IEP: Intramural esophageal pseudodiverticulosis

1. Definition

Esophageal pseudocysts are rare, non-epithelial-lined fluid collections that form in the esophageal wall or paraesophageal region, often secondary to trauma, inflammation, or pancreatic disease. Unlike true cysts, they lack an epithelial lining and are typically walled off by granulation tissue or a fibrous capsule.

2. Causes and Etiology

Primary Causes:

Pancreatic pseudocyst extension: Due to close anatomical relationships, pseudocysts originating in the pancreas (particularly from the tail) may extend to the mediastinum or esophageal hiatus, causing compression or adherence to the esophagus.
Esophageal perforation or trauma:

Iatrogenic (e.g., endoscopic instrumentation, surgery)
Traumatic (blunt or penetrating trauma)
Spontaneous (e.g., Boerhaave syndrome)

Post-inflammatory reaction:

Severe esophagitis (infectious, corrosive, eosinophilic)
Chronic gastroesophageal reflux disease (GERD)

Cystic degeneration of esophageal duplication cysts (rarely)
Mediastinal abscesses or hematomas that organize into pseudocystic collections

3. Pathogenesis

The pathogenesis of esophageal pseudocysts involves:

Disruption of tissue planes due to inflammation, trauma, or enzymatic digestion (particularly by pancreatic enzymes).
Leakage of luminal contents (esophageal or pancreatic fluid) into surrounding soft tissue.
Formation of localized walled-off fluid collections composed of fibrin, inflammatory cells, and granulation tissue.
In the case of pancreatic etiology, enzymatic digestion of mediastinal tissue may cause secondary pseudocyst formation that compresses or adheres to the esophageal wall.

Esophageal wall pseudocysts specifically may arise within the muscularis propria or between the muscular and adventitial layers.

4. Epidemiology

Incidence: Extremely rare. True esophageal pseudocysts are seldom reported in the literature.
Age: More common in adults, particularly in the 4th to 6th decades for pancreatic-related causes; traumatic causes may affect younger individuals.
Sex: No definitive sex predilection, though alcoholic pancreatitis-related pseudocysts may show a male predominance.
Risk Factors:

Chronic pancreatitis
Pancreatic trauma
Severe esophageal inflammation
Esophageal instrumentation
Alcohol abuse
Previous mediastinal surgery or radiation

5. Clinical Symptoms

Symptoms vary depending on the size, location, and mass effect of the pseudocyst:

Common Symptoms:

Dysphagia: Due to extrinsic esophageal compression
Odynophagia: If inflammation involves surrounding structures
Retrosternal chest pain
Regurgitation or vomiting
Hoarseness: If the recurrent laryngeal nerve is involved
Cough or dyspnea: Compression of the trachea or bronchi
Fever: Suggests secondary infection or abscess formation

Systemic Symptoms (if secondary to pancreatitis):

Nausea, vomiting, epigastric pain
Weight loss
Elevated serum amylase/lipase

6. Imaging Findings

CT Scan (Contrast-Enhanced CT Chest/Abdomen):

Hypodense, fluid-attenuation lesion adjacent to or within the esophageal wall
No enhancing epithelial lining (key to differentiate from duplication cyst)
May demonstrate communication with the mediastinum or the pancreas
Possible displacement of the esophagus or airway
Secondary signs of pancreatitis (calcifications, ductal dilation, etc.)

MRI:

T1 hypointense, T2 hyperintense cystic lesion
No mural enhancement
Helps differentiate fluid from soft tissue or blood

Endoscopic Ultrasound (EUS):

Hypoechoic, anechoic lesion in the esophageal wall
Lack of epithelial lining or mural stratification
May guide aspiration or biopsy

Esophagography (Barium Swallow):

Extrinsic compression of the esophageal lumen
Smooth impression on the posterior or lateral esophageal wall
Rarely, fistulous communication

PET-CT (in case of suspicion for malignancy):

Pseudocysts are typically non-FDG avid unless infected

7. Treatment

Treatment depends on etiology, symptomatology, and complications.

