Post on 30-Jul-2020
transcript
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Early Diagnosis and Intervention of Vascular Anomalies
(Infantile Hemangiomasand Malformations)
Bernard A. Cohen, MD, FAAPProfessor of Dermatology and PediatricsJohns Hopkins Center
Linda Rozell-Shannon, PhDPresident and FounderVascular Birthmarks Foundation
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Disclaimer Presenter Bernard A. Cohen, MD, FAAP
o I have no financial disclosures.
o I am a member of the International Society for the Study of Vascular Anomalies (ISSVA).
o There is finally a US Food and Drug Administration (FDA)-approved treatment for infantile hemangiomas.
o I will discuss off-label use of medications for infantile hemangiomas.
o Darrow DH, Greene AK, Mancini AJ, Nopper AJ, American Academy of Pediatrics Section on Dermatology, Section on Otolaryngology–Head and Neck Surgery, and Section on Plastic Surgery. Diagnosis and management of infantile hemangioma. Pediatrics. 2015;136(4):e1060–e1104.
Statements and opinions expressed are those of the authors and not necessarily those of the American Academy of Pediatrics.
Mead Johnson sponsors programs such as this to give healthcare professionals access to scientific and educational information provided by experts. The presenters have complete and independent control over the planning and content of the presentation, and are not receiving any compensation from Mead Johnson for this presentation. The presenters’ comments and opinions are not necessarily those of Mead Johnson. In the event that the presentation contains statements about uses of drugs that are not within the drugs' approved indications, Mead Johnson does not promote the use of any drug for indications outside the FDA-approved product label.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
What’s new (and old but relevant) onInfantile Hemangiomas
(and other assorted vascular lesions)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Webinar Outline
Definitions…vascular tumors of infancy
Pathogenesis, morphology, course
Management of uncomplicated infantile hemangioma (IH)
High risk lesions…segmental, PHACES
New variants you should know
Rx options for complicated lesions
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
First a little quiz…Hemangioma (A) or malformation (B)?
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
A CapillaryB VenousC LymphaticD ArteriovenousE Combined
Hemangioma (A) or malformation (B)?
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
A CapillaryB VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
A Capillary
B Venous
C Lymphatic
D Arteriovenous
E Combined
Hemangioma (A) or malformation (B)?
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Hemangioma (A) or malformation (B)?
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.Hemangioma (A) or malformation (B)?
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.Hemangioma (A) or malformation (B)?
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.Hemangioma (A) or malformation (B)?
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma (A) or malformation (B)?
A Capillary B VenousC LymphaticD ArteriovenousE Combined
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Vascular Tumor vs Vascular Malformation
Vascularanomalies
Vasculartumor
Infantilehemangioma
Kaposiform hemangioendo-
thelioma
Tufted angioma and other tumors
Vascular malformation
Venous, arterial, AVM, capillary,
lymphatic
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69(3):412–422; Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (new issues). Adv Dermatol. 1997;13:375–423; and Workshops of the International Society for the Study of Vascular Anomalies (ISSVA): 1996 and April 2014 (www.issva.org/workshops).
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Definitions: Distinguish from Vascular Malformations
Infantile Hemangioma
Usually not presentat birth
Dynamic
Regressing
Proliferative
Vascular Malformation
Present at birth
Static
Persistent
Non-proliferative
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Infantile Hemangiomas
Incidence 8–10% at 2 months
(<1% of newborns)
Family history 8–10%
Locationo Head, neck 50%
o Trunk 30%
o Extremities 20%
(Data from multiple observational studies over last 5 decades.)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Newer Epidemiologic Data Female predominance: 2.4:1
o Previously published: 1.4–4:1 White, non-Hispanic Twins – usually only 1 affected Mothers – increased age, placental abnormalities Prematurity Low birth weight
o #1 risk factor for IHo For every 500-g decrease in birth weight, risk of IH increases 25%o Hemangiomas in 1 in 4 infants <1,000 g
Chamlin SL, Haggstrom AN, Drolet BA, et al. Multicenter prospective study of ulcerated hemangiomas. J Pediatrics. 2007;151(6):684–689; Drolet BA, Swanson EA, Frieden IJ, Hemangioma Investigator Group. Infantile hemangiomas: an emerging health issue linked to an increased rate of low birth weight infants. J Pediatr. 2008;153(5):712–715; Darrow DH, Greene AK, Mancini AJ, Nopper AJ, American Academy of Pediatrics Section on Dermatology, Section on Otolaryngology–Head and Neck Surgery, and Section on Plastic Surgery. Diagnosis and management of infantile hemangioma. Pediatrics. 2015;136(4):e1060–e1104.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Pathogenesis
Dysregulation of angiogenesis
Imbalance of pro- and anti-angiogenic factors
Dysregulation of endothelial cell proliferation
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Storch CH, Hoeger PH. Propranolol for infantile haemangiomas: insights into the molecular mechanisms of action. Br J Dermatol. 2010;163(2):269–274.
VEGF & bFGF in serum during proliferative phase and in involuting phase
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Infantile Hemangiomas: Morphology
Superficial
Deep (not to be confused with venous malformation)
Most both
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Superficial
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Subcutaneous
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Combined superficial/deep
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangioma Patterns
Focal (localized)
Multifocal (multiple localized)
Segmental
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Infantile Hemangioma
Size 1 mm–20 cm
Number 85% 1 lesion
Rare >100
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Infantile Hemangioma: Early Course
Early, pale macule, central telangiectasia
Growth phase (510 patients [Lampe, 1965])50% until 6 months50% until 12 months80–90% < X 2
5% X 32% X 4
85% peak by 3 months (most rapid growth 2–7 weeks)
Certain lesions with prolonged growth phaseBrandling-Bennett HA, Metry DW, Baselga E, et al. Infantile hemangiomas with unusually prolonged growth phase: a case series. Arch Dermatol. 2008;144(12):1632–1637, and Darrow DH, Greene AK, Mancini AJ, Nopper AJ, American Academy of Pediatrics Section on Dermatology, Section on Otolaryngology–Head and Neck Surgery, and Section on Plastic Surgery. Diagnosis and management of infantile hemangioma. Pediatrics. 2015;136(4):e1060–e1104.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
1 week 2 weeks 3 weeks
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Uncomplicated Infantile Hemangioma: Prognosis
(Generally) independent ofo Sizeo Numbero Sexo Locationo Growtho Prematurityo Presence of deep component
But focal vs multifocal vs segmental is important
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Uncomplicated Infantile Hemangioma: Management
Complete physical examination
Close observation
Photodocumentation (website)
Avoidance of aggressive therapy
Parent counseling
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Infantile Hemangiomas: Complications
Involvement of vital structureso Airway
o Eye
o Urethra, anus
o Gastrointestinal (GI) tract
Infection/ulceration (local, sepsis)
Pain
Cardiac failure (liver lesions, and large lesions anywhere)
Body image
Special patterns (eg, segmental)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Risk of Complications Data from 1,058 cohort
o Haggstrom AN, Drolet BA, Baselga E. Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment. Pediatrics. 2006;118(3):882–887
o Darrow DH, Greene AK, Mancini AJ, Nopper AJ, American Academy of Pediatrics Section on Dermatology, Section on Otolaryngology–Head and Neck Surgery, and Section on Plastic Surgery. Diagnosis and management of infantile hemangioma. Pediatrics. 2015;136(4):e1060–e1104.
Increased risk with large size, facial location, segmental morphology for short term outcome = complication (24%) and treatment (38%)
Rx for ulcer (23%), eye (6.9%), airway (1.8%), auditory canal (1.1%), cardiac (0.4%)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Labial hemangioma
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Buttock hemangioma
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Disfiguring Eye/airway involvement
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
High Risk Subtypes
Chang LC, Haggstrom AN, Drolet BA, et al. Growth characteristics of infantile hemangiomas: implications for management. Pediatrics. 2008;122(2):360–367.
Large, facial segmental PHACES
Nasal tip, ear, large facial Disfigurement, scarring
Periorbital or retrobulbar Ocular axis occlusion, astigmatism, amblyopia, tear duct obstruction
Segmental “beard area” (S3) Airway IH
Perioral Ulceration, disfigurement, feeding difficulties
Segmental over lumbosacral spine
Tethered cord, genitourinary anomalies, PELVIS
Perineal, axilla, neck, perioral Ulceration
Anogenital area
_______________________Multifocal
Caudal regression syndromes (PELVIS, SACRAL, LUMBAR)_______________________________Visceral involvement (liver, GI)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Lumbosacral Hemangiomas
Spinal dysraphism
Anomalies of bony and soft tissue cord
Consider ultrasound before 6 months
Magnetic resonance study after 6 months—requires sedation
Many subsets of segmental hemangiomas
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Complicated Presentations Cervicofacial (Beard IH)
63% association with upper
airway hemangiomas
33% of airway hemangiomas with cutaneous hemangioma
Stridor, cough, cyanosis, hoarseness
~40% with airway hemangioma will require tracheostomy
Orlow SJ, Isakoff MS, Blei F. Increased risk of symptomatic hemangiomas of the airway in association with cutaneous hemangiomas in a "beard" distribution. J Pediatr. 1997;131(4):643–646, and Chatrath P, Black M, Jani P, Albert DM, Bailey CM. A review of the current management of infantile subglottic haemangioma, including a comparison of CO(2) laser therapy versus tracheostomy. Int J Pediatr Otorhinolaryngol. 2002;64(2):143–157.
Orlow SJ, Isakoff MS, Blei F. Increased risk of symptomatic hemangiomas of the airway in association with cutaneous hemangiomas in a "beard" distribution. J Pediatr. 1997;131(4):643–646, with permission from Elsevier.
Orlow SJ, Isakoff MS, Blei F. Increased risk of symptomatic hemangiomas of the airway in association with cutaneous hemangiomas in a "beard" distribution. J Pediatr. 1997;131(4):643–646, with permission from Elsevier.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.Segmental/field/non-localized hemangioma
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
PHACES
Posterior fossa vascular malformations Hemangiomas Arterial anomalies Coarctation of aorta, cardiac defects Eye abnormalities (microphthalmia, optic nerve
hypoplasia, cataracts, increasedretinal vascularity)
Sternal clefting +/- supraumbilicalraphe
Metry D, Heyer G, Hess C, et al. Consensus statement on diagnostic criteria for PHACE syndrome. Pediatrics. 2009;124(5):1447–1456.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Complicated Presentations Facial Segmental
Waner M, North PE, Scherer KA, Frieden IJ, Waner A, Mihm MC Jr. The nonrandom distribution of facial hemangiomas. Arch Dermatol. 2003;139(7):869–875.
Frontonasal
(S4)
Maxillary (S2)
Mandibular
(S3)
Frontotemporal
(S1)
V
I
Higher correlation with structural cerebral and cerebrovascular anomalies
Higher correlation with ventral and cardiac defects, including coarctation
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
PHACE(S)
Female-to-male ratio: 9:1
>20% infants with facial IH: segmental hemangioma
o 2% of all hemangiomas
o ? More common than Sturge-Weber
Diagnosis with hemangioma and 1 extracutaneous finding
Metry DW, Haggstrom AN, Drolet BA, et al. A prospective study of PHACE syndrome in infantile hemangiomas: demographic features, clinical findings, and complications. Am J Med Genet A. 2006;140(9):975–986.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Another Segmental Hemangioma
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Segmental Hemangiomas
Complications
Greater need for treatment
Worse outcome
Associated structural anomalies
Segmental lesions: risk for visceral hemangiomas
o Liver > GI, brain, mediastinum
o 25% mortality
Chiller KG, Passaro D, Frieden IJ. Hemangiomas of infancy: clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex. Arch Dermatol. 2002;138(12):1567–1576; Metry DW, Hawrot A, Altman C, Frieden IJ. Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Arch Dermatol. 2004;140(5):591–596; and Drolet BA, Dohil M, Golomb MR, et al. Early stroke and cerebral vasculopathy in children with facial hemangiomas and PHACE association. Pediatrics. 2006;117(3):959–964.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Infantile Hemangiomas: Risk of Slow Regression or Scarring
Parotid
Lip
Tip of nose
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Infantile Hemangioma Variants
RICH (rapidly involuting)
NICH (non-involuting)
PICH (partially involuting)
Features of IH, vascular malformations
Glut-1 negative
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Rapidly Involuting Congenital Hemangioma
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
RICH
Develop in utero
No postnatal growth
50% gone by 7 months
Glut-1 negative – not = IH
Some histologic features of IH
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Non-involuting Congenital Hemangioma
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
NICH
Develop in utero
May grow somewhat
Glut-1 negative – not = IH
High flow = IH
Persistent
Surgical excision – not recurrence
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Another VariantMinimal Growth Infantile Hemangioma
Suh KY, Frieden IJ. Infantile hemangiomas with minimal or arrested growth: a retrospective case series. Arch Dermatol. 2010;146(9):971–976.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangiomas: Rx
Systemic corticosteroidsEdgerton (1967) Esterly (1968)Brown (1972) Feingold (1978)
Intralesional steroidso Central retinal artery occlusiono Eyelid necrosiso Atrophy, hematomao Eyelid depigmentationo Growth delay
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Systemic Steroids: Other Considerations
Hypertensiono Particularly with high doseso Monitoring?o Long-term risk
Growtho Dose, length of Rxo Catch up growth
Behavioral changes/central nervous system development Adrenal suppression Fungal infection Other risks…
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Newly approved stuff…
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Therapy: Propranolol
Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A. Propranolol for severe hemangiomas of infancy. New Engl J Med. 2008:358 (24):2649–2651.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Vasoconstriction of supplying capillaries
o Visible color change in first 48 hours
o From decreased release of nitric oxide
Inhibition of angiogenesis
o Effects on pro-angiogenic growth factors, VEGF &
bFGF, MMP-2 & MMP-9
o Arrest of growth
Induction of apoptosis
o Regression of IH
65
Storch CH, Hoeger PH. Propranolol for infantile haemangiomas: insights into the molecular mechanisms of action. Brit J Dermatol. 2010;163(2):269–274.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
July 22, 2008
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
August 13, 2008 (before Rx)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
August 14, 2008 (day 1)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
August 15, 2008 (day 2)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
August 16, 2008 (day 3)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
August 28, 20082 weeks later
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
September 22, 20081 month later
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Our Experience: Methods
Retrospective analysis in 70 patients with function-threatening or disfiguring cutaneous hemangiomas treated with propranolol
o Response to therapy
o Complications (hypoglycemia, hypotension, cool hands and feet, etc.)
