vascular anomalies of newborn,approach to infantile hemangioma

1. Vascular Anomalies of
newborn,Approach to infantile
hemangioma
Date-26-02-2021
Dr.Santhosh Reddy
Moderator-Dr.Arnab Sengupta

2. • The International Society for the Study of Vascular Anomalies
(ISSVA) has recently (2018) released an expanded
classification.
• Ninety percent of lesions can be diagnosed by history and
physical examination. Vascular anomalies are classified based
on their clinical behavior and cellular characteristics
• There are two broad types of vascular anomalies:
• Tumors
• Malformations.

3. •
•
Vascular Anomalies
Tumours
Infantile
hemangioma
- Congenital
hemangioma
- Pyogenic
granuloma
Kaposiform
hemangioendothe
lioma
Malformatio
ns
Slow flow
Capillary
malformation
- Venous
malformation
- Lymphatic
malformation
Fast flow
Arteriovenous
malformation
- Arteriovenous
fistula
Overgrowth
syndromes
CLOVES
Klippel–Trenaunay
Sturge–Weber
Parkes Weber

4. • Tumors typically arise postnatally and demonstrate
endothelial proliferation.
• Vascular tumors demonstrate cellular hyperplasia There are
four major lesions:
1. Infantile hemangioma (IH),
2. Congenital hemangioma,
3. Kaposiform hemangioendothelioma (KHE),
4. Pyogenic granuloma (PG)

5. • Vascular malformations are errors in vascular development,
are present at birth, and have minimal endothelial turnover. It
can comprise a single malformed and dysplastic vessel or a
combination of such vessels (Enjolras and Mulliken, 1997, and
Finn et al.1983).
There are four major categories:
1. Capillary malformation,
2. Lymphatic malformation,
3. Venous malformation, and
4. Arteriovenous malformation

6. INFANTILE HEMANGIOMA
• MC tumor of infancy, affecting 4% to 5% of infants.
• Risk factors
 Prematurity,
 Multiple gestations,
 Preeclampsia,
 Placenta previa,
 Invasive antenatal procedures,
 Advanced maternal age,
 Assisted reproductive techniques, and
 Female sex.
• The median age of appearance is 2 weeks.

7. Pathogenesis
• IHs form as a result of dysregulation of both vasculogenesis
and angiogenesis.
• There are many proposed theories as to the trigger that
initiates this dysregulation, without any one unifying cause,
but it is likely that hypoxia plays an important role.
• There is strong evidence in support of IHs being derived from
endothelial stem and progenitor cells (Khan et al., 2008).
• Isolation of CD133+ endothelial progenitor cells from infantile
hemangioma tissue
• Injection of these progenitor cells into mice lead to formation
of tumors with the unique immunohistochemical and growth
characteristics of IHs (Khan et al.2008).

8. • The endothelial cells that were isolated from IHs were also
found to be clonal in nature, suggesting that these tumors
were caused by a somatic mutation in one or more genes
regulating endothelial cell proliferation (Boye et al., 2001).
• The endothelial cells in infantile hemangioma express a
unique phenotype of cell surface markers with positive
staining for glucose transporter 1 (GLUT1).

9. • Histologically, IHs have increased cellularity and form small
sinusoidal vascular channels.
• Further evidence suggestive of this link relates to the
increased risk of development of IHs in low birth weight
infants—particularly those whose mothers had preeclampsia
or placenta previa, both of which are associated with
placental hypoxia.

10. • Hypoxia likely plays a large role in the development of IHs and
is thought to be an important triggering signal for their
development(Drolet and Frieden, 2010; Léauté- Labrèze et al., 2017), with in
utero hypoxia as a risk factor for localized hemangiomas and
regional hypoxia from arterial abnormalities as a risk factor for
segmental hemangiomas.

11. Clinical features
• At birth, IHs are either absent or barely evident.
• Within the first few weeks after birth, a precursor lesion is
present, which can appear as either a pale area of
vasoconstriction or a telangiectatic red macule or a “bruise-
like” area .
• IH is red when it involves the superficial dermis and can
appear bluish if it is located beneath the skin.
• There is a short latency period of 1 to 3 weeks before
initiation of a rapid proliferation phase in most hemangiomas.

