This dual case study details the multidisciplinary diagnosis and management of two young patients, with differing symptoms and etiology, who were supported by the Esophageal and Airway Treatment Program at Children’s Hospital of Philadelphia.
Patient 1
Background
A 9-month-old full-term male presented with chronic cough, noisy breathing and feeding difficulty. Prior evaluations at multiple institutions – including investigations for aspiration, H-type tracheoesophageal fistula (TEF), and diffuse lung disease – were unrevealing, and his symptoms persisted without improvement.
After independently learning about the Esophageal and Airway Treatment Program at CHOP (EAT@CHOP), his parents sought evaluation in our multidisciplinary clinic.
Given the refractory nature of his symptoms, our team recommended a comprehensive triple scope, microlaryngoscopy and bronchoscopy (MLB), flexible bronchoscopy, and esophagogastroduodenoscopy (EGD), as the first diagnostic step.
Diagnostic findings
1/23/25: Triple endoscopy demonstrated severe distal tracheomalacia with significant dynamic collapse and copious secretions throughout the tracheobronchial tree. What had been interpreted elsewhere as a possible tracheal diverticulum was clarified to be an atretic tracheal bronchus.
No laryngeal cleft, H-type TEF, or esophageal pathology was identified. These findings confirmed that his chronic cough and noisy breathing were attributable to tracheomalacia.
Treatment
Initial therapy included Atrovent® TID, bethanechol TID, and hypertonic saline during respiratory illnesses. Despite optimized medical management, the baby continued to experience persistent noisy breathing and wet cough, with symptoms worsening in winter months. After thorough shared decision-making, the family elected to pursue surgical intervention.
7/24/25: The baby underwent thoracoscopic rotational esophagoplasty and posterior tracheopexy. The thoracic esophagus was mobilized away from the trachea, and under bronchoscopic guidance, the posterior tracheal membrane was secured to the anterior longitudinal ligament of the spine, restoring airway patency during exhalation.
Outcomes
The patient recovered remarkably well, with discharge on post-operative day 2. At follow-up, his symptoms had completely resolved. According to his parents, he had experienced “a complete turn-around” and was now “living his best life.” They shared that CHOP was the first place they “felt heard,” and that after treatment at EAT@CHOP, they finally “had [their] baby back.”
Patient 2
Background
This 3-month-old male was born with Type C esophageal atresia with distal tracheoesophageal fistula (EA/TEF) and underwent early right thoracotomy with TEF ligation and primary EA repair. His initial postoperative course was uncomplicated, and he was discharged from the NICU in stable condition.
Two months later, he experienced an apnea episode associated with feeding, prompting emergency evaluation and hospitalization. EAT@CHOP was consulted, and a triple scope was arranged.
Diagnostic findings
9/2/25: Triple endoscopy revealed a broad posterior tracheal wall and severe tracheomalacia at the proximal thoracic trachea, with diffuse bronchial edema and copious thin secretions.
CTA demonstrated marked dynamic narrowing of the proximal thoracic trachea at the level of the crossing innominate artery.
Treatment
The infant was started on medical therapy for tracheomalacia while a surgical plan was developed. Before surgery could be scheduled, however, he suffered another apnea episode with feeding and required readmission, prompting urgent operative intervention.
9/24/25: The baby underwent cervical posterior tracheopexy, redo right thoracotomy, rotational esophagoplasty, thoracic tracheoplasty, and posterior tracheopexy, reconstituting the normal shape of the trachea and effectively opening his airway.
Outcomes
The infant required two days of PICU monitoring and was discharged post-operative day 8. At follow-up, he tolerated goal feeds without respiratory distress, demonstrated excellent growth, and had no further apneic episodes.
A recent viral illness was managed successfully at home, an important milestone given his prior instability. His parents shared that EAT@CHOP not only “saved [their] son’s life,” but also allowed them to “cuddle [their] little man tonight, and every night for as long as he’ll let [them].”
Discussion
Tracheomalacia is defined as dynamic collapse of ≥50% of the tracheal lumen during expiration, leading to symptoms such as noisy breathing, barky cough, feeding-related respiratory distress, recurrent lower respiratory infections, and cyanotic or “blue spells.” It is strongly associated with EA/TEF, yet is frequently misdiagnosed as asthma, delaying definitive treatment.
At the Esophageal and Airway Treatment Program at CHOP, diagnosis is established through a multidisciplinary triple-scope evaluation combining ENT-performed MLB, pulmonology-performed flexible bronchoscopy, and gastroenterology-performed endoscopy. This approach ensures a comprehensive assessment of vocal cord function, laryngeal anomalies, TEF recurrence, any esophageal pathologies, and dynamic airway collapse in real time.
All findings are reviewed at our multidisciplinary airway conference, where individualized management plans are developed. Most patients improve with optimized medical management, but surgical intervention is recommended for severe symptoms, especially recurrent respiratory infections, or life-threatening events such as apnea or cyanosis.
Surgical strategies aim to eliminate dynamic tracheal collapse and are tailored to each patient’s anatomy and disease location.
Anterior approach
- Aortopexy/Innominopexy: involves elevating vascular structures off the trachea
- Anterior tracheopexy: includes lifting anterior cartilage and anchoring it to the sternum
Posterior approach
- Rotational esophagoplasty: involves mobilizing the esophagus away from the posterior trachea
- Posterior tracheopexy: includes anchoring the posterior membrane to the anterior longitudinal ligament of the spine
These procedures may be performed via open or minimally invasive techniques, depending on patient anatomy and prior surgical history.
The authors are all from Children’s Hospital of Philadelphia and collaborated in the care of these patients. Duy T. Dao, MD, MPH, is an attending pediatric surgeon with the Division of Pediatric General, Thoracic and Fetal Surgery. Joseph Piccione, DO, MS, is Co-Director of the Advanced Diagnostic and Interventional Bronchoscopy Center, and Pulmonary Co-Director for the Center for Pediatric Airway Disorders and Esophageal and Airway Treatment Program. Richard Paul Boesch, DO, Pelton A. Phinizy, MD, Aoife Corcoran, MD, and Antoinette Wannes Daou, MD, are attending physicians with the Division of Pulmonary and Sleep Medicine. Michael A. Manfredi, MD, is a gastroenterologist with the Division of Gastroenterology, Hepatology and Nutrition. Thomas E. Hamilton, MD, is an attending pediatric surgeon with the Division of Pediatric General, Thoracic and Fetal Surgery. Dr. Manfredi and Dr. Hamilton are co-directors of the Esophageal and Airway Treatment (EAT) Program.
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This dual case study details the multidisciplinary diagnosis and management of two young patients, with differing symptoms and etiology, who were supported by the Esophageal and Airway Treatment Program at Children’s Hospital of Philadelphia.
Patient 1
Background
A 9-month-old full-term male presented with chronic cough, noisy breathing and feeding difficulty. Prior evaluations at multiple institutions – including investigations for aspiration, H-type tracheoesophageal fistula (TEF), and diffuse lung disease – were unrevealing, and his symptoms persisted without improvement.
After independently learning about the Esophageal and Airway Treatment Program at CHOP (EAT@CHOP), his parents sought evaluation in our multidisciplinary clinic.
Given the refractory nature of his symptoms, our team recommended a comprehensive triple scope, microlaryngoscopy and bronchoscopy (MLB), flexible bronchoscopy, and esophagogastroduodenoscopy (EGD), as the first diagnostic step.
Diagnostic findings
1/23/25: Triple endoscopy demonstrated severe distal tracheomalacia with significant dynamic collapse and copious secretions throughout the tracheobronchial tree. What had been interpreted elsewhere as a possible tracheal diverticulum was clarified to be an atretic tracheal bronchus.
No laryngeal cleft, H-type TEF, or esophageal pathology was identified. These findings confirmed that his chronic cough and noisy breathing were attributable to tracheomalacia.
Treatment
Initial therapy included Atrovent® TID, bethanechol TID, and hypertonic saline during respiratory illnesses. Despite optimized medical management, the baby continued to experience persistent noisy breathing and wet cough, with symptoms worsening in winter months. After thorough shared decision-making, the family elected to pursue surgical intervention.
7/24/25: The baby underwent thoracoscopic rotational esophagoplasty and posterior tracheopexy. The thoracic esophagus was mobilized away from the trachea, and under bronchoscopic guidance, the posterior tracheal membrane was secured to the anterior longitudinal ligament of the spine, restoring airway patency during exhalation.
Outcomes
The patient recovered remarkably well, with discharge on post-operative day 2. At follow-up, his symptoms had completely resolved. According to his parents, he had experienced “a complete turn-around” and was now “living his best life.” They shared that CHOP was the first place they “felt heard,” and that after treatment at EAT@CHOP, they finally “had [their] baby back.”
