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Hepatic artery ligation for cardiac failure due to hepatic hemangioma in the newborn
Hepatic Artery Ligation for Cardiac Failure Due to Hepatic Hemangioma in the Newborn By Leone Mattioli, Kyo Rak Lee, and Thomas M. Holder
EPATIC VASCULAR TUMORS may lead to cardiac failure in the neonatal period with the mechanism of large systemic arteriovenous fistulas and when treated with anticongestive measures alone have had poor prognosis) Among the recognized modes of therapy 2-4 aiming at reducing the size of the shunt, ligation of the common hepatic artery has been successfully employed in previously reported cases without damage to the liver parenchyma) '5 We have recently treated a newborn of 6 wk of age with intractable cardiac failure, due to hepatic hemangioma, by this technique. The common hepatic artery was ligated proximal to the gastroduodenal artery, and the infant underwent a remarkable clinical and hemodynamic improvement. There was, in addition, complete resolution of the intrahepatic arteriovenous connections as shown by arteriography. There were no duodenal or liver complications. At 2 yr of age the infant was alive and growing normally. Ligation of the common hepatic artery is a simple and safe procedure, which should be employed more often and may become the procedure of choice in the treatment of hepatic hemangioma.
H
CASE REPORT A 27-day-old female white infant was admitted to K.U.M.C because of feeding difficulty, tachypnea, mild cyanosis, and hepatomega!y. A Grade I11 precordial systolic murmur was present shortly after birth. On admission (June 29, 1971) physical examination revealed a mildly cyanosed infant iu acute distress. Respiratory rate was 80/min, and heart rate, 130/rain. The precordiam was hyperactive with a right venti:icular heave. There was a Grade III/1V systolic ejection murmur at the left upper sternal border. SI was normal and $2 was closely split. P2 was accentuated. No rules were heard. The liver was palpable 4icm below the right costal margin. A loud continuous bruit was heard over the entire liver. No cutaneous hemangiomas were noted on the skin. Routine laboratory studies were within normal limits, The electrocardiogram showed right axis deviation, biventricular hypertrophy, and first degree AV block. The chest roentgenogram showed cardiac enlargement With pulmonary plethora. She was digitalized and underwent cardiac catheterization on June 30, 1971. Mild pulmonary hypertension was present without other significant abnormalities (Table 1). Since the liver was enlarging progressively to 7 cm below the costal margin, hepatic arteriography was performed on July 3, 1971. The arteriogram showed a large common hepatic artery with dilated hepatic branches and prolonged pooling of contrast material in multiple vascular lakes in both lobes of the liver (Fig. IA). An early drainage of the dilated hepatic vein was seen during the late arterial phase (Fig. 1B). Because of the progressively downhill course, hepatic artery ligation was performed (TH) on July 4, 1971. At operation, multiple hemangiomas diffusely involving the liver were found. A very large common hepatic artery was ligated proximal to the gastroduodenal artery. No thrills were palpable distal to the ligation. The infant showed a dramatic improvement postoperatively. Within
From Departments of Pediatrics, Diagnostic Radiology, and Surgery, University of Kansas Medical Center, Kansas City, Kansas. Address for reprint requests: Leone MattiolL M.D., University of Kansas Medical Center, 39th and Rainbow Blvd., Kansas City, Kansas 66103. 9 1974 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vok 9, No. 6 (December), 1974
859
860
MATTIOLI, LEE, AND HOLDER
Table 1. Cardiac Catheterization Preop. 6/30/71 (30 Days of Age) Site Superior vena cava Right atrium Azygos vein Hepatic vein Right ventricle Pulmonary artery Wedge Left ventricle Aorta
Pressures (mm Hg)
Oxygen Saturation (%)
Postop. 3/20/72 (9 Mo of Age) Pressures (ram Hg)
Oxygen Saturation (%)
(3)*
79 75
63 (8)* 61
77
50/8 40/20
64
90/8 90/50*(80)
91 89
28/4 24/12 5
75 73
105/6,5*(80)
94
*Figures in parentheses represent mean pressures.
