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Diaper Area Hemangiomas: a unique set of concerns.

Karla Hall, Medical Research, Executive Director, Hemangioma Newsline
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. Current research shows that the aggressive use of pulsed dye, 585nm laser can selectively cause a thermal reaction in the vasculature resulting in photocoagulation and rapid resolution of the ulcerated lesion. There have also been reports of similar results using the Argon laser, 488 and 514nm wavelengths. However this laser is controversial because of the increased potential for scarring. (1)
The most common complication of superficial hemangioma is ulceration. Ulceration of hemangiomas occurs in up to 10% of all lesions during the growth period. As the hemangioma grows the skin can not keep up. The elasticity of the skin is reduced, it then splits and opens. This causes 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 not repair itself, healing may not occur for months. Ulcerated hemangiomas are a great risk for infection. Perianal and urogenital lesions are at an increased risk for infection because of urine and feces. Even the most careful diapering can't 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 babies can't be bathed, water alone causes severe pain. Untreated ulceration that becomes infected can lead to more serious cellulitus. Standard barrier methods of protection become ineffective and can irritate the child more. Using a semipermeable dressing over the wound and changing it frequently may ease pain. (Vigilon is one product used. )
In addition to the pain and the risk of infection associated with ulcerated "diaper" area hemangioma, all ulcerated hemangioma leaves residual scarring. Although in the diaper area the cosmetic concerns are not as critical, the destruction of normal sensitive nerve tissue as a result of scar tissue accumulation can not be ignored.
The treatment of diaper area hemangioma becomes essential to reduce the pain and risk of infection to the child. Standard treatments include, observation, steroid, laser, surgical excision and in extreme cases interferon. Since most diaper area hemangiomas are associated with ulceration, pain, bleeding and infection observation should be excluded. Early intervention of diaper area hemangioma may prevent any of the complications from occurring. Aggressive Laser treatment of the area is documented to be the most effective tool in the management of diaper area hemangiomas. Lasering the ulcerated area can improve the patients discomfort however this is related to the depth of the ulceration. The deeper the ulceration thre grater the tendency towards pain and the longer the healing period. Although some facilities use a gentle laser approach, it is this authors opinion; based on the medical literature that aggressive laser proves to be the most effective. The literature shows that pulsed dye laser treatment leads to a rapid decrease in pain and to initiation of the healing process. According to reports from Albany Medical Center, NY, Arkansas Children's Hospital, Buffalo Children's Hospital, Charlotte Plastic Surgery and Laser Center and University of Colorado School of Medicine, 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 best window of opportunity for eradication of the lesion is prior to 10-12 months of age. Reports show that 70% of ulcerated lesions heal entirely before 2 weeks following single treatment. It is the parent's view that pain was decreased almost immediately as noted by behavior changes in the infants. Sleep improved, cries decreased during bathes 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) It can be suggested that the pulsed dye laser is a safe and effective method of treatment of all ulcerated hemangioma. Primary care givers need to be informed of the benefits of early intervention with laser for hemangiomas of the diaper area, which are at high risk for ulceration and complication. Dr. Mitlon Waner has documented similar findings in a more comprehensive study. 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. The Prednisone class of drugs is 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 frighten most parents. The side effects of oral steroid use include gastric reflux, and stomach irritation. These symptoms can be eliminated with the use of prescription Zantac or Propulsid at 3-5mg/kg-body weight. 