Double pylorus in cirrhosis

Abstract

Double pylorus is an uncommon clinical condition that can be acquired or congenital. Most acquired cases arise as a complication of peptic ulcer disease and less commonly from other conditions such as gastric malignancy. We present a case of double pylorus in a cirrhotic patient diagnosed during surveillance endoscopy for esophageal varices.

Double pylorus (DP) is an extremely rare endoscopic finding involving a double communication between the gastric antrum and duodenum. It has been observed in <0.4% of upper gastrointestinal endoscopies and is more frequently observed in men. ¹ DP is either congenital or arises as a consequence of peptic ulcer disease. Most reported cases describe DP as a complication of a penetrating ulcer, commonly referred to as acquired DP. ² However, there are documented cases describing DP as a congenital abnormality, either isolated or in combination with other congenital abnormalities, such as pancreatic divisum. ³

CASE DESCRIPTION

A 63-year-old man presented to the gastroenterology clinic for follow-up of decompensated cirrhosis. He was known to have Child class A liver cirrhosis due to previous alcohol abuse and chronic hepatitis C (treated with ledipasvir/sofosbuvir with documented sustained virologic response), esophageal varices with multiple bandings, a history of alcohol abuse (quit alcohol 6 years prior), and poorly controlled type 2 diabetes mellitus. There was no prior history of Helicobacter pylori infection or gastric or duodenal ulcers on previous endoscopies. The patient had undergone eight upper gastrointestinal endoscopies before the diagnosis of DP was made.

At his clinic visit, the patient was asymptomatic. He denied episodes of confusion or forgetfulness, jaundice, hematemesis, hematochezia, melena, abdominal pain, or abdominal distension. Review of systems was negative for weight loss, alcohol use, and nonsteroidal anti-inflammatory drug use. His medications included nadolol 40 mg orally in the morning and 20 mg orally in the evening and pantoprazole 40 mg orally daily.

On examination, the patient was hemodynamically stable. His blood pressure was 129/69 mm Hg; pulse, 57 beats/minute; respiratory rate, 16 breaths/minute; and temperature, 97.6°F. Bowel sounds were normal, and the abdomen was soft, nontender, and nondistended. Neither the liver nor the spleen were palpable. Cardiovascular, respiratory, and neurologic examinations were unremarkable. Hemoglobin was 11.3 g/dL (baseline 11 g/dL); hematocrit, 33.7%; mean corpuscular volume, 83.4 fL; platelet level, 87,000/mm³; international normalized ratio, 1.1; blood urea nitrogen, 11 mg/dL; creatinine, 0.9 mg/dL; albumin, 3.2 g/dL; alkaline phosphatase, 81 IU/L; aspartate aminotransferase, 27 IU/L; alanine aminotransferase, 17 IU/L; and total bilirubin, 1.0 mg/dL.

During the patient’s last upper endoscopy in March 2018, he had three columns of grade II varices in the lower third of the esophagus without any red wale signs or platelet plugs, and six bands were used to achieve complete eradication. The patient canceled follow-up endoscopy and was lost to follow-up for about 20 months. Upper gastrointestinal endoscopy in November 2019 revealed a DP (Figure 1), a 6 mm benign duodenal nodule, mild portal hypertensive gastropathy, scarring of the lower third of the esophagus from prior bandings, and two columns of grade I esophageal varices. The endoscope could be traversed easily through both pyloric orifices, and the presence of DP was not noted to be documented on prior upper endoscopies. On clinical follow-up 3 weeks later, he remained asymptomatic.

Figure 1.

Endoscopic view from antrum demonstrating double pylorus. The openings are separated by a bridging tissue.

DISCUSSION

DP was first reported in 1969 by Smith and Tuttle. ⁴ It is reported to be found in 0.001% to 0.4% of upper gastrointestinal endoscopies and is more common in men, with a male to female ratio of 2:1. ¹ Congenital cases of DP can be found in association with pancreatic divisum, ³ heterotrophic pancreatic tissue, ⁵ and gastric duplication. ⁶

The development of congenital DP occurs during the early embryonic stages of foregut development; its diagnosis requires the presence of normal mucosa and muscularis mucosa, lack of peptic ulcer disease, and lack of radiologic and endoscopic evidence of ulcer. ⁷ It is mostly an incidental finding, is mostly asymptomatic, and does not require any therapy or extensive investigation.

Acquired cases mostly arise as a complication of chronic peptic ulcer disease, in which a fistulous tract forms from the gastric antrum to the duodenal bulb. These cases present with signs and symptoms similar to peptic ulcer disease, including abdominal pain, dyspepsia, and gastrointestinal bleeding, or can remain asymptomatic. ⁸ Rarely, DP has been documented as a complication of gastric cancer. ⁹ Other comorbidities including diabetes mellitus, chronic obstructive pulmonary disease, rheumatoid arthritis, cirrhosis, Behçet’s disease, systemic lupus erythematosus, chronic kidney disease, and radiotherapy have been associated with the development of acquired DP, possibly due to the poor healing of ulcers. ^10–12

Management includes treatment with proton pump inhibitors and H2 receptor antagonists. Most patients respond well to conservative treatment. Helicobacter pylori infection should be tested for and eradicated, if present. ⁸ After treatment, the fistula remains open in 67% of patients, fuses with the normal pyloric opening in 28% of patients, and disappears in 5% of patients. ⁶ For patients with signs and symptoms of gastric outlet obstruction, endoscopic division of bridging tissue using a biliary sphincterotome should be considered because reestablishment of the normal pyloric aperture will alleviate the symptoms. Indications for surgery include complications such as free perforation, refractory bleeding, and failure of healing despite optimal medical or endoscopic therapy. ^{13 , 14}

In conclusion, our patient likely had acquired DP. Although he denied taking nonsteroidal anti-inflammatory drugs, he might have taken them as over-the-counter preparations since his previous endoscopy, eventually leading to peptic ulcer disease and acquired DP. The fact that DP was not seen on eight previous endoscopies further confirms the diagnosis of acquired DP. It is also possible that DP was missed during those studies. ¹⁵ The patient is taking daily pantoprazole.