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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3825_Библиотеки_им_академика_М_И_Перельмана
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230
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Fig. 15.14 Same patient
15.13: by limb
of Fig.
lifting, blood ows out
shrinking the foot and
demonstrating the
extension of the venous
dysplastic mass
R. Mattassi and V. Pozzoli
Fig. 15.15 Swelling of
the lateral site of foot and
ankle in a patient with
AVM.Details of the same
patient of Fig.15.9
15 Vascular Malformations andEdema
Fig. 15.16 Swelling of
the limb in lymphatic
truncular dysplasia
231
15.6 Clinical Picture ofEdema inCVM
In venous malformations, swelling may have different aspects. The classical edema
that involves foot, ankle, and sometimes also the calf is often visible. However,
because of great variability inlocation of dysplastic tissue, sometimes swelling may
be seen mainly in a part of the foot rather than in the whole distal extremity
(Fig.15.19).
Another cause of swollen appearance of a limb in CVM is a “false edema,” not
due to uid stasis, but because of other causes:
– Subcutaneous fat overgrowth in the site of the malformation. This condition
increases volume of the involved area but is not due to edema. New data demon-
strate that it may be related to gene mutations, like PIK3Ca [16, 17] (Fig.15.20).
– Intramuscular CVM with enlargement of the involved muscle. The most com-
mon cause is venous malformation; lymphatic and also AVM are possible.
Intramuscular bro-adipose vascular anomaly, called FAVA, a rare entity due to
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Fig. 15.17 Patient with a
combined truncular lymphatic
(absence of the deep
lymphatics) and venous
extratruncular malformations.
Supercial nevus, dilated
abnormal veins, and limb
overgrowth. This case can be
dened as “KlippelTrenaunay syndrome” (KTS).
Diagnosis of KTS is often not
correct, as the syndrome
requires a combination of
malformations (venous and
lymphatic) and not AVM.The
term “Klippel-TrenaunayWeber” is also incorrect
because it is a mixture
between KTS (without AVM)
and Parkes Weber syndrome
(with AVM). The incorrect
terminology is often the cause
of confusion
R. Mattassi and V. Pozzoli
intramuscular inltration of brofatty tissue, together with venous anomalies
and muscle contraction may also create limb enlargement and simulate true
edema [18] (Fig.15.21).
15.7 Diagnosis
Clinical examination is the rst step. Location of edema and extension on the foot
and on the calf should be noticed. Effect of compression of the swollen part is helpful to distinguish true edema (compressible subcutaneous uid) (Fig.15.22), from
limb enlargement due to subcutaneous dilated vessels (Fig.15.23) (compressible
but slowly lling again- venous; pulsating and quickly lling again- arteriovenous)
or dysplastic masses. Lymphatic extratruncular malformations are normally not
compressible (Fig. 15.11). Venous extratruncular malformations may be easily
compressible and ll out slowly. Abnormal located supercial veins, cutaneous
nevus, limb enlargement, and limb length discrepancy are all signs of vascular malformations. Abnormal supercial masses, pulsating or not, are also signs that should
orient on a vascular defect.
15 Vascular Malformations andEdema
Fig. 15.18 Young girl
with extensive
extratruncular lymphatic
malformation. Visible
masses are rm and not
compressible. This patient
was treated by surgical
removal, step by step, of
the malformation with
signicant improvement
233
Fig. 15.19 Swelling of
the medial part of the
dorsum of the foot and of
the rst toe, due to a
venous malformation. In
this case, swollen aspect
was increased by fat
overgrowth (see text)
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Fig. 15.20 Increase of
volume of the calf due to
small venous
malformations surrounded
by extensive fat
overgrowth
R. Mattassi and V. Pozzoli
The second step of diagnosis is color Doppler examination. This exam is as much
effective as the examiner has experience in CVM and knows what can be found. A
standard color Doppler investigation is currently an incomplete test. Deep and
supercial veins, their caliber and ow, as well as arterial ow should be studied.
Aplasia or hypoplasia should be investigated (Fig.15.24). Existence of a marginal
vein is a mandatory step in a case with a clinical picture that suggests a venous
malformation. Search of intramuscular vascular masses is also necessary. The quantity of non-vascular tissue (relation tissue/vessels) is useful for the correct treatment: in case of a high non-vascular tissue component of a dysplastic mass,
sclerotherapy is ineffective (Fig.15.25). High ow areas (AVM) should be recognized as well as no ow vascular tissue (lymphatic).
