On September 11, 1994 Bob & Karla Hall had their second child.  She was born with an infantile hemangioma located in the inter-gluteal crease.   The lesion ulcerated, became infected and casued extreme pain for their child.  After months of searching the Halls located a physician that was able to treat the child and end the over 18 months of pain and suffering.  It is because of this experience that the Halls joined forces with the DuBiels and formed NOVA.  Simply put they wanted to help others affected by hemangioma and vascular malformation.

Hemangioma is the most common benign tumor of infancy and childhood. It has been reported that 10% of all children are diagnosed with a benign vascular tumor during the first year of life. 70% of hemangiomas occur on the head and neck. The remaining 30% can occur anywhere on the body either internal or externally. One of the most complicated areas a hemangioma can develop in is the urogenital area or anogenital area. These lesions commonly called diaper area hemangiomas are associated with pain, bleeding, recurring infection and ulceration. Traditional treatments included wound care, cold compresses of Burrows solution, antibiotic ointments and zinc oxide base creams. These treatments provide simple lesions with some protection from urine and feces but provide little or no improvement for the ulcerated urogenital or anogenital lesion. The medical research reports that the use of pulsed dye, 585nm laser can selectively cause photocoagulation and rapid resolution of the ulcerated lesion. There are several manufacturers of this type of laser and  new models have high tech cooling devices that can prevent burning of the skin which was one of the most common complications in the 1990's. (1)

The most common complication of superficial hemangioma is ulceration. Ulceration of hemangiomas occurs in up to 10% of all lesions during the initial growth period. The exact mechanism of ulceration is not understood, however the normal skin in the area of the hemangioma has decreased elasticity.  It is thought by some that as a hemangioma proliferates (grows) the skin does not stretch enough, it then splits and opens. This results in the wound we call ulceration. Because the skin over a hemangioma is compromised it is not unusual for it to split after just a slight bump. Since the hemangioma is growing faster then the skin, the skin can't repair itself, healing may not occur for months. Ulcerated hemangiomas are a risk for infection.

The most common areas for ulceration to occur are the high friction areas of the "diaper area" and the lip.  However; ulceration may occur on any hemangioma.

Perianal and urogenital lesions are at an increased risk for infection because of urine and feces. Even the most careful diapering can't always prevent infection. Once ulcerated all hemangiomas become painful. In the presence of a wet or soiled diaper this pain can be exasperating. Cleaning the area becomes more difficult and thus the risk of infection becomes greater. Some infants can't be bathed; water alone causes severe pain. Untreated ulceration that becomes infected can lead to more serious condition called cellulitus. Standard barrier methods of protection become ineffective and can irritate the child more. Using a semi-permeable dressing over the wound and changing it frequently may ease pain. (Vigilon is one product that is used.)

Typical ulceration in diaper area

In addition to the pain and the risk of infection associated with ulcerated “diaper” area hemangioma,  is the residual scaring that is left in its wake.  All ulcerated hemangioma will leave a scar.  Although in the diaper area the cosmetic concerns of ulceration are not as critical as for ulceration in the face, the destruction of normal sensitive nerve tissue that may occur as a result of scar tissue accumulation should be considered in the decision to treat.

The treatment of diaper area hemangioma becomes essential to reduce the pain and risk of infection to the child. Standard treatments for hemangioma include, observation, steroid, laser, surgical excision and other medications including vincristine.  Since most diaper area hemangiomas are associated with ulceration, pain, bleeding and infection observation is often not an option.

Early intervention of diaper area hemangioma may prevent any of the complications from occurring. Laser treatment of the area is documented to be an effective tool in the management of diaper area hemangiomas. Laser treatment of the ulcerated area can improve the patients discomfort; however relief is related to the depth of the ulceration. The deeper the ulceration, the grater the tendency towards pain and the longer the healing period. The literature shows that pulsed dye laser treatment may lead to a rapid decrease in pain and to initiation of the healing process.

