What is Esophageal Intramural Pseudodiverticulosis?
Diffuse intramural esophageal diverticulosis is a rare condition where numerous tiny, flask-like pouches develop in the wall of the esophagus, which is the tube that connects your mouth to your stomach. These pouches form from the ducts of small glands inside the esophagus wall. Therefore, the condition is also referred to as esophageal intramural pseudodiverticulosis (EIP). This was first identified by Mendl and his team in 1960.
Typically, people with this condition experience difficulty in swallowing and food getting stuck due to a narrowing in the upper part of the esophagus. The condition can be diagnosed through x-rays or endoscopy, which uses a flexible tube with a camera to view the esophagus, and by studying the tissue from the esophagus under a microscope.
What Causes Esophageal Intramural Pseudodiverticulosis?
The exact cause of this condition isn’t fully clear and more research needs to be done. However, several studies associate tobacco smoking and alcohol consumption as major risk factors. There are suggestions in medical studies about a potential link between this condition and esophageal moniliasis, a type of fungal infection in the esophagus.
Other connections mentioned in medical literature include gastroesophageal reflux disease (a condition where stomach acid frequently flows back into the tube connecting the mouth and stomach), hiatal hernia (a condition in which part of the stomach pushes up through the diaphragm muscle), diabetes, corrosive ingestion (accidental swallowing of a harmful substance), Plummer-Vinson syndrome (a rare disease characterized by difficulty swallowing), and esophageal carcinomas (cancer of the esophagus). Some reports also suggest that motility disorders, conditions that affect the body’s ability to move food through the digestive system, could be a predisposing factor for this condition.
Risk Factors and Frequency for Esophageal Intramural Pseudodiverticulosis
Diffuse intramural esophageal diverticulosis is a rare non-cancerous disease, with only around 200 cases reported worldwide. In one study involving 14,350 patients who underwent a diagnostic procedure known as a barium swallow esophagram, only 21 patients were diagnosed with the disease, meaning the occurrence of this disease is approximately 0.15%.
- The disease can affect people of any age, from as young as 8 months to as old as 86 years, with the average age of diagnosis being 53.5 years.
- The disease seems to affect more males (58%) than females (42%).
- However, other studies have shown that the disease commonly affects people in two age groups: teenagers and individuals in their 50s and 60s. This is known as a bimodal pattern of age distribution.
Signs and Symptoms of Esophageal Intramural Pseudodiverticulosis
Diffuse intramural esophageal diverticulosis is a condition where the esophagus develops small pockets or pouches. Most patients experience difficulty swallowing on a regular basis. This could be constant, occur at intervals, or steadily worsen over time. It’s usually difficult for them to swallow solid foods and sometimes they might get food stuck, although this often clears up on its own. However, some people with this condition don’t have any symptoms and they learn about their condition during tests for other health concerns. On average, it takes about 60.5 months, ranging from just two days to 26 years, from the onset of swallowing difficulties to getting a diagnosis. Other possible symptoms include chest pain, tightness in the chest, painful swallowing, and bleeding in the upper gastrointestinal tract.
- Regular difficulty swallowing, which might be constant, intermittent, or progressively worse.
- Difficulty swallowing solid foods.
- Occasional food impaction that often resolves by itself.
- Chest pain and tightness.
- Pain when swallowing.
- Bleeding in the upper portion of the gastrointestinal tract.
Testing for Esophageal Intramural Pseudodiverticulosis
Radiological examinations, which are tests done using x-rays or other types of radiation, are better at detecting a condition known as esophageal intramural pseudodiverticulosis (EIP) compared to endoscopic exams. EIP is when tiny, flask-shaped pockets (diverticula) form in the wall of your esophagus. These pockets can be hard to spot with an endoscope, a device used to look inside your body, because they’re so small.
Endoscopy, however, is still important because it can help diagnose or rule out co-existing conditions such as strictures (narrowed parts of the esophagus), Barrett’s esophagus (damaged esophagus lining), esophagitis (inflammation of the esophagus), or cancer. This is done by directly viewing the affected area and/or taking a sample (biopsy).
