Review

Rafiq and Sitrin report an interesting case of a portal vein aneurysm that has been followed for over 10 years.¹ During that time, the aneurysm increased only slightly in diameter, was asymptomatic, and was managed with conservative treatment. The only potential etiologic factor encountered was alcoholic pancreatitis, but it was not severe, leading the authors to suggest that a congenital origin was most likely the cause of the aneurysm.

Rafiq and Sitrin have presented sound etiopathogenic discussion and specific criteria for the definition and diagnosis of portal vein aneurysms, as have other authors.^2–3 However, a careful review for published cases in the Ovid MEDLINE-indexed literature found at least 95 medical articles on portal vein aneurysms, which translated into approximately 150 cases since the first case described by Barzilai and Kleckner in 1956.⁴ Excluding cases written in languages other than English, more than 120 cases remain.^2–70 Therefore, portal vein aneurysm should not be considered an exceptionally rare disease. Certainly, the discovery of portal vein aneurysms has been made easier with the wider availability of noninvasive abdominal imaging studies, including in utero imaging.^5–6 Portal vein aneurysms have been found in 0.07% of ultrasonographic studies for the liver⁷ and in 0.1% of abdominal noninvasive imaging studies for varied indications.⁸ Because the clinical presentation of intrahepatic and extrahepatic aneurysms appears to be similar, they can be considered together.

There is no standardization for the treatment of portal vein aneurysms because treatment has been based on anecdotal reports. The main questions that result are: what is the natural history of portal vein aneurysms, and how does it compare to the risks of iatrogenic complications? The lack of cohort or controlled studies prevent us from answering these questions. Except in life-threatening situations such as rupture and acute thrombosis or in symptomatic cases such as compression of adjacent viscera, there is no consensus on whether patients with portal vein aneurysms should be treated or followed. When these patients are followed, a progressive increase in aneurysm diameter constitutes an empiric indication for surgery.

When treatment is indicated, the direct surgical approach consists of aneurysmorrhaphy or aneurysm resection. Portal decompression procedures are effective if the aneurysm is caused by portal hypertension.^3,9 This technique is straightforward in the case of noncirrhotic portal hypertension but complicated in cirrhotic patients, due to increased surgical risk and to technical issues regarding future liver transplantation for these patients. For decompensated cirrhotic patients, it is best to wait for liver transplant. Surgical risk is also increased for compensated cirrhotics, so surgery should be balanced against the natural history of portal vein aneurysms. A recent case study of radiologic treatment of a hemorrhagic aneurysm further corroborates the need for conservative management, if radiologic invasive procedures are available to the patient.¹⁰ On the other hand, some gastroenterologists advocate a more aggressive approach to aneurysms associated with portal hypertension because these cases are potentially more predisposed to rupture and bleeding.³

For the sake of therapeutic discussion, the portal vein aneurysms discussed above can arbitrarily be classified as complicated, whereas portal vein aneurysms that are asymptomatic or cause tolerable symptoms can be classified as uncomplicated. Most patients with uncomplicated aneurysms have been followed conservatively, but some patients have been operated prophylactically.^11–12 The follow-up of 10 years in the case described by Rafiq and Sitrin, in addition to the fact that many of the reported cases consist of incidental findings, aid in defending the perspective that uncomplicated portal vein aneurysms can be managed conservatively.

Therefore, at this time, and probably for a long time hereafter, the management of uncomplicated portal vein aneurysms must remain individualized. Gastroenterologists must take into account the individual surgical risk and make a decision in conjunction with the patient, as appropriately demonstrated by Rafiq and Sitrin.