Diagnosis: The history and photos
are most suggestive of macrocystic lymphatic
malformation or commonly called "cystic hygroma".
However, MRI is needed to confirm the diagnosis and
also the assess the extent of the lesion. There may
be minimal skin discoloration with these lesions
(should not be confused with venous malformations).
Also, some of these lesions are combined lesions,
These lesions are benign conditions;
however, there may be sudden bleeding into
the lesion, which may cause significant enlargement
of the lesion rapidly. This typically happens in
the lesions located in the orbit, behind the
eyeball. In those cases, with sudden enlargement
of the lesion, it is relatively common to see
permanent optic nerve damage. In the case
presented here, sudden airway compromise is a
possibility if there is intralesional bleeding.
This is also true for sclerotherapy,
since sclerotherapy generally causes some
enlargement of the lesion, particularly if
absolute alcohol is used.
MRI is the gold standard modality in vascular
anomalies, particularly for lymphatic malformations.
MRI is much more reliable in diagnosis and more
importantly to show the extend of the lesion. It is
particularly important in this patient, since there
is possible airway involvement. If sclerotherapy is
performed without assessing the malformation in
detail, it may cause significant swelling in the
area and may cause obstruction of the airway
partially or completely. Therefore, almost every
lymphatic malformation patient, particularly if the
lesion is located in the head and neck, needs MRI.
Treatment: To my knowledge,
currently OK432 is not available. One other issue
with OK-432 is that it is difficult to find a
physician (or hospital) who would be interested in
using this agent since it is not FDA approved. This
means that most hospitals do not allow their
physicians use this agent considering
the fact that there is too much risk. There are
other sclerosant agents available, with similar
success rates. One of these agents is Doxycycline.
This agent is quite safe and long term possible
complications are well established. For
the patient demonstrated here,
alternative to sclerotherapy would a surgical
excision depending on the extend of the malformation
in the neck.