o No serious adverse effects
o Drop in blood pressure with first dose but not clinically important
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Ongoing experience…
Over 1,000 babies
No serious complications
Our current dosing: 2 mg/kg/d (We are off-label!)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
New stuff…
First drug ever approved for treating IH
Great safety and efficacy data
Most exciting drug discovery and implementation in my career
Easy to access
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Hemangeol (propranolol hydrochloride 4.28 mg/mL)
Phase II/III clinical trial
60.4% complete or nearly complete resolution compared to 3.6% placebo
88% improved at week 5
Most common adverse drug reactions: ~10% sleep disorders, aggravated upper respiratory infections, diarrhea, vomiting
<2% stopped medication for safety
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
With permission, Puttgen K.
Topical timolol for superficial IH, PG, etc.
6.29.156.1.15
8.25.15 8.25.15
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
More Cool Stuff…
Topical beta-blockers
Collaboration with pedsplastics on rebounding IH
Scalp, periocular, nasal tip lesions
Identification of IH requiring early intervention
Other segmental IH
Risk factors for hemangiomas
Hemangiomatosis and visceral lesions
Vascular malformations (oral sirolimus, topical sirolimus, etc.)
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Impact of Vascular Anomalies on the Family System Linda Rozell-Shannon (April 2017)
For new parents, having an infant is stressful, but when that infant is diagnosed with a potential disfiguring vascular anomaly, parents will experience additional stress.
Wandering from doctor to doctor to find an accurate diagnosis and appropriate treatment plan can result in symptoms of acute stress, disruption to normal family routines, and feelings of helplessness and hopelessness by the entire family, primarily the parents.
Additionally, the stress from the uncertainty over how large or disfiguring these lesions can become can interfere with maternal bonding as the mother becomes fixated on treatment, missing significant milestones in the infant’s normal development.
To further complicate the matter, insurance companies routinely deny the treatment of benign vascular anomalies leaving the families feeling helpless and hopeless.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Studies conducted to assess the impact of having an infant with a facial vascular anomaly concluded that there was an interference with normal maternal bonding, the fear of the unknown negatively impacted the family system, and inconsistent information from physicians and potential denial of treatment by insurance providers resulted in symptoms of acute stress.
Due to the lack of consistent information regarding the diagnosis and treatment of these lesions, families often seek outside sources, such as not for profits, that provide accurate information and support to the affected families.
Outdated medical information that promotes a “benign neglect” philosophy further complicates the fact finding of parents who learn about early treatment options on the internet through organizations such as the Vascular Birthmarks Foundation.
Online medical advice provided by vascular anomalies experts provides hope to the families but is often impeded by insurance companies who deny out of network treatment. As a result, families experience cyclical highs and lows as they wander from doctor to doctor and internet resources to internet resources trying to find appropriate treatment options.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
What can be done????
Affected families need to know they are not alone. Support groups, such as the Vascular Birthmarks Foundation, exist, which can connect families newly diagnosed with families who have successfully navigated the diagnosis and treatment process.
Affected families need to know there are treatment options available, and these options should be presented and discussed, weighing pros and cons.
Outdated benign neglect protocol needs to be abandoned for a more appropriate approach to early intervention.
Babies need to be referred early for treatment, following the 4-week well baby check up.
If needed, practitioners need to provide documentation for the medical necessity for treatment.
Last, but not least, physicians need to be cognizant and sensitive to the fact that having an infant with a potentially problematic and disfiguring vascular anomaly can interfere with maternal bonding, as well as the entire family system. In some instances, families may need to be referred to a social worker or appropriate mental health expert to resolve any psychosocial issues.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Treat with confidence. Trusted answers from the American Academy of Pediatrics.Treat with confidence. Trusted answers from the American Academy of Pediatrics.
Visit Pediatric Care Online today for additional information on this and other topics.
http://pediatriccare.solutions.aap.org
Pediatric Care Online is a convenient electronic resource for immediate expert help with virtually every pediatric clinical information need with must-have resources that are
included in a comprehensive reference library and time-saving clinical tools.
Don’t have a subscription to PCO?
Then take advantage of a free trial today!Call Mead Johnson Nutrition at 888/363-2362 or,
for more information, go to http://pediatriccare.solutions.aap.org/SS/Free_Trial.aspx