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    Pediatrics
    October 2015, VOLUME 136 / ISSUE 4
    From the American Academy of Pediatrics
    Clinical Report

    Diagnosis and Management of Infantile Hemangioma

    David H. Darrow, Arin K. Greene, Anthony J. Mancini, Amy J. Nopper, the SECTION ON DERMATOLOGY, SECTION ON OTOLARYNGOLOGY–HEAD AND NECK SURGERY, and SECTION ON PLASTIC SURGERY
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    • FIGURE 1
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      FIGURE 1

      RICH is fully formed at birth (A) and then involutes, mostly during the first year of life. B, The same lesion seen at 8 months of age.

    • FIGURE 2
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      FIGURE 2

      Pyogenic granulomas have some clinical and histologic features similar to IHs, but they are generally smaller, pedunculated, and more likely to bleed.

    • FIGURE 3
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      FIGURE 3

      A, The venous blood contained within a venous malformation imparts a bluish hue that may lead to misdiagnosis as a deep IH. B, Bleeding into surface vesicles of a lymphatic malformation may lead to misdiagnosis as an IH.

    • FIGURE 4
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      FIGURE 4

      Proliferative phase IH. Well-circumscribed lobules of closely packed capillaries composed of plump endothelial cells and pericytes are separated by normal-appearing dermal stromal elements (hematoxylin and eosin stain; original magnification ×100; photo courtesy of Paula North, MD.)

    • FIGURE 5
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      FIGURE 5

      Involutive phase IH. Lesional capillaries are set within loose fibro-adipose tissue and are less densely packed than in the proliferative phase. Note the thickened and hyalinized basement membranes studded with apoptotic debris, reflective of the involutive process. Residual lining endothelial cells are mitotically inactive. (Photo courtesy of Paula North, MD)

    • FIGURE 6
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      FIGURE 6

      Cutaneous IHs may be classified on the basis of their depth. A, Superficial IHs are visible only at the skin surface and may be focal (as shown) or segmental. B, Deep IHs have no surface involvement. C, Mixed, or compound, IHs have both superficial and deep components.

    • FIGURE 7
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      FIGURE 7

      Abortive IHs are macular, telangiectatic patches that have failed to fully proliferate.

    • FIGURE 8
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      FIGURE 8

      (A) Patterns of segmental IH of the face extracted from image analysisdefined. Seg1 (frontotemporal), Seg2 (maxillary), Seg3 (mandibular), and Seg4 (frontonasal). (B) An ulcerated segmental IH in the maxillary distribution.

    • FIGURE 9
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      FIGURE 9

      Multifocal cutaneous IHs in a child with IH of the liver.

    • FIGURE 10
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      FIGURE 10

      Ulcerated segmental IH of the perineal/perianal region.

    • FIGURE 11
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      FIGURE 11

      A, The presence of multiple IHs in the “beard” distribution is associated with a higher likelihood of airway involvement (reproduced with permission from J Pediatr. 1997;131(4):643–646 ©Elsevier).106 B and C, Patient with airway involvement requiring tracheotomy is shown with “beard” involvement at the lip and chin (B) as well as the parotid area and neck (C).

    • FIGURE 12
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      FIGURE 12

      A, Frontotemporal segmental IH typical of PHACE syndrome. B, Sternal clefting characteristic of PHACE syndrome (scar is congenital, not surgical).

    • FIGURE 13
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      FIGURE 13

      Uncomplicated IHs. These lesions generally do not require medical or surgical intervention.

    • FIGURE 14
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      FIGURE 14

      IH of the left eye causing visual field cut and astigmatism. Untreated, the lesion could proliferate, potentially resulting in deprivation amblyopia.

    • FIGURE 15
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      FIGURE 15

      Airway IH extending from the vocal folds inferiorly into the subglottic space, the narrowest region of the pediatric airway. (Photo courtesy of Jonathan Perkins, DO.)

    • FIGURE 16
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      FIGURE 16

      Open rhinoplasty approach to nasal tip IH. These lesions (H) originate within intercartilaginous ligament of the lower lateral nasal cartilages (arrows), rotating them outward.

    • FIGURE 17
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      FIGURE 17

      A, Compound IH of the nasal tip with significant surface involvement. B, Nasal tip after treatment with PDL to salvage tip skin before surgical resection.

    • FIGURE 18
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      FIGURE 18

      Ulcerated IH of the lower lip has resulted in distortion of the soft tissue and obliteration of the vermilion-cutaneous border.

    Tables

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    • TABLE 1

      Classification of Cutaneous Vascular Anomalies, 2014

      Vascular malformations
       Venous malformations
       Lymphatic malformations
       Capillary malformations
       Arteriovenous malformations and fistulae
       Mixed (combined) malformations
      Vascular tumors
       Benign
       Infantile hemangioma (IH)
       Congenital hemangioma (rapidly involuting [RICH]; non-involuting [NICH])
       Lobulated capillary hemangiomas (LCH) (pyogenic granuloma)*
       Tufted angioma (TA)
       Others
       Locally aggressive
       Kaposiform hemangioendothelioma (KHE)
       Kaposi sarcoma
       Others
       Malignant
       Angiosarcoma
       Others
      • Adapted from the International Society for the Study of Vascular Anomalies, 2014, ref 1 (issva.org/classification).

