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Multiple Congenital Esophageal Stenoses in a 15-Month-old Child

Butler Tjaden, Naomi E.; Barton, Keisha R.; Walsh, Seema M.†,‡; Mahmood, Nadia F.‡,§; Vogel, Adam M.‡,||; Fishman, Douglas S.†,‡

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Journal of Pediatric Gastroenterology and Nutrition: September 2020 - Volume 71 - Issue 3 - p e97
doi: 10.1097/MPG.0000000000002766
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A 15-month-old previously healthy boy presented with 2 months of dysphagia and weight loss. At 13 months he began refusing solids with frequent regurgitation. Upper gastrointestinal series demonstrated 3 irregular but concentric esophageal strictures (Fig. 1). Three discrete segments with normal overlying mucosa and 1 diverticular component were confirmed by esophagogastroduodenoscopy (Fig. 2). Mediastinal abnormalities (vascular rings or pulmonary slings on computed tomographic angiography) were absent, and biopsies were negative. Without history of caustic ingestion, we diagnosed multiple congenital esophageal stenosis (CES). Computed tomography and endoscopic ultrasound failed to demonstrate evidence of tracheobronchial remnants (TBRs). The distal 2 strictures have significant recoil during dilations, and he continues undergoing serial dilations to facilitate oral tolerance.

FIGURE 1
FIGURE 1:
Upper GI reveals multiple concentric strictures throughout the esophagus with areas of dilatation, indicative of 3 distinct congenital strictures (white arrows). A, Frontal view. B, Lateral view.
FIGURE 2
FIGURE 2:
Endoscopy reveals stenosis (A and B) and diverticula (C.).

CES is rare (1:25,000–50,000 live births), with 3 histologically defined subtypes: fibromuscular hypertrophy, membranous diaphragm, and TBR (1). TBRs are often approached surgically, whereas serial balloon dilations can treat fibromuscular hypertrophy and membranous diaphragm. Endoscopic incisional therapy (2) can be used in refractory CES, resulting in fewer surgical interventions (3). Most children with isolated CES without concomitant esophageal atresia present with dysphagia, food impaction, vomiting, or impaired growth (4,5). Multiple CES lesions are exceedingly rare, with stenoses reported in the distal two third of the esophagus (6,7). This case demonstrates 3 stenoses, including a proximal lesion.

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