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Journal Reading Diagnosis and Management of Infantile Hemangioma : Executive Summary Preceptor : dr. Robert ,

Diagnosis and Management of Infantile Hemangioma :

Reading Diagnosis and Management of Infantile Hemangioma : Executive Summary Preceptor : dr. Robert , Sp.A
Executive Summary
Executive Summary
and Management of Infantile Hemangioma : Executive Summary Preceptor : dr. Robert , Sp.A Presented by

Preceptor : dr. Robert , Sp.A Presented by : dr. Melisa Ayu

and Management of Infantile Hemangioma : Executive Summary Preceptor : dr. Robert , Sp.A Presented by
INTRODUCTION
INTRODUCTION

Infantile Hemangiomas (IH) : the most common tumours of childhood.

Resolve without intervention or consequences

Associated with complications, pain, functional impairment, or permanent disfigurement.

NOMENCLATURE
NOMENCLATURE

Infantile Hemangioma (IH)

Vascular malformation —> capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation.

Congenital hemangioma —> rapidly involuting (RICH) and non involuting (NICH)

Pyogenic granuloma or lobular capillary hemangioma

NOMENCLATURE
NOMENCLATURE

“Cavernous hemangioma” —> deep IH or venous malformation

Kasabach-Merritt phenomenon (consumptive coagulopathy) —> not associated with IH

deep IH or venous malformation • Kasabach-Merritt phenomenon (consumptive coagulopathy) —> not associated with IH
EPIDEMIOLOGY
EPIDEMIOLOGY

5% of infants, female/male ratio = 1.4:1 to 3:1

Risk factors of IH :

white race

prematurity

low birth weight

advanced maternal age

multiple gestation pregnancy

placenta previa

preeclampsia

EPIDEMIOLOGY
EPIDEMIOLOGY

Other risk factors :

in utero diagnostic procedure

use of fertility drugs or erythropoietin

breech presentation

first born

PATHOGENESIS & HISTOPATHOLOGY

PATHOGENESIS & HISTOPATHOLOGY • Pathogenesis of IH : • Intrinsic factors —> angiogenic & vasculogenic
PATHOGENESIS & HISTOPATHOLOGY • Pathogenesis of IH : • Intrinsic factors —> angiogenic & vasculogenic

Pathogenesis of IH :

Intrinsic factors —> angiogenic & vasculogenic factors

Extrinsic factors —> tissue hypoxia & developmental field disturbances

• Endothelial progenitor cells —> IH from clonal expansion —> vasculogenesis; alternatively, fetal progenitor cells arising from disruption of the placenta during gestation or birth.

Aberrant proliferation and differentiation of a pluripotent progenitor cell —> migrates to locations in which growth of placenta-like tissue is favorable —> IH

• Histologically : • Proliferative IHs —> well-defined masses of capillaries lined by plump endothelial

• Histologically :

• Histologically : • Proliferative IHs —> well-defined masses of capillaries lined by plump endothelial cells,

Proliferative IHs —> well-defined masses of capillaries lined by plump endothelial cells, arranged in lobules, often with enlarged draining veins.

Involuting IHs —> fibrofatty stroma, residual “ghost” vessels, mast cells, and apoptotic bodies.

• Immunohistochemical staining of IH is positive for glucose trans- porter 1 (GLUT1), CD31, CD34, factor VIII–related antigen, and others;

CLINICAL PRESENTATION
CLINICAL PRESENTATION

Appear before : 4 weeks, complete : 5 months, and continue up to 12 months

Deep IH —> Later & Longer. Involution phase : 6 - 12 months —> Flatten & shrink with fading of the color

IHs leaves residual skin changes :

including : telangiectasia, fibrofatty tissue, rendundant skin, atrophy, dyspigmentation, and scar

Proliferative phase, based on its depth :

• Proliferative phase, based on its depth :

Classification based on anatomic configuration :