      • ↵* Reactive proliferating vascular lesion

    • TABLE 2

      Consensus Algorithm for the Diagnosis of PHACE Syndrome

      PHACE SyndromePossible PHACE Syndrome
      Facial hemangioma >5 cm in diameter plus 1 major criterion or 2 minor criteriaFacial hemangioma >5 cm in diameter plus 1 minor criterionHemangioma of the neck or upper torso plus 1 major criterion or 2 minor criteriaNo hemangioma plus 2 major criteria
      • Adapted from ref 25.

    • TABLE 3

      Consensus Diagnostic Criteria for PHACE Syndrome

      Organ SystemMajor CriteriaMinor Criteria
      CerebrovascularAnomaly of major cerebral arteriesDysplasiaa of the large cerebral arteriesbArterial stenosis or occlusion with or without moyamoya collateralsPersistent embryonic artery other than trigeminal arteryProatlantal intersegmental artery (types 1 and 2)Primitive hypoglossal arteryPrimitive otic artery
       Absence or moderate-severe hypoplasia of the large cerebral arteries
       Aberrant origin or course of the large cerebral arteriesb
       Persistent trigeminal artery
       Saccular aneurysms of any cerebral arteries
      Structural brainPosterior fossa anomalyDandy-Walker complex or unilateral/bilateral cerebellar hypoplasia/dysplasiaEnhancing extraaxial lesion with features consistent with intracranial hemangiomaMidline anomalyc
      Neuronal migration disorderd
      CardiovascularAortic arch anomalyVentricular septal defect
       Coarctation of aortaRight aortic arch (double aortic arch)
       Dysplasiaa
       Aneurysm
      Aberrant origin of the subclavian artery with or without a vascular ring
      OcularPosterior segment abnormalityAnterior segment abnormality
       Persistent hyperplastic primary vitreous MicrophthalmiaSclerocornea
       Persistent fetal vasculature Coloboma
       Retinal vascular anomalies Cataracts
       Morning glory disc anomaly
       Optic nerve hypoplasia Coloboma
       Peripapillary staphyloma
      Ventral or midlineSternal defectHypopituitarism
       Sternal cleftEctopic thyroid
       Supraumbilical raphe
       Sternal defects
      • Adapted from ref 25.

      • ↵a Includes kinking, looping, tortuosity, and/or dolichoectasia.

      • ↵b Internal carotid artery, middle cerebral artery, anterior cerebral artery, posterior cerebral artery, or vertebrobasilar system

      • ↵c Callosal agenesis or dysgenesis, septum pellucidum agenesis, pituitary malformation, or pituitary ectopia.

      • ↵d Polymicrogyria, cortical dysplasia, or gray matter heterotopia.

    • TABLE 4

      Treatment Options in the Management of Ulcerated IH

      Wound CareAdjuvant Therapies
      DressingsAntimicrobials
       White petrolatum–impregnated gauze Metronidazole gel
       Nonadherent dressings (eg, Mepitel [Mölnlycke Health Care; Gothenburg, Sweden], Telfa [Covidien/Medtronic; Minneapolis, MN]) Mupirocin, gentamicin, bacitracin ointmentPain controlTopical
       Hydrocolloid dressings (eg, DuoDERM [ConvaTec; Luxembourg])  Anesthetics (eg, lidocaine, benzocaine)Oral
      Topical agents  Acetaminophen with or without narcotics
       White petrolatum, Aquaphor [Beiersdorf Inc.; Hamburg, Germany], Silver sulfadiazine (Silvadene; Monarch Pharmaceuticals; Bristol, TN)
      Other
       Becaplermin gel
       Topical timolol
       PDL
       Early excision
       Oral propranolol or steroids
      • Adapted from ref 368.

    • TABLE 5

      Contraindications and Potential Complications Associated With Propranolol Therapy

      ContraindicationsComplications
      Sinus bradycardiaSinus bradycardia
      HypotensionHypotension
      Greater than first-degree heart blockDiarrhea
      Heart failureCool extremitiesSleep disturbanceReactive airways
      Cardiogenic shockHypoglycemia/seizures
      Reactive airways
      Hypoglycemia
      Hypersensitivity to propranolol hydrochloride
      • Adapted from ref 161.

    • TABLE 6

      Potential Adverse Effects of Systemic Corticosteroids

      HPA axis suppression
      Cushingoid features
      Growth deceleration
      Weight gain/increased appetite
      Hypertension
      Gastric irritation
      Irritability
      Insomnia
      Immune suppression
      Cardiomyopathy
      Steroid myopathy
      Osteopenia
      Ocular adverse effects (glaucoma, cataracts)
      • HPA, hypothalamic-pituitary-adrenal.

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    Diagnosis and Management of Infantile Hemangioma
    David H. Darrow, Arin K. Greene, Anthony J. Mancini, Amy J. Nopper, the SECTION ON DERMATOLOGY, SECTION ON OTOLARYNGOLOGY–HEAD AND NECK SURGERY, and SECTION ON PLASTIC SURGERY
    Pediatrics Oct 2015, 136 (4) e1060-e1104; DOI: 10.1542/peds.2015-2485

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    Diagnosis and Management of Infantile Hemangioma
    David H. Darrow, Arin K. Greene, Anthony J. Mancini, Amy J. Nopper, the SECTION ON DERMATOLOGY, SECTION ON OTOLARYNGOLOGY–HEAD AND NECK SURGERY, and SECTION ON PLASTIC SURGERY
    Pediatrics Oct 2015, 136 (4) e1060-e1104; DOI: 10.1542/peds.2015-2485
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