stimec-_Opmaak 1 23/10/12 16:38 Pagina 302

JBR–BTR, 2012, 95: 302-305.

GASTRIC VARICES WITHOUT PORTAL HYPERTENSION: ROLE OF LEFT INFERIOR VENA CAVA?

B.V. Stimec 1, L.A.B. Grønvold 2, D. Ignjatovic 2,3 , J.H.D. Fasel 1

A variant vascular anatomy was detected during regular analysis of multidetector computed tomography angio- graphy of the in a 70 year-old female patient, referred to the department of surgery for laparoscopic right colectomy for colon cancer. The anomalous vessel was located left to the aorta, and was consistent with a persistent left inferior vena cava. It was connected by two retroaortic rootlets to the dorsal surface of the regular right inferior vena cava and had four notable tributaries – an anastomosis with the iliolumbar trunk, ovarian , and the . In the upper abdomen, the left inferior vena cava took a tortuous course, passing at first between the spleen and the diaphragm, then curving below the inferior splenic border and terminating in an irregu - lar network in the posterior region of gastric fundus and cardia, close to the splenic hilum, without supradiaphrag - matic continuation. Despite this extraordinary termination, there were no signs of portal hypertension or data on previous occurrence of this condition.

Key-words: Stomach, varices – Magnetic resonance (MR), vascular studies – , MR.

Variations of the inferior vena cava (IVC) and its tributaries were once regarded as numerous in form and rare in incidence. These varia - tions, however, have been more fre - quently reported with the advent of multislice helical CT imaging modal - ities. A persistent left inferior vena cava is found in 0.2%-0.5% and a double IVC in 0.2-3% of the general population in autopsy series (1-5). The case of the gastric varices we report hereinafter deserves attention for its atypical appearance, lack of pertinent clinical etiology and the direct anatomical framework of the persistent inferior left vena cava.

Case presentation

The investigation was conducted Fig. 1. — Synoptical coronal CT of the abdomen, level of the in accordance with Ethical Principles portal vein confluence (asterisk). for Medical Research Involving Human Subjects (6). A 70-year-old woman was referred to the department of sur - left-sided ovarian adenocarcinoma pression of the left ureter by the gery for colon cancer, located in the for which she underwent a bilateral pelvic tumor and consequent ascending colon, just proximal to the salpingo -oophorectomy, supra - hydronephrosis. hepatic flexure. The laparoscopic vaginal hysterectomy and recto- There were no biochemical, radio - right colectomy was performed, but sigmoid resection two years earlier, logical or anamnestic findings sug - the patient developed signs of anas - followed by a re-operation for gesting clinically significant portal tomotic leakage and underwent re- anastomotic leakage, and a sigmoid- hypertension, such as low platelet operation with a resulting ileostomy. ostomy. No retroperitoneal lym - count, splenomegaly, portal vein Final histopathology showed an ade - phadenectomy was performed. She diameter above the upper limit, nocarcinoma (stage Dukes C) with had been treated by adjuvant schistosomiasis, excessive alcohol metastases to local lymph nodes. chemotherapy and had undergone intake, melena, hematemesis, etc. The patient had had a previous introduction of a left JJ (pig-tail) Therefore, the upper endoscopy was history of progressive and infiltrating stent, due to the long-standing com - not indicated. The pre- and postoperative helical CT was performed with a 64-detector CT scanner (Toshiba, TS_Aquillion64). From: 1. Faculty of Medicine, Department of Cellular Physiology and Metabolism, Portal-venous phase images were Anatomy Sector, University of Geneva, Geneva, Switzerland, 2. Vestfold Hospital Trust, obtained after contrast media Department of Surgery, Tonsberg, Norway, 3. Department of Digestive Surgery, administration (320 mg/ml, 80 ml), Akershus University Hospital, University of Oslo, Lørenskog, Norway. Address for correspondence: Dr B.V. Stimec, M.D., Ph.D., Faculty of Medicine, using a power injector at a rate of Department of Cellular Physiology and Metabolism, Anatomy Sector, University of 2.5 ml/s. The CT parameters were as Geneva, Rue Michel-Servet 1, 1211 Geneva, Switzerland. follows: pitch was 0.828, tube volt - E-mail: [email protected] age 120 kV, maximum tube current stimec-_Opmaak 1 23/10/12 16:38 Pagina 303

GASTRIC VARICES WITHOUT PORTAL HYPERTENSION — STIMEC et al 303

A

Fig. 3. — Grayscale outline of the LIVC. Captions: ILV – com - mon trunk for iliolumbar / left ; LRV – left renal vein, LOV – left ; LIPV – left inferior phrenic vein. Other captions as in Figure 2.

