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CT Findings of Diffuse Esophageal Spasm: Case Report

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Diffuse esophageal spasm (DES) is characterized by substernal chest pain, dysphagia, and a manometric pat- tern of frequent simultaneous contractions with inter- mittently normal peristalsis (1). Most patients with DES show abnormal esophageal motility at esophagography, although the findings are nonspecific and require clini- cal and manometric correlation (1). We report a case of DES in which CT demonstrated relatively long, sym- metric, segmental esophageal wall thickening and an epiphrenic esophageal diverticulum.

Case Report

A 62-year-old man who for several years had suffered from intermittent chest pain presented with dysphagia, first experienced two months earlier. Chest CT of the esophagus, demonstrated diffuse circumferential wall thickening of the distal third of the esophagus, from the carina to the gastroesophageal junction (Fig. 1A-C). The long segment of the esophagus was involved, though

periesophageal fat was normal. At the level of the hepat- ic dome, double intraluminal air densities were present (Fig. 1b), and at the lower level, adjacent to the esopha- gus, an air cavity thought to be an esophageal diverticu- lum was noted (Fig. 1C). Subsequent esophagography revealed an intermittent absence of primary peristalsis, but prominent tertiary activity (Fig. 1D) which involved the distal one third of the esophagus. No mucosal lesion or mass was found, but an epiphrenic diverticulum was present (Fig. 1d). Similarly, endoscopy revealed no mu- cosal lesion, but esophageal manometry demonstrated long duration and high amplitude spontaneous esophageal contraction, consistent with a diagnosis of DES.

Discussion

DES is characterized by intermittent abnormal esophageal motility, chest pain, and dysphagia. The chest pain is usually spontaneous, not related to swal-

J Korean Radiol Soc 2004;50:115-117

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CT Findings of Diffuse Esophageal Spasm:

Case Report

1

Sung Bin Park, M.D.1, 2, Koun-Sik Song, M.D.2, Joon Beom Seo, M.D.2, Jin Seong Lee, M.D.2, In Sun Lee, M.D.2, Kyung-Hee Lee, M.D.2

1Department of physical examination for conscription, Seoul Regional Military Manpower Office

2Department of Radiology, Asan Medical center, University of Ulsan, College of Medicine Received May 24, 2003 ; Accepted October 17, 2003

Address reprint requests to : Sung Bin Park, M.D., Department of physical examination for conscription, Seoul Regional Military Manpower Office.

159-1 Singil-dong, Yeongdeungpo-gu, Seoul 150-057, Korea.

Tel. 82-2-820-4552 Fax. 82-2-820-4548 E-mail: [email protected]

We report the CT findings of diffuse esophageal spasm (DES) in a patient with dys- phagia. Although an uncommon condition, DES should be included in the differential diagnosis if relatively long and symmetric segmental esophageal wall thickening and an epiphrenic esophageal diverticulum are noted at CT.

Index words :Esophagus, abnormalities Esophagus, motility

Computed tomography (CT)

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lowing, and can simulate angina. The degree of dyspha- gia varies, and is not always associated with chest pain.

A diagnosis of DES is based on manometric findings of long duration and high amplitude, during simultaneous contraction, more than 10% of initiated swallows and intermittently normal primary peristalsis (1). The patho- genesis of the disorder has not been clearly defined, but appears to reflect an imbalance between excitatory and inhibitory neural pathways affecting esophageal smooth

muscle. Pathologically, the esophageal musculature may be relatively normal or markedly thickened due to hypertrophy. If this has occurred, the circular layer of muscularis propria is most often affected. Unlike achala- sia, in which they are reduced or absent, ganglionic cells are invariably preserved (2).

Esophagography after barium swallowing in patients with DES may reveal normal or nonspecific findings such as disruption of primary peristalsis and prominent

Sung Bin Park, et al: CT Findings of Diffuse Esophageal Spasm

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A B

C

D Fig. 1. A. CT scan obtained at subcarinal level shows circumferential esophageal wall thickening with preservation of adjacent soft tissue plane. Dilatation of prox- imal esophagus was also noted (not shown).

B. CT scan at hepatic dome level shows double intraluminal air densities (arrow- heads), which is thought to be folding of the thickened esophageal wall.

C. CT scan obtained at gastroesophageal junction level shows exophytic air den- sity due to epiphrenic esophageal diverticulum (arrow).

D. Double contrast esophagogram shows proximal dilatation and “cork screw”

appearance due to prominent tertiary wave without mucosal abnormality (ar- rows). Also noted is epiphrenic diverticulum at gastroesophageal junction (arrow- heads). Primary peristalsis was not seen on fluoroscopic evaluation after barium swallow (not shown).

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tertiary activity. The contractions are usually repetitive and simultaneous, causing the typical “cork screw”or

“curling”appearance (3). Coexisting diverticula sec- ondary to marked increased in intraluminal pressure, as in our case, are not uncommon. The reported radiologic sensitivity of esophagography in the diagnosis of DES is about 75%, though with careful fluoroscopic observa- tion of five single swallows, the detection rate would probably be improved (4).

The esophagus is easily evaluated by CT, a sensitive method of demonstrating esophageal abnormalities, in- cluding esophageal wall thickening, if careful attention is paid (5). For optimal study conditions, intravenous contrast material should be administered, since this bet- ter defines the tissue planes. The thickness of the esophageal wall depends on the degree of esophageal distention, and should normally not exceed 3-5 mm (5-7). Thickening is a nonspecific response to a variety of esophageal conditions, and although these can be classified into patterns and evaluated for length of esophageal involvement, it has still not usually been possible to conclusively identify the cause of wall thick- ening on the basis of CT appearance alone (5). Benign or malignant tumors of the esophagus are usually manifest- ed at CT as focal, asymmetric thickening of the esophageal wall (5, 7), while wall thickening in esophagitis has been shown to be concentric and cir- cumferential, involving a relatively long segment of the esophagus in 75% of patients (8).

Nino-Murcia et al. (3) reported that the CT findings of DES in three patients were smooth, symmetric, circum- ferential wall thickening of the distal two-thirds of the esophagus, with normal periesophageal fat. While DES is a benign condition that is not dissimilar to esophagitis,

certain findings are indicative of DES. In our case, an epiphrenic esophageal diverticulum was detected at CT, as well as relatively long, symmetric, segmental esophageal wall thickening. In addition, the esophageal lumen contained double intraluminal air densities, a finding thought to be due to folding of the thickened esophageal wall.

We suggest that if CT demonstrates relatively long, symmetric, segmental esophageal wall thickening and an epiphrenic esophageal diverticulum in patients with chest pain or dysphasia, DES should be included in the differential diagnosis and further evaluation such as esophagogrphy and/or manometry is required.

References

1. Chen YM, Ott DJ, Hewson EG, et al. Diffuse esophageal spasm:

radiographic and manometric correlation. Radiology 1989;170:807- 810

2. Enterline H, Thompson J. Motility disorders of the esophagus and spontaneous rupture of the esophagus. In: Enterline H, Thompson J, eds. Pathology of the esophagus New York: Springer-Verlag, 1984:

59-62

3. Nino-Murcia M, Stark P, Triadafilopoulos G. Esophageal wall thickening: a CT finding in diffuse esophageal spasm. J Comput Assist Tomogr 1997;21(2):318-321

4. Ott DJ, Chen YM, Hewson EG, et al. Esophageal motility: assess- ment with synchronous video tape fluoroscopy and manometry.

Radiology 1989;173:419-422

5. Reinig JW, Stanley JH, Schabel SI. CT evaluation of thickened esophageal walls. AJR Am J Roentgenol 1983;140(5):931-934 6. Halber MD, Daffner RH, Thompson WM. CT of the esophagus: I.

Normal appearance. AJR Am J Roentgenol 1979;133(6):1047-1050 7. Desai RK, Tagliabue JR, Wegryn SA, Einstein DM. CT evaluation

of wall thickening in the alimentary tract. Radiographics 1991;11(5):771-783

8. Berkovich GY, Levine NS, Miller WT, Jr. CT findings in Patients with Esophagitis. AJR Am J Roentgenol 2000;175:1431-1434 J Korean Radiol Soc 2004;50:115-117

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대한방사선의학회지 2004;50:115-117

미만성 식도 연축의 CT 소견: 증례 보고1

1서울지방 병무청 징병검사과

2서울아산병원 방사선과

박성빈1, 2・송군식2・서준범2・이진성2・이인선2・이경희2

연하곤란을 보인 미만성 식도 연축(Diffuse esophageal spasm)환자의 CT 소견을 보고하고자 한다. 미만성 식도 연축은 드문 증례이나 CT 소견상 긴 분절에 걸쳐 대칭적 식도 벽의 비후와 동반된 식도게실이 있다면 미만성 식 도 연축이 감별진단으로 포함되어야 할 것이다.

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