Introduction

Hepatic hydatid disease can cause multiple medical problems, including vascular complications. These complications are infrequent but are associated with high morbidity [1Gruttadauria S, Luca A, Cintorino D, Doria C, Scott VL, Marino IR (2003) Hepatic hydatid cyst causing thrombosis of the inferior vena cava and complicated by hemofilia. Dig Dis Sci 48: 358-364.-10Kayaalp C (2007) Hydatid liver cyst ruptured into vena cava inferior. Neth J Med 65: 402-403.]. They occur when a liver hydatid cyst (LHC) compresses or erodes one of the great vessels: portal vein, hepatic veins and/or inferior cava vein (IVC), or smaller branches [6Mekeel KL, Hemming AW (2007) Combined resection of the liver and the inferior vena cava for hydatid disease. J Gastrointest Surg 11: 1741-1743.,11Agarwal N, Dewan P (2010) Portal hypertension as a rare complication of hydatid cyst: diagnosis and management. Int J Surg 9: 1-6.]. Here we describe a patient with a recurrence of a giant LHC causing compression of the portal vein and IVC, resulting in portal hypertension and re-distribution of venous flow via theazygos system causing hematemesis from esophagealvarices.

Case Report

A 39-year-old Moroccan male came to the Emergency Department after frank hematemesis. Blood analysis revealed the following noteworthy values: hemoglobin: 8.4 gr/dl, 8400 leukocytes/ul (29.1% eosinophils), and alkaline phosphatase: 180 U/l, GGT: 128 U/l. Upper gastrointestinal endoscopy was performed and esophagealvarices with recent bleeding were seen. Past medical history included liver and lung surgery due to hydatid cysts at age of 7 (no medical reports). Abdominal ultrasound and CT showed a well-defined multivesicular cyst, with small foci of calcium in the wall, measuring 20 x 15 x 13 cm, occupying the segments IV, V and VIII of the liver, displacing and partially compressing a dilated portal vein and causing complete obstruction of the IVC (Figure 1). The radiological diagnosis was relapsed LHC. Cavography was performed, demonstrating a complete obstruction of IVC due to an extrinsic mass effect caused by the LHC. A large left renal vein redirected the venous flow through an enlarged azygos and hemiazygos system emptying into the superior cava vein. Hydatid serology was positive (1/320). We performed surgery to open the cyst, and then emptied and sterilized it (Figure 2). As we considered that radical cystectomy was impossible, we performed a subtotal cystectomy and left a large drain in the cyst cavity. Postoperatively, the patient presented low output biliary fistula that did not require any treatment. The microbiological study of the liquid of the cyst confirmed Echinococcusgranulosus infestation. In 18 months of follow-up, no relapse has been observed. The most recent CT showed a residual cavity of 2.7 cm, normalization of portal flow but no IVC flow; flow by collateral circulation continues. Esophagealvarices disappeared and serology of hydatidosis is currently negative.

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   Figure 1:

   Portal compression. A: sagittal B: coronal.
   
   
   

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   Figure 1: Portal compression. A: sagittal B: coronal.

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   Figure 2:

   Operative surgical view. C: Cyst. PV: portal vein.
   
   
   

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   Figure 2: Operative surgical view. C: Cyst. PV: portal vein.

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Discussion

LHC may cause serious vascular complications, affecting portal vein and its branches, the IVC, or both veins, as in our case [2Zubiarre L, Oyarbazal I, Ruiz I, Guisasola E (2006) Invasion of the portal vein by a hydatid cyst. Review of the literature. Gastroenterol Hepatol 29: 405-408.,6Mekeel KL, Hemming AW (2007) Combined resection of the liver and the inferior vena cava for hydatid disease. J Gastrointest Surg 11: 1741-1743.].

The portal vein may be affected by extrinsic compression of a LHC producing portal flow obstruction which could cause portal vein thrombosis or, in exceptional cases, portal cavernomatosis [11Agarwal N, Dewan P (2010) Portal hypertension as a rare complication of hydatid cyst: diagnosis and management. Int J Surg 9: 1-6.]. Until 2010, only 11 publications had appeared in PubMed, including 25 patients with hydatidosis and portal hypertension. Eighty per cent of these patients had alveolar hydatid disease [11Agarwal N, Dewan P (2010) Portal hypertension as a rare complication of hydatid cyst: diagnosis and management. Int J Surg 9: 1-6.]. Clinically, LHC can cause prehepatic, hepatic and posthepatic portal hypertension (Budd-Chiari) [2Zubiarre L, Oyarbazal I, Ruiz I, Guisasola E (2006) Invasion of the portal vein by a hydatid cyst. Review of the literature. Gastroenterol Hepatol 29: 405-408.,7Gil-Egea MJ, Alamaeda F, Girvent M, Riera R, Sitges A (1998) Hydatid cyst in the hepatic hilum causing a cavernous transformation in the portal vein. Gastroenterol Hepatol 21: 227-229.,8Kayacetin E, Hidayetoglu T (2004) Hydatid cyst of the liver causing a cavernous transformation in the portal vein and complicated by intrabiliary and intraperitoneal rupture. J Gastroenterol Hepatol 19: 1223-1224.,11Agarwal N, Dewan P (2010) Portal hypertension as a rare complication of hydatid cyst: diagnosis and management. Int J Surg 9: 1-6.]. A fistula between the LHC and the portal vein may occur, passing daughter vesicles into the bloodstream. This clinical situation has a high mortality [2Zubiarre L, Oyarbazal I, Ruiz I, Guisasola E (2006) Invasion of the portal vein by a hydatid cyst. Review of the literature. Gastroenterol Hepatol 29: 405-408.,7Gil-Egea MJ, Alamaeda F, Girvent M, Riera R, Sitges A (1998) Hydatid cyst in the hepatic hilum causing a cavernous transformation in the portal vein. Gastroenterol Hepatol 21: 227-229.,8Kayacetin E, Hidayetoglu T (2004) Hydatid cyst of the liver causing a cavernous transformation in the portal vein and complicated by intrabiliary and intraperitoneal rupture. J Gastroenterol Hepatol 19: 1223-1224.]. Surgery for LHC may resolve the portal compression if the period of compression has been short but in cases of cavernomatosis of the portal vein, the venous flow is not usually restored.

Involvement of the IVC is more common in patients with alveolar hydatid disease related to diffuse injury that causes this type of infestation [6Mekeel KL, Hemming AW (2007) Combined resection of the liver and the inferior vena cava for hydatid disease. J Gastrointest Surg 11: 1741-1743.]. Compression of the IVC by the LHC may cause either complete or incomplete obstruction of the venous flow and may create a true thrombosis of the IVC, even cavernomatosis in extreme cases like ours [6Mekeel KL, Hemming AW (2007) Combined resection of the liver and the inferior vena cava for hydatid disease. J Gastrointest Surg 11: 1741-1743.]. In exceptional cases, a fistula between the LHC and the IVC may occur. In these patients, some hydatid membranes may pass from the cyst to the right atrium and indirectly produce a hydatid pulmonary embolism [1Gruttadauria S, Luca A, Cintorino D, Doria C, Scott VL, Marino IR (2003) Hepatic hydatid cyst causing thrombosis of the inferior vena cava and complicated by hemofilia. Dig Dis Sci 48: 358-364.,3Bahamondes JC, Sandoval P, Losada H, Meriño G, Salaman J, et al. (2010) Quiste hidatídico hepático complicado con invasión a vena cava inferior y embolización de membranas hidatídicas a aurícula derecha. Caso Clínico. Rev Chil Cir 62: 179-182.-5Karunajeewa HA, Jones RM, Hardy KJ, Buxton BF, Richards MJ (2002) Hydatid disease invading the inferior vena cava: successful combined medical and surgical Management. ANZ J Surg 72: 1 59-160.,9Sirmali M, Gezer S, Yol S, Kaya S (2006) Hydatid cyst of the pulmonary artery secondary to hepatic hydatid cyst. Acta Chir Belg 106: 441-442.]. Despite the existence of a real fistula between IVC and LHC, exsanguination is exceptional because the pressure of the cyst is higher than the IVC and the pericystic wall hinders the passage of blood to the cyst. But when a fistula is present, anaphylactic or hemodynamic shock may occur and has a high morbidity and mortality [10Kayaalp C (2007) Hydatid liver cyst ruptured into vena cava inferior. Neth J Med 65: 402-403.]. Patients with involvement of the IVC due to LHC usuallyhave large, centrally-located cysts. Thrombosis of the IVC due to LHC can cause acute symptoms but in most cases patients gradually manage to create tributary veins to resolve the obstruction andmay, as in our case, be asymptomatic for a long period. Approximately 50% of patients with IVC thrombosis are asymptomatic when they are diagnosed [5Karunajeewa HA, Jones RM, Hardy KJ, Buxton BF, Richards MJ (2002) Hydatid disease invading the inferior vena cava: successful combined medical and surgical Management. ANZ J Surg 72: 1 59-160.].

The limited number of patients with vascular complications of LHC makesit difficult to draw conclusions about optimal treatment. We think that patients with vascular involvement due to LHC should always be treated surgicallyif their condition permits even if they are asymptomatic, before they develop severe future complications. Previous albendazole therapy is advisable. Some patients have been treated with PAIR, sometimes as single therapy due to severe co-morbidities and/ora high surgical risk, or as a prelude to surgery [8Kayacetin E, Hidayetoglu T (2004) Hydatid cyst of the liver causing a cavernous transformation in the portal vein and complicated by intrabiliary and intraperitoneal rupture. J Gastroenterol Hepatol 19: 1223-1224.,12Rajagopal KV, Bishwas R (2002) Hydatid cyst of the liver presenting as inferior vena cava obstruction. J Clin Ultrasound 30: 114-116.]. In the case of anaphylactic or hemodynamic shock, emergency surgery is mandatory but mortality is extremely high.

In patients with portal vein involvement, the surgical technique performed depends on the degree of involvement and type of portal thrombosis. If there is only compression or a short contact with no (or only mild) portal thrombosis, radical surgery can be planned if it is not precluded by other clinical problems, but for cases with large-scale contact (either lengthwise or circumferential) or severe thrombosis with portal hypertension, conservative surgery is the best option [2Zubiarre L, Oyarbazal I, Ruiz I, Guisasola E (2006) Invasion of the portal vein by a hydatid cyst. Review of the literature. Gastroenterol Hepatol 29: 405-408.,7Gil-Egea MJ, Alamaeda F, Girvent M, Riera R, Sitges A (1998) Hydatid cyst in the hepatic hilum causing a cavernous transformation in the portal vein. Gastroenterol Hepatol 21: 227-229.,8Kayacetin E, Hidayetoglu T (2004) Hydatid cyst of the liver causing a cavernous transformation in the portal vein and complicated by intrabiliary and intraperitoneal rupture. J Gastroenterol Hepatol 19: 1223-1224.]. In a very few cases, portal resection or prosthetic substitution has been performed.

Typically, in patients like ours with vascular involvement of the IVC, conservative surgery is chosen because of the difficulty of performing radical procedures [12 Rajagopal KV, Bishwas R (2002) Hydatid cyst of the liver presenting as inferior vena cava obstruction. J Clin Ultrasound 30: 114-116.]. When only lateral contact exists, total cystectomy is possible in selected cases. Some authors have performed liver resection with partial resection of the IVC [1Gruttadauria S, Luca A, Cintorino D, Doria C, Scott VL, Marino IR (2003) Hepatic hydatid cyst causing thrombosis of the inferior vena cava and complicated by hemofilia. Dig Dis Sci 48: 358-364.,2Zubiarre L, Oyarbazal I, Ruiz I, Guisasola E (2006) Invasion of the portal vein by a hydatid cyst. Review of the literature. Gastroenterol Hepatol 29: 405-408.,6Mekeel KL, Hemming AW (2007) Combined resection of the liver and the inferior vena cava for hydatid disease. J Gastrointest Surg 11: 1741-1743.], and a very small number of IVC resections with prosthetic replacement due to LHC have been reported [6Mekeel KL, Hemming AW (2007) Combined resection of the liver and the inferior vena cava for hydatid disease. J Gastrointest Surg 11: 1741-1743.]. This technical option is controversial; some authors consider the degree of surgery to be excessive for a benign disease, but on occasion it is the only possible solution to a severe problem [6Mekeel KL, Hemming AW (2007) Combined resection of the liver and the inferior vena cava for hydatid disease. J Gastrointest Surg 11: 1741-1743.,10Kayaalp C (2007) Hydatid liver cyst ruptured into vena cava inferior. Neth J Med 65: 402-403.].

In patients with Budd-Chiari syndrome, three options have been used: LHC surgery alone, shunt, or placement of percutaneous endoluminal prosthesis [11 Agarwal N, Dewan P (2010) Portal hypertension as a rare complication of hydatid cyst: diagnosis and management. Int J Surg 9: 1-6.,13Sarawagi R, KeshavaSk, Surendrababu NRS, Zachariah UG, Eapen EC (2011) Budd-Chiari syndrome complicating hydatid cyst of the liver managed by venoplasty and stenting. Cardiovasc Intervent Radiol 34: s202-205].

To conclude, vascular complications due to LHC are infrequent. The portal vein, the IVC, or both, may be affected. Degrees of severity range from compression to cavernomatosis. Patient may be asymptomatic, but extreme clinical situations could occur. In patients with vascular involvement due to LHC, conservative surgery is preferred because radical surgery is usually considered too risky.