According to interviews with physicians and a review of the medical literature, infants treated with pulsed dye laser from 1 week of age until 18 months saw rapid improvement and healing with one to three treatments. Laser energy used is 6.0Jouls –6.5 Joules/cm2. Lesions larger then 25cm2 may require 3-4 treatments. Healing of the painful ulcerations does not always mean total eradication of the lesion. The optimal results and eradication of the lesion occurred in infants treated with laser prior to 10-12 months of age.

Reports in the medical literature document that 70% of ulcerated lesions heal entirely before 2 weeks following single treatment. Interviews with parents reveal that pain was decreased almost immediately as noted by behavior changes in the infants. Sleep improved, cries decreased during baths and diaper changes, infants became less irritable. In some cases where severe ulcerations are noted the addition of steroids to the treatment is necessary. Use of steroids depends on the patient's age and the rate of growth of the lesion.

In a report by Dr. Joseph Morelli, 76% of the infants studied with ulceration occurred in the diaper area and all ulcerations occurred before 10 months of age. (2) Many experienced physicians suggest that the pulsed dye laser is a safe and effective method of treatment of all ulcerated hemangioma. Primary care providers should consider the benefits of early intervention with laser for hemangiomas of the diaper area, hemangioma that are at high risk for ulceration and complication.   A comprehensive study of the benefits of laser was published by Dr. Milton Wane.  He notes that complete resolution and a return of normal skin with no textural changes and residual scarring when treated before the end of the first year of life. (3)

Proliferating hemangiomas respond well to oral steroid treatment. Gluco-cortico steroids including Prednisone may be used to slow the growth of proliferating hemangiomas. Brand names include, prelone, and deltasone. Corticosteroids are natural hormones produced by the adrenal glands. They have potent anti-inflammatory properties. Recent studies confirm that 30% of patients respond to doses of Prednisone of 2-3mg/kg-body weight. (4) An Israel study of over 20 years reported in 1996 that doses of 5mg/kg body weight showed a rapid improvement over the lower dose without increasing the side effects. (5) However steroid treatment is only useful during the proliferative phase in most cases.

Oral steroids do have side effects that require careful monitoring by an experienced physician. The side effects of oral steroid use include gastric reflux, and stomach irritation. These symptoms can be eliminated with the use of prescription medications like Zantac Increased irritability, increased susceptibility to infection, and impairment of the natural immune response to infection are other complications of steroid use. These effects can result in a delay of normal vaccines for infants. Children may become “cushnoid” in that they can become “chubby” and appear round in the face. In rare cases they may have a growth in body hair. Prolonged steroid use can depress the ability of the body's adrenal glands to produce natural corticosteroid. Abruptly stopping oral steroids can lead to corticosteroid insufficiency and immune system complications. Withdrawal of steroid should be accomplished by gradual tapering. The tapering may also reduce the risk of re-growth of the lesion commonly called rebound growth.

The key to the success of steroid therapy with minimal side effects is to give the appropriate does to achieve maximum results. Treatment must continue during the growth of the lesion or until the 7-8 month of age when growth naturally slows. Once it is decided which dose is optimum for the infant, usually between 2-5mg/kg body weight dose at this amount must continue for 3-4 weeks and then decrease slowly over 8-10 weeks. If re growth begins the full dose may be resumed for 2 weeks and the decrease restarted. In some cases this process is repeated until the lesion no long grows. Children on steroids must be monitored weekly. Weight gain must be recorded as well as blood pressure and urine checks.

In the treatment of diaper area hemangiomas early laser may eliminate the need for steroid use. Many parents prefer to not treat with steroid because of the side effects. Evaluation by a physician experienced with hemangioma management or a multidisciplinary team is recommended when making treatment decisions.

