A woman, aged about 70 years came to the ER with a history of haematemesis and vomiting after eating sweets. Severe pain in the neck and throat, specifically on swallowing, was also present. The blood reports were normal and a CT neck scan showed no sign of the presence of any foreign body. She was admitted for further investigation and diagnosis with the possibility of a foreign body to be ruled out.

A 70-degree rigid endoscope was used to perform a video laryngoscopy under local anaesthesia. A smooth hemorrhagic lesion was observed in the post-cricoid region. Congested and edematous arytenoids which was seen to be greater on the left side than on the right. While the true cords were normal and had equal bilateral movement, and pyriform fossae were normal, there was also mild edema of the false cords.

An upper GI endoscopy, done after obtaining the opinion of a gastroenterologist, showed linear submucosal haematoma from 14 to 22 cms. The other aspects of the study were normal. Proton pump inhibitors were prescribed and the pain began to subside and the patient was able to start consuming soft solids. Cardiology consultation obtained was normal. Platelet count, PT, PTT and INR was normal.

Discussion

The second layer of submucosa in the esophagus contains esophagal glands, Meissner nerve plexus and blood vessels. Intramural hematoma of the esophagus (IHE), also known as dissecting intramural hematoma, is a rare condition, with acute mucosal and submucosal injuries that result in interlayer blood collection.

IHE may be either spontaneous or secondary to injury caused by toxic substance ingestion, foreign bodies or iatrogenic intervention related.  Based on etiology, IHE can be broadly classified as:

1. Spontaneous
2. Secondary to the trauma caused by the ingestion of a foreign body
   a. Valsalva maneuver
   b. Lifting of heavy weights
   c. The rapid swallowing of large bulky bolus
   d. Severe vomiting or retching
3. Latrogenic-during procedural intervention such as Nasogastric tube insertion, Upper gastric endoscopy or ERCP, Endotracheal intubation and transesophageal echocardiogram, etc. has been documented in literature mainly in the form of case reports.
4. Abnormal haemostasis-leukemia, hemophilia, thrombocytopenia, renal failure, anticoagulant or antiplatelet therapy
5. Being related to heart disease

Epidemiology

Because of the rarity of IHE, it is difficult to estimate the incidence and prevalence of the condition. One report mentioned bimodal age distribution that peaks at about 45 years. A second peak at 75 years has also been noted. For reasons that are yet to be determined, elderly females are twice as likely to develop IHE as compared to males. Also at high risk are those with some underlying coagulopathy disorder such as hemophilia or inpatients taking antiplatelet or anticoagulants. IHE is also common amongst the elderly who are antiplatelet or anticoagulant therapy. The increased and improved availability of diagnostic technologies has resulted in the earlier recognition of the condition.

Pathophysiology

It has been proposed that sudden pressure changes in the esophagus coupled with a bleeding tendency are the mechanisms that trigger spontaneous IHE. secondary IHE is thought to be the result of an acute injury that is similar to Mallory-Weiss tear and Boerhaave syndrome. The sudden bleeding between the mucosa and the muscularis propria of the esophagus wall is the initiating cause. The progressive submucosal dissection leads to severe pain and signs of the esophageal lumen being blocked.  There is a higher risk of IHE in patients one anticoagulant therapy.

History and Physical Issues

IHE normally presents with sudden chest or retrosternal pain. The triad of symptoms is retrosternal chest pain, hematemesis and dysphagia.  At least 2 of these normally appear. Any history of bleeding or use of anticoagulant medication should be part of the evaluation. Any history of violent retching, vomiting or instrumentation of the esophagus should for part of the evaluation. In rare cases, a foreign body may have been ingested and is present.

Physical examination rarely reveals any symptoms except tachycardia and pallor. Extreme care should be taken to ensure that IHE is not a case of acute chest pain as the use of anticoagulants could make a cardiac condition worse. If dysphagia or odynophagia are present, differentiating IHE for retrosternal pain is simpler.

Evaluation

Multiple modalities are currently being used for diagnosis. A CT scan with high contrast is the most common. This may show a smooth filling defect in the lumen of the esophagus, a thickened esophageal wall. A high contrast CT will show the anatomical relationship between the esophagus, aorta and mediastinal structures. Oral contrast and imaging are advisable when transmural perforation is suspected. Endoscopic ultrasound will show submucosal lesions better than plain endoscopy and will also evaluate adjacent structures. MRI can help to distinguish IHE from other mediastinal pathologies.

It is best to delay endoscopy until the integrity of the esophageal wall is determined. EKG, cardiac markers and chest x-rays can be sued to exclude cardiopulmonary conditions. The presence of pneumothorax, pneumomediastinum, or pleural effusion may indicate transmural injuries to the esophagus.

It is proposed that the severity be graded as follows:

• Stage 1 – isolated hematoma
• Stage 2 – hematoma surrounded by tissue edema
• Stage 3 – compression of the esophageal lumen
• Stage 4 – The esophageal lumen is obliterated by the hematoma.

Treatment

Conservative treatment is advisable. In the initial stages, oral intake is withheld and IV fluids and proton pump inhibitors are used. As the condition improves, oral intake is gradually permitted. A serial CT or contrast swallow study may be required to monitor the recovery.

Recurrent bleeding should raise suspicion of hematoma leakage or a rupture into the esophageal lumen. In such cases, emergency airway protection or therapeutic angiography may be required. Surgery often has poor outcomes but may be required if conservative treatment does not produce the required response or if a massive hemorrhage occurs.

Differential Diagnosis

This will consist of ascertaining the presence of:

• Mallory weiss mucosal tear
• Esophageal perforation
• Boerhaav transmural rupture
• Aortoesophageal fistula
• Esophageal varices rupture
• Esophagitis
• Malignant tumour
• Acute MI
• Pulmonary embolism
• Aortic dissection

All cardiothoracic possibilities must be considered for the differential diagnosis of IHE. A chest x-ray could show pneumomediastinum, pneumothorax, and pleural effusions. Myocardial infarction, aortic dissection and pulmonary embolisms should be ruled out. Hematemesis and dysphagia or odynophagia, present in IHE may be used to differentiate from other critical diseases.

Conclusion

We have presented this case of intramural haematoma of the esophagus as it is a rare disorder. This condition responds well to conservative management. It is also important to remember the more life threatening differential diagnosis and evaluate for the same especially if there is no good response to conservative line of management.

This case was particularly unusual as the haematoma was in the proximal esophagus which is extremely rare and there was an associated post-circoid haematoma separate from the esophageal haematoma. In our search of literature, this type of presentation is being reported for the first time.

References:
https://www.ncbi.nlm.nih.gov/books/NBK459228/
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3668800/