Adulthood of patients after oesophageal atresia repair

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– General Surgeon’s Guide

Pełnoletniość pacjentów po operacji naprawczej atrezji przełyku

– wskazówki dla specjalistów chirurgii ogólnej

Anna Tokarz

^1,2AEF

, Wojciech Rogula

^1,2AEF

, Karolina Tokarska

^1,2AEF

, Maciej Tarsa

^2AEF

, Witold Urban

^2AEF

,

Karolina Zbroja

^2AEF

, Wojciech Górecki

^1AEF

1Department of Pediatric Surgery, University Children’s Hospital, Jagiellonian University Medical College, Krakow, Poland;

Head: Wojciech Górecki MD PhD

2Student’s Scientific Group of Pediatric Surgery, Jagiellonian University Medical College, Krakow, Poland

Article history: Received: 03.12.2020 Accepted: 23.03.2021 Published: 25.03.2021

ABSTRACT: Introduction: Despite proper surgical management in neonatal period, congenital oesophageal atresia (OA) may present a serious source of lifelong disorders.

Aim: The objective of our study is to present the most important facts about oesophageal atresia, types of treatment and possible complications which might demand surgical management in adult life. We would like to familiarize general surgeons with this matter to provide OA patients with proper medical care.

Material and methods: We made a thorough literature review of Medline database and Cochrane Library with "(o)esophageal atresia", "long gap", "surgery" and “follow-up” used as keywords.

Results: Long-term observation reveals that patients with OA may need help from general surgeons, however do not often look for it. Even after surgical repair in neonatal period, OA indisposes to multiple concomitant disorders including gastroesophageal reflux disease, oesophagitis, strictures, epithelial metaplastic changes in the oesophagus or hiatal hernia development.

Endoscopic follow-up should be systematically performed in OA patients’ population in order to early detect possible pathologies.

Conclusions: Advancement in medicine allowed to improve survival rates and adulthood is reached more often in the population of OA patients. In the face of a variety of concomitant problems, systematic long-term follow-up seems to be crucial in proper medical care in these patients.

KEYWORDS: adult, endoscopy, follow-up studies, oesophageal atresia, surgeons

STRESZCZENIE: Wprowadzenie: Pomimo prawidłowego leczenia chirurgicznego w okresie noworodkowym, wrodzona atrezja przełyku (ang. oesophageal artesia; OA) może stanowić poważne źródło zaburzeń odczuwanych przez całe życie.

Cel: Celem niniejszej pracy jest przedstawienie najistotniejszych informacji na temat OA, rodzajów jej leczenia i możliwych powikłań, mogących wymagać leczenia chirurgicznego po osiągnięciu przez pacjenta pełnoletniości. Chcielibyśmy zapoznać specjalistów chirurgii ogólnej z problematyką dotyczącą tego schorzenia w celu zapewnienia właściwej opieki medycznej pacjentom z OA w wywiadzie.

Materiał i metody: Dokonano gruntownego przeglądu literatury zgromadzonej w bazie danych Medline i bibliotece Cochrane z wykorzystaniem fraz: (o)esophageal atresia, long gap, surgery i follow-up jako słów kluczowych.

Wyniki: Wyniki długoterminowej obserwacji wskazują, że pacjenci z OA mogą wymagać pomocy ze strony chirurgów ogólnych, choć nie zawsze tej pomocy poszukują. Nawet po wykonaniu operacji naprawczej w okresie noworodkowym, OA predysponuje do licznych chorób współistniejących, takich jak: choroba refluksowa przełyku, zapalenie przełyku, zwężenie przełyku, metaplazja nabłonka przełyku czy przepuklina rozworu przełykowego. Pacjentów z OA należy systematycznie poddawać kontrolnym badaniom endoskopowym, mającym na celu wczesne wykrycie ewentualnych zaburzeń.

