PEDIATRIC SURGERY UPDATE ©

VOLUME 50 NO 03 MARCH 2018

Peliosis

Peliosis hepatis (PH) is a very rare benign disease characterized by multiple small blood-filled cysts of various sizes and shape  within the liver parenchyma. Peliosis comes from the Greek word ‘pelios' that means reddish or bluish (extravasated blood). What triggers PH is unknown, but dilation of sinusoids might be due to an altered outflow damaging the sinusoid wall and creating dilation of the central vein of the hepatic lobules. Peliosis can be focal or widespread with most cases involving the right hepatic lobe. Peliosis can also occur on other organs such as the spleen, bone marrow, lymph nodes, etc. Etiologic factors associated with PH include drugs, autoimmune mechanisms and infectious causes. Drugs associated with PH include steroids, oral contraceptives, tamoxifen, methotrexate, thiopurine, azathioprine and iron chelators. Alcohol consumption can trigger PH. Imaging studies such as CT-Scan and MRI angiography suggest the diagnosis, but cannot be precised enough since PH cannot be differentiated from adenomas, hemangiomas, focal nodular hyperplasia, Caroli disease or multiple abscess. Lesions are from few millimeters in diameter to 4 cm. Ultrasound may show a pseudocystic lesion of the hepatic parenchyma with intra- or perilesional vascularity. Angiography demonstrates multiple hypervascularized nodules during the late arterial phase with enhancement more pronounced during the parenchymal phase which persists during the venous phase. MRI when combined with hepatospecific contrast material represents the gold standard for radiological diagnosis of PH. Due to the high risk of bleeding an open biopsy using intraoperative US is needed to establish the diagnosis of PH. The disease can cause stenosis of the vena cava when developed in young age. Though mainly asymptomatic, PH can rupture and produce spontaneous hemoperitoneum. Management is usually in the acute setting due to bleeding and consists of either hepatic lobectomy, transplantation or percutaneous embolization.

References:
1- Crocetti D, Palmieri A, Pedulla G, Pasta V, D'Orazi V, Grazi GL: Peliosis hepatis: Personal experience and literature review. World J Gastroenterol. 21(46):13188-94, 2015
2- Hong GS, Kim KW, An J, Shim JH, Kim J, Yu ES: Focal type of peliosis hepatis. Clin Mol Hepatol. 21(4):398-401, 2015
3- Iwata T, Adachi K, Takahashi M: Peliosis Hepatis Mimicking Malignant Hypervascular Tumors. J Gastrointest Surg. 21(6):1095-1098, 2017
4- Dai YN, Ren ZZ, Song WY, Huang HJ, Yang DH, Wang MS, Huang YC, Chen MJ, Zhang JJ, Tong YX, Pan HY: Peliosis hepatis: 2 case reports of a rare liver disorder and its differential
diagnosis. Medicine (Baltimore). 96(13):e6471, 2017
5- Tan CHN, Soon GST, Kow WCA: Liver lesions detected in a hepatitis B core total antibody-positive patient masquerading as hepatocellular carcinoma: a rare case of peliosis hepatis and a review of the literature. Ann Hepatobiliary Pancreat Surg. 21(3):157-162, 2017
6- Biswas S, Gogna S, Patel P: A Fatal Case of Intra-Abdominal Hemorrhage Following Diagnostic Blind Percutaneous Liver Biopsy in a Patient With Peliosis Hepatis. Gastroenterology Res. 10(5):318-321, 2017

        Prophylactic Thyroidectomy

*Edited by: Humberto Lugo-Vicente, MD, FACS, FAAP
P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico 00922-0426
Tel (787)-999-9450 Fax (787)-720-6103 E-mail: TITOLUGO@COQUI.NET
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Pediatric Surgery Update ISSN 1089-7739
Last updated: April 2017