1. R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

2. 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

3. Choroidal hemangioma OS

4. Hemangiomas also present on right upper lid and right hip

5. Hemangioma resolution at 2 yr F/U

6. Types of Congenital Hemangiomas Typical infantile hemangioma NICH (Non-involuting congenital hemangioma) RICH (Rapidly involuting congenital hemangioma)

7. Typical Infantile Hemangiomas 1-2 % of neonates Up to 12 % of Caucasian infants by 1 year Female > Males (3-5:1) Up to 23 % of premature infants Manifest postnatally (median of 2 weeks)

8. Typical Infantile Hemangiomas Grows rapidly during 1 st year of life Involutes slowly from 1 – 7 years Completely regresses by 8 –12 years Glucose transporter-1 protein present

9. 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

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

11. 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

12. Typical IH NICH RICH

13. Possible Theoretical Model Hemangioma VEGF bFGF + + Interferon (trophoblastic origin) - 1.6 X greater in 1 st than 3 rd trimester 2.RICH/NICH from localized area of low [IFN] 3.Typical IH from sudden decrease in [IFN] without placental circulation

14. Treatment Observation, observation, observation!!! (>90 % 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

15. Skin / Lid hemangiomas Usually capillary type Induces astigmatism May cause amblyopia May require intervention Sterker I, Grafe G. Strabismus. 2004 Jun;12(2):103-10.

16. 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

17. 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%)

18. 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

19. Any Questions? BEAT OU !!