Wnioski: Postęp w medycynie pozwolił na poprawę wskaźników przeżycia, w związku z czym pacjenci z OA częściej osiągają pełnoletniość. W obliczu różnorodnych problemów, będących następstwem atrezji, kluczowe znaczenie w prawidłowej opiece medycznej u tych osób wydaje się mieć długoterminowa obserwacja kontrolna.

SŁOWA KLUCZOWE: atrezja przełyku, chirurdzy, dorośli, endoskopia, obserwacja kontrolna

Authors’ Contribution:

A – Study Design B – Data Collection C – Statistical Analysis D – Manuscript Preparation E – Literature Search F – Funds Collection

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allows for primary anastomosis, longer would cause massive tension, leakage or stenosis and reflux [2, 7]. The oesophageal pouches grow faster than an infant so gaps longer than 2 vertebral bodies might allow for a delayed primary anastomosis at 8 to 12 weeks of age [2]. Other approaches that can be used when the primary repair is not possible are traction techniques or oesophageal replacement with the stomach segments, small intestine or colon [8].

The most recent approach postulates the superiority of traction techniques and primary anastomosis over the graft even in gaps longer than 3.5 cm [9].

Surgeons divide OA in terms of length between oesophageal pouches, but the division is quite fluent and depends on a particu- lar surgeon [9, 10]. Some surgeons claim that long-gap OA is “in the eye of the beholder” [9]. In practice, most of them consider OA as a long gap when an experienced surgeon is unable to perform primary anastomosis [10]. Recently, International Network of Esophageal Atresia stated that LGOA is every case of OA with an absence of intra-abdominal air, which means every OA without distal TOF [11]. It is recommended to refer these LGOA patients to special pediatric surgical centers which are experienced in tre- ating such conditions [11].

SURGICAL MANAGEMENT OF SHORT-GAP

OESOPHAGEAL ATRESIA

Thoracotomy

The main aim of the procedure is ligation of TOF (when presented) and reconstruction of the oesophageal continuity. Right po- sterolateral thoracotomy is performed with carefulness to avoid muscle damage. The most recommended approach is entry through the 4^th intercostal space. When the right-sided aortic arch is presented there is a need for an analogous left thoracotomy. With the preferred, extrapleural approach the posterior mediastinum is reached and the azygos vein is exposed. Then the fistula is disclosed, dissected and ligated (transfixing sutures are recommended). It is important to identify correctly the me- diastinal structures such as the major bronchus or aorta to avoid their accidental dissection. Then the upper oesophageal pouch is dissected, mobilized and both pouches are sewn together with a single layer of absorbable sutures to create an anastomosis. In this case interrupted sutures are the preferred option [4, 12, 13]. It is relevant to insert a transanastomotic tube to avoid subsequent lumen strictures. In most cases there is no need for chest drain [13].

Thoracoscopy

According to the recent publication from the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA), thoracoscopy is a viable option in the treatment of OA. This approach offers multiple advantages (possibility of magnification, better cosmesis, fewer musculoskeletal deformities), but concurrently requires longer operative time and background in this field. There are contradictory opinions on whether thoracoscopy provides faster recovery as compared to thoracotomy. When it comes to technical demands, surgery cannot last longer than 3 hours and the insufflation pressure of CO2 cannot be higher than 5 mmHg [13]. Evenone of the first retrospective reviews on

ABBREVIATIONS

BO – Barrett’s oesophagus

ERNICA – European Reference Network for rare Inherited Congenital Anomalies

GORD – gastro-oesophageal reflux disease LC – lesser gastric curvature

LGOA – long gap oesophageal atresia OA – oesophageal atresia

TOF – tracheo-oesophageal fistula

INTRODUCTION

The term oesophageal atresia (OA) with or without tracheo-oeso- phageal fistula (TOF) describes a cluster of congenital anomalies, where the continuity of the oesophagus is interrupted and inappropriate connections (fistulas) between the oesophageal and tracheal lumen may appear. OA occurs with a prevalence of roughly 1 in 2500–3500 births [1, 2]. In Poland, this problem concerns 75–90 newborns annually [3].