Asymptomatic Cases:

Observation and follow-up imaging

Symptomatic or Complicated Pseudocysts:

Pancreatic Pseudocyst-Related:

Treat underlying pancreatitis
Drainage (transgastric, transesophageal EUS-guided)
Surgical cystgastrostomy or cystojejunostomy if inaccessible endoscopically

Infectious Pseudocysts:

Broad-spectrum antibiotics
Percutaneous or endoscopic drainage

Esophageal perforation-related:

Conservative management if small and contained
NPO, TPN, IV antibiotics
Surgical repair in severe or non-contained cases

Mediastinal extension:

Mediastinal drainage via thoracoscopy, VATS, or thoracotomy
Esophageal stenting if a fistula is present

8. Prognosis

Prognosis depends on:

Underlying cause
Presence of infection
Patient comorbidities
Promptness of diagnosis and intervention

Favorable Prognosis:

Small, sterile pseudocysts that resolve with conservative care
Effective drainage of pancreatic-associated pseudocysts

Unfavorable Prognosis:

Delay in diagnosis leading to rupture, sepsis, or mediastinitis
Recurrent pseudocyst formation in chronic pancreatitis
Fistula formation or airway compromise

Mortality is rare but may occur in complicated cases involving mediastinitis, sepsis, or hemorrhage (due to vessel erosion).

9. Differential Diagnosis

Esophageal duplication cyst
Bronchogenic cyst
Mediastinal abscess
Lymphangioma
Neuroenteric cyst
Mediastinal hematoma
Neoplastic cystic degeneration (e.g., cystic esophageal carcinoma)

Conclusion

Esophageal pseudocysts are a rare but clinically significant entity that often arise secondary to trauma, esophageal perforation, or pancreatitis. Differentiation from congenital or neoplastic cysts is critical. Imaging plays a central role in diagnosis, with CT and EUS being most informative. Treatment is tailored based on symptoms, complications, and underlying pathology. With appropriate management, the prognosis is generally favorable, although vigilance is required for infectious or obstructive complications.

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Case study: A 54-Year-Old Woman with Dysphagia
Intramural Esophageal Pseudodiverticulosis

History and Imaging Findings

A 54-year-old woman presented with dysphagia and gastroesophageal reflux symptoms, particularly during the ingestion of solid foods.
She did not experience any difficulty swallowing liquids.
At the time of presentation, she was taking a proton pump inhibitor (PPI).
The patient had a long-standing history of ulcerative colitis and had been on multiple immunosuppressive agents.
Esophagographic imaging is provided below.

Quiz 1

How would you describe the abnormal findings?
(1) Small, flask-shaped outpouchings measuring 1–4 mm filled with barium
(2) Small, well-defined ulcerations with the perforated appearance
(3) Large, flat, and shaggy ulcers
(4) Longitudinally elevated mucosal plaques
(5) Cobblestone-like raised plaques
What type of pathology do the esophageal abnormalities reflect?
(1) Ulceration
(2) Plaque
(3) Pseudodiverticulum
(4) Stricture

Explanation: Barium filling defects typically suggest elevated lesions such as plaques, whereas central pooling of contrast material within a cystic structure generally indicates ulceration. However, in this case, the barium is actually filling pseudodiverticula.
Double-contrast studies are superior for diagnosing esophageal dilatation and strictures.
(1) True
(2) False

Explanation: Single-contrast studies are generally more effective for detecting esophageal dilatation and strictures, whereas double-contrast studies provide better evaluation of the mucosal surface.

CT Imaging

Quiz 2

Which imaging modality is the most sensitive for diagnosing this condition?
(1) CT
(2) MRI
(3) Barium esophagography
(4) Upper gastrointestinal series

Explanation: Barium esophagography is more sensitive than endoscopy for diagnosing this condition. CT may only reveal nonspecific findings such as esophageal wall thickening and luminal narrowing.
What is the underlying pathogenesis of these abnormalities?
(1) Virus-related infection
(2) Fungal infection
(3) Corrosive substances or medications causing mucosal injury
(4) Ductal dilatation of the submucosal glands of the esophagus

Explanation: Intramural pseudodiverticulosis arises from the dilatation of excretory ducts of the submucosal glands of the esophagus. The cause of this ductal dilatation remains unclear. It is a rare and benign radiographic finding, seen in approximately 1% of barium esophagograms.
Which of the following is a potential complication of this condition?
(1) Esophageal adenocarcinoma
(2) Stricture
(3) Odynophagia
(4) Infection

Explanation: Strictures are frequently associated with eosinophilic esophagitis (EoE), a chronic inflammatory condition marked by abnormal accumulation of eosinophils in the esophageal mucosa. These strictures are most commonly found in the upper and mid esophagus and can lead to dysphagia, chest pain, and reflux-like symptoms.
Which of the following is another risk factor associated with this condition?
(1) Chronic alcoholism
(2) Ulcerative colitis
(3) Gallstones
(4) Scleroderma

Explanation: Esophageal intramural pseudodiverticulosis (EIP) has been associated with chronic alcoholism in approximately 15% of patients, and with diabetes mellitus, gastroesophageal reflux disease (GERD), and esophageal candidiasis in up to 20% of cases.

