A 33-year-old man comes to the office for a routine follow-up visit. He was recently treated for esophageal achalasia with a pneumatic dilation. He reports a significant improvement in symptoms of dysphagia and nocturnal regurgitation. His past medical history is also significant for borderline hypertension and an anxiety disorder. He is an ex-smoker with a 5 pack-year smoking history. His mother suffered from Sjogren's disease and lymphoma. His medications include omeprazole and clonazepam.
His blood pressure is 135/85 mmHg and his pulse is 67/min. His BMI is 30 kg/m². His physical examination is otherwise unremarkable.
Over the next 10 years, the patient is at the highest risk for which of the following?

Question

Quizplus · Accepted Answer

Achalasia is of unknown etiology and involves decreased peristalsis of the distal esophagus with failed relaxation of the lower esophageal sphincter (LES). Patients typically present with dysphagia with solids (91%) and liquids (85%), difficulty belching (85%), and other nonspecific symptoms (40-60%, including weight loss, chest pain, heartburn). Achalasia confers an increased risk for esophageal cancer (usually squamous cell carcinoma). Stasis and fermentation cause mucosal inflammation, epithelial hyperplasia, multifocal dysplasia, and eventual squamous cell carcinoma in some patients. As a result, these patients may need periodic surveillance, although a firm surveillance interval has not yet been established for these patients. (Choice A) Fibrosing mediastinitis is a rare but potentially serious disease caused by long-standing mediastinal inflammation. It involves diffuse fibrosis of the soft tissues of the mediastinum, and it is usually a consequence of prior granulomatous disease (e.g., histoplasmosis, tuberculosis, etc.) or radiation treatment, but not with achalasia. (Choice B) Achalasia does not usually increase the risk of lymphoma. However, non-Hodgkin's lymphoma may lead to pseudoachalasia by causing gastroesophageal obstruction. (Choice C) Patients with achalasia may have or develop other GI motility disorders such as gastroparesis, intestinal pseudoobstruction, etc. Isolated pyloric stenosis is usually either congenital (seen mainly in the first 2 years of life) or secondary to a mucosal disease such as a mass lesion or peptic ulcer. (Choice E) Zenker's diverticulum is a pulsion diverticulum which occurs in the upper esophagus. It is related to cricopharyngeal dysfunction and not achalasia, which affects the LES and not the UES. Educational objective: Patients with achalasia are at an increased risk of subsequent development of squamous cell cancer of the esophagus. __________ References: Long-term risk of esophagitis, Barrett's oesophagus and oesophageal cancer in achalasia patients (http://www.ncbi.nlm.nih.gov/pubmed/16782616)

A 33-Year-Old Man Comes to the Office for a Routine