Transition in Esophageal Atresia: Medical, Psychosocial, and Structural Aspects of Lifelong Care

Abstract

Esophageal atresia (EA) is often perceived as a pediatric surgical diagnosis with treatment concluded after repair. In reality, EA represents a complex chronic condition requiring lifelong monitoring. Beyond surgical sequelae, patients are at risk for gastrointestinal, respiratory, and musculoskeletal long-term complications, frequently accompanied by associated malformations and psychosocial challenges. Transition from pediatric to adult-centered healthcare is particularly critical but remains insufficiently structured in Germany. Comprehensive, multidisciplinary teams for adults are scarce, and existing pilot projects are limited to single institutions. Consequently, pediatricians often act as key coordinators during transition, while patient organizations provide essential support through information, peer networks, and practical tools. Sustainable care structures – including dedicated transition centers, interdisciplinary networks, and standardized transfer protocols – are urgently needed to ensure continuity of care. Such frameworks would help to secure medical safety, psychosocial stability, and an independent quality of life for individuals with EA into adulthood.

Keywords

oesophageal atresia/esophageal atresia - transition - adult medicine - chronic condition - holistic care

Introduction

Esophageal atresia (EA) has traditionally been understood as a pediatric surgical condition. Parents are often left with the impression that the disease is resolved following surgery and childhood. However, the reality is more complex: the patient journey does not end with surgical correction nor with the attainment of adulthood. While some families develop considerable acceptance of ongoing medical problems, this often results in relevant complications remaining undetected or untreated. The transition from pediatric to adult medical care is especially challenging. In Germany, major gaps persist, as specialized multidisciplinary care teams for adults with EA are still rarely established. This article highlights the key medical, psychosocial, and structural aspects of transition into adult care.

Transition in Medicine – Definition, Current Status, and Relevance in Esophageal Atresia

Defining Transition vs. Transfer

Transition (from the Latin transitio = passage) refers to the planned, multi-year process of moving from pediatric to adult health care. Its goal is to promote self-management, psychosocial maturity, and health-related autonomy [1], [2]. By contrast, transfer refers to the specific moment of handover, such as referral to an adult clinic [3]. Without preparatory transition, this often results in care discontinuities and poorer health outcomes [1], [2], [3]. National and international guidelines therefore emphasize structured preparation, individualized planning, and interprofessional coordination [4], [5], [6]. The German S3 guideline explicitly calls for the involvement of adolescents, parents, and adult health providers [6].

Current Situation in Germany

Germany currently lacks a nationwide, standardized transition strategy [7], [9]. Model initiatives such as TRANSLATE-NAMSE have provided valuable approaches, but remained locally limited [7], [12]. Surveys indicate that most pediatric departments have not implemented formal concepts [8], [9]. Moreover, the legal age threshold of 18 often enforces an abrupt transition, with insufficient preparation and psychosocial support.

A survey from pediatric nephrology illustrates this gap: more than 80% of clinics had no written agreements in place, yet emphasized the need for structured transition strategies [8]. Similarly, adults with EA are often inadequately connected to primary care structures [15] (see [Table 1]).

Table 1 Gaps in Care in Germany – Current vs. Desired State.

Area	Current State	Target State
Transition Programs	Only pilot projects, locally restricted	Implemented nationwide
Age Limit	Abrupt at 18 years	Flexible, individually adapted
Adult Care Structures	Few specialized centers	Multidisciplinary centers nationwide
Follow-up Planning	Rarely documented	Standardized plans with handover meeting
Pediatrics–Adult Communication	Fragmented	Clear interfaces, designated contacts
Psychosocial Support	Insufficient	Integral part of transition concept

Esophageal Atresia – An Example of Complex Follow-up Care

Esophageal atresia (EA) is a congenital malformation of the esophagus and trachea that cannot be fully corrected by anastomotic surgery alone [33], [34]. Consequently, lifelong monitoring is essential. Long-term care must address both medical and psychosocial aspects, including additional anomalies associated with VACTERL as well as long-term sequelae of surgical treatment. This underscores the importance of continuous, interdisciplinary follow-up care that integrates both physical and psychological dimensions and actively includes family systems.

Gastroesophageal Long-term Problems

Recent reviews highlight increasing evidence for the lifelong gastrointestinal vulnerability of patients with EA [10]. While surgical reconstruction restores anatomical continuity, it only incompletely replaces the complex neuromuscular control of the esophagus. This frequently results in persistent motility-associated complaints. A personalized follow-up approach, guided by functional symptoms as well as endoscopic and manometric findings, is increasingly advocated [10].

Motility Disorders and Neural Control

Many patients suffer from disturbances of the oral phase, bolus transport, and lower sphincter control. Damage to the vagus nerve and its branches–particularly the recurrent laryngeal nerve–is thought to play a role [28], [29]. As these pathways are essential for motility, swallowing, and vocal cord function, dysfunction can cause not only dysphagia but also hoarseness, vocal cord paresis, and an increased risk of aspiration. These problems often emerge only in later childhood or adolescence, requiring interdisciplinary follow-up (e.g., gastroenterology, speech therapy, otolaryngology).