Findings and Diagnosis

Findings

Esophagographic Findings:
Primary esophageal peristalsis was preserved. A small hiatal hernia with mild gastroesophageal reflux was noted. Multiple small, flask-shaped outpouchings were observed along the lateral contour of the mid esophagus, consistent with segmental intramural pseudodiverticulosis involving the mid to distal esophagus.

Chest CT Findings:
The distal esophagus demonstrated non-dependent mural thickening measuring up to 6 mm, suggestive of possible esophagitis.

Differential Diagnosis

Infectious esophagitis (e.g., HSV esophagitis typically presents with small ulcerations)
Drug-induced esophagitis
Intramural esophageal pseudodiverticulosis (IEP)
Crohn’s disease (may present with small aphthoid esophageal ulcers)

Final Diagnosis:
Intramural Esophageal Pseudodiverticulosis (IEP)

Discussion

Intramural Esophageal Pseudodiverticulosis (IEP)

Epidemiology
IEP is a rare, benign condition, identified in less than 1% of barium esophagographic studies. It has been associated with chronic alcoholism in approximately 15% of patients, and with diabetes mellitus, gastroesophageal reflux disease (GERD), and esophageal candidiasis in about 20% of cases. The condition exhibits a bimodal age distribution, predominantly affecting adolescents and individuals in their 50s to 60s.

Pathophysiology
IEP results from the dilatation of the excretory ducts of the submucosal glands of the esophagus. The involvement may be segmental or diffuse.

Clinical Presentation
While IEP is frequently asymptomatic, symptomatic patients often present with dysphagia. Stricture-related symptoms may also occur, especially in cases with extensive involvement.

Imaging Features

Barium Esophagography
Barium swallow is more sensitive than endoscopy for detecting IEP.

Classic findings include multiple 1–4 mm, flask-shaped outpouchings aligned longitudinally along the esophagus.
Incomplete filling may falsely suggest a lack of communication with the esophageal lumen; however, in this case, adequate barium coating confirmed continuity with the lumen.
In severe cases, bridging between adjacent pseudodiverticula may be seen. When viewed en face, pseudodiverticula can mimic ulcers.
Segmental involvement of the distal esophagus may be associated with peptic strictures, suggesting a sequela of reflux esophagitis.
Low-density (thin) barium tends to penetrate diverticula more effectively than high-density barium.
Strictures are most commonly found in the upper to mid-esophagus.

Chest CT
CT imaging typically demonstrates esophageal wall thickening, luminal narrowing, and in some cases, intramural gas retention.

Treatment
Most patients do not require specific treatment.

Proton pump inhibitors (PPIs) may relieve symptoms related to coexisting esophagitis.
Endoscopic dilation of strictures can also provide symptom relief.

Proton pump inhibitors (PPIs) reduce gastric acid secretion by inhibiting the hydrogen-potassium ATPase enzyme system of gastric parietal cells. These agents are commonly used for GERD, peptic ulcer disease, gastritis, and Helicobacter pylori eradication. Representative PPIs include omeprazole, lansoprazole, and esomeprazole. While generally safe, long-term use can be associated with adverse effects such as decreased calcium absorption (leading to osteoporosis) and vitamin B12 deficiency.

Complications
Strictures are infrequently observed in IEP but are more likely to occur in the upper or mid-esophagus in patients with radiographically visible intramural tracts. In contrast, those without visible tracts tend to develop strictures in the distal esophagus.
There is also an increased risk of developing esophageal carcinoma.

References

(1) Levine M, Moolten D, Herlinger H, Laufer I. Esophageal intramural pseudodiverticulosis: A reevaluation. AJR Am J Roentgenol. 1986;147(6):1165-1170. doi:10.2214/ajr.147.6.1165.

(2) Chon YE, Hwang S, Jung KS, et al. A case of esophageal intramural pseudodiverticulosis. Gut Liver. 2011;5(1):93-95. doi:10.5009/gnl.2011.5.1.93.

(3) Brühlmann WF, Zollikofer CL, Maranta E, et al. Intramural pseudodiverticulosis of the esophagus: Report of seven cases and literature review. Gastrointest Radiol. 1981;6(1):199-208. doi:10.1007/BF01890250.

(4) Luedtke P, Levine MS, Rubesin SE, Weinstein DS, Laufer I. Radiologic diagnosis of benign esophageal strictures: A pattern approach. Radiographics. 2003;23(4):897-909. doi:10.1148/rg.234025717.

(5) Canon CL, Levine MS, Cherukuri R, Johnson LF, Smith JK, Koehler RE. Intramural tracking: A feature of esophageal intramural pseudodiverticulosis. AJR Am J Roentgenol. 2000;175(2):371-374. doi:10.2214/ajr.175.2.1750371.

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