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Case Reports Indian Pediatrics 2000;37: 780-784 |
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Infantile Hepatic Hemangio-endothelioma: Treatment by Coil Embolization of the Hepatic Artery |
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Shyam S. Kothari
Arteriovenous malformations are an uncommon but important cause of high output heart failure in infants. In appropriate cases a surgical or catheter based intervention directed at the malformation may result in cure of the heart failure. Infantile Hepatic Hemangio-endothelioma (IHH) is one such disorder where the heart failure may be life threatening during early infancy. We report a case of IHH in view of its rarity and discuss the management. To the best of our knowledge, coil embolization of the hepatic artery has not been previously reported in Indian literature.
A 3½-month-old male infant presented with poor feeding and tachypnea of one month duration. Examination revealed an irritable child weighing 4.4 kg, in respiratory distress with a respiratory rate of about 60/min. The heart rate was 130/min and the pulse was high volume. The neck veins were full but the JVP was not ascertained. There was cardiomegaly, a grade 3/6 ejection systolic murmur over the left precordium and a left ventricular third heart sound. There were fine crackles over both the lungs. Cutaneous hemangiomas on the chest, back and ear lobes were noted. The liver was massively enlarged, measuring about 13 cm below the costal margin. The chest X-ray showed cardiomegaly and pulmonary venous congestion. The EKG was normal except for sinus tachycardia. The hemoglobin was 10 g/dL and the total and differential counts were normal. There was evidence of prerenal azotemia (blood urea of 55 mg/dl and serum creatinine of 0.5 mg/dL). Serum bilirubin and liver enzymes were normal. An echocardiogram showed left and right ventricular dilatation. The LV end-diastolic and end-systolic dimensions were 25 and 14 mm, respectively. Left ventricular contractility was normal. The valves were normal and the atrial and ventricular septae were intact. There was no PDA or coarctation. Contrast echo did not show any evidence of pulmonary arterio-venous fistula. An ultra-sound of the abdomen showed multiple hypo-echoic areas all over the liver with dilated hepatic venous channels. The portal vein was normal. This picture was consistent with the diagnosis of infantile hepatic hemangio-endothelioma (Fig. 1). The child was started on oral prednisolone at a dose of 24 mg on alternate days along with cefotaxime and amikacin for suspected lung infection. Decongestive therapy with parenteral frusemide was instituted. There was, however, no improvement in the patient’s condition and he was taken up for arteriography on the 9th day. Access was obtained through the right femoral artery and a 5F sheath was placed. After a non-selective descending aortogram with a pigtail catheter, celiac artery was hooked with a celiac artery catheter. Selective injection into the right hepatic artery was done by passing a 0.18 Tracker catheter through the celiac artery catheter into the right hepatic artery. This showed increased vascularity and tumor blush over the right lobe of liver. Three 0.18 coils were used to occlude the right hepatic artery. The left hepatic was not embolized at this time. The patient did not show significant improvement over the next seven days and therefore was taken up for embolization of the left hepatic artery which was done with two 0.18 coils. Selective injection into the right hepatic artery showed significant reduction in flow to the right lobe of liver and absence of the tumor blush (Fig. 2 ). After the second procedure, there was dramatic improvement in the child’s condition with significant reduction of the liver size. There was no elevation of liver enzymes and the child was discharged on the 6th post-procedure day. The child was doing well at three months after discharge. There was no evidence of congestive failure and an ultrasound of the abdomen showed remarkable regression of the malformation and the dilated hepatic venous channels (Fig. 3 ).
Fig. 1. Ultrasound of the abdomen showing multiple hypoechoic areas in the liver with dilated hepatic venous channels. This picture is characteristic of hepatic hemangioendothelioma.
Infantile hemangioendothelioma is the most common benign hepatic tumor in children, accounting for more than 50% of the cases(1). Despite being histologically benign, it carries a high mortality because of cardiac failure due to the massive arteriovenous shunting. The malformation typically presents in the first six months of life and is often associated with hemangiomas in other organs and tissues, especially the skin (in about 50% of cases). The triad of enlarged liver, high output cardiac failure and multiple cutaneous hemangiomas is usually diagnostic. Ultrasound shows diffuse involvement of both lobes of the liver by lesions which can vary from being anechoic to hyperechoic. The smaller malformations are usually asymptomatic; even the large ones regress after the first year of life. But mortality in the first year of life has been reported to be as high as 70%(2), largely because of the occurrence of high output cardiac failure. Oral or intravenous corticosteroids have been the mainstay of treatment(3). Holcomb and colleagues(4) reported a series of 16 cases of IHH seen over a period of 32 years, 11 of whom had received steroids either as sole therapy or along with another modality. Nine of these patients were alive and well at the time of their report. The mechanism of action of steroids in inducing tumor regression in this disorder is not known. Steroids have been postulated to restore reactivity of the immature endothelial cells to circulating catecholamines as shown in adrenalectomized rats(3). Radio-therapy has been used for the treatment of hepatic hemangioendothelioma in the past, but it’s efficacy is largely unsubstantiated. For those infants in whom the cardiac failure does not respond, as was the case with our patient, aggressive therapy is indicated. Hepatic artery ligation, first performed by deLorimier(5) has consistently been shown to be highly effective in ameliorating the congestive failure. More recently, selective hepatic arteriography and embolization has largely replaced surgical ligation. Wire coils, detachable balloons and polyvinyl alcohol have all been used(6). Hepatic artery occlusion does not generally cause liver necrosis because of adequate blood flow from the portal circulation. However, focal hepatic necrosis and subsequent disseminated intravascular coagulation has rarely been reported after occlusion of the hepatic artery either by surgical or catheter based techniques. Hepatic injury in this setting can be minimized by preventing hypotension in the immediate post-procedure period. Hepatic artery embolization is not useful when the tumor’s primary blood supply is from the portal system(4). The prevalence of this variation in tumor blood supply is not known and should be excluded by recording the venous phase of the superior mesenteric arteriogram after hepatic artery embolization. The optimal modality of therapy in such cases is not known. In conclusion, percutaneous, catheter-based techniques are highly successful in treating arteriovenous fistulae when technically feasible. The results of treatment can be extremely gratifying as illustrated by the present case.
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