Monday, September 1, 2008

Early Surgical Intervention for Proliferating Hemagiomas of the Scalp -- An Article Review

First I want to repost this information on hemangiomas from last July. It was included in a post on Vascular Birthmarks.
 
Hemangiomas are classified as superficial when they appear on the surface of the skin ("strawberry marks") and cavernous when found deeper below the skin's surface. They can be slightly raised and bright red and sometimes aren't visible until a few days or weeks after a baby is born. Cavernous, deep hemangiomas may be bluish because they involve blood vessels in deeper layers of the skin. Hemangiomas grow rapidly during the first 6 months or so of life, but usually shrink back and disappear by the time a child is 5 to 9 years old. Some, particularly larger ones, may leave a scar as they regress that can be corrected by minor plastic surgery. Most are on the head or neck, although they can be anywhere on the body, and can cause complications if their location interferes with sight, feeding, breathing, or other body functions.

Approximately 30% of all hemangiomas are visible at birth. The remaining 70% become visible within one to four weeks after birth. They affect approximately one in 10 to 20 Caucasian children, with a 3:1 predilection for the female sex. The incidence in non-Caucasian infants is lower.
 
Hemangiomas are endothelial neoplasms. To date, there is no universally accepted model for the etiology of hemangiomas. There is general consensus on the importance of endothelial cells, but the source of the endothelial cells is speculative and there is no consensus on the possible mechanisms by which the hemangioma endothelium interacts with surrounding cells. There is a scientific debate on whether they are of mutational or placental origin or fit in a developmental field disruption. In the past hemangiomas were once thought to be due to maternal behavior or thoughts during pregnancy. The term vascular birthmark itself implies a connection between birth and a vascular lesion. The term strawberry nevus indicates that the mother's intake of red fruits (strawberries) was once thought to cause birthmarks. It is important for the family to remember that the cause of hemangiomas has not been determined, and neither parent should bear guilt over the occurrence or appearance of one of these birthmarks. Approximately 83% percent occur on the head and neck area. The remaining 17% appear throughout the rest of the body (both externally and internally).
 
At birth, a precursor lesion may be present as an erythematous patch or a telangiectasia. Within weeks the lesion expands rapidly. Hemangiomas can grow for up to 18 months and then begin a long slow regression known as involution. This involution can last from 3- 10 years. This dramatic/fast growth of a hemangioma may seem alarming, but usually no treatment is indicated. This is because this rapid growth phase is followed by a gradual involution (regression) in the following years. At the age of 5 years, 50 percent of the hemangiomas have involuted, and at the age of 9 years 90 percent have.
 
The ones that do need treatment are the ones that ulcerate, obstruct the airway, or cause visual deficits or cardiovascular symptoms. Current treatment may include intralesional or systemic corticosteroid treatment, systemic interferon treatment, local bleomycin treatment, and surgery. Early intervention has been shown to reduce the need for corrective surgery after "involution" has occurred; or to, at least, minimize extensive corrective surgeries in the future. Psycho-social scarring which occurs when a child has been forced to live with a facial deformity until "involution" has been completed can be avoided by early, aggressive intervention.
 
 
Now, I want to review the information in the first reference below (photo to the right from that article). It is basically a report of six cases and how they were treated. Their conclusions are:
 

Although patience may be virtuous for many hemangiomas, we feel that large hemangiomas of the scalp deserve special scrutiny.

Besides the commonly accepted reasons for surgical intervention (e.g., ulceration, hemodynamic instability, airway obstruction), large (and even not so large) hemangiomas of the scalp may invite a more aggressive surgical approach for the following reasons.

First, even if a hemangioma undergoes complete involution, the remaining fibrofatty skin is often atrophic. The dermal layer is extremely thin and devoid of normal skin appendages. This can lead to large alopecic areas and/or derangement of the natural hairline..........

Second, hemangiomas of the parietal scalp may impinge on the ear. Because of the well-known pliability of the neonatal ear, thought to be secondary to the effects of maternal estrogens, extrinsic deforming forces may result in a permanent deformity. Thus, there is an opportunity to both remove the deforming force and reshape the ear within an early postpartum window of opportunity. As demonstrated in cases 4 through 6, permanent ear deformity was avoided by early removal of the deforming hemangioma.

Finally and perhaps most importantly, the scalp of a newborn infant possesses significant elasticity because of a relative tissue excess and an inherent pliability resulting from the absence of a thick fibrous galeal layer. As the infant ages, this considerable redundancy of scalp tissue dissipates, which may limit the possibility of primary closure (with or without scalp rotation flaps) without the need for tissue expansion. It should be noted that in the case of neonates born prematurely, the optimal timing for surgical intervention needs to be considered individually, as the relative operative risk will vary with the degree of prematurity.

One reason for traditionally waiting (other than that most will regress) is the concern that excision of hemangiomas in infants can be associated with life-threatening bleeding. They offer three important technical points are emphasized to minimize this risk.


First, all excisions are performed only after infiltrating the lesion and surrounding tissues with a tumescent solution of dilute epinephrine.

Second, hemostatic polypropylene sutures may be placed around the hemangioma to limit inflow to the lesion.

Third, the plane of dissection is outside of the lesion in the avascular galeal layer. Although bleeding of any amount in an infant is potentially dangerous, application of these simple principles will reduce the chance of a potentially disastrous hemorrhage.

I would encourage pediatrician and family doctors to go ahead and make the referral to a plastic or ENT surgeon if the hemangioma is large and in the head/scalp region. The surgeon may choose to watch with you, but the patient will already be in the pipeline if something needs to be done early.

 

REFERENCES:

Early Surgical Intervention for Proliferating Hemangiomas of the Scalp: Indications and Outcomes; Plastic and Reconstructive Surgery:Volume 122(2)August 2008pp 457-462; Spector, Jason A. M.D.; Blei, Francine M.D.; Zide, Barry M. D.M.D., M.D.


Hemangiomas and Vascular Malformations in Infants and Children: A Classification Based on Endothelial Characteristics; The Pathogenesis of Hemangiomas: A Review; Plastic and Reconstructive Surgery:Volume 117(2)February 2006; Bauland, Constantijn G. M.D.; van Steensel, Maurice A. M. M.D., Ph.D.; Steijlen, Peter M. M.D., Ph.D.; Rieu, Paul N. M. A. M.D., Ph.D.; Spauwen, Paul H. M. M.D., Ph.D.

Progress to ward Understanding Vascular Malformations [Special Topic]; Plastic and Reconstructive Surgery:Volume 107(6)May 2001; Breugem, Corstiaan C. M.B.Ch.B.; van der Horst, Chantal M. A. M. M.D., Ph.D., and; Hennekam, Raoul C. M. M.D., Ph.D

Vascular Birthmarks: Hemangioma and Malformations. by Mulliken, John B. MD; Young, Anthony E. MD; Textbook--W.B. Saunder, Philadelphia, PA, 1988.

Hemangiomas and Vascular Malformations of the Head and Neck edited by Milton Waner, MD and James Suen, MD--Wiley-Liss, NY 1999

2 comments:

NeoNurseChic said...

This is a very interesting article. I have a personal curiosity about hemangiomas because I developed a rather unusual one at age 16. I first discovered a small, color-less (skin tone was unchanged) pimple-sized bump on the left side of my nose in October of 1996. By January, the lump was quite large - and while it looked like I had a small bump on the left side of my nose - it was actually not noticeable by most people. It had grown inwards also, blocking a good bit of my left nasal passage. The skin was completely unchanged in color and appearance over this lump. I wish I had a picture, but I'm really not sure if we took any pictures of my face before the surgery I ended up having.

The search for "what the bump was" was not an easy one. First family doctor told us it was an ingrown pimple and to put a hot compress on it. Next family doctor said it was a cyst and referred to dermatologist. Dermatologist said he didn't know what it was, but it was not a cyst, and he recommended to get it looked at by an ENT immediately as it had grown from pimple sized to golf-ball sized in 3 months. ENT initially felt that no matter what it was, it had to come out as it was blocking my airway. So I had surgery in February of 1997.

After I awoke from surgery, the first question I asked the ENT was, "Was it a cyst?" He said it wasn't, but he wasn't sure what it was at that point. He told my mom he was concerned about the consistency of the tumor. (He referred to it as a tumor...) He told us at the follow up visit that he had been concerned it was cancerous and he would have referred to Sloan-Kettering. He said that fortunately it was not. It was a lobular capillary hemangioma.

Fortunately ENT was certified in plastics, and the scar runs right up the left side of my nose in the crease - not a noticeable scar by any means. Of course, by 2005 I ended up having corrective sinus surgery to correct deviated septum (was not present at time of hemangioma) and remove scar tissue that had blocked the left nasal passage as a result of the 1st surgery. A month and a half later, I had a massive bleed and had further surgery for cauterization. ENT said blood was just oozing from everywhere, not one offending vessel. Finally, after a year, I felt back to normal.

I know my story isn't about a scalp hemangioma so doesn't exactly fit with the article, but I truly have always been interested in the topic of hemangiomas ever since then. Mine always seemed a bit unusual as it came on when I was 16 and had no effect on the skin's color or appearance, other than the bulge that formed on the left side of my nose. I did note occasional pulsating feeling in the lump, and to this day on very rare occasions I'll feel a pulsating where it used to be - usually just random out of the blue.

It was nice to read a review of hemangiomas and treatment practices!

Take care,
Carrie :)

Dreaming again said...

I had a friend whose baby was born with one. Her pediatrician said "ahh, just a strawberry! nothing to worry about" her mamma's instinct told her to watch.
It grew rapidly, and doc said "ahh, by the time she's two it will lighten and go away'
Her mamma's instinct said "I want a second opinion"
Doc said "no, if you don't trust me, find a new doc" (he'd been HER pediatrican as a child!!)
So, she broken heartidly found a new pediatrician.
Found one, alarmed he sent her immediately to specialist here in Tulsa who said it had already grown too severe for a Tulsa doc and sent her to an Expert in Little Rock.

I'm thinking Dr. Weiner?

She had to have a surgery or two and 3 or 4 laser treatments.

An early treatment, might have prevented all that.