Steakhouse Syndrome in Myotonic Dystrophy

Area of Science:

• Gastroenterology
• Neurology
• Genetics

Background:

• Myotonic dystrophy (MD) is a multisystem disorder affecting various organs, including the gastrointestinal tract.
• Gastrointestinal dysmotility is a known complication of myotonic dystrophy, potentially impacting esophageal function.

Observation:

• A 70-year-old male patient with myotonic dystrophy presented with severe vomiting and reduced food intake.
• Diagnostic evaluation revealed an acute esophageal obstruction caused by a large bolus of impacted steak, a condition termed 'steakhouse syndrome'.

Findings:

• The esophageal food impaction in this MD patient is hypothesized to be linked to underlying esophageal functional changes secondary to the disease's gastrointestinal pathology.
• Endoscopic intervention successfully removed the impacted food bolus, leading to complete resolution of the patient's symptoms without recurrence.

Implications:

• This case highlights 'steakhouse syndrome' as a potential, albeit uncommon, gastrointestinal manifestation in patients with myotonic dystrophy.
• Clinicians should consider esophageal food impaction in the differential diagnosis for patients with myotonic dystrophy presenting with upper gastrointestinal symptoms.
• Early recognition and endoscopic management are crucial for favorable outcomes in MD patients experiencing food bolus obstruction.