Localised (focal) IH

Segmental IH

Indeterminate IH

Multifocal IH

based on anatomic configuration : • Localised (focal) IH • Segmental IH • Indeterminate IH •
Segmental Hemangioma
Segmental Hemangioma

segmen 1: frontotemporal

segmen 2 : maxillary

segmen 3 : mandibular

segmen 4 : frontonasal

Penyebaran sesuai dengan embriologi tonjolan wajah. segemn 1, pertumbuhan frontonasal membentuk philthrum dan memisahkan
Penyebaran sesuai dengan embriologi tonjolan wajah. segemn 1,
pertumbuhan frontonasal membentuk philthrum dan memisahkan
miaxilla. segmen mandibula menyatu di tengah. kadang hemangioma
mengenai >1 segmen, entirely atau sebagian
PHACE
PHACE

Kriteria PHACE :

Hemangioma in head (face atau scalp) >5cm2 + 1 major criteria or 2 minor criteria

Major criteria

anomali arteri cerebral mayor

Dandy-walker complex

aortic arch anomaly

posterior segment anomaly of the eye

sternal defects

Minor criteria : congenital cardiac lesions seperti ASD, VSD, PDA, etc

LUMBAR
LUMBAR

LUMBAR, which refers to

• Lower body IH and other cutaneous defects,

• Urogenital anomalies and Ulceration,

• Myelopathy,

• Bony deformities,

• Anorectal malformations, Arterial anomalies, and

• Renal anomalies.

Usually segmental lumbosacral or anogenital lesions. LUMBAR, also described under the acronyms “SACRAL” and “PELVIS,” may be thought of

as the “lower half of the body” variant of PHACE.

COMPLICATION
COMPLICATION

Function-threatening or life-threatening complications, including:

Ulceration

• risk factors: larger lesions, segmental IH, and distribution in the head, neck, perioral, and perineal or perianal locations

Bleeding

Visual impairment: include ptosis, amblyopia, astigmatism

Feeding impairment: IH on the lips or in the aerodigestive tract

Auditory impairment

Nasal deformities

Congestive heart failure: may occur with large IHs, as a result of arteriovenous shunting

Airway obstruction: risk of air- way IH increased with facial IH in the “beard” distribution

Hypothyroidism: with diffuse hepatic IH—> excess production of type 3 iodothyronine deiodinase

IMAGING
IMAGING

• Imaging of IH is not usually necessary.

• Imaging may be necessary when:

• the diagnosis is uncertain

• evaluation of extent is necessary

• the IH is a possible marker of PHACE or LUMBAR syndrome

• response to therapy must be monitored.

• When imaging of IH is performed, ultrasound is the preferred modality for diagnosis, whereas MRI is better to assess extent of the lesion.

CLINICAL APPROACH TO IH
CLINICAL APPROACH TO IH

• The indications for intervention for IH include:

• Emergency treatment of potentially life-threatening complications

• Urgent treatment of existing or imminent functional impairment, pain, or bleeding

• Evaluation to identify important structural anomalies potentially associated with IH

• Elective treatment to reduce the likelihood of long-term or permanent disfigurement

• Factors affecting the determination of appropriate modality and timing of intervention for IH :

• Age of the patient

• Growth phase of the lesion

• Location and size of the lesion

• Degree of skin involvement

• Severity of complications and urgency of intervention

• Potential for adverse psychosocial consequences

• Parental preference

• Physician experience

MANAGEMENT OF ULCERATED IH

MANAGEMENT OF ULCERATED IH • FOCUS : wound care, pain control, controlling IH growth, and prevention
MANAGEMENT OF ULCERATED IH • FOCUS : wound care, pain control, controlling IH growth, and prevention

• FOCUS : wound care, pain control, controlling IH growth, and prevention and treatment of secondary infection.

• First line therapy :

• Vigilant wound care

• Use of barrier ointments or dressings

• Topical antibiotics

• Ulcerated IH with suboptimal response to topical care :

• Medical —> propanolol

• Pulsed dye laser

• Surgical

MEDICAL THERAPY FOR IH

MEDICAL THERAPY FOR IH
Beta Blocker Therapy
Beta Blocker Therapy

Mechanism of action

Beta Blocker Therapy • Mechanism of action • vasoconstriction • inhibition of angiogenesis • down regulation

vasoconstriction

inhibition of angiogenesis

down regulation of matrix metaloproteinase and interleukin 6

regulation of Renin-Angiostensin System—> decrease renin

inhibition of NO

Stimulasi apoptosis

Pretreatment EGC

HR below normal for age :

newborns (<1 month old), <70 bpm

infants (1-12 months old), <80 bpm

children (>12 months old), <70 bpm

Family history of congenital heart conditions / arrythmias/maternal history of connective tissue disease

history of arrythmia or an arrythmia is auculated during examination

Contraindication

• cardiogenic shock,

• sinus bradycardia,

• hypotension,

• heart murmur, or family heart block greater than first degree, arrythmia

• heart failure,

• wheezing, asthma or reactive airway disease

• hypersensitivity to the drug.

• tachypnea, dyspnea

• poor feeding

propanolol pd PHACE syndrome meningkatkan terjadinya resiko stroke dengan menurunkan TD dan melemahkan aliran darah
propanolol pd PHACE syndrome
meningkatkan terjadinya resiko stroke
dengan menurunkan TD dan melemahkan
aliran darah pada pembuluh darah yang
teroklusi, sempit, dan stenotik
menyebabkan TD labil -> meningkatkan
resiko untuk stroke

• Special precautions : PHACE syndrome

Propranolol use in PHACE syndrome
Propranolol use in PHACE syndrome

Arterial anomalies : Increase risk for stroke—> dropping BP & attenuating blood flow & increasing variability in systolic BP

Cardiac & aortic arch anomalies : require Echocardiography —> consultation with cardiology

Extensive facial hematoma —> prime candidates for propranolol therapy

Evaluation : MRI/MRA of the head & neck, and cardiac imaging include aortic arch

Consultation & co-management with Neurology

Propranolol use in PHACE syndrome
Propranolol use in PHACE syndrome

Use the lowest dose

slow dosage titration upward

close observation including hospitalization in high risk infants

3 times daily dosing to minimize abrupt changes in systolic BP

Recommended administration of propranolol :

1 to 3.4 mg/kg per day divided into 2 or 3 doses, min 6 hours interval

Duration of therapy : 8 to 12 months of age, or between 3 and 12 months of therapy

Common adverse effects:

• sleep disturbance including nightmares, somnolence, cold hands and feet, diarrhea, gastroesophageal reflux, and bronchial hyperreactivity.

Serious adverse effects :

• asymptomatic hypotension

• Pulmonary symptoms related to direct blockade of adrenergic bronchodilation

• Hypoglycemia or hypoglycemic seizures

• asymptomatic bradikardia

• Hyperkalemia

Hypoglycemia
Hypoglycemia

Precautions to reduce the risk of hypoglycemia :

• administration of medication after feeding

• holding doses when the patient is ill with decreased oral intake, vomiting, or diarrhea

• avoiding prolonged intervals of longer than 6 hours between feedings.

Mild hypoglicemia :

• sweating, shakiness, takikardia, anxiety, & hunger

Severe hypoglycemia :

• lethargy, stupor, poor feeding, seizure, apnea, loss of consciousness, & hypothermia

Hyperkalemia
Hyperkalemia
Hyperkalemia • Hypothesis : • caused by tumor lysis syndrome (TLS) • impaired potassium uptake into

Hypothesis :

caused by tumor lysis syndrome (TLS)

impaired potassium uptake into cells caused by Beta-blockade

Start treatment of anti hyperkalemia when potassium level exceeds 6 mmol/L

Furosemid : bolus 1 mg/kg —> 1mg/kg/day —> 0.8 mg/kg/day divided 3 doses —> Day 10 : oral furosemid 0.5mg/ kg/day

Fluids

Nebulized salbutamol 6 times daily

Inpatient Hospitalization Criteria
Inpatient Hospitalization Criteria

infants <=8 weeks gestation, corrected age

infant with inadequate social support

any age infant with comorbid conditions affecting Cardiovascular system, respiratory system including symptomatic airway hemangiomas or blood glucose maintenance.

Monitoring patient in Propranolol therapy
Monitoring patient in Propranolol therapy

electrolyte status

blood glucose

hemodynamic monitoring

Corticosteroid Therapy
Corticosteroid Therapy

Oral prednisolone or prednisone at 2 to 3 mg/kg/day in a single morning dose. Treatment is more successful when initiated during IH proliferation. Side effects of long term use :

irritability

sleep disturbance

gastric irritation

hypertension

immunosuppresion

HPA axis suppression

Linear growth deceleration

cushingoid facies

Intralesional steroid injections : small, bulky, well- localised IH lesions

May systemically absorbed

Other Medical Therapies
Other Medical Therapies

Interferon Alpha

A promising treatment from 1980 - 1990

Significant neurotoxicity

Laser Treatment of IH

Laser Treatment of IH

Laser Treatment of IH

Useful for :

early non proliferating superficial lesions

salvaging critical skin

controlling ulceration

treating persisting post involution telangiectasia

Pulsed-dye laser : the light is preferentially absorbed by haemoglobin

The use of pulsed-dye laser on proliferating and segmental IH —> ulceration

The potential complications are :

atrophic scar

hypopigmentation

SURGICAL THERAPY FOR IH

SURGICAL THERAPY FOR IH

Indication of surgery during infancy:

failure of other therapy for a critical IH

a focal lesion in a favorable location

elective surgery leaving a scar that would be the same if the lesion were removed after involution

Delaying surgery until after infancy : safer, shorter scar, and a higher likelihood of a favourable outcome

Hemangiomas expand the skin —> after removed —> the wound usually can be reconstructed by linear closure

The goal of treatment : to improve the appearance of the child

For circular lesions located on the face —> circular excision and purse-string closure

IHs WITH SPECIAL ANATOMIC CONCERNS

IHs WITH SPECIAL ANATOMIC CONCERNS
IHs WITH SPECIAL ANATOMIC CONCERNS
EYELID IH
EYELID IH

IH can deform cornea & obstruct visual axis —> astigmatism, strabismus, amblyopia.

Propanolol has largely supplanted by intralesional steroid injection of the upper eyelid —> as an alternative for refractory lesions.

AIRWAY IH
AIRWAY IH

Occuring in isolation —> greater risk in patient with large cutaneous lesions involving the lower face and neck (‘beard’ distribution).

Most lesions : subglottic —> biphasic stridor and barky cough

Operative endoscopy : to identify lesions and assess their extent

NASAL TIP IH
NASAL TIP IH

Early management : combination of pharmacotherapy and laser —> reduces:

long term deformity from skin expansion

atrophy and of injury to the underlying cartilage.

Reconstruction of involuted nasal tip :

excision of the lesion

reconstruction of the cartilaginous framework

excision and redraping of the skin.

LIPS & PERINEUM IH
LIPS & PERINEUM IH

Higher risk of ulceration —> early pharmacotherapy

Perineal IH : reduction of friction using topical lubrication with or without a barrier dressing

Vermilion area of the lip : transverse mucosal incision.

Bulkier lesion of the lip : wedge excision

Eversion of the lower lip : excision of the mucosal strip, Inversion : lyophilises dermal implant or dermal graft

LIVER IH
LIVER IH

• Infants with >= 5 cutaneous hemangiomas —> greater risk of hepatic lesions—> screening by ultrasonography.

• Liver hemangiomas can be focal, multifocal, or diffuse.

• Focal lesions —> RICHs : fully grown at birth and rapidly involute

• Multifocal and diffuse lesions—> true IH and often with cutaneous lesions.

• Symptomatic multifocal and diffuse lesions —> systemic pharmacotherapy.

• Infants with significant multifocal or diffuse hemangiomas —> thyroid hormone screening—> hormone replacement

• Diffuse lesions may present with hepatomegaly —>abdominal compartment syndrome.

CONCLUSIONS

Although many IHs can be observed without treatment, others will clearly benefit from medical or surgical intervention.

When complications are likely or the threshold for intervention is uncertain, referral to an experienced specialist or a multidisciplinary vascular anomaly center may be advantageous.

It is important for providers of pediatric care to keep abreast of advances in IH management as continued research leads to pathogenesis-directed therapeutic options and as the types of intervention and their use evolve.

THANK YOU
THANK YOU