Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity.

OBJECTIVE: This paper highlights the management of 5 patients affected by symptomatic ecchordosis physaliphora (EP), treated via endoscopic endonasal transsphenoidal-transclival approach and contextual multilayer skull base reconstruction. A detailed analysis of each case is provided, along with the review of the current body of literature. METHODS: A retrospective review of patients treated by means of endoscopic endonasal approach for EP from 2010 to 2020 in the Otolaryngology and Neurosurgery Departments of a tertiary-care referral center for endoscopic skull base surgery was analyzed. Only adult patients with a definitive histopathological and immunohistochemical diagnosis of EP were included in the study. A systematic literature review according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed for EP. RESULTS: Five cases of EP were retrieved and included in the study. Four patients presented with CSF leakage: in two cases after minor head trauma, in one case with associated bacterial meningitis, and in one case as only referred symptom. One patient complained diplopia due to VI cranial nerve palsy. No complications or recurrences of the disease were observed after a median follow-up of 37.2 months (range, 18-72 months). A total of 27 studies were identified with the systematic literature review, encompassing 30 patients affected by symptomatic EP who were addressed to surgical treatment. Twenty-five patients underwent complete surgical removal of the EP, while in 5 cases, only subtotal resection was performed. CONCLUSIONS: EP might result in a "locus minoris resistentiae" of the skull base, predisposing, in rare cases, to CSF leakage and meningitis, spontaneously or after minor trauma. In cases of symptomatic EP, endoscopic endonasal transsphenoidal-transclival approach represents a safe and effective technique for both EP resection and contextual skull base reconstruction.

Modified Posterior Pedicle Middle Turbinate Flap: An Additional Option for Skull Base Resurfacing.

OBJECTIVES/HYPOTHESIS: Although the Hadad-Bassagusteguy flap represents the first choice for middle and posterior skull base reconstruction and coverage of exposed bony areas, in some cases it is unavailable. The aim of this study is to describe, as an alternative option in selected cases, a modified posterior pedicle middle turbinate flap (mPPMTF) extended to the lacrimal area. Anatomical features, step-by-step harvesting technique, and surgical applications are presented. STUDY DESIGN: Anatomic dissection study and case report. METHODS: Four mPPMTFs were raised in two fresh-frozen cadaver heads. A study of the vascular supply and measurements of length, width, and area of the flap were performed. The ability of the flap to cover the ventral skull base, particularly the upper clivus area, was tested. A clinical case in which an mPPMTF was used for clivus resurfacing after osteoradionecrosis is reported. RESULTS: The vascular supply of the mPPMTF was identified as the middle turbinate branch of the sphenopalatine artery. The flap had a mean length of 6.92 cm, mean maximum width of 1.08 cm, and mean total area of 5.33 cm2 . The flap was able to reach the upper clivus, with a clival coverage ratio of 70.66%. In the clinical case, good surgical outcomes were observed, with accelerated re-epithelization without complications. CONCLUSIONS: The mPPMTF represents an alternative to the Hadad-Bassagusteguy flap for posterior cranial fossa and nasopharynx resurfacing. The main drawbacks are its technically demanding and time-consuming harvesting. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E767-E774, 2021.

Identification of OTX1 and OTX2 As Two Possible Molecular Markers for Sinonasal Carcinomas and Olfactory Neuroblastomas.

OTX homeobox (HB) genes are expressed during embryonic morphogenesis and during the development of olfactory epithelium in adult organisms. Mutations occurring in these genes are often related to tumorigenesis in human. No data are available today regarding the possible correlation between OTX genes and tumors of the nasal cavity. The aim of this work is to understand if OTX1 and OTX2 can be considered as molecular markers in the development of nasal tumors. We selected nasal and sinonasal adenocarcinomas to investigate the expression of OTX1 and OTX2 genes through immunohistochemical and real-time PCR analyses.Both OTX1 and OTX2 were absent in all the samples of sinonasal Intestinal-Type Adenocarcinomas (ITACs). OTX1 mRNA was identified only in Non-Intestinal Type Adenocarcinomas (NITACs) while OTX2 mRNA was expressed only in Olfactory Neuroblastomas (ONs). We have demonstrated that the differential gene expression for both OTX1 and OTX2 genes might be a useful molecular marker to distinguish the different types of sinonasal tumors.

Endoscopic endonasal resection of adenoid cystic carcinoma of the sinonasal tract and skull base.