Patient 2
Background
This 3-month-old male was born with Type C esophageal atresia with distal tracheoesophageal fistula (EA/TEF) and underwent early right thoracotomy with TEF ligation and primary EA repair. His initial postoperative course was uncomplicated, and he was discharged from the NICU in stable condition.
Two months later, he experienced an apnea episode associated with feeding, prompting emergency evaluation and hospitalization. EAT@CHOP was consulted, and a triple scope was arranged.
Diagnostic findings
9/2/25: Triple endoscopy revealed a broad posterior tracheal wall and severe tracheomalacia at the proximal thoracic trachea, with diffuse bronchial edema and copious thin secretions.
CTA demonstrated marked dynamic narrowing of the proximal thoracic trachea at the level of the crossing innominate artery.
Treatment
The infant was started on medical therapy for tracheomalacia while a surgical plan was developed. Before surgery could be scheduled, however, he suffered another apnea episode with feeding and required readmission, prompting urgent operative intervention.
9/24/25: The baby underwent cervical posterior tracheopexy, redo right thoracotomy, rotational esophagoplasty, thoracic tracheoplasty, and posterior tracheopexy, reconstituting the normal shape of the trachea and effectively opening his airway.
Outcomes
The infant required two days of PICU monitoring and was discharged post-operative day 8. At follow-up, he tolerated goal feeds without respiratory distress, demonstrated excellent growth, and had no further apneic episodes.
A recent viral illness was managed successfully at home, an important milestone given his prior instability. His parents shared that EAT@CHOP not only “saved [their] son’s life,” but also allowed them to “cuddle [their] little man tonight, and every night for as long as he’ll let [them].”
Discussion
Tracheomalacia is defined as dynamic collapse of ≥50% of the tracheal lumen during expiration, leading to symptoms such as noisy breathing, barky cough, feeding-related respiratory distress, recurrent lower respiratory infections, and cyanotic or “blue spells.” It is strongly associated with EA/TEF, yet is frequently misdiagnosed as asthma, delaying definitive treatment.
At the Esophageal and Airway Treatment Program at CHOP, diagnosis is established through a multidisciplinary triple-scope evaluation combining ENT-performed MLB, pulmonology-performed flexible bronchoscopy, and gastroenterology-performed endoscopy. This approach ensures a comprehensive assessment of vocal cord function, laryngeal anomalies, TEF recurrence, any esophageal pathologies, and dynamic airway collapse in real time.
All findings are reviewed at our multidisciplinary airway conference, where individualized management plans are developed. Most patients improve with optimized medical management, but surgical intervention is recommended for severe symptoms, especially recurrent respiratory infections, or life-threatening events such as apnea or cyanosis.
Surgical strategies aim to eliminate dynamic tracheal collapse and are tailored to each patient’s anatomy and disease location.
Anterior approach
- Aortopexy/Innominopexy: involves elevating vascular structures off the trachea
- Anterior tracheopexy: includes lifting anterior cartilage and anchoring it to the sternum
Posterior approach
- Rotational esophagoplasty: involves mobilizing the esophagus away from the posterior trachea
- Posterior tracheopexy: includes anchoring the posterior membrane to the anterior longitudinal ligament of the spine
These procedures may be performed via open or minimally invasive techniques, depending on patient anatomy and prior surgical history.
The authors are all from Children’s Hospital of Philadelphia and collaborated in the care of these patients. Duy T. Dao, MD, MPH, is an attending pediatric surgeon with the Division of Pediatric General, Thoracic and Fetal Surgery. Joseph Piccione, DO, MS, is Co-Director of the Advanced Diagnostic and Interventional Bronchoscopy Center, and Pulmonary Co-Director for the Center for Pediatric Airway Disorders and Esophageal and Airway Treatment Program. Richard Paul Boesch, DO, Pelton A. Phinizy, MD, Aoife Corcoran, MD, and Antoinette Wannes Daou, MD, are attending physicians with the Division of Pulmonary and Sleep Medicine. Michael A. Manfredi, MD, is a gastroenterologist with the Division of Gastroenterology, Hepatology and Nutrition. Thomas E. Hamilton, MD, is an attending pediatric surgeon with the Division of Pediatric General, Thoracic and Fetal Surgery. Dr. Manfredi and Dr. Hamilton are co-directors of the Esophageal and Airway Treatment (EAT) Program.
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