A 2-year-old girl is brought to the office for follow-up after recent hospitalization. Two weeks ago, she had abrupt onset of dysphagia, drooling, and vomiting after eating sausage. She underwent urgent esophagoscopy, which showed complete occlusion of the esophageal lumen with impacted food. Impaction was removed and there were no mucosal lesions or strictures at the site of the impaction. Follow-up barium esophagography reveals a deep impression on the posterior aspect of the esophagus at about the level of T4. Recovery was uneventful. The parents report that this is her second episode of food impaction. The patient drinks liquids well but has always had frequent choking with solid foods. Medical history also includes frequent respiratory tract infections, including 2 episodes of middle lobe pneumonia in the last year. Her weight was advancing along the 50th percentile until approximately age 9 months, when her growth began to slow; weight is now at the 15th percentile. On examination, the child appears small for her age. The oral cavity and oropharynx are clear, the lungs are clear, and heart rate is regular with no murmurs. Which of the following is the most likely etiology of this patient's condition?

Question

A 2-year-old girl is brought to the office for follow-up after recent hospitalization.  Two weeks ago, she had abrupt onset of dysphagia, drooling, and vomiting after eating sausage.  She underwent urgent esophagoscopy, which showed complete occlusion of the esophageal lumen with impacted food.  Impaction was removed and there were no mucosal lesions or strictures at the site of the impaction.  Follow-up barium esophagography reveals a deep impression on the posterior aspect of the esophagus at about the level of T4.  Recovery was uneventful.  The parents report that this is her second episode of food impaction.  The patient drinks liquids well but has always had frequent choking with solid foods.  Medical history also includes frequent respiratory tract infections, including 2 episodes of middle lobe pneumonia in the last year.  Her weight was advancing along the 50th percentile until approximately age 9 months, when her growth began to slow; weight is now at the 15th percentile.  On examination, the child appears small for her age.  The oral cavity and oropharynx are clear, the lungs are clear, and heart rate is regular with no murmurs.  Which of the following is the most likely etiology of this patient's condition?

Quizplus · Accepted Answer

11ec25c8_d90d_57f8_bc81_79c8feec21df_MD0003_00 Vascular rings encompass congenital malformations of the aortic arch system that encircle the trachea and/or esophagus and cause compressive symptoms. Tracheal compression leads to biphasic stridor that increases with increased work of breathing (eg, crying, feeding). Vascular rings can also present with esophageal compression symptoms, as in this patient with severe solid-food dysphagia. She was gaining weight well as an infant likely due to being on an all-liquid diet (requiring less esophagus distension). With the introduction of solid foods (around age 6 months), dysphagia became more pronounced and the patient's growth rate slowed due to increasing reliance on solid foods for her caloric needs. She had recurrent food impactions at the site of esophageal compression, seen on fluoroscopic esophagography at around the level of the aortic arch (eg, T3-T4). Her episodes of middle-lobe pneumonia were possibly due to aspiration. CT scan can delineate the anatomy forming the vascular ring and evaluate associated tracheal abnormalities. Due to possible concurrent cardiac and airway abnormalities, patients require direct laryngoscopy, bronchoscopy, and echocardiogram. Treatment is surgical division of the structures creating the ring. (Choice B) Eosinophilic esophagitis can cause dysphagia to solids, repeated food impactions and, potentially, normal esophagoscopy (although mucosa biopsy would show eosinophils). However, posterior esophageal compression is not seen. (Choice C) Primary achalasia is characterized by inflammatory degeneration of esophageal neurons, leading to impaired peristalsis and incomplete relaxation of the lower esophageal sphincter. It can cause regurgitation of undigested food and heartburn. However, dysphagia is typically to both solids and liquids, and esophagography shows esophageal dilation with a "bird-beak" appearance. "bird-beak" appearance (Choice D) Foreign body (eg, coin) ingestion is common in this age group and could cause food impaction. However, esophagoscopy would show the foreign body, and there would be no signs of external compression. (Choice E) Esophageal candidiasis commonly presents with odynophagia and dysphagia to solids. It occurs most often in immunosuppressed individuals and is often concurrent with thrush. White mucosal plaques are seen on esophagoscopy. thrush (Choice F) Esophageal adenocarcinoma typically arises within an area of Barrett esophagus. Although it presents with solid-food dysphagia and weight loss, patients are usually obese adults with gastroesophageal reflux disease. Mucosal changes would be seen on esophagoscopy, and the esophagus would show intraluminal obstruction rather than external compression. Barrett esophagus Educational objective: Vascular rings encircle the trachea and/or esophagus. Compression of the trachea may present with stridor; compression of the esophagus may present with dysphagia, vomiting, or recurrent food impactions. References: Recurrent respiratory tract infections and dysphagia in a child with an aortic vascular ring. (http://www.ncbi.nlm.nih.gov/pubmed/11846259) Vascular rings. (http://www.ncbi.nlm.nih.gov/pubmed/27301603)

A 2-Year-Old Girl Is Brought to the Office for Follow-Up