OA is usually diagnosed in the first 24 hours after birth [4].

It should be suspected when neonate is choking, frothing, sali- vating excessively and presenting with respiratory distress [5].

However, congenital OA may be a serious source of lifelong surgical issues despite the fact it demands appropriate surgical help in the neonatal period. This review aims to familiarize mainly general surgeons with this issue to provide this group of patients with a proper medical care.

MATERIALS AND METHODS

The PubMed and Cochrane Library online databases were thoro- ughly searched using (o)esophageal atresia as a keyword. Only ar- ticles in English that referred to OA in human species and infants (birth – 23 months) were included. To focus on surgical methods of OA repair, especially long-gap oesophageal atresia (LGOA), and on possible long-term complications, we included additional keywords:

“long-gap”, “surgery” and “follow-up” in the next step of searching.

The selection based on titles, abstracts and relevant articles was cho- sen and subsumed. We also used relevant information from pediatric surgery textbooks to make this review as complete as possible.

CLASSIFICATION

First classification of OA was made by Vogt in 1929, and it is still used nowadays. It segregates variations of this condition according to anatomical differences. The other classifications made by Gross, Ladd and Kluth, are subsequent modifications of Vogt’s work [1, 6]. Differences and similarities between Vogt, Gross, Ladd and Kluth classifications are shown in Fig. 1. The most common ver- sion of OA is atresia with distal TOF (Vogt IIIB/Gross C type) [1].

SURGICAL MANAGEMENT OF OA

Surgical management mainly depends on the size of gap between oesophageal pouches: smaller than 2 vertebral bodies or 1 cm

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Fig. 1. Differences and similarities between Vogt, Gross, Ladd and Kluth classifications of OA [1, 6].

There was not any known case

Kluth VII subtypes Kluth IIIa subtypes

Kluth VIII subtypes Kluth IIIb subtypes

Kluth IIIc subtypes

<1%

7% 2% 2%

4%

Kluth I and II – many subtypes

Kluth IIIb₁₉

Ladd IV Ladd V

Gross D Gross E Gross F

Vogt III C Vogt IV

Ladd I Ladd II Ladd III

Gross A Gross B Gross C

Vogt II

Vogt I Vogt III A Vogt III B

Kluth I₁

thoracoscopic repair of OA with TOF since 2005 has shown that the thoracoscopic approach is safe and effective [14]. The leak rate (7.6%), stricture rate (3.8%) and fundoplication rate (24.0%) were comparable to the results of open operations. Patients needed oesophageal dilatation (31.7% of cases), however the overall mortality was low (in the quoted review: 2.8%, three deaths verified, one related to a surgery complication, among all the 104 patients) [14]. Thoracoscopic technique has been improved during recent years, as shown by a meta-analysis from 2020. Perusal of 17 studies with 1043 cases of OA showed that thoracoscopy compared with thoracotomy provides for a shorter hospital stay and lower risk of: leakage (OR 1.92), stricture formation (OR 2.66), stricture dilatation (OR 1.90) and mortality (OR 1.18). However, speciali- sts emphasize higher necessity for antireflux surgery (OR 2.12) and still longer duration of thoracoscopic operation compared to thoracotomy [15]. Thoracoscopic repair can be performed through transpleural or extrapleural approach. In the first case the insufflation of the pleural space causes right lung collapse.

This procedure provides a good view of the superior vena cava, azygos vein, phrenic and vagus nerves. The main disadvanta- ges are strictures, present in 50% of cases [16]. The extrapleural access is obtained by dissection of intercostal muscles and in- serting a trocar avoiding the pleura. This procedure allows for insufflation of carbon dioxide to obtain an additional operative area. It is believed this approach prevents a leak to the pleural space and empyema [17].

There is a necessity of a randomized trial, which could compare these two methods [14, 15].

SURGICAL MANAGEMENT OF LONG-GAP

OESOPHAGEAL ATRESIA

There are two main courses: elongation of the oesophageal pouches followed by delayed primary anastomosis or grafts derived from other parts of the gastrointestinal tract. Current research has revealed the superiority of oesophageal reconstruction over oesophageal replacement. Most pediatric surgeons believe that preservation of the native oesophagus should be a priority, being a more long-term and satisfactory approach [18].

Elongation and delayed primary anastomosis

One of the traction techniques was described by Foker et al. [19].

With transpleural approach, sutures are placed around the oesophageal pouches and in the prevertebral fascia. It is believed that using many small, tension-free sutures allows to obtain an anastomosis capable of holding out an intense stress. After 3 or 4 days the chest is reopened. If the distance is still too large, the sutures are placed with traction on the patient’s back. The growth is sti- mulated by gradually increasing tension and observed on X-ray.

The anastomosis is performed when the distance is about 1 cm.

From 1984 to 2005 the University of Minnesota Hospital performed 5 operations with internal traction (gap between segments:

3.7–6.0 cm), 23 operations with external (gap 3.7–10.0 cm) or mi- xed traction (gap 3.6–12.5 cm). The complications were: pulling out the sutures because of tension, adhesions and lack of progress.

These problems required reoperations, but in every case the primary anastomosis was achieved. There was no evidence of over- stretching or damage of the oesophageal muscle. After operation the coordinated peristalsis occurred only to the anastomotic line, but the passage was possible owing to spontaneous contractions.

Patients had a tendency toward reflux and stricture. In those cases the dilations and Nissen fundoplications were performed [9, 19].

One of the latest methods of oesophageal elongation is thoracoscopic traction technique which has been developed as a minimal invasive procedure preceding a delayed primary anastomosis. There is a technique described by Foker but performed thoracoscopically. In 2015, a retrospective single-center review with regard to this technique was published by Van der Zee et al. Authors described their experience with the treatment of 10 patients with LGOA by that new method and also an evolution of the technique. Primarily, they started with performing gastrostomy – then followed by pla- cing traction sutures into both pouches of the oesophagus. Later, they started to perform laparoscopic gastropexy against the anterior wall of the abdomen to stop the dislocation of the stomach to the chest, so that there was no need to perform gastrostomy anymore.

The change in the gap length was assessed by pre- and postoperative chest X-rays. The traction was maintained for approxima- tely a few days and then the primary anastomosis was performed.

The procedure was successful in 8 children. Due to the traction,

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Group I Group II Group III birth weight > 1500 g

WITHOUT severe heart defects birth weight < 1500 g OR

severe heart defects birth weight < 1500 g AND severe heart defects

survival rate according to Spitz [1] 97% 59% 22%

survival rate in years 1975–1989 [38] 94.3% 70.0% 20.0%

survival rate in years 1990–2011 [38] 100% 94.1% 50.0%

Tab. I. Survival rates according to the criteria of Spitz [1, 38].

an increased risk of gastro-oesophageal reflux disease (GORD) was observed. Summarizing, the thoracoscopic traction technique is a minimally invasive procedure to deal with LGOA and it can be performed directly after birth without the use of accompanying gastrostomy; however, the procedure is not always effective [20].

Another traction technique is extrathoracic oesophageal elongation known as Kimura’s technique. This method consists of initial oesophagostomy, sequential extrathoracic oesophageal elongation and definite reconstruction. The incision is made above the right clavicle, lateral to the retracted sternocleidomastoid muscle.

The upper oesophageal segment is identified, dissected and bro- ught to the skin. During the recovery, patients develop the swal- lowing mechanism by sham feeding and simultaneously are fed by gastrostomy. The elongation operation consists of dissection and stretching of the oesophagus and it can be performed every 2 or 3 months. Every time the gap is measured by means of fluoroscopic assessment. When the primary anastomosis is possible, it is performed thoracoscopically or with open reconstruction [21, 22].

