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Downhill Azygos Vein Secondary to Occlusion of the Superior Vena Cava in Behcet's Disease
Downhill Azygos Vein secondary to Occlusion of the Superior Vena Cava in Behcet's Disease* Minoru Suk igara, M.D .; Shinichi Takamoto, M.D .; and Ryozo Omoto , M.D .
A patient with downhill azygos venous 80w is reported. This reverse 80w was due to an obstruction in the superior vena cava and the azygos entry due to Beheets disease. Color 80w mapping of the azygos vein, using transesophageal real-time two-dimensional Doppler echography, was used for both anatomic and hemodynamic analysis. (Chest 1988;
94:1308.(9)
D
own hill esophageal varices and a downhill azygos vein, phenomena which occur when the superior vena cava is occluded, are collaterals that drain the venous flow from the head, neck, upper extremities and thorax. In the past, angiography and computed tomography (Cf) have been the main methods for the assessment of collaterals, I but we employed transesophageal real-time two-dimensional Doppler echography (TE2DD), a newly developed ultrasound method .' This report presents the first published case of downhill azygos vein that has been demonstrated b y TE2DD. CASE REPORT A 44-year-old man wasadmitted in to the Saitama Medical School Hospital suffering from Behcets disease with dyspnea on exertion and edema of the face. Two years before admission, he had iridocyclitis and oral ulceration. Right external jugular venography showed complete obstruction of the innominate and subclavian veins and the superior vena cava (SVC). Many downhill collateral veins were noted in the upper thorax instead of SVC (Fig 1). As shown in the lateral view, these collateral veins joined the azygos vein (Fig 2). Intercostal veins (asterisks) , draining into the azygos vein at almost a right angle, were also visualized. Of those, the uppermost one, which was thickest, was thought to be the supreme intercostal vein. The CT scanning, obtained at a level just below the tracheal carina, showed a thick azygosvein anterior to the vertebrae or posterior to the right side of the esophagus (data not shown). Endoscopic examination revealed small nonhazardous esophageal varices in the upper third of the esophagus. The TE2DD was used to visualize the collateral veins. The ultrasonic apparatus used was a 5 MHz, endoscopic convex array transducer integrated into the end of a Bexibleinsertion tube .' The scan direction was perpendicular to the insertion tube. The examination took about ten minutes. With regard to color display, the blood Bowapproaching the transducer was displayed in warm, reddish colors, while the Bowaway from it was shown in blue. The brightness varied with the Bow velocity. Thirty centimeters from the incisors, we could see the thick azygos vein posterior to the esophagus (Fig 3, left). The azygos vein was displayed in dark blue, while the thick intercostal veins, spouting into the azygos vein, are seen in bright red , the brightness reBecting the narrow angle between the ultrasound beam and the intercostal veins. Figure 3, right, is the schema of left. The direction of the azygos venous Bow wascaudad, the opposite of the normal. The uppermost and thickest *From the First Department of Surgery, Saitama Medical School, Moroyama-machi, lruma-gun, Saitama, Japan. Reprint requests : Dr. Sukigawa, 1st Department ofSurgery, Saitama Medical School , Moroyamamachi , lruma-gun, Saitama-kun, Japan
350-04
1308
FIGURE 1. Right external jugular venogram displaying obstruction of both the innominate vein and the superior vena cava and welldeveloped collateral vessels, anteroposterior view. intercostal vein was thought to be the supreme intercostal vein. The esophageal varices were not observed by TE2DD. DISCUSSION Downhill azygos vein is a result ofocclusion ofthe superior vena cava. The diseases which may result in such obstruction include bronchogenic carcinoma, thyroid gland enlargement, mediastinal fibrosis," and Behcets disease .' When the superior vena cava is occluded, blood from the upper extremities, head, and thorax must flow into the right atrium through collateral veins. The main collateral routes are the internal mammary, lateral thoracic, vertebral, and esophageal veins, communicating with the azygos vein or the inferior vena cava, and thereby, circumventing the obstructed superior vena cava .'.6 In the present case, the superior vena cava was obstructed in its entire course, including the azygos entry. However, the esophageal varices were confined to the upper third of the thoracic esophagus. This was, perhaps, due to the well-developed azygos venous
FIGURE 2. External jugular venogram showing the thick azygos vein, into which the intercostal veins (asterisks) drain. (Lateral view.) Downhill Azygos Vein (SukigarB, Takamoto, Omoto)
Oral
Anal
FIGURE 3. Color flow mapping of the azygos vein and the intercostal veins joining the former. Left, echogram taken 30 cm from the incisors. Right, the schema of left.