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 also reduces the risk of regrowth of the lesion 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 3-5mg/kgbody weight dose at this amount must continue for 3-4 weeks and then decrease slowly over 8-10 weeks. If regrowth begins the full dose can 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. In the treatment of diaper area hemangiomas early aggressive laser can eliminate the need for steroid use. Many parents would rather not treat with steroid because of the side effects. Evaluation by a physician experienced with hemangioma management or a multidisciplinary team is recommended. Some physicians prefer interlesional 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 is not the treatment of choice. Injections are extremely painful and require sedation. Injection does not reduce the side effects. A second pharmacological treatment of hemangioma is interferon. Alpha 2a interferon is an antiviral drug developed in the research of cancer treatments. It was discovered that it had antiangiogenic application. (Ability to shrink blood vessel tumors) This drug was promising until it was shown that infants on interferon experience a delay in motor development and in severe cases spastic dysplasia. These neurological complications are cause for concern. Many physicians are not using the drug in infants. Some are still cautiously using the drug only if steroid treatment fails. Other antiageigenic agents are being evaluated for use in the treatment of complicated or endangering hemangioma. If life threatening conditions such as congestive heart failure, airway obstruction, visual obstruction, thrombocytopenia( Kasabach Merritt syndrome) exist it would be prudent to contact one of the major treating facilities for treatment protocols. (6) Interferon is not usually a treatment for diaper area lesion. Surgical excision of the urogenital hemangioma or analgenital hemangioma is difficult because of the sensitive organ structures involved. If surgical excision is recommended be sure to determine why the other treatment options have been rule out first. General surgeons with limited experience in the treatment of vascular lesions should be avoided. Many have recommended extreme surgeries for superficial lesions. My own child was advised to have a colestomy for surgical excision by a local pediatric surgeon the hemangioma was later resolved by 2 outpatient laser procedures. Research any surgeon before considering excision of diaper area lesions. Ask to speak to other patients with similar lesions. Children with diaper area hemangiomas are in pain. Even the smallest lesion can ulcerate if it is near the rectum. The ulceration will 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 aggressive laser proves to be the best tool available for the treatment of this type of lesion. The statistics reported in the literature support early intervention and the clinic reports support aggressive application for maximum results and complete resolution.
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 methods that may help ease the pain until proper treatment by an experienced physician is received. Some of these methods are also used following laser surgery. 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 a good choice as well as generic zinc oxide cream. AquaPhor ointment is another excellent barrier against urine and feces. It is like petroleum jelly except is water-soluble so it will was away without wiping. Several doctors advise using it after laser surgery also. 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 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, never wipe 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. 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 3-year-old child who was born with a large perianal hemangioma and a second hemangioma on the labia. The lesion was treated "gently " with laser from 2 months of age until 5 months of age. These treatments demonstrated no response on the hemangioma. It 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 colestomy 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. The child's pain was gone that same day. For the first time she slept through the night. A second procedure ended their long ordeal. With the assistance of many of the physicians and researchers and parents she met along the way Karla started Hemangioma Newsline, an informational resource for families and physicians in the management of Hemangioma.
Endnotes
1. Achauer, Bruce M., VandeerKam, Victoria, Ulcerated anogenital hemangioma of infancy. Plastic and Reconstructive Surgery, Vol.87, No. 5, May 1991.
2. Morelli, Joseph, treatment of ulcerated hemangiomas in infancy. Arch Pediatric Adolesc. Medicine, vol. 148, 1994.
3. 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.
4. Ibid. Waner, Milton, 1994.
5. Sadan, Naum, Wolach, Baruch, Treatment of hemangiomas of infants with high doses of predsisone. The Journal of Pediatrics, Vol.128: 1, Jan. 1996.
6. Shannon, Linda, Marshall, Connie, Birthmarks: A Guide to hemangiomas and Vascular Malformations, Women's Health Publishing, Nevada, 1997.
Other References
7. Low, David, Hemangiomas and Vascular Malformations, Seminars in Pediatric Surgery, Vol. 3:2, May 1994.
8. Mulliken John B. Boon, L.M., Folkman, Judah, Pharmcologic therapy for endangering hemangiomas. Current Opinion in dermatology 1995:109-113.
9. Freeman, M. Sean, Hemangioma Treatment Protocol, submitted for publication 1997
10. Achauer, Bruce, VanderKam, V., Management of Hemangiomas of Infancy, Review of 245 Patients. Plastic and Reconstructive Surgery, April 1997 1301-1308.