KEY WORDS
Algorithm - management - vascular malformation
INTRODUCTION
Vascular anomalies are congenital lesions of vascular origin, which have been both
confusing and challenging to manage. Most of the confusions pertaining to them have
largely been put to rest with the advent of biological classification.[1] The classification based on the correlation of physical features, natural history
and histology categorises vascular anomalies into vascular tumours featuring hyperplasia
and vascular malformation which results from localised maldevelopment of vascular
morphogenesis. This taxonomy has removed the confusing terminologies, which led to
improper diagnosis and treatment earlier. However, the challenge involved in the treatment
still remains. Although multidisciplinary approach in management is largely established,
we still find patients shuttling between specialities as ‘nomads’. Most of the times,
these patients are managed by reconstructive surgeons who play a central role in their
management, but still there is a lack of a standard and universally acceptable management
protocol for these cases. We have been following an algorithmic approach in the management
of vascular anomalies. In this study, we present our data of vascular anomaly patients
of over a decade.
MATERIALS AND METHODS
This study includes a retrospective review of patients of vascular anomalies treated
at our department from 2002 to 2012. The biological classification of Mulliken and
Glowacki was used to categorise patients.[1] The imaging modalities used were plain radiographs, Doppler study, magnetic resonance
(MR) imaging with MR angiography and contrast computed tomography for the evaluation
of patients. After diagnosis and classification, the further course of management
was on the basis of the protocol [Figure 1a and b].
Figure 1: (a) Algorithm for vascular tumours and haemangiomas. (b) Algorithm for vascular malformation
In cases of haemangiomas, by default, we exercised masterly inaction keeping the patient
under regular observation during the proliferative phase. The indications of intervention
during this phase were rapidly growing hemangiomas which ulcerate or bleed, encroach
upon vital ares like eyes, nose & mouth, compromise oral intake or causing airway
obstruction.
If any of the above-mentioned indications was there at presentation or during any
time in the proliferative phase, pharmacotherapy was prescribed. The most common indications
were rapid growth and ulcerations. From 2002 to 2008, the pharmacological intervention
was either systemic prednisolone or intralesional triamcinolone. Intralesional triamcinolone
(25 mg/mL) was administered for small localised haemangiomas at a dosage of 3–5 mg/kg
every 4–6 weeks. Systemic prednisolone was prescribed for large lesions at a dosage
of 2–3 mg/kg/day till the child was 10–12 months of age. The dosage was tailored as
the child gained weight. The dosage was tapered slowly every 2–4 weeks before discontinuing
prednisolone. In cases where there was rebound growth after discontinuation of corticosteroid,
another course of 4–6 weeks was instituted. Since 2009, oral propranolol was the mainstay
pharmacotherapeutic agent. It was decided to switch over to propranolol as there was
faster response and fewer side effects. It was prescribed at a dose of 2–3 mg/kg/day
in three divided dosages. The dose was gradually tapered every 4 weeks and discontinued
by about 10–12 weeks of age. In case a rebound growth was noticed after stoppage of
propranolol, a 4-week course was repeated. Cases which presented with post-involution
residual lesion were addressed surgically.
The protocol for vascular malformation was separate for low? and high-flow varieties.
Localised venous, lymphatic and lymphatico-venous malformations (LVMs) in areas where
there were no aesthetic and functional consideration were excised. Extensive lesions
where significant post-excision functional and/or aesthetic deficit were foreseen,
excision was not done initially to avoid post-excision functional loss and disfigurement,
for example, in face and hand. Instead sclerotherapy was done with Sodium tetradecyl
sulphate (0.5-2 mL of 1% solution intra-lesionally) or Bleomycin (1 mg/kg body weight,
intralesionally) in venous malformation (VM) and lymphatic malformation (LM), respectively.
The number of sclerotherapy sessions was guided by the size of the lesion and the
response to the treatment. The endpoint of the therapy was either complete resolution
of the lesion or no further reduction in size. In latter cases, the residual malformation
was surgically excised. Capillary malformations (CMs) were categorised into flat and
nodular lesions. In former cases, pulse dye laser therapy was recommended, and in
the latter cases, surgical excision was done in localised and excisable lesions. Camouflage
was advised in the rest. Small high-flow vascular malformations were excised after
encircling the lesion with haemostatic sutures. Larger lesions were also excised where
proximal control was possible with tourniquet. In cases where proximal control was
not possible, like in head and neck malformations with intra-abdominal components,
preliminary embolisation was done followed by excision or debulking 24 h later.
