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Congenital
hemangioma (RICH)
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Congenital hemangioma or Rapidly
Involuting Congenital Hemangioma (RICH) is
a similar benign tumor (birthmark) to
infantile hemangioma, but it differs
from infantile hemangioma in that it has
a fully developed presentation at birth,
with rapid involution and regression.
Overall, congenital hemangioma is a rare
entity.
Photo#1
shows a moderately sized congenital
hemangioma (birthmark) or rapidly involuting
congenital hemangioma in the
thigh. There are central ulcer
like skin changes. Photo#2 shows a
similar congenital hemangioma
lesion in the scalp.
These birthmarks may be difficult to
differentiate from other congenital
lesions, particularly from congenital
fibrosarcoma. Recognizing these
congenital birthmarks and differentiating
them from other congenital lesions or tumors
is very important to reduce unnecessary
invasive diagnostic procedures (surgical
biopsies or major surgical excision
procedures) in these newborns, because
most of these birthmarks involute
rapidly. |
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Diagnostic
Tests? The
appropriate diagnostic test is
ultrasonography with Doppler and/or
magnetic resonance imaging (MRI). The
imaging features of these tumors
(birthmarks) also greatly differ from more
common types
of
infantile hemangiomas, but
may be confused with arteriovenous
malformations (AVM) or other tumors
(fibrosarcoma etc.). Various sized
cystic vascular lesions with
arteriovenous connections similar to
high-flow lesions (AVM) are commonly
seen on ultrasound examinations or MRI
scans. Interpretation of these scans (US
or MRI) by an experienced radiologist or
interventional radiologist is important
for accurate diagnosis. Some tumors may
require tissue sampling (biopsy) for
definite diagnosis.
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Figure#1
(T1 weighted MRI image) shows a
large lesion (rapidly involuting
congenital hemangioma) in the left
upper thigh with multiple dark
vessels due to rapid blood flow,
unusual appearance for common
infantile hemangiomas. Figure#2
is an ultrasound
image of rapidly involuting
congenital hemangioma. There are
multiple high-flow relatively
large vessels in the tumor
tissue. Figure#3 is
an catheter arteriogram of rapidly
involuting congenital hemangioma
(in the head). There are multiple
aneursyms and arteriovenous
connections similar to AVM. |
What
is the treatment ?
These
lesions (birthmarks) usually
rapidly
involute (shrink), usually much faster
than common hemangiomas. However, some
patients may develop significant health
problems
(e.g., cardiac failure, bleeding
similar to kaposiform
hemangioendothelioma) due to
arteriovenous shunting. Therefore,
patients with these rare birthmarks need
to be followed closely and appropriate
therapeutic intervention should be
implemented whenever needed. Embolization
should be used if needed. Medical
intervention (pharmacological) is
similar to that of infantile hemangiomas.
Photo#1
is at 6 days, photo#2 at 12 days,
photo#3 at 45 days, and photo#4
at 8 months. This patient was treated
with systemic steroids for a few
months.
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