Consequence of an Unforeseen Diagnosis of Scleroderma Esophagus in a Patient With Prior Fundoplication

Abstract

In systemic sclerosis (SSc), in addition to the skin, the GI system, and in particular the esophagus, are commonly affected. The term “scleroderma esophagus” is used to describe the nature of esophageal dysfunction in patients with SSc and is characterized by esophageal aperistalsis and hypotensive esophagogastric junction pressure. First line treatment for scleroderma esophagus and GERD symptoms is pharmacotherapy with PPIs and behavioral modifications. Anti-reflux surgeries, such as fundoplication, are considered a relative contraindication as they have been associated with high rates of post-operative esophagitis and dysphagia. This case reflects the experience of a patient who received a fundoplication, initially achieved the anticipated GERD symptom improvement, and later in life developed scleroderma with esophageal manifestations. An 83 year-old woman presented with recurrent episodes of aspiration pneumonia and dysphagia for 4 yrs. An EGD revealed food in the esophagus suggestive of esophageal stasis. She had a Nissen fundoplication 20yrs prior and was without symptoms of dysphagia for 16yrs. Also, the patient was diagnosed with Raynaud's disease 10yrs after her fundoplication. On exam she had sclerodactyly and a facial telangiectasia. Barium esophagram showed a dilated and aperistaltic esophagus with retained debris. Ordered for suspicion of scleroderma, an anti-centromere antibody (ACA) was strongly positive at >1:640 (nl < 1:40), suggestive of limited type scleroderma which often presents with components of CREST syndrome. An upper endoscopy was then performed that again demonstrated dilation of the entire esophagus with food present. An intact Nissen fundoplication was clearly visualized and the wrap indentation which was 2cm distal to the esophagogastric junction was dilated to 20mm. The patient continued to have aspiration events resulting in hospitalizations for pneumonia. For this reason, and because of numerous additional co-morbidities further dilation was deemed too high risk and the patient underwent placement of a 20F PEG for feedings. It could not be predicted at the time of this patient's fundoplication that she would go on to develop scleroderma. This case demonstrates how the combination of the prior fundoplication likely contributed to development of severe dysphagia and recurrent aspiration pneumonias in the setting of her new onset scleroderma 16yrs post-operatively, ultimately necessitating placement of a PEG tube for nutrition.Figure: Intact Nissen fundoplication with dilation of entire esophagus and food in the esophagus was visualized on EGD.Figure: Esophagram demonstrating an esophagus filled with ingested debris. The esophagus was diffusely dilated with absence of peristalsis in the distal two-thirds. The gastroesophageal junction measured a maximum of 10mm in diameter.