OBJECTIVES: Adenoid cystic carcinoma (ACC) is a locally aggressive salivary gland malignancy prone to perineural invasion and local recurrences. In the literature, few data exist to guide treatment when this tumor involves the paranasal sinuses and skull base. We report our experience in the management of sinonasal adenoid cystic carcinoma through an endoscopic endonasal approach. METHODS: Retrospective analysis of patients affected by sinonasal ACC treated through an endoscopic endonasal approach from 1997 to 2015, managed at the Universities of Varese and Brescia, Italy. RESULTS: Thirty-four patients were included in the analysis. The ethmoid sinus (55.9%), nasal septum (17.7%), maxillary sinus (11.7%), and sphenoid sinus (5.9%) were the primary tumor sites encountered. Twenty patients (58.8%) presented with T3 or T4, without any systemic spreading. Twenty-nine patients underwent endoscopic transnasal resection, whereas the involvement of the anterior skull base in five cases required a transnasal endoscopic craniectomy. Overall, 20 of 34 (58.8%) patients received some form of adjuvant radiotherapy. The follow-up ranged from 12 to 202 months (mean of 73.2 months). The 5-year overall, disease-specific, and recurrence-free survival rates were 86.5% ± 7.39%, 86.5% ± 7.39%, and 71.8% ± 8.67%, respectively. CONCLUSIONS: The endoscopic approach is safe and effective for selected sinonasal ACC, reducing the comorbidities of the external approaches while producing similar oncological results. High T-stage, grade III histology, positive surgical margins, and perineural infiltration all have an important negative prognostic value. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:1071-1077, 2019.

Sinonasal Non-Intestinal-Type Adenocarcinoma: A Retrospective Review of 22 Patients.

OBJECTIVE: To analyze outcomes and prognostic factors of sinonasal nonsalivary non-intestinal-type adenocarcinoma (n-ITAC.) METHODS: A retrospective review of 22 consecutive patients with n-ITAC was performed. RESULTS: Average follow-up time was 77 months. The 5-year overall survival and disease-specific survival were 95.2%. The 5-year overall survival and disease-specific survival were 100% for pT1, pT2, and pT3 tumors and 83.3% for pT4a and pT4b tumors; 100% for G1 tumors and 87.5% for G3 tumors; and 100% for tumors with negative surgical margin and 50% for tumors with positive surgical margin. Stage, grade, and surgical margins were independent prognostic factors. Adjuvant radiotherapy was performed for high-grade and high-stage tumors. CONCLUSIONS: Surgery followed by radiotherapy has remained a mainstay for management of n-ITAC, and the endoscopic transnasal approach, when correctly planned and indicated, is the surgery of choice. Adjuvant radiotherapy is recommended in cases of high-stage (T3 and T4) and high-grade tumors. n-ITAC is associated with a favorable outcome. High grade, pT4 stage, and positive surgical margins are independent negative prognostic factors.

Endoscopic endonasal approach for sinonasal and anterior skull base malignancies in the elderly.

BACKGROUND: The purpose of this study was to report the outcomes of endoscopic transnasal resection for sinonasal and anterior skull-base cancers in elderly patients. METHODS: A retrospective review was performed. The patients were divided into 2 groups, <70 years old and ≥70 years old and compared by univariate analysis. Prognostic factors were evaluated with a multivariate analysis. Survival rates were also calculated. RESULTS: Two hundred three elderly patients and 397 younger patients were enrolled in this study. The elderly patients reported lower survival rates than the younger patients. When melanoma and esthesioneuroblastoma were censored, the disease-specific survival (DSS) and recurrence-free survival (RFS) were similar. Complication rates were 17.5% without any statistical significance between the groups. Multivariate analysis revealed that histology, stage, surgical margins, and surgical approaches were independent predictors of survival in elderly patients. CONCLUSION: The endoscopic transnasal approach reported low mortality and morbidity rates also in geriatric patients, and age itself is not to be considered as a contraindication.

OTX1 and OTX2 as possible molecular markers of sinonasal carcinomas and olfactory neuroblastomas.

OTX Homeobox genes are involved in embryonic morphogenesis and in the development of olfactory epithelium in adult. Mutations occurring in the OTX genes are reported to be associated to tumorigenisis in human. No reports correlate the expression of OTX genes and neoplasms of the nasal cavity. Thus, through immunohistochemical and Real-time PCR analysis we investigated OTX1 and OTX2 expression in the more frequent types of nasal and sinonasal tumours. Variable expression of both genes were found in normal sinonasal mucosa and in tumours. Interestingly, no expression of both OTX genes were detected in sinonasal intestinal-type adenocarcinomas; only OTX1 was found in non-intestinal-type adenocarcinomas and OTX2 was selectively expressed in olfactory neuroblastomas. In conclusion, OTX1 and OTX2 genes might have a role in the pathogenesis of different types of sinonasal neoplasms.

