Intramural hematoma of the esophagus (IHE) is a rare form of injury to the gullet which is caused by esophageal mucosal dissection when bleeding occurs intramurally. It frequently occurs in elderly patients who are on anticoagulant or antiplatelet therapy. This type of injury has been reported to occur spontaneously or following instrumentation as well as after the ingestion of a foreign body resulting in mucosal damage. An iatrogenic injury caused by instrumentation of the esophagus has also been postulated to lead to the development of this condition. Nasogastric tube insertion and transoesophageal echocardiogram are two of procedures that have been reported to cause intramural hematoma of the esophagus. Females have a preponderance to develop this condition.
This acute esophageal condition frequently occurs among the 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. IHE more commonly occurs in elderly females who are twice as likely to develop this condition when compared to males.
Intramural hematoma of the esophagus is thought to occur as 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 which occurs 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 mucosa which confines the hematoma can occur at a later stage, and this then presents as hematemesis. Although this acute esophageal condition has been reported to occur spontaneously, risk factors include increased esophageal pressure and iatrogenic trauma. Patients on anticoagulants therapy are at a higher risk factor in developing this problem. Presence of hypertension may also be a contributory factor for the development of this condition.
Intramural hematoma of the esophagus usually presents as a sudden onset of a chest or retrosternal pain. Patients complain of developing acute oesophageal symptoms when a submucosal esophageal hematoma occurs. A majority of patients present with at least two of the classical triad of symptoms which are retrosternal chest pain, hematemesis, occurs rare dysphagia or odynophagia. There may be an associated history of violent retching, vomiting or instrumentation of 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 reason for the retrosternal pain
Multiple modalities have been used to diagnose this condition. A CT scan with intravenous contrast is the preferred primary investigation which 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. An 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. Endoscopy may inadvertently worsen the situation causing a perforation while insufflation and may not result in the visualization of any mucosal damage. Endoscopic ultrasound carries the same risks but is superior to plain endoscopy in that it can demonstrate submucosal lesions as well as evaluating adjacent structures. MRI can show an intramural hematoma on T1 and T2 weighted images as well as displaying soft tissue planes around the aorta. Plain x-ray is usually non-contributory to the primary diagnosis, but the presence of associated pneumothorax, pneumomediastinum, or pleural effusion raises 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 esophageal lumen, and stage VI when there is an obliteration of the esophageal lumen by the hematoma.
Intramural hematoma of the esophagus has a benign course in most instances. Most cases have been reported to resolve in 3 to 4 weeks time. The treatment is conservative. The patient needs hospitalization and monitoring in a medical center with radiological and endoscopic facilities. The initial management of the patient involves withholding oral intake, adequate nutritional support, correction of associated coagulopathy, and administration of proton pump inhibitors. The patient is gradually allowed oral feeding as the symptoms improve. Progress can be monitored by serial CT or contrast swallow. Medical and conservative treatment results in full recovery in most cases. Appropriate surgery or therapeutic angiography is necessary for those who do not respond to conservative therapy or have massive hemorrhage leading to hemodynamic instability.
Intramural hematoma of the esophagus is rare, but there is an increasing incidence in the elderly. This condition presents as an acute esophageal condition in patients who are on anticoagulation therapy. It is commoner in those who have had esophageal instrumentation or a possible traumatic mucosal injury. With an increasingly older population who have cardiovascular risks on anticoagulation, it is essential to recognize and diagnose this condition early. Confusion with acute cardiac pain could lead to a potentially dangerous complication with the use of anticoagulation or thrombolytic therapy.
Intramural esophageal hematoma in most cases is due to a traumatic event. The condition is best managed by an interdisciplinary team because the diagnosis can be difficult. Whenever the esophageal hematoma is diagnosed, serial imaging studies are needed in symptomatic patients to ensure that there is no frank perforation of the esophagus. A leak of esophageal contents in the chest cavity is usually fatal without treatment. There are several reports of anticoagulant drugs that have resulted in an intranural esophageal hematoma, thus the importance of the monitoring therapeutic coagulation times by the pharmacist or nurse practitioner. If they become aware of a complication or a change in coagulation times, the monitoring providers should discuss further therapeutic changes with the clinician overseeing the care of the patient. The best outcomes will be achieved by an interdisciplinary approach to monitoring the care of these patients.  [Level V]
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