12. • Proliferating phase-first 3 months rapid growth occurs. with most of
the growth occurring between 5 and 8 weeks of age continue to
grow more slowly up until 9 to 12 months of age.
• Up to 80% of IHs have completed their growth by 3 months of age.
• Involuting phase-After 12 months, the tumor begins to
regress.After involution, one-half of the children will have a residual
deformity.
• Involuted phase-Regression of a hemangioma occurs slowly over
many years, with approximately 90% regression noted by 4 years of
age (Bauland et al., 2011).

13. • Clinical signs of regression include dulling of the bright red color to a more
purple color and a central gray white discoloration that spreads
centrifugally. If the white discoloration occurs in infants younger than 3
months, it can sometimes be a marker of impending ulceration.
• Regression does not indicate complete resolution and normalization of
the underlying skin in all cases.
• There can be remaining telangiectasias, distortion of facial anatomy, and
permanent textural changes characterized by residual fibrofatty tissue and
skin laxity because of loss of elastic fibers causing fine wrinkling of tissue.
• Permanent scarring can also result from hemangiomas that were
previously ulcerated.

14. • While proliferation is often a key characteristic of IHs, it
should not be considered an absolute defining feature.
• There is a unique subset of IHs known as abortive
hemangiomas or infantile hemangiomas with minimal or
absent growth in which the proliferation phase is absent (Suh
and Frieden, 2010;).
• These hemangiomas present preferentially on the lower
extremities and sometimes can be confused with capillary
malformations (CMs).
• Although they lack the proliferation phase, they do
spontaneously regress.

15. IH-Superficial haemangioma
• Superficial hemangiomas are located in the upper dermis and present as
elevated bright-red, well-demarcated, papules or plaques, which are
sometimes in lay language referred
to as strawberry hemangiomas.
• In their early proliferation phase,
small bright-red papules can be
seen arising from fine telangiectatic
vessels, which can be a distinguishing
feature between IH and CMs that
can have a similar appearance in early
infancy.

16. IH-Deep haemangioma
• Deep infantile hemangiomas are confined to the deep dermis and
subcutis and present as bluish, dome-shaped tumors or nodules, with ill-
defined borders.
• They can present in isolation or more commonly as a mixed infantile
hemangioma with both superficial and deep components.
• The deep component of IHs often has a distinct growth pattern in which
they tend to appear later, around 2 to 3 months of age, and grow for a
longer period, sometimes
even over years, as compared
with their superficial counterparts
(Brandling-Bennett et al., 2008; Chang et al.,
2008).

17. IH-Mixed
• Mixed infantile hemangioma with both superficial and deep
components.

18. IH-Focal
• Focal hemangiomas seem to grow from a single point with
minimal or absent growth.

19. IH-Segmental
• Segmental hemangiomas are thought to arise from an
embryonic developmental unit or placode and comprise a
pattern on the skin correlating with these developmental
units.

20. • Segmental hemangiomas are at risk of associated anomalies
and syndromes. Large facial or scalp hemangiomas should be
evaluated for the possibility of -
• PHACE (posterior fossa abnormalities, infantile hemangioma,
arterial,cardiac, eye abnormalties)
• LUMBAR (lower body hemangioma, urogenital
abnormalities, ulceration, myelopathy, bony deformities,
anorectal malformations, and renal abnormalities) syndrome.
• Segmental hemangiomas, like deep IHs, can sometimes have a
prolonged growth phase, which in rare cases may last for
years.

21. • Local Complications
• Ulceration. it can be seen in up to 30% of cases. It can cause
significant pain and discomfort and permanent scarring in the area
of ulceration.
• Ulceration has been described to be more likely to occur during two
points during the life cycle of the hemangioma: either just before
the rapid proliferation phase, which can be the presenting sign of
the hemangioma, or at the end of the growth phase (usually around
4 months of age).
• The exact mechanism causing ulceration is unknown but is thought
to be related to tissue hypoxia, with the tumor outgrowing its blood
supply (Chamlin et al., 2007).

22. • Ulceration is more commonly seen in large hemangiomas, those
with segmental distribution, and those with mixed morphology with
both superficial and deep components (Morelli et al., 1994; Mulliken et
al., 1995; Chamlin et al., 2007).
• Areas of friction or those exposed to moisture for a long time, such
as the lower lip, neck, intertriginous areas, and anogenital and
diaper region, are at high risk of ulceration.

