Hereditary Hemorrhagic Telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) describes the presenting manifestations of a disorder that is characterized by pathologic blood vessels. HHT is inherited as an autosomal dominant trait with variable penetrance. The abnormal vascular structures (dysplasias) can affect all the organs in the human body. The link between a physical stimulus and new lesion development has been established for mucosal trauma owing to nasal airflow turbulence, for ultraviolet exposure to the fingers, and for mechanical trauma to the dominant hand. The pressing question then is whether HHT treatment constitutes a stimulus that is sufficient to trigger new lesion development.

Influence of temporary nasal occlusion (tNO) on epistaxis frequency in patients with hereditary hemorrhagic telangiectasia (HHT).

The objective of the study was to evaluate the influence of temporary nasal occlusion (tNO) with hypoallergenic tape on the frequency and severity of epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). This prospective observational study included 20 HHT patients who were undergoing Nd:YAG laser therapy at regular intervals. Over a 3-month period, laser therapy was supplemented by tNO with hypoallergenic tape for 5 h/day on average. On a 0-10 numeric rating scale, the patients reported significantly greater satisfaction in epistaxis terms after tNO treatment, with mean scores of 5 before and 7 after 3-month tNO (p = 0.05). The Epistaxis Severity Score also fell significantly from a median of 3.59-2.43 after 3-month tNO compared with laser therapy alone (p = 0.01). The patients' hemoglobin levels remained stable during the study (median: 12.2 g/dL before tNO; median: 11.7 g/dL after tNO; p = 0.387). Overall, the present study confirms the positive influence of tNO on epistaxis in HHT patients and on subjective satisfaction. This simple and inexpensive strategy is therefore a helpful option, especially in addition to regular Nd:YAG laser therapy, and is recommended by the authors.

Update on Clinical Strategies in Hereditary Hemorrhagic Telangiectasia from an ENT Point of View.

OBJECTIVES: Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of vascular malformations with an absence of capillaries between arteries and veins. One major manifestation site is the nasal mucous membrane where recurrent nosebleeds occur. Our clinical strategy to treat patients with HHT has the aim to reduce nasal bleeding long-term with minimal local and general side effects. METHODS: We describe staged diagnosis and therapy including individual medical treatments of 97 patients with HHT. The success of treatment is monitored with a systematic questionnaire. RESULTS: The neodymium-doped yttrium aluminium garnet (Nd:YAG) laser therapy remains standard treatment of choice with no major side effects despite the need for repeated treatment. In addition new treatment strategies like nasal occlusion, local drug therapy, and nasal septal splinting show initial success. CONCLUSION: Improvement of the quality of life of HHT patients can be achieved by a multimodal concept. Several new treatment strategies like nasal septal splinting and nasal occlusion successfully expand the range of established methods. Further studies have to prove the safety and long-term effectiveness of the described individual medical treatments.

Comparison of HPV prevalence in HNSCC patients with regard to regional and socioeconomic factors.

HPV infection is considered as an independent risk factor for head and neck squamous cell carcinomas (HNSCC). Due to highly variable prevalence results in numerous studies, it is, however, difficult to estimate the relevance of HPV infection as risk factor for a specific patient collective. This study aimed to elucidate the disparities of HPV prevalence by analyzing socioeconomically and regionally different patient collectives. Two age, gender, stage and tumor location matched cohorts of 18 private health insured (PHIP) and 16 statutory health insured patients (SIP) suffering from an oropharyngeal squamous cell carcinoma (OSCC) and treated at a university hospital were screened for p16 overexpression and HPV infection by immunohistochemistry and PCR. In addition 85 HNSCC patients of an otolaryngology private practice (PPP) in a rural area were screened for p16 overexpression and positive cases were tested for HPV infection. HPV prevalence was 72.2% in the PHIP collective in comparison to 25.0% (p = 0.015) in the SIP collective with a significantly improved 5-year overall survival (p = 0.003) of the PHIP collective. The total HPV prevalence of PPP group was 7.1% with the highest infection rate in tonsillar carcinomas (33.3%) and a larger percentage of female patients in the HPV positive group (p = 0.037). This study shows that variable HPV infection rates in HNSCC can be caused by the selection of particular patient collectives, which suggest taking socioeconomic and regional factors into account for a decision on HPV testing, if it is not performed on a routine basis.

Netherton syndrome - Why ENT surgeons should be aware of this rare disease - report of a case.

BACKGROUND: Comèl-Netherton syndrome is an inherited ichthyosis that is associated with highly impaired epidermal cornification and barrier function. Literature sparsely reports of the occurrence of early onset skin cancer in people with Netherton syndrome. To the best of our knowledge the suitability of the severely altered skin in patients with Netherton syndrome for techniques of facial plastic reconstructive surgery has not been discussed in literature yet. CASE PRESENTATION: We present a 31-year-old caucasian female patient with Netherton syndrome who developed a defect of the right nasal ala. Biopsy revealed a well differentiated squamous cell carcinoma.We describe the reconstruction of a full thickness nasal defect with a paramedian forehead flap and an epidermal turn-in flap in Netherton syndrome. Despite the altered skin texture, reconstruction and healing were uneventful and the surgical result was favourable. CONCLUSION: Therefore the authors state that the development of cutaneous malignancies should be included as a possible complication in patients with Netherton syndrome. Standard techniques of surgical facial reconstruction can be applied in these patients; healing and outcome do not appear to be negatively affected by the underlying disease.

Triple test as predictive screen for unilateral weakness on caloric testing in routine practice.

OBJECTIVE: To investigate in vertigo patients in routine practice to what extent a rapid and straightforward triple bedside test (spontaneous nystagmus, head-shaking nystagmus, and the head impulse test) can predict a normal result on caloric testing. STUDY DESIGN: Prospective, single-blind, diagnostic study. SETTING: Tertiary referral center. PATIENTS: 151 patients (78 male and 73 female subjects; mean age, 52.5 +/- 16.4 yr) presenting with acute or recent symptoms of vertigo. INTERVENTION: Diagnostic evaluation. MAIN OUTCOME MEASURE: The negative predictive value (NPV) of the triple test in relation to a normal caloric test response. RESULTS: In unilateral weakness (UW) on caloric testing (UW, >=25%), the triple test had sensitivity of 63.6%, specificity of 85.4%, a positive predictive value (PPV) of 71.4%, and an NPV of 80.4%. In other words, 80.4% of patients with a negative triple test also had a normal response on caloric testing. In pronounced canal paresis (UW, >=50%), the triple test had sensitivity of 81.8%, specificity of 81.4%, a PPV of 55.1%, and an NPV of 94.1%. Significant differences were found between 2 subgroups assessed by examiners with differing levels of experience (p < 0.05). CONCLUSION: The triple test represents a good screening tool that quickly and reliably excludes unilateral weakness and in particular pronounced canal paresis on caloric testing.

Influence of Surface Processing on the Biocompatibility of Titanium.

Surface conditioning of titanium middle ear implants results in an improved biocompatibility, which can be characterized by the properties of fibroblasts cultured on conditioned surfaces. Titanium has been established as a favorable biomaterial in ossicular chain reconstruction. The epithelization of the surface of the implants is important for their integration and stable positioning in the middle ear. Mouse fibroblast cells were cultured on platelets made from pure Grade 2 titanium. Platelets that had been etched along their production process were compared to unetched platelets. The DNA in the cell nuclei was stained with DAPI and the actin filaments of the cytoskeleton were stained with FITC-conjugated phalloidin in order to analyze the cells grown on etched and unetched platelets by fluorescence microscopy. SEM (scanning electron microscopic) images were used to compare the surface structure of etched and unetched titanium platelets. There was a statistically significant increase of the area covered by the cytoplasm and increased actin expression by fibroblasts grown on the etched titanium platelets. In addition, the area of the platelets covered by nuclei on the etched platelets exceeded on average the one on unetched platelets, although this difference was not significant. The SEM pictures comparing unetched and etched titanium platelets showed a clear difference in surface structure. Surface conditioning of titanium implants improved the epithelization by fibroblasts and consequently etched titanium should be the preferred biomaterial for reconstructive middle ear surgery.