from 2005 to 2019 underwent a total of 16 esophageal reconstructions with PJIG. Results Median age at PJIG was 1.6 (range: 0.2–15) years. Underlying conditions were esophageal atresia (EA) (n = 11) or native esophagus lost by trauma or infection (n = 3). Eight patients with EA underwent PJIG as primary reconstruction and three as a rescue operation after complications in primary repair. Significant surgical complications occurred in 43% of patients. Major reoperations in six (43%) patients included resection and reanastomosis of strictured proximal PJIG (n = 1) and redo PJIG after failure of the first operation (n = 2). Surgical mortality was nil. After a median follow-up of 6.5 (range: 0.7–14) years, 13 (93%) patients survived, and 1 died of congenital heart disease. PJIG failed in three (23%) survivors of whom two underwent graft removal because of life-threatening aspiration and one did not start oral feeds at all. Ten survivors (77%) have full enteral feeds. Respiratory function in the survivors is satisfactory. Two patients have moderate and three mild gastroesophageal reflux symptoms. Conclusion PJIG was a functional option for a variety of conditions that required esophageal reconstruction. However, significant early and late complications required major surgical revisions.