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CHAPTER
6
Defective development of the
peripheral lymphatic system:
Lymphatic malformations
B.B. Lee
a
, M. Amore
b,c
a
Vascular surgery, George Washington University Hospital, Washington,
DC, United States
b
Phlebology and Lymphology Unit, Cardiovascular Surgery Division,
Central Military Hospital, Buenos Aires, Argentina
c
III Chair of Anatomy, Buenos Aires University, Buenos Aires, Argentina
General aspects
Like other perfusion systems, a defective development can occur
in the lymphatic system during the complicated lymphangiogenic
development and termed lymphatic malformation (LM). LM is
therefore one of the various forms of congenital vascular malforma-
tions (CVMs) affecting only the lymphatic conduction system as the
outcome of defective development during various stages of lym-
phangiogenesis [1–4].
LM lesion can present as a solitary independent lesion or coexist
with other CVMs often as a hemolymphatic malformation (HLM)
[5–8] (Fig. 1). In other words, LM exists with other kinds of CVM
as part of a complex birth defect affecting the entire blood circula-
tory system and lymphatics including arteries, veins, capillaries,
and the lymphatic system. This combined form of CVM is often
one of the vascular malformation components of “syndromic” enti-
ties (e.g., Klippel-Trenaunay syndrome). For example, LM coexists
with venous malformation (VM) [9–12] and capillary malformation
109Lymphatic Structure and Function in Health and Disease
https://doi.org/10.1016/B978-0-12-815645-2.00006-X
Copyright © 2020 Elsevier Inc. All rights reserved.
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(CM) [13,14] as the vascular malformation component of Klippel-
Trenaunay Syndrome (KTS) [15–18]. When they are further com-
bined with arteriovenous malformation (AVM) [19–22], it is defined
as Parkes Weber syndrome (PWS) [23,24]. Hence, LM develops as a
sole CVM lesion or one of multiple CVM lesions coexisting with
other forms of CVM lesions at the same time, for example, VM,
CM, and/or AVM.
Embryological aspects
During the embryogenesis, the blood and lymphatic systems in
particular encounter extremely strenuous conditions to serve as the
logistics to provide necessary support to the rapidly growingembryo:
from single fertilized egg to billions of cells to form various tissues
and organs. Hence, the embryogenesis of this circulation system goes
through an intricate sequential process involving evolution, involu-
tion, generation, and degeneration to fulfill such critical role to meet
its mandate properly [25–28]. As such, the complicated process of
embryological development of the lymphatic system gets involved
in anomalous involution to form an abnormal network as a conse-
quence of the persistence of the primitive structure. Therefore, it is
quite conceivable the embryonic lymphatic vessels/system would
encounter such a high risk of defective development in view of exten-
sive evolutional process till it reaches complete maturation, together
with blood circulation system, before birth [29–32].
(B)
(C)
(A)
FIG. 1 Hemolymphatic malformation (KTS: Klippel-Trenaunay syndrome). Photo-
graph showing (A) lymphatic malformation (LM), lymphocutaneous fistula,
(B) capillary malformation (CM), and (C) venous malformation (VM).
110 6. Defective development of the peripheral lymphatic system
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A defective development of the lymphatic system, therefore, can
occur throughout any stage of lymphangiogenesis to result in vari-
ous conditions of malformed lymphatic system [33–36] For example,
developing lymphatic sacs, two jugular and two iliac, can fail to
make normal maturation in early eighth week of the gestation to
have defective development to remain a sequestration of primitive
lymphatic tissue as isolated clusters of amorphous lymphatic tissues
(e.g., lymphangioma) [37,38].
When these primitive lymphatic tissues fail to make normal
development, they are sequestered as an isolated amorphous cluster
of lymphatic tissue in various regions with no direct involvement to
the lymphatic transport systems; they continue to dilate to possess a
unique multicystic morphology in various extents: macrocystic and
microcystic lesion, termed as “cystic/cavernous lymphangioma”
[39–42]. This lesion affecting the head and neck has been named
“cystic hygroma,” with notorious recurrence by its nature/evolu-
tion potential to grow when stimulated by surgical excision, etc.
Such malformed lymphatic tissue has a unique characteristic of
the mesenchymal cell as a residual embryonic tissue remnant. These
“prematured” lymphatic tissues are classified separately as
“extratruncular” lesions, based on the embryonic stage when such
developmental arrest arise, since its clinical outcome is profoundly
different depending upon when such event occurred [43] ( Fig. 2).
(
A
)
(B)
FIG. 2 Extratrucular lymphatic malformation/lymphangioma. Photograph showing
(A) clinical findings of gluteal lymphangioma, affecting right buttock, and
(B) ultrasonography view of macrocystic-type lesion, right gluteal region.