When using a CT scan, which gives more detailed pictures of your body’s tissues and organs, the esophageal wall might appear thicker than normal with some uneven narrowing of the inside.
Other tests may use manometry, a technique that measures the pressure inside your esophagus. This can help assess a range of esophagus muscle movement issues. These might include localized or widespread lack of muscle contractions (aperistalsis), lower strength with normal or no contractions, higher strength contractions, widespread esophageal spasms, or normal strength with unusual, uncoordinated contractions (synchronous tertiary contractions).
Treatment Options for Esophageal Intramural Pseudodiverticulosis
Treatment for this health issue focuses mainly on addressing any other existing health conditions and providing relief from symptoms. In fact, about 10% of people do not need to undergo any specific treatment at all for this condition. If the patient also has acid reflux or esophagitis, taking medications to reduce acid reflux can help alleviate their symptoms. Additionally, if a patient has a yeast infection in their esophagus, known as esophageal candidiasis, treating it can help improve their condition.
In some cases, medical professionals may also suggest a procedure called ‘mechanical dilatation’ for patients who have developed esophageal strictures. Esophageal strictures are narrowings of the esophagus which can make swallowing difficult. In the mechanical dilatation procedure, doctors use a special device to gently expand the narrowed parts of your esophagus. This method can significantly improve the patient’s condition, and in some cases, it can even reduce the number of diverticula. Diverticula are small pouches that can form along your esophagus. In some situations, this procedure can lead to the complete disappearance of these pouches.
What else can Esophageal Intramural Pseudodiverticulosis be?
There are several other health conditions that can exhibit similar symptoms to diffuse intramural esophageal diverticulosis and can be mistaken for it. These conditions often show up with EIP and are seen as risk factors. They include:
- Gastroesophageal reflux disease (a long-term condition where stomach acid comes up into the esophagus)
- Esophagitis (inflammation that can damage tissues of the esophagus)
- Esophageal stricture (a narrowed esophagus)
- Esophageal cancer
- Motility disorders (problems with the movement of food through the digestive tract)
What to expect with Esophageal Intramural Pseudodiverticulosis
Diverticula, in basic terms, are small pouches that can form in your digestive system. They might not cause problems or have any significant impact on your health, and their presence or absence after treatment doesn’t generally affect your health outcomes. However, these diverticula can often indicate that there may be other underlying health conditions.
Most complications aren’t usually a direct result of having diverticulosis – which is the condition of having these pouches in your digestive system. These complications could be related to similar underlying health issues. Hence, despite the presence of diverticula being generally harmless, it has been found to be associated with esophageal cancer in some cases.
Possible Complications When Diagnosed with Esophageal Intramural Pseudodiverticulosis
The primary complication for patients with widespread esophageal diverticulosis (tiny pouches forming in the esophagus) is the development of an esophageal stricture. This is a narrowing of the esophagus, affecting 76% to 90% of patients. It’s typically found firstly in the upper part of the esophagus, then in its lower part, and finally in the middle.
An increased occurrence of esophageal intramural pseudodiverticulosis (EIP) has also been observed in patients with esophageal cancer. It’s worth noting, though, that the link between EIP and esophageal cancer isn’t definitive and calls for further study. It’s currently undetermined whether EIP causes esophageal cancer, results from it, or if both conditions share the same root causes.
Lastly, a rare but serious complication is the formation of abnormal connections or ‘fistulas’ with the anterior mediastinum (the space in the chest between the lungs).
Most Common Complications:
- Esophageal stricture
- Increased prevalence of EIP in patients with esophageal cancer
- Fistula formation with the anterior mediastinum
Preventing Esophageal Intramural Pseudodiverticulosis
Diffuse intramural esophageal diverticulosis is a less-known medical condition, but doctors keep it in mind when working out why a patient is having trouble swallowing. On a related note, it’s important for anyone to understand that difficulty swallowing is not a normal occurrence and it’s always a good idea to reach out to a doctor or healthcare professional as soon as this happens.
