weekly_question 3/11/2024

[Admin]

An 8-month-old girl presents with choking with every feeding. She had a previous proximal esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) repair at birth with an uneventful recovery although she is still taking antireflux medication. An esophagram shows minimal narrowing at the anastomosis but a smooth narrowing in the distal esophagus with contrast passing into the stomach. What is the most appropriate step in management?

a increase proton pump inhibitor dose

b esophagoscopy

c laryngoscopy and bronchoscopy

d balloon dilation

e surgical resection Discussion

[Admin]

Correct answer
D balloon dilation

Congenital esophageal stenosis (CES) is a rare disorder occurring in roughly one in 25,000 to 50,000 births. CES may be associated in roughly 15% of patients with esophageal atresia and tracheoesophageal fistula (EA/TEF). CES may be due to fibromuscular hypertrophy or tracheobronchial remnants.

Patients with CES will present with dysphagia, choking and vomiting. Patients with CES and EA/TEF will often present at a younger age. Esophagram is typically obtained to evaluate the anastomosis and should also rule out any additional strictures as in this case.

Dilation with balloon dilators or bougies is the first line in treatment. Although classic teaching is that dilation is often not successful and surgical intervention is required, recent studies have shown successful treatment with serial dilations in 84 to 95% of patients with CES; however, most series are small and results vary. Although balloon dilation is most commonly used, flexible bougie dilators may be more effective and associated with a lower perforation risk in some reports. CES with tracheobronchial remnants are less likely to respond to serial dilations and are at higher risk of perforation.

If a patient does not respond to dilations then surgical excision should be considered as the presence of a tracheobronchial remnant is likely. Imaging study (transesophageal ultrasound, compuertized tomography or magnetic resonance imaging) can confirm the presence of cartilaginous remnants and the need for surgical excision. Newer technology such as the Endoflip™, which uses high-resolution impedance planimetry to measure luminal geometry and pressure during dilations may aid in diagnosing tracheobronchial remnants at the time of dilation leading to early surgical intervention.

Surgical excision of CES may be associated with postoperative gastroesophageal reflux. Thus, some advocate for an antireflux operation at the time of excision. In patients with EA/TEF and CES, antireflux operations may worsen the underlying esophageal dysmotility associated with these diseases. Alternatively, in patients with fibromuscular hypertrophy, a longitudinal myotomy with transverse closure should be considered.

Increasing the PPI dose may be warranted if symptoms were due to gastroesophageal reflux disease. Esophagoscopy may be helpful to confirm the diagnosis and rule out other causes. Laryngoscopy is helpful in ruling out laryngeal clefts and bronchoscopy for tracheomalacia/bronchomalacia but this patient’s symptoms are not consistent with these diagnoses.

[Bilal]

D

[luai]

I say B, in order to know is the Problem narrowing or reflux
If it the narrowing then start with D for management

[Ayman]

C

[Baashe]

C.. r/o missed or recurrent tef

[Meddz81]

C, to rule out a missed proximal fistula or recurrence

[ortho-onc]

Why would we assess the airway (C. laryngoscopy & bronchoscopy) if we know the problem is more posteriorly?
Personally, I would D, then reassess with A or E down the line.

[Reem Mohammed]

C

[Abd El wahed]

D

[Khulood]

C
To rollout missed fistula

[Ismailmohamed]

C

[Admin]

Correct answer
D balloon dilation

Congenital esophageal stenosis (CES) is a rare disorder occurring in roughly one in 25,000 to 50,000 births. CES may be associated in roughly 15% of patients with esophageal atresia and tracheoesophageal fistula (EA/TEF). CES may be due to fibromuscular hypertrophy or tracheobronchial remnants.

Patients with CES will present with dysphagia, choking and vomiting. Patients with CES and EA/TEF will often present at a younger age. Esophagram is typically obtained to evaluate the anastomosis and should also rule out any additional strictures as in this case.

Dilation with balloon dilators or bougies is the first line in treatment. Although classic teaching is that dilation is often not successful and surgical intervention is required, recent studies have shown successful treatment with serial dilations in 84 to 95% of patients with CES; however, most series are small and results vary. Although balloon dilation is most commonly used, flexible bougie dilators may be more effective and associated with a lower perforation risk in some reports. CES with tracheobronchial remnants are less likely to respond to serial dilations and are at higher risk of perforation.

If a patient does not respond to dilations then surgical excision should be considered as the presence of a tracheobronchial remnant is likely. Imaging study (transesophageal ultrasound, compuertized tomography or magnetic resonance imaging) can confirm the presence of cartilaginous remnants and the need for surgical excision. Newer technology such as the Endoflip™, which uses high-resolution impedance planimetry to measure luminal geometry and pressure during dilations may aid in diagnosing tracheobronchial remnants at the time of dilation leading to early surgical intervention.

Surgical excision of CES may be associated with postoperative gastroesophageal reflux. Thus, some advocate for an antireflux operation at the time of excision. In patients with EA/TEF and CES, antireflux operations may worsen the underlying esophageal dysmotility associated with these diseases. Alternatively, in patients with fibromuscular hypertrophy, a longitudinal myotomy with transverse closure should be considered.

Increasing the PPI dose may be warranted if symptoms were due to gastroesophageal reflux disease. Esophagoscopy may be helpful to confirm the diagnosis and rule out other causes. Laryngoscopy is helpful in ruling out laryngeal clefts and bronchoscopy for tracheomalacia/bronchomalacia but this patient’s symptoms are not consistent with these diagnoses.