Authors

  • R.O. Sulaymonova
  • S.K. Yulchiev
  • T.Kh. Adjimamatov

DOI:

https://doi.org/10.71337/inlibrary.uz.jnci.97656

Keywords:

Keywords: Esophageal atresia tracheoesophageal fistula pure esophageal atresia neonatal surgery congenital esophageal anomaly surgical management thoracic surgery delayed anastomosis primary anastomosis postoperative care

Abstract

Annotation: This article explores the selection of surgical treatment strategies for congenital esophageal atresia without tracheoesophageal fistula in children—one of the most complex and severe forms of esophageal malformations in neonates. This condition poses significant diagnostic challenges, presents with critical clinical symptoms, and necessitates urgent surgical intervention. The study analyzes various clinical presentations, preoperative diagnostic methods, general condition assessments, and the development of individualized surgical plans tailored to each patient. Special emphasis is placed on thoracic surgical approaches, the use of minimally invasive techniques, gastrostomy, primary versus delayed anastomosis, as well as postoperative care and long-term outcomes. The conclusion underscores that proper selection of the surgical method plays a crucial role in improving survival rates and enhancing the overall quality of life for affected children.


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SURGICAL MANAGEMENT STRATEGIES FOR ESOPHAGEAL

ATRESIA WITHOUT TRACHEOESOPHAGEAL FISTULA IN NEONATES

R.O. Sulaymonova, S.K. Yulchiev, T.Kh. Adjimamatov

Andijan State Medical Institute, Republic of Uzbekistan

Annotation:

This article explores the selection of surgical treatment strategies for

congenital esophageal atresia without tracheoesophageal fistula in children—one of
the most complex and severe forms of esophageal malformations in neonates. This
condition poses significant diagnostic challenges, presents with critical clinical
symptoms, and necessitates urgent surgical intervention. The study analyzes various
clinical presentations, preoperative diagnostic methods, general condition assessments,
and the development of individualized surgical plans tailored to each patient. Special
emphasis is placed on thoracic surgical approaches, the use of minimally invasive
techniques, gastrostomy, primary versus delayed anastomosis, as well as postoperative
care and long-term outcomes. The conclusion underscores that proper selection of the
surgical method plays a crucial role in improving survival rates and enhancing the
overall quality of life for affected children.

Keywords:

Esophageal atresia, tracheoesophageal fistula, pure esophageal

atresia, neonatal surgery, congenital esophageal anomaly, surgical management,
thoracic surgery, delayed anastomosis, primary anastomosis, postoperative care

YANGI TUG‘ILGAN CHAQALOQLARDA TRAXEOEZOFAGEAL

FISTULASIZ QIZILO‘NGACH ATREZIYASINI JARROHLIK YO‘LI BILAN

DAVOLASH STRATEGIYALARI

R.O`. Sulaymonova, S.K. Yo‘lchiyev, T.X. Adjimamatov

Andijon davlat tibbiyot instituti, O‘zbekiston Respublikasi

Annotatsiya:

Ushbu maqolada bolalarda uchraydigan tug‘ma qizilo‘ngach

atreziyasi (TQA) ning eng murakkab shakllaridan biri — oqmasiz turi (atresia
esophagus without tracheoesophageal fistula) klinik holatlari va ularni xirurgik
davolash usullarini tanlash masalalari tahlil qilinadi. Mazkur patologiya erta yoshdagi
bolalarda og‘ir klinik kechish, tashxis qo‘yishdagi murakkabliklar va tezkor jarrohlik
aralashuvining zarurligi bilan ajralib turadi. Tadqiqot davomida kasallikning turli
klinik shakllari, preoperatsion diagnostika usullari, bemorlarning umumiy ahvoli,
ularning organizmiga moslashgan individual jarrohlik strategiyalari o‘rganildi.
Maqolada torakal jarrohlik, minimal invaziv texnologiyalar, gastrostomiya, birlamchi
va ikkilamchi anastomozlar qo‘llanilishi, shuningdek, operatsiyadan keyingi parvarish
va uzoq muddatli natijalarga alohida e’tibor qaratildi. Xulosa sifatida, davolash usulini


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to‘g‘ri tanlash bolaning yashab qolish imkoniyatini va hayot sifatini sezilarli darajada
oshirishi ta’kidlandi.

Kalit so‘zlar:

Qizilo‘ngach atreziyasi, traxeoezofageal fistula, sof qizilo‘ngach

atreziyasi, neonatal jarrohlik, tug‘ma qizilo‘ngach anomaliyalari, jarrohlik muolajasi,
torakal jarrohlik, kechiktirilgan anastomoz, birlamchi anastomoz, operatsiyadan
keyingi parvarish

ХИРУРГИЧЕСКИЕ СТРАТЕГИИ ЛЕЧЕНИЯ АТРЕЗИИ ПИЩЕВОДА БЕЗ

ТРАХЕОПИЩЕВОДНОГО СВИЩА У НОВОРОЖДЁННЫХ

Р.О. Сулаймонова, С.К. Юлчиев, Т.Х. Аджимаматов

Андижанский государственный медицинский институт,

Республика Узбекистан


Аннотация:

В данной статье рассматриваются особенности выбора

хирургической

тактики

при

врожденной

атрезии

пищевода

без

трахеопищеводного свища — одной из наиболее тяжелых форм патологии
пищевода у новорожденных. Эта аномалия отличается сложной диагностикой,
тяжелым течением и требует срочного оперативного вмешательства. В
исследовании проанализированы различные клинические варианты заболевания,
методы предоперационной диагностики, общее состояние пациентов и
индивидуальные подходы к выбору хирургической тактики. Особое внимание
уделено методам торакохирургии, применению малоинвазивных технологий,
гастростомии, первичным и отсроченным анастомозам, а также ведению
послеоперационного периода и отдаленным результатам. В заключении
подчеркивается, что правильный выбор метода лечения значительно повышает
шансы на выживание и улучшает качество жизни ребенка.

Ключевые слова:

Атрезия пищевода, трахеопищеводный свищ, чистая

атрезия пищевода, неонатальная хирургия, врождённые аномалии пищевода,
хирургическая тактика, торакальная хирургия, отсроченный анастомоз,
первичный анастомоз, послеоперационный уход

Esophageal atresia (EA) is a congenital anomaly characterized by an interruption

in the continuity of the esophagus. It occurs in approximately 1 in 2,500 to 4,000 live
births and represents one of the most critical conditions encountered in neonatal
surgery. While the majority of cases (around 85–90%) are associated with a
tracheoesophageal fistula (TEF), approximately 7–10% of patients present with
isolated or "pure" esophageal atresia, where no communication exists between the
esophagus and trachea. This rare variant is often associated with more complex clinical
management, owing to its unique anatomical configuration and the absence of gastric


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air on radiographs, which complicates early diagnosis.Pure esophageal atresia typically
presents with signs of excessive salivation, feeding difficulties, and respiratory distress
shortly after birth. However, the lack of a TEF can delay recognition and intervention,
especially in centers where neonatal surgical expertise is limited. In addition, this form
of EA is often accompanied by other congenital anomalies, including cardiac, renal,
and vertebral defects, which further complicate management and prognosis. The
decision-making process regarding surgical correction is particularly challenging in
these cases, as the esophageal segments are often significantly separated, making
primary anastomosis technically difficult or even impossible in the neonatal period.The
importance of timely and appropriate surgical intervention cannot be overstated. In the
absence of a tracheoesophageal fistula, neonates are unable to swallow saliva or feed
orally, necessitating prompt establishment of alternative nutritional routes, such as
gastrostomy or parenteral nutrition. Furthermore, the timing and type of esophageal
repair—whether primary, delayed primary, or esophageal replacement—must be
carefully tailored to each patient’s anatomy, overall condition, and associated
anomalies. In recent decades, advances in neonatal intensive care, surgical techniques,
and perioperative management have significantly improved outcomes; however,
morbidity and mortality remain higher in this subgroup compared to other types of EA.

Given the rarity and complexity of pure esophageal atresia, optimal surgical

strategy remains a subject of ongoing debate among pediatric surgeons. Various
approaches have been described in the literature, including delayed primary
anastomosis after esophageal elongation, staged reconstruction with initial
gastrostomy, cervical or thoracic esophagostomy, and, in severe cases, esophageal
substitution using stomach, colon, or jejunal interposition. Each method carries its own
risks and benefits, and the decision largely depends on factors such as the length of the
gap between the proximal and distal esophageal segments, the neonate’s general
condition, and the presence of associated anomalies. Despite improvements in
perioperative care, complications such as anastomotic leakage, strictures,
gastroesophageal reflux, and long-term feeding difficulties remain significant
concerns.Moreover, in low- and middle-income countries, including Uzbekistan, the
management of such complex congenital anomalies is further challenged by limitations
in neonatal intensive care resources, delayed referral, and variable access to advanced
imaging and minimally invasive surgical techniques. As a result, standardized
treatment protocols are often lacking, and individualized surgical decision-making
becomes essential to improving outcomes.

The present study aims to evaluate and analyze surgical management strategies

employed in neonates with pure esophageal atresia treated at a regional pediatric
surgical center. Through retrospective review and clinical assessment, we aim to
identify effective treatment algorithms based on gap length, patient stability, and


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postoperative outcomes. By comparing our clinical experience with existing
international standards, we seek to contribute to the development of practical
guidelines suited to resource-constrained settings and to enhance overall survival and
quality of life in affected neonates.

Esophageal atresia has been a subject of surgical interest since the first successful

repair was reported by Cameron Haight in 1941. Over the decades, the management of
EA with a tracheoesophageal fistula has become relatively standardized; however,
isolated or pure esophageal atresia remains a major clinical challenge due to the
absence of a distal fistula and the typically long gap between the esophageal segments.
The literature suggests that these patients account for 7–10% of all EA cases and often
require highly individualized treatment plans.Numerous studies have focused on
classifying esophageal gap length and its influence on treatment strategy. According to
Spitz and others, long-gap EA is defined by an inability to bring the two esophageal
ends together without tension, and this is frequently the case in isolated EA. In such
instances, initial management typically involves the placement of a gastrostomy tube
to allow enteral nutrition, while various methods are employed to encourage
esophageal growth, such as bougienage, traction sutures, or magnetic compression
anastomosis. Delayed primary anastomosis, usually performed after 6–12 weeks, has
shown promising outcomes in centers with specialized neonatal surgical teams and
access to modern intensive care.

Alternative techniques, such as esophageal replacement with gastric transposition,

colonic interposition, or jejunal grafts, have been employed when native esophageal
repair is not feasible. These methods, however, are associated with significant long-
term morbidity, including strictures, dysmotility, reflux, and nutritional challenges.
Studies by Kimura et al. and Foker et al. introduced and refined traction-based
techniques that allow the esophagus to elongate over time, enabling primary
anastomosis in many previously inoperable cases. However, these procedures require
specialized expertise and infrastructure not always available in low-resource
settings.Several publications have emphasized the importance of multidisciplinary care
in improving surgical outcomes, including the roles of neonatologists, radiologists,
anesthesiologists, and nutritionists. Despite advances in surgical technique, the
literature indicates that mortality and morbidity remain higher in patients with pure EA
compared to those with TEF-associated variants, particularly in cases with concomitant
anomalies or delayed diagnosis.

To date, very few studies from Central Asia, including Uzbekistan, have

documented institutional experience with pure EA, especially regarding decision-
making in resource-limited environments. Therefore, our study seeks to fill this gap by
analyzing clinical cases managed in a regional surgical center, offering insights into
practical, adaptable surgical approaches suited to similar healthcare contexts.This


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study was conducted as a retrospective clinical review at the Pediatric Surgery
Department of Andijan State Medical Institute, Uzbekistan, over a six-year period from
January 2018 to December 2023. The primary aim was to analyze the surgical
approaches, intraoperative findings, and postoperative outcomes in neonates diagnosed
with isolated (pure) esophageal atresia, specifically cases lacking a tracheoesophageal
fistula. The study was approved by the institutional ethical committee, and all patient
information was handled with confidentiality according to international ethical
standards.A total of

18 neonates

were included in the study based on specific inclusion

and exclusion criteria. The

inclusion criteria

were as follows:

A confirmed diagnosis of esophageal atresia without any associated

tracheoesophageal fistula.

Neonates admitted within the first 7 days of life.

Availability of full preoperative, intraoperative, and postoperative medical

records.

Birth weight of ≥1500 grams and gestational age of ≥32 weeks.

Exclusion criteria involved:

Esophageal atresia cases associated with any type of tracheoesophageal fistula

(Types C, D, or E).

Severe life-threatening comorbidities incompatible with surgery (e.g., lethal

cardiac defects).

Neonates who expired before surgical intervention or whose records were

incomplete.

Diagnosis was established based on a combination of clinical signs and imaging

studies. Key clinical indicators included:

Excessive drooling and accumulation of oral secretions.

Inability to pass a nasogastric tube beyond 10–12 cm from the gum line.

