Pediatr Surg Int DOI 10.1007/s00383-014-3472-3
CASE REPORT
Surgical management of hepatic arterioportal fistula in a neonate Priya Ramachandran • N. P. Shanmugam M. Vij • M. Rela
•
Accepted: 9 January 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Congenital arterioportal fistulae in the liver are rare malformations which can lead to portal hypertension. We report a hepatic arterioportal fistula in a neonate who presented with intestinal hypoperfusion. Computerised tomography angiography showed a fistulous communication between the left hepatic artery and portal vein with hypoperfusion of small and large bowel. A formal left hepatectomy was done followed by clinical improvement and reduction in portal venous pressures. The case and the literature pertaining to it are discussed. Keywords Arterioportal fistulae Intestinal hypoperfusion Intestinal hypoxia
Introduction Fistulous communications between the hepatic artery and portal vein can lead to increased portal pressures with reversal of flow in the main portal vein [1]. There are reports in the literature of infants and older children presenting with features of portal hypertension (PH) [2]. We report here a neonate with hepatic arterioportal fistula (HAPF) who had shunting of blood from the aorta through the celiac axis and
P. Ramachandran (&) Department of Pediatric Surgery, Kanchi Kamakoti Child Trust Hospital and Child Trust Medical Research Foundation, 12A, Nageswara Road, Nungambakkam, Chennai 600034, India e-mail: [email protected] P. Ramachandran N. P. Shanmugam M. Vij M. Rela Department of Hepatobiliary Surgery and Liver Transplantation and National Foundation for Liver Research, Global Health City, Chennai, India
hepatic artery into the low resistance fistulous communication leading to mesenteric hypoperfusion.
Case report A 2.7 kg male presented on day 17 of life with a history of decreased activity, refusal of feeds, loose stools, non-bilious vomiting and weight loss. He was born to a 19-yearold mother whose antenatal history was uneventful. On clinical examination, he was dehydrated and lethargic but hemodynamically stable. He had abdominal distension and tenderness. The liver was palpable to 4 cm below the costal margin. The spleen was also palpable. His basic blood parameters were normal and his initial liver functional test showed total bilirubin of 6.1 with direct of 1.1, aspartate transaminase of 68, alanine transaminase of 113 and serum alkaline phosphate 439. He continued to have ileus with a large amount of bilious nasogastric aspirate and bloodstained stools. Ultrasound examination showed an anomalous markedly ectatic and tortuous vein arising from the left branch of the portal vein. After initial stabilization, a 320-slice triphasic CT of the abdomen was performed which showed an AV fistula between the left hepatic artery and the portal vein with reduction in caliber of abdominal aorta and iliac and mesenteric arteries, distal to the celiac origin and hypoperfusion of the large and small bowel (Fig. 1). During laparotomy, an arteriovenous malformation was seen in segment 4 near the Rex recess. Two hepatic arteries were identified supplying the left lobe, one arising from the left gastric artery and the other from the common hepatic artery. Pressure in the main portal vein was measured at 25 mmHg. The pressure remained the same after clamping both arteries. A formal left hemihepatectomy
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Pediatr Surg Int
Fig. 1 CT angiogram showing aortoportal fistula with narrowing of abdominal aorta below probably due to steal phenomenon of blood going into low pressure portal system
was performed after which the pressure in the main portal vein fell to 12 mmHg. This indicated that, in addition to the main branches of the hepatic artery, there were segmental arterial communications with the portal venous system in the substance of the liver causing portal hypertension. The post-operative period was uneventful. An ultrasound prior to discharge showed normalization of aortic caliber after the celiac take-off, and the portal vein measured 3 mm in size with normal flow on Doppler (Fig. 2a, b). The malformative lesion, on histology, showed ectatic variably muscularized veins and arteries with marked fibrotic intimal thickening, interrupted elastic lamina and luminal narrowing. All these features were typical of a high pressure arteriovenous malformation (Fig. 3).
