Congenital esophageal stenosis

Digestive Diseases and Sciences, Vol. 35, No. 2 (February 1990), pp. 263-266

CASE REPORT

Congenital Esophageal Stenosis A Rare Cause of Food Impaction in the Adult MAJOR PETER R. McNALLY, DO, CAPTAIN EDWARD H. COLLIER, III, MD, CAPTAIN MARK C. LOPIANO, MD, L I E U T E N A N T C O L O N E L THOMAS G. BREWER, MD, and C O L O N E L ROY K. H. WONG, MD KEY WORDS: esophagus; stenosis; congenital anomalies.

Esophageal stenosis is a rare congenital anomaly (1 per 25,000 live births), which usually presents during infancy (1). The stenosis is typically aperistaltic, and the onset of symptoms depends upon the degree of deformity. In severe stenosis, symptoms of vomiting or regurgitation occur at birth or with the introduction of solids (2, 3). In mild cases, patient adaptation may delay the diagnosis (1, 4). We report an unusual case of congenital esophageal stenosis, where abnormal peristalsis was preserved within the stenotic segment and presentation as acute food impaction was delayed until adulthood. Endoscopic, radiographic, and esophageal manometric findings of congenital esophageal stenosis are described, and the therapeutic alternatives discussed. CASE REPORT A 22-year-old white male was admitted to the Eisenhower Army Medical Center with a food impaction. Severe retrosternal chest discomfort occurred after a hurried meal of rice, vegetables, and cashew nuts. The patient's first recollection of "choking" occurred at about 8 years of age. These episodes would occur four to six times per month, predictably with consumption of meats and raw vegetables Manuscript received March 30, 1989; revised manuscript received October 17, 1989; accepted October 20, 1989. From the Gastroenterology Service, Department of Medicine, and Department of Radiology, Eisenhower Army Medical Center, Augusta, Georgia 30905-5100; and Gastroenterology Service, Department of Medicine, Walter Reed Army Medical Center 20907-5100. The opinions and assertions contained herein are the private ones of the authors and are not to be construed as official policy or reflecting the views of the Army or the Department of Defense. Address for reprint requests: Major Peter R. McNally, DO, Division of Medicine, Walter Reed Institute of Research, Walter Reed Army Medical Center, Washington, DC 20307-5100.

or hurried meals. These episodes of probable food impactions were relieved with repetitive forced swallows of liquids or self-induced vomiting. Since the patient was otherwise healthy and choking episodes were transient and clearly associated with dietary indiscretion or hurried meals, he was not sent for medical evaluation by his parents. At 19 years of age the patient experienced a prolonged episode of food impaction (4 hr), after a hurried meal. While awaiting emergency room evaluation, the impaction spontaneously passed and he did not pursue further medical attention, but rather resumed his judicious eating habits. Past medical history was remarkable for an inguinal herniorrhaphy and that the patient had poor dentition as a child and was edentulous by 18 years of age. Only after enlistment in the United Stated armed forces was dental care available to the patient. When complete upper and lower dentures were provided, swallowing improved and led to a 20 lb. weight gain. Physical examination during this hospitalization was unremarkable, and roentgenogram of the chest and screening laboratory tests including hemogram, urinalysis, and SMA-20 were normal. When the patient presented to our institution with acute dysphagia, a limited barium esophagram was done and showed a stenotic esophagus with a food bolus trapped at the level of the diaphram (Figure 1A). Administration of glucagon 0.5 mg intravenously did not relieve the impaction. Fiberoptic endoscopy with a smaller-diameter endoscope (Olympus OES-XP10, 7.9 mm OD) revealed the cervical esophagus from 18 to 25 cm to be dilated. Below this level the esophagus conically tapered into a stenotic centrally located lumen. The endoscope was passed through the stenosis against resistance causing a long linear mucosal tear (Figure

Digestive Diseases and Sciences, Vol. 35, No. 2 (February 1990)

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M c N A L L Y ET A L

Fig 1. Barium esophagram showing (A) the cashew nut impacted at the level of the diaphragmatic hiatus and (B) proximal esophageal dilatation with long smooth stenosis below.

2). Esophageal mucosa was otherwise normal in appearance with a distinct squamocolumnar junction located at the diaphragmatic hiatus. A 10-mm cashew nut was retrieved with a polypectomy snare. An upper gastrointestinal series confirmed the endoscopic findings of an abnormal esophagus (Figure 1B). Esophageal peristalsis appeared normal during wet swallows of barium. The duodenal cloop and terminal ileum were normally located, and there was no evidence of malrotation or duplication. Esophageal manometry was done using a lowcompliance water perfused system (Arndorfer Specialties, Inc.). The mean lower esophageal sphincter (LES) pressure was elevated at 41 mm Hg and LES relaxation was normal. Esophageal motility was abnormal (Figure 3). Each 5-cc wet swallow was followed by a prolonged, 9-sec, "doublehumped" contraction wave, with a mean pressure of 80 mm Hg. The first peak occurred simultaneously throughout the esophagus and probably rep-

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resented esophageal noncompliance, while the second peak occurred sequentially throughout the esophagus and represented esophageal peristalsis. Maloney dilators were used to dilate the esophagus. Starting with a 26 Fr, three progressively larger sized dilators were passed during four sessions over a three-week period. Moderate chest pain was experienced after each dilation and was treated with nifedipine 10 mg sublingual. Esophageal dilatations were stopped at a maximum of 36 Fr, and the patient has enjoyed improved swallowing without further episodes of dysphagia during a five-month follow-up period.