24 hr the respiratory and pulse rates fell to normal. There was no audible bruit over the liver. The precordial murmur had also disappeared. The liver enzymes (SGOT, SGPT), and bilirubin were measured daily for 4 days and were normal. On July 14, 1971 she was discharged from the hospital without medications. At 9 mo. of age, she showed a normal growth. Weight was 8.3 kg and height, 73 cm. The Physical examination was entirely normal, except for a small cutaneous hemangioma measuring 1.0 x 1.5 cm over the right upper abdomen. The electrocardiogram and chest x-ray were normal. Repeat cardiac catheterization and hepatic arteriography were performed. The hemodynamic findings were normal (Table 1). The arteriogram demonstrated a complete resolution o f the hemangiomas (Fig. 2A). The right and left hepatic arteries were normally opacified through the collateral routes o f the left gastric artery and the pancreaticoduodenal arteries (Fig. 2B). At the age of 2, the child continued to grow well at the 15th percentile for height and 25th percentile for weight. There was no clinical evidence o f recurrence of the hepatic hemangioma.
Fig. 1. Preoperative hepatic arteriogram demonstrates multiple capillary hemangiomas diffusely in the liver. (A) Arterial phase shows the dilated common hepatic artery (closed arrows) and intrahepatic branches Iopen arrows). (B) The hepatic veins are densely opacified and dilated, indicating an extensive arteriovenous shunting.
HEPATIC ARTERY LIGATION
861
RIGHT INFERIOR PHRENICARTER7 Y GASTRO'-DUODENAL ARTERY\ ,.~,~
LEFT.EPAT,CARTERY\\
MIDDLE HEPATICARTERY ~ ~
RIGHT HEPATICAR E R Y ~
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.
f
r'- '~
\
I 4
~"
"~
./4r~:,
/~
/=
LEFT INFERIOR PHRENIC ARTERY
'
"~"~
AC ARTERY
/ ANTERIOR ond POST RIOR
PANCREAT~O-OUOOENAL ARTERY
~GHT~ c
LEFT RENAL ARTERY
....9
/E
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~ T E R Y ~ }~
Fig. 2. (A) Postoperative hepatic arteriogram demonstrates a complete resolution of the hemangiomas. (B) Schematic drawing shows excellent collaterals from the left gastric and the pancreaticoduodenal arteries to the proper hepatic, right and left hepatic arteries. DISCUSSION
Large hepatic arteriovenous fistulas in infants are exclusively caused by hemangioendotheliomas. ~ They lead to progressive circulatory and cardiac overload with a mechanism which is common to other peripheral arteriovenous fistulas. The heart is subjected to increasing volume overload which, unlike the aortopulmonary connections, is independent of pulmonary vascular resistance changes. Symptoms of circulatory embarrassment are, therefore, likely to appear shortly after birth. 2
862
MATTIOLI, LEE, AND HOLDER
Clinically, the diagnosis of hepatic hemangiomatosis is suspected by auscultating a continuous or systolic bruit over the enlarged liver and confirmed by arteriography. Characteristic arteriographic findings are abnormal arterial vessels fed by an enlarged hepatic artery, pooling of contrast material, and an early hepatovenous drainage. 6 Digitalis and diuretics are of little avail. In a series of 25 cases gathered in the literature, ~ there were three spontaneous cures related to spontaneous involution of the tumor. Various means have been employed to reduce the size of the fistulas which include radiation, hepatic lobectomy, cortisone, hepatic artery ligation. 