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

Highlights • Systemic scleroderma in severe cases, such as gastroesophageal reflux disease, a lung transplant cannot be performed.• Although gastroesophageal reflux disease can be medically treated, a poor response will warrant an anti-reflux surgery.• We propose an open gastrectomy with roux-en-Y anastomosis as an alternative to the Nissen fundoplication.• The decision to provide a surgical intervention must be individualized.


Introduction
Scleroderma is a systemic autoimmune disease that affects the connective tissue and has life-threatening cardiovascular, gastrointestinal, and respiratory complications. Based on the 2006 International Society of Heart and Lung Transplant (ISHLT) guidelines, a lung transplant is warranted when the consequences produce diffuse interstitial lung disease and pulmonary hypertension. One of the contraindications of this is gastroesophageal reflux disease (GERD). This condition can be medically treated, but if this fails a surgical approach is often required to ensure eligibility for a lung transplant. The downside to the surgical approach is recurrence of disease, up to 21% in patients with connective tissue diseases [1]. We present the following case report as a successful alternative surgical therapeutic approach in these patients to ensure eligibility for a lung transplant. The following approach was completed in the Fundacion Cardioinfantil -Instituto de Cardiologia (IC), in Bogota, Colombia. This case report is written according to surgical case report (SCARE) criteria [2].

Case presentation
Patient is a 56-year-old female with body mass index of 22.5 kg/m 2 and previous clinical history of diffuse scleroderma, intestinal pneumonitis, mild pulmonary hypertension, secondary gastroesophageal reflux, and severe esophageal motility disease. Receiving immunosuppression, antihypertensive, proton pump inhibitor and prokinetic management with weak response, patient is admitted for surgical management after a 1-year follow-up with cardiology, pneumology, and gastroenterology.
At admission, patient was symptomatic with a previous diagnosis of esophageal aperistalsis ( Fig. 1) and dysphagia with poor response to medical therapy. Physical examination revealed no significant findings. The lung transplant surgical team determined the patient was not a candidate given the severity of the GERD, due to a high risk of primary graft dysfunction. This led to a medical committee deciding an open total gastrectomy with roux-en-Y anastomosis  prior to lung transplant for this particular case. This case report reveals a surgical alternative in patients with GERD secondary to scleroderma despite a high risk of disease recurrence.
Follow-up one week later with esophagogram revealed normal esophageal morphology, no stenosis or motility difficulties, adequate esophago-jejunal anastomosis diameter, no extravasation of the contrast medium and an adequate transit of the medium to the small intestine. There is no evidence of reflux of the medium (Fig. 2). In out-patient consult, an upper gastrointestinal endoscopy is performed within a 3-month period which revealed mild esophago-jejunal anastomosis stricture resolving after three balloon dilations of 11 mm, 15 mm, and 18 mm, respectively (Figs. [3][4][5]. Currently, the patient continues follow-up consults, with an adequate postoperative state, asymptomatic according to the Gastroesophageal Reflux Disease Health Related Quality of Life (GERD-HRQL) instrument and currently awaits lung transplant.

Discussion
Scleroderma is a connective tissue autoimmune disease which primarily affects the skin and other organs. The most commonly affected organs are the kidney, digestive tract, and the lungs. The major cause of mortality in scleroderma is a development of interstitial pulmonary disease [3]. In the digestive tract, the esophagus is affected in 50-80% of patients, with its primary effect problems in peristalsis and inferior esophageal sphincter damage [4]. In this case report, the patient had a terminal lung disease which required lung transplant. However, this patient was not a candidate due to concomitant GERD, given the high risk [5] between GERD and obliterating bronchiolitis syndrome leading to a primary graft dysfunction.
GERD can be treated medically and when there is poor response, an anti-reflux surgery is a therapeutic alternative. There are several case report series which document a recurrence rate of up to 21% [6] and dysphagia between 31-71% after the Nissen fundoplication [7][8][9][10]. In the scleroderma context, a surgical approach is controversial given the ineffective esophageal motility ruling out a total Nissen fundoplication, some opting out for a partial Nissen fundoplication with recurrence rates of up to 19% [1]. For this reason, the surgical alternatives in GERD are limited. Other options include a total duodenal derivation [11] or an esophagectomy [12], both high morbidity approaches. Hence the Nissen fundoplication remains the standard, albeit controversial, surgical option in scleroderma patients.
In this case report, the advanced gastrointestinal disease secondary to scleroderma limited the standard surgical approach given the high recurrence rate. In this situation, an open gastrectomy    with roux-en-Y anastomosis was decided as the best approach to solve the GERD and abide by the ISHLT standards for a lung transplant given the high risk of recurrence as well as potential complications given the comorbidities associated. The University of Pittsburgh first published the comparison between the laparoscopic Nissen fundoplication, the open gastrectomy with roux-en-Y anastomosis, and the esophagectomy in GERD patients secondary to scleroderma. They concluded that the gastrectomy approach had a lower rate of recurrence and lower reported dysphagia in the postoperative state compared to the other two alternatives [13,14].

Conclusions
In summary, we propose an open gastrectomy with roux-en-Y anastomosis as a surgical alternative to the Nissen fundoplication in patients with advanced connective tissue disease. In Fig. 6, we also propose a new protocol for evaluating gastrointestinal disease in patients with a severe lung disease requiring lung transplant. The decision to provide a surgical intervention must be individualized, considering the expertise of the surgeons and transplant teams. An interdisciplinary treatment is very important, including a lung transplant and gastrointestinal team to ensure a successful intervention for these patients.

Declaration of Competing Interest
The authors declare they have no conflicts of interest.

Funding
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Ethical approval
The Ethical and Research Committee of the Fundación Cardioinfantil -IC and the General Surgery Research Group at the Fundación Cardioinfantil -IC.

Consent
Written consent was obtained from the patient for publication of this report. Any details identifying the individuals to the clinical history and images associated were eliminated as to remain anonymous.