Gastroesophageal Reflux Disease (GERD)

GERD is one of the most common long-term complications, affecting about half of adolescents and nearly all adults with EA [18], [19]. Symptoms are often nonspecific, and even asymptomatic patients may develop clinically significant pathology. Possible complications include strictures, eosinophilic esophagitis, dumping syndrome, Barrettʼs metaplasia, and even carcinoma [18]. Regular surveillance endoscopies (EGD), ideally every 5 – 10 years, are therefore mandatory [18], [19].

Nutrition and Growth

Children with EA often develop aversions to certain foods or fear of choking, [16] which may lead to eating disorders and growth impairment. In adulthood, distinct differences from the general population become apparent: 21% of patients are underweight, while only 2% are obese [15]. Speech and nutritional therapy remain important beyond childhood and adolescence to prevent malnutrition and related complications [15], [17]. This is particularly relevant in cases where primary care was surgical only and long-term structured follow-up was lacking.

Respiratory Long-term Complications

Respiratory problems are among the central sequelae of EA [11], [13], [14]. Tracheomalacia, occurring in up to one-third of patients, [11] is especially common. Tracheal instability may lead to chronic cough, recurrent infections, atelectasis, and eventually bronchiectasis. Symptoms such as stridor, wheezing, or dyspnea are often misinterpreted in childhood as asthma or croup. Microaspiration-related bronchitis is also frequent.

Diagnostics

Assessment is interdisciplinary and includes pulmonary function tests, flexible and rigid bronchoscopy, and imaging studies. Low-dose CT or real-time MRI with dynamic breathing maneuvers allow visualization of tracheal and bronchial collapse phenomena [13], [14], [35].

Therapy and Management

Therapeutic options range from inhalations (saline, N-acetylcysteine) and structured respiratory physiotherapy (e.g., autogenic drainage, PEP systems) to surgical interventions such as tracheopexy in severe cases [11], [13], [14]. Antibiotic strategies modeled after cystic fibrosis care are also established [14]. Education of parents–and later patients themselves–on disease management, including keeping standby antibiotics for acute situations, is crucial, even in adulthood.

Structured Long-term Care

Ongoing respiratory physiotherapy should be prescribed as a standard part of follow-up care [13]. In cases of recurrent infections or signs of chronic lung damage, transition to specialized bronchiectasis clinics (pediatric or pulmonary) is advisable to ensure regular monitoring and early intervention [14]. For further details, see “Pulmonary Problems in Children with Esophageal Atresia” in this issue.

Musculoskeletal Sequelae and Scoliosis

After open thoracotomy, many EA patients develop musculoskeletal sequelae such as rib fusions, thoracic asymmetries, and scoliosis [17], [32]. Minimally invasive thoracoscopic techniques significantly reduce this risk [20].

A recent prospective MRI study from Leipzig provided the first morphological and functional evidence in real time: children after open EA repair showed rib fusions and adhesions (78%) as well as scoliosis (15%) significantly more often than thoracoscopically operated children or healthy controls [32]. Thoracic development was also impaired, with reduced right-sided lung volumes and asymmetric thoracic movement. The number of thoracotomies emerged as a key risk factor for reduced thoracic volumes.

These findings highlight that musculoskeletal and pulmonary sequelae after thoracotomy are frequently underestimated and require targeted follow-up. In addition to clinical and imaging surveillance, physiotherapy, breathing exercises, and sports activities should be actively encouraged. Appropriate sports not only support physical development but also strengthen psychosocial stability (See “Supporting Physical Literacy in Patients with Esophageal Atresia”).

Psychosocial Aspects and Quality of Life

Despite substantial medical progress, many patients continue to report impairments in everyday life. These include reduced physical capacity, limitations in school, education, or work, and challenges in relationships and quality of life [15], [21], [22], [23]. Dysphagia-related problems in social situations (e.g., eating together) and school absenteeism due to recurrent respiratory infections are particularly distressing, contributing to emotional stress and reduced quality of life [21], [22], [31].

Moreover, studies show an increased prevalence of psychiatric disorders such as depression, ADHD, and autism in EA patients [24]. Therefore, psychological health should be routinely assessed during follow-up. In addition to medical care, involvement of patient support groups (e.g., KEKS, SoMA) and empathetic, open physician–patient communication are critical factors for sustaining long-term quality of life (see [Table 1]).

Recommendations for Outpatient Care (See [Table 2])

Table 2 Recommendations for Outpatient Follow-Up in Esophageal Atresia.

