The Pulse | Thursday, October 24, 2019
The Unpopular Esophagus: From ‘Tics’ to ‘Sword Throats’
At first glance, the esophagus seems primitive, but with a closer inspection and deeper understanding of its physiology, it truly is a magnificent organ. More “popular” disease states such as GERD, Barrett’s Esophagus and achalasia garner all the attention. However, several less common conditions deserve equal attention, as they will surely cross paths with true “esophagologists.”
Diverticula (or “tics”) are outpouchings of the esophagus, caused by a primary motility disorder or an abnormality of the upper esophageal sphincter (UES) or lower esophageal sphincter (LES). In 1877, Friedrich Albert von Zenker, a German pathologist, first described the most common diverticulum, arising from the cervical esophagus. Many diverticula are asymptomatic, but when bothersome, patients may complain of dysphagia, chest pain and regurgitation of undigested food. An esophagram will make the diagnosis, and surgical excision with an esophagomyotomy is the treatment of choice.
Vascular rings and slings are congenital anomalies of the great vessels that cause compression of the esophagus. Dysphagia lusoria (mild dysphagia to solids, but not liquids) is common, and an esophagram or angiography will confirm the diagnosis. Surgical repair via a median sternotomy and cardiopulmonary bypass is nearly 100% successful.
Esophageal rings, first described by Richard Schatzki in 1945, are characterized by a concentric symmetrical narrowing found at the squamocolumnar junction. Characterized by esophageal mucosa above and gastric mucosa below, they are responsible for episodic aphagia. This is the abrupt onset of dysphagia causes by the intermittent obstruction of the nondistensible ring by solid food (aka the “Steakhouse syndrome”). An esophagram or an esophagogastroduodenoscopy (EGD) will often make the diagnosis, and treatment is bougienage if symptomatic.
Esophageal webs are thin membranous structures consisting of both squamous epithelium both above and below the web, distinguishing them from a Schatzki’s ring. They cause partial or complete obstruction and are found throughout the esophagus. They may be congenital or acquired, and dysphagia is the most common symptom. Diagnosis starts with an esophagram first, followed by an EGD. Treatment depends upon the nature of the web (thin vs. thick). Options include bougienage, piecemeal excision with biopsy forceps, laser lysis, balloon dilatation and surgical mucosal resection.
Benign tumors represent less than 1% of all esophageal neoplasms, with leiomyomas being the most common (60%). These are intramural, solitary lesions that are classified as a Gastrointestinal stromal tumor (GIST). Most are asymptomatic with a characteristic appearance on an esophagram. Upper endoscopy reveals a smooth, well-defined, noncircumferential mass. If suspected, it is important to avoid biopsy as this hinders surgical enucleation.
Foreign bodies are no stranger to the esophagus, especially in the pediatric population. Seventy percent of foreign bodies will be found at the level of the UES or thoracic inlet. Fifteen percent will be located at the level of the mid-esophagus just above the aortic notch. The remaining 15% will be found in the distal esophagus just above the LES. Emergent endoscopy is indicated in cases of obstruction or the presence of disk batteries or sharp objects. Urgent endoscopy should be performed for partial food impaction, magnets and non-sharp objects. Coins in the esophagus may be observed for up to 24 hours if asymptomatic.
Sword swallowing dates back over 4,000 years, and the first ever EGD was performed in a sword swallower in 1868. Not for the faint of heart, it takes from 3 to 10 years to learn how to swallow a single sword, and at least 29 deaths have been reported from sword swallowing injuries. Side effects range from “sword throats” and chest pain, to esophageal perforation and intestinal bleeding requiring transfusion. Cost of medical care for these injuries can exceed $50,000. Ironically, most sword swallowers return to work following a brush with death as there is “too much unfinished business.”
The disease states and occurrences listed above are just a brief look at some of the uncommon and even unusual anomalies of the esophagus. With a better understanding of these entities, may your appreciation and curiosity for this wonderful organ continue to grow with enthusiasm.
Dr. Nikolai A. Bildzukewicz is an assistant professor of clinical surgery at the Keck School of Medicine of USC. He is also the associate program director for the general surgery residency, as well as the advanced GI/MIS fellowship. Originally from Pennsylvania, he obtained his medical degree from Jefferson Medical College in Philadelphia. Upon graduating from medical school, Dr. Bildzukewicz was commissioned as a lieutenant in the United States Navy. He completed a general surgery internship at the Naval Medical Center San Diego and served several months as a general medical officer (GMO) at Naval Hospital Great Lakes. He reported for training at the Naval Aerospace Medical Institute in Pensacola, FL, graduating as a Naval Flight Surgeon. He spent the next two years on active duty, providing medical care to military pilots, aircrew, and sailors ashore and at sea. In 2005, he returned to Jefferson to complete his general surgery training. In 2012, he completed his fellowship training in minimally-invasive, bariatric, and robotic surgery at the University of California San Diego.
His clinical interests include the diagnosis and treatment of gastroesophageal reflux disease (GERD), hiatal and paraesophageal hernias, and esophageal motility disorders including achalasia. He also focuses on the advanced laparoscopic repair of both inguinal and ventral hernias. His research interests are in advancing minimally-invasive surgical techniques.