system, through which the venous flow of the upper half of the body drained into the inferior vena cava. In the past, evaluation of collateral vessels was commonly performed by angiography and CT. Transcutaneous ultrasonography is not capable of demonstrating the collateral veins around the esophagus because of intervening bone and intrapulmonary air. However, in TE2DD, the distance between the transducer and these vessels is reduced, and there are fewer obstacles, and so, visualization is easier. Moreover, with TE2DD , both anatomic and hemodynamic information can be obtained simultaneously. If a facility for a fast Fourier transform were incorporated in the ultrasound scanner, a quantitative evaluation of flow would also be possible. Although there are some limitations such as the small field of view and the long learning period required for the operator to be able to recognize the anatomy and interpret the ultrasound images, TE2DD is an extremely useful method for the study of collaterals and other vessels in the vicinity of the esophagus. REFERENCES
2
3
4 5 6 7
Hirose J, Takashima T, Suzuki M, Matsui 0 : Downhill varices of the esophagus demonstrated by dynamic computed tomography. J Com put Assist Tomogr 1984; 8:1007-09 Sukigara M, Komazaki T, YamazakiT, Ansai H, Koyama I, Omoto R. Colour flow mapping of oesophagogastric varices and vessels in and around the liver with trans-oesophageal real -time twodimensional Doppler ultrasound. Clin Radiol 1987; 38:487-94 Sorokin JJ, Levine SM, Moss EG, Biddle CM . Downh ill varice s: report of a substernal thyroid gland . Gastroenterology 1977; 73:345-48 Rosenthal T, Halkin H , Shani M, Deutch V. Occlusion of the graft veins in the Behcet syndrome. Angiology 1972; 23:600-05 Johnson LS, Kinnear DG , Brown RA, Mulder DS. Downhill esophageal varices. Arch Surg 1978; 113:1463-64 Felson B, Lessure AP. Downhill varices of the esophagus. Dis Chest 1964; 46:740-46 Miura K, Omoto R. Cautions for clinical use of the instrument. In: Omoto R, ed . Color atlas of real-time two-dimensional Doppler echocardiography, 2nd ed. Tokyo: Shindan-To-Chiryo Co, 1987:29-36
Epithelial Necrosis and Alveolar cOllarse in the Pathogenesis of Usua Interstitial Pneumonla* Jeffrey L. Myers, M.D ., F.G.G.P.; and Anna-Lu/se A Katzenstein, M .D.
We report ultrastructural evidence of epithelial necrosis and alveolar coUapse in a patient with usual interstitial pneumonia (UIP~ These changes were focal and confined to smaU areas characterized histologicaUy by aggregates of interstitial 6broblasts embedded within a myxoid stroma (6broblastic foci), UltrastructuraUy, the denuded epithelial basal lamina in these areas showed deep infoldings into the interstitium, and the luminal surfaces of the resultant clefts often were re-epithelialized. These findings suggest that the 6broblastic foci commonly seen in UIP represent sites of acute lung injury, and that alveolar coUapse foUowing epithelial necrosis is an important mechanism of lung remodeling. In addition to new insights regarding the pathogenesis of 6brosis in UIP, these observations may have important implications for assessing prognosis and selecting treatment strategies. (Cheat 1988; 94:1309-11)
T
he pathogenesis of interstitial fibrosis in usual interstitial pneumonia (UIP) has been the subject of extensive investigation.' Several studies have suggested that UIP begins as alveolitis, and that fibrosis develops as a result of interstitial 6broblast proliferation and collagen deposition.' Incorporation of intraluminal fibrosis into the interstitium has also been described as a mechanism of interstitial fibrosts.s Recently, ultrastructural observations in patients with the acute form of interstitial pneumonia (HammanRich disease, acute interstitial pneumonia) have shown that epithelial necrosis followed by collapse and apposition of alveolar walls is an important mechanism of parenchymal remodeling. 3 We present evidence that this same mechanism ·From the Division of Surgical Pathologg University of Alabama at Birmingham. Reprint requests: Dr. Myers, DitMion ofSurgical Ehthology. University HospUal. 619 South 19th Street, Birmingham 35233 CHEST I 94 I 6 I DECEMBER. 1988
1309
A patient with downhill azygos venous 80w is reported. This reverse 80w was due to an obstruction in the superior vena cava and the azygos entry due to Beheets disease. Color 80w mapping of the azygos vein, using transesophageal real-time two-dimensional Doppler echography, was used for both anatomic and hemodynamic analysis. (Chest 1988;