However, this technique takes a few months to gain some length.

Nowadays, in the presence of other available methods, this beco- mes unacceptable.

Internal static traction is another alternative method of surgical repair of LGOA. This method, described by Patkowski, consists of thoracoscopic placement of a traction suture between both pouches. Planned rethoracoscopy is performed after 4 days.

Depending on the size of the gap, either repetitive traction suture placement or oesophageal anastomosis is performed. This technique allows to maintain traction internally and avoid infectious complications resulting from unnecessary interference with the outside. It also protects patients from negative consequences of an open surgery. The negative side of this procedure is the need for reoperation [23, 24]. Within 4-10 days, with 2–4 thoracoscopic traction procedures, however, primary anastomosis is achievable, and the avoidance of gastrostomy is possible [24].

Another method of surgery for LGOA is suture fistula technique.

It was first described in 1974 by Shafer and David [25]. A few years later Schullinger et al. also presented the effects of that method among their patients [26]. This technique consists of division and closure of TOF followed by closure of the lower oesophageal pouch and its approximation to the mobilized but unopened upper pouch. The next step of the procedure is placement of a tight central suture through the lumen of both oesophageal pouches.

Unprompted fistulization should occur a few weeks after surgery. The fistula was subsequently dilated by using a woven silk bo- ugie. A noticeable drawback after that procedure was sympto- matic GORD. Stringel et al. also described a similar technique based on suture approximation of both oesophageal segments and

fistulization following endoscopic and fluoroscopic placement of a string for guided dilatations [10].

Hendren and Hale proposed an alternative method of lengthening the oesophageal pouches by using metal bougies and electromagnetic field to approximate both pouches and perform subsequent anastomosis [27]. In 2016 Dorman et al. published a case report of a patient treated by endoscopic magnetic compression anastomosis. They accomplished the anastomosis with multi-stage approach consisting of thoracoscopic internal Foker procedure, then open thoracotomy with tracheoesophageal fistula closure and oesophageal lengthening by suture approximation and finally placement of catheters with magnets in both oesophageal pouches. The last step of the procedure allowed them to achieve a connection between both pouches without reoperation [28].

Circular myotomy can also be used in the management of LGOA.

It was first used by Livadatis to lengthen the proximal part of the oesophagus [29]. The basis of this method is to supply blood to the proximal pouch from the thyrocervical trunk. It is suggested that this should be an adjunct procedure before primary anastomosis in LGOA [30].

Oesophageal replacement with other parts of the

gastrointestinal tract

Gastric transposition

This method involves stomach mobilization, distal oesophageal pouch dissection, pyloroplasty, proximal oesophageal pouch dissection, creating space in the posterior mediastinum, pulling up the stomach with its blood supply to the mediastinum and anastomosis with the cervical oesophagus [31]. Critics emphasize that the stomach can constrict lungs and thus be the cause of recurrent aspirations, and that vagotomy is connected with delayed gastric emptying [8]. However, the experience with 26 patients showed no respiratory problems. Delayed gastric emptying was noticed in five patients and only three of them demonstrated it at the last follow-up. Gastric transposition is an effective method and could be performed in complications following primary repair, e.g. reflux, massive stricture or oesophageal dysfunction [32].

Gastric tube

The oesophagus is made of the greater curvature of the stomach.

This procedure consists of division of the gastrocolic omentum and right gastro-epiploic artery, placement of a chest tube in the stomach to preserve an appropriate amount of tissue to form a gastric tube, division of short gastric vessels, gastric tube creation with a stapler and anastomosis with the cervical oesophagus.

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survival rate of patients with OA. However, they are included in Tab.

I. with the outcomes of more recent studies to show the improve- ment through the years.

Long-term surgical complications

From the general surgeon’s point of view, it is important to be fami- liar with possible long-term complications of OA repair which may demand appropriate surgical management in adult life.

