While certain small vascular tumours and malformations can be well-managed in the primary care setting, more complex and severe cases would greatly benefit from a multidisciplinary specialty care approach. Read more about how the vascular anomalies teams at the SingHealth Duke-NUS Vascular Centre can help to manage these patients, utilising multimodal treatment for optimal outcomes.
Vascular anomalies are a heterogeneous group of conditions that involve abnormal growth or arrangement of various forms of blood or lymphatic vessels in the body. They can range from mild to severe conditions that can lead to significant physical, psychological and emotional impairment.
Small infantile haemangiomas or capillary malformations, especially those not on critical sites (e.g., arms, legs and trunk) can be managed in the primary care setting.
However, more complex and severe vascular anomalies should ideally be managed under the care of a multidisciplinary team in centres specialising in their management. A combination of various treatments involving input from various sub-specialties may be required for optimal results.
Complex and complicated vascular anomalies require
multimodal and multidisciplinary management to
Typical treatment modalities include:
The multidisciplinary vascular anomalies teams
at KK Women’s and Children’s Hospital (KKH) and
Singapore General Hospital (SGH) are part of the
SingHealth Duke-NUS Vascular Centre (SDVC), and
provide a one-stop centre for the diagnosis and
management of a wide range of vascular anomalies
in adults (seen at SGH) and children and adolescents (seen at KKH).
The teams comprise experts in
the fields of:
Initial presentation and history
Patient M presented to the Vascular Anomalies Clinic at KKH
with extensive venous malformations of her lip, tongue and
buccal mucosa (Figure 1A). These were present at birth and had gradually increased in size over the years. Imaging revealed
deeper involvement including the pharynx and larynx, with
narrowing of her upper airways.
While the patient had previously sought advice from other
healthcare providers, no single treatment had been deemed
effective and safe due to the extent of airway involvement.
A multidisciplinary management plan
With discussion amongst the members of the multidisciplinary
vascular anomalies team at KKH, a targeted and holistic management plan was put in place for the patient.
This involved multimodal treatment with oral sirolimus, multiple treatment sessions for sclerotherapy, and pulsed dye laser (PDL) and neodymium-doped yttrium aluminium garnet (Nd:YAG) laser treatment over two to three years. She was under continued review by the multidisciplinary teams at both KKH and SGH.
Post-treatment, there was marked improvement in the size of the
venous malformations, both externally and internally (Figure 1B). The patient and her family were happy with the results, and she is
now a more confident young woman. M continues to be reviewed
by her multidisciplinary care team.
According to the International Society for the Study
of Vascular Anomalies (ISSVA), vascular anomalies can be classified into two broad categories – vascular
tumours and vascular malformations (Figure 2). This classification has led to standardisation of
the nomenclature. This is important as there now
exists a variety of treatment options targeted at the various vascular tumours and malformations. Accurate
diagnosis is key for appropriate treatments and
Figure 2 ISSVA Classification of Vascular Anomalies 2018 (abridged)CM: capillary malformation, VM: venous malformation, LM: lymphatic malformation, AVM: arteriovenous malformation
1. INFANTILE HAEMANGIOMAS
Infantile haemangiomas (IH) are a type of vascular
tumour that presents in the first three to four weeks of life as enlarging red or bluish papules
or nodules (Figure 3). They grow in breadth and depth over four to five months and thereafter, they
slow in growth until the child is about a year old
before beginning to involute.
IHs are the most common vascular tumours in
children, affecting 2-4% of infants. They are more
common in females, premature infants and twins.
Most IHs are uncomplicated with minimal cosmetic
concern and can be observed for spontaneous
resolution. Small IHs can involute to nearnormal
skin. Large IHs, however, if left untreated,
may involute with residual fibrofatty change, telangiectasia and skin atrophy.
A handful of IHs may lead to life-threatening or
function-threatening complications or have the
potential to cause severe cosmetic disfigurement.
Complicated IHs require prompt referral to a
specialist centre, as early treatment within the
first six months of life can reduce the following
Both systemic (e.g., propranolol) and topical (e.g.,
timolol) beta-blockers have been shown to be
highly effective in the treatment of complicated
IHs if started within the first year of life.
Lasers, such as PDL, can also be used to treat
Occasionally, reconstructive surgery may
be required for large, pedunculated IHs or
for treatment of residual cosmetic concerns.
Radiological interventions such as embolisation
may be required for large visceral IHs that are
associated with high-output cardiac failure.
2. KASABACH-MERRITT SYNDROME,
AND TUFTED ANGIOMA
Kasabach-Merritt syndrome (KMS) is characterised
by thrombocytopenia, coagulopathy and microangiopathic haemolytic anaemia, and can lead to
severe bleeding in a child due to low platelets and
The syndrome is associated with a rapidly
expanding kaposiform haemangioendothelioma
(KHE) (Figure 4) and tufted angioma (TA), which are uncommon vascular tumours presenting in infancy.
The vascular lesion commonly becomes enlarged
and indurated, and infants can present with
bleeding manifestations such as ecchymoses,
epistaxis, haematochezia and haematuria.
Although both KHE and TA can be locally
aggressive, they may also undergo spontaneous
involution. Diagnosis of KHE and TA is made based
on clinical, radiological (ultrasound or magnetic
resonance imaging [MRI]) and histological features.
Treatment of KMS, KHE and TA may require different
regimens and a multidisciplinary approach.
Management options include systemic high-dose
corticosteroids, antifibrinolytics, interferons,
vincristine and antithrombotics.
More recently, the use of mTOR (mammalian target
of rapamycin) inhibitors has proven to be effective
in reducing both KMS as well as the size of the
underlying tumour. Surgery, embolisation and
radiation may also be occasionally required.
1. CAPILLARY MALFORMATIONS /
Capillary malformations or port-wine stains (PWS)
initially appear flat (Figure 5) but may thicken and appear hypertrophic during adulthood. The
commonest form of vascular malformations, they
usually present at birth and grow in proportion to
Certain PWS may result in significant cosmetic
disfigurement. Pyogenic granuloma-like lesions
that lead to recurrent bleeding can also develop.
Large PWS affecting the forehead or eyelids may
be associated with Sturge-Weber syndrome (SWS),
with cranial and ophthalmological associations.
These patients require brain imaging and regular
eye examinations. Seizures and developmental
delays are common neurological manifestations
PWS can be treated with lasers such as PDL or
Nd:YAG laser. Treatments can begin within the first
few months of life and require multiple sessions,
usually one to two months apart.
Hypertrophic PWS may require reconstructive
surgery or interventional ablation.
2. VENOUS AND LYMPHATIC
The next most common forms of vascular malformations,
venous and lymphatic malformations,
appear as soft, bluish-to-purplish swellings (Figure 6).
They usually present within the first few years of
life but can also become prominent during puberty,
or less commonly, in adulthood.
Although mostly asymptomatic, they can present
with pain, bleeding, thrombosis and secondary
infection. Diagnosis is made on clinical, radiological
(e.g., ultrasound and MRI) and occasionally,
Recently, an increasing number of somatic genetic
mutations have been discovered in these vascular
anomalies (e.g., PIK3CA). Genetic testing may be offered in some cases to guide management.
Small, asymptomatic lesions may be left alone, while treatment is recommended for larger lesions,
especially if symptomatic. Various treatment
modalities are often combined for optimal results,
including systemic medications (e.g., mTOR or PIK3CA inhibitors), sclerotherapy, lasers and surgery.
3. VASCULAR MALFORMATION SYNDROMES
Some vascular malformations may be associated
with other abnormalities, such as tissue overgrowth,
bony and visceral involvement, soft tissue tumours
and epidermal nevi. These ‘vascular malformations
syndromes’ are rare but have the potential to lead
to severe physical and psychosocial impairment.
Somatic mosaic mutations in PIK3CA have been found in the PIK3CA-related overgrowth spectrum (PROS). These include Klippel-Trenaunay
syndrome (Figure 7) and congenital lipomatous overgrowth, vascular malformations, epidermal
nevi and scoliosis/skeletal/spinal anomalies
(CLOVES) syndrome. Patients with PROS present
with asymmetric limb overgrowth associated with
various slow-flow vascular malformations.
Multimodal and multidisciplinary treatment is
required for these patients, including sclerotherapy,
embolisation, systemic medications (e.g.,
mTOR and PIK3CA inhibitors), lasers and surgery.
Vascular anomalies comprise vascular tumours and
vascular malformations. They commonly present
in childhood but can also occur in adolescents and
The ISSVA classification has provided a basis for
accurate and timely diagnosis of vascular anomalies.
Accurate diagnosis is of paramount importance in
deciding on the management of these patients.
Complicated vascular anomalies should be diagnosed
and managed by a multidisciplinary vascular
anomalies team, utilising multimodal treatment for
optimal treatment outcomes.
Clin Assoc Prof Mark Koh Jean AanService Chief @ KKH, SingHealth Duke-NUS Vascular Centre;Head & Senior Consultant, Department of Dermatology,KK Women’s and Children’s Hospital
Clinical Associate Professor Koh is a dermatologist who specialises in paediatric
dermatology and dermatopathology. He is also a visiting consultant at Singapore General
Hospital, Sengkang General Hospital and the National Cancer Centre Singapore. He is a
Clinical Associate Professor at Duke-NUS Medical School; Adjunct Associate Professor at
the Lee Kong Chian School of Medicine, Nanyang Technological University; and Senior
Clinical Lecturer at the Yong Loo Lin School of Medicine, National University of Singapore.
Dr Luke Toh Han WeiSenior Consultant, SingHealth Duke-NUS Vascular Centre;Head, Department of Diagnostic and Interventional Imaging;Interventional Services, KK Women’s and Children’s Hospital
Dr Luke Toh received his fellowship training in paediatric interventional radiology, and is a
diagnostic radiologist with special interest in the management of vascular malformations.
He also a visiting consultant at Singapore General Hospital. Dr Toh is a Clinical Assistant
Professor at Duke-NUS Medical School and Adjunct Assistant Professor at the Department
of Anatomy, Yong Loo Lin School of Medicine, National University of Singapore.
Clin Assoc Prof Chan Mei YokeSenior Consultant, SingHealth Duke-NUS Vascular Centre;Haematology/Oncology Service, KK Women’s and Children’s Hospital
Clinical Associate Professor Chan Mei Yoke is a paediatric haematologist/oncologist
and palliative care specialist. She trained at the Vascular Anomalies Center at Boston
Children’s Hospital, USA and contributed to the development of the multidisciplinary Vascular Anomalies Clinics at KK Women's and Children's Hospital and Singapore General
Hospital. She is a Clinical Associate Professor with Duke-NUS Medical School; Yong Loo
Lin School of Medicine, National University of Singapore; and Lee Kong Chian School of Medicine, Nanyang Technological University.
GPs can call the SingHealth Duke-NUS Vascular Centre for appointments at the following hotlines:
Singapore General Hospital: 6326 6060Changi General Hospital: 6788 3003Sengkang General Hospital: 6930 6000KK Women’s and Children’s Hospital: 6294 4050National Heart Centre Singapore: 6704 2222
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