Episodes of coughing and cyanosis upon feeding attempts.

Radiological confirmation was made using plain chest and abdominal X-rays

showing:

Coiling of the nasogastric tube in the proximal esophageal pouch.

Absence of intra-abdominal gas — a hallmark of isolated EA.

Additional diagnostic modalities such as contrast esophagograms or computed

tomography were utilized in selective cases to assess gap length or rule out associated
anomalies.Each neonate underwent thorough preoperative evaluation, including
echocardiography and abdominal ultrasound, to identify coexisting congenital
anomalies (cardiac, renal, vertebral, or anorectal), which were documented and
considered in the overall treatment planning. Preoperative stabilization included
thermoregulation, intravenous fluids, antibiotics, head-up positioning, and continuous
suctioning of the upper pouch to prevent aspiration.


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The choice of surgical strategy for each patient was determined based on the

clinical condition of the neonate, the anatomical findings during surgery, and the
estimated gap length between the proximal and distal esophageal segments. Gap length
was assessed intraoperatively using a calibrated bougie introduced through the
proximal pouch and, in selected cases, by contrast imaging. Based on the feasibility of
tension-free anastomosis, patients were divided into two main groups:

1.

Primary Anastomosis Group (n = 7):

These neonates had a relatively short esophageal gap (≤2.5 cm) and underwent

one-stage thoracotomy with end-to-end esophageal anastomosis. The procedure was
performed via a right posterolateral extrapleural thoracotomy through the fourth
intercostal space. The proximal and distal esophageal ends were mobilized, and a
single-layer interrupted or double-layer continuous suturing technique using
absorbable sutures (5-0 or 6-0 polydioxanone) was used depending on tissue condition.

2.

Staged Repair Group (n = 11):

Patients with a long esophageal gap (>2.5 cm) or significant preoperative

instability underwent an initial

gastrostomy

for enteral feeding and

cervical

esophagostomy

or conservative upper pouch suctioning. Delayed primary anastomosis

was planned after 8–12 weeks, following weight gain and improved clinical stability.
In some cases, esophageal elongation techniques (serial bougienage or traction sutures)
were employed prior to definitive repair. In cases where native esophageal continuity
could not be achieved, esophageal substitution (gastric pull-up or colonic interposition)
was considered.

All procedures were performed under general anesthesia with preoperative and

postoperative intensive care monitoring. Postoperative management included
mechanical ventilation as required, intravenous antibiotics, parenteral nutrition or
feeding via gastrostomy, and serial chest X-rays to monitor for complications such as
anastomotic leaks or pneumothorax.

Clinical outcomes were evaluated based on several key parameters:

Early postoperative complications:

anastomotic leakage, strictures, wound

infection, and pneumonia.

Late complications:

gastroesophageal reflux, feeding intolerance, need for

esophageal dilatation.

Survival rate:

recorded at discharge and at 6-month follow-up.

Length of hospital stay

and

time to initiation of oral feeding

were also noted.

Follow-up assessments were carried out at 1, 3, and 6 months postoperatively

through clinical examination, feeding evaluation, and radiological imaging
(esophagogram) when indicated.



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CONCLUSION

The surgical management of esophageal atresia without tracheoesophageal fistula

remains one of the most challenging areas in neonatal surgery, particularly in resource-
limited settings. Our clinical experience over a six-year period demonstrates that
individualized treatment strategies based on gap length, neonatal stability, and
available surgical resources can lead to satisfactory outcomes even in cases with
complex anatomical presentations.

Primary esophageal anastomosis is feasible and effective in selected cases with a

short gap, offering the advantage of early restoration of esophageal continuity.
However, in long-gap variants, a staged approach involving initial gastrostomy,
conservative management, and delayed repair remains a safe and pragmatic solution,
especially when advanced traction techniques or endoscopic tools are not accessible.

Despite the inherent risks of complications such as anastomotic strictures,

gastroesophageal reflux, and feeding difficulties, careful perioperative care and timely
surgical decision-making significantly improve survival and quality of life. Our study
highlights the importance of multidisciplinary coordination, early diagnosis, and the
need for practical, adaptable surgical protocols in regions with limited specialized
infrastructure.

Future directions should include the development of standardized algorithms

tailored to low- and middle-income healthcare systems, capacity building in neonatal
intensive care, and adoption of innovative yet affordable techniques to improve
outcomes in neonates with pure esophageal atresia.

REFERENCES

1.

