The Quarterly Consultis a quarterly supplement to the Bulletin highlighting pediatric clinical expertise. If you have an item of interest to include in the Quarterly Consult, contact Dr. Steve Dassel at 206-625-7373, mailbox 8588.

This edition of Consult is a discussion of vascular anomalies with Dr. Jonathan Perkins. Jonathan is an associate professor of otolaryngology at the University of Washington and the chief of the Vascular Anomalies Clinic at Seattle Children's Hospital.

Q: Jonathan, how are vascular anomalies classified?

A: They are classified as either vascular tumors or vascular malformations. Tumors such as hemangiomas and kaposiform hemangioendotheliomas have neoplastic properties.

Vascular malformations are disorganized clusters of vessels and can be arterial, venous or lymphatic.

Q: What is the importance of this distinction?

A: Medication is not curative for vascular malformations that need either surgery or interventional radiology procedures for treatment.

However, medical therapy for vascular malformations that are lymphatic, venous or arterial is frequently necessary to manage symptoms.

On the other hand, vascular tumors, with their rapid growth potential, can compromise function such as vision or breathing and can also ulcerate. Medical therapy can decrease and stop vascular tumor growth, which can prevent functional compromise and allow ulcers to heal.

Vascular tumors are also associated with a Kasabach-Merritt phenomenon with thrombocytopenia, which requires medical therapy.

Q: So, these contrasting properties explain the different characteristics of a vascular malformation such as a port wine stain and a vascular tumor such as a hemangioma?

A: That's right.

Q: How have approaches to vascular anomalies changed? What is new?

A: We have a much better understanding of the biology and natural history of vascular anomalies. With more experience, we have more confidence in treating them.

For example, the surgical treatment of an airway hemangioma is much less likely to involve tracheotomy now, than, say, 20 years ago. With more surgical experience, we can be more aggressive, while avoiding significant bleeding or making matters worse in any way.

We are also more comfortable with medical, rather than surgical, management that can improve the course of some of these lesions.

Q: What are some examples of drugs in your armamentarium?

A: We have used both injectable and parenteral steroids for years to treat rapidly enlarging vascular anomalies. We are becoming more familiar with the antiangiogenic drugs, such as interferon and vincristine.

Antibiotics are sometimes used to prevent repeated swelling. Nonsteroidal anti-inflammatory medications can be necessary in situations where chronic pain is present.

Q: How may vascular anomalies present?

A: Classically, a hemangioma makes its appearance shortly after birth and grows at a faster rate than the patient. Vascular malformations are usually present at birth and grow with the patient.

However, a venous or arteriovenous malformation may undergo rapid growth at the time of hormonal change of puberty and present as pain due to nerve compression.

Lymphatic malformations may rapidly enlarge with associated viral infection or local trauma. Vascular anomalies may present as a mass or as a cutaneous lesion, or may present as symptoms of compromised function, such as stridor or amblyopia.

They may also present with bleeding, such as GI bleeding from an intestinal vascular anomaly. Occasionally, unexplained infections may be due to an unidentified immune deficiency associated with a vascular anomaly.

Q: When should we refer?

A: We think all unexplained vascular masses should be evaluated. Evaluation allows distinction between types of vascular anomalies and determination of appropriate treatment when indicated.

Q: What are the vascular anomalies that warrant urgent referrals?

A: Urgent appointments are reserved for hemangiomas on a nasal tip, ear and lip, and other vascular tumors that become ulcerated, bleed, are painful, have massive swelling, or are located on the eyelid or in and around the airway.

Commonly, hemangiomas are located in the head, neck, genital or axillary regions of the body. Genital hemangiomas are "semiurgent" in that they typically ulcerate.

Complicated hemangiomas and other vascular tumors (greater than one centimeter in diameter) should be seen within two to three weeks in either the Vascular Anomalies Clinic or the Dermatology Clinic, depending upon the location of the anomaly.

You can refer a new patient by fax, 206-985-3121, or by phone, 206-987-2080.

Q: What about your garden-variety hemangioma?

A: Uncomplicated vascular tumors such as hemangioma are not urgent referrals. By uncomplicated I mean less than one centimeter in diameter.

They can be referred to the Vascular Anomalies Clinic or the Dermatology Clinic for the next available appointment.

Q: What if we are just not sure?

A: When it is unclear how a patient should be scheduled, the coordinator involved with the vascular anomalies program can be contacted directly.

Urgent consultations can be directed to Maureen Lofgren, RN, the dermatology resident or the pediatric otolaryngology fellow. Or you can call the physician operator at 206-987-7777 to speak with the on-call provider.

Q: Do you mind getting referrals when the primary care provider is comfortable in managing the lesion but the parent is apprehensive?

A: Absolutely not. We often see patients in that situation. The primary care provider correctly says that this can just be followed. The patient goes home with a diagnosis and a name, accesses the Internet and comes away with all sorts of questions, and contacts us.

Half of new patients to Vascular Anomalies Clinic are parent (self) referrals. So, parent curiosity is definitely an indication for referral.

Q: There are not enough physicians to see every hemangioma out there.

A: I agree. Use your judgment with your referrals, but we are available.

Q: What are some examples of "benign" vascular anomalies that may have a deeper significance?

A: Superficial capillary malformations may have an underlying arteriovenous malformation. Varicosities in the leg or arm are very uncommon in children and may signify Klippel-Trenaunay syndrome.

Large hemangiomas of the head and neck overlying the mandible ("beard" distribution) associated with stridor suggest an airway component of that lesion. Large hemangiomas of the head and neck can be associated with PHACES syndrome.

Multiple hemangiomas can be associated with internal hemangiomas and hypothyroidism. Bluish cutaneous venous malformations and GI bleeding can be associated with Blue Rubber Bleb Nevus syndrome.

Q: What about hemangiomas around the eye?

A: It has been known for some time that rapid enlargement of eyelid hemangiomas may obscure vision and result in deprivation amblyopia.

In addition, simple compression of the eyeball by an enlarging hemangioma can distort the eyeball shape, changing the eye's focal length, resulting in astigmatic amblyopia. Of most concern is when the hemangioma is on the upper medial eyelid, or lower eyelid above the eyelid crease.

Q: Anything else?

A: As previously mentioned, hemangiomas can be part of the PHACES syndrome. The other parts of the association are posterior fossa lesions, arterial anomalies, coarctation of the aorta or cardiac defects, eye problems and a sternal cleft.

Q: Finally, Jonathan, what mistakes do you see primary care physicians making in our approach to vascular malformations in children?

A: As I mentioned before, 50% of patients new to the Vascular Anomalies Clinic come without a physician referral. So, it's important to recognize parents' concerns or just curiosity, when they want a referral to a vascular anomalies clinic.

Often parental ideas about vascular anomaly treatment differ from that of physicians, and open discussion about these ideas usually helps patient care. Another problem is failure to recognize hemangioma ulceration and its associated pain and bleeding potential.

Early treatment of some ulcerated hemangiomas, such as genital hemangiomas, can make a big difference in patient well-being. Over the years, vascular tumors have all been labeled hemangiomas.

Advances in our understanding of the natural history and associated problems (i.e. bony and soft tissue overgrowth, pain, infection, swelling) of these growths have resulted in improved therapy.

Often early intervention can reduce vascular anomaly growth and the need for subsequent treatment interventions.

Q: Regarding the waiting and watching of hemangiomas, I was taught that many will regress, reaching their smallest point around 4 years of age or so, and that was the best time to refer for surgical excision because the scar would be smallest.

A: It is true that the 50% of hemangiomas appearing around the time of birth will regress markedly and we may not do anything about them until 4 years of age, but with increasing access to medical information and a resultant heightening of anxiety, parents are less willing to sit back and wait for four years.

Though controversial, there is evidence that children become aware of their appearance by 3 years of age.

For this reason, intervention to reduce hemangioma redness (laser therapy) or size (medical and/or surgical therapy) can be done prior to age 3.

Q: This has been very informative, Jonathan. Thank you.