Purpose: The purpose of the study is to establish the natural history,
probable nature, and optimal treatment of lesions within the orbit de
scribed previously as lymphangiomas or orbital varices. Methods: The c
linical and radiologic records of 158 patients with these lesions were
reviewed, Of these, 91 had surgery, and histologic specimens from 57
were re-examined. Results: Most patients were infants or children with
a dark blue swelling in the superomedial part of the orbit, orbital h
emorrhage, or proptosis, Computed tomography showed smooth, contoured
lesions denser than brain extending posteriorly. Half enlarged with th
e Valsalva maneuver, 31% contained phleboliths. Surgery was performed
in 91 patients, mainly for cosmesis. Excised tissue included endotheli
um-lined channels containing blood in the orbit and clear fluid in man
y superficial specimens, Conclusions: The behavior of these lesions an
d their prevalence in infancy and childhood favor a hamartoma. The aut
hors observed a seamless range of clinical features that they could no
t subdivide, particularly in relation to any connection with the orbit
al veins. Many bleed and enlarge permanently and need excision, but su
rgery is difficult and management should be as conservative as possibl
e. The origin of these lesions cannot be determined by histopathologic
analysis, although the authors have evidence of venous features in th
e orbit and lymphatic features more superficially. The authors' clinic
al findings support a venous origin, Two-thirds have either a free ven
ous connection or phleboliths. Their distribution corresponds with tha
t of the normal orbital veins, and at surgery they derive from or repl
ace those veins. ''Orbital venous anomaly'' is the most accurate term
for their description.