Role of endoscopic surgery in the management of sinonasal and skull base schwannomas.

BACKGROUND: The purpose of this study was to report our experience with the endoscopic management of sinonasal schwannomas, analyzing the advantages, limitations, and outcomes of the technique. METHODS: A retrospective analysis was carried out on 11 patients treated endoscopically between 2000 and 2014 at a single institution. RESULTS: Eight patients underwent an exclusive endoscopic endonasal approach, whereas, in 3 patients, an osteoplastic flap was combined because of massive or lateral frontal sinus involvement. The tumor extended into the orbit in 5 cases, and involved the skull base in 5 patients who required a concomitant endoscopic duraplasty. No evidence of disease was observed in 10 patients after a mean follow-up of 90.1 months (range, 14-189 months). One patient was alive with persistence of disease, although asymptomatic. CONCLUSION: The endoscopic endonasal approach is a valid alternative for the vast majority of sinonasal schwannomas with minimal morbidity for the patient. © 2016 Wiley Periodicals, Inc. Head Neck 38: E2074-E2082, 2016.

Endoscopic endonasal orbital transposition to expand the frontal sinus approaches.

BACKGROUND: The management of frontal sinus disease still remains a challenging issue for rhinologists. Although endoscopic frontal sinus surgery has significantly advanced over the past years, there are some conditions in which the external approaches are still necessary. Recently, the increasing experience in endoscopic sinus surgery together with important technologic advancements have allowed expansion of the indications for selected lesions localized more and more lateral into the frontal sinus. OBJECTIVE: The aim of this study was to describe the endoscopic endonasal approach to the far lateral portion of the frontal sinus and to evaluate the outcomes of this technique. METHODS: A retrospective evaluation was carried out with patients at a single institution, who, from 2004 to 2012, underwent an exclusive endoscopic transnasal approach to the far lateral portion of the frontal sinus at a single institution. The surgical technique included the Draf IIb procedure, a modified Lothrop procedure (Draf III), and endoscopic lateralization of the orbit, differently associated according to the needs of each case. RESULTS: Twenty-four patients were eligible for the present study. The lesions included were mucoceles in 9 cases, fibro-osseous lesions in 10 cases, and inverted papillomas in 5 cases. The overall follow-up ranged from 12 to 120 months (mean, 40.6 months). At the time of the last follow-up, no recurrence of disease was observed in the patients treated; in one case of osteoma, a small and stable in size (1 mm) persistent lesion was observed in an asymptomatic patient. One scheduled and one accidental cerebrospinal fluid leak with immediate skull base reconstruction occurred. No intraoperative or postoperative early or late orbital complication was observed in any case. CONCLUSION: The endoscopic endonasal orbital transposition seems to facilitate the endoscopic management of selected far-lateral frontal sinus lesions. However, it is a demanding procedure that requires experience and skill to avoid intraoperative skull base and orbital complications.

Bone regeneration after sinonasal mucocele marsupialization: What really happens over time?

OBJECTIVE: To evaluate the necessity of reconstructing the eroded bony boundaries after mucocele marsupialization when the mucoperiosteum has been spared. STUDY DESIGN: Retrospective review of 308 patients treated for a sinonasal mucocele. Of these, 116 showed areas of bone reabsorption in their preoperative computed tomography (CT) scan. METHODS: Of 116 patients showing one or more areas of bone reabsorption who underwent marsupialization of the mucocele, whether using a purely endonasal endoscopic approach or a combined approach, the common factor was that the mucoperiosteum of the paranasal sinus had always been spared and the eroded bone had never been reconstructed. After rigorous selection, 12 adult patients were enrolled to undergo a postoperative CT scan in order to verify what had happened to the eroded bone at least 3 years following the surgical marsupialization of the mucocele. RESULTS: In 66,6% of patients, the postoperative CT scan showed complete self-reconstruction of bone that had previously been eroded by the mucocele. No enophthalmus, meningocele, or other facial deformities were noted in our selection group, despite not having undergone surgical reconstruction of the bone. CONCLUSIONS: Even taking into account the small number of patients enrolled in the present study, indications are that there is no need to reconstruct the eroded bone, as would appear from our results that sparing the mucoperiosteum is enough to enable the bone to regenerate. Nevertheless, larger scale studies of the subject are merited.

Endoscopic endonasal resection of respiratory epithelial adenomatoid hamartomas of the sinonasal tract.