testinal cancer, with enlarged paraaortic lymph nodes. During this B period the patient was also diag - nosed with an aggressive leukemia Fig. 2. — A. 3D volume rendering. CT – celiac trunk, LIVC – left and died shortly thereafter due to inferior vena cava (ur – upper root, lr – lower root), SMA – supe - rior mesenteric , SMV – superior mesenteric vein, SV – sepsis. splenic vein, PV – portal vein, RIVC – right inferior vena cava. The CT disclosed a 10 mm by B. Detail from Fig. 2 A: arborization of the upper part of LIVC 10 mm gallstone in the gallbladder, with tortuous gastric varices. otherwise the liver and the bile ducts were considered normal. The bipolar diameter of the spleen was 11.7 cm, and the calibers of the splenic vein, 250 mAs, 3 mm reconstructed sec - superior mesenteric vein and the tion thickness, 3 mm reconstruction interval. Analysis was performed by means of 2D multiplanar reconstruc - tion with maximum intensity projec - Table I. — Morphometry of the persistent LIVC. tion and 3D volume rendering tech - nique using the Osirix algorithm. Skeletotopy Caliber (cm) Length (cm) In the later clinical follow-up, Upper root L2 / L3 0.884 4.330 diagnostic imaging revealed a tumor Lower root L3 (lower half) 0.646 5.771 in the distal ileum, highly suspicious Trunk L3 – T12 / L1 1.039 10.144 of a local recurrence of her gastroin - stimec-_Opmaak 1 23/10/12 16:38 Pagina 304

304 JBR–BTR, 2012, 95 (5)

portal vein were 0.872 cm, 0.840 cm Discussion cardinal vein systems are relatively and 0.976 cm, respectively (Fig. 1). isolated (5, 7-9). On the other hand, Both the pre- and postoperative The embryogenesis of the IVC is a the duplication of the vena cava may CTs revealed a vessel located left to composite process, with some also be associated with a retrocaval the aorta, consistent with a persist - stages still uncertain or under ureter, which descends between the ent left inferior vena cava (LIVC). Two debate. It is based on sequential for - venae cavae and the aorta (4). In our venous rootlets emerged from the mation, regression, anastomosing case, no congenital anomalies of the dorsal surface of the regular right and fusion of multiple paired axial urinary system were observed. IVC, passed to the left retro-aortical - venous channels: postcardinal, sub - Further, taking into account the cau - ly, and merged into the trunk of LIVC cardinal, supracardinal, azygos and docranial direction of the blood flow (Fig. 2). subcentral. The double IVC derives for the ovarian vein and the iliolum - There were four notable tributar - from the persistence of both the bar vein, it would be unlikely that the ies/branches to the LIVC (Fig. 3 and right and left supracardinal . initial gynecological operation has 4, Table I): Although the left IVC is considered to elicited the development of a collat - be strictly subrenal (3-5, 7, 8), there eral venous channel. – common trunk for iliolumbar / left are extremely rare reports of its The ratio between the two IVC cal - ascending lumbar vein; supra renal hemiazygos continua - ibers has not been often addressed – stump of the left ovarian vein; tion (9). In the case reported by Lao in the literature, only with regard to – left renal vein; and et al. (10), the left vena cava crossed the determination of duplication – left inferior phrenic vein the midline to the right and emptied type (2) or the regression of the right The first branch (common trunk into the azygos vein. IVC (5). Our case shows the persist - for iliolumbar / left ascending lumbar Embryologic changes affecting ent LIVC to have a caliber approxi - vein) emerged from the lower root the veins are considered to also mately one-third of its counterpart. and not from the LIVC trunk itself. In induce variations in lymphatic The most enigmatic part of our the upper abdomen, the LIVC took a drainage (1). LIVC case was its cranial portion. tortuous course, passing at first With regard to the two IVC conflu - Unlike the reports in which the per - between the spleen and the ence and/or anastomoses, the LIVC sistent LIVC continues the hemiazy - diaphragm, then curving below the crosses the aorta anteriorly (5, 7, 8), gos course and opens into the coro - inferior splenic border and terminat - or posteriorly (4), at the level of the nary sinus (5), in our case the per - ing in an irregular network in the left renal vein. A highly positioned sistent vessel did not traverse the posterior region of gastric fundus, preaortic crossing may lead to inter - diaphragm. Instead, it dispersed in a close to the splenic hilum (Fig. 2B). mittent celiac trunk compression network in the region of gastric fun - In relation to the gastric wall, this syndrome (3). Our case had two dus, resembling the MDCT picture of network was found to lie extramural - retroaortic roots connecting the right gastric varices (11). It has been noted ly (Fig. 4). According to the Sarin and left IVC at the levels of second that, unlike the cardiac veins and classification, these gastric varices and third lumbar vertebra, respec - varices which are continuous with would correspond to group IGV-1. tively. In addition, the number of the esophageal varices, fundic varices The left ureter coursed lateral and vertebrae was normal, and no major develop independently and can be deep to the inferior portion of LIVC. vascular anomalies were found very large in caliber (12). This is a There was no supradiaphragmatic except the LIVC in question. This is in consequence of the hepatopetal continuation of the LIVC. The right accordance with the fact that the direction of blood flow towards the IVC was unremarkable. developmental anomalies of the stomach and inferior diaphragmatic

