Nasal Endoscopy in the Clinical Diagnosis of Hereditary Hemorrhagic Telangiectasia.

OBJECTIVES: To evaluate the role of nasal endoscopy for early clinical diagnosis of hereditary hemorrhagic telangiectasia (HHT) in children and to investigate the characteristics of epistaxis and mucocutaneous telangiectases in our pediatric population. STUDY DESIGN: From May 2016 to December 2019, a cross-sectional observational study was conducted, recruiting children aged 2-18 years with a parent affected by HHT. To identify the Curaçao criteria, all children underwent collection of clinical history, mucocutaneous examination, and nasal endoscopy. The clinical data were then compared with the genetic data acquired subsequently. RESULTS: Seventy children (median age, 10.8 years) were included. All underwent nasal endoscopy without complications. Forty-six children were positive by genetic testing; of these, 26 % had skin and oral telangiectases and 91 % had nasal telangiectases. The diagnostic sensitivity of the Curaçao criteria increased from 28 % (95 % CI, 16%-43 %) to 85 % (95 % CI, 71%-94 %; P < .0001) when the nasal telangiectases were included. CONCLUSIONS: The magnified and complete endoscopic view of the nasal cavities proved useful in increasing the diagnostic sensitivity of the Curaçao criteria. Such an examination turned out to be feasible and safe. For this reason, we believe that nasal endoscopy should be included in the diagnostic assessment of pediatric patients with suspected HHT.

"TuNa-saving" endoscopic medial maxillectomy: a surgical technique for maxillary inverted papilloma.

The maxillary sinus is the most common site of sinonasal inverted papilloma. Endoscopic sinus surgery, in particular endoscopic medial maxillectomy, is currently the gold standard for treatment of maxillary sinus papilloma. Although a common technique, complications such as stenosis of the lacrimal pathway and consequent development of epiphora are still possible. To avoid these problems, we propose a modification of this surgical technique that preserves the head of the inferior turbinate and the nasolacrimal duct. A retrospective analysis was performed on patients treated for maxillary inverted papilloma in three tertiary medical centres between 2006 and 2014. Pedicle-oriented endoscopic surgery principles were applied and, in select cases where the tumour pedicle was located on the anterior wall, a modified endoscopic medial maxillectomy was carried out as described in this paper. From 2006 to 2014 a total of 84 patients were treated. A standard endoscopic medial maxillectomy was performed in 55 patients (65.4%), while the remaining 29 (34.6%) had a modified technique performed. Three recurrences (3/84; 3.6%) were observed after a minimum follow-up of 24 months. A new surgical approach for select cases of maxillary sinus inverted papilloma is proposed in this paper. In this technique, the endoscopic medial maxillectomy was performed while preserving the head of the inferior turbinate and the nasolacrimal duct ("TuNa-saving"). This technique allowed for good visualization of the maxillary sinus, good oncological control and a reduction in the rate of complications.

A novel device for intraoperative cauterization of bleeding points in endoscopic sinus surgery.

Hemostasis is a critical point in endoscopic sinus and skull base surgery. A variety of techniques are presently available for reducing intraoperative bleeding; however, several limitations of the classical instruments should be stated. For example, reaching bleeding points in an anatomically angled site with straightforward bipolar devices could be quite difficult. With the aim of solving this problem, we developed a simple system using a standard curved suction tube, a rubber catheter and a monopolar system. This device provides an integrated suction function and is able to reach all paranasal and skull base areas, making it extremely useful in gaining precise access to the site of bleeding while providing excellent endoscopic vision. The described monopolar suction tube has proven to be a valid instrument for intraoperative hemostasis in endoscopic procedures; moreover, it does not add any further cost, making it applicable in particular healthcare settings, such as those in developing countries.

Fluorescein-guided intraoperative endoscopy in patients with hereditary hemorrhagic telangiectasia: first impressions.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disease that results in mucocutaneous telangiectasias and arteriovenous visceral malformations. Nasal telangiectasias lead to recurrent epistaxis, which affects up to 96% of patients. Different morphologic classifications and methods of visualization of nasal lesions have been described in the literature. We developed a new method of intraoperative endoscopy based on the intravenous administration of fluorescein. Preliminary data of this technique are reported. METHODS: After the intravenous administration of sodium fluorescein, an intraoperative fluorescein-guided endoscopy was carried out using photographic customized yellow filters on top of a 0-degree, 4-mm endoscope. RESULTS: In 2015, 65 HHT patients underwent surgery for their epistaxis in our institution, and in 7 patients (3 males, 4 females; mean age, 54 years) an intraoperative fluorescein-guided intraoperative nasal endoscopy was performed. No adverse events or complications were observed. CONCLUSION: First impressions regarding the usage of this technique in HHT patients seem to be promising and positive in terms of efficacy and safety. However, further studies with larger cohorts of patients should be performed in order to better investigate the use of this method for diagnostic and surgical purposes in HHT.