94:1308.(9)
D
own hill esophageal varices and a downhill azygos vein, phenomena which occur when the superior vena cava is occluded, are collaterals that drain the venous flow from the head, neck, upper extremities and thorax. In the past, angiography and computed tomography (Cf) have been the main methods for the assessment of collaterals, I but we employed transesophageal real-time two-dimensional Doppler echography (TE2DD), a newly developed ultrasound method .' This report presents the first published case of downhill azygos vein that has been demonstrated b y TE2DD. CASE REPORT A 44-year-old man wasadmitted in to the Saitama Medical School Hospital suffering from Behcets disease with dyspnea on exertion and edema of the face. Two years before admission, he had iridocyclitis and oral ulceration. Right external jugular venography showed complete obstruction of the innominate and subclavian veins and the superior vena cava (SVC). Many downhill collateral veins were noted in the upper thorax instead of SVC (Fig 1). As shown in the lateral view, these collateral veins joined the azygos vein (Fig 2). Intercostal veins (asterisks) , draining into the azygos vein at almost a right angle, were also visualized. Of those, the uppermost one, which was thickest, was thought to be the supreme intercostal vein. The CT scanning, obtained at a level just below the tracheal carina, showed a thick azygosvein anterior to the vertebrae or posterior to the right side of the esophagus (data not shown). Endoscopic examination revealed small nonhazardous esophageal varices in the upper third of the esophagus. The TE2DD was used to visualize the collateral veins. The ultrasonic apparatus used was a 5 MHz, endoscopic convex array transducer integrated into the end of a Bexibleinsertion tube .' The scan direction was perpendicular to the insertion tube. The examination took about ten minutes. With regard to color display, the blood Bowapproaching the transducer was displayed in warm, reddish colors, while the Bowaway from it was shown in blue. The brightness varied with the Bow velocity. Thirty centimeters from the incisors, we could see the thick azygos vein posterior to the esophagus (Fig 3, left). The azygos vein was displayed in dark blue, while the thick intercostal veins, spouting into the azygos vein, are seen in bright red , the brightness reBecting the narrow angle between the ultrasound beam and the intercostal veins. Figure 3, right, is the schema of left. The direction of the azygos venous Bow wascaudad, the opposite of the normal. The uppermost and thickest *From the First Department of Surgery, Saitama Medical School, Moroyama-machi, lruma-gun, Saitama, Japan. Reprint requests : Dr. Sukigawa, 1st Department ofSurgery, Saitama Medical School , Moroyamamachi , lruma-gun, Saitama-kun, Japan
350-04
1308
FIGURE 1. Right external jugular venogram displaying obstruction of both the innominate vein and the superior vena cava and welldeveloped collateral vessels, anteroposterior view. intercostal vein was thought to be the supreme intercostal vein. The esophageal varices were not observed by TE2DD. DISCUSSION Downhill azygos vein is a result ofocclusion ofthe superior vena cava. The diseases which may result in such obstruction include bronchogenic carcinoma, thyroid gland enlargement, mediastinal fibrosis," and Behcets disease .' When the superior vena cava is occluded, blood from the upper extremities, head, and thorax must flow into the right atrium through collateral veins. The main collateral routes are the internal mammary, lateral thoracic, vertebral, and esophageal veins, communicating with the azygos vein or the inferior vena cava, and thereby, circumventing the obstructed superior vena cava .'.6 In the present case, the superior vena cava was obstructed in its entire course, including the azygos entry. However, the esophageal varices were confined to the upper third of the thoracic esophagus. This was, perhaps, due to the well-developed azygos venous
FIGURE 2. External jugular venogram showing the thick azygos vein, into which the intercostal veins (asterisks) drain. (Lateral view.) Downhill Azygos Vein (SukigarB, Takamoto, Omoto)
Oral
Anal
FIGURE 3. Color flow mapping of the azygos vein and the intercostal veins joining the former. Left, echogram taken 30 cm from the incisors. Right, the schema of left.
