Mediastinoscopy-Assisted Esophagectomy as an Effective Treatment for IgG4-Related Esophageal Stenosis: A Case Report

Abstract

Introduction: Immunoglobulin G4-related disease (IgG4-RD) rarely involves the esophagus, typically causing stenosis that presents significant diagnostic and therapeutic challenges. Due to its rarity and its mimicry of other conditions, obtaining a definitive preoperative diagnosis can be difficult. This report details a case of IgG4-RD-induced esophageal stenosis with initial diagnostic ambiguity, which was successfully managed with mediastinoscopy-assisted esophagectomy (MAE), highlighting this minimally invasive approach in a patient with comorbidities.

Case presentation: A 70-year-old male with comorbidities, including obstructive pulmonary disorder, presented with progressive dysphagia and epigastric discomfort. Endoscopy revealed a persistent mid-esophageal ulcer and a non-passable circumferential stricture; multiple biopsies were nondiagnostic for malignancy or infection. Given the refractory nature of the stenosis, MAE with gastric conduit reconstruction was performed. The postoperative course was uneventful, and the patient achieved symptom resolution without medication. Histopathological examination of the resected esophagus confirmed IgG4-RD, showing obliterative phlebitis and a dense infiltrate of IgG4-positive plasma cells (80/high-power field; IgG4/IgG ratio 80/85).

Conclusions: This case underscores that IgG4-RD should be considered in the differential diagnosis of refractory esophageal stenosis, even with initially inconclusive biopsies. While serum IgG4 measurement has low sensitivity, it is still recommended. For benign esophageal stenosis of unclear etiology, particularly in patients with significant comorbidities, MAE can be a useful and potentially curative surgical option, offering symptom resolution and the possibility of a drug-free outcome.

Keywords: IgG-4 related disease; esophageal stenosis; mediastinoscopy-assisted esophagectomy.

Fig. 1. Representative endoscopic findings. (A) Initial presentation: Circumferential ulceration was observed. (B) Remission phase: No mucosal damage or stenosis was observed. (C) Relapse phase: Erosion and mild stenosis were noted.

Fig. 2. Current endoscopic findings. Stenosis and a deep ulcer were observed at the same location as previous findings. (A) Distant view. (B) Close-up view. (C) X-ray fluoroscopy: A severe 3-cm-long stricture was present at 35 cm from the incisors.

Fig. 3. CT findings. Circumferential stenosis was observed in the same segment as the endoscopic findings. (A) Sagittal view. (B) Dilatation of the proximal esophagus was noted. (C) Circumferential stenosis.

Fig. 4. Surgical findings. Mediastinoscopy-assisted transcervical and transhiatal esophagectomy was performed. (A) Transcervical view: The cervical and upper esophagus were mobilized and encircled with a tape around the carina. (B) Transhiatal view: The lower and middle esophagus were mobilized using the previously placed tape as a guide.

Fig. 5. Histopathological features of the resected esophagus. (A) Macroscopic findings: Circumferential stenosis with ulceration is observed in the lower esophagus. (B) Hematoxylin and eosin staining shows dense submucosal fibrosis accompanied by a plasma cell–rich inflammatory infiltrate (objective magnification, ×20). (C) Elastic van Gieson staining highlights obliterative phlebitis, with concentric fibrotic occlusion of the vascular lumen (×20). (D) Immunohistochemistry for IgG4 reveals numerous IgG4-positive plasma cells, exceeding 50 cells per high-power field in hotspot areas (×20).

IgG4, immunoglobulin G4