IS Case 70: Boerhave's Syndrome

Sam McCabe, MD


Imaging Sciences URMC 2008
Publication Date: 2009-05-19

History

A 27-year-old male presented with leukocytosis and epigastric pain 10 hours after a prolonged episode of severe vomiting and retching.

Findings

Plain radiographs revealed ileus and subtle lucent streaks in the region of the medial left diaphragm. Non IV contrast enhanced CT after administration of PO contrast demonstrated a small amount of extraluminal gas in the region of the gastroesophageal (GE) junction as well as a small left pleural effusion. Subsequent esophagram demonstrated focal contrast extravasation from the left lateral esophageal wall, just above the GE junction.

Diagnosis

Boerhave's Syndrome

Discussion

Boerhave's syndrome is defined as spontaneous esophageal rupture due to increased intraluminal pressure from retching against a closed glottis. It accounts for only 15% of esophageal perforations, most being due to penetrating or less commonly blunt trauma, ingestion, or iatrogenic injury. In simple Boerhave's, the lesion is invariably located in the left lateral distal esophagus, 2-3 cm above the GE junction. This location has a thin muscular layer and lacks adjacent supporting structures, making it an anatomical weak point. Prior surgery, radiation, adjacent neoplasms or other insults to the esophagus may cause focal weakening and predispose to other sites of rupture. The tear is usually vertically oriented and 1-4 cm in length.

Plain radiographs are often normal. Findings may include pneumomediastinum, especially around the left costovertebral angle. This has been referred to as the V-sign of Naclerio. Other findings may include mediastinal widening, pleural effusion, atelectasis, and hydropneumothorax.

CT typically reveals small gas collections around the esophagus. Other additional findings may include extravasation of oral contrast, esophageal thickening, or periesophageal or mediastinal fluid collections. CT is limited in its ability to define the site or extent of the tear.

The diagnostic procedure of choice remains the esophagram, which can confirm the location and extent of the tear. Nonionic contrast should be used instead of gastrografin, due to the risk of aspiration. If no leak is seen, barium should be administered orally. Barium may demonstrate small leaks not visible initially.

Early diagnosis is key. Delayed diagnosis and treatment lead to fluminant mediastinitis. This carries a mortality rate of 70%, the highest of any GI perforation.

Treatment for large tears is emergent thoracotomy and attempted primary repair. Small tears tend to heal on their own and may be managed conservatively with broad spectrum antibiotics and parenteral nutrition.

References

  1. Rubesin SE, Levine MS. Radiologic diagnosis of gastrointestinal perforation. Radiol Clin North Am. 2003 Nov;41(6):1095-115. [PMID: 14661660]
  2. Zylak CM, Standen JR, Barnes GR, Zylak CJ. Pneumomediastinum revisited. Radiographics. 2000 Jul-Aug;20(4):1043-57. [PMID: 10903694]

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