Ann Thorac Surg 2006;81:354-356
© 2006 The Society of Thoracic Surgeons
Case report
Dysphagia in the Young Male: The Ringed Esophagus
Michael A. Smith, MD
a
,
G. Alexander Patterson, MD
b
,
*
,
Joel D. Cooper, MD
b
a Department of Cardiothoracic Surgery, University of Southern California, Keck School of Medicine, Los Angeles, California
b Division of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri
Accepted for publication October 8, 2004.
* Address correspondence to Dr Patterson, Division of Cardiothoracic Surgery, Washington University School of Medicine, Washington University Medical Center, Suite 3108, Queeny Tower, One Barnes-Jewish Hospital Plaza, St. Louis, MO 63110-1013 (Email: pattersona{at}msnotes.wustl.edu).
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Abstract
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The endoscopic view of the multi-ringed esophagus readily explains why the term "tracheal esophagus" is applicable. This entity may be undiagnosed until dysphagia and impactions secondary to strictures occur in the young male. Several factors point to a congenital rather than an acquired disorder. Treatment consists of slow, progressive dilatations that are repeated for recurrent dysphagia.
The pathologic findings and clinical course of the multi-ringed, tracheal, or corrugated esophagus are rarely reported in thoracic literature. As a variant of congenital esophageal stenosis, this entity is frequently obscured in discussions of webs, rings (Schatzki's), or distal esophageal stenosis, which may exhibit tracheobronchial remnants and reflux esophagitis.
Ten years ago, a man who is currently 40 years old was transferred to our service having sustained a cervical esophageal perforation during endoscopy for removal of an impacted apple fragment. He gave the classic history of a patient with a ringed esophagus, having had dysphagia since age 8, essentially existing on a soft and liquid diet. He recovered with conservative management and later underwent manometry, which revealed a minimally hypertensive lower sphincter with no motor disorder. A barium study showed only slight narrowing of the gastroesophageal junction with reduced caliber of the entire esophagus. At endoscopy, a stricture was encountered just beneath the cricopharyngeus that initially permitted passage of only an 8-mm Savary dilator (Cook Medical, Bloomington, IN). Careful, progressive dilatation through a 15-mm Savary (Cook Medical) now allowed passage of the pediatric endoscope (Olympus GIF-160; Olympus America, Melville, NY) into the stomach. Upon withdrawal, concentric, multiple, thin, submucosal fibrous rings involving the upper and mid esophagus could be visualized (Fig 1), with linear mucosal lacerations overlying each ring (Fig 2).
The severity of the stricture dictated monthly dilatations until a No. 46 Maloney bougie could be passed, and each dilatation produced broad superficial mucosal lacerations. Since those initial dilatations, there have been no episodes of impaction and the patient now returns for dilatations at 2-year intervals. We are treating this patient's father who has a history of lifelong dysphagia and who has required dilatation of his strictured multi-ringed esophagus. Three other young males with histories of longstanding dysphagia underwent periodic dilatation of their ringed esophagi when symptoms of dysphagia recurred. In the past, one of these individuals had sustained an esophageal perforation during removal of an impacted meat bolus. This patient's father also gave a history of having had solid food dysphagia for many years. Others have reported this finding in father and son, which we believe is further evidence of a congenital factor [1]. In our 5 patients, the mucosa has appeared normal throughout, without showing any evidence of esophagitis. The rigid rings were covered by a taut mucosa and were not obliterated by insufflation of the esophageal lumen. Only 1 of our patients presented with barium studies showing the serrated edges seen with multiple esophageal rings (Fig 3).
The gastroenterologic literature is replete with case reports of the ringed esophagus [1–3], the diagnosis and treatment of which was first reported by Kelly and Frazier [4] in 1966. This is predominantly found in the young male who may recall symptoms dating back to early childhood, and who frequently existed on a modified diet. Being termed a slow eater, forceful regurgitation of an impacted food bolus and prolonged mastication of food are common complaints of the patient with the multi-ringed esophagus.