3,5,7 The ligation of the common hepatic artery has been suggested as the most appropriate method of therapy for diffuse hepatic hemagiomas) '5 As the spectrum of operative indications has broadened, hepatic artery ligation has been employed with increasing frequency) Liver function as measured by the activity of SGOT, SGPT, LDH, and bilirubin has not been compromised in the previously reported cases as well as in our own. In patients subjected to ligation of the hepatic artery proper, for the purpose of reducing oxygen supply to hepatic tumor cells, the activity of SGOT, SGPT, and L D H increased transiently but returned to the normal level after 1 wk. 9 Increasing evidence suggests that the arterial blood supply to the liver is not essential to the survival of the liver parenchyma when blood flow and oxygen content of the portal vein are adequately maintained) ~ Thus~ low-flow states should be carefully avoided in the postoperative period after hepatic artery ligation. SUMMARY
A 6-wk-old infant born with large hepatic hemangioma underwent successful ligation of the common hepatic artery with marked clinical and hemodynamic improvement of her profound cardiac failure. No alteration of liver enzymes was noted. At 2 yr of age, this infant is clinically normal. We feel on the basis of this case and of two other previously reported cases that ligation of the common hepatic artery is a safe and effective means of therapy for cardiac failure due to diffuse intrahepatic arteriovenous fistulas in the newborn. REFERENCES 1. Delorimer AA, Simpson EB, Baum RS, et al: Hepatic artery ligation for hepatic hemangiomatosis. N Engl J Med 277:333-337, 1967 2. Leonidas JC, Strauss L, Beck AR: Vascular tumors of the liver in newborns: A pediatric emergency. Am J Dis Child 125:505-510, 1973 3. Graivier L; Votteler TP, Dorman GW: Hepatic hemangiomas in newborn infants. J Pediatr Sarg 2: 299-307, 1967 4. Tawes RL Jr, Nelson JA, Hyde GA Jr: Hepatic hemangioma: Successful resection in a neonate. Surgery 70: 782-785, 1971 5. Rake MO, Liberman MM, Dawson JL, et al: Ligation of the hepatic artery in the treatment ot heart failure due to hepatic hemangiomatosis. Gut 11: 512-515, 1970
6. Moss AA, Clark RE, Palubinskas AT, et al: Angiographic appearance of benign and malignant hepatic tumors in infants and children. Am J Roentgenol Radium Ther Nucl Med 113:6i-69, 1971 7. Darte JM: Radiation therapy in childhood. Prog Radiat Ther 3: 141-171, 1965 8. Madding GF, Kennedy PA: Hepatic artery ligation. Surg Clin North Am 52:3: 7t9728, 1972 9. Almersjo O, Bengmark S, Engevik L, et al: Serum enzyme changes after hepatic dearterialization in man. Ann Surg 167: 9-17, 1968 10. Mays ET: Observations and management after hepatic artery ligation. Surg Gynecol Obstet 124".801-807, 1967
EPATIC VASCULAR TUMORS may lead to cardiac failure in the neonatal period with the mechanism of large systemic arteriovenous fistulas and when treated with anticongestive measures alone have had poor prognosis) Among the recognized modes of therapy 2-4 aiming at reducing the size of the shunt, ligation of the common hepatic artery has been successfully employed in previously reported cases without damage to the liver parenchyma) '5 We have recently treated a newborn of 6 wk of age with intractable cardiac failure, due to hepatic hemangioma, by this technique. The common hepatic artery was ligated proximal to the gastroduodenal artery, and the infant underwent a remarkable clinical and hemodynamic improvement. There was, in addition, complete resolution of the intrahepatic arteriovenous connections as shown by arteriography. There were no duodenal or liver complications. At 2 yr of age the infant was alive and growing normally. Ligation of the common hepatic artery is a simple and safe procedure, which should be employed more often and may become the procedure of choice in the treatment of hepatic hemangioma.