The data of the patients were maintained in terms of diagnosis, age, sex, site, tissue
involved, treatment given and final outcome. The data were compiled and analysed in
Microsoft Excel®.
RESULTS
A total of 784 cases of vascular anomalies were included in the study who presented
to this hospital from 2002 to 2012. Out of these, 196 were vascular tumours and 588
were vascular malformations. In the vascular tumour category, 192 were haemangiomas
and 4 were haemangioendotheliomas. The distribution of the haemangioma patients in
terms of gender and site is shown in [Table 1]. The nature of intervention in relation to haemangioma is shown in [Table 2]. In majority of the patients (110/196), masterly inaction was followed so that the
lesions could involute spontaneously by an age ranging from 5 to 7 years. Thirty-seven
cases were operated for residual lesions after involution, out of which 22 were in
face and neck area, 9 in extremities and 6 in trunk. Eight patients with facial lesions
needed tissue expansion. Rest of the residual lesions were excised and primarily closed.
Forty patients needed pharmacological intervention during proliferative phase. The
indications for pharmacotherapy are shown in [Table 3]. The most common indication being rapidly growing tumour leading to ulceration and
repeated bleeding. Nineteen patients required systemic prednisolone. Intralesional
triamcinolone was given to nine patients. Twelve patients were treated with propranolol
[Figure 2]. Only nine patients required surgery.
Figure 2: Haemangioma of the face, treated by propranolol
Table 1
Distribution of hemangiomas according to site
|
Distribution
|
Hemagioma
|
Hemagioendothelioma
|
|
Congenital
|
Infantile
|
|
5
|
187
|
4
|
|
Location
|
Male
|
Female
|
Total
|
|
Cervicofacial
|
27
|
91
|
118
|
|
Trunk
|
12
|
24
|
36
|
|
Extremities
|
23
|
19
|
42
|
|
62
|
134
|
196
|
Table 2
Interventions in hemangioma patients
|
Interventions
|
Number
|
|
Pharmacotherapy
|
40
|
|
Systemic Prednisolone
|
19
|
|
Intralesional Triamcinalone
|
9
|
|
Propranolol
|
12
|
|
Surgical
|
9
|
|
Intervention for residual lesion
|
37
|
|
None
|
110
|
Table 3
Pharmacotherapy in hemangioma patients
|
Indications for Pharmacotherapy
|
Number
|
|
Bleeding
|
13
|
|
Visual obstruction
|
4
|
|
Nostril obstruction
|
2
|
|
Oral cavity obstruction
|
6
|
|
Bleeding diathesis
|
5
|
|
Rapid growth with Necrosis
|
10
|
|
Total
|
40
|
A total of 588 cases of vascular malformation were included in the study. On the whole,
87.24% (513/588) of cases were low-flow and 12.76% (75/588) were high-flow lesions.
The distribution of types of vascular malformations as per gender and site is shown
in [Tables 4]
[5], respectively. The most common malformations were venous and lympho-venous accounting
for 53.74% followed by LMs (22.1%), arterial/arterio VM (AVM) (12.76%) and the least
common being CMs (11.39%). The overall gender distributions of the lesions were almost
similar in both sexes with a slight male preponderance. Male predominance was evident
in VM/LVM, LM and arterial malformation (AM)/AVM, but females significantly outnumbered
the CM cases. Majority of the CM (47.74%) cases were in the cervico-facial region
followed by 33.83% in the extremities. All the CMs were intradermal in location. LM
and AM/AVM were distributed equally in cervico-facial area and extremities, but in
case of VM/LVM, 56.12% were in extremities and 28.48% were in cervico-facial region.