23. • Treatment of ulceration include topically administered brimonidine,
0.2% or timolol, 0.5%, topical antibiotics with mupirocin or
metronidazole, pulsed dye laser treatment, and systemic therapy
with propranolol. Topical analgesics can also be useful to minimize
pain and discomfort (Cheng and Friedlender, 2016).
• Disfigurement. In addition to the transient disfigurement,
hemangiomas located on the central face, large hemangiomas, and
those with a significant superficial component can predispose the
affected child to permanent scarring.

24. • IHs involving the nasal tip are at risk of a bulbous nasal tip or a
“Cyrano” deformity . This is caused by splaying of the alar cartilage
during the proliferative growth phase. Early intervention with
initiation of propranolol therapy can preserve the contour of the
nose (Perkins et al., 2014).
• If a persistent deformity develops, surgical debulking may be
needed.
• Similarly, IHs located on the lip are at risk of deformity and
disruption of the natural contours of the lips in addition to being at
high risk of ulceration and permanent scarring (Yanes et al., 2016).

25. • Large hemangiomas of the central chest or hemangiomas involving
the breast tissue in females can also be quite disfiguring, leading to
permanent breast hypoplasia in some cases (Theiler et al., 2016).
• Disfigurement alone, regardless of threat to function, is a
reasonable indication for medical therapy in certain cases and
should be considered.
• Regression of hemangiomas does not always ensure complete
normalization of the underlying skin and therefore can lead to
lifelong psychosocial and emotional sequelae experienced by both
the family and the patient (Zweegers and Van der Vleuten, 2012).

26. • Functional Complications
• Periocular Hemangiomas. Hemangiomas located on the lid or
around the orbit are at risk of causing visual impairment and can
lead to amblyopia in severe cases.
• Amblyopia can be caused by direct pressure on the globe, causing
astigmatism or myopia, or because of the size of the hemangioma
there can be visual axis obstruction or strabismus (Spence-Shishido et al.,
2015).
• Larger and segmental hemangiomas in the periorbital area pose the
greatest risk of ocular complications.

27. • Deep retrobulbar IHs may present with proptosis and can also cause
strabismus and visual acuity changes. Deep and mixed infant
hemangiomas can also cause tear duct obstruction and exposure
keratopathy.
• Aggressive and early initiation of treatment along with evaluation
by a pediatric ophthalmologist can help prevent some of these
complications (Spiteri Cornish and Reddy, 2011; Xue and Hildebrand, 2013).
• Further imaging, such as MRI, may be needed to assess the extent
of the hemangioma or the presence of a deeper component.

28. • Auricular Hemangiomas. With infantile hemangiomas of the ear
there is high risk of physical deformity of the ear, cartilage
destruction, ulceration, potential infection when ulceration is
present, and potential hearing alterations.
• Segmental hemangiomas in this region have a higher rate of
complications, and with them there can also be a risk of
sensorineural and conductive hearing loss.

29. Potential Life-Threatening Complications
• Airway Hemangiomas. Airway hemangiomas can occur with or
without the presence of cutaneous IHs.
• The highest risk occurs with hemangiomas located in a “beard
distribution” specifically involving the left or right preauricular
areas, chin, lower lip, and anterior part of the neck (Orlow et al., 1997).
• Clinically, airway hemangiomas present most commonly between 6
and 12 weeks of age with biphasic stridor or a hoarse, croup-like
cry.
• The subglottis is the most common site of involvement, but the oral
cavity, oropharynx, hypopharynx, larynx, and upper trachea can also
be involved.

30. • Referral to a pediatric otolaryngologist is important for evaluation
of the airway, and systemic treatment should be started
immediately (O et al., 2009).
• Treatment of airway hemangiomas may require a combination of
multiple medical and surgical treatments depending on the extent
of involvement, which may include propranolol, oral and
intralesional corticosteroids, vincristine, interferon alpha, surgical
excision, and laser therapy.

31. • Hepatic Hemangiomas. The presence of an IH in the liver can lead
to potential serious complications such as congestive heart failure
and consumptive hypothyroidism.
• Individuals with five or more cutaneous IHs of any size and in any
location should be screened for the possibility of a hepatic IH (Horii et
al., 2011).
• Individuals with large or segmental IHs do not seem to be at greater
risk of hepatic IHs (Boon et al., 1996a; Horii et al., 2010).

