AJR 2003; 181:807-808
© American Roentgen Ray Society
Gastric Wall Weakening Resulting in Separate Perforations in a Patient with Duchenne's Muscular Dystrophy
David Dinan1,
Marc S. Levine1,
Andrew R. Gordon1,
Stephen E. Rubesin1 and
John L. Rombeau2
1 Department of Radiology, Hospital of the University of Pennsylvania, 3400
Spruce St., Philadelphia, PA 19104.
2 Department of Surgery, Hospital of the University of Pennsylvania,
Philadelphia, PA 19104.
Received October 30, 2002;
accepted after revision January 16, 2003.
Address correspondence to M. S. Levine.
Introduction
The muscular dystrophies are a group of hereditary diseases characterized
by skeletal muscle degeneration and progressive muscular weakness. Smooth
muscle in the gastrointestinal tract may also be affected, particularly in
patients with Duchenne's muscular dystrophy, a fatal X-linked recessive
disease. Autopsy studies have shown that gastrointestinal involvement by the
muscular dystrophies is characterized by edema, fatty infiltration,
fragmentation, fibrosis, and waxy degeneration of smooth muscle, resulting in
atrophy and thinning of the bowel wall
[1,
2]. These changes can lead to
severe functional disturbances of the gastrointestinal tract with
gastroparesis [3,
4], acute gastric dilatation
[5-7],
and intestinal pseudoobstruction
[8].
We recently encountered a patient with Duchenne's muscular dystrophy who
developed separate gastric perforations, the first after colectomy and
nasogastric intubation and the second after surgical repair of the initial
perforation. To our knowledge, gastric perforation has not been previously
reported in the radiology literature as a complication of Duchenne's muscular
dystrophy. Nevertheless, it is important to be aware of the increased risk of
gastric perforation associated with nasogastric intubation or surgical
intervention in these patients. We therefore present the findings in this case
and discuss their significance.
Case Report
A 20-year-old man with Duchenne's muscular dystrophy underwent surgery for
recurrent intestinal pseudoobstruction and sigmoid volvulus managed without
complications by colonic decompression via a rectal tube. Because of the
severity and frequency of these episodes, the patient elected to undergo a
total colectomy. At surgery, he was found to have a diffuse megacolon that was
nearly three times the length and five times the width of a normal colon. He
underwent a total colectomy with creation of a Hartmann's pouch and a
temporary diverting ileostomy. An 18-French nasogastric tube (Bard, Covington,
GA) was placed in the stomach at surgery for gastric decompression during the
early postoperative period.
The patient developed abdominal pain and nausea 4 days after surgery, at
which time an abdominal radiograph revealed marked gastric dilatation despite
placement of the nasogastric tube. Fourteen days after surgery, the patient
developed clinical signs of peritonitis. An upper gastrointestinal study with
water-soluble contrast medium (Gastroview [diatrizoate meglumine and
diatrizoate sodium solution], Lafayette Pharmaceuticals, Lafayette, IN) showed
the tip of the tube abutting the greater curvature of the gastric body with
focal extravasation of contrast material from this region into a large
collection in the left upper quadrant that communicated superiorly with the
left subphrenic space (Fig.
1A). An emergent laparotomy revealed focal perforation on the
greater curvature of the midgastric body. The stomach was also found to have a
paper-thin wall without evidence of peptic ulcer disease or other
abnormalities. The perforation was closed, the peritoneal cavity was
débrided, and multiple drains were placed in the upper abdomen. No
nasogastric tube was placed at the time of the second operation.
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Fig. 1A. —20-year-old man with Duchenne's muscular dystrophy and
separate gastric perforations. Left posterior oblique spot radiograph from
upper gastrointestinal study with water-soluble contrast medium shows focal
extravasation of contrast material (open arrow) from greater
curvature of gastric body into large collection (curved arrows) in
left upper quadrant that communicates superiorly with subphrenic space. Thin
track (straight white arrow) also extends inferiorly from this
collection. Note nasogastric tube (black arrows) in stomach with tip
near site of perforation.
|
|
Sixteen days after the gastric repair, the patient developed recurrent
signs of peritonitis. A repeated upper gastrointestinal study with
water-soluble contrast medium revealed a new leak from the gastric fundus near
the gastroesophageal junction with focal extravasation of contrast material
into a large collection in the left upper quadrant that communicated with the
peritoneal cavity in the right side of the abdomen (Figs.