Area	Recommended Measure	Frequency/Comment
Gastrointestinal	EGD with biopsies (strictures, GERD, Barrettʼs, EoE)	Every 5 – 10 years or symptom-based
Respiratory	Pulmonary function, if needed bronchoscopy, respiratory therapy	Every 1 – 2 years, immediately if infections occur
Nutrition	Weight, BMI, eating behavior	Annually
Musculoskeletal	Inspection for posture, scoliosis	Annually
Psychosocial	EA-QoL, screening for depression/ADHD/autism	Regularly
Vaccinations	Pneumococcus, influenza, pertussis, Hib, optional: RSV (Respiratory Syncytial Virus) and COVID-19	Check vaccination status regularly
Networking	KEKS/NEKS health folder, participation in self-help groups	Continuously

Transition into adult medicine is particularly critical, as specialized multidisciplinary teams remain rare in Germany. Against this background, the following recommendations apply for outpatient care:

• Gastrointestinal follow-up: Regular endoscopies (EGD) with biopsies, even if asymptomatic, to detect complications such as strictures, GERD, Barrettʼs metaplasia, or eosinophilic esophagitis early.

• Respiratory diagnostics: Pulmonary function testing every 1 – 2 years; bronchoscopy for recurrent infections or chronic cough; respiratory physiotherapy and encouragement of age-appropriate physical activity.

• Nutritional status: Regular assessment of weight, BMI, and eating behavior; early referral to nutrition specialists and speech therapists if needed.

• Musculoskeletal screening: Clinical exam for postural issues and scoliosis; imaging if indicated.

• Psychosocial support: Routine assessment of quality of life (e. g., EA-QoL questionnaire); referral to psychotherapy or social services as appropriate.

• Prevention: Verification and updating of vaccinations, particularly pneumococcus, influenza, pertussis, and Hib, optional: RSV (Respiratory Syncytial Virus) – new vaccination options: e. g., nirsevimab (passive immunization during the first winter of life), especially for infants with risk factors. Consider also: COVID-19 (according to STIKO recommendations for children from 6 months of age with underlying conditions).

• Self-help and networking: Recommendation to engage with patient organizations such as KEKS or SoMA; use of the KEKS health folder as a structured documentation and communication tool.

These recommendations emphasize that follow-up care for EA patients must be understood not as a completed surgical intervention but as a lifelong interdisciplinary task.

Stakeholders in Comprehensive Follow-up Care

Pediatricians as Key Actors

Pediatricians, who often accompany chronically ill children for many years, play a central role in the transition process. With their knowledge of disease course, family dynamics, and psychosocial factors, they can initiate transition early, integrate age-appropriate education, and develop individualized plans.

Targeted assessments enable evaluation of self-management, disease processing, and psychosocial maturity. On this basis, measures such as transition clinics, internships in adult outpatient care, or structured handover discussions can be planned. In practice, however, these approaches often fail due to lack of resources and insufficient networking with adult medicine. Adult physicians are frequently unprepared for the complex needs of EA patients, underscoring the importance of binding communication structures and interdisciplinary collaboration.

Patient Organizations as Bridge Builders

Self-help organizations such as KEKS or SoMA complement medical care with information, peer support, and transition programs. Their offerings range from workshops and transition booklets to mentoring by older patients. These initiatives promote health literacy, adherence, and quality of life [25], [26].

In addition, patient organizations act as mediators between families and care structures: they address fears, clarify expectations, and build trust in new medical teams. Their continuous involvement helps mitigate disruptions in the transition process [30].

Designing a “Transfer Arrival Station”

A central goal for future care structures should be the development of a Transfer Arrival Station–a real or virtual space where handovers from pediatric to adult medicine are coordinated and supported [7], [27]. Essential components could include:

• Joint transition clinics with pediatric and adult providers

• Structured transfer plans with relevant disease and treatment history

• Transition coordinators or “navigators”

• Psychosocial support from social work and psychology

• Participatory follow-up discussions to evaluate the transition

Such a “station” would be more than just a handover point: it would provide empowerment, validation, and collaboration, closing the “black hole” between care systems and offering adolescents and families both safety and orientation.

Conclusion

The transition of children and adolescents with chronic diseases–particularly rare and complex conditions such as esophageal atresia–remains a critical yet insufficiently structured phase of care. While surgical repair resolves the acute phase, numerous gastrointestinal, pulmonary, musculoskeletal, and psychosocial sequelae necessitate continuous interdisciplinary follow-up.

Currently, Germany faces major deficits: lack of nationwide transition programs, rigid age limits, insufficient networking with adult medicine, and very few established multidisciplinary structures. These care gaps pose not only medical risks but also impair quality of life and psychosocial stability.

Until structured transition concepts are institutionally anchored, pediatricians remain key actors: as continuous points of contact, they can actively shape transition through early education, motivation, and referral to supportive networks. Patient organizations such as KEKS and SoMA provide essential additional resources by offering information, peer support, and practical assistance.

In the long term, however, binding political, institutional, and financial frameworks are required: transition centers, interdisciplinary networks, structured handover processes, and defined “arrival stations” that coordinate and accompany the move into adult care. Only through such sustainable structures can the transition truly succeed–ensuring not only the prevention of complications but also a stable, healthy, and self-determined adult life for individuals with esophageal atresia and comparable conditions.

Conflict of Interest

The authors declare that they have no conflict of interest.

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Publikationsverlauf

Artikel online veröffentlicht:
10. Oktober 2025