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Joseph Junewick, MD FACR
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Esophageal Lieomyomatosis

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: 7 months ago
Updated: 7 months ago
Tags: PEDS
Modality/Study Types: CT FL DR
Activities:
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History

Teenage female with dysphagia and diagnosed with achalasia on upper GI. She had a child with neonatal onset of similar symptoms who was diagnosed with esophageal leiomyomatosis and subsequently underwent CT.


Case Images


Diagnosis

Esophageal Leiomyomatosis

Findings

DR – PA chest radiograph shows right paraspinal soft tissue mass.
FL – Esophagram shows concentric narrowing of the esophagus, most pronounced distally.
CT – Marked isodense mural thickening of the esophagus from the thoracic inlet to the cardia of the stomach.

Discussion

Esophageal leiomyomatosis is a benign, neoplastic condition that allows for the proliferation of circular and longitudinal smooth muscle in the distal esophagus. This growth acts to thicken the esophageal wall and may cause corresponding dysphagia, gastroesophageal reflux, peptic stricture, or defects of the gastric fundus. Patients are predominantly pediatric (average age of 11 years, 6 months to adulthood) and present with insidious-onset dysphagia. The inheritance of esophageal leiomyomatosis can be familial or sporadic, and may be seen in individuals with Alport syndrome; genetic defect of collagen IV. Esophageal leiomyomatosis should not be confused with primary achalasia or idiopathic muscular hypertrophy of the esophagus which typically occur in older populations. Malignant tumors may also exhibit similar clinical manifestations. However, symptoms associated with malignant tumors of the esophagus display a more rapid onset. On upper GI, the esophagus is dilated with long segment tapering (as opposed to achalasia which typically involves a short segment). Cross-sectional imaging shows marked concentric mural thickening extending into the cardia of the stomach. There have been no reports of sarcomatous degeneration. Treatment is based on the degree of dysphagia and ranges from medical management to myotomy to esophagectomy/gastroesophagectomy.

Reference

Levine MS, Buck JL, Pantongrag-Brown L, Buetow PC, Lowry, MA, & Sobin, LH. 1996. Esophageal Leiomyomatosis. Radiology. 199(2): 533-536. DOI: 10.1148/radiology.199.2.8668807

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Abigail Rossman



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