Patient comfort following FESS and Nasopore® packing, a double blind, prospective, randomized trial.

BACKGROUND: The use of nasal packing after functional endoscopic sinus surgery (FESS) is often associated with pain and a feeling of pressure for patients. The aim of the present work was to investigate a modern wound dressing made of polyurethane (Nasopore®) that makes removal of the nasal packing unnecessary and is focussed on patient comfort. METHODOLOGY: Following bilateral FESS, after randomisation, one side was packed with Nasopore® while the other side was without packing as a control. The following parameters from 47 patients were determined daily in two centres from post-operative day 1 for the duration of the inpatient stay in a double-blinded setting: side-specific post-operative bleeding, nasal breathing and feeling of pressure as well as the general parameters sleep disturbance, headaches and general well-being. Which side patients considered subjectively the better was also recorded. RESULTS: No significant differences were determined between the two sides in terms of the rates of post-operative bleeding and nasal breathing. The feeling of pressure was slightly less on the side packed with Nasopore® on post-operative days 2 and 3. No trend could be observed regarding which side patients described as being subjectively better. CONCLUSION: There were only slight differences in patient comfort between the Nasopore® side and the control. Because the feeling of pressure in the midface was significantly less and there were no complications, this suggests there is greater patient comfort when using Nasopore® compared to using no nasal packing.

Congenital erythropoietic porphyria presenting with recurrent epistaxis: a case report.

BACKGROUND: Congenital erythropoietic porphyria (CEP), also known as pink tooth or Gunther disease, is a rare hereditary disorder caused by an enzyme mutation in the heme biosynthesis pathway, which leads to the accumulation of immature and non-physiological protoporphyrin rings in various tissues. CEP is characterized by sun-exposed bullous skin lesions, hemolytic anemia, red/brown urine, and teeth staining. CASE PRESENTATION: We present a unique case of a 10-year-old Asian boy with CEP who presented with recurrent epistaxis, an unusual presentation for this condition. Based on clinical presentation and laboratory findings, including elevated urine uroporphyrin and coproporphyrin I and III levels, microcytic anemia, a higher red cell distribution width (RDW), and a lower platelet count, a thorough assessment and detailed workup resulted in a diagnosis of CEP. The patient underwent a successful splenectomy and recovered without any complications. CONCLUSION: This case report aims to raise awareness among healthcare professionals about the uncommon and atypical presentation of CEP and its management options.

Port-wine stains are more than skin-deep! Expanding the spectrum of extracutaneous manifestations of nevi flammei of the head and neck.

UNLABELLED: It is well known that port-wine stains of the upper part of the face may herald abnormalities of the brain or eye in the form of Sturge-Weber syndrome. This study focuses on other extracutaneous anomalies in patients with nevi flammei of the head and neck, giving rise to functional complications. PATIENTS AND METHODS: A retrospective study was performed on patients with port-wine stains involving the head and neck area. Records were reviewed for demographic parameters, extent of the lesion, clinical complications, diagnostic measures, previous treatments, ultimate therapeutic approach, and outcome. RESULTS: Nine patients, mean age 50.4 years, with port-wine stains and clinical symptoms due to extracutaneous involvement, were admitted and treated from 2006 to 2009. Major clinical features included macrocheilia in three cases, gingival bleeding in two, dysphonia with globus sensation, painful parotideal swelling with recurrent otitis, painful lingual swelling, recurrent epistaxis, and nasal obstruction in one case each. Cases with lower lip hypertrophy were treated by conventional surgical approaches. Recurrent epistaxis and nasal obstruction due to affected inferior turbinate were treated by Nd:YAG laser therapy, and globus sensation and dysphonia by speech therapy. Patients with gingival affection and recurrent otitis were treated by local ear care. CONCLUSION: Port-wine stains in the head and neck may develop extracutaneous manifestations causing severe problems. A multimodal and interdisciplinary approach is mandatory for an appropriate treatment.

Local sclerotherapy with polydocanol (Aethoxysklerol®) for the treatment of Epistaxis in Rendu-Osler-Weber or Hereditary Hemorrhagic Telangiectasia (HHT): 15 years of experience.

Hereditary Haemorrhagic Telangiectasia or Rendu-Osler-Weber syndrome is a rare autosomal dominant vascular disease characterized by mucocutaneous and gastrointestinal telangiectases and localized arteriovenous malformations in lung, brain and liver. Epistaxis, due to rupture of telangiectases of the nasal mucosa, is the most frequent clinical manifestation, leading in many cases to severe impairment of the quality of life in the patients. Though several treatments have been used to reduce epistaxis, none have been completely effective, with the exception of polydocanol (Aethoxysklerol®) in submucosal or subpericondrial injections, which was first presented in 2000 with very good results. After fifteen years using polydocanol in submucosal injections on 45 patients and with nearly 300 injections, we have observed that in 95% of all cases, their nose bleeds improved with respect to frequency and quantity without any important side effects. There was just one case of septal perforation, another with increased septal perforation, and one patient who suffered from dizziness and blurred vision for a few minutes. In this paper the results obtained using this technique over a fifteen-year period will be presented and evaluated.

Treatment of acute epistaxis secondary to guttural pouch mycosis with transarterial nitinol vascular occlusion plugs in three equids.

CASE DESCRIPTION: 2 horses and 1 pony were evaluated for right-sided (1 horse and the pony) and left-sided (1 horse) acute epistaxis of 1 day's to 1 month's duration. CLINICAL FINDINGS: Endoscopic examination of the 3 equids revealed that the hemorrhage originated from the right maxillary artery in 2 equids and from the left internal carotid artery in the third. Mycosis of the auditory tube diverticulum (guttural pouch) was detected in all 3 equids. TREATMENT AND OUTCOME: All 3 equids underwent surgery, and transarterial nitinol intravascular plugs were placed to occlude affected blood vessels. All equids survived for a long period (ie, a minimum of 1 to 2 years) and returned to their previous use. All had complete regression of clinical signs of guttural pouch mycosis without additional medical treatment. CLINICAL RELEVANCE: The use of transarterial nitinol intravascular plugs appeared to be an effective alternative to other techniques for the treatment of epistaxis secondary to guttural pouch mycosis, including transarterial occlusion devices such as embolization coils.

Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy: results from the von Willebrand Disease Prophylaxis Network.

Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed in severe haemophilia A, may also be observed. The bleeding patterns of VWD can affect quality of life, and may be life-threatening. The von Willebrand Disease Prophylaxis Network is an international study group formed with the goal of investigating the role of prophylaxis in clinically severe VWD. The objective of the present study is to investigate the response to prophylaxis focusing primarily on epistaxis, joint bleeding, gastrointestinal bleeding, and heavy bleeding associated with menses. Data from 105 subjects, 10 enrolled in a prospective study and 95 in a retrospective study between 2008 and 2013, were available for analysis. The median annualized rate reductions in bleeding were significant for epistaxis (P < 0.0001), gastrointestinal bleeding (P = 0.0003), joint bleeding (P < 0.0001), and menorrhagia (P = 0.008). Doses on a group level were approximately the same prior to and during prophylaxis, but more patients with gastrointestinal bleeding had prophylaxis three or more times per week as well as higher dosages. Our study, which primarily used retrospective data, indicates that prospective studies are needed to better delineate the doses and dose intervals that should be used for prophylactic treatment of VWD.

Management of pregnancy in osteogenesis imperfecta: new perspectives.

Osteogenesis imperfecta during pregnancy presents increased risk to mother and fetus. In addition to the well-known skeletal changes, other recently recognized metabolic abnormalities also may lead to maternal and fetal problems during labor and delivery. A discussion of risk factors and their managment is presented. The importance of considering the possibility of fetal osteogenesis imperfecta is stressed, and cesarean section is presented as the method of choice for delivery. Important genetic factors including the lack of correlation between the severity of involvement of parent and offspring and the use of pyrophosphate analyses are discussed. An illustrative case is presented.

[Impact of prior biological assessment of coagulation on the hemorrhagic risk of fiberoptic bronchoscopy]. / Bilan de coagulation et risque hémorragique de la fibroscopie bronchique.

INTRODUCTION: The goals of the study were to test the value of coagulation tests to predict bleeding events during fiberoptic bronchoscopy (FOB) and to identify risk factors. METHODS: A monocentric prospective study of 426 procedures performed by one physician was realized. A standardized questionnaire and coagulation tests including prothrombine time, activated cephaline time and platelets counts were performed before the procedure. Bleeding events, defined by a loss of more than 50 ml of blood, were recorded for all patients. RESULTS: 44 patients (10.3%) had bleeding events, modifying the procedure in 19 cases. No death occurred following FOB during the study. Among the 17 patients with abnormalities on coagulation test before FOB, only one had significant bleeding. Two risk factors were found as predictors of bleeding events: nose or gum bleeding (OR=4.99, CI (95%) [2.6-9.5]; p<0.001) and left cardiac failure (OR=4.53, CI (95%) [1.7-12.1]; p<0.01). CONCLUSION: This study shows that abnormalities on coagulation tests are not predictive for bleeding events. Nose or gum bleeding and left cardiac failure may be risk factors for bleeding events during FOB.

A self-controlled comparative clinical trial to explore the effectiveness of three topical hemostatic agents for stopping severe epistaxis in pediatrics with inherited coagulopathies.

OBJECTIVE: The aim of this study was to assess the effectiveness of localized treatments to persistently stop epistaxis in patients with inherited bleeding disorders. METHODS: In a self-controlled comparative clinical trial, to offer the best solution to stop epistaxis at home (within 10 minutes), patients with inherited bleeding disorders were treated using three different topical hemostatic agents, including Tranexamic acid impregnated tampon, EpiCell tampon prepared from oxidized regenerated cellulose pad, and ChitoHem tampon (reinforced with chitosan). The results of using these different products on three groups of randomly selected patients were ultimately compared using the χ(2) and Fisher's exact test statistics. RESULTS: A total of 31 patients, 5 females and 26 males with a mean age of 5.6 years, were included in the study. Twenty-three patients had Glanzmann disease, four had von-Willebrand disease, two had Bernard soulier syndrome, two had activated factor VII deficiency, and one patient had impaired secretion of adenosine deaminase. The study exhibited that statistically there was no significant difference between EpiCell tampon and Tranexamic acid impregnated tampon treatments with respect to the hemostasis duration. However, ChitoHem tampon was more efficient than Tranexamic acid impregnated tampon (P value <0.001) and EpiCell tampon (P value < 0.05). CONCLUSION: ChitoHem tampon, the chitosan-reinforced product, was the best therapy solution to stop epistaxis. We recommend further research on the use of other hemostatic agents for localized bleeding in patients with inherited bleeding disorders.

Effects of dilution and prolonged storage with preservative in a polyethylene container on Bevacizumab (Avastin™) for topical delivery as a nasal spray in anti-hereditary hemorrhagic telangiectasia and related therapies.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia and severe, recurrent epistaxis is a common clinical phenotype associated with HHT. An intranasal treatment regime of diluted Avastin™ (Bevacizumab; recombinant humanized anti-vascular epithelial growth factor immunoglobin G1) using apulsatile nasal irrigator has proven efficacious in clinical practice. However, concerns regarding the stability of Avastin™ following dilution and prolonged storage in standard containers used for drug delivery, such as polyethylene bottles, have so far prevented a more widespread clinical use. Compatibility with the preservative benzalkonium chloride was also unknown. OBJECTIVE: This study aimed at determining, whether dilution, prolonged refrigerated storage and the presence of the preservative benzalkonium chloride - as required for novel Avastin™ formulations - affected the biochemical and electrochemical properties of the drug. METHODS: We performed a detailed biochemical and electrochemical analysis of Avastin™, including native and sodium dodecyl sulfate polyacrylamide gel electrophoresis, enzyme-linked immunosorbent assay and isoelectric focusing. RESULTS: We did not detect any evidence of degeneration or aggregation following dilution and prolonged, refrigerated storage or from the presence of benzalkonium chloride. All biochemical and electrochemical properties of Avastin™ after dilution and prolonged, refrigerated storage were undistinguishable from control. CONCLUSIONS: Our data provide important insight into the stability of Avastin™ and allow the consideration of novel Avastin™ formulations, including its use in a metered-dose nasal spray for the treatment of HHT and other applications.

Manejo odontológico da Síndrome de Rendu-Osler-Weber: relato de caso / Dental management of Rendu-Osler-Weber Syndrome: case report

A Telangiectasia Hemorrágica Hereditária (THH) ou Síndrome de Rendu-Osler-Weber (SROW) é uma displasia fibrovascular sistêmica que apresenta alterações na camada muscular da parede dos vasos sanguíneos. Isto faz com que os vasos sejam sujeitos a rupturas frequentes.Este diagnóstico pode ficar a cargo do cirurgião dentista visto que os primeiros sinais podem ser observados em boca. Podem ser identificados telangiectasias e hemorragias na mucosa bucal.Por segurança, o manejo odontológico deste paciente deve ser focado na sua condição de saúde geral. Assim, este artigo tem por objetivo a descrição de um caso clínico de tratamento odontológico de uma paciente com SROW, esclarecendo as principais características dessa síndrome e relatando as peculiaridades do seu manejo.

The Rendu-Osler-Weber Syndrome or Hereditary Hemorrhagic Telangiectasia (HHT) is a rare systemic fibrovascular dysplasia, which causes a defect in the elastic and muscle layer of the blood vessel wall, turning them more vulnerable to traumas and spontaneous ruptures. The dentists can play an important role in this diagnostic because the first signs often appear in the mouth, such as telangiectasia and recurrent bleeding in the oral mucosa. The management of this patient should be appropriate to its systemic profile to ensure the safety and effectiveness of dental treatment. This work aims to describe the main characteristics of this syndrome, its peculiarities and limitations of management during dental treatment, followed by the presentation of a clinical case.