Background/Objectives: The endoscopic transsphenoidal approach constitutes an excellent technique for adrenocorticotropin hormone (ACTH)-producing pituitary tumours. It is associated with subnormal postoperative serum cortisol levels, which may guide decisions regarding immediate re-operation. Methods: The authors retrospectively reviewed patients with Cushing's disease who had undergone endoscopic transsphenoidal surgery between 2013 and 2023. All operations were performed by neurosurgeons and skull-base otolaryngologists. Surgical outcomes were evaluated in combination with prognostic factors such as cortisol and ACTH levels in terms of long-term remission and late recurrence rates of Cushing's disease. Results: Fifty patients aged between 15 and 69 (average 37.8) years were evaluated, having undergone 50 operations. The median follow-up was 76.5 months (range: 23-122 months). Major complications with a transient CSF leak resulting from the surgical approach occurred in three patients. Two patients in the series experienced minor complications, developing a deep vein thrombosis, and thirteen patients developed transient diabetes insipidus. The initial remission rate was 84% (n = 42/50). Initial non-remission occurred in eight (8) patients (16%), with three macro- and five microadenomas. A total of 3 of the 42 patients with initial remission had a late recurrence after 50 months follow-up and required repeat transsphenoidal surgery. Seven patients (16.6%) who did not exhibit early postoperative cortisol reduction subsequently achieved remission. Male gender was the only factor that was significantly associated with lower remission rates in either short- or long-term follow-up (p = 0.003 and 0.038, respectively). An immediate postoperative ACTH nadir of ≤5 pg/mL was significantly related to long-term remission (p = 0.004). In our study, a significant correlation was confirmed between remission of the disease and 24 h urinary cortisol values, both early and late (p = 0.019), and serum cortisol <138 nmol/L. In this retrospective study from a single institution specialising in pituitary tumour management, the endoscopic transsphenoidal approach was shown to be both safe and effective. Additionally, we found that the risk of relapse in patients with Cushing's disease persisting for more than 5 years after surgery is real but low. Moreover, failure to achieve an early postoperative cortisol reduction does not preclude a subsequent remission. Conclusions: Our findings demonstrate that ACTH, postoperative serum cortisol, and urinary free cortisol are valuable predictors of relapse over a five-year period and are closely correlated to each other.

Background. Transsphenoidal surgery is the treatment of choice for Cushing’s disease. Successful surgery is associated with subnormal postoperative serum cortisol concentrations and cortisoluria levels, which may guide decisions regarding immediate reoperation. Remission is defined as the biochemical reversal of hypercortisolism with the re-emergence of diurnal circadian rhythm. Methods. A single-center prospective cohort study was conducted among thirty-three patients who underwent transsphenoidal pituitary surgery for Cushing’s disease. Postoperative surgical outcomes, daily morning cortisolemia, and 24 h urinary-free cortisol from the first to the fifth morning were evaluated. Results. All patients underwent surgery, with a remission rate of 81.2%. Of the 26 patients who achieved early remission, 92% remained in remission. Two patients (7.7%) showed recurrence of Cushing’s disease during a mean follow-up of 81.7 months. Early postoperative hypocortisolism suggests complete removal of the tumor, correlating with high rates of remission (p < 0.001). Also, in 12.5% of patients with early cortisol values >138 nmol/L, there was a gradual late remission. Conclusions. In our cohort of patients, the endoscopic transsphenoidal approach was safe and effective in the treatment of Cushing’s disease. We demonstrated that serum and urinary cortisol concentrations did not experience significant fluctuations from the first to the fifth day. This constitutes an accurate predictor of durable remission, comprising a distinctive finding in the intermediate term by our team.

OBJECTIVE:Endoscopic ear surgery is no longer a promising technique, but a well-established one. This study aims to compare endoscopic and microscopic tympanoplasty based on current literature evidence, in terms of their efficacy and safety characteristics. DATA SOURCES:We conducted a systematic literature search of four medical databases (Pubmed, Cochrane Library, Scopus, ClinicalTrials.gov), focusing on randomized controlled or observational studies comparing microscopic to endoscopic tympanoplasty. REVIEW METHODS:Data related to the efficacy and safety of each technique were extracted. Outcome data were summarized using pooled mