A 53-year-old female patient with SSc was referred to our department for further evaluation and treatment of an esophageal stricture. The patient was diagnosed with SSc with associated GERD (Fig. 1a) and interstitial pneumonia (IP) and had been taking medication continuously for the past 15 years, including a proton pump inhibitor (PPI), mycophenolate mofetil (1.5 g), tacrolimus (3 mg), and prednisolone (5 mg). The patient had been undergoing regular checkups but developed symptoms of reflux and cough and was admitted for examination 5 years ago. Endoscopy revealed mild reflux esophagitis and an esophageal hiatus hernia (Fig. 1b). Esophageal manometry showed that the integrated relaxation pressure (IRP) was 14.3 mmHg and a complete absence of peristalsis. Esophagography showed no peristalsis; however, there was an obstruction (Fig. 2a). The 24-h multichannel intraluminal impedance-pH (24 h MII-pH) monitoring revealed significant non-acid reflux. A small bowel contrast study indicated weak peristalsis of the small intestine. The patient's medication was adjusted, and her condition remained stable.

Endoscopy showed a reflux esophagitis of Los Angeles classification Grade B 15 years ago (a). Endoscopy revealed Grade M reflux esophagitis 5 years ago (b), but it further worsened to Grade C 3 years ago (c). Endoscopy showed 20 mm esophageal ulcer (arrowhead) and benign stenosis due to scar tissue in the middle thoracic esophagus 1 year ago (d). Endoscopy revealed restenosis 30 cm from the incisors despite multiple balloon expansions and Grade D reflux esophagitis when the patient was referred to our department (e, f)

Esophagogram showed no expansion or constriction but the esophagus was in a state of aperistalsis 5 years ago (a), but it remained in a state of aperistalsis 3 years ago (b). Esophagogram revealed an esophageal stricture 30 mm in the middle thoracic esophagus (c)

However, the patient’s dysphagia worsened, and esophagography revealed esophageal aperistalsis and liquid passage disturbance, leading to readmission for examination 3 years ago (Fig. 2b). Endoscopy revealed moderate reflux esophagitis (Fig. 1c), and no stricture or dilation was detected. Esophageal manometry confirmed the aperistalsis; the lower esophageal sphincter pressure (LESP) was 14.2 mmHg, and the IRP was 6.8 mmHg, which was lower than in the previous examination. The PPI dose was increased, and the patient was discharged.

The GERD symptoms had worsened a year ago despite ongoing medication. Endoscopy revealed severe reflux esophagitis and ulceration (Fig. 1d). The IRP and LESP were 5.8 and 12.6 mmHg, respectively, on manometry. The 24-h MII-pH monitoring could not provide an accurate evaluation, because the esophagus was filled with liquid. The patient had developed a membranous stricture and edema of the middle thoracic esophagus 3 months after the previous examination. A radical incision and cutting (RIC) procedure was performed to alleviate the severe dysphagia without any adverse events.

After treatment, the patient remained stable for 6 months; however, stricture recurrence occurred, and another RIC procedure was performed. As the progression of stenosis worsened despite treatment, the patient underwent several endoscopic balloon dilations; however, the procedures were unsuccessful. Finally, the patient was referred to our department for surgery.

On admission, the patient had severe dysphagia, regurgitation, skin induration, and tightening of the face and fingers. Endoscopy revealed severe reflux esophagitis and esophageal stenosis (Fig. 1e, f), and esophagography confirmed the presence of a stricture (Fig. 2c). Contrast-enhanced computed tomography (CT) revealed dilatation and liquid pooling in the proximal esophagus and a stable fibrosing non-specific interstitial pneumonia pattern in the lungs (Additional file 1: Fig. S1a, b). There were no atypical cells on biopsy. With good control of the IP, the cardiopulmonary function was normal.

Endoscopic treatments for the severe esophageal stricture caused by GERD and SSc, were unsuccessful. Therefore, the patient underwent thoracoscopic subtotal esophagectomy and laparoscopic gastric tube reconstruction through the retrosternal route. Despite multiple endoscopic treatments, no adhesions were observed around the esophagus. The operation time was 318 min, and the blood loss was 80 mL without any adverse events. The surgical specimen contained an esophageal stricture 50 × 30 mm in size (Fig. 3a), and histopathological examination showed thinning and tearing of the muscular layer and fibrous tissue replacement of the muscle tissue (Fig. 3b).

Macroscopic finding of esophageal stenosis, 5.0 × 3.0 cm in size (a). Microscopic examination revealed thinning of the muscular layer and an increase in fibrous tissue (b, (arrowhead). (Hematoxylin and eosin stain, 10 ×)

After the surgery, pneumothorax occurred due to inadequate air drainage, but it was successfully managed by inserting a chest tube for 3 days. In addition, the patient required a 1-month hospitalization for tacrolimus adjustment. Finally, the patient was discharged in stable condition. The patient continued regular checkups and showed no symptoms or findings of GERD or esophageal stricture 6 months after surgery.