Endoscopic Management of Recurrent Food Impactions and Dysphagia in a Patient With Allgroveʼs Syndrome

Abstract

Clinical Vignette: A 23-year-old male with Allgrove's syndrome complicated by multiple esophageal strictures with esophageal diverticula, and recurrent food impactions presented with dysphagia. He was diagnosed with achalasia in infancy and was previously treated by Heller myotomy and fundoplication. Endosopcy was performed and a gastroscope could not pass an esophageal stricture, just below the upper esophageal sphincter. Instead, an ultraslim nasal scope was successfully used and a jagwire was passed under fluoroscopic guidance into the stomach. The jagwire then assisted scope navigation through the esophagus. A long stricture was identified from 30 cm to 40 cm with a food bolus located proximally and removed by Roth net. The stricture was serially dilated using a TTS CRE balloon from 4 to 6 mm (Image 1). The nasal scope was then advanced distally to 40 cm where another food bolus was fragmented using biopsy forceps and deposited into the stomach. The patient tolerated the procedure well and his diet was advanced. He was discharged with outpatient follow-up for stricture dilation. Discussion: Allgrove's syndrome is a rare autosomal recessive disorder characterized by a triad of adrenal insufficiency, achalasia, and alacrima often associated autonomic dysfunction. Esophageal abnormalities are similar to idiopathic achalasia caused by a thickening of the intramuscular layer, loss of the myenteric ganglia and decreased nitric oxide signaling. Traditional treatments include Heller myotomy and endoscopic dilation. The most frequently used dilators for achalasia are the wire-guided polyvinyl dilators (Savary-Gilliard) and the TTS CRE balloon dilators. The Savary-Gilliard dilator exerts a radial force as it gradually passes distally, though the dilating force is transmitted longitudinally secondary to shearing effects. In contrast, the balloon dilators lack longitudinal force and deliver the radial force instantly. Longitudinal force and lack of visualization pose a significant risk for perforation and are contraindicated in multiple, long, or complex strictures, or when diverticula are present. Our patient has a complex esophageal stricture due to asymmetry, diameter < 12mm, and inability to pass the endoscope. While no clear advantage has been demonstrated among dilator types, this case highlights the benefit of endoscopic visualization with the CRE balloon dilator to accurately position the balloon across the stricture.Figure 1