Esophageal Hematoma

Etiology

The proposed mechanism involves a rapid alteration in intrathoracic and intraesophageal pressures triggered by events such as vomiting, retching, and coughing, leading to the formation of an intramural hematoma that may rupture into the esophageal lumen. Additionally, the use of anticoagulants, antiplatelet medications, and coagulopathic disorders are associated with the development of intramural esophageal hematoma. In certain cases, intramural esophageal hematoma can occur spontaneously without an identifiable cause.[4]

Intramural hematoma of the esophagus can be associated with various etiologies and broadly classified as either spontaneous or secondary to trauma and iatrogenic procedural interventions. Secondary causes include traumatic ingestion of foreign bodies, Valsalva maneuver, lifting heavy weights, hurried large bulky bolus swallowing, nasogastric tube insertion, upper gastrointestinal endoscopy or endoscopic retrograde cholangiopancreatography, endotracheal intubation, and transesophageal echocardiogram, as documented mainly in case reports in the literature.[5][6][7][8][9][10]

Epidemiology

Esophageal traumatic injuries, including traumatic penetration and perforation, are rare, making it challenging to estimate the incidence or prevalence of intramural hematoma of the esophagus.[11] Most incidents have been reported in case reports. A large case series review notes a bimodal age distribution, with peaks around the ages of 45 and 75. Intramural hematoma of the esophagus is more commonly observed in older females, who are twice as likely to develop this condition compared to males, for reasons that are not yet understood.

Patients with underlying coagulopathy disorders, such as hemophilia or inpatients taking antiplatelet or anticoagulant medications, are at higher risk of developing both spontaneous and secondary intramural hematoma of the esophagus.[12] Although the development of this entity is rare, it is increasingly being recognized early due to the widespread availability of modern radiological and endoscopic facilities.[13][14][15]

Pathophysiology

In patients with normal blood clotting, vomiting can lead to the formation of a submucosal hematoma, typically in the lower esophagus. This suggests that the pressure from vomiting can damage the mucosa and submucosal vessels. In a reported case, the esophageal submucosal hematoma was located on the right anterior wall of the distal esophagus—an area prone to increased intra-abdominal pressure. Initially forming in the lower esophagus, the hematoma likely resulted from mucosal laceration, leading to bleeding and collapse. This bleeding from ruptured submucosal vessels likely extended into the upper esophagus, resulting in a widespread esophageal submucosal hematoma.[16]

A sudden pressure change in the esophagus and an underlying bleeding tendency have been proposed as mechanisms of spontaneous intramural hematoma of the esophagus. Secondary intramural hematoma of the esophagus is thought to result from an acute injury similar in mechanism to a Mallory-Weiss tear and Boerhaave syndrome, with the intramural hematoma representing an intermediate stage. The proposed initiating cause for the development of this condition is sudden bleeding between the mucosa and muscularis propria of the esophageal wall, sometimes involving a long segment of the esophagus. This progressive submucosal dissection due to bleeding leads to symptoms ranging from severe pain to signs of esophageal lumen obstruction. Later, a breach of the mucosa containing the hematoma can occur, presenting as hematemesis.[15] 

History and Physical

Intramural hematoma of the esophagus usually presents as a sudden onset of chest or retrosternal pain. Most patients (80%) present with at least 2 of the classical triad of symptoms—retrosternal chest pain (84%), dysphagia or odynophagia (59%), and hematemesis (56%). While taking the patient's history, it is important to inquire about any bleeding diathesis or anticoagulation medication. A history of violent retching, vomiting, or instrumentation of the esophagus may also be present. Rarely, a history of foreign body ingestion is present.

Physical examination may only reveal nonspecific findings such as tachycardia and pallor. In addition, it is crucial to distinguish esophageal hematoma from other causes of acute chest pain. The presence of dysphagia or odynophagia can help exclude significant cardiac causes of retrosternal pain.[17]

Evaluation

Multiple modalities have been used to diagnose this condition.[18] The preferred primary investigation is a computed tomography (CT) scan with intravenous contrast. This noninvasive technique can be performed quickly and is available in most centers. The typical CT finding is a thickened esophageal wall with luminal compression or, in large hematomas, obliteration of the lumen. An intraesophageal mass or filling defect may be visualized, sometimes resembling a double-barrel or dual lumen.

A contrast-enhanced CT will delineate the anatomical relationship between the esophagus, aorta, and mediastinal structures. Often, the contrast study of the esophagus will demonstrate a smooth filling defect in the lumen of the esophagus. Administration of oral contrast and imaging should be performed when transmural perforation is suspected. The extravasation of oral contrast extraluminally is diagnostic and can demonstrate the location of the rent in the mucosa.

