A novel approach to manage recurrent epistaxis in outpatients with hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is characterized by telangiectasic vascular malformations of the skin and of the digestive and respiratory mucosa. Epistaxis is the most common otorhinolaryngologic manifestation, with potential complications as septal perforations and, in cases of serious bleeding, anemia. Given that some therapeutic approaches are burdened by failure, whereas others are invasive and painful, the possibility of using simple and effective approaches to manage recurrent epistaxis, especially in outpatients, is needed and useful. Recently, the use of Surgiflo, (OMRIX biopharmaceuticals Ltd.MDA Blood Bank, Ramar-GanPOB, Kiryat Ono, Israel) a gelatin-thrombin matrix, currently used as an alternative to the nasal packing, has been proposed. We evaluated the effectiveness of Surgiflo in the treatment of recurrent epistaxis in outpatients with HHT. The present investigation reports the case of 3 patients with recurrent HHT-related epistaxis treated with Surgiflo. We also review the literature discussing available treatment options. Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a rare systemic fibrovascular dysplasia with autosomal-dominant inheritance. It is characterized by a spectrum of telangiectasic vascular malformations occurring in the skin and in the digestive and respiratory mucosa. Other manifestations of HHT occur in the lungs, liver, or central nervous system [1] and [2]. Otorhinolaryngologic manifestations are frequent, and epistaxis caused by spontaneous bleeding of nasal mucosa telangiectasis is the most common. The potential complications of epistaxis are septal perforations and, in cases of serious bleeding, anemia [3]. Treatment of HHT is aimed at controlling symptoms. For instance, it is possible to stop nose bleeding by applying direct pressure to the nose or performing anterior and/or posterior packing [4] and [5]. If these measures are insufficient and the frequency and duration of episodes impair the patient's quality of life, surgical cauterization, photocoagulation laser, or septal mucosal dermoplasty may be recommended [1] and [6]. To prevent the onset of chronic anemia resulting from severe epistaxis, the endovascular treatment with microembolization could be performed. Chemical cauterization should always be avoided because it may harm nasal structures [1]. Although brachytherapy and fibrin glue injection in the nasal septum and inferior turbinates submucosa are efficacious, they bring about only temporary symptom improvement [3]. Given that some therapeutic approaches are burdened by failure and some others are invasive and painful, the possibility of using simple and effective approaches to manage recurrent epistaxis, especially in hematology units and in outpatients, is needed and useful. Recently, the use of Surgiflo, a gelatin-thrombin matrix, currently used in neurosurgery and in thoracic and vascular surgery, has been proposed as an alternative to the nasal packing after functional endoscopic sinus surgery. In addition, some authors have evaluated the use of Surgiflo in the management of recurrent epistaxis [4] and [7]. Therefore, we evaluated the effectiveness of Surgiflo in the treatment of recurrent epistaxis in patients with HHT. The present investigation reports 3 cases of outpatients with recurrent HHT-related epistaxis treated with Surgiflo. We also review the literature discussing available treatment options. Surgiflo is a dehydrated gelatin-thrombin matrix in a syringe. For intranasal use, its gelatinous consistency must be maintained to remain in the nasal cavity (reconstitution with up to 3.5 to 4 mL of sterile water). A 16-cm-long applicator is put through the nasal vestibule to the rhinopharynx. The rhinopharynx and the nasal cavity are filled in with Surgiflo from back to front. A.V., female, 63 years old, had been complaining of mild recurrent epistaxis for 20 years, with worsening in the 6 months before treatment. She did not use salicylate-based drugs. The patient underwent cauterization of varices of the base of the tongue 7 years before and of the nasal septum 1 year before, with many anterior packings, followed by immediate epistaxis after each packing removal. The patient was transferred to ENT consultation in April 2011; nasal endoscopy showed crusts and hematic points spread throughout nasal septal mucosa. Blood test showed hemoglobin (Hb) levels of 10.50 g/dL and iron levels of 29 μg/dL. Surgiflo was applied inside both nasal cavities through nasal endoscopy without anesthesia, and the patient left the hospital after 2 hours of monitoring without recurrence of bleeding. No epistaxis has recurred until now. G.C., male, 49 years old with HHT, had been complaining of a right anterior epistaxis for 5 years. He underwent repeated right anterior packings that were effective, but bleeding recurrences were immediate after each packing removal. Nasal endoscopy showed hematic points throughout the right nasal cavity. Blood tests showed Hb levels of 10.90 g/dL and iron levels 33 μg/dL. In September 2011, he was treated with Surgiflo without anesthesia. The patient left the hospital after 2 hours of monitoring without bleeding. No epistaxis has recurred until now. G.E., male, 62 years old, was affected by HHT; he had been complaining of epistaxis for 7 years, treated by repeated anterior packings followed by immediate bleeding recurrences. During his physical examination at ENT Unit, we noticed hematic points spread throughout nasal septum. Blood tests showed Hb levels of 10.30 g/dL and iron levels 30 μg/dL. We applied Surgiflo in July 2012 inside his nasal mucosa through nasal endoscopy without anesthesia. The patient left the hospital after 2 hours of monitoring without bleeding. No epistaxis has recurred until now. Epistaxis is the earliest and the most common symptom of HHT. Ninety-five percent of individuals with HHT experience recurrent epistaxis, with a mean frequency of 18 episodes per month [1]. Although the nasal packing allows stopping most of bleedings, the disadvantages of local infections, septic complications, pain, mucosal traumatism, and bleeding recurrence still remain. An alternative to the traditional packing could be the use of absorbable packing such as Surgicel (ETHICON, LLC San Lorenzo, Puerto Rico) (oxidized cellulose polymer) or Gelfoam (Pharmacia and Upjohn Company, Kalamazoo, MI, USA) (absorbable gelatin sponge), whose placement is, anyhow, painful in outpatients and less effective for posterior bleeding [4]. For instance, some authors have evaluated the use of the gelatin-thrombin matrix after functional endoscopic sinus surgery with encouraging outcomes, reporting the prevention of bleeding after surgery in 96.7% of cases [7]. Furthermore, Buiret et al [4] report the cases of 2 patients, one with thrombocytopenia and the other with thrombopathy, hospitalized for recurrent epistaxis and, after repeated failed treatments, successfully treated with local application of Surgiflo. In the present study, we report 3 cases of patients with recurrent HHT-related epistaxis treated with Surgiflo. Our preliminary encouraging findings suggest that the use of local application of gelatin-thrombin matrix could be an effective alternative to the traditional anterior/posterior nasal packing, especially in outpatients at higher risk for bleeding, in which often the bleeding occurs again after the removal of nasal packing. Moreover, this technique is not invasive or potentially dangerous for outpatients. Surgiflo is effective, repeatable over time, and easy to apply, even without anesthesia. In addition, it could avoid the use of surgical procedures such as cauterization. In our opinion, Surgiflo can represent a valid tool for the management recurrent epistaxis in HHT, particularly in outpatients, because it is free of disadvantages related to the anterior packing or surgical procedures, effective, and reabsorbable. Furthermore, the cost of this device is widely offset by the reduction of hospitalization [4].