Abstract
Background
Epistaxis is the most common symptom in patients with hereditary hemorrhagic telangiectasia (HHT). In severely affected cases, cessation of the nasal airflow seems to be the only long-term effective treatment. Such procedure deeply affects patient’s quality of life (QoL) and is sometimes refused.
Methods
This study investigated the effectiveness of a tailored silicone nasal swab on 2 patients affected by HHT and severe epistaxis.
Results
In both cases, we observed a good clinical effect, with significant reduction of epistaxis and improved QoL.
Conclusions
Our preliminary results indicate that the silicone nasal swab can be considered an effective way of treating severe epistaxis in patients with HHT. Its advantages, in terms of maintained airflow patency, reversibility, and improved QoL, are highlighted.
1
Introduction
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that is characterized by multiple vascular hemorrhagic sites placed anywhere in the body. Depending on the site of the angiodysplasia, the disease can be really problematic and sometimes life threatening. From a clinical point of view, one of the most typical manifestation of HHT is nose bleeding or epistaxis .
The degree of epistaxis is variable. Many patients have sporadic nose bleeding, without a significant change of the hemoglobin (Hb) value, whereas a few patients have daily and severe epistaxis, and multiple transfusions are needed. About one fifth of the patients presents an important epistaxis with a tremendous impact on their quality of life (QoL) . Obviously, management of epistaxis is different in relation to the degree of blood loss and to the frequency of the manifestations. From a therapeutic point of view, laser techniques have been proposed in mild to moderate cases ; but unfortunately, results are transitory. Furthermore, in severe cases, laser has been reported to be of limited value . In severe and rebellious cases, the closure of the nostrils seems to be the most effective solution in terms of controlling the epistaxis with stability in time . Unfortunately, not every patient is compliant with this proposal. Taking this into consideration, we started closing the nostrils in a simple and reversible way by applying a silicone nasal swab tailored to the patient’s anatomy. In this article, we present our preliminary experience with 2 patients in the management of severe cases of epistaxis refractory to every conservative treatment. This article was approved by the Ethical Committee of the University of Pisa.
2
Illustrative case 1
A 35-year-old woman, diagnosed at age 25 years with cutaneous, pulmonary, and nasal HHT, was referred to our center after 9 years of severe epistaxis. The patient had undergone different laser applications at another institution with a great experience in this technique, with only temporary control of the epistaxis. Furthermore, during the years, she was admitted several times, in different hospitals, for epistaxis and underwent nasal packing. She also learned to pack her own nose and only in really severe cases did she go to the hospital. During the years, the clinical situation worsened; and she was transfused many times (>10), in some critical periods almost daily. At the time of her admission at the hospital, the endoscopic appearance of the nasal fossae was typical; and her laboratory tests showed a severe iron-deficient anemia with Hb value at around 8 g/dL. The Short Form (SF)–36 questionnaire showed significantly lowered values in all domains. Based on this situation, the patient underwent to a superselective embolization of both the maxillary arteries in the Neuroradiological Unit of our hospital. Notwithstanding the benefits of this therapy, they were only temporary; and the epistaxis reappeared briefly after the embolization. Based on the failure of each conservative treatment, we proposed the closure of the nostrils; but the patient refused. So we then proposed to close the nostril by means of a silicone swab, and this was accepted by the patient. A customized double halved silicon nose swab was realized and tailored to the nostrils of the patient ( Fig. 1 A). The patient wore the silicon swab day and night, without removing it, until the number and severity of epistaxis happened to decrease. More specifically, we applied the nasal swabs in April 2007 ( Fig. 1 B) for 6 months, when the patient had no more episodes of epistaxis. Her main Hb value increased to 9.5 g/dL; the SF-36 questionnaire showed an increment of the QoL. At the time of removal of the nasal obturator, no mucosal damage was observed related to it. In November 2007, the patient presented 2 episodes of epistaxis, one mild and the other severe. After the first episode, the patient wore again the silicone swab; and during the subsequent episode of severe epistaxis, we managed to easily remove the nasal swab and to pack the nasal fossae in a standard fashion. After these 2 episodes, no more epistaxis was observed. Unfortunately, the patient presented a severe right heart failure due to the presence of a severe arterovenous malformation; and she was in the heart-lung transplantation program. She eventually died from the disease.
2
Illustrative case 1
A 35-year-old woman, diagnosed at age 25 years with cutaneous, pulmonary, and nasal HHT, was referred to our center after 9 years of severe epistaxis. The patient had undergone different laser applications at another institution with a great experience in this technique, with only temporary control of the epistaxis. Furthermore, during the years, she was admitted several times, in different hospitals, for epistaxis and underwent nasal packing. She also learned to pack her own nose and only in really severe cases did she go to the hospital. During the years, the clinical situation worsened; and she was transfused many times (>10), in some critical periods almost daily. At the time of her admission at the hospital, the endoscopic appearance of the nasal fossae was typical; and her laboratory tests showed a severe iron-deficient anemia with Hb value at around 8 g/dL. The Short Form (SF)–36 questionnaire showed significantly lowered values in all domains. Based on this situation, the patient underwent to a superselective embolization of both the maxillary arteries in the Neuroradiological Unit of our hospital. Notwithstanding the benefits of this therapy, they were only temporary; and the epistaxis reappeared briefly after the embolization. Based on the failure of each conservative treatment, we proposed the closure of the nostrils; but the patient refused. So we then proposed to close the nostril by means of a silicone swab, and this was accepted by the patient. A customized double halved silicon nose swab was realized and tailored to the nostrils of the patient ( Fig. 1 A). The patient wore the silicon swab day and night, without removing it, until the number and severity of epistaxis happened to decrease. More specifically, we applied the nasal swabs in April 2007 ( Fig. 1 B) for 6 months, when the patient had no more episodes of epistaxis. Her main Hb value increased to 9.5 g/dL; the SF-36 questionnaire showed an increment of the QoL. At the time of removal of the nasal obturator, no mucosal damage was observed related to it. In November 2007, the patient presented 2 episodes of epistaxis, one mild and the other severe. After the first episode, the patient wore again the silicone swab; and during the subsequent episode of severe epistaxis, we managed to easily remove the nasal swab and to pack the nasal fossae in a standard fashion. After these 2 episodes, no more epistaxis was observed. Unfortunately, the patient presented a severe right heart failure due to the presence of a severe arterovenous malformation; and she was in the heart-lung transplantation program. She eventually died from the disease.
