Total gastrectomy in systemic scleroderma when anti-reflux surgery is not viable: A case report.

INTRODUCTION

Systemic scleroderma is an autoimmune disease that can affect the respiratory system and the gastrointestinal tract. When diffuse lung disease and pulmonary hypertension develop, a lung transplant is usually considered as treatment. This option, however, is not feasible in the presence of concomitant gastroesophageal reflux disease. In this case, medical therapy is initially warranted. If this fails, surgical approach may be considered in order for the patient to be a lung transplant candidate.

CASE PRESENTATION: A 56-year-old female, with previous history of intestinal pneumonitis, mild pulmonary hypertension and gastroesophageal reflux secondary to systemic scleroderma, is considered for lung transplant. Initially, due to persistent gastroesophageal reflux, a transplant was not a viable. This was corrected with an open gastrectomy with roux-en-Y anastomosis. Follow-up one week later revealed normal anatomy, adequate esophageal-jejunal anastomosis, and adequate contrast medium transit via esophagogram. Additionally, there was no evidence of contrast medium reflux indicating a resolved gastroesophageal reflux disease. This led to the patient becoming a candidate for lung transplant.

DISCUSSION: We suggest an open gastrectomy with roux-en-Y anastomosis as an alternative to the Nissen fundoplication for patients with connective tissue disease that develop terminal pulmonary consequences and require a lung transplant.