r.i.c.h. grand rounds october 2005 speaker: jay c. bradley, md discussant: michael j. shami, md...

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

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R.I.C.H.

Grand Rounds

October 2005

Speaker: Jay C. Bradley, MD

Discussant: Michael J. Shami, MD

Rapidly Involuting Congenital Hemangioma

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

Page 3: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

Choroidal hemangioma OS

Page 4: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

Hemangiomas also present on right upper lid and right hip

Page 5: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

Hemangioma resolution at 2 yr F/U

Page 6: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

Types of Congenital Hemangiomas

• Typical infantile hemangioma

• NICH (Non-involuting congenital hemangioma)

• RICH (Rapidly involuting congenital hemangioma)

Page 7: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

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)

Page 8: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

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

Page 9: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

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

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

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

Page 12: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

Typical IH

NICH RICH

Possible Theoretical Model

Hemangioma

VEGF

bFGF

Interferon (trophoblastic origin)

1. 6 X greater in 1st than 3rd trimester2. RICH/NICH from localized area of

low [IFN]3. Typical IH from sudden decrease in

[IFN] without placental circulation

Page 14: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

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

Page 15: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

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.

Page 16: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

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

http://www.nyee.edu/images/v118.jpg

Page 17: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

• 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%)

Page 18: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

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

Page 19: R.I.C.H. Grand Rounds October 2005 Speaker: Jay C. Bradley, MD Discussant: Michael J. Shami, MD Rapidly Involuting Congenital Hemangioma

Any Questions?

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