Tuesday, January 20, 2009


28 October 2008

To whom it may concern.

Dear Sir/Madam


NUH Ref Number : XJ3154931

4 months old Indonesian boy with haemangioendothelioma of the liver with

heart failure.

Samuel is a 4 months old Indonesian boy who was diagnosed to have haemangioendothelioma (abnormal blood vessels) in his liver 9 weeks ago and this has caused him to go in to heart failure and needing respiratory support with the ventilator. He is improving with 3 embolisation (non-surgical medical method to block) of the abnormal blood vessels but he will likely to require another 4 weeks of ventilation support.

Samuel is currently a 4 months old baby boy from Indonesia. He presented with progressive heart failure and a rapidly enlarging liver in Indonesia. Multiple tests done did not reveal his exact diagnosis and hence there was considerable delay

in Indonesia. He turned blue when he was feeding soon after birth and he had progressive enlargement of the abdomen over the first few months of life. We initially thought that he may have a form of tumour affecting young infants (infantile neuroblastoma 4S type). He was medically evacuated to NUH about 7 weeks ago on 17 August 2008 on ventilator as the medical evacuation team found that he was gasping and n heart failure.

On examination in NUH, he was in heart failure and massive enlargement of the liver to 10cm below the right costal margin (normal is about 3cm), this is almost as large as an adult liver! He had also a strange bruit over his abdomen and a small haemangioma at his back. His heart was markedly enlarged (See figure 1) with gallop rhythm confirming the heart failure.

We finally diagnosed him to have massive hepatic haemangio-endothelioma with intractable heart failure.

Figure 1. Samuel’s Chest X ray at initial presentation which shows a massively enlarged heart due to his heart failure. His liver is also exceedingly large displacing his intestines to the left.

2-D echocardiography (18/8/08 and later 3/9/08) showed no structural abnormality of the heart with good fractional shortening of 35%. This shows that his heart is normal but is unable to cope with the massive load. We started him initially on oral prednisolone to attempt to shrink the haemangioma with little success. We added escalating doses of interferon-alpha

based on the AIEOP experience Med Pediatr Oncol 2002 Feb;38(2):118-9 in view of the lack of response to steroids. The liver size remained about 10cm below the costal margn although there was improvement in the heart failure. The surrogate haemangioma at his back grew larger despite the interferon-alpha. In view of the laci of definitive improvement, we decided to attempt localised embolisation. 

Haemangiogram carried out by Prof Lenny Tan, our inventional radiologist showed massive shunting of blood vessels from the celiac and superior mesenteric arteries to the abnormal haemangiothelioma of the liver (Figure 2). We attempted 4 weeks of prednisolone and escalating doses of interferon-alpha therapy which has been previously shown to be successful in some patients. But there was little response. He remained on very high ventilator setting despite therapy.

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