BACKGROUND: Respiratory epithelial adenomatoid hamartomas (REAHs) are rare benign tumors and may manifest as either isolated lesions or in association with sinonasal polyposis. The aim of this study is to report our experience in the management of patients with REAH and to analyze the long-term results of the endoscopic endonasal approach. METHODS: A retrospective analysis of a database dedicated to patients with REAH treated between May 2003 and December 2012 was performed. Clinical presentation, demographic, histologic and radiographic features, operative findings, and follow-up data were examined. RESULTS: Twenty-seven patients with REAH, 14 males and 13 females, with a mean age of 51 years, underwent endoscopic sinus surgery (ESS). The most frequent reported symptoms were nasal obstruction (80%), headache (12%), mucous rhinorrhea (20%), and hyposmia (40%). Seventeen cases (first group) were present as isolated masses, 10 cases (second group) were associated with nasal polyposis. The first group with a preoperative diagnosis of REAH was submitted to a more aggressive resection with subperiosteal dissection and drilling of the underlying bone. The patients in the second group, because of the unrevealed diagnosis of REAH and due to the presence of nasal polyposis, underwent standard ESS. No evidence of recurrence in either of the subgroups after a mean follow-up of 61.2 months. CONCLUSION: REAH is a benign well-defined pathological entity but is still unfamiliar. REAH should be kept in mind as a differential diagnosis from more aggressive lesions to avoid unnecessary surgical procedure. A complete but conservative endoscopic resection appears to be curative.

The endoscopic transnasal paraseptal approach to a sphenoid sinus osteoma: case report and literature review.

Osteomas are the most common benign tumors of the nose and paranasal sinuses. Their symptoms, which are nonspecific, occur as the result of a blocked nasal airflow or, in some rare cases, the involvement of nearby structures. Isolated sphenoid sinus osteomas are very rare, as only 20 cases have been previously reported in the literature. Most authors advise surgical treatment for symptomatic lesions. Surgical access to the sphenoid sinus has traditionally been a challenge for surgeons. We describe an endoscopic transnasal paraseptal resection of a sphenoid osteoma in a 35-year-old man. We also discuss surgical access and review the evolution of the surgical approaches to the sphenoid sinus.

A rare localization of actinomycosis mimicking ulcerative malignancy.

Actinomycosis is a chronic, suppurative, and granulomatous process caused by Actinomycetes, saprophytic bacteria normally residing in the oral cavity. It can involve any organ, but the cervicofacial disease is the most frequent. Pharyngolayngeal involvement is rare and usually occurs secondary to the oral or cervical disease. There are few cases of primary pharyngolaringeal actinomycosis described in the literature. A rare case of pharyngeal actinomycosis mimicking an ulcerative malignancy in a 63-year-old man is reported. The patient was treated successfully with long-term antibiotic therapy. The clinical and pathological features and the aspects of diagnosis and treatment of cervicofacial actinomycosis are discussed.

A novel technique for tailoring frontal osteoplastic flaps using the ENT magnetic navigation system.

CONCLUSION: The ENT magnetic navigation system is potentially useful and offers the most accurate technique for harvesting frontal osteoplastic flaps. It represents a valid tool in the wide range of instruments available to rhinologists. OBJECTIVE: Precise delineation of the boundaries of the frontal sinus is a crucial step when harvesting a frontal osteoplastic flap. We present a novel technique using the ENT magnetic navigation system. METHODS: Nineteen patients affected by different pathologies involving the frontal sinus underwent an osteoplastic flap procedure using the ENT magnetic navigation system between January 2009 and April 2011. RESULTS: The ENT magnetic navigation system was found to be a safe and accurate tool for delineating the frontal sinus boundaries. No intraoperative complications occurred during the osteoplastic procedures.

Endoscopic transnasal craniectomy in the management of selected sinonasal malignancies.

BACKGROUND: Because of a better understanding of the anatomy from an endoscopic perspective, the acquisition of surgical experience, and concomitant technological advances, endoscopic resection of the anterior skull base (ASB) and overlying dura has now become a reality, opening new possibilities in the management of sinonasal malignancies. Here, the authors review a series of 62 patients, the largest reported to date, who underwent endoscopic transnasal craniectomy (ETC) and endoscopic dural repair for the management of selected sinonasal malignancies. Special emphasis is placed on the surgical technique, technical tricks, choice of materials for endoscopic dural repair, postoperative management, and complications. METHODS: From 2004, 62 patients underwent ETC at two referral hospitals, which extended anteroposteriorly from the frontal sinus to planum sphenoidale and laterolaterally from the nasal septum to the lamina papyracea (unilateral resection, n = 28; 45%) or from papyracea to papyracea (bilateral resection, n = 34; 55%). Duraplasty with a three-layer technique was performed using the iliotibial tract and fat tissue. RESULTS: The most frequent histotypes were adenocarcinoma (58%) and olfactory neuroblastoma (22%). Forty-five (73%) patients were previously untreated. The incidence of early (T1-2, Kadish A-B) and advanced (T3-4, Kadish C) tumors was similar. The complication rate was 15%, mostly cerebrospinal fluid leaks (13%). Its prevalence did not correlate with patient age, medical comorbidities, previous treatment, presence of ASB involvement, or whether ETC was mono- or bilateral, but tended to correlate with advanced tumor stage, dural involvement, and the period of treatment. After a mean follow-up of 17.5 months (range, 1-54 months), 58 (94%) patients had no evidence of disease. CONCLUSION: In correctly selected patients with sinonasal tumors involving the ASB, ETC offers a less invasive alternative than resection by an open approach with an acceptable morbidity.

Endoscopic endonasal management of encephaloceles in children: an eight-year experience.

OBJECTIVE: Skull base encephaloceles are difficult to diagnose and to treat. Traditionally, they are approached externally via craniotomic routes. Endoscopic management of skull base defects is the standard treatment in adults. Our aim is to evaluate the efficacy and safety of endoscopic endonasal repair of basal encephaloceles in the paediatric population. METHODS: Retrospective evaluation of paediatric encephaloceles managed endoscopically at a tertiary referral centre. RESULTS: Eleven subjects fitted the criteria of the study. Mean age at surgery was 6.1+/-4.6 SD years, while mean follow-up was 46.5+/-28 SD months. Most of the defects were located in the anterior cranial fossa. All patients were managed successfully with no complications. CONCLUSIONS: Endonasal endoscopic management of congenital encephaloceles is feasible in paediatric patients. Meticulous pre-op neuroimaging is of paramount importance when dealing with such cases.

Endonasal endoscopic repair of Sternberg's canal cerebrospinal fluid leaks.

OBJECTIVES: Management of cerebrospinal fluid leaks or encephaloceles of Sternberg's canal is challenging. Transnasal visualization of this area is difficult, especially when large pneumatization is present. External approaches to this region involve aggressive surgery and are often associated with significant morbidity. The aim of the study was to assess the real effectiveness of an endoscopic endonasal approach for treating cerebrospinal fluid leaks of the lateral recess of the sphenoid sinus. STUDY DESIGN: The authors conducted a retrospective evaluation. METHODS: Clinical charts of patients with cerebrospinal fluid leaks and/or encephaloceles of Sternberg's canal treated at our institution were retrospectively reviewed. All these patients were managed with an endonasal endoscopic procedure. RESULTS: Fifteen patients (9 female and 6 male) were included in this study. Nine patients underwent a transethmoidal-pterygoidal-sphenoidal approach with a multilayer reconstructive technique. No cerebrospinal fluid leak recurrences were observed during follow up (mean follow up 37.6 +/- 21.7 standard deviation months) CONCLUSIONS: The transethmoidal-pterygoidal-sphenoidal approach provides a wide, safe, and direct route to the lateral recess of the sphenoid sinus. Multilayered reconstruction of the skull base defects must be considered the first option for this kind of lesion.

Endonasal endoscopic duraplasty: our experience.

OBJECTIVE: Endoscopic repair of cerebrospinal fluid (CSF) leaks is a recognized technique. We consider our experience and evaluate the outcomes in patients who underwent endoscopic repair of CSF leaks. METHODS: A retrospective case note review of 135 patients who underwent anterior cranial repairs of CSF leaks between August 1995 and December 2004 at a tertiary referral center. We describe the technical details and outcomes of care by purely endoscopic procedures. RESULTS: Thirteen patients had combined transcranial and endonasal repairs and 122 patients had their repairs using an endoscopic approach only. There were 64 males and 71 females with ages that ranged from 1 to 75 years (mean age 42 years, median age 44 years). The success rate for first attempt only was 93.4%. Eight of the 122 patients (6.6%) needed a second surgical repair. In one patient a bicoronal approach was necessary while in the other cases a revision endoscopic procedure was appropriate. The period of follow-up ranged from 2 months to 9 years (mean 5 years, median 39 months). CONCLUSIONS: Our experience confirmed that endoscopic surgery is an effective and safe method of treatment for most CSF leaks. A variety of different endoscopic techniques allowed CSF leaks to be repaired in almost every site of the anterior skull base with very few exceptions.