As a congenital disease impairing the oesophagus, OA predisposes to multiple oesophageal disorders even after proper surgical repair. OA is almost always connected with dysfunction of oesophageal motility as a result of inborn abnormalities and also surgical treatment [1, 39]. Congenital or postoperative vagal nerve damage predisposes to delayed gastric emptying [40]. Aforementioned factors as well as excessive anastomotic tension, mainly in LGOA, increase the risk of GORD [40, 41]. That disorder tends to persist throughout life [41–43]. Over time, patients become accustomed to the reflux symptoms, so they may not report them spontaneously. However, endoscopy follow-up should be systematically performed to avoid negative GORD consequences [42, 43]. GORD may lead to mucosal damage, oesophagitis, strictures and also epithelial metaplastic changes [42, 44, 45]. Antireflux medications such as proton pump inhibitors are recommended as the first-line treatment [46]. Fun- doplication is recommended in case of conservative therapy failure, although specific indications vary among different centers. Oeso- phagitis frequently occurs in this group of patients and predisposes to development of premalignant lesion called Barrett’s Oesophagus (BO) [40]. It is defined as a presence of intestinal metaplasia within the oesophageal epithelium. BO is associated with an increased risk of progression to oesophageal adenocarcinoma [44]. Anastomotic stenosis may appear as a result of excessive tension between both ends of the oesophagus in LGOA and also as the cause of GORD. It requires an endoscopy or contrast X-ray scan to establish a proper diagnosis. Repeated endoscopic dilations remain the first-line treatment [40]. Previous oesophageal surgery and presence of short oesophagus predispose to hiatal hernia development and may be a next factor increasing GORD symptoms. Operation of sliding hernia is required in case of conservative anti-reflux treatment failure or when the complications e.g. strictures, ulceration or repeated bleeding are present. Due to the high risk of complications, paraoesophage- al hernia should always be surgically repaired. Operative management usually includes hiatus repair or Nissen fundoplication [47].

GUIDANCE FOR ENDOSCOPY

Endoscopy follow-up should be performed:

1. every 5–10 years, 2. if new symptoms occur,

3. if long-lasting symptoms get worse [38, 46].

During endoscopy, it is relevant to exclude the possibility of eosinophilic esophagitis, especially when the patient displays following symptoms: recurrent strictures, long-lasting dys- phagia, treatment resistance. An increased risk of eosinophilic esophagitis results not only from complications of OA surgery (e.g. GORD), but also from common mutations in the For- khead box gene, taking part in the formation of both conditions.

Blood supply is provided by the left gastro-epiploic artery [8, 33].

According to the summary of literature data, the gastric tube has twice as many cases of leaks and strictures as every other method (leaks – 69% patients, strictures – 53%). However, these complications did not impact the number of deaths (4% patients) [32].

Schärli’s technique

A gap amounting to 6–8 cm can be compensated by elongation of the lesser gastric curvature (LC). Originally, the surgeon must ligate the left gastric artery, preferably after the second branch, with an aim of eliciting a good supply to the distal oesophagus and concurrently to release LC from vascular fixation. Afterwards the surgeon performs oblique incision of LC. The new oesophagus can be transferred intrathoracically or retrosternally. However, the second route shows fewer pulmonary complications and does not demand thoracotomy. Among eight patients treated with this method, the most frequent complications were stenosis (4 cases), aspiration (4 cases) and anastomotic leak (2 cases) [34].

Colon interposition

Using a colon graft is another method of oesophageal replacement.