Spitz, L. (2007). Oesophageal atresia. Orphanet Journal of Rare Diseases, 2(1), 24.
https://doi.org/10.1186/1750-1172-2-24

2.

Foker, J. E., Linden, B. C., Boyle, E. M., & Marquardt, C. (1997). Development of
a true primary repair for the full spectrum of esophageal atresia. Annals of Surgery,
226(4), 533–541. https://doi.org/10.1097/00000658-199710000-00011

3.

Kimura, K., Soper, R. T., & Kimura, S. (1994). Esophageal elongation with traction
sutures in the treatment of long-gap esophageal atresia. Journal of Pediatric
Surgery, 29(9), 1205–1207.

4.

Myers, N. A., & Morgan, A. S. (1983). Esophageal replacement in children: current
status. Progress in Pediatric Surgery, 16, 96–115.

5.

Tovar, J. A., & Fragoso, A. C. (2013). Current controversies in the surgical
management of esophageal atresia and tracheoesophageal fistula. World Journal of
Gastroenterology, 19(30), 4813–4820. https://doi.org/10.3748/wjg.v19.i30.4813

6.

Harmon, C. M., & Coran, A. G. (2009). Congenital anomalies of the esophagus. In
A. G. Coran (Ed.), Pediatric Surgery (7th ed., pp. 1051–1071). Elsevier Saunders.


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7.

Bhatnagar, V. (2012). Long gap esophageal atresia: An Indian experience. Journal
of Neonatal Surgery, 1(3), 36. https://doi.org/10.21699/jns.v1i3.100

8.

Ein, S. H., & Shandling, B. (1984). The long gap esophageal atresia—The case for
delayed primary anastomosis. Journal of Pediatric Surgery, 19(3), 313–316.

9.

Leva, E., Macchini, F., Duci, M., & Morandi, A. (2015). Esophageal atresia: full
spectrum management in a single center. European Journal of Pediatric Surgery,
25(02), 123–129. https://doi.org/10.1055/s-0034-1387930

10.

Zani, A., & Pierro, A. (2010). Esophageal replacement in children: Past and
present. Journal of Pediatric Surgery, 45(1), 206–210.

Author Information:

O‘g‘iloy Rashidjon qizi Sulaymonova

– Master's Student,

Department of Pediatric Surgery, Andijan State Medical Institute,

Republic of Uzbekistan

Email address:

Ugiloyrashidovna@gmail.com


References

Spitz, L. (2007). Oesophageal atresia. Orphanet Journal of Rare Diseases, 2(1), 24. https://doi.org/10.1186/1750-1172-2-24

Foker, J. E., Linden, B. C., Boyle, E. M., & Marquardt, C. (1997). Development of a true primary repair for the full spectrum of esophageal atresia. Annals of Surgery, 226(4), 533–541. https://doi.org/10.1097/00000658-199710000-00011

Kimura, K., Soper, R. T., & Kimura, S. (1994). Esophageal elongation with traction sutures in the treatment of long-gap esophageal atresia. Journal of Pediatric Surgery, 29(9), 1205–1207.

Myers, N. A., & Morgan, A. S. (1983). Esophageal replacement in children: current status. Progress in Pediatric Surgery, 16, 96–115.

Tovar, J. A., & Fragoso, A. C. (2013). Current controversies in the surgical management of esophageal atresia and tracheoesophageal fistula. World Journal of Gastroenterology, 19(30), 4813–4820. https://doi.org/10.3748/wjg.v19.i30.4813

Harmon, C. M., & Coran, A. G. (2009). Congenital anomalies of the esophagus. In A. G. Coran (Ed.), Pediatric Surgery (7th ed., pp. 1051–1071). Elsevier Saunders.

Bhatnagar, V. (2012). Long gap esophageal atresia: An Indian experience. Journal of Neonatal Surgery, 1(3), 36. https://doi.org/10.21699/jns.v1i3.100

Ein, S. H., & Shandling, B. (1984). The long gap esophageal atresia—The case for delayed primary anastomosis. Journal of Pediatric Surgery, 19(3), 313–316.

Leva, E., Macchini, F., Duci, M., & Morandi, A. (2015). Esophageal atresia: full spectrum management in a single center. European Journal of Pediatric Surgery, 25(02), 123–129. https://doi.org/10.1055/s-0034-1387930

Zani, A., & Pierro, A. (2010). Esophageal replacement in children: Past and present. Journal of Pediatric Surgery, 45(1), 206–210.