Hereditary hemorrhagic telangiectasia (HHT) is a rare, autosomal dominant disease characterized by the presence of recurrent epistaxis and small characteristic malformations of the peripheral blood vessels near the surface of the skin or mucosal linings. Arteriovenous malformations (AVM) of the lung, liver, and CNS are also known clinical findings. The purpose of this study was to examine quality of life (QoL) in patients with HHT in Norway. Sixty-six affected patients (39 women and 27 men) were included. QoL on overall-, health-related (HR-QoL), and disease-specific levels were measured with Cantril's Ladder (CL), Short Form 36 (SF-36), and a Symptom-specific QoL question in HHT patients (SFB-HHT-Q), respectively. Comparisons were made between patients and an age and gender adjusted normative sample from the Norwegian population (N = 990). Overall, the results reflected that several HHT disease-related variables were associated with reduced QoL on all three levels; overall QoL (CL), HR-QoL (SF36) as well as disease-specific QoL (SFB-HHT-Q), while demographic variables impacted HR-QoL in HHT patients. Compared to the normative sample, all subscales of SF36, but bodily pain, were significantly poorer in the HHT patients. HHT disease variables had the strongest association with QoL compared to demographic variables. The results substantiate that disease severity is associated with poorer QoL in this patients. Pain contributed independently to all levels of QoL.

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The report reflects an agreement based on the consensus conference of the International Standardization Committee on the Objective Assessment of the Nasal Airway in Riga, 2nd Nov. 2016. The aim of the conference was to address the existing nasal airway function tests and to take into account physical, mathematical and technical correctness as a base of international standardization as well as the requirements of the Council Directive 93/42/EEC of 14 June 1993 concerning medical devices. Rhinomanometry, acoustic rhinometry, peak nasal inspiratory flow, Odiosoft-Rhino, optical rhinometry, 24-h measurements, computational fluid dynamics, nasometry and the mirrow test were evaluated for important diagnostic criteria, which are the precision of the equipment including calibration and the software applied; validity with sensitivity, specificity, positive and negative predictive values, reliability with intra-individual and inter-individual reproducibility and responsiveness in clinical studies. For rhinomanometry, the logarithmic effective resistance was set as the parameter of high diagnostic relevance. In acoustic rhinometry, the area of interest for the minimal cross-sectional area will need further standardization. Peak nasal inspiratory flow is a reproducible and fast test, which showed a high range of mean values in different studies. The state of the art with computational fluid dynamics for the simulation of the airway still depends on high performance computing hardware and will, after standardization of the software and both the software and hardware for imaging protocols, certainly deliver a better understanding of the nasal airway flux.

Compared with other populations, the grading of epistaxis in Norwegian patients with HHT gave generally similar results. A multicenter epidemiological study is required to get a larger study population. A common internationally accepted grading or classification system for epistaxis in HHT is highly recommended.

Objective and Importance: Cerebrospinal fluid (CSF) fistula from the middle cranial fossa into the sphenoid sinus is a rare condition. In the past, the treatment of choice has been closure via a craniotomy. Only few geriatric cases are known, which were successfully operated by endoscopic surgery. We present a further case of nontraumatic CSF fistula originating from the middle cranial fossa. A new endoscopic technique was applied. We discuss treatment options for this rare defect. Clinical Presentation: A 76-year-old patient presented with a 2-year history of rhinorrhea. High levels of b-trace protein pointed to a diagnosis of CSF fistula. The defect was located at the anterior and inferior aspect of the pterygoid recess of the left sphenoid sinus. Intervention: The patient was operated using an endoscopic trans-sphenoidal approach. After endoscopic opening of the maxillary and sphenoid sinus, a complete posterior ethmoidectomy was performed. The medial part of the pterygoid process was removed, allowing endoscopic exposure and closure of the defect. At 1-year follow-up, the CSF fistula had not recurred and the patient had no sequel from the surgical procedure. Conclusion: In selected cases, this new endoscopic partial transpterygoid approach to the middle cranial fossa is recommended for surgical repair of CSF fistula involving the lateral extension of the sphenoid sinus. To our knowledge, ours is the oldest patient with this condition successfully operated by endoscopic means at the world's most northern university hospital.