H
CASE REPORT A 27-day-old female white infant was admitted to K.U.M.C because of feeding difficulty, tachypnea, mild cyanosis, and hepatomega!y. A Grade I11 precordial systolic murmur was present shortly after birth. On admission (June 29, 1971) physical examination revealed a mildly cyanosed infant iu acute distress. Respiratory rate was 80/min, and heart rate, 130/rain. The precordiam was hyperactive with a right venti:icular heave. There was a Grade III/1V systolic ejection murmur at the left upper sternal border. SI was normal and $2 was closely split. P2 was accentuated. No rules were heard. The liver was palpable 4icm below the right costal margin. A loud continuous bruit was heard over the entire liver. No cutaneous hemangiomas were noted on the skin. Routine laboratory studies were within normal limits, The electrocardiogram showed right axis deviation, biventricular hypertrophy, and first degree AV block. The chest roentgenogram showed cardiac enlargement With pulmonary plethora. She was digitalized and underwent cardiac catheterization on June 30, 1971. Mild pulmonary hypertension was present without other significant abnormalities (Table 1). Since the liver was enlarging progressively to 7 cm below the costal margin, hepatic arteriography was performed on July 3, 1971. The arteriogram showed a large common hepatic artery with dilated hepatic branches and prolonged pooling of contrast material in multiple vascular lakes in both lobes of the liver (Fig. IA). An early drainage of the dilated hepatic vein was seen during the late arterial phase (Fig. 1B). Because of the progressively downhill course, hepatic artery ligation was performed (TH) on July 4, 1971. At operation, multiple hemangiomas diffusely involving the liver were found. A very large common hepatic artery was ligated proximal to the gastroduodenal artery. No thrills were palpable distal to the ligation. The infant showed a dramatic improvement postoperatively. Within
From Departments of Pediatrics, Diagnostic Radiology, and Surgery, University of Kansas Medical Center, Kansas City, Kansas. Address for reprint requests: Leone MattiolL M.D., University of Kansas Medical Center, 39th and Rainbow Blvd., Kansas City, Kansas 66103. 9 1974 by Grune & Stratton, Inc. Journal of Pediatric Surgery, Vok 9, No. 6 (December), 1974
859
860
MATTIOLI, LEE, AND HOLDER
Table 1. Cardiac Catheterization Preop. 6/30/71 (30 Days of Age) Site Superior vena cava Right atrium Azygos vein Hepatic vein Right ventricle Pulmonary artery Wedge Left ventricle Aorta
Pressures (mm Hg)
Oxygen Saturation (%)
Postop. 3/20/72 (9 Mo of Age) Pressures (ram Hg)
Oxygen Saturation (%)
(3)*
79 75
63 (8)* 61
77
50/8 40/20
64
90/8 90/50*(80)
91 89
28/4 24/12 5
75 73
105/6,5*(80)
94
*Figures in parentheses represent mean pressures.
24 hr the respiratory and pulse rates fell to normal. There was no audible bruit over the liver. The precordial murmur had also disappeared. The liver enzymes (SGOT, SGPT), and bilirubin were measured daily for 4 days and were normal. On July 14, 1971 she was discharged from the hospital without medications. At 9 mo. of age, she showed a normal growth. Weight was 8.3 kg and height, 73 cm. The Physical examination was entirely normal, except for a small cutaneous hemangioma measuring 1.0 x 1.5 cm over the right upper abdomen. The electrocardiogram and chest x-ray were normal. Repeat cardiac catheterization and hepatic arteriography were performed. The hemodynamic findings were normal (Table 1). The arteriogram demonstrated a complete resolution o f the hemangiomas (Fig. 2A). The right and left hepatic arteries were normally opacified through the collateral routes o f the left gastric artery and the pancreaticoduodenal arteries (Fig. 2B). At the age of 2, the child continued to grow well at the 15th percentile for height and 25th percentile for weight. There was no clinical evidence o f recurrence of the hepatic hemangioma.
Fig. 1. Preoperative hepatic arteriogram demonstrates multiple capillary hemangiomas diffusely in the liver. (A) Arterial phase shows the dilated common hepatic artery (closed arrows) and intrahepatic branches Iopen arrows). (B) The hepatic veins are densely opacified and dilated, indicating an extensive arteriovenous shunting.
HEPATIC ARTERY LIGATION
861
RIGHT INFERIOR PHRENICARTER7 Y GASTRO'-DUODENAL ARTERY\ ,.~,~
LEFT.EPAT,CARTERY\\
MIDDLE HEPATICARTERY ~ ~
RIGHT HEPATICAR E R Y ~
"
.