In all types of malformations, trunk was least affected. Most of the LM cases were
either subcutaneous (46.15%) or both subcutaneous and intramuscular (51.54%) involvement
and only three had pure muscular involvement. Most of the VM/LVMs (76.58%) were in
the subcutaneous plane and 20.25% were in both subcutaneous and muscular planes. However,
significantly ten (3.16%) VM/LVM lesions were intramuscular and all of them were in
extremities. In cases of AM/AVM, 34.66% were in subcutaneous tissue and 25.33% had
involvement of both subcutaneous tissue and muscle. The ablative modalities used for
different types of lesions are shown in [Table 6], and the reconstructive option used for the post-excision defect is shown in [Table 7]. CM cases were either referred to the dermatology department for laser or were excised.
The former option was exercised in 43.28% (29/67) of cases and 34.33% (23/67) of cases
were excised. Excision was done in cases where the lesions were small and localised,
and in some cases, the response of laser was suboptimal. Most of them (17) were primarily
closed after excision, but seven required local flaps, three were closed by pre-expanded
flaps and five defects were skin grafted. Fifteen (22.39%) patients with extensive
CM either had incomplete ablation or could not be treated by any modality. Out of
130 LMs, 60 were excised and 11 were sclerosed by bleomycin and 24 patients underwent
excision after one or multiple sessions of sclerotherapy. Thirty-five (26.92%) patients
had either partial debulking or no excision owing to the extensive nature of the lesion.
Table 4
Gender distribution of vascular malformations
|
Lesions
|
Male
|
Female
|
Total
|
|
CM
|
22
|
45
|
67
|
|
LM
|
72
|
58
|
130
|
|
VM and LVM
|
170
|
146
|
316
|
|
AM, AVM, AVF
|
42
|
33
|
75
|
|
306
|
282
|
588
|
Table 5
Distribution vascular malformations according to site
|
Lesion
|
Location
|
Total
|
|
Cervicofacial
|
Total
|
Trunk
|
Total
|
Extremities
|
Total
|
Multiple sites
|
|
ID
|
SC
|
IM
|
SC + IM
|
ID
|
SC
|
IM
|
SC + IM
|
ID
|
SC
|
IM
|
SC + IM
|
ID
|
SC
|
IM
|
SC + IM
|
|
CM
|
32
|
0
|
0
|
0
|
32
|
8
|
0
|
0
|
0
|
8
|
22
|
0
|
0
|
0
|
22
|
5
|
0
|
0
|
0
|
67
|
|
LM
|
0
|
31
|
0
|
22
|
53
|
0
|
12
|
0
|
6
|
18
|
0
|
13
|
3
|
38
|
54
|
0
|
4
|
0
|
1
|
130
|
|
VM and LVM
|
0
|
69
|
|
21
|
90
|
0
|
35
|
0
|
8
|
43
|
0
|
135
|
10
|
32
|
177
|
|
3
|
|
3
|
316
|
|
AM, AVM, AVF
|
0
|
27
|
0
|
5
|
32
|
0
|
3
|
0
|
1
|
4
|
0
|
26
|
0
|
13
|
39
|
0
|
0
|
0
|
0
|
75
|
|
Total
|
32
|
127
|
0
|
48
|
207
|
8
|
50
|
0
|
15
|
73
|
22
|
174
|
13
|
83
|
292
|
5
|
7
|
0
|
4
|
588
|
Table 6
Interventions in vascular malformations
|
Intervention
|
|
Lesion
|
Laser
|
Excision
|
Sclerotherapy
|
Sclerotherapy +Excision
|
Pre-op embolisation +Excision
|
No or incomplete treatment
|
Total
|
|
CM
|
29
|
23
|
0
|
0
|
0
|
15
|
67
|
|
LM
|
0
|
60
|
11
|
24
|
0
|
35
|
130
|
|
VM and LVM
|
0
|
109
|
78
|
108
|
0
|
21
|
316
|
|
AM, AVM, AVF
|
0
|
37
|
1
|
0
|
29
|
8
|
75
|
|
Total
|
29
|
229
|
90
|
132
|
29
|
79
|
588
|
Table 7
Surgical intervention in vascular malformations
|
Surgery
|
|
Lesion
|
Pry Closure
|
Local Flap
|
Regional Flap
|
Free Flap
|
Tissue Expander
|
Skin grafting
|
Total
|
|
CM
|
17
|
7
|
0
|
0
|
3
|
5
|
32
|
|
LM
|
83
|
9
|
4
|
0
|
0
|
15
|
111
|
|
VM and LVM
|
133
|
33
|
5
|
4
|
0
|
42
|
217
|
|
AM, AVM, AVF
|
61
|
3
|
3
|
3
|
0
|
2
|
72
|
|
Total
|
294
|
52
|
12
|
7
|
3
|
64
|
434
|
In 83 patients, the post-excision defect could be closed primarily whereas nine and
four cases required local and regional flaps, respectively, and 15 defects were skin
grafted. Venous and lympho-VMs were managed by only excision, only sclerotherapy sclerotherapy
followed by excision. Seventy-eight patients were treated by sclerosant injection
only [Figure 3]. Two hundred and seventeen cases were operated upon, and out of that, 109 cases
were excised [Figures 4]
[5] and 108 were excised after sclerotherapy. In 21 patients with extensive lesion,