Nowadays two surgical approaches are used. First of them is based on using the right colon in a retrosternal position. Blood supply is provided by ileocolic vessels. The alternative method is left or transverse colon interposition based on the left colic artery in the left re- trohilar position by using the transpleural technique [33]. The procedure consists of colon mobilization, division of appropriate blood vessels and dissection of selected segments of the colon. Restora- tion of gastrointestinal tract continuity is achieved by end-to-end anastomosis. In the right colon interposition, neck incision is made, oesophageal pouches are mobilized, a selected segment of the colon with the vascular pedicle is transferred through the retrosternal tunnel and anastomosis is performed. In the left/transverse colon interposition either single thoracoabdominal incision or separa- te abdominal and thoracic incisions are made. Colon is displaced through an incision in the diaphragm and through the left pleural cavity into the neck. Then, anastomosis with both oesophageal pouches is performed [33].

Small bowel interposition

It consists of division of the jejunal segment with its vascular pedicle, restoration of jejunal continuity, graft transposition into the thoracic cavity and anastomosis with both oesophageal pouches [35]. The biggest problem is blood supply, so some surgeons tried to use microvascular anastomosis to the internal mammary artery to improve the results [8]. Although it is a demanding technique, it is preferred because of the lower risk of GORD in comparison to other graft methods and owing to graft ability to grow with the same speed as the child. Also, the risk of necrosis is low in experienced hands and it seems to be beneficial in some LGOA cases [11, 36, 37].

Survival rates

Spitz determined survival rates depending on birth weight and presence of heart defects [1]. Admittedly the techniques of opera- ting OA have been changing through the last decades and the criteria of Spitz stopped to be sufficient determinants to predict the

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report disturbing symptoms – some of them could get used to va- rious symptoms present since childhood [50].

CONCLUSIONS

Advancement in medicine allowed to improve the survival rates in the population of patients with diagnosed OA. This is thanks to better surgical management as well as higher level of neonatal care. This means more patients with OA reach adulthood and need help from general surgeons. It is relevant that general surgeons possess knowledge on this condition and procedures resto- ring the continuity of the oesophagus. Therefore, it is important to know there is a variety of surgical management procedures, all the more some of them are not often used nowadays and fast access to the information can be constricted. Furthermore, patients with repaired OA need accurate follow-up in adulthood. Despite being at a higher risk of gastroesophageal diseases such as GORD, BO and carcinoma, they do not often seek medical care. On the contrary, they get used to symptoms experienced since childhood or develop adaptive behaviors. That is why it is so relevant to treat them with a deep understanding of this condition and alert- ness, also oncological.

The possible portrayal in endoscopy:

1. swelling, exudate – in child population,

2. swelling, corrugated oesophagus, longitudinal grooves, crêpe- paper mucosa in adult population,

3. no visible changes: 7–32% cases [48].

BO in endoscopy in patients after OA repair is described as “velve- ty red tongue-like structure” above the gastroesophageal junction.

It is relevant to remember that anatomical terms of location are not always preserved and there is a need for locating gastric folds, especially in gastric transposition [49]. If we suspect BO, biopsies should be taken in four quadrants in every centimeter [46].

Endoscopists should always keep in mind the higher risk of squamous cell carcinoma or adenocarcinoma in this group of patients.

The very early symptoms of these conditions are small mucosal irregularity. There are simple methods to improve their detecta- bility. First of them uses acetic acid for high visibility of the squ- amocolumnar junction. Equally helpful can be Lugol’s iodine, which is not taken at sites of squamous dysplasia [46]. The best way to diagnose carcinoma is to perform endoscopy very carefully and always keep in mind that it is possible even if patients do not

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The full terms of this license are available on: https://creativecommons.org/licenses/by-nc/4.0/legalcodeode Wojciech Rogula MD; Department of Pediatric Surgery, University Children’s Hospital, Jagiellonian University Medical College, Krakow; Wielicka street 265, 30-663 Krakow, Poland; Phone: +48 12 333 91 52, +48 12 333 91 53;

E-mail: woj.rogula@gmail.com

Tokarz A., Rogula W., Tokarska K., Tarsa M., Urban W., Zbroja K., Gorecki W.: Adulthood of patients after oesophageal atresia repair – General Surgeon’s Guide; Pol Przegl Chir 2021; 93(3): 27-34

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