111Embryological aspects
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LM caused by developmental arrest in the “earlier” stage of lym-
phangiogenesis is referred to as the extratruncular lesion because this
lesion is caused by embryonic tissue remnants from the reticular
stage, which maintains “evolutional potential,” a unique embryonic
characteristic originated from the mesenchymal cells. Therefore,
they steadily grow by various intrinsic and extrinsic factors/stimu-
lations (e.g., menarche, pregnancy, female hormone, trauma, and
surgery). In other words, LMs are further classified into two groups:
“extratruncular” and “truncular” type, and an extratruncular LM
lesion represents the outcome of the defective development follow-
ing the developmental arrest in the “earlier” stage of
“lymphangiogenesis” [44–47], while truncular LM lesion represents
the lesion/defective development during the “later” stage of lym-
phangiogenesis before the completion of fully matured normal lym-
phatic trunk formation. The extratruncular lesion, better known as
“lymphangioma,” continues to grow whenever the condition
should be met due to this unique characteristic of mesenchymal
cells/lymphangioblasts, while “truncular” lesion cannot continue
to grow since it no longer possesses this evolutional potential to
grow (cf. extratruncular lesion) since it is the outcome of develop-
mental arrest in the “later” stage. However, when disruption of
the lymph vessels and node formation processes occurs along the
main lymphatic trunk formation after the ninth week of gestation,
various structural abnormalities would develop in the lymph-
transporting system [48,49]. Hence, truncular LM lesions give a
much more serious impact to overall lymph-transporting function
due to the direct involvement to the lymph vessels and/or
nodes/system as various structural if not functional defects, known
clinically as “primary” lymphedema, since this defective develop-
ment occurs during lymphatic trunk formation [50,51] (Fig. 3).
Clinical aspects
Primary lymphedema represents a clinical manifestation of the
defective lymphatic system, occurring in the “late” stage of lym-
phangiogenesis with direct involvement of lymphatic vessels and
nodes. Various defective developments result in hypoplasia,
112 6. Defective development of the peripheral lymphatic system
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aplasia, numerical hyperplasia, or dilation (lymphangiectasia) of
lymphatic vessel trunk with valvular incompetence [52–55].
Lymph node dysplasia, known as “lymphnododysplasia,” is gen-
erally involved together with lymphangiodysplasia, but selective
lymph node dysplasia alone can cause primary lymphedema
[56,57]. However, not all primary lymphedema accompanies such
anatomical defects to cause lymph stasis, and some have only defec-
tive function with no visible defect. Such conditions that are limited
to a functional defect with very little structural derangement are
caused by some effect that is molecular in origin (e.g., Milroy’s dis-
ease) [58–61], with all primary lymphedemas being genetically
derived. In other words, truncular LMs are not always accompanied
by morphologically detectable defects of the lymphatic system.
“Milroy-Meige syndrome” does not have any apparent structural
defects of the lymphatic system but rather has a functional impair-
ment at the capillary lymphatics/initial lymphatics level. Also,
“lymphedema-distichiasis syndrome” exhibits impairment of the
endoluminal valves, which cause lymphatic reflux.
There are 41 syndromes with peripheral primary lymphedema,
added to 85 syndromes with primary generalized lymphedema:
1. Noonan syndrome
2. Turner syndrome
(
A
)(
B
)(
C
)
FIG. 3 Truncular lymphatic malformation/primary lymphedema affecting right
lower extremity. Photograph showing (A) anterior view, (B) posterior view, and
(C) lymphoscintigraphic findings.
113Clinical aspects
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3. Yellow nail syndrome
4. Nevo syndrome
5. Aplasia cutis + primary lymphedema (Bronspiegel syndrome)
6. Cholestasis + primary lymphedema (Aagenaes syndrome)
7. Progressive encephalopathy with edema, hypsarrhythmia, and
optic atrophy (PHEO) syndrome
8. Cerebral arteriovenous malformation + primary lymphedema
(Avasthey syndrome)
9. Cleft palate + primary lymphedema (Figueroa syndrome)
10. Hypoparathyroidism + primary lymphedema (Dahlberg
syndrome)
11. Distichiasis + primary lymphedema
12. Microcephaly + primary lymphedema
These 12 syndromes are most frequently mentioned among many
others. Such syndromes are often detected among the newborns and
1-month-old pediatric patients, with primary lymphedema com-
bined with various conditions such as uni- or bilateral Wilms’
tumor, unilateral suprarenal cysts, superficial and deep venous mal-
formations, KTS, PWS, neurofibromatosis, and combined angiodys-
plastic syndromes.
Rarely, such a condition becomes more complicated with the
Gorham-Stout syndrome, Haferkamp syndrome, Proteus syn-
drome, lipodysplasias, exudative enteropathies, and chylous reflux
syndromes.
The classification of primary lymphedema into three groups of
congenital,praecox,andtardatypesisbasedontheageatfirst
clinical manifestation, but they all have similar dysplastic and or
functional causes. Instead, primary lymphedema can be graded
based on its expression in grades (0–3). In terms of incidence
and prevalence statistics, degrees 0–1arenotreportedbutare
themostfrequentones[62,63].Hence,manybelievethat
“primary” lymphedema is caused either by intrinsic “defects”
[64–67] or a malfunction of the lymph conducting elements
[68–71] due to a genetically determined abnormality of lymphatic
anatomy or function. Therefore, a malformation may require detec-
tion or definition in molecular or other functional terms since it
may not necessarily provide sufficient structural attributes that
can be imaged [54,55,72,73].
114 6. Defective development of the peripheral lymphatic system
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