Our patients, and most of the reported cases having this entity, had no history of heartburn, reflux, or caustic ingestion. Barium study reports are often normal in these individuals; however, double contrast images are more apt to demonstrate the characteristic serrated appearance. Manometry will usually reveal normal findings and rarely helps to make a diagnosis of ringed esophagus, which will often be initially diagnosed by endoscopy. Biopsies of the rings have been variously reported as showing hyperkeratosis, basal cell hyperplasia with elongation of the submucosal papillae, and fibrosis of the submucosa and muscularis lamina propria [5]. These rings should not be confused with esophageal felinization, which is a transitory contraction of the circular esophageal muscle with ridging of the muscularis mucosa. There are those who maintain that basal cell hyperplasia with elongation of the submucosal papillae is indicative of active esophagitis, and some have stated that the multi-ringed esophagus is entirely due to gastrointestinal reflux disease [6]. The presence of scattered mucosal eosinophils is said to be a specific finding in reflux esophagitis, but some pathologists believe this is a nonspecific marker of any inflammatory process. This should raise the question of why women, who have gastrointestinal reflux disease just as often as men do, rarely have a true multi-ringed esophagus.
Certainly in any large series of patients, some will obviously have active esophagitis, which will be readily apparent at endoscopy, but as we also noted, most reports stress the lack of esophagitis in this group of patients. This and other factors make us believe that the rings are not acquired secondary to reflux, but are congenital. To us, a much more plausible answer for the onset of dysphagia in the young male is that the fibromuscular elements of the congenital rings begin to contract, thus giving rise to varying degrees of stricture. Certainly the mucosa overlying these rings is not contributory to any obstruction. The same mucosal microscopic changes that repeatedly appear in case reports may well result from the slow passage of solid food over many years and may be the irritant that gives rise to these findings. One should recall the findings seen in stasis esophagitis of an early, untreated achalasia, which microscopically shows basal cell hyperplasia with elongated papilla. Certainly these finding are not related to gastrointestinal reflux disease.
Occasionally the ringed esophagus may be found in patients having eosinophilic esophagitis [7]. Again, there is a predominance of males with this entity, and an allergic history is commonly present. Eosinophils may be very numerous in the mucosa and may actually infiltrate the entire esophageal wall. Dysphagia is a feature of this disease, but the small caliber esophagus, a long segment stricture, rather than the multi-ringed strictures is more commonly found [8]. The same caveat pertains when first seeing these patients or any patient with the multi-ringed esophagus. Perforation is not an uncommon complication in this group of individuals. It should be stressed that linear mucosal lacerations, or shearing, occur after most dilatations, even though no perceptible resistance to the bougie is encountered. Reintroduction of the esophagoscope should be carried out after every procedure to rule out frank perforation.
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References
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- Harrison CA, Katon RM. Familial multiple congenital esophageal ringsreport of an affected father and son. Am J Gastroenterol 1992;87:1813-1815.[Medline]
- Shiflett DW, Gilliam JH, Wu WC, Austin WE, Ott DJ. Multiple esophageal webs Gastroenterology 1979;77:556-559.[Medline]
- Oh CH, Levine MS, Katzka DA, et al. Congenital esophageal stenosis in adultsclinical and radiographic findings in severe patients. Am J Roentgenol 2001;176:1179-1182.[Abstract/Free Full Text]
- Kelley ML, Frazer JP. Symptomatic mid-esophageal webs JAMA 1966;197:183-186.[Free Full Text]
- Morrow JB, Vargo JJ, Goldblum JR, Richter JE. The ringed esophagushistological features of GERD. Am J Gastroenterol 2001;96:984-989.[Medline]
- Janisch HD, Eckardt VF. Histological abnormalities in patients with multiple esophageal webs Dig Dis Sci 1982;27:503-506.[Medline]
- Fox VL, Nurko S, Furuta GT. Eosinophilic esophagitisit's not just kid's stuff. Gastrointest Endosc 2002;56:260-270.[Medline]
- Vasilopoulos S, Murphy P, Auerbach A. The small-caliber esophagusan unappreciated cause of dysphagia for solids in patients with eosinophilic esophagitis. Gastrointest Endosc 2002;55:99-106.[Medline]
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