 |
 |
 |
 |
 |
 |
 |
|
|
|||||||
| Discussion |
A 10 day-old-male child presented with scalp abscess following forceps
delivery. The abscess was drained and the child was admitted in the NICU
where he developed septicemia and signs of heart failure. On examination,
he had moderate hepatomegaly. A clinical diagnosis of hepatic abscesses
was made.
RADIOLOGICAL Examination:
On ultrasonography, the liver showed gross hepatomegaly involving both the
lobes with multiple mixed echoic lesions of varying sizes - ranging from
2 to 4 cm.( Fig. 1: Left lobe liver; Figs. 2, 3: Right lobe liver).
 |
 |
 |
|
Fig.
1
|
Fig.
2
|
Fig.
3
|
On Doppler study, the
blood vessels supplying these masses were tortuous and dilated and demonstrated
nonphasic low resistance brisk blood flow(Fig. 4). The celiac axis showed
markedly increased flow velocity (Fig.5).
 |
 |
|
Fig.
4
|
Fig.
5
|
 |
|
|
Fig.
6
|
Plain and contrast enhanced
CT scans of the abdomen showed the liver to be massively enlargemed and
studded with multiple markedly enhancing nodules of varying sizes involving
both the lobes suggestive of hemangioendotheliomas. (Fig.7, Fig.8)
 |
 |
|
Fig.
7
|
Fig.
8
|
Diagnosis:
Infantile Hemangioendothelioma.
The child died after 1 week due to congestive heart failure. Autopsy specimen
showed the liver studded with multiple nodules in both the lobes.(Fig. 9)
 |
|
|
Fig.
9
|
Differential
diagnosis:
Three other liver tumors should be considered as differentials: hepatoblastoma,
mesenchymal hamartoma and metastatic neuroblastoma. Hepatoblastoma is rarely
seen in children less than one year of age. The alpha-fetoprotein level
is usually markedly elevated - whereas in cases of infantile hemangioendothelioma,
it is usually normal or only mild to moderately increased. On CT, hepatoblastomas
are more heterogenous than infantile hemangioendothelioma - especially after
contrast administration. Mesenchymal hamartomas usually occur in the infant
and are not associated with elevated alpha- fetoprotein levels. Mesenchymal
hamartomas typically appear as multilocular cystic masses - rarely as a
solid lesions. When solid, mesenchymal hamartomas are avascular or hypo
vascular masses on angiography, scintigraphy, and Doppler ultrasound. Metastatic
neuroblastoma may be confused with the multicentric form of infantile hemangioendothelioma.
With metastatic neuroblastoma, urinary levels of catecholamines are almost
always elevated while the primary tumor, often an adrenal mass, can be seen.
On contrast enhanced CT scans, marked enhancement is usually noted in cases
of infantile hemangioendothelioma while with metastatic neuroblastoma enhancing
areas present normal residual liver.
DISCUSSION:
Infantile hepatic hemangioendothelioma
(IHHE) is a rare benign vascular tumour of liver, usually presenting in
infancy with cardiac failure.
Benign hepatic tumors account for about one third of all hepatic tumors
in children. The majority of these tumors are of vascular origin, usually
hemangioendotheliomas or cavernous hemangiomas. IHHE is a type of capillary
hemangioma which is seen almost exclusively in children.
IHHE is the most common symptomatic vascular tumor with 85% of cases presenting
in the first six months of life. The tumor may appear as an asymptomatic
abdominal mass or more commonly presents with congestive heart failure,
thrombocytopenia, jaundice or hemorrhage. The heart failure is usually secondary
to arterio-venous shunting within the tumour. Additionally, 50% of patients
have cutaneous haemangiomas.
Microscopically, they are capillarized vascular channels lined by one or
more layer of plump endothelial cells secondary to extensive proliferation
of endothelial cells forming multiple spongy nodules of varying sizes. Proliferation
of the endothelial cells may obliterate and sequestrate the bile ducts within
the tumor. The lesions show arteriovenous malformations, hemorrhage, thrombosis,
fibrosis and calcification and is termed 'giant' if size is more than 7
cm in size. They have a high frequency of malignant degeneration progressing
on to malignant hemangio endothelial sarcomas.
The tumor has a proliferating phase and an involuting phase. In the proliferating
phase, the tumor grows rapidly - reaching peak growth rate by approximately
the fourth month. By about one year, it enters an involuting stage where
it stops growing. Over the next 3-5 years it involutes and disappears spontaneously.
Management:
In an asymptomatic child, no treatment may be necessary; although follow-up
imaging is recommended in order to demonstrate tumor regression. Patients
with congestive heart failure are treated with prednisone, digoxin, and
furosemide. Prednisone appears to accelerate regression of the lesion. If
conservative treatment fails, embolization therapy or surgical resection
may be necessary. Liver transplantation is an option in cases with diffuse
tumor involvement or uncontrollable heart failure.