Continuing Education Activity

Intramural hematoma of the esophagus (IHE) or dissecting intramural hematoma of the esophagus is an uncommon condition that presents with acute esophageal symptoms. The patient may present with dysphagia. In most cases, there is no specific treatment as the hematoma will spontaneously regress. This activity describes the pathophysiology of an intramural esophageal hematoma and highlights the role of the interprofessional team in the management of these patients.

Objectives:

• Identify the etiology of intramural esophageal hematoma.
• Review the presentation of a patient with an intramural esophageal hematoma.
• Outline the treatment and management options available for esophageal hematoma.
• Explain interprofessional team strategies for enhancing care coordination and communication to advance the management of intramural esophageal hematoma and improve outcomes.

Introduction

The esophagus is approximately 25-centimeter muscular tube that connects the pharynx to the stomach. Histologically it comprises of innermost mucosal layer, submucosal layer, muscular, and adventitial layer. submucosal layer. The second layer submucosa which connects the mucosal and muscular layer contains blood vessels, Meissner nerve plexus, and esophageal glands.  Intramural hematoma of the esophagus (IHE) or dissecting intramural hematoma is a very rare manifestation of acute mucosal and submucosal injuries resulting in an interlayer collection of blood. IHE can be spontaneous and secondary to trauma with foreign body, toxic substance ingestion, or iatrogenic intervention related.  Patients with IHE can present with symptoms mimicking other acute cardiopulmonary diseases. Classic triad associated with IHE is acute chest pain, odynophagia or dysphagia, and hematemesis. Usually, IHE is a very rare and benign condition. Prognosis is usually excellent with proper diagnosis and management.[1][2][3]

Etiology

Intramural hematoma of the esophagus (IHE) has been associated with multiple etiology and broadly can be classified as spontaneous or secondary to trauma and iatrogenic procedural intervention. Secondary causes such as traumatic ingestion of foreign body, Valsalva maneuver, lifting heavyweight, hurried large bulky bolus swallowing, severe retching, vomiting, Nasogastric tube insertion, Upper gastric endoscopy or ERCP, Endotracheal intubation and transesophageal echocardiogram, etc. has been documented in literature mainly consisted with case reports.[4][5][6][7][8][9]

Epidemiology

In general esophageal traumatic injury, including traumatic penetration and perforation itself, are very rare, which makes it difficult to estimate IHE incidence or prevalence[10]. Most of the incidence has been reported in case reports. One such large case series review mentions bimodal age distribution with the first peak around 45 years and the second peak around 75. IHE more commonly has been observed in elderly females who are twice as likely to develop this condition when compared to males for unknown reasons. Patient with some underlying coagulopathy disorder such as hemophilia or inpatient taking antiplatelet or anticoagulant carries a higher risk of developing both spontaneous and secondary IHE[11]. This acute esophageal condition frequently occurs among elderly individuals who are on antiplatelet or anticoagulant therapy. Although the development of this entity is a rare occurrence, it is increasingly being recognized early with the easy availability of modern radiological and endoscopic facilities.[12][13][14]

Pathophysiology

Sudden pressure change in the esophagus and underlying bleeding tendency has been proposed as a mechanism of spontaneous intramural hematoma of the esophagus. Secondary IHE of the esophagus is thought to occur as a result of an acute injury that is similar in mechanism to Mallory-Weiss tear and Boerhaave syndrome; although, IHE represents an intermediate stage. The proposed initiating cause for the development of this condition is the sudden bleeding between the mucosa and muscularis propria of the esophageal wall, at times involving a long segment of the esophagus. This progressive submucosal dissection due to bleeding leads to symptoms varying from severe pain to signs of obstruction of the esophageal lumen. Breach of the mucosa, which confines the hematoma, can occur at a later stage, and this then presents as hematemesis. Patients on anticoagulants therapy are at a higher risk factor in developing IHE.[14] 

History and Physical

Intramural hematoma of the esophagus usually presents as a sudden onset of chest or retrosternal pain. Most patients present with at least two of the classical triad of symptoms which are retrosternal chest pain, hematemesis, and dysphagia or odynophagia. History of Bleeding diathesis or anticoagulation medication should be included in the evaluation. There may be an associated history of violent retching, vomiting, or instrumentation of the esophagus. Rarely a history of foreign body ingestion may be present. Physical examination may not reveal any specific findings except tachycardia and pallor. It is essential to differentiate this condition from any cause of acute cardiac pain, as any anticoagulant therapy will worsen the situation. The presence of dysphagia or odynophagia can help to exclude significant cardiac reasons for the retrosternal pain.

