Multidisciplinary Approach to Congenital Multiple Arterio-Porto-Caval Malformation: Case Report

Abstract

A case of intrahepatic arteriovenous malformation connected with umbilical, hepatic, portal, and arterial systems in a fetus diagnosed at 36 weeks of gestation is presented. Prenatal color Doppler ultrasonography in the fetal liver demonstrated complicated vascular connections fed by arterial and portal branches. Postnatal color Doppler, power Doppler, 3D power Doppler, abdominal multidetector computed tomography (MDCT), and angiography CT verified the prenatal diagnoses. Extended right hepatectomy was performed successfully on postnatal day 19.

Keywords

Arteriovenous malformation - doppler ultrasound - 3-D ultrasound - MDCT - hepatic vascular abnormalities - management

Publication History

Publication Date:
02 April 2008 (online)

• REFERENCES

• 1 Lima M, Lalla M, Aquino A, Dòmini M. et al. Congenital symptomatic intrahepatic arteriovenous fistulas in newborns: management of 2 cases with prenatal diagnosis. J Pediatr Surg 2005; 40: e1-e5
• 2 Prefumo F, Thilaganathan B, Tekay A. Antenatal diagnosis of fetal intra-abdominal umbilical vein dilatation. Ultrasound Obstet Gynecol 2001; 17: 82-85
• 3 Fung TY, Leung TN, Leung TY, Lau TK. Fetal intra-abdominal umbilical vein varix: what is the clinical significance?. Ultrasound Obstet Gynecol 2005; 25: 149-154
• 4 Sepulveda W, Mackenna A, Sanchez J, Corral E, Carstens E. Fetal prognosis in varix of the intrafetal umbilical vein. J Ultrasound Med 1998; 17: 171-175
• 5 Gembruch U, Baschat AA, Gloeckner-Hoffmann K, Gortner L, Germer U. Prenatal diagnosis and management of fetuses with liver hemangiomata. Ultrasound Obstet Gynecol 2002; 19: 454-460
• 6 Mejides AA, Adra AM, O'Sullivan MJ, Nicholas MC. Prenatal diagnosis and therapy for fetal hepatic vascular malformation. Obstet Gynecol 1995; 85: 850-853
• 7 Tseng JJ, Chou MM, Lee YH, Ho ESC. Prenatal diagnosis of intrahepatic arteriovenous shunts. Ultrasound Obstet Gynecol 2000; 15: 441-444
• 8 Hartung J, Chaoui R, Kalache K, Tennstedt C, Bollmann R. Prenatal diagnosis of intrahepatic communications of the umbilical vein with atypical arteries (A-V fistulae) in two cases of trisomy 21 using color Doppler ultrasound. Ultrasound Obstet Gynecol 2000; 16: 271-274
• 9 Achiron R, Hegesh J, Yagel S, Lipitz S, Cohen SB, Rotstein Z. Abnormalities of the fetal central veins and umbilico-portal system: prenatal ultrasonographic diagnosis and proposed classification. Ultrasound Obstet Gynecol 2000; 16: 539-548
• 10 Viora E, Sciarrone A, Bastonero S. et al. Anomalies of the fetal venous system: a report of 26 cases and review of the literature. Fetal Diagn Ther 2004; 19: 440-447
• 11 Kalache K, Romero R, Goncalves LF. et al. Three-dimensional color power imaging of the fetal hepatic circulation. Am J Obstet Gynecol 2003; 189: 1401-1406
• 12 Moore KL, Persaud TVN. The Developing Human. Clinically Oriented Embryology. Philadelphia: WB Saunders; 1998: 350-355
• 13 Paley MR, Farrant P, Kane P, Heaton ND, Howard ER, Karani JB. Developmental intrahepatic shunts of childhood: radiological features and management. Eur Radiol 1997; 7: 1377-1382
• 14 Agarwala S, Dutta H, Bhatnagar V, Gulathi M, Paul S, Mitra D. Congenital hepatoportal arteriovenous fistula: report of a case. Surg Today 2000; 30: 268-271
• 15 Hashimoto M, Tate E, Nishii T, Watarai J, Shioya T, White RI. Angiography of hepatic vascular malformations associated with hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol 2003; 26: 177-180
• 16 Luks FI, Yazbeck S, Brandt ML, Bensoussan AL, Brochu P, Blanchard H. Benign liver tumors in children: a 25-year experience. J Pediatr Surg 1991; 26: 1326-1330
• 17 Fasouliotis SJ, Achiron R, Kivilevitch Z, Yagel S. The human fetal venous system: normal embryologic, anatomic, and physiologic characteristics and developmental abnormalities. J Ultrasound Med 2002; 21: 1145-1158
• 18 Hofstaetter C, Plath H, Hansmann M. Prenatal diagnosis of abnormalities of fetal venous system. Ultrasound Obstet Gynecol 2000; 15: 231-241
• 19 Gallego C, Miralles M, Marin C, Muyor P, Gonzales G, Garcia-Hidalgo E. Congenital hepatic shunts. Radiographics 2004; 24: 755-772
• 20 Adaletli I, Kurugoglu S, Kilic F, Senyuz OF, Dervisoglu S. Radiological evaluation with Doppler sonography and multidetector CT angiography in congenital hepatic arteriovenous malformation in a newborn. Pediatr Radiol 2006; 36: 1204-1207
• 21 Chavan A, Caselitz M, Gratz KF, Lotz J. et al. Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations. Eur Radiol 2004; 14: 2079-85
• 22 Jouannic JM, Jacquemard F, Mirlesse V. et al. Intrahepatic arteriovenous fistula. Prenatal diagnosis, physiopathological study and neonatal management. [in French] J Gynecol Obstet Biol Reprod (Paris) 1998; 27: 90-94
• 23 Rouveyrol F, Meyer M, Lusson JR, Gaspard F, Lachaux A, Stephan JL. Idiopathic intrahepatic portosystemic shunts in 4 children. [in French] Arch Pediatr 2006; 13: 251-255