Some physicians prefer intra-lesional injection of steroid to the oral use. This technique works well in localized and small lesions. Recent reports show no advantage of injection over oral use. Injection in the diaper area does not seem to be the treatment of choice. Injections are painful and may require sedation or topical anesthetic creams. Steroid injection does not reduce the side effects compared to oral steroids.

Doctors do have other drugs in their arsenal to treat complicated hemangioma.  These drugs have serious side effects and all the risks vs. benefits must be considered.  Only a physician with experience in treating vascular anomalies can outline the best treatment option for a patent.  Alpha 2a interferon is an antiviral drug developed in the research of cancer treatments. Interferon was used to treat complicated hemangiomas that did not respond to steroid management. This drug was promising for the management of hemangioma until it was shown that infants on interferon experience a delay in motor development and in severe cases spastic dysplasia. Today it is only recommend for use in the treatment of Hemangioma in life cases where the child's life is endangered. The drug vincristine came on the front lines of hemangioma management in 2002. This antiangiogenic drug is used for life threatening hemangioma, KHE lesions and those that do not respond to steroids.  This drug is used for patients that do not respond to oral steroids.  Life threatening complications of hemangioma included congestive heart failure, airway obstruction, visual obstruction, thrombocytopenia( Kasabach Merritt syndrome associated with tufted angioma and KHE anomaly) There are several Vascular Anomaly Centers with multidisciplinary teams located throughout the United States. These centers can advise and treat in accordance with acceptable standards and protocols.

Surgical excision of the diaper area hemangioma is difficult because of the sensitive organ structures involved. If surgical excision is recommended parents should ask why the other treatment options have not been considered first. Parents should consult Vascular Anomaly Centers and or physicians with experienced in the management of hemangioma. Often surgery can be avoided by using oral steroids and or laser. It is always a good idea to get 2-3 medical opinions before deciding on any surgery.

Children with ulcerated diaper area hemangiomas are in pain. Even the smallest lesion can ulcerate if it is near the rectum. The ulceration may continue to tear with each bowel movement. These lesions can grow into the vagina or rectum. They can obstruct urinary flow, or normal bowel movement. Untreated an older toddler may hold his daily bowel movements for fear of the pain and this can result in severe constipation and further digestive complications. Early intervention with laser often proves to be the best tool available for the treatment of this type of lesion.

NOTE:  Hemangioma located in the intergluteal crease or other midline regions require evaluation for spinal concerns.  It is advised by many physicians that a MRI/MRA be performed to rule out serious complications.  Some complications include Tethered Spine and fistula.  For more information about these complications please contact NOVA at admin@mail.novanews.org

Homeopathic suggestions to ease the pain of ulcerated hemangioma.

Ulcerated Hemangiomas are always associated with pain. Over the years, when medical treatment was not available mothers and physicians developed ways to ease the pain. Here is a list of suggestions that may help ease the pain until evaluation by an experienced physician is received. Some of these methods are also used following laser surgery. As always check with your physician prior to using any of these suggestions.

Zinc Oxide creams provide a barrier to urine and feces as well as keep the area moist. Some physicians express concern for infection while using these products. Read the labels, some products contain irritating ingredients. Stay away from fragrances, Desitin seems to be used by many parents as well as generic zinc oxide cream.

Aqua-Phor ointment is another excellent barrier against urine and feces. It is like petroleum jelly except is water-soluble making removing easier. Several doctors advise using it after laser surgery to provide a barrier.

Second Skin burn pads . These products draw the heat away from the skin into a moist gel pad, which does not stick to the skin. It can be used after laser to draw the heat away.

• Bathing is a concern for ulcerated diaper area hemangioma. Sea Salt Baths seem to soothe the child while normal water baths can cause pain. Salt-water baths should be very dilute, like contact saline without the preservatives. Sea Salt can be purchased at health food stores read the directions for dilution.
• Air drying the area where possible is best, avoid wiping with a towel to dry.
• Squirt bottles filled with water or saline solution instead of wipes or wet cloths to clean the area is best. It is better to leave a small amount of soil behind and get it in the bath later then to wipe the skin.

