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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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Vascular malformations may be sited in almost any part of the body. About 50%
of cases have CVM on the lower limbs [5]; as this chapter is dedicated to edema, it
will be focused on lower limbs.
R. Mattassi and V. Pozzoli
15.2 Type ofDefects
15.2.1 Venous Defects (VM)
Venous malformations of the lower limbs may be of different types:
– Aplasia or hypoplasia of the main deep veins
– Persistence of marginal vein
– Venous aneurysms
– Dysplastic supercial veins
– Dysplastic venous areas inltrating tissues
15.2.2 Arteriovenous Malformations (AVM)
Two types of anomalies are possible:
(1) Direct communication between two main vessels (truncular forms)
(2) More or less small stulous vessels inltrating tissues
15.2.3 Lymphatic Malformations (LM)
(1) Defects of the main lymphatics (aplasia or hypoplasia) and lymph nodes
(2) Areas of dysplastic small lymphatics inltrating tissues
15.3 Pathology andClinical Picture
Aplasia or hypoplasia of main deep veins is an uncommon anomaly. Main vein
tracts involved in the lower limbs are supercial femoral, popliteal, and common
femoral vein [6]. Iliac veins and even inferior cava vein aplasia are also possible
(Fig. 15.1). As these anomalies appear during intrauterine life, collateral venous
circulation develops. This patient shows supercial dilated veins, more or less
extended, with atypical varicose, located mainly on the lateral part of the limb
(Fig.15.2). In case of iliac aplasia, abdominal subcutaneous collateral circulation
may be visible which bypasses the obstructed venous tract connecting with supercial thoracic veins, draining in the superior cava territory (cava–cava bypass).
Marginal vein, is an abnormal, valveless, more or less dilated vein, located on the
lateral edge of the lower limb. This vein is a remnant of an embryonic vessel that
15 Vascular Malformations andEdema
Fig. 15.1 MR
demonstrating an aplasia
of the right iliac vein
(arrows)
Fig. 15.2 Dysplastic
supercial veins on the
lateral side of the limb
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R. Mattassi and V. Pozzoli
normally disappears before birth. Marginal vein may have different extension, ending through abnormal lateral perforators or through gluteal perforators in the deep
venous system [7] (Fig.15.3).
Marginal vein may be of different caliber: sometimes not larger than a normal
saphena, but in other cases the vessel can be extremely large and connected to the
deep system through huge perforators. Clinically, the abnormal vein may be visible
on the lateral side of the limb. However, sometimes the vein may be not visible
because of smaller caliber and subcutaneous fat. Other dilated veins, extended until
the dorsum of the foot, can be seen (Fig.15.4)
Venous aneurysms may be located in different parts of the body. The most frequent site is in the lower limbs, like popliteal vein, supercial femoral, and common
femoral vein [8]. Clinically, they are rarely visible because of the deep location,
except in the inguinal region.
Dysplastic supercial veins are anomalous subcutaneous veins located often in
atypical sites, like the lateral edge of a limb. They are incontinent and with reux [9]
(Fig.15.5).
Inltrating extratruncular venous malformations of the lower limb are a mass of
dysplastic venous structures which inltrate tissues, mainly muscles. These defects
may manifest clinically by an enlargement of the interested area, like in the thigh or
in the calf (Figs.15.6 and 15.7).
Fig. 15.3 Marginal vein
(arrows). A huge perforator
on the proximal lateral side
of the thigh is the upper
connection with the deep
venous system
15 Vascular Malformations andEdema
Fig. 15.4 Dilated
dysplastic veins on
external ankle and dorsum
of the foot
Fig. 15.5 Supercial
dysplastic veins extended
to the dorsum of the foot.
Right foot is hypoplastic
due to compression of the
hypertonic veins on bones
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Fig. 15.6 Remarkable
swelling of left thigh due
to area of dysplastic veins.
Notice edema of the calf
R. Mattassi and V. Pozzoli
Fig. 15.7 Swelling of the
calf because of dysplastic
veins
15 Vascular Malformations andEdema
225
Direct arteriovenous malformations are direct connections between a main
artery and a vein. This type of anomaly, fortunately uncommon (3% in a series of
261 cases: [10]), may create a high overloading of the circulation and of the heart.
As located on main vessels, they are usually deep sited and not visible; sometimes
they manifest with visible dilated supercial veins.
Inltrating or peripheral AVM: this type of defect may be limited or inltrating
and diffuse; it may be sited more or less supercially or deep inside the tissues. It
may manifest as a mass more or less pulsating, sometimes with visible supercial
veins or as a swelling of the whole limb (Figs.15.8 and 15.9).
Truncular lymphatic defects are mainly an aplasia or hypoplasia of the main
lymphatic draining trunks and/or lymph nodes [11]. Lower limbs are the most common site of that defect. Main lymphatic trunks of this area are the deep and the
supercial system, the deep one following main artery and the supercial one following the great saphenous vein. Absence of a segment or of the whole lymphatic
trunk is the most common defect; aplasia of both systems is rare, but possible.
