Osler's disease, also referred to as haemorrhagic telangiectasis (HHT), is a rare hereditary condition in which the body's capillaries dilate to produce red skin spots and mucous membranes. Symtoms are expressed in the fingertips, face, lips, mouth, inside the nose and in the digestive tract. Breathing difficulties, bleeding in the lungs and, potentially, blood clots on the brain. The cause for the dilation in the capillaries is unknown; scientists believe that genes control the way in which the veins grow.

Those with this condition find that mild trauma can instigate the onset of nose bleeding that can be profuse in the absence of normal vasoconstriction. In fact, in more than 90 percent of these patients, a nosebleed is the most common presenting symptom. Over the years a multitude of different treatment options have been described for control of the epistaxis in patients with HHT including laser treatment, embolisation and the systemic use of estrogens which is controversial due to their systemic side effects.

Since 1997, German otolaryngologists have been successfully using Argon Plasma Coagulation (APC) in the treatment of patients with known HHT and bleeding telangiectasia of the nasal mucosa. The technology is based on a high frequency electrical current applied in a non-contact way through a plasma beam to the tissue surface. When compared to the C02 laser, the APC: (1) has a limited temperature of 100 degrees C (boiling water temperature), and does not cause damage to adjacent tissue; (2) does not require safety measures necessary for laser surgery; (3) uses flexible applicators available to reach the lower airway; (4) produces no bleeding lesions; and (5) allows no vaporization of tissue and danger of contamination with virus.

This research presents the first results of a large-scale study that utilized a combined treatment approach of APC and topical estriol, one of the three naturally occurring estrogens, in Olser's disease and recurrent nosebleeds. Estrogens are known to induce an epithelial metaplasia by changing the ciliary columnar epithelium into a keratinizing squamous epithelium. The authors of "Plasma Surgery and Estriol" are Haneen Sadick MD, Karl M. Hormann MD/Prof, and Wolfgang Bergler MD/Prof, all from the Department of Otolaryngology, Head and Neck Surgery, University Hospital Mannheim, Mannheim, Germany. Their findings are to be presented at the American Academy of Otolaryngology--Head and Neck Surgery Foundation Annual Meeting and OTO EXPO, September 22-25, 2002, at the San Diego Convention Center, San Diego, CA.

Methodology: Between 1997 and 2001, 79 patients with diagnosed HHT and recurrent epitaxis (nosebleeds) were treated with Argon Plasma Coagulation (APC) and postoperative topical estriol application. Forty-one patients were female and 48 patients male with a mean age of 57 years (57,3 +/- 12,7 years; range 6 to 81 years). Out of the 79 patients, 52 patients could be followed up over a mean period of 18 months. The most common symptoms were recurrent epistaxis in 99 percent (51 patients), telangiectasia in 97 percent (50 patients), a family history of the disorder in 95 percent (49 patients), and organic manifestation of the disorder other than the nose and the skin in 29 percent (15 patients). Out of these 15 patients, three patients had cerebral angiodysplasia (cAVM), six patients had a hepatic involvement, four patients had gastrointestinal bleeding, and two patients had pulmonary arteriovenous shunts (pAVM).

Of the subjects, 49 patients had already experienced different treatment approaches to control their recurrent nasal bleeding. Forty-three patients had undergone nasal packing, seven patients already had undergone septodermoplasty, 15 patients had received unsuccessful treatment with CO2 and Nd:YAG laser before referral, and four patients had undergone embolization. In five patients, a septal perforation following several treatment attempts with the laser was present.

Argon Plasma Coagulation (APC) was performed in all 52 patients by two surgeons. In 32 cases, the operation was performed under general anesthesia. After APC, the nasal cavities were filled with an ointment containing 0.1 percent estriol. The patients were instructed to apply the estriol ointment twice a day. Within the first two weeks, only in exceptional cases, crusts and fibrin layers which were attached to the nasal mucosa were removed mechanically.

To evaluate the long-term results of the combined treatment approach, pre- as well as 18 months postoperatively, the patients had to undergo rhinoscopical and endoscopical examination of their nasal cavity and were given a questionnaire concerning the bleeding frequency and intensity. Over a follow-up period of 18 months, blood samples were drawn from each patient for serum estriol level measurements by an enzyme-linked immunosorbent assay (ELISA). All estriol- blood samples as well as control-blood samples were fixed and analyzed twice, and the estriol serum concentrations were determined in pg/ml.

Results: Before treatment with APC and topical estriol, 51 patients (98 percent) claimed to have daily episodes of recurrent epistaxis. Only one patient (2 percent) had nosebleeds which occurred a few times a week. The bleeding intensity in 47 patients (90 percent) was equal to a "grade III" bleeding, making a bowl or similar utensil necessary to hold beneath the nose. After the combined treatment approach with APC and topical estriol, more than 94 percent of the patients noticed a significant reduction of the bleeding frequency as well as the bleeding intensity when compared to their preoperative findings. In one patient the bleeding frequency and intensity remained the same and could not be influenced essentially under APC and topical estriol treatment. All together, 98 percent of the patients claimed to have an improved quality of life. From 52 patients, 50 patients were willing to undergo the same operation again. Only two patients were dissatisfied with the treatment, stating a discomfort during local anesthesia. However, these two patients were willing to undergo the same procedure again, but under general anesthesia. The serum estriol levels under long-term application of topical estriol did not increase significantly when compared to the preoperative findings.

Conclusions: The results from the present study showed that 18 months after APC therapy and topical estriol application, the patients were satisfied with the treatment result and were willing to undergo the same treatment again. More than 94 percent of the patients claimed a significant reduction in the bleeding frequency as well as bleeding intensity. This was verified by rhinoscopic and endoscopic examination of the nose. Former berry-like telangiectasia of the nose were covered by a thickened epithelial lining which made them appear more flat and even to the tissue surface, thus making them less vulnerable to local traumas.

In none of the patients did serum estriol levels increased significantly after long-term topical estriol application. No systemic side effects were noticed in the study patients. Researchers regard the topical estriol application on the nasal mucosa as a promising adjuvant treatment modality to APC. Whereas APC is used to control the acute state of bleeding from telangiectasia of the nasal mucosa, topical estriol ointment served as a protecting film on the nasal mucosa by re-establishing the nasal mucosa with thick layers of squamous epithelium, thus making the treated telangiectasia less vulnerable to local trauma

Log on to http://www.entnet.org/healthinfo/nose/nosebleeds.cfm for more information about diagnosis and treatment of this disorder.

MEDIA CONTACT
Register for reporter access to contact details
CITATIONS

American Academy of Otolaryngology--Head and Neck Surgery Foundation Annual Meeting and OTO EXPO