32. • Parotid Hemangiomas. Parotid hemangiomas can be isolated to the
parotid gland or can be present as part of a segmental IH in the
maxillary distribution of facial segment .
• Parotid hemangiomas have a unique pattern of growth as they can
have a longer proliferative growth phase than typical IHs.
• This can lead to longer treatment courses (Brandling-Bennett et al.,
2008).

33. • They have also been associated with functional complications
relating to deformity of adjacent structures such as the ear and lip
and conductive hearing loss caused by narrowing of the external
auditory canal.
• Life-threatening complications can also arise, including an
association with subglottic hemangiomas, and less commonly
congestive heart failure and consumptive hypothyroidism (Greene, 2004;
De Corti et al., 2015).

34. IH Clinical Findings At Risk For
Life-threatening - “Beard-area” IH
≥5 cutaneous IHs
Obstructive airway hemangiomas
Liver hemangiomas,cardiac
failure,hypothyroidism
Functional impairment
Periocular IH (>1 cm)
IH involving lip or oral cavity
Astigmatism, anisometropia, proptosis,
amblyopia
Feeding impairment
Ulceration -Segmental IH: IH of any size
involving any of the following sites:
lips,
columella, superior helix of ear, gluteal
cleft and/or perineum, perianal
skin, and other intertriginous areas (eg,
neck, axillae, inguinal region)
Increased risk of ulceration
Associated structural anomalies
Segmental IH of face or scalp
Segmental IH of lumbosacral and/or
perineal area
PHACE syndrome
LUMBAR syndrome

35. Disfigurement -
Segmental IH,
especially of face
and scalp
Facial IH: nasal tip
or lip or any facial
location ≥2 cm
High risk of scarring and/or permanent disfigurement
Risk of disfigurement via distortion of anatomic landmarks and/or
scarring
and/or permanent skin changes
Scalp IH >2 cm Permanent alopecia (especially if the hemangioma becomes thick or
bulky); profuse bleeding if ulceration develops
Neck, trunk, or
extremity IH >2 cm
superficial IH (eg,
≥2 mm thickness)
Greater risk of leaving permanent scarring and/or permanent skin
changes depending on anatomic location
Breast IH (female
infants)
Permanent changes in breast development (eg, breast asymmetry) or
nipple contour

36. Diagnosis
• In most cases a hemangioma can be diagnosed by its clinical
appearance and characteristic pattern of evolution.
• Doppler ultrasound examination can be easily performed and may
be helpful in distinguishing between an infantile hemangioma and
another low-flow malformation or nonvascular tumor.
• Other imaging modalities—MRI or angiography—may be indicated
for large or obstructive lesions(e.g., ocular, upper airway) to help
define the extent of involvement or associated abnormalities (e.g.,
PHACE or LUMBAR syndrome) (Baker et al., 1993; Esterly, 1995).

37. • Skin biopsy is diagnostic for nonvascular tumors, which can mimic
vascular birthmarks (e.g., pilomatricoma, juvenile xanthogranuloma
[JXG], Langerhans cell histiocytosis, infantile myofibromatosis,
rhabdomyosarcoma).
• IHs can also be differentiated from other vascular tumors by
staining with GLUT1, an immunohistochemical marker that is highly
selective and specific for IHs.

38. Congenital Hemangiomas
• Congenital hemangiomas are an uncommon and distinct type of
vascular proliferation that are fully formed at birth.
• They do not undergo the characteristic proliferative growth pattern
in postnatal life as seen with IHs, but rather their proliferative phase
occurs in utero.
• They are also GLUT1 negative, unlike IHs.
• Congenital hemangiomas can be divided into three major subtypes
1. Rapidly involuting congenital hemangiomas (RICHs),
2. Noninvoluting congenital hemangiomas (NICH), and
3. Partially involuting congenital hemangiomas (PICHs) on the
basis of their clinical progression.
• Congenital hemangiomas are usually solitary in nature and are more
common on the extremities and head and neck (Boull and Maguiness,
2016).

39. Pyogenic Granulomas
• Pyogenic granulomas, also known as lobular capillary
hemangiomas, are benign acquired vascular tumors that are
commonly seen in infants and children (Patrice et al., 1991;).
• They have also been seen to develop within an existing port-wine
stain. They present clinically as a rapidly growing solitary, red
papule that can sometimes be exophytic or pedunculated in nature.
•
• They often present with a crusted or eroded surface because of
their friable nature.
• They do not spontaneous involute, and treatment is often pursued
because of recurrent episodes of bleeding and their friability.

40. • Treatment - simple curettage or shave excision with electrocautery
of the base,most definitive and curative.
• Topical therapies, such as topically administered timolol therapy, a
beta blocker, or imiquimod therapy, or pulsed dye laser treatment,
which have variable success with prolonged treatment courses.
• Recurrence is possible

41. Kaposiform hemangioendothelioma
• KHE is an extremely rare vascular neoplasm. It is present at birth in
50% of patients. The tumor is often >5 cm in dimensions and
appears as a flat, reddish-purple, edematous lesion.
• Seventy percent of patients have Kasabach–Merritt phenomenon
(KMP) (thrombocytopenia <25,000/mm3 , petechiae, bleeding).
• KHE partially regresses after 2 years of age, although it usually
persists long term causing chronic pain and stiffness.
• Most lesions are extensive, involving multiple tissues, and well
beyond the limits of resection.
•

42. • Vincristine is first-line therapy; the response rate is 90%. KHE does
not respond as well to second-line drugs, interferon (50%), or
corticosteroid (10%).
• Recently, patients have been treated with sirolimus as first-line
therapy with good efficacy.
•
• Thrombocytopenia is not significantly improved with platelet
transfusion which should be avoided
unless there is active bleeding or
a surgical procedure is planned.
• By 2 years of age, the tumor
usually has undergone partial
involution and the platelet count normalizes.

43. Treatment of IH
• Most IHs are uncomplicated and spontaneously regress, but there is
a subset of approximately 10%–15% of IHs that result in
complications requiring treatment (Mulliken et al., 1995; Hoeger et al.,
2015).
• Treatment is indicated in cases complicated by disfigurement or risk
of disfigurement, ulceration, or functional compromise.
• There are various treatment modalities, including topical or
systemic medications, surgery, or laser therapy, that are chosen on
the basis of various factors, including the stage of growth, location,
potential complications, and associated conditions.

44. • Active Nonintervention. For most lesions the initial treatment of
choice is “active nonintervention.”
• This is reserved for hemangiomas that are not at risk of causing
disfigurement, ulceration, or causing any functional impairment.
• These often tend to be small focal hemangiomas in nonfacial
locations.
• Anticipatory guidance should be discussed with the family in regard
to the natural course of IHs and the initial period of rapid growth in
the first few months of life and then slow rate of involution over
many years.

45. • There is also a common concern of risk of significant hemorrhage.
This is rare in IHs.
• Minor episodes of bleeding can result from trauma and respond to
short-term compression, like any superficial wound.
• Although the large majority of IHs do not require treatment and
spontaneously regress.

46. Topical Therapies
• Topically administered timolol maleate is a nonselective β-
adrenergic receptor blocker. It has been shown to be most effective
for superficial and thin hemangiomas
• In general, timolol is well tolerated, without significant systemic
side effects.
• Caution should be used in preterm infants with postmenstrual age
less than 44 weeks and low birth weight infants weighing less than
2500 g at the time of initiation of treatment as there have been a
few case reports of symptomatic bradycardia in this group (Frommelt
et al., 2016).

47. Oral Propranolol
• Dosing is typically 1 to 3 mg/kg/day in three divided doses, given
till there is response (duration 6 to 12 months) and then gradually
tapered every 4 weeks.
• Approximately 90% of tumors will stop growing or regress.
• Risks (<3%) include bronchospasm, bradycardia, hypotension,
hypoglycemia, seizures, and hyperkalemia.
• Preterm infants and those <3 months of age are more likely to have
adverse events.

48. • Patients usually have cardiology consultation; electrocardiogram;
echocardiogram; glucose/electrolyte measurements; and frequent
blood pressure, heart rate, and respiratory examinations.
• Inpatient initiation of treatment is used for premature or infants <3
months of age.
• Potential contraindications include asthma, glucose abnormalities,
heart disease, hypotension, bradycardia, and PHACES (posterior
fossa abnormalties, hemangioma, arterial lesions, cardiac
abnormalities, eye problems, sternal notch or dimple) association.

49. Intralesional corticosteroid injections
• May be used in small, localized hemangiomas in cosmetically
sensitive areas with high rates of morbidity such as the lip, nasal tip,
and eyelid (Couto and Greene, 2014; Herlihy et al., 2016).
• This can be used as a therapy adjuvant to other topical or systemic
treatments.
• Complications include cutaneous atrophy, skin necrosis, and, for
intralesional periocular injections, ophthalmic artery occlusion and
blindness.
• Thus periocular intralesional steroid injection should be performed
only by experienced pediatric ophthalmologists.

50. Laser Therapy
• Pulsed dye laser treatment has been used.There have been some
controversial data regarding its use as a monotherapy for
uncomplicated hemangiomas, and it seems to be more efficacious
when used in conjunction with propranolol or timolol (Asilian et al.,
2015).
• It has also been used to heal ulcerations (David et al., 2003) and to
decrease residual erythema and telangiectasias in an involuting
hemangioma.
• However, a randomized prospective controlled trial of 121 infants
found that pulsed dye laser treatment in uncomplicated
hemangiomas is no better than watchful waiting (Batta et al., 2002).

51. • Pulsed dye laser treatment cannot prevent the preprogrammed
growth pattern of hemangiomas and has a limited role during the
proliferative phase.
• One risk of laser use during the proliferative phase can be
ulceration. In contrast, it can be useful during involution, if there is
residual redness or telangiectasias, which may be resolved faster
than with the natural course of involution, but has no effect on
fibrofatty residuum.

52. Surgical Excision
• Surgical excision can be done in cases of medically refractory
hemangiomas that are symptomatic and proliferating, in an
emergency situation where there is lifethreatening functional
compromise, or in situations with recurrent profuse bleeding.
• It may also be beneficial in situations where there is a large
disfiguring pedunculated lesion that will leave behind significant
fibrofatty residual or scar.

53. Wound Care
• Ulceration is a therapeutic challenge, but all ulcers benefit from
local wound care and potential occlusive dressings. The type of
dressing chosen depends on the amount of exudate and on the
location of ulceration.
• For sites that are difficult to dress, frequent and liberal application
of petrolatum jelly is effective.
• Various dressing materials, including petrolatum-impregnated gauze
and seaweed-derived alginate dressings, are often recommended.
• Agents for pain control should be considered, including topical
anesthetics and oral analgesics such as acetaminophen or ibuprofen.

54. • A high index of suspicion should be maintained for secondary
infection, with appropriate use of topical or oral antibiotics as
needed.
• If conservative therapy is unsuccessful, pulsed dye laser treatment
may relieve pain and speed reepithelialization (Achauer and Vander
Kam, 1991; Morelli et al., 1991, 1994).
• The ulcers will heal but will inevitably leave scars.

55. Capillary malformation
• Capillary malformations (CM) include the
1. nevus simplex (stork bite, angel kiss) and
2. cutaneous and/or mucosal CM (portwine stain).
• The cutaneous CM further include
(a) nonsyndromic CM,
(b) CM with CNS and/or ocular anomalies (Sturge Weber
syndrome)
(c) CM with bone and or soft tissue overgrowth,
(d) diffuse CM with overgrowth
• These lesions is obvious at birth, and the pink-purple
skin discoloration can cause psychosocial distress.

56. • Over time, cutaneous CM darkens and the soft tissue and bone may
enlarge underneath the stain.
•
• Nevus simplex, referred to as an “angel kiss” or “stork bite,” present in
one-half of Caucasian newborns, is located on the forehead, eyelids, nose,
upper lip, or posterior neck.
• This lesion is a fading capillary stain; no treatment is necessary because it
lightens over the first 2 years of life.
• The mainstay of treatment for portwine stain is is pulsed dye laser.
• Pulsed dye laser is less effective for capillary malformations that have
progressed to a dark color with cutaneous thickening.
• Surgical procedures are indicated to correct overgrowth caused by the
malformation.

57. Lymphatic malformation
• Lymphatic malformation is defined by the size of its channels: macrocystic,
microcystic, or combined.
• The most commonly affected sites are the neck and axilla.
• Lymphatic malformation can cause infection, bleeding, and psychosocial
morbidity.
• Macrocystic lesions contain cysts large enough to be accessed by a needle
(typically ≥5 mm) and are amenable to sclerotherapy.
• Microcystic lesions have cysts that are too small to be cannulated by a
needle (usually <5 mm) and thus cannot be treated by sclerotherapy.
• Approximately one-half of lymphatic malformations are not purely
macrocystic or microcystic;
• They contain both macrocysts and microcysts. Small, superficial lymphatic
malformations do not require further diagnostic evaluation.
• Large or deep lesions are evaluated by magnetic resonance imaging (MRI).

58. • Lymphatic malformation is benign, and, thus, intervention is not mandatory. Small,
asymptomatic lesions may be observed.
• First-line management for a large or problematic macrocystic/combined lymphatic
malformation is sclerotherapy.
• Sclerosant most commonly used is bleomycin.
• Generally, sclerotherapy gives superior results
and has lower morbidity compared to resection.
• Resection of a macrocystic lymphatic malformation is indicated if sclerotherapy is
no longer possible or if excision may be curative because the lesion is small.
• Symptomatic microcystic lesions are managed by resection which is typically
subtotal.
• Sirolimus recently has shown efficacy for very problematic microcystic lymphatic
malformations.

59. Venous malformation
• Lesions are blue, soft, and compressible. Hard calcified phleboliths may be
palpable.
• Lesions cause psychosocial morbidity as well as pain secondary to
congestion, thrombosis, and phlebolith formation.
• Patients with venous malformations are not at risk for thromboembolism
unless a large phlebectatic vein is connected to the deep venous system.
• Small, superficial venous malformations do not require further diagnostic
workup.
• Large or deep lesions are evaluated by MRI.
• Individuals with recurrent discomfort are given low-dose daily aspirin to
prevent phlebothrombosis.

60. • Intervention is reserved for symptomatic lesions or asymptomatic
phlebectatic areas at risk for thromboembolism.
• If possible, intervention should be postponed until after 12 months
of age when the risk of anesthesia is lowest.
• Therapy for lesions causing a visible deformity should be considered
before 4 years of age to limit psychological morbidity.
• Sclerotherapy typically is first-line treatment and is generally safer
and more effective than resection.
• Sclerosant most commonly used is sodium tetradecyl sulfate.
• Resection of a venous malformation should be considered for small
lesions that can be completely removed or for persistent symptoms
after completion of sclerotherapy

61. Arteriovenous malformation
• Arteriovenous malformation has an absent capillary bed which
causes shunting of blood directly from the arterial to the venous
circulation through a fistula (direct connection of an artery to a
vein) or nidus (abnormal channels bridging the feeding artery to the
draining veins).
• Lesions have a pink-red cutaneous stain, are warm, and can have
palpable pulsations.
• Patients are at risk for disfigurement, destruction of tissues, pain,
ulceration, bleeding, and congestive heart failure.
• Handheld Doppler examination shows fast flow.
• MRI is usually obtained to confirm the diagnosis and determine the
extent of the lesion.

62. • An angiogram is obtained if the diagnosis remains unclear following
ultrasound and MRI or if embolization is planned.
• Because the lesion is often diffuse and involves multiple tissue
planes, cure is rare.
• An asymptomatic arteriovenous malformation should be observed
unless it can be removed for possible cure with minimal morbidity.
• Embolization is generally first-line therapy for a symptomatic lesion.
•
• It is generally not curative, and most arteriovenous malformations
will reexpand following treatment.

63. • Resection of an arteriovenous malformation, when feasible, has a
lower recurrence rate when compared to embolization.
•
• Indications for resection include
(i) a well-localized lesion,
(ii) correction of a focal deformity, or
(iii) a symptomatic arteriovenous malformation that has
failed embolization.
• When excision is planned, preoperative embolization will facilitate
the procedure by minimizing blood loss.
• Excision should be carried out 24 to 72 hours after embolization,
before recanalization restores blood flow to the lesion.

64. Early infantile hemangioma in the “beard” distribution; this child was found
to have a subglottic hemangioma

65. Infantile hemangioma with minimal to arrested growth. (a) Telangiectatic patch with
peripheral pallor and small component of proliferation (darker red) at superior portion; (b)
Vascular patch studded with tiny proliferative vascular papules

66. Focal infantile hemangioma

67. References
• Avery's Diseases of the Newborn-10th edition
• Cloherty and Stark's Manual of Neonatal Care-south asian edition.
• American academy of pediatrics Clinical Practice Guideline for the
Management of Infantile Hemangiomas Daniel P. Krowchuk et al. Volume
143, number 1, January 2019: