A 67-year-old woman presented with weight loss, loss of appetite, and early satiety. Her symptoms had been exacerbated by stress. She had no other signiﬁcant medical history and was not taking any regular medication. An upper gastrointestinal (GI) series was performed (Figure 1).
Mamata Chithriki, MD, Shahzad Sadiq, MD,
Moneer Jaibaji, MD,
St. Joseph Mercy Oakland Hospital, Pontiac, MI.
A 67-year-old woman presented with weight loss, loss of
appetite, and early satiety. Her symptoms had been exacerbated by
stress. She had no other significant medical history and was not
taking any regular medication. An upper gastrointestinal (GI)
series was performed (Figure 1).
The upper GI series showed an antral ulcer with a fistulous
connection to the duodenum, also known as a "double pylorus."
Esophagogastroduodenoscopy was performed and revealed two openings
from the stomach: one the normal pylorus and one opening that
appeared edematous at the margins with a clean-based ulcer,
representing a gastroduodenal fistula (Figure 2). Biopsies of the
ulcerative region were negative for
. The patient was treated with a proton pump inhibitor, and her
symptoms resolved. Afollow-up endoscopy 8 weeks later revealed
resolution of the inflammatory changes with a persistent
gastroduodenal fistula (Figure 3).
Double pylorus or gastroduodenal fistula is an unusual, but
recognized, condition in which two pyloric openings connect the
gastric antrum to the duodenal bulb. The two openings are separated
by a septum or bridge of tissue covered by gastric epithelium.
Congenital and acquired forms of this condition can occur. The
congenital form is reported to occur equally in both sexes and its
etiology is unknown. Criteria for entertaining a congenital origin
include: A) no clinical history of peptic ulcer disease and no
evidence of ulcerative changes on imaging studies either
radiographically or endoscopically; B) recognition at a very young
age; C) histologic features, including the presence of mucosa,
submucosa, and muscularis propria in the second channel; D)
spontaneous or drug-induced motility in both channels; and E) other
associated congenital abnormalities, such as heterotopic pancreatic
tissue or gastric duplication.
Congenital origin of the double pylorus is rare in comparison with
the acquired form; one series reported that it occurred in only 5
of 60 cases.
Complete tubular duplication of the pylorus was reported in one
series of congenital lesions of the upper GI tract as occurring in
only 1 anomaly in a collection of 281 lesions.
As with GI duplications at any level, ectopic gastric mucosa can
exist in the second pyloric channel. These areas can be
symptomatic, as they have a propensity for similar complications,
such as peptic ulceration that can arise in normal gastric mucosa.
The acquired form of double pylorus, which appears more commonly,
has a male predominance of 2:1
and is postulated to be a sequela of peptic ulcer disease creating
a GI fistula.
The sequence of events has repeatedly been shown as follows: 1)
formation of peptic ulcer in gastric antrum or duodenal bulb; 2)
penetration of this ulcer leading to adhesion formation between the
adjacent stomach and duodenal walls; and 3) continued penetration
along the muscular layer resulting in a fistulous tract.
Generally, patients present with symptoms of abdominal pain and
GI bleeding or anemia. Most patients reported having experienced
symptoms for >2 years prior to the identification of the
Patients with the acquired form have an increased incidence of
associated systemic disorders. Studies have failed to elucidate a
more active progression of peptic ulcer disease resulting from the
existence of the double pylorus. There have been, to our knowledge,
no known cases of free bowel perforation or gastric outlet
obstruction, nor have there been reports of localized infection or
bile reflux gastritis. There have been no known associations with
the development of this condition with gastric malignancy, nor is
there any known association of increased gastric acid secretion or
Radiographic findings on double-contrast studies are
characteristic, showing two linear barium-lined channels in the
pyloric region separated by a nonopacified segment, the septum.
Reports have documented this appearance being misinterpreted as
polyps, tumors, or large mucosal folds, however.
Prone barium-filled views frequently reveal the double pylorus
optimally. The application of pressure on the abdomen may assist in
better opacification of both channels with barium.
Unless the radiologist is aware of this entity, it may be
overlooked on contrast studies. The findings on endoscopy clinch
the diagnosis, with the orifice of the fistula being noticeably
visible adjacent to the pylorus. However, on occasion, insertion of
the biopsy forceps or the endoscope itself through the fistula may
be essential in documenting the fistulous connection. Care must be
taken to adequately distend the antrum with air such that large
gastric folds do not obscure a small fistula.
Ulcer formation most commonly occurs on the lesser curve of the
gastric antrum adjacent to the fistulous tract and connects to the
superior aspect of the duodenal bulb. Only about half of patients
with double pylorus have been shown to harbor
The patient reported here also failed to demonstrate the presence
Treatment recommendations have included avoidance of ulcerogenic
medications and the institution of intensive and complete antiulcer
treatment as soon as possible. Triple therapy should probably be
recommended in patients who are positive for
, though no significant benefit has been shown from its eradication
in symptom resolution, decreasing ulcer recurrence, or in fistula
closure. Fistula closure has not been shown to occur in the
majority of patients with this condition despite resolution of
symptoms, as was the scenario with the above-described case.
Fistulas often remain patent or converge to form a large single
opening following the destruction of the septum from penetrating
ulcer formation. Surgery is an alternative for the treatment of
double pylorus, particularly in patients with refractory symptoms,
recurrent ulcers or other complications.
The findings of one series that reviewed a total of 53 cases
suggested that only 20% of cases required surgery, while 80% had
been successfully managed medically.
The double pylorus appears to be a complication of peptic ulcer
disease, which in many cases can be managed by medical therapy
without complications. Surgical intervention may be necessary for
the usual critical complications of peptic ulcer disease or