phenylephrine-hydrochloride and Telangiectasia--Hereditary-Hemorrhagic

Patients with hereditary haemorrhagic telangiectasia can present with a multitude of symptoms caused by telangiectasia and arteriovenous malformations in the nose, brain, gastrointestinal tract, liver and spinal cord. Clinicians should be aware of the potential diagnosis of hereditary haemorrhagic telangiectasia and how to manage these patients both in the acute and chronic setting. Identifying these patients and optimising their management can help reverse the reduced life expectancy back to that of the normal population. The management of these patients is complex and often requires a multidisciplinary approach, with difficult discussions to be had around screening for arteriovenous malformations and genetic testing. The stepwise management ladder can be used in both the medical and surgical strategies; there are multiple pharmacological and surgical options available, all with their own side effects and risks. Patient education is key to help informed decision making. This article outlines the clinical characteristics of the disease and management options available.

Topics: Arteriovenous Malformations; Epistaxis; Genetic Testing; Humans; Nose; Telangiectasia, Hereditary Hemorrhagic

Topics: Aged, 80 and over; Embolization, Therapeutic; Enbucrilate; Epistaxis; Humans; Male; Nose; Telangiectasia, Hereditary Hemorrhagic; Tissue Adhesives; Vascular Malformations

A rare clinical case of recurrent nosebleeds associated with Randu-Osler disease, in which drug, hemostatic, transfusion therapy and combined nasal tamponade were ineffective, required ligation of the external carotid arteries on both sides, is presented.. В статье приведено описание редкого клинического случая рецидивирующего носового кровотечения на фоне болезни Рандю—Ослера, при котором медикаментозная, гемостатическая, трансфузионная терапия и комбинированная тампонада носа оказались неэффективными; это вызвало необходимость выполнения перевязки наружных сонных артерий с обеих сторон.

Topics: Carotid Arteries; Carotid Artery, External; Epistaxis; Humans; Ligation; Nose; Telangiectasia, Hereditary Hemorrhagic

Nosebleeds (epistaxis) are usually minor. Medical intervention is only necessary in about 6% of cases. The source of bleeding is frequently located in the anterior region of the nose (Kiesselbach's plexus). The estimated lifetime prevalence of epistaxis is 60%. Diffuse epistaxis is often a manifestation of systemic disease. Epistaxis is the leading symptom of Rendu-Osler-Weber disease (hereditary hemorrhagic telangiectasia, HHT). If intervention is required, the first-choice of treatment is bidigital compression for several minutes. Common therapeutic measures include local hemostasis using electrocoagulation or chemical agents, e.g., silver nitrate. Resorbable anterior nasal tampons or tampons with a smooth surface are also frequently employed. In case of failed surgical closure of the sphenopalatine artery, angiographic embolization is the method of choice.. Meist handelt es sich beim Nasenbluten (Epistaxis) um kleinere Blutungen. Medizinische Maßnahmen sind nur in etwa 6 % der Fälle erforderlich. Oft liegt die Blutungsquelle im vorderen Nasenabschnitt (Locus Kiesselbachii). Die geschätzte Gesamtlebenszeitprävalenz für Epistaxis beträgt 60 %. Tritt die Epistaxis in diffuser Form auf, so ist das häufig die Manifestation einer systemischen Erkrankung. Beim M. Rendu-Osler-Weber (hereditäre hämorrhagische Teleangiektasie, HHT) stellt die Epistaxis das Leitsymptom dar. Erstmaßnahme der Wahl bei Interventionsbedarf ist die bidigitale Kompression über mehrere Minuten. Verbreitete Therapiemaßnahmen sind u. a. die lokale Blutstillung mittels Elektrokoagulation oder mit chemischen Mitteln wie Silbernitrat. Auch resorbierbare vordere Nasentamponaden oder Tamponaden mit glatter Oberfläche werden häufig eingesetzt. Beim Versagen des chirurgischen Gefäßverschlusses der A. sphenopalatina ist die angiographische Embolisation Methode der Wahl.

