Laser Treatment of Epistaxis and Oral Bleeding in Hereditary Hemorrhagic Telangiectasia

Background: Hereditary hemorrhagic telangiectasia (HHT) is a relatively common, albeit under-recognized autosomal-dominant multisystemic vascular disorder. Epistaxis due to telangiectases in the nasal mucosa is the most common and often the earliest symptom of HHT. As many as 90% of affected individuals eventually experience recurrent epistaxis, with a mean frequency of 18 episodes per month. Prompted by the limitations of invasive treatment, researchers have directed attention to laser photocoagulation as an alternative nonsurgical treatment modality with promising results. Purpose: The purpose of the present study was to describe our experience with the state-of-the-art 980 nm diode laser for the treatment of bleeding lesions of the skin, nasal and oral mucosa in patients with HHT. Methodology/Principal: We treated 16 HHT patients with intractable bleeding from telangiectasias using the 980-nm diode laser as an office procedure using local anesthesia. We recorded hemoglobin levels before and after treatment and used disability questionnaires. Results: All patients treated had an improvement in hemoglobin levels and disability scores. In patients with multiple lesions hemoglobin levels improved from a mean of 8.4 to 11.2 (p = 0.008). The disability index in this group improved from a mean of 5.3 to 2.8 (p = 0.007). Follow-up ranged between 4 and 12 months. Conclusions: The 980-nm diode laser is a good office based solution for bleeding in HHT.

Skull base chordomas review of current treatment paradigms

Background:Chordomas are locally invasive neoplasms,arising from notochordal remnants and can appear anywhere along the axial skeleton.Local recurrences are common,and distant metastases may occur years after the initial presentation.Methods:Literature review of current treatment strategies for chordomas of the skull base.Results:Surgery is the mainstay of treatment and complete resection has paramount importance for prognosis.When complete resection is not achieved recurrent disease is common.The anatomical complexity of the skull base makes resection complex.Endonasal endoscopic approaches to the clivus has become increasingly favored in recent years although addressing reconstruction of the skull base to prevent CSF leak may be challenging.Evidence suggests that radiotherapy should not be considered as a primary single modality when trying to achieve cure of the disease.Nonetheless,immediate post-operative radiotherapy improves survival.Many strategies have been suggested to preserve sensitive vital structures in the skull base during treatment but as for survival there is no evidence of advantage when comparing adjuvant therapy with photon radiotherapy,gamma knife surgery,proton beam therapy,and carbon ion radiation therapy.There is no evidence to support cytotoxic chemotherapy in the treatment of chordomas but targeted therapies have started to show promise.Several optional molecular targets exist.Brachyury is overexpressed in 95%of chordomas but not in other mesenchymal neoplasms.However,its precise role in chordoma pathogenesis is currently unclear,and its cellular location in the nucleus makes it difficult to target.The inhibition of brachyury in chordoma cell lines induces growth arrest and apoptosis.This does not have clinical application to date.There are retrospective results with different molecular targeted therapies for advanced chordomas with some effectiveness.Conclusion:Despite improvements made in the past 10 years in our knowledge of chordoma biology,available therapies still offer a limited benefit.There is an unmet need for new therapeutic options for patients with advanced disease.Therefore,patients with advanced disease should be encouraged to participate in clinical trials when and where available.