Other investigative modalities, such as endoscopic ultrasound and magnetic resonance imaging (MRI), can help diagnose intramural hematoma of the esophagus. Endoscopy should be postponed until the integrity of the esophageal wall has been established. If needed, an endoscopy can be performed with care after confirming the integrity of the esophageal wall. An endoscopy will reveal a bluish swelling with or without a mucosal tear. Endoscopic ultrasound is superior to conventional endoscopy because it can demonstrate submucosal lesions and evaluate adjacent structures. MRI can show a mass of intermediate density on T1- and T2-weighted images, distinguishing intramural hematoma of the esophagus from other mediastinal pathologies and displaying soft tissue planes around the aorta.

An electrocardiogram (ECG), chest radiograph, and cardiac markers are useful in excluding other cardiopulmonary diseases. The presence of pneumothorax, pneumomediastinum, or pleural effusion should raise a strong suspicion of a transmural injury to the esophagus and an intramural hematoma.

A proposed grading of the degree of luminal involvement by the hematoma includes:

• Stage I: Isolated hematoma
• Stage II: Hematoma surrounded by tissue edema
• Stage III: Hematoma causing compression of the esophageal lumen
• Stage VI: Obliteration of the esophageal lumen by the hematoma [10]

Treatment / Management

Treatment for intramural hematoma of the esophagus is usually conservative. Initial management involves withholding oral intake, administering intravenous fluids, correcting any associated coagulopathy, and administering proton pump inhibitors. Serial CT scans or contrast swallow studies are needed to monitor clinical progress and hematoma resolution. The patient is gradually allowed oral feeding as symptoms improve. In most cases, medical and conservative treatment typically result in the complete recovery of patients (see Image. Progression and Healing Stages of Esophageal Hematoma).

Recurrent bleeding or increased difficulty swallowing should raise suspicion for hematoma leakage or rupture into the esophageal lumen and expanding hematoma, respectively. This situation should be managed as an acute emergency with airway protection and hemodynamic resuscitation. Therapeutic angiography may become necessary in cases of recurrent massive hematemesis to stop bleeding and hematoma expansion via transarterial embolization. Surgery, typically associated with poor outcomes, may be necessary for patients who do not respond to conservative therapy or who experience massive recurrent hemorrhage leading to hemodynamic instability or severe esophageal luminal obstruction or perforation.[19][20][21][22][18](A1)

Differential Diagnosis

During the initial presentation of a patient with suspected intramural hematoma of the esophagus, it is essential to consider all acute cardiothoracic emergencies in the differential diagnosis. Classic differentials include Mallory-Weiss syndrome (mucosal tear) and Boerhaave syndrome (transmural perforation), as intramural hematoma of the esophagus can be an intermediate stage between these two conditions. While a history of severe vomiting often precedes Mallory-Weiss and Boerhaave syndromes, it is not a reliable factor for exclusion.

A chest x-ray may reveal pneumomediastinum, pneumothorax, or pleural effusion in cases of Boerhaave syndrome. Acute retrosternal chest pain is also common in acute myocardial infarction, aortic dissection, and pulmonary embolism, which should be ruled out through careful history-taking, physical examination, and appropriate diagnostic tests. Hematemesis and dysphagia or odynophagia in intramural hematoma of the esophagus can help differentiate it from other critical conditions when used alongside other diagnostic modalities.[22]

Once an accurate diagnosis is established, appropriate treatment should be administered based on the patient's specific symptoms and condition. Treatment options for spontaneous intramural hematoma of the esophagus include the following:

• Conservative treatment: This involves medical approaches such as fasting, acid suppression, hemostasis, and protection of the esophageal and gastric mucosa. This method is suitable for cases where the muscle layer is unaffected, and the hematoma can be absorbed within 1 to 3 weeks.

• Interventional embolization: This treatment is appropriate for complications such as mediastinal hematoma or hemothorax.

• Surgery: This is necessary in cases of esophageal rupture.[18]

Prognosis

Intramural hematoma of the esophagus is considered a benign condition, with a generally favorable prognosis when managed promptly and appropriately. Most patients fully recover with conservative treatment, including rest, pain management, and dietary modifications. Patients typically experience significant symptom relief within days to weeks, and intramural hematoma of the esophagus fully resolves in 3 to 4 weeks.[15] However, it is essential for clinicians to monitor for any signs of deterioration, especially in patients with underlying risk factors or comorbidities.

Complications

Although rare, complications of intramural hematoma of the esophagus can be severe and require prompt attention. They are mainly related to intraluminal bleeding secondary to rupture of hematoma in the esophageal lumen and expanding hematoma causing esophageal luminal obstruction. Increasing swallowing difficulty should raise suspicion of expanding hematoma. The most significant complication is esophageal perforation, which can lead to mediastinitis and sepsis and necessitate surgical intervention.

Other potential complications include secondary infection of the hematoma, stricture formation, and persistent dysphagia due to scar tissue. Additionally, there is a risk of rebleeding, especially in patients with underlying coagulopathies or those on anticoagulant therapy. Aspiration of blood or necrotic tissue into the lungs can lead to aspiration pneumonia. Early and accurate diagnosis through imaging and endoscopic evaluation is crucial to prevent complications and ensure a positive outcome. Regular follow-up and monitoring are essential to promptly detect and address any developing complications.[23]