Esthesioneuroblastoma: an update on the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy.

Objectives To update the Massachusetts General Hospital (MGH) and Massachusetts Eye and Ear Infirmary (MEEI) experience in the management of esthesioneuroblastoma (ENB) with multimodality therapy and to reassess treatment outcomes and complications in a larger cohort with longer follow-up times. Design A retrospective chart review. Setting A tertiary referral center. Participants All patients presenting with ENB and managed at the MGH and MEEI from 1997 to 2013. Main Outcome Measures Disease-free and overall survival. Results Twenty-two patients were identified with an average follow-up of 73 months. Ten patients presented with Kadish stage B disease and 12 with stage C disease. A total of six patients (27%) developed regional metastases. Treatment for all patients included craniofacial resection (CFR) followed by proton beam irradiation with or without chemotherapy. The 5-year disease-free and overall survival rates were 86.4% and 95.2%, respectively, by Kaplan-Meier analysis. Negative margins were a significant factor in disease-free survival. One patient experienced severe late-radiation toxicity. Conclusions ENB is safely and effectively treated with CFR followed by proton beam irradiation. The high incidence of regional metastases warrants strong consideration for elective neck irradiation. Proton beam radiation is associated with lower rates of severe late-radiation toxicity than conventional radiotherapy.

Anteriorly based pedicled flaps for skull base reconstruction.

The expansion of endoscopic skull base surgery has resulted in the creation of large defects that must be repaired to ensure separation of the cranial vault from the nasal cavity. The workhorse of anterior skull base reconstruction remains the nasoseptal or Hadad-Bassagasteguy flap. Despite its success, the nasoseptal flap is limited in its ability to reach extremely anterior defects including those involving the frontal break, posterior frontal table, and anterior cribiform plate. Alternative approaches utilizing anteriorly pedicled flaps have been described which exploit the vascular supply to the anterior septum and lateral nasal wall. The diminutive nature of the anterior septal blood supply has led to the elaboration of a bipedicled anterior septal flap which is capable of reliable reconstruction of both the frontal beak as well as posterior frontal table defects. Similarly anteriorly based inferior turbinate flaps pedicled largely on anterior ethmoid arborizations have been used successfully to reconstruct anterior defects. In light of the limitations of the nasoseptal flap, the addition of anteriorly pedicled flaps to the clinical armamentarium offers the opportunity to provide vascularized mucosal coverage of virtually any region of the skull base which can be reached endoscopically.

Novel techniques and the future of skull base reconstruction.

The field of endoscopic skull base surgery has evolved considerably in recent years fueled largely by advances in both imaging and instrumentation. While the indications for these approaches continue to be extended, the ability to reconstruct the resultant defects has emerged as a rate-limiting obstacle. Postoperative failures with current multilayer grafting techniques remain significant and may increase as the indications for endoscopic resections continue to expand. Laser tissue welding represents a novel method of wound repair in which laser energy is applied to a chromophore doped biologic solder at the wound edge to create a laser weld (fig. 1). These repairs are capable of withstanding forces far exceeding those exerted by intracranial pressure with negligible collateral thermal tissue injury. Recent clinical trials have demonstrated the safety and feasibility of endoscopic laser welding while exposing the limitations of first generation hyaluronic acid based solders. Novel supersaturated gel based solders are currently being tested in clinical trials and appear to possess significantly improved viscoelastic properties. While laser tissue welding remains an experimental technique, continued success with these novel solder formulations may catalyze the widespread adoption of this technique for skull base repair in the near future.