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Unusual cause of intra-abdominal perforation
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  1. James Hong-En Kang,
  2. Matthew Williams
  1. Gastroenterology department, James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, UK
  1. Correspondence to Dr James Hong-En Kang, James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, UK; james.kang{at}nhs.net

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A 57-year-old man presented with abdominal distension and severe abdominal pain, on a background of two stone unintentional weight loss over 4 months. Examination revealed a distended, tender and rigid abdomen. Cross-sectional imaging of the abdomen and pelvis was undertaken (figure 1A,B).

Figure 1

CT abdomen (A) demonstrating gastric dilatation with calibre change at the third part of the duodenum between the superior mesenteric artery and aorta. There is gastric intramural gas and intraperitoneal gas consistent with gastric perforation. CT abdomen (B) showing a dilated oesophagus, consistent with the diagnosis of achalasia.

Question

What is the diagnosis?

Answer

CT (figure 1A,B) shows gastric dilatation with calibre change at the third part of the duodenum between the superior mesenteric artery (SMA) and aorta (arrow, figure 1B)—the SMA syndrome. There is gastric intramural gas and intraperitoneal gas consistent with gastric perforation (arrow, figure 1A).

SMA syndrome occurs due to compression of the third part of the duodenum between the SMA and the abdominal aorta. Normally, the SMA comes off the aorta at a 45° angle. Loss of mesenteric adipose tissue secondary to rapid weight reduction can decrease this angle, causing obstruction.1

Before admission, the patient had been undergoing outpatient investigation for intermittent chest pain and dysphagia. Gastroscopy revealed food and liquid debris in the oesophagus. Following admission, barium studies were performed that showed a tight stricture at the gastro-oesophageal junction—the classical ‘birds beak’ appearance (arrow, figure 2). Subsequent high-resolution oesophageal manometry was consistent with Chicago classification type 2 achalasia.

Figure 2

Barium swallow study showing a gastro-oesophageal stricture with a classical ‘bird's beak’ appearance, consistent with the diagnosis of achalasia.

Achalasia is characterised by oesophageal aperistalsis and incomplete lower oesophageal sphincter relaxation on swallowing. It commonly presents as dysphagia for both solids and liquids, often with unintentional weight loss. It is diagnosed by endoscopy, contrast studies and oesophageal manometry.2

The patient underwent emergency laparotomy with decompression, closure of the gastric perforation and gastrojejunostomy to bypass the obstruction.

He was later treated successfully by pneumatic dilatation as an outpatient.

Unusually, the weight loss that led to SMA syndrome in this patient was due to previously undiagnosed achalasia. Duodenal obstruction would usually cause profuse vomiting. However, no vomiting episodes were recorded in the hospital inpatient notes. In this patient, it is likely that duodenal obstruction secondary to SMA syndrome, together with the absence of transient lower oesophageal sphincter relaxations present in achalasia, created a closed system that could not decompress distally or proximally. We hypothesise that this is the reason that the stomach distended to the point of perforation, which would otherwise be a very uncommon occurrence in a patient with duodenal obstruction who is able to vomit normally.

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References

Footnotes

  • Contributors MW examined the patient and conceived the idea for the case report. JH-EK wrote the first draft of the manuscript. Both authors reviewed and amended the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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