The next step is to choose an imaging investigation and that should be guided by
the result of the Duplex scan test. In case of low ow (venous) or no ow (lymphatic) data, MR is the best test (Fig.15.26 a, b). Contrast media is not necessary in
venous and lymphatic defects as a high-quality exam will demonstrate all necessary
15 Vascular Malformations andEdema
Fig. 15.21 Inltrating
venous malformation of
the calf with muscle
retraction and equinism, in
a case of FAVA.Pain by
walking and edema of the
ankle and foot
235
Fig. 15.22 Effect of digit
pressure on true edema
236
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Fig. 15.23 Swelling of
the foot due to inltration
of diffuse venous
malformations. These
masses are compressible
and ll out again after
release, slowly. In case of
AVM, lling is quick and
often pulsation is
perceptible
R. Mattassi and V. Pozzoli
Fig. 15.24 Aplasia of left supercial femoral vein, demonstrated by echo Doppler
data about the location and extension of the malformation [19]. In case of high ow
signal (AVM), MR with contrast media or also Angio-MR is the best test. Angio-CT
with 3D images can also be useful (Figs.15.27 and 15.28).
Angiography is no more a rst option diagnostic test, as MR and CT offers excellent results. It should be reserved only to AVM in an intention to treat procedure,
after diagnostic process has been completed [20]. There is no indication for angiography in venous and lymphatic malformations [2].
15 Vascular Malformations andEdema
Fig. 15.25 Echo Doppler
of a venous malformation
(bounded area) with
prevalent non-vascular
component. Vessels are
indicated by arrows; the
other area is composed by
compact tissue. That type
of defect, if treated by
sclerosis, will not
disappear
237
a
Fig. 15.26 (a) MR without contrast of a venous malformation of the calf. Frontal projection. The
malformation is well demonstrated. Contrast media is not necessary for venous or lymphatic malformations. (b) MR without contrast of the same case in a transverse projection. The extension into
the deep tissues is demonstrated. At least two projections are necessary to have a complete image
of location, extension, and inltration of tissues of the malformation
b
In case of whole swollen limb or in case of diffuse extended venous malformation, a lymphoscintigraphy should be performed. However, rather than a standard
outow test, a morphologic study of the main, supercial, and deep lymphatic
trunks should be done. This test will demonstrate anomalies of the main lymphatic
trunks or in lymph nodes [21] (Fig.15.29 a, b).
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Fig. 15.27 Angio-CT
with 3D elaboration of
images in a case of AVM
of the planta pedis.
Anatomy of vessels and
site of “nidus” of AVM are
demonstrated. This report
should be compared with
echo Doppler in order to
have complete data about
site and hemodynamic of
the malformation
Fig. 15.28 Angio-CT
with 3D elaboration of
images in case of an
intramuscular limited AVM
of the thigh. Some feeders
of the AVM mass are
visible, as well as a large
outow vessel
R. Mattassi and V. Pozzoli
15 Vascular Malformations andEdema
239
Fig. 15.29 (a)
Lymphoscintigraphy for
selective study of the deep
lymphatic system by
injection of the tracer on
the planta pedis. Complete
absence of the deep
lymphatic trunk on the left
lower limb; (b) Selective
lymphoscintigraphy of the
supercial lymphatic
system by injection of the
tracer in the interdigital
spaces on the foot. On the
left limb, some ow
through abnormal and
dilated lymphatics, which
do not proceed beyond the
knee. The right limb shows
a single supercial outow
vessel. Popliteal lymph
nodes are not visible. This
patient has edema on the
left limb but a clinically
normal right one. The
absence of edema in limbs
with dysplastic lymphatics
in CVM is not uncommon
a
b
15.8 Treatment
Vascular malformations are considered a difcult issue for treatment. However,
often the difculty is due to an incomplete or absent diagnostic procedure because
of lack of knowledge and experience in the management of CVM.If the vascular
defect has been correctly recognized, the choice of the best treatment will be much
more easier [22]. Detailed discussion of the single technique is beyond the scope of
this chapter; an overview of it will be given here.
Elastic compression can be the rst choice in slight forms without symptoms.
However, as the disease has the tendency to progress, regular controls should be
performed [23].
Venous hypoplasia with supercial dilated veins can be treated successfully by
surgery, as deep veins are able to dilate after removal of the supercial veins. That
happens because supercial veins act as a functional bypass: if removed, blood ow
is forced again to ow through deep veins which dilate spontaneously. This
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