Diverticulum o f the inferior vena cava: A case report F. Hasan, FRCS, F. Gleeson, MRCP, FRCR, M. R. Lock, FRCS, R. Williams, M B B S , and D. Grant, M R C P , F R C R , London, United Kingdom Anomalies of the inferior vena cava are rare. We describe a case of a diverticulum arising from the inferior vena cava, which has not been described to date. (J VASC SURG 1992;15:578-80.)
Abnormalities o f the inferior vena cava (IVC) are uncommon. Surgeons and radiologists, however, must be aware o f their existence to avoid potential pitfalls. These variations are thought to be a reflection of the anomalies arising in the complex embryologic process, which results in the formation o f the IVC. T o date, four major anomalies have been described) ,2 We report a case of a diverticulum arising from the IVC, which has not yet been described (Medline Search 1971-1991). Its implication for the surgeon, radiologist, and patient care are discussed.
CASE REPORT A 59-year-old man was admitted with a history of right upper quadrant pain compatible with biliary colic. He had had congenital syphilis and as a result had become blind in the left eye because of optic atrophy. At the age of 40 years he had a vagotomy and pyloroplasty performed for a perforated duodenal ulcer. He had otherwise been well. His examination was unremarkable except for evidence of previous surgery and a hemangioma involving the right hemiscrotum and lateral aspect of his right leg. This had been present from birth. Ultrasonography of his right upper quadrant showed multiple gallstones and a large cystic lesion related to the posterolateral aspect of the right kidney. In view of this a CT scan was obtained. This revealed an oval, homogenous, well-circumscribed mass within the right hypochondrium measuring 5 x 5.5 cm. It appeared to be separate from the liver, right kidney, right psoas muscle, and the IVC. The scan sequence was repeated after intravenous contrast enhancement, and the mass demonstrated only partial enhancement. There was no evidence of dilated intrahepatic or extrahepatic ducts or choledochal cysts (Fig. 1). From the Departmentof Surgery(Hasan, Lock,and Williams) and Department of Radiology (Gleeson), Whittington Hospital, London. Reprint requests: Mr. M. R. Lock, Department of Surgery, Whittington Hospital, Highgate Hill, London, NI9 5NF. 24/4/32841 578
Further tests including a barium meal, gastric hormone screen, vanillyl mandelic acid levels, and serologic tests for treponemal antibodies were all normal. A laparotomy was undertaken via an upper midline, incision, which confirmed the presence of a gall bladder containing gallstones. It also revealed a large diverticulum arising from the posterolateral aspect of the IVC and extending between the right kidney and the duodenum (Fig. 2). The mass was carefully mobilized and the neck was clamped. It was then excised and the neck oversewn with 5-0 Prolene sutures (Ethicon Inc., Somerville, N.J.). A routine cholecystectomywas performed; the perioperative cholangiogram was normal. His postoperative course was uneventful, and he was discharged 10 days after his laparotomy. The diagnosis of a diverticulum of the IVC was made by histology, which displayed a full thickness venous wall, characterized by a muscular wall with an endothelial lining.
DISCUSSION The development o f the IVC is a complex process. It takes place in the fetus between the sixth and tenth week. 3'* Three parallel pairs o f veins, the supracardinal, subcardinal, and infracardinal veins appear, develop extensive anastomosis, and partially regress to give rise to the definitive IVC. Eventually the right subcardinal vein forms the suprarenal segment o f the IVC, the right supracardinal vein forms the infrarenal segment, and the postcardinal veins form the iliac bifurcation. The subcardinal and supracardinal veins both contribute to the renal veins. Anomalies o f the IVC are uncommon. Four major embryologic anomalies have been described; 1,2,4-7these include a double IVC (incidence o f 0.2% to 3%), a left-sided IVC (incidence o f 0.2% to 0.5%), a retroaortic left renal vein (incidence of 1.2% to 2.4%), and a circumaortic left renal vein (incidence of 1.5% to 8.7%). Other abnormalities have been described but most are variations on the above themes and some are described only in animals. 2 A
Volume 15 Number 3 March 1992
Inferior vena cava diverticulum
579
Fig. 1. CT scan of abdomen, shows inferior vena cava diverticulum (A), inferior vena cava (B), and aorta (C).
diverticulum arising from the IVC has not been previously described. The clinical significance of IVC abnormalities to the surgeon and radiologist is crucial. The former may be referred patients with an undiaguosed abdominal mass, as in this case, or may discover a "mass" as an incidental finding during laparotomy. Patients are frequently referred to a radiologist for percutaneous image-guided biopsy of abdominal lesions, and clearly an appreciation of these anomalies is vital to avoid potential disaster. Most anomalies of the IVC can be diagnosed by CT scanning or ultrasonography. 4'6-s If there is doubt, an inferior venocavogram will delineate the precise anatomy. 2,9 Venography in this situation should be performed via a left femoral puncture, since this will opacify both IVCs if duplication is present and will opacify the left IVC if transposition exists. During investigation for undiagnosed abdominal masses due consideration should be given to possible IVC abnormalities to avoid puncturing a potential IVC diverticulum.