A B C

Fig. 4. — 2D multiplanar reconstruction. A) and B) coronal views; C) axial view: arrows – fundic termination of LIVC. Caption as in Figure 3. stimec-_Opmaak 1 23/10/12 16:38 Pagina 305

GASTRIC VARICES WITHOUT PORTAL HYPERTENSION — STIMEC et al 305

veins, which plays a buffer role in References Kathmandu Univ Med J (KUMJ) , portal hypertension (13). However, in 2006, 4: 253 -255. our case there was no data or any 1. Castillo O.A., Sanchez-Salas R., 10. Lao W.T., Chan W.P., Lee R.C., et al.: clinical findings in favor of portal Alvarez J.M., et al.: Inferior vena cava Duplicated infrarenal IVC with azy - hypertension. We also considered anomalies during laparoscopic gous continuation of left IVC: Pre- operative CT and 3-D reconstruction the possibility of congenital extra - retroperitoneal lymph node dissec - tion. J Endourol , 2008, 22: 327-331. image findings. Chin J Radiol , 2003, hepatic portocaval shunt – the 2. Natsis K., Apostolidis S., Noussios G., 28: 249-253. Abernethy syndrome (14), or similar et al.: Duplication of the inferior vena 11. Zhao L.Q., He W., Ji M., et al.: 64-row malformations reported in ani - cava: anatomy, embryology and clas - multidetector computed tomography mals (15), but, as already mentioned, sification proposal. Anat Sci Int , 2010, portal venography of gastric variceal there were no other abnormalities of 85: 56-60. collateral circulation. World J Gastro- the . 3. Wartmann C.T., Kinsella C.R. Jr., enterol , 2010, 16: 1003-1007. The only plausible explanation we Tubbs R.S., et al.: A rare case of a 12. Arakawa M., Masuzaki T., Okuda K.: can offer could be an enlarged complete left inferior vena cava asso - Pathomorphology of esophageal and gastric varices. Semin Liver Dis , 2002, venous communication between the ciated with the symptoms of Dunbar syndrome. Clin Anat , 2011, 24: 262- 22: 73-82. persistent LIVC, the left gastric/ 265. 13. Fujimura I., de Carvalho C.A., gastroomental veins and the left infe - 4. Baldridge E.D. Jr., Canos A.J.: Venous Rodrigues Júnior A.J.: Angio- rior phrenic vein. A similar pattern, an anomalies encountered in aortoiliac architecture of the esophagogastric anastomosis between an abnormally surgery. Arch Surg , 1987, 122: 1184- veins in portal hypertension. Acta enlarged vein communicating 1188. Anat (Basel) , 1990, 139: 374-379. between the left gastric vein and the 5. Castro E.C., Devine W., Galambos C.: 14. Collard B., Maleux G., Heye S., et al.: esophageal tributary of the left infe - The anatomy of a novel malformation Value of carbon dioxide wedged rior phrenic vein, which opened into of the cardinal vein system. Pediatr venography and transvenous liver biopsy in the definitive diagnosis of the left renal vein, has been found to Dev Pathol , 2010, 13: 318-321. 6. World Medical Association Abernethy malformation. Abdom occur in 40% of apparently healthy Declaration of Helsinki: Ethical Imaging , 2006, 31: 315-319. specimens (16). There have been Principles for Medical Research 15. Brown J.C. Jr., Chanoit G., Reeder J.: reports on portosystemic shunts Involving Human Subjects, 59 th Complex extrahepatic portocaval involving the left inferior phrenic vein WMA General Assembly, Seoul, shunt with unusual caval features in a and draining into the renal vein (17), October 2008. Available at: http:// cat: computed tomographic charac - but they were intrahepatic and prob - www.wma.net/en/30publications/ terisation. J Small Anim Pract , 2010, ably acquired through iatrogenic or 10policies/b3/index.html Accessed 51: 227-230. traumatic episodes, or as a conse - November 23, 2011. 16. Loukas M., Louis R.G. Jr., Hullett J., et al.: An anatomical classification of the quence of portal hypertension. 7. Honma S., Tokiyoshi A., Kawai K., et al.: Left inferior vena cava with variations of the inferior phrenic vein. In the light of the fact that the regressed right inferior vena cava. Surg Radiol Anat , 2005, 27: 566-574. number of the patients undergoing Anat Sci Int , 2008, 83: 173-178. 17. Hirota T., Yamagami T., Matsumoto T., multiple complex surgical proce - 8. Khamanarong K., Woraputtaporn W., et al.: Intrahepatic portosystemic dures is increasing, the preoperative Teerakul S., et al.: Left sided inferior venous shunt passing through the left awareness for the new forms of vas - vena cava: a case report. J Med Assoc inferior phrenic vein and draining cular variations and anomalies, such Thai , 2010, 93: 998 -1000. into the left renal vein. Br J Radiol , as the case here presented, becomes 9. Kumar S.: An anomaly of inferior 2004, 77: 966-968. mandatory. vena cava: a rare case report.