Lymph nodes may lose their function and become a blockade of lymph outow. The
deep lymphatic trunk is the most common site of a defect; very often the supercial
system is able to compensate even completely so that there may be a clinically
Fig. 15.8 Diffuse
swelling of the right lower
limb in a 6-month-old
child due to extensive
AVM on the thigh. Duplex
scan recognizes a
hypoplasia of the
supercial femoral vein.
Two years later, femoral
vein dilates spontaneously
and stulas disappear
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Fig. 15.9 Diffuse
swelling of the right limb
because of an inltrating
AVM on the ankle and
foot. Medications are due
to ulcers on ngers
R. Mattassi and V. Pozzoli
normal picture and the defect is discovered only after a specic test, like lymphoscintigraphy. Foot or calf and, sometimes the whole limb, may be increased in volume (Fig.15.10).
Extratruncular lymphatic malformations are areas of dysplastic lymphatics inltrating tissues. They are composed by lymphatic cysts of different size: microcystic,
in case of small cysts, or macrocystic, in case of larger size of cysts. They manifest
as swollen, limited area, non-compressible (Fig.15.11).
All these malformations may show a cutaneous nevus, more or less extended
(Fig.15.12).
15.4 Hemodynamics ofVascular Malformations
oftheLower Limbs
There are three mechanisms that cause edema in CVM:
– Venous reux and venostasis in venous malformations
– Venous hypertension in arteriovenous malformations
– Lymphatic stasis in defects of the main draining trunks
15 Vascular Malformations andEdema
Fig. 15.10 Diffuse edema
of the whole right limb due
to a truncular lymphatic
dysplasia. Extratruncular
lymphatic malformations
are also present on the foot
and calf
227
Fig. 15.11 Swelling of
the dorsum of the foot due
to extratruncular lymphatic
malformation. The area is
compact and not
compressible
(1) Venous reux exists in abnormal insufcient veins, like marginal vein, super-
cial dysplastic veins, and deep insufcient main veins. In a study with color
doppler about hemodynamic defects in 52 cases of complex venous dysplasia
of the lower limbs, reux was recorded in all dysplastic supercial veins and
marginal veins; in 22 of 52 cases (42%) a reux in the deep venous system was
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Fig. 15.12 Diffuse
cutaneous nevus in venous
malformation
R. Mattassi and V. Pozzoli
noticed. A venous stasis without reux was recorded in extratruncular inltrating malformations, mainly intramuscular, and also in 8 cases (16%) of abnormal dilatation of calf veins [9].
(2) Venous hypertension in AVM is due to an increased inow of blood in the
venous system. The shunt between arterial and venous system is a low resistance condition in which more blood than normal enters the veins. Outow
veins dilate and pulsating venous ow may be sometimes recognized. Peripheral
veins, located distally to the shunt, have stasis with hypertension [12].
(3) Lymphatic outow defects, due to an obstruction of the main draining lym-
phatic trunks. As explained before, absence or stenosis of a main trunk may
create distal lymph stasis. However, even in the absence of a main lymphatic,
the phenomenon of dermal backow is very rare in CVM [13].
15.5 Symptomatology
Aplasia or hypoplasia of main veins of the lower limb may even cause no symptoms, especially if larger veins are obstructed, like iliac or cava vein, because development of collateral circulation is possible. In more distal obstruction, like
15 Vascular Malformations andEdema
229
supercial or popliteal vein, main disturbances are due to venous stasis, in the normal main veins and in supercial dysplastic veins.
Extranuclear venous malformations my cause heaviness and edema, due to
venous stasis. Pain, due to blood stasis, or even related to a localized thrombosis in
the malformation may be claimed by the patient. If this type is sited on the foot,
inltrating the plantar muscles or extending subcutaneously, the clinical picture will
be of a swollen foot. However, rather than due to edema, the swollen aspect is
caused by the dysplastic vascular mass itself. In diffuse inltration of the foot, this
malformation may substitute main part of the muscles. By elevation of the limb,
typically the outow of the resting blood will be thinning out the foot (Figs.15.13
and 15.14).
Arteriovenous malformations on the limbs may show a hyperemic area with
swelling and abnormal pulsation (Fig.15.15). Pain appears with worsening of the
condition. If ulcers appear they may be complicated by sudden bleeding, which is a
sign of progression of the disease. Heart decompensation is rare and is more common in direct AVM.
Lymphatic defects often manifest with edema by aplasia or malfunction of main
lymphatic trunks. The deep lymphatic system is much more often involved. The
defect exists since birth but, for unknown reasons, edema may appear late (Fig.15.16).
In complex and extended venous malformations, there may coexist lymphatic
dysplasia of the main trunks or even extratruncular lymphatic anomalies: this is the
so-called Klippel-Trenaunay syndrome [14, 15] (Fig.15.17).
Extratruncular lymphatic malformations are less common on the lower limbs.
They manifest as lymphatic masses inltrating muscles. The microcystic form is
much more common in lower limbs. These defects, if truncular lymphatic malformations do not coexist, rarely originate edema (Fig.15.18).
Fig. 15.13 Diffuse
inltrating extratruncular
venous malformations of
the calf and foot. The
malformation also
inltrates extensively
muscles of calf and also
planta pedis. Patient
complains swelling of
dysplastic veins by gravity
in standing position
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