Examine | Case Reports: Tough to Swallow

Managing a rare case of dysphagia

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An esophagogram revealed an asymmetric, regular and tapered narrowing of a short segment of the distal esophagus. (Photo courtesy Journal of Radiology Case Reports)

A coronal CT reconstruction of the chest shows a circumferential, diffuse, slightly asymmetric and regular thickening of the distal esophageal wall (arrow). (Photo courtesy Journal of Radiology Case Reports)

In this installment of Examine: Case Reports, we look at congenital esophageal stenosis, a rare anomaly in pediatric patients. This case report was originally published in the June issue of the Journal of Radiology Case Reports. The complete version, including discussion, references, and interactivity, can be accessed at www.radiologycases.com.

Congenital esophageal stenosis (CES) is a rare anomaly in pediatric patients caused by incomplete separation of the respiratory tract from the primitive foregut. The first clinical signs are abnormalities of the swallowing mechanism, due to intrinsic narrowing of the esophagus.

Diagnosis is usually delayed and requires an accurate history and high level of suspicion, in addition to an esophagogram. Treatment usually involves surgery, depending on the severity, location, and type of stenosis.

The incident rate of CES is estimated at 1 per 25,000 to 50,000 births. The condition may be the result of an intrauterine stress or anoxia, which leads to incomplete separation of the respiratory tract from the primitive foregut. An association with other anomalies, particularly esophageal atresia, is reported in 17 percent to 33 percent of cases.

The three anatomic types of CES are: esophageal membranes (EM); fibromuscular stenosis (FMS); and tracheobronchial remnants (TBR). TBR, the most common form, involves preferentially the lower third of the esophagus. The other two forms are often seen in the middle third portion of the esophagus.

Generally, high lesions present with respiratory symptoms, while low lesions present with vomiting. Symptoms usually start around the weaning period with dysphagia to solids. An esophagogram is the main diagnostic tool in CES. It generally reveals a concentric, aperistaltic, sometimes asymmetric narrowing of the upper, mid, or distal esophagus (frequently within 3.5 cm of the gastric cardia), with variable length (about 1 cm to 2 cm), smooth contours and tapered borders.

Patients with reflux esophagitis stenosis generally have severe gastroesophageal reflux (GER), and show areas that lack distensibility or transverse and converging folds. Peristalsis is disorganized, although it can be absent. The vascular ring due to double aortic arch occurs as bilateral and posterior compressions of the esophagus, which remain constant regardless of peristalsis. The right indentation is usually slightly higher than the left, and the posterior compression is wide and courses downward as it goes from right to left.

Our case involved an 18-month-old toddler diagnosed with CES. The male infant, born at term with hypospadia, had a 12-month history of postprandial vomiting of undigested food particles, which began in the weaning period. No weight loss was reported. A clinical exam was unremarkable.

Differential diagnosis included GER, achalasia and stenosis secondary to GER, a foreign body, and congenital or neoplasic causes. An esophagogram revealed an asymmetric, regular, tapered, and aperistaltic narrowing of the distal esophagus approximately 2 cm to 3 cm long. Proximal dilatation of the esophagus and tertiary contractions were found, but GER was not present. Tertiary contractions were observed along the esophagus, except in the narrowed segment, which showed no peristalsis. The proximal portion of the lower esophagus was dilated.

No signs of esophagitis were found and a biopsy was not performed. Manometry pointed to a distal tumor versus a primary phase of achalasia. Given the worsening of the clinical condition, clinicians placed a feeding tube.

An oral and intravenous contrast enhanced chest CT characterized the lesion and provided a pre-surgery assessment. The scan revealed a diffuse, circumferential, asymmetric, nonenhanced wall thickening of the distal esophagus that was discretely lateralized to the left.

The lesion showed a maximum thickness of 10 mm, and 2.5 cm of longitudinal length, being localized 2.5 cm distal to the carina, while sparing the last 2 cm of the esophagus. Excision of a 5 cm segment by thoracotomy with an end-to-end esophageal anastomosis was performed under a probable diagnosis of CES versus neoplasic stenosis. Histology confirmed CES due to TBR. Post-operative recovery was uneventful, with steady weight gain and normal development at follow-up.

Eva Serrao, Alexandra Santos, Ana Gaivao, Ana Tavares, and Sergio Ferreira are with the department of imaging at the Hospital Professor Doutor Fernando Fonseca in Amadora, Portugal. This case report was originally published in the June issue of the Journal of Radiology Case Reports. The complete version, including discussion, references, and interactivity can be accessed at www.radiologycases.com. Direct comments and questions to editorial@rt-image.com.