Ri0htRe0a, Vein
(I
Fig. 2. Inferior vena cava shows diverticulum.
We thank Mr. A. Wilson for the diagram and Miss M. Chaudry, Superintendant Radiographer, CT Scanning, for the CT scans.
580
Journal of VASCULAR SURGERY
Hasan et al.
REFERENCES 1. Philipps E. Embryology, normal anatomy and anomalies. In: Ferris FJ, Hipora FA, Kahn PC, Philipps E, Shapiro JR, eds. Venography of the inferior vena cava and its branches. Baltimore: Williams and Wilkins, 1969:1-32. 2. Giordano JM, Trout I-IH. Anomalies of the inferior vena cava. J V ~ c SURG 1986;3:924-8. 3. Chaung VP, Mena E, Hoskins PA. Congenital anomalies of the inferior vena cava: review ofembryogenesis and presentation of a simplified classification. Br J Radiol 1974;47:206-13. 4. Mayo J, Gray R, St. Louis E, Grossman H, McLoughlin M, Wise D. Anomalies of the inferior vena cava. AJR 1983;140: 339-45. 5. Royal SA, Collen P. CT evaluation of the anomalies of the inferior vena cava and left renal vein. AJR 1979;132:759-63.
6. Richardson ML, Richard KE, Levesque PH. Inferior vena cava duplication: demonstration by sonography. J Clin Ultrasound 1983;11:225-8. 7. Lee JKT, Sagel SS, Stanley Rj'. Computed body tomography with MRI correlation, 2nd Ed. Raven Press 1989;chap. 17:718-26. 8. Faer MJ, Lynch RD, Evan HO, Chin FK. Inferior vena cava duplication: demonstration by computed tomography. Radiology 1979;130:707-9. 9. Brener BJ, Darling C, Frederick PL, Longton RR. Major venous anomalies complicating abdominoaortic surgery. Arch Surg 1974;108:160-5.
Submitted Feb. 8, 1991; accepted July 29, 1991.
Abnormalities o f the inferior vena cava (IVC) are uncommon. Surgeons and radiologists, however, must be aware o f their existence to avoid potential pitfalls. These variations are thought to be a reflection of the anomalies arising in the complex embryologic process, which results in the formation o f the IVC. T o date, four major anomalies have been described) ,2 We report a case of a diverticulum arising from the IVC, which has not yet been described (Medline Search 1971-1991). Its implication for the surgeon, radiologist, and patient care are discussed.
CASE REPORT A 59-year-old man was admitted with a history of right upper quadrant pain compatible with biliary colic. He had had congenital syphilis and as a result had become blind in the left eye because of optic atrophy. At the age of 40 years he had a vagotomy and pyloroplasty performed for a perforated duodenal ulcer. He had otherwise been well. His examination was unremarkable except for evidence of previous surgery and a hemangioma involving the right hemiscrotum and lateral aspect of his right leg. This had been present from birth. Ultrasonography of his right upper quadrant showed multiple gallstones and a large cystic lesion related to the posterolateral aspect of the right kidney. In view of this a CT scan was obtained. This revealed an oval, homogenous, well-circumscribed mass within the right hypochondrium measuring 5 x 5.5 cm. It appeared to be separate from the liver, right kidney, right psoas muscle, and the IVC. The scan sequence was repeated after intravenous contrast enhancement, and the mass demonstrated only partial enhancement. There was no evidence of dilated intrahepatic or extrahepatic ducts or choledochal cysts (Fig. 1). From the Departmentof Surgery(Hasan, Lock,and Williams) and Department of Radiology (Gleeson), Whittington Hospital, London. Reprint requests: Mr. M. R. Lock, Department of Surgery, Whittington Hospital, Highgate Hill, London, NI9 5NF. 24/4/32841 578
Further tests including a barium meal, gastric hormone screen, vanillyl mandelic acid levels, and serologic tests for treponemal antibodies were all normal. A laparotomy was undertaken via an upper midline, incision, which confirmed the presence of a gall bladder containing gallstones. It also revealed a large diverticulum arising from the posterolateral aspect of the IVC and extending between the right kidney and the duodenum (Fig. 2). The mass was carefully mobilized and the neck was clamped. It was then excised and the neck oversewn with 5-0 Prolene sutures (Ethicon Inc., Somerville, N.J.). A routine cholecystectomywas performed; the perioperative cholangiogram was normal. His postoperative course was uneventful, and he was discharged 10 days after his laparotomy. The diagnosis of a diverticulum of the IVC was made by histology, which displayed a full thickness venous wall, characterized by a muscular wall with an endothelial lining.
DISCUSSION The development o f the IVC is a complex process. It takes place in the fetus between the sixth and tenth week. 3'* Three parallel pairs o f veins, the supracardinal, subcardinal, and infracardinal veins appear, develop extensive anastomosis, and partially regress to give rise to the definitive IVC. Eventually the right subcardinal vein forms the suprarenal segment o f the IVC, the right supracardinal vein forms the infrarenal segment, and the postcardinal veins form the iliac bifurcation. The subcardinal and supracardinal veins both contribute to the renal veins. Anomalies o f the IVC are uncommon. Four major embryologic anomalies have been described; 1,2,4-7these include a double IVC (incidence o f 0.2% to 3%), a left-sided IVC (incidence o f 0.2% to 0.5%), a retroaortic left renal vein (incidence of 1.2% to 2.4%), and a circumaortic left renal vein (incidence of 1.5% to 8.7%). Other abnormalities have been described but most are variations on the above themes and some are described only in animals. 2 A
Volume 15 Number 3 March 1992
Inferior vena cava diverticulum
579
Fig. 1. CT scan of abdomen, shows inferior vena cava diverticulum (A), inferior vena cava (B), and aorta (C).
diverticulum arising from the IVC has not been previously described. The clinical significance of IVC abnormalities to the surgeon and radiologist is crucial. The former may be referred patients with an undiaguosed abdominal mass, as in this case, or may discover a "mass" as an incidental finding during laparotomy. Patients are frequently referred to a radiologist for percutaneous image-guided biopsy of abdominal lesions, and clearly an appreciation of these anomalies is vital to avoid potential disaster. Most anomalies of the IVC can be diagnosed by CT scanning or ultrasonography. 4'6-s If there is doubt, an inferior venocavogram will delineate the precise anatomy. 2,9 Venography in this situation should be performed via a left femoral puncture, since this will opacify both IVCs if duplication is present and will opacify the left IVC if transposition exists. During investigation for undiagnosed abdominal masses due consideration should be given to possible IVC abnormalities to avoid puncturing a potential IVC diverticulum.
Ri0htRe0a, Vein
(I
Fig. 2. Inferior vena cava shows diverticulum.
We thank Mr. A. Wilson for the diagram and Miss M. Chaudry, Superintendant Radiographer, CT Scanning, for the CT scans.
580
Journal of VASCULAR SURGERY
Hasan et al.
REFERENCES 1. Philipps E. Embryology, normal anatomy and anomalies. In: Ferris FJ, Hipora FA, Kahn PC, Philipps E, Shapiro JR, eds. Venography of the inferior vena cava and its branches. Baltimore: Williams and Wilkins, 1969:1-32. 2. Giordano JM, Trout I-IH. Anomalies of the inferior vena cava. J V ~ c SURG 1986;3:924-8. 3. Chaung VP, Mena E, Hoskins PA. Congenital anomalies of the inferior vena cava: review ofembryogenesis and presentation of a simplified classification. Br J Radiol 1974;47:206-13. 4. Mayo J, Gray R, St. Louis E, Grossman H, McLoughlin M, Wise D. Anomalies of the inferior vena cava. AJR 1983;140: 339-45. 5. Royal SA, Collen P. CT evaluation of the anomalies of the inferior vena cava and left renal vein. AJR 1979;132:759-63.
6. Richardson ML, Richard KE, Levesque PH. Inferior vena cava duplication: demonstration by sonography. J Clin Ultrasound 1983;11:225-8. 7. Lee JKT, Sagel SS, Stanley Rj'. Computed body tomography with MRI correlation, 2nd Ed. Raven Press 1989;chap. 17:718-26. 8. Faer MJ, Lynch RD, Evan HO, Chin FK. Inferior vena cava duplication: demonstration by computed tomography. Radiology 1979;130:707-9. 9. Brener BJ, Darling C, Frederick PL, Longton RR. Major venous anomalies complicating abdominoaortic surgery. Arch Surg 1974;108:160-5.
Submitted Feb. 8, 1991; accepted July 29, 1991.
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