f
r'- '~
\
I 4
~"
"~
./4r~:,
/~
/=
LEFT INFERIOR PHRENIC ARTERY
'
"~"~
AC ARTERY
/ ANTERIOR ond POST RIOR
PANCREAT~O-OUOOENAL ARTERY
~GHT~ c
LEFT RENAL ARTERY
....9
/E
1 ('~
~ T E R Y ~ }~
Fig. 2. (A) Postoperative hepatic arteriogram demonstrates a complete resolution of the hemangiomas. (B) Schematic drawing shows excellent collaterals from the left gastric and the pancreaticoduodenal arteries to the proper hepatic, right and left hepatic arteries. DISCUSSION
Large hepatic arteriovenous fistulas in infants are exclusively caused by hemangioendotheliomas. ~ They lead to progressive circulatory and cardiac overload with a mechanism which is common to other peripheral arteriovenous fistulas. The heart is subjected to increasing volume overload which, unlike the aortopulmonary connections, is independent of pulmonary vascular resistance changes. Symptoms of circulatory embarrassment are, therefore, likely to appear shortly after birth. 2
862
MATTIOLI, LEE, AND HOLDER
Clinically, the diagnosis of hepatic hemangiomatosis is suspected by auscultating a continuous or systolic bruit over the enlarged liver and confirmed by arteriography. Characteristic arteriographic findings are abnormal arterial vessels fed by an enlarged hepatic artery, pooling of contrast material, and an early hepatovenous drainage. 6 Digitalis and diuretics are of little avail. In a series of 25 cases gathered in the literature, ~ there were three spontaneous cures related to spontaneous involution of the tumor. Various means have been employed to reduce the size of the fistulas which include radiation, hepatic lobectomy, cortisone, hepatic artery ligation. 3,5,7 The ligation of the common hepatic artery has been suggested as the most appropriate method of therapy for diffuse hepatic hemagiomas) '5 As the spectrum of operative indications has broadened, hepatic artery ligation has been employed with increasing frequency) Liver function as measured by the activity of SGOT, SGPT, LDH, and bilirubin has not been compromised in the previously reported cases as well as in our own. In patients subjected to ligation of the hepatic artery proper, for the purpose of reducing oxygen supply to hepatic tumor cells, the activity of SGOT, SGPT, and L D H increased transiently but returned to the normal level after 1 wk. 9 Increasing evidence suggests that the arterial blood supply to the liver is not essential to the survival of the liver parenchyma when blood flow and oxygen content of the portal vein are adequately maintained) ~ Thus~ low-flow states should be carefully avoided in the postoperative period after hepatic artery ligation. SUMMARY
A 6-wk-old infant born with large hepatic hemangioma underwent successful ligation of the common hepatic artery with marked clinical and hemodynamic improvement of her profound cardiac failure. No alteration of liver enzymes was noted. At 2 yr of age, this infant is clinically normal. We feel on the basis of this case and of two other previously reported cases that ligation of the common hepatic artery is a safe and effective means of therapy for cardiac failure due to diffuse intrahepatic arteriovenous fistulas in the newborn. REFERENCES 1. Delorimer AA, Simpson EB, Baum RS, et al: Hepatic artery ligation for hepatic hemangiomatosis. N Engl J Med 277:333-337, 1967 2. Leonidas JC, Strauss L, Beck AR: Vascular tumors of the liver in newborns: A pediatric emergency. Am J Dis Child 125:505-510, 1973 3. Graivier L; Votteler TP, Dorman GW: Hepatic hemangiomas in newborn infants. J Pediatr Sarg 2: 299-307, 1967 4. Tawes RL Jr, Nelson JA, Hyde GA Jr: Hepatic hemangioma: Successful resection in a neonate. Surgery 70: 782-785, 1971 5. Rake MO, Liberman MM, Dawson JL, et al: Ligation of the hepatic artery in the treatment ot heart failure due to hepatic hemangiomatosis. Gut 11: 512-515, 1970
6. Moss AA, Clark RE, Palubinskas AT, et al: Angiographic appearance of benign and malignant hepatic tumors in infants and children. Am J Roentgenol Radium Ther Nucl Med 113:6i-69, 1971 7. Darte JM: Radiation therapy in childhood. Prog Radiat Ther 3: 141-171, 1965 8. Madding GF, Kennedy PA: Hepatic artery ligation. Surg Clin North Am 52:3: 7t9728, 1972 9. Almersjo O, Bengmark S, Engevik L, et al: Serum enzyme changes after hepatic dearterialization in man. Ann Surg 167: 9-17, 1968 10. Mays ET: Observations and management after hepatic artery ligation. Surg Gynecol Obstet 124".801-807, 1967