R.I.C.H.

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R.I.C.H.
Rapidly Involuting Congenital Hemangioma

• Grand Rounds
• October 2005
• Speaker Jay C. Bradley, MD
• Discussant Michael J. Shami, MD

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Report of Case

• Premature female referred for choroidal
  hemangioma OS discovered at ROP screening exam
• 1 pound 10 ounces, 27 weeks gestational age at
  first exam
• MRI of brain/orbits/liver c/s contrast NL

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Choroidal hemangioma OS
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Hemangiomas also present on right upper lid and
right hip
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Hemangioma resolution at 2 yr F/U
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Types of Congenital Hemangiomas

• Typical infantile hemangioma
• NICH (Non-involuting congenital hemangioma)
• RICH (Rapidly involuting congenital hemangioma)

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Typical Infantile Hemangiomas

• 1-2 of neonates
• Up to 12 of Caucasian infants by 1 year
• Female gt Males (3-51)
• Up to 23 of premature infants
• Manifest postnatally (median of 2 weeks)

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Typical Infantile Hemangiomas

• Grows rapidly during 1st year of life
• Involutes slowly from 1 7 years
• Completely regresses by 8 12 years
• Glucose transporter-1 protein present

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N.I.C.H.

• Present before birth
• Does not involute
• Grows in proportion with patient
• Similar to RICH in appearance, location, size,
  and sex distribution

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R.I.C.H

• Fully grown at birth
• Male Female
• Diagnosis possible as early as 12 weeks gestation
  by U/S
• Rapid 2nd trimester growth with 3rd trimester
  plateau
• Involuted rapidly by 12 18 months
• No glucose transporter-1 protein

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R.I.C.H.

• Most common locations
• Head neck
• Extremities, close to joint (ie elbow, shoulder,
  knee or hip)
• Rare on trunk / liver / sacrococcygeal area
• No previously reported choroidal involvement
• Inhomogeneous areas and larger flow voids on MRI
• Aneurysms on angiography

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Possible Theoretical Model

• 6 X greater in 1st than 3rd trimester
• RICH/NICH from localized area of low IFN
• Typical IH from sudden decrease in IFN without
  placental circulation

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Treatment

• Observation, observation, observation!!! (gt90
  require no treatment, without relation to size)
• Unless
• Equivocal diagnosis (rule out congenital
  fibrosarcoma and other malignancy)
• Ulceration, hemorrhage, visual obstruction
• AV shunting with CHF
• Kasabach-Merritt phenomenon
• Residual excess skin or telangiectasias after
  rapid involution
• Then excision /- steroids /- interferon

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Skin / Lid hemangiomas

• Usually capillary type
• Induces astigmatism
• May cause amblyopia
• May require intervention

Sterker I, Grafe G. Strabismus. 2004
Jun12(2)103-10.
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Choroidal hemangiomas

• Usually cavernous type
• Isolated
• Reddish orange
• Well-circumscribed
• Discovered during routine exam or secondary to
  induced hyperopia from tumor or serous detachment
• Not usually associated with Sturge-Weber syndrome

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• Diffuse
• Tomato catsup fundus
• May be dicovered during Dx/Tx of associated
  developmental glaucoma or amblyopia in children
• Associated with Sturge-Weber syndrome
• Encephalotrigeminal angiomatosis
• Ipsilateral facial nevus flammeus (port-wine
  stain) V1/2 (87)
• Sporadic inheritance
• Seizures (72-93)/ glaucoma (30-71) / choroidal
  hemangioma (40) / MR (50-75)

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Choroidal hemangioma

• Overlying cytic change alone to frank
  neurosensory detachment secondary to choroidal
  exudation and RPE dysfunction
• Hard exudates are not commonly seen
• Treatment
• No symptoms ? observation
• Serous detachment affecting fovea ? Laser
  photocoagulation, cryopexy, external beam and
  plaque radiation, or PDT

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