NOTE: Be careful not to overuse topical antibiotic creams without consulting your doctor, these can cause irritation and make things worse.

Karla Hall is the mother of a 13-year-old child who was born with a large perianal hemangioma and a second hemangioma on the labia.  The hemangiomas ulcerated and caused extreme pain for the child. Simple diaper changes and baths became prolonged painful and tearful events. Local surgeons suggested surgical excision involving a colostomy but most said to just leave it alone it would go away. The Halls began a national search for a physician, which led them to California , Atlanta , Philadelphia , Denver , Boston , NY , and Arkansas , and eventually in their own backyard of Charlotte NC. At 18 months of age the child was treated with the pulsed dye laser and the flash lamp laser by Dr. M. Sean Freeman. The child's pain ended that same day. For the first time she slept through the night. A second procedure ended their long ordeal. NOVA was founded as a result of Karla's passion to help other families with hemangioma.   Karla Hall and Lillian Dubiel published the first edition of Hemangioma Newsline in 1996 in a attempt to reach other parents of children with hemangioma.  Together they founded NOVA and have worked tirelessly for over 10 years. 

The above article was first published in Hemangioma Newsline in 1996.Revised 2002, 2007.

Special Thanks to all the parents and physicians that contributed to this article.

Endnotes

•  Achauer, Bruce M., VandeerKam , Victoria , Ulcerated anogenital hemangioma of infancy. Plastic and Reconstructive Surgery, Vol.87, No. 5, May 1991.

•  Morelli, Joseph, treatment of ulcerated hemangiomas in infancy. Arch Pediatric Adolesc. Medicine, vol. 148, 1994.

•  Waner, Milton, Suen, James Yee, Dinehart, S., Mallory, S.B., Laser photocoagulation of superficial proliferating hemangiomas. Journal of Dermatological Surgical Oncology, 20:43-46, 1994.

•  Ibid. Waner, Milton , 1994.

•  Sadan, Naum, Wolach, Baruch, Treatment of hemangiomas of infants with high doses of predsisone. The Journal of Pediatrics, Vol.128: 1, Jan. 1996.

•  Shannon, Linda, Marshall, Connie, Birthmarks: A Guide to hemangiomas and Vascular Malformations , Women's Health Publishing, Nevada , 1997.

Other References

•  Low, David, Hemangiomas and Vascular Malformations, Seminars in Pediatric Surgery, Vol. 3:2, May 1994.

•  Mulliken John B. Boon, L.M., Folkman , Judah , Pharmcologic therapy for endangering hemangiomas. Current Opinion in dermatology 1995:109-113.

•  Freeman, M. Sean, Hemangioma Treatment Protocol, submitted for publication 1997

•  Achauer, Bruce, VanderKam, V., Management of Hemangiomas of Infancy, Review of 245 Patients. Plastic and Reconstructive Surgery, April 1997 1301-1308.

•  Waner, Milton MD, Suen James Y MD, Hemangiomas and Vascular Malformations of the Head and Neck 1999 Wiley LISS Inc. New York

NOVA PROVIDES THIS INFORMATION TO FAMILIES AS A RESOURCE. IT IS NOT INTENDED TO ENGAGE IN THE PRACTICE OF MEDICINE OR TO REPLACE THE PHYSICIAN. NOVA DOES NOT CLAIM TO HAVE MEDICAL KNOWLEDGE. NOVA DOES NOT ENDORSE ANY PARTICULAR PHYSICIAN, TREATING FACILITY OR TREATMENT PROTOCAL. IN ALL CASES NOVA AND ITS BOARD OF DIRECTORS RECOMMENDS THAT YOU SEEK THE OPINION OF A PHYSICIAN EXPERIENCED IN THE MANAGEMENT OF HEMANGIOMAS AND VASCULAR MALFORMATIONS. 

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