Topics: Embolization, Therapeutic; Epistaxis; Humans; Nose; Telangiectasia, Hereditary Hemorrhagic

Nasal closure, also known as the modified Young's procedure was introduced in Denmark in 2008, as a surgical solution to severe epistaxis in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of this study was to report the overall satisfaction of the procedure from a patient's point of view as well as the occurrence of complications.. All the HHT patients who underwent nasal closure from 2008 to 2018 were included in the study. The patients were evaluated for postoperative complications and subjective outcome using Glasgow Benefit Inventory (GBI).. Ten patients were included in the study and were observed for a mean of 64 months. None of the patients was completely free of complications, and reversal was requested in a single case. Haemoglobin levels rose with an average of 2.8 g/dl. The average GBI score after surgery was 38.05. Nine of ten patients would recommend nasal closure to fellow HHT patients.. Nasal closure is highly recommended among patients, but due to the rate of postoperative complications, the procedure should be reserved for a carefully selected group of HHT patients.

Topics: Adult; Aged; Cohort Studies; Denmark; Epistaxis; Female; Humans; Male; Middle Aged; Nasal Mucosa; Nasal Surgical Procedures; Nose; Patient Reported Outcome Measures; Patient Satisfaction; Surgical Flaps; Telangiectasia, Hereditary Hemorrhagic; Treatment Outcome

Topics: Bandages; Epistaxis; Humans; Nose; Telangiectasia, Hereditary Hemorrhagic

Hereditary hemorrhagic telangiectasia (HHT) is characterized by recurrent epistaxis that can lead to a feeling of losing control. We assessed potential benefits and side effects of different nasal packings used by patients themselves.. An online-questionnaire in English and German was used to analyze nasal self-packings.. 588 of the 915 respondents suffered from HHT with most of them having moderate or severe epistaxis. Almost two thirds of the patients had already treated themselves with nasal packings. While one quarter used non-pneumatic nasal packings (NPNP) or pneumatic nasal packings (PNP), nearly half of the patients took only tissues to stop the bleeding. Patients with severe epistaxis used PNP more often than NPNP. Using nasal packings, most patients could stop their nosebleeds after a while. Patients using PNP reported the feeling of losing control less often and significant improvements in quality of life with a positive GBI.. Our study showed that most patients with HHT using nasal self-packings could stop the bleeding after a while. Nasal self-packing is a user-friendly and secure method leaving patients more self-confident and independent.

Topics: Epistaxis; Humans; Nose; Quality of Life; Self Care; Surveys and Questionnaires; Telangiectasia, Hereditary Hemorrhagic

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that leads to frequent epistaxis. It can have a significant impact on quality of life. Many reports exist regarding various therapies to address the epistaxis.. We presented our technique for addressing the epistaxis associated with HHT.. Patients are treated in the operating room while they are under general anesthesia. A local anesthetic is injected sublabially, and oxymetazoline is dripped into the nose. The coblation wand is used to treat the telangiectasias. Bevacizumab is then injected into the nasal cavity bilaterally.. The coblation wand, with or without adjunctive bevacizumab injection, is a technically feasible intervention for patients with HHT that all providers can perform.

Topics: Anesthesia, Local; Bevacizumab; Cautery; Electrosurgery; Endoscopy; Epistaxis; Humans; Nose; Oxymetazoline; Practice Guidelines as Topic; Telangiectasia, Hereditary Hemorrhagic; Video Recording

Nasal closure has been shown to effectively manage severe epistaxis refractory to other treatments in patients with hereditary hemorrhagic telangiectasia (HHT). The nasal closure procedure may be underutilized because of its surgical complexity and flap breakdown.. This work is a retrospective review of 13 HHT patients treated for severe epistaxis with nasal closure between 2005 and 2013. Operating room (OR) time, need for revision surgery, preprocedure, and postprocedure epistaxis severity score (ESS), complete blood count values, and Glasgow Benefit Inventory (GBI) questionnaire results were collected for each patient. The technique is described. We characterize a typical nasal closure patient and compare outcomes based on our experience with the traditional 3-flap closure and a simplified 2-flap nasal closure procedure.. The average candidate for nasal closure in this series had an ESS of 7.88, hemoglobin (Hgb) of 8.3 g/dL, and received multiple transfusions, iron therapy, and cautery/coagulation procedures. Average ESS subsequent to nasal closure using the 2-flap method is 0.92 and mean GBI score is 56.3. Comparison of 5 patients who underwent the traditional 3-flap nasal closure procedure and 8 patients receiving the 2-flap nasal closure showed no significant difference in postoperative ESS or GBI metrics. Mean operating room times of the traditional and simplified methods were 3.12 hours and 1.44 hours (p = 0.0001). Mean time to first revision for 8 nasal closure patients was 21.5 months.. In short-term follow-up, the 2-flap procedure showed comparable effectiveness with significantly reduced complexity and operative time compared to the traditional nasal closure method.

Topics: Adult; Aged; Epistaxis; Female; Humans; Male; Middle Aged; Nose; Otorhinolaryngologic Surgical Procedures; Surgical Flaps; Telangiectasia, Hereditary Hemorrhagic; Treatment Outcome

Topics: Aged; Cheek; Epistaxis; Gastrointestinal Hemorrhage; Humans; Male; Nose; Telangiectasia, Hereditary Hemorrhagic

Application of endosseous implants in prosthetic craniofacial reconstruction represents a secure and reliable method and is a well-established surgical procedure. In areas of low bone presentation, standardized plate-like titanium implants are available. For allowing a congruent fitting to the recipient site, these contemporary implants have to be manually adapted--implicating drawbacks in terms of time consumption, technical complexity, and insufficient functional outcome. Owing to these limitations, a custom-made patient-specific implant is introduced based on Digital Imaging and Communications in Medicine (DICOM) data and designed for optimal prosthetic reconstruction.. For the first time, the application of a prefabricated patient-specific implant for retaining a craniofacial prosthesis is described. In a 64-year-old man with partial nasal defect standardized plate-like implants failed because of compromised bone quality due to Osler disease. To realize an implant-retained prosthetic reconstruction, a patient-specific implant was fabricated based on computer-aided design and computer-aided manufacturing (CAD/CAM) technology. This technique allows for considering the implant's ideal geometry as well as its correct placement of the required magnetic abutments. Furthermore, the surface of the implant can be designed for optimal hygienic conditions.. The patient-specific implant was successfully inserted in a time effective operating procedure. Follow-up at 6 months showed an excellent functional and aesthetic outcome.. Application of prefabricated patient-specific implants offers prospectively an ideal tool for retaining craniofacial prostheses and should be considered a viable option in standard cases, but obligatory in anatomically demanding defects.

Topics: Alloys; Bone Screws; Computer-Aided Design; Follow-Up Studies; Humans; Image Processing, Computer-Assisted; Imaging, Three-Dimensional; Magnets; Male; Middle Aged; Nose; Nose Deformities, Acquired; Operative Time; Patient-Specific Modeling; Printing, Three-Dimensional; Prostheses and Implants; Prosthesis Design; Prosthesis Implantation; Prosthesis Retention; Telangiectasia, Hereditary Hemorrhagic; Titanium; Tomography, X-Ray Computed; Treatment Outcome

In recent years, the positive effect of topically applied estriol nose ointment in the adjuvant therapy of Morbus-Rendu-Osler (HHT) has been proven. Due to the induced metaplasia, a complete destruction of the ciliated cells may be expected. However, data regarding the ciliary function of HHT patients with and without the use of topical estriol application are currently lacking.. Ciliated samples were obtained by gently brushing the inferior nasal turbinate of 19 healthy volunteers and 15 patients with known HHT (8 of them regularly using 0.1% estriol nose ointment for 2 years (HHTwE) and 7 of them not using the ointment in the last 12 months (HHTwoE)). Analysis was done with an inverted phase contrast microscope connected to a high-speed digital camera. Recorded parameters were the visual integrity (VI) of the ciliary beat and its frequency (CBF) in Hz.. The VI index of all samples showed an undisrupted, even beating pattern with a difference between the three groups. The mean CBF in all HHT patients was reduced compared to the control group`s mean CBF. Within the HHT group itself, the mean CBF was reduced in the HHTwE group compared to the HHTwoE group.. The ciliary beat frequency of HHT patients is impaired compared to the control group and even more so if the HHT patients topically apply estriol more than 6 months. An undisrupted beating pattern is found in the HHTwE group despite the fact that estrogens induce a transformation of the ciliated columnar into a keratinizing squamous epithelium. This data may justify the adjuvant application of estriol as a nose ointment in the treatment of epistaxis in HHT patients without the fear of damage to the nose`s mucus clearance.

Topics: Administration, Topical; Adult; Cilia; Estriol; Female; Humans; Male; Middle Aged; Nose; Ointments; Telangiectasia, Hereditary Hemorrhagic

Teleangiectases are the source of hemorrhage in many HHT patients. Most frequent site of bleeding is the nose and more than 90% of all individuals with HHT suffer from recurrent epistaxis. Despite all efforts, treatment of epistaxis in HHT continues to be a problem for many otorhinolaryngologists, who can alleviate recurrent nosebleeds by Septodermoplasty or laser therapy, but rarely can stop nasal hemorrhages permanently. Recurrence is almost inevitable, but the mechanisms of recurrence are not fully understood.. Prior to routine Nd:YAG laser therapy of nasal telangiectases the nasal mucosa of 17 patients with HHT according to the clinical diagnostic criteria of the HHT Foundation International was examined with a 0 degrees contact rhinoscope in areas with clinically visible telangiectases as well as in clinically normal mucosa. The digitally recorded images were compared to findings of a group of five healthy volunteers and the findings of five patients with polypoid sinusitis.. Visualization of subepithelial vessels was feasible in all individuals of the study group as well as the control groups. Dilated vascular loops and tortuous vessels could be found in the study groups as well as in the control group, but the overall density of telangiectatic vessels was on an average higher in the HHT group. The process of vessel dilatation and tortuous configuration seemed to progress with age.. Contact endoscopy allows the investigation of the angioarchitecture of capillaries of the nasal mucosa in vivo. This observation may be of significance for studies of nasal diseases, which are accompanied by epistaxis. With this regard it seems to be of special interest for studies of HHT.

Topics: Adolescent; Adult; Aged; Child; Endoscopy; Epistaxis; Female; Humans; Laser Therapy; Male; Middle Aged; Nose; Recurrence; Telangiectasia, Hereditary Hemorrhagic

Epistaxis is a common complication in patients with hereditary hemorrhagic telangiectasia. Its treatment is generally aimed at controlling the frequency and severity of nasal hemorrhage and involves surgery, transcatheter embolization, topical treatment, or a combination. Despite this multitude of treatment methods, the long-term prognosis for many patients remains poor. We describe a patient in whom direct intralesional injections of bleomycin successfully palliated severe recurrent epistaxis for almost 2 years without the need for adjuvant therapy.

Topics: Anesthesia, General; Angiography, Digital Subtraction; Antimetabolites, Antineoplastic; Bleomycin; Dose-Response Relationship, Drug; Drug Administration Schedule; Epistaxis; Follow-Up Studies; Humans; Injections, Intralesional; Male; Middle Aged; Nose; Retreatment; Telangiectasia, Hereditary Hemorrhagic

In seven patients with hereditary haemorrhagic teleangiectasia (HHT) only one patient had lasting benefit from treatment with the flashlamp-pulsed dye laser. In seven other patients who had received other treatment before, none had any observable effect of this laser. The flashlamp-pulsed dye laser therefore does not seem to be effective in the treatment of nasal teleangiectasias.

Topics: Humans; Laser Coagulation; Nose; Telangiectasia, Hereditary Hemorrhagic

A 69-year-old male patient with hereditary hemorrhagic telangiectasia presented with severe epistaxis requiring repeated transfusions. Both nasal passages were densely populated with these vascular malformations that involved the entire nasal mucosa. A total rhinotomy was performed and all the nasal mucosa and turbinates were excised. Both nasal passages were completely resurfaced with a free radial forearm skin flap. The vascular pedicle was delivered from the nasal passage via the maxillary antrum to anastomose with the facial vessels in the cheek. Following surgery the patient had no further significant epistaxis.

Topics: Aged; Epistaxis; Humans; Male; Nose; Surgical Flaps; Telangiectasia, Hereditary Hemorrhagic

Percutaneous embolization to control the nasal blood circulation was used in 19 patients with vascular nasal disorders. Embolization was the initial treatment for intractable posterior epistaxis in 11 patients, 9 (82%) of whom were controlled without further treatment. Used as an adjunct to surgery on vascular nasal tumors, embolization reduced intraoperative blood loss in four patients to an average of 800 cc. In four patients with Osler-Weber-Rendu disease, treatment with embolization did not significantly alter the course of their disease. Overall, however, this relatively new technique enhances successful management of difficult epistaxis cases.

Topics: Embolization, Therapeutic; Epistaxis; Histiocytoma, Benign Fibrous; Humans; Nose; Nose Neoplasms; Punctures; Telangiectasia, Hereditary Hemorrhagic

Topics: Adult; Biopsy; Diagnosis, Differential; Humans; Middle Aged; Mouth Mucosa; Nose; Telangiectasia, Hereditary Hemorrhagic

Topics: Adult; Female; Hemorrhage; Humans; Nose; Telangiectasia, Hereditary Hemorrhagic

Topics: Adolescent; Bandages; Blood Coagulation Disorders; Carotid Artery, External; Epistaxis; Female; Hemostasis; Humans; Hypertension; Hypnotics and Sedatives; Ligation; Male; Maxillary Artery; Middle Aged; Nasal Decongestants; Nose; Nose Neoplasms; Pressure; Recurrence; Telangiectasia, Hereditary Hemorrhagic; Wounds and Injuries

Topics: Adult; Epistaxis; Follow-Up Studies; Humans; Male; Methods; Nose; Skin Transplantation; Surgery, Plastic; Telangiectasia, Hereditary Hemorrhagic; Transplantation, Homologous

Topics: Adolescent; Adult; Aged; Blood Transfusion; Cautery; Child; Cryosurgery; Epistaxis; Estrogens; Female; Humans; Lubrication; Male; Middle Aged; Nose; Postoperative Complications; Pressure; Skin Transplantation; Telangiectasia, Hereditary Hemorrhagic

Topics: Epistaxis; Humans; Male; Methods; Middle Aged; Nasal Septum; Nose; Osteotomy; Skin Transplantation; Telangiectasia, Hereditary Hemorrhagic; Transplantation, Autologous

Topics: Carotid Arteries; Epistaxis; Humans; Nasal Septum; Nose; Nose Neoplasms; Seasons; Telangiectasia, Hereditary Hemorrhagic

Topics: Aged; Blood Transfusion; Electrocoagulation; Epistaxis; Female; Humans; Nose; Pedigree; Peptic Ulcer Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Telangiectasia, Hereditary Hemorrhagic; Uterine Hemorrhage

Topics: Cryosurgery; Emergencies; Epistaxis; Humans; Hypertension; Intubation; Methods; Nose; Telangiectasia, Hereditary Hemorrhagic

Topics: Humans; Nasal Surgical Procedures; Nose; Replantation; Telangiectasia, Hereditary Hemorrhagic; Telangiectasis