system, through which the venous flow of the upper half of the body drained into the inferior vena cava. In the past, evaluation of collateral vessels was commonly performed by angiography and CT. Transcutaneous ultrasonography is not capable of demonstrating the collateral veins around the esophagus because of intervening bone and intrapulmonary air. However, in TE2DD, the distance between the transducer and these vessels is reduced, and there are fewer obstacles, and so, visualization is easier. Moreover, with TE2DD , both anatomic and hemodynamic information can be obtained simultaneously. If a facility for a fast Fourier transform were incorporated in the ultrasound scanner, a quantitative evaluation of flow would also be possible. Although there are some limitations such as the small field of view and the long learning period required for the operator to be able to recognize the anatomy and interpret the ultrasound images, TE2DD is an extremely useful method for the study of collaterals and other vessels in the vicinity of the esophagus. REFERENCES
2
3
4 5 6 7
Hirose J, Takashima T, Suzuki M, Matsui 0 : Downhill varices of the esophagus demonstrated by dynamic computed tomography. J Com put Assist Tomogr 1984; 8:1007-09 Sukigara M, Komazaki T, YamazakiT, Ansai H, Koyama I, Omoto R. Colour flow mapping of oesophagogastric varices and vessels in and around the liver with trans-oesophageal real -time twodimensional Doppler ultrasound. Clin Radiol 1987; 38:487-94 Sorokin JJ, Levine SM, Moss EG, Biddle CM . Downh ill varice s: report of a substernal thyroid gland . Gastroenterology 1977; 73:345-48 Rosenthal T, Halkin H , Shani M, Deutch V. Occlusion of the graft veins in the Behcet syndrome. Angiology 1972; 23:600-05 Johnson LS, Kinnear DG , Brown RA, Mulder DS. Downhill esophageal varices. Arch Surg 1978; 113:1463-64 Felson B, Lessure AP. Downhill varices of the esophagus. Dis Chest 1964; 46:740-46 Miura K, Omoto R. Cautions for clinical use of the instrument. In: Omoto R, ed . Color atlas of real-time two-dimensional Doppler echocardiography, 2nd ed. Tokyo: Shindan-To-Chiryo Co, 1987:29-36
Epithelial Necrosis and Alveolar cOllarse in the Pathogenesis of Usua Interstitial Pneumonla* Jeffrey L. Myers, M.D ., F.G.G.P.; and Anna-Lu/se A Katzenstein, M .D.
We report ultrastructural evidence of epithelial necrosis and alveolar coUapse in a patient with usual interstitial pneumonia (UIP~ These changes were focal and confined to smaU areas characterized histologicaUy by aggregates of interstitial 6broblasts embedded within a myxoid stroma (6broblastic foci), UltrastructuraUy, the denuded epithelial basal lamina in these areas showed deep infoldings into the interstitium, and the luminal surfaces of the resultant clefts often were re-epithelialized. These findings suggest that the 6broblastic foci commonly seen in UIP represent sites of acute lung injury, and that alveolar coUapse foUowing epithelial necrosis is an important mechanism of lung remodeling. In addition to new insights regarding the pathogenesis of 6brosis in UIP, these observations may have important implications for assessing prognosis and selecting treatment strategies. (Cheat 1988; 94:1309-11)
T
he pathogenesis of interstitial fibrosis in usual interstitial pneumonia (UIP) has been the subject of extensive investigation.' Several studies have suggested that UIP begins as alveolitis, and that fibrosis develops as a result of interstitial 6broblast proliferation and collagen deposition.' Incorporation of intraluminal fibrosis into the interstitium has also been described as a mechanism of interstitial fibrosts.s Recently, ultrastructural observations in patients with the acute form of interstitial pneumonia (HammanRich disease, acute interstitial pneumonia) have shown that epithelial necrosis followed by collapse and apposition of alveolar walls is an important mechanism of parenchymal remodeling. 3 We present evidence that this same mechanism ·From the Division of Surgical Pathologg University of Alabama at Birmingham. Reprint requests: Dr. Myers, DitMion ofSurgical Ehthology. University HospUal. 619 South 19th Street, Birmingham 35233 CHEST I 94 I 6 I DECEMBER. 1988
1309