partial excision or no ablation was done [Figure 6]. After excision, primary closure was possible in 133 cases, 33 needed local flaps,
5 needed regional flaps and 4 defects were reconstructed by free tissue transfer and
42 defects were skin grafted. Arterial and AVMs were either excised after securing
control of the feeding artery or excised following embolisation [Figures 7]
[8]. The option of securing proximal control during operation was tourniquet, clamping
or ligation of feeding vessel, encircling haemostatic sutures and occlusion with intestinal
non-crushing clamps. Thirty-seven cases could be excised with pre-operative proximal
control and 29 were removed after pre-operative embolisation. One patient of multiple
arteriovenous fistulae of foot was managed with sclerotherapy under tourniquet. The
patient had only temporary relief. In eight patients, ablation was not attempted owing
to the extensive nature of the lesion or due to involvement of vital structures. Out
of 66 cases of high-flow lesions which were operated upon, the post-excision defect
could be primarily closed in 61, local, regional and free flaps were used in three
cases each and 2 were skin grafted. In vascular malformations managed by sclerotherapy,
the most common complication was overlying skin necrosis. The most common complication
of excision in the cases of vascular malformation was haematoma.
Figure 3: Venous malformation of the lower lip managed by sclerotherapy only
Figure 4: Localised lymphatic malformation of the neck, excised
Figure 5: Excision of intramuscular venous malformation
Figure 6: Extensive lymphovenous malformation of the face. Multiple sclerotherapy followed
by partial debulking
Figure 7: Intramuscular arterial malformation with feeding vessel from internal iliac artery.
Preoperative embolisation and per-abdominal ligation of internal iliac artery followed
by complete excision
Figure 8: Arterial malformation temporal region with intractable bleeding. Ligation of external
carotid artery followed by excision and skin grafting
DISCUSSION
The vascular anomalies were first classified by Virchow in 1876.[2] Although the classification stood the test of time for nearly a century, it did
not allow standardisation of treatment of vascular anomalies mainly because of lack
of understanding of the natural behaviour of the lesions and erroneous terminologies.
It was in 1982 Mulliken and Glowacki proposed a biological classification based on
endothelial characteristics integrating physical findings, natural history and histology.[1] This system divided vascular anomalies into vascular tumours and vascular malformations.[1]
[3] The fundamental difference between the two is presence of endothelial proliferation
in the former and vascular architectural aberration in the latter. This classification
is now accepted worldwide, has brought a lot of clarity in the understanding of these
lesions and has paved the way for standardised treatment protocol. The treatment protocol
used in the present study is based on this classification.
In this study, haemangiomas account for 25% and vascular malformations account for
75% of all vascular anomalies. Haemangioma is the most common infantile tumour in
Western population where it occurs in 4%–10% of the population and 3–5 times more
common in females. Haemangiomas are not as common in our country as the population
is racially different. Sachin et al. reported that haemangiomas were only 15.56% of the vascular anomalies in their study[4] and 31.59% in a study by Ye et al.[5] The male-to-female ratio among haemangiomas in our study was 1:2.16 which is less
than what is seen in European population.[6] Lesser female predominance of 1:1.49 was also reported by Ye et al.[5] Regarding regional predilection, majority (60.2%) of the lesions in the present
study were cervico-facial followed by extremities and trunk which is reflected in
other studies[4]
[5] as well.
In the present study, 43.88% (86/196) of patients of haemangiomas required some form
of interventions, which is in consonance with studies from rest of the world.[7]
[8]
[9]
[10]
[11] Following the advent of use of propranolol, a pharmacotherapeutic agent for haemangioma,
propranolol replaced prednisolone in our protocol from 2009 onwards, as it had a faster
response and fewer side effects. Although there is a controversy regarding admission
for propranolol administration,[12] all patients in our study were admitted for propranolol administration. The mechanism
of action of propranolol remains vague, but it is believed to be due to regulation
of vascular growth factors and haemodynamic cytokines.[13] In most studies, the majority of the cases were low flow but the proportion of high
flow varied. In the study by Ye et al., high flow accounted for 23.3%.[5] Although CMs are the most common malformations reported in literature, in this study,
the venous and lympho VMs accounted for majority of cases (53.74%). In the literature,
the reported prevalence of CM was 0.3% in children,[13] LM was 1: 2000–4000 live births[14] and VM was 1: 10000.[15] The treatment protocol for vascular malformation was fundamentally governed by three
aspects, namely, its type, site and tissues involved. CMs are ideal for laser ablation.
The Pulse dye laser is reported to be most efficacious in these although argon, potassium
titanyl phosphate and 755 nm laser have also been used in advanced cases.[16]
[17] The laser treatment was done by dermatology department, and only cases with long-standing
CM with tissue hypertrophy were managed by us. Excision was the preferred modality
in these cases. No treatment could be offered to six cases of extensive CMs who had
presented in adult age and in whom lesions were thick precluding laser therapy, and
in nine cases, incomplete removal was only possible. Such situations have been reported
in literature as well.[18] Lymphatic malformations were mostly excised after sclerotherapy. Since many of the
lesions had concomitant skin lesions, most of the extirpation left significant skin
defects warranting either skin grafting or flap cover. This aspect of LM warranting
complex reconstruction is reported in literature.[19] Several sclerotherapy options are available for sclerotherapy of LMs, namely, ethanol,
bleomycin, OK-432 and doxycycline.[20]
[21]
[22] In our series, we have used bleomycin only. There was no sclerosant-related complications
in LMs. Carbon dioxide laser has also been reported to be used in intra-oral LMs.[23] The management of VMs is also a multimodality approach which includes surgery, neodymium-doped
yttrium aluminium garnet (Nd:Yag) laser and sclerotherapy.[24] Nd:Yag laser is reported to be very effective as it causes shrinkage and thrombosis
of the aberrant venous channels.[24] Sclerotherapy is also an effective modality for VMs, and the sclerosants commonly
used are sodium tetradecyl sulphate, ethanol, bleomycin and OK-432.[25]
[26]
[27] In our series, complete ablation of most of the VMs was possible with surgical excision
either primarily or after sclerotherapy. However, for VMs in superficial plane, especially
in face and hand, only sclerotherapy was tried at first to avoid post-surgical scarring
and deformity. The gold standard of management of high-flow or AVM is excision with
or without pre-operative embolisation of feeding vessel.[28] Most of the high-flow malformations in our series were also excised.
CONCLUSION
The protocol has brought an element of much-needed objectivity in the management of
vascular anomalies. This has helped us to define the management of particular lesion
considering its pathology, extent and aesthetic and functional consequences of ablation
to a certain extent. The basic premise of our management protocol is preservation
of form and function vis-à-vis complete ablation. It is always wise to lean towards
sclerotherapy in cases of LM and VM of face and hand. Sometimes, it is prudent to
settle for a suboptimal excision to preserve aesthetic and function.
The algorithmic protocol also helps in counselling of patients regarding the course
of management and possible outcome. The study reiterates the importance of seamless
coordination of surgeon, reconstructive surgeon, intervention radiologist, cardiologist
and laser therapist in managing these complex lesions. In a significant number of
patients where no treatment could be offered underlines the fact that the final frontier
in the management of vascular anomalies is yet to be reached.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patient(s) has/have given his/her/their consent for his/her/their
images and other clinical information to be reported in the journal. The patients
understand that their names and initials will not be published and due efforts will
be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