Evaluation

Multiple modalities have been used to diagnose this condition. A CT scan with intravenous contrast is the preferred primary investigation that can be done quickly, available in most centers, and is non-invasive. Very often, the contrast study of the esophagus will demonstrate a smooth filling defect in the lumen in the esophagus. The typical CT finding is a thickened esophageal wall with luminal compression or, in large hematomas, the obliteration of the lumen. A CT scan shows an intra-esophageal mass or filling defect, which may sometimes resemble a double barrel or dual lumen. A contrast-enhanced CT will also delineate the anatomical relationship between the esophagus, aorta, and mediastinal structures. Administration of oral contrast and imaging should be performed in instances where the transmural perforation is suspected. 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 MRI are found to be useful in diagnosing intramural hematoma of the esophagus. Endoscopy should be postponed until the integrity of the esophageal wall had 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 plain endoscopy in that it can demonstrate submucosal lesions as well as can evaluate adjacent structures. MRI can show a mass of intermediate density on T1- and T2-weighted images and can distinguish IHE from other mediastinal pathologies as well as displaying soft tissue planes around the aorta. EKG, chest x-ray, and cardiac markers are useful to exclude other cardiopulmonary diseases. The presence of pneumothorax, pneumomediastinum, or pleural effusion should raise the strong suspicion of a transmural injury to the esophagus along with the intramural hematoma. A proposed grading of the severity of the luminal involvement by the hematoma is stage 1 when there is an isolated hematoma, stage II hematoma surrounded by tissue edema, stage III when there is compression of the esophageal lumen, and stage VI when there is an obliteration of the esophageal lumen by the hematoma.[9]

Treatment / Management

Treatment for IHE is usually conservative. The initial management of the patient involves withholding oral intake, intravenous fluids, correction of associated coagulopathy, and administration of proton pump inhibitors. A serial CT scan or contrast swallow study is needed for monitoring of clinical progress and resolution of hematoma. The patient is gradually allowed oral feeding as the symptoms improve. Medical and conservative treatment results in full recovery in most cases. Recurrent bleeding or increasing difficulty should raise suspicion for hematoma leakage or rupture into the esophageal lumen and expanding hematoma, respectively, and should be managed as an acute emergency with airway protection and hemodynamic resuscitation. Therapeutic angiography may become necessary in the case of recurrent massive hematemesis to stop bleeding and hematoma expansion with transarterial embolization. Surgery usually is associated with poor outcomes but may become necessary for those who do not respond to conservative therapy or have massive recurrent hemorrhage leading to hemodynamic instability or associated severe esophageal luminal obstruction or perforation.[15][16][17][18]

Differential Diagnosis

On presentation, consideration must be given to almost all acute cardiothoracic emergencies for differential diagnosis of IHE, but the classic clinical differential diagnosis includes Mallory Weiss (mucosal tear) and Boerhaave syndrome (transmural perforation) as IHE is an intermediate stage of these two conditions. History usually includes preceding severe vomiting in Mallory Weiss and Boerhaave syndrome; however not a reliable factor to exclude two conditions. A chest x-ray may show pneumomediastinum, pneumothorax, and pleural effusion in case of later. Acute retrosternal chest pain is a common symptom in acute myocardial infarction, aortic dissection, and pulmonary embolism and should be ruled out with careful history, physical examination, and appropriate diagnostic tests as these conditions are more common compared to IHE. The presence of hematemesis and dysphagia or odynophagia in IHE can be useful in differentiating from other critical diseases in conjunction with other diagnostic modalities.[18]

Prognosis

IHE is considered a benign condition. Most patients fully recover with conservative treatment. The prognosis is excellent, and IHE resolves in 3 to 4 weeks.[14] 

Complications

Complications of IHE 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 for expanding hematoma. Endoscopic or surgical decompression may be needed in such a case.[19]

Consultations

Management of IHE and related complications needs an interprofessional approach which includes gastroenterology, interventional radiology, and cardiothoracic surgery services.

Deterrence and Patient Education

Patients with IHE must stay compliant with dietary restrictions and serial imaging follow-up to avoid complications. The patient must be educated on symptoms such as difficulty swallowing, blood in vomiting, dark stool, or melena for prompt diagnosis of complication. Efforts should be directed to avoid any such act which can increase intraesophageal pressure or trauma such as heavy weightlifting, Valsalva maneuvers, abrupt and fast swallowing of large food bolus which otherwise can result in exacerbation of symptoms and complication. Patients on antiplatelet or anticoagulants for other reasons should closely follow up with their physician regarding the resumption of these medications safely.

Pearls and Other Issues

Intramural hematoma of the esophagus is rare. This condition presents as an acute esophageal condition in patients who either have a bleeding tendency or are on anticoagulation therapy. It is more common in those who have had esophageal instrumentation or a possible traumatic mucosal injury though spontaneous IHE is also possible. With an increasingly older population who have cardiovascular risks on anticoagulation, it is essential to recognize and diagnose this condition early. Misdiagnosis of IHE as other cardiopulmonary diseases could lead to a potentially dangerous complication with the use of anticoagulation or thrombolytic therapy.

Enhancing Healthcare Team Outcomes

Intramural esophageal hematoma is best managed by an interprofessional team which includes surgery and medical subspecialist and Nursing staff because of relatively rare diagnosis and associated complication. Physicians and nursing staff should be aware of the clinical course, pathology, and complications of disease for early recognition of symptoms related to complications and timely intervention by medical and surgical subspecialists if needed. Given the rarity of IHE, the article is based on mainly observational studies, expert opinions, and case reports, hence considered as weak evidence level [Oxford CEBM Level V] [20][17]