Objective: To review the rare clinical condition of venous gangrene of
the extremities, particularly in association with malignant disease;
to develop a treatment scheme for this difficult condition. Data Sourc
es: Surgical articles on phlegmasia cerulea dolens, venous gangrene an
d other forms of noninfectious, nonarterial gangrene of the extremitie
s from 1937 to the present were identified from published indexes and
computer databases; experience with four additional cases treated by t
he authors is incorporated. Study Selection: Studies selected for deta
iled review include those involved directly with venous gangrene and i
ts investigation and treatment, and those relating to investigation of
clinically similar conditions. Data Extraction: Because of the rarity
of venous gangrene, much of the available literature is descriptive,
involves small numbers of cases and describes only limited experience
with investigation and treatment; data extraction was done by a single
reviewer. Data Synthesis: Because of advances in diagnostic technolog
y and thrombosis research, the assessment of suspected venous gangrene
has changed significantly in recent years. Despite these advances, th
e prognosis for patients with this condition continues to be poor. The
possible confounding roles of paradoxical reactions to anticoagulants
and of primary hypercoagulable states are considered. Conclusions: Ob
jective demonstration of extensive thrombosis of the large veins of th
e involved extremity remains central to the diagnosis. The presence an
d stage of any associated malignant lesion must be rapidly determined
to allow planning for further treatment. Because conventional anticoag
ulation frequently fails, it may be necessary to consider less well-es
tablished modes of therapy.