1B and
1C). A second emergency
laparotomy revealed extensive inflammation and adhesions involving the
peritoneal cavity. An additional drain therefore was placed in the collection
in the left upper quadrant without an attempted closure of the leak. The
patient slowly improved over the next week, and a third upper gastrointestinal
study 8 days after the last operation revealed a dilated stomach without
evidence of a residual leak. He was discharged in stable condition to a
rehabilitation facility for further care.
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Fig. 1B. —20-year-old man with Duchenne's muscular dystrophy and
separate gastric perforations. Frontal spot radiograph from repeated upper
gastrointestinal study with water-soluble contrast medium after surgical
repair of perforation shows new leak of contrast material (white
arrow) from gastric fundus near gastroesophageal junction into collection
(arrowheads) in left upper quadrant. Also note large extraluminal gas
collection (black arrows) in upper abdomen.
|
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Fig. 1C. —20-year-old man with Duchenne's muscular dystrophy and
separate gastric perforations. Right posterior oblique spot radiograph from
same study as B shows accumulation of contrast material and debris in
large collection (small black arrows) in left upper quadrant below
the esophagus (straight white arrow). Also note tiny amount of
contrast material (curved arrow) entering extraluminal gas collection
(large black arrows) to right of midline.
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Discussion
It has been well documented that abnormal smooth muscle in the wall of the
stomach may cause acute gastric dilatation in patients with Duchenne's
muscular dystrophy, occasionally necessitating placement of a nasogastric tube
for urgent decompression of the stomach
[5-7].
In our patient, however, nasogastric intubation was complicated by gastric
perforation and peritonitis. Although the nasogastric tubes used by our
surgeons are relatively rigid, such tubes are routinely used for patients who
undergo laparotomy and cause no complications in almost all cases. Surgery
revealed focal perforation of the greater curvature of the gastric body near
the tip of the nasogastric tube (Fig.
1A), presumably because of the traumatic effect of the tube on a
dilated stomach with a paper-thin wall. After surgical repair of the
perforation, the patient developed recurrent signs of peritonitis, and
repeated surgery revealed a separate perforation of the gastric fundus near
the gastroesophageal junction (Figs.
1B and
1C). The fact that the second
perforation occurred at a site remote from the original perforation lends
credence to the notion that our patient had a fragile gastric wall at
increased risk for perforation.
Multiple gastric perforations have been reported in the pathology
literature as a cause of death in a patient with Duchenne's muscular dystrophy
[1]. At autopsy, the patient
had areas of ulceration and necrosis in the stomach. In the same autopsy
series, other patients with muscular dystrophy were found to have abnormal
stomachs or small intestines with marked atrophy and edema of smooth muscle
fibers, resulting in thinning of the bowel wall
[1]. We therefore believe that
gastric wall atrophy and thinning in patients with Duchenne's muscular
dystrophy increase the risk of gastric perforation after nasogastric
intubation or surgical procedures involving the stomach.
In summary, radiologists should be aware of the increased risk of gastric
perforation associated with nasogastric intubation or surgical procedures on
the stomachs of patients who have Duchenne's muscular dystrophy because of the
thinning and increased fragility of the gastric wall.
References
- Bevans M. Changes in the musculature of the gastrointestinal tract
and in the myocardium in progressive muscular dystrophy. Arch
Pathol 1945;40:225
-238
- Huvos AG, Pruzanski W. Smooth muscle involvement in primary muscle
disease. II. Progressive muscular dystrophy. Arch
Pathol 1967;83:234
-240[Medline]
- Barohn RJ, Levine EJ, Olson JO, Mendell JR. Gastric hypomotility in
Duchenne's muscular dystrophy. N Engl J Med1988; 319:15
-18[Abstract]
- Chung BC, Park HJ, Yoon SB, et al. Acute gastroparesis in
Duchenne's muscular dystrophy. Yonsei Med J1998; 39:175
-179[Medline]
- Crowe GG. Acute dilatation of stomach as a complication of muscular
dystrophy. Br Med J1961; 5236:1371
- Robin GC, Falewski GL. Acute gastric dilatation in progressive
muscular dystrophy. Lancet1963; 27:171
-172
- Bensen ES, Jaffe KM, Tarr PI. Acute gastric dilatation in Duchenne
muscular dystrophy: a case report and review of the literature.
Arch Phys Med Rehabil1996; 77:512
-514[Medline]
- Leon SH, Schuffler MD, Kettler M, Rohrmann C. Chronic intestinal
pseudoobstruction as a complication of Duchenne's muscular dystrophy.
Gastroenterology1986; 90:455
-459[Medline]
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Introduction
Case Report