DISCUSSION The esophagus develops from the embryonic foregut by the end of the first fetal month (5). Between the 7th and 10th fetal weeks, vacuoles in the esophagus coalesce, and a lumen is formed (6). Intrauterine anoxia or stress during this period is felt to Digestive Diseases and Sciences, Vol. 35, No. 2 (February 1990)

CONGENITAL ESOPHAGEAL STENOSIS

Fig 2. Endoscopic photograph showing the mucosal tear beginning at the inlet of'the esophageal stenosis.

cause the defective cannulation of the esophageal lumen, leading to esophageal stenosis (2). There are basically three types of congenital esophageal stenosis: esophageal web, segmental stenosis composed of tracheobronchial elements (7, 8), and an esophageal muscular ring composed of collagenous scar (1) or maldeveloped muscular mucosae replaced by haphazardly arranged circular muscle fibers (9, 10). The stenosis is typically found in the middle third of the esophagus, is 1-2 cm in length, and is aperistaltic (1, 2). In the patient described in this report, esophageal peristalsis was preserved within a long stenotic segment, which is one possible explanation for his delayed presentation. The patient's poor dentition may have also contributed to the delay in diagnosis, as he was unable to eat large quantities of solid food prior to acquiring dentures. Esophageal stenosis can be diagnosed by radiologic and/or endoscopic examination: a dilated portion of the esophagus is seen above a narrowed segment of variable length. The picture differs from that of a caustic stricture, in that the stenotic opening leading from the dilated portion of the Digestive Diseases and Sciences, Vol. 35, No. 2 (February 1990)

esophagus usually leaves centrally, whereas the opening of a caustic stricture is often eccentrically placed (4). Also, multiple esophageal stenoses and mucosal irregularities are more typical of caustic strictures (1, 11, 12). The importance of thorough mastication by patients with esophageal stenosis is emphasized in this patient who, edentulous in his teens, gained 20 lb with dentures. Bulimic/anorectic patients suffer from perimylolysis and periodontal disease due to long-standing regurgitation (13). A similar dental injury is seen in this patient, as food impactions were relieved by self-induced vomiting since childhood. Muscular congenital esophageal stenosis has been successfully treated with Hurst, Maloney, and Tucker dilators (1, 4), as well as surgical resection (14). In this patient the Maloney-type dilator proved to be ideal, even at small French sizes. The proximal conically tapered stenosis guided the tip of the dilator, and no misdirection or buckling occurred. Most typically dilation to a lumen size of 38 Fr is attempted. In this patient dilatations were stopped at 36 Fr as the risk of

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M c N A L L Y ET AL SUMMARY W h e n e s o p h a g e a l p e r i s t a l s i s is p r e s e r v e d , the presentation of congenital esophageal stenosis may b e d e l a y e d u n t i l a d u l t h o o d . Serial M a l o n e y dilat i o n s are a safe a n d effective m e t h o d o f t r e a t i n g muscular congenital esophageal stenosis.

REFERENCES

Fig 3. Esophageal manometry. Simultaneous esophageal pressure tracing of wet swallows (WS) recorded at the level of the lower esophageal sphincter (LES) and at three orifices placed at 5-cm intervals proximally. The timing of each WS was recorded with a pneumatic neck collar. Paper speed: 1 mm/min; GBL: gastric baseline. p e r f o r a t i n g this l o n g e s o p h a g e a l s t e n o s i s w i t h furt h e r d i l a t i o n w a s felt to b e g r e a t e r t h a n t h e likelihood of recurrent food impaction.

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1. Bluestone CD, Kerry R, Sieber WK: Congenital esophageal stenosis. Laryngoscope 79:1095-1101, 1969 2. Dominguez R, Zarabi M, OH KS, Bender TM, Girdany BR: Congenital esophageal stenosis. Clin Radiol 36:263-266, 1985 3. Boyle JT: Congenital disorders of the esophagus. In Diseases of the Esophagus. S Cohen, RD Soloway (eds). New York, Churchill Livingstone, 1982, pp 97-120 4. Holinger PH, Schild JA: Anomalies of the esophagus. In Gastroenterology, 3rd ed. HL Bockus (ed). Philadelphia, WB Saunders, 1974, pp. 183-190 5. Arey LB: The digestive tube and associated glands. In Developmental Anatomy, 7th ed. LB Arey (ed). Philadelphia, WB Saunders, 1974, pp 245-248 6. Greenough WG: Congenital esophageal strictures. Am J Roentgenol 92:994-999, 1964 7. Anderson GS, Schakelford GD, Mancilla-Jimenez R, McAIister WH: Cartilaginous esophageal ring: A cause of esophageal stenosis in infants and children. Pediatr Radiol 108:665-666, 1973 8. Fonkalsrud EW: Esophageal stenosis due to tracheobronchial remnants. Am J Surg 124:101-103, 1972 9. Groote AD, Laurini RN, Polman HA: A case of esophageal stenosis. Hum Pathol 11:1170-1171, 1985 10. Heymann MB, Berquist WE, Fonkalsurd EW, Lewin KJ, Ament ME: Esophageal muscular ring and the Vacteral association: A case report. Pediatr 67:683-686, 1981 11. Cello JP, Fogel RP, Boland R: Liquid caustic ingestion-spectrum of injury. Arch Intern Med 140:501-504, 1980 12. Tucker JA, Yarington CT: Treatment of caustic ingestion. Otolaryngol Clin North Am 12:343-350, 1979 13. Abrams RA, Ruff JC: Oral signs and symptoms in the diagnosis of bulimia. J Am Dent Assoc 113:761-764, 1986 14. Aprigliano F: Esophageal stenosis. Ann Otol 89:391-396, 1980

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