Discussion HAPF causes symptoms pertaining to mesenteric vascular congestion and portal hypertension like chronic diarrhea, malabsorption, variceal bleeding and ascites [2, 3]. Increased blood flow in the hepatic artery may contribute to hypoperfusion of distal organs resulting in a ‘‘steal’’ phenomenon with reduced blood flow in the superior and inferior mesenteric arteries [4]. Gryboski [4] described a HAPF causing decreased blood flow in the superior mesenteric artery leading to fatal small bowel infarction in an
123
Fig. 2 a Ultrasonography of abdomen showing narrowed caliber abdominal aorta. b Normalization of aortic caliber post left hemihepatectomy
18-week-old infant. Percutaneous transarterial embolisation (TAE) using various materials has been attempted in many cases [5]. However, multiple sessions are required [6]. Definitive therapy aimed at reducing the portal hypertension and restoring mesenteric perfusion is possible only with surgical excision of HAPF followed by vascular repair, ligation of hepatic artery branches or partial hepatectomy [2]. Although the morbidity and mortality is less with TAE, selection of procedure depends on the complexity of the lesion. A classification has been proposed wherein unilateral lesions supplied by one branch (type 1) are easier to treat with TAE rather than bilateral and complex lesions (types 2, 3) [2]. Since our patient presented at 17 days of life, features of portal hypertension had not become established. The main symptoms of feed intolerance and ileus were pertaining to mesenteric hypoperfusion and ‘‘steal’’ phenomenon. Restoring the normal hemodynamics of blood flow and prevention of portal hypertension were the goals of our treatment. At 2.7 kg, the neonate was not considered suitable for TAE. During the operation it was clearly demonstrated that the portal pressures remained high even after occlusion of both arteries supplying the lesion. Only after
Pediatr Surg Int
Fig. 3 Abnormal artery like vascular structures (H&E, original magnification 940) (a). Abnormally fibrotic and muscularized vessel (H&E, original magnification 940) (b). Vessel with markedly thickened wall (H&E, original magnification 940) (c). Elastin Van
Gieson’s stain also shows the presence of thick-walled arterial vessels with disrupted internal elastic lamina (EVG, original magnification 940) (d)
classic left hemihepatectomy did the portal pressures normalize, probably because of anomalous communications within the substance of the liver. There was no morbidity from the procedure. Our case of HAPF in a neonate is the first of its kind to be reported in the literature.
2. Norton SP (2006) The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr 43:248–253 3. Alkim C (1999) A case report of congenital intrahepatic arterioportal fistula. Am J Gastroenterol 94:522–523 4. Gryboski JD (1967) Congenital hepatic artery aneurysm with superior mesenteric artery insufficiency: a steal syndrome. Pediatrics 39(2):344–347 5. Cil BE (2004) Transhepatic embolisation of a recanalised congenital hepatic arterioportal fistula with NBCA and coils. Cardiovasc Interv Radiol 27(2):172–174 6. Marchand V (1999) Congenital hepatic arterioportal fistula in a 3-year-old child. J Pediatr Gastroenterol Nutr 28(4):435–441
References 1. Billing JS (1997) Hepatic arterioportal fistula: a curable cause of portal hypertension in infancy. HPB Surg 10:311–314
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CASE REPORT
Surgical management of hepatic arterioportal fistula in a neonate Priya Ramachandran • N. P. Shanmugam M. Vij • M. Rela
•
Accepted: 9 January 2014 Ó Springer-Verlag Berlin Heidelberg 2014
Abstract Congenital arterioportal fistulae in the liver are rare malformations which can lead to portal hypertension. We report a hepatic arterioportal fistula in a neonate who presented with intestinal hypoperfusion. Computerised tomography angiography showed a fistulous communication between the left hepatic artery and portal vein with hypoperfusion of small and large bowel. A formal left hepatectomy was done followed by clinical improvement and reduction in portal venous pressures. The case and the literature pertaining to it are discussed. Keywords Arterioportal fistulae Intestinal hypoperfusion Intestinal hypoxia
Introduction Fistulous communications between the hepatic artery and portal vein can lead to increased portal pressures with reversal of flow in the main portal vein [1]. There are reports in the literature of infants and older children presenting with features of portal hypertension (PH) [2]. We report here a neonate with hepatic arterioportal fistula (HAPF) who had shunting of blood from the aorta through the celiac axis and
P. Ramachandran (&) Department of Pediatric Surgery, Kanchi Kamakoti Child Trust Hospital and Child Trust Medical Research Foundation, 12A, Nageswara Road, Nungambakkam, Chennai 600034, India e-mail: [email protected] P. Ramachandran N. P. Shanmugam M. Vij M. Rela Department of Hepatobiliary Surgery and Liver Transplantation and National Foundation for Liver Research, Global Health City, Chennai, India
hepatic artery into the low resistance fistulous communication leading to mesenteric hypoperfusion.
Case report A 2.7 kg male presented on day 17 of life with a history of decreased activity, refusal of feeds, loose stools, non-bilious vomiting and weight loss. He was born to a 19-yearold mother whose antenatal history was uneventful. On clinical examination, he was dehydrated and lethargic but hemodynamically stable. He had abdominal distension and tenderness. The liver was palpable to 4 cm below the costal margin. The spleen was also palpable. His basic blood parameters were normal and his initial liver functional test showed total bilirubin of 6.1 with direct of 1.1, aspartate transaminase of 68, alanine transaminase of 113 and serum alkaline phosphate 439. He continued to have ileus with a large amount of bilious nasogastric aspirate and bloodstained stools. Ultrasound examination showed an anomalous markedly ectatic and tortuous vein arising from the left branch of the portal vein. After initial stabilization, a 320-slice triphasic CT of the abdomen was performed which showed an AV fistula between the left hepatic artery and the portal vein with reduction in caliber of abdominal aorta and iliac and mesenteric arteries, distal to the celiac origin and hypoperfusion of the large and small bowel (Fig. 1). During laparotomy, an arteriovenous malformation was seen in segment 4 near the Rex recess. Two hepatic arteries were identified supplying the left lobe, one arising from the left gastric artery and the other from the common hepatic artery. Pressure in the main portal vein was measured at 25 mmHg. The pressure remained the same after clamping both arteries. A formal left hemihepatectomy
123
Pediatr Surg Int
Fig. 1 CT angiogram showing aortoportal fistula with narrowing of abdominal aorta below probably due to steal phenomenon of blood going into low pressure portal system
was performed after which the pressure in the main portal vein fell to 12 mmHg. This indicated that, in addition to the main branches of the hepatic artery, there were segmental arterial communications with the portal venous system in the substance of the liver causing portal hypertension. The post-operative period was uneventful. An ultrasound prior to discharge showed normalization of aortic caliber after the celiac take-off, and the portal vein measured 3 mm in size with normal flow on Doppler (Fig. 2a, b). The malformative lesion, on histology, showed ectatic variably muscularized veins and arteries with marked fibrotic intimal thickening, interrupted elastic lamina and luminal narrowing. All these features were typical of a high pressure arteriovenous malformation (Fig. 3).
Discussion HAPF causes symptoms pertaining to mesenteric vascular congestion and portal hypertension like chronic diarrhea, malabsorption, variceal bleeding and ascites [2, 3]. Increased blood flow in the hepatic artery may contribute to hypoperfusion of distal organs resulting in a ‘‘steal’’ phenomenon with reduced blood flow in the superior and inferior mesenteric arteries [4]. Gryboski [4] described a HAPF causing decreased blood flow in the superior mesenteric artery leading to fatal small bowel infarction in an
123
Fig. 2 a Ultrasonography of abdomen showing narrowed caliber abdominal aorta. b Normalization of aortic caliber post left hemihepatectomy
18-week-old infant. Percutaneous transarterial embolisation (TAE) using various materials has been attempted in many cases [5]. However, multiple sessions are required [6]. Definitive therapy aimed at reducing the portal hypertension and restoring mesenteric perfusion is possible only with surgical excision of HAPF followed by vascular repair, ligation of hepatic artery branches or partial hepatectomy [2]. Although the morbidity and mortality is less with TAE, selection of procedure depends on the complexity of the lesion. A classification has been proposed wherein unilateral lesions supplied by one branch (type 1) are easier to treat with TAE rather than bilateral and complex lesions (types 2, 3) [2]. Since our patient presented at 17 days of life, features of portal hypertension had not become established. The main symptoms of feed intolerance and ileus were pertaining to mesenteric hypoperfusion and ‘‘steal’’ phenomenon. Restoring the normal hemodynamics of blood flow and prevention of portal hypertension were the goals of our treatment. At 2.7 kg, the neonate was not considered suitable for TAE. During the operation it was clearly demonstrated that the portal pressures remained high even after occlusion of both arteries supplying the lesion. Only after
Pediatr Surg Int
Fig. 3 Abnormal artery like vascular structures (H&E, original magnification 940) (a). Abnormally fibrotic and muscularized vessel (H&E, original magnification 940) (b). Vessel with markedly thickened wall (H&E, original magnification 940) (c). Elastin Van
Gieson’s stain also shows the presence of thick-walled arterial vessels with disrupted internal elastic lamina (EVG, original magnification 940) (d)
classic left hemihepatectomy did the portal pressures normalize, probably because of anomalous communications within the substance of the liver. There was no morbidity from the procedure. Our case of HAPF in a neonate is the first of its kind to be reported in the literature.
2. Norton SP (2006) The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr 43:248–253 3. Alkim C (1999) A case report of congenital intrahepatic arterioportal fistula. Am J Gastroenterol 94:522–523 4. Gryboski JD (1967) Congenital hepatic artery aneurysm with superior mesenteric artery insufficiency: a steal syndrome. Pediatrics 39(2):344–347 5. Cil BE (2004) Transhepatic embolisation of a recanalised congenital hepatic arterioportal fistula with NBCA and coils. Cardiovasc Interv Radiol 27(2):172–174 6. Marchand V (1999) Congenital hepatic arterioportal fistula in a 3-year-old child. J Pediatr Gastroenterol Nutr 28(4):435–441
References 1. Billing JS (1997) Hepatic arterioportal fistula: a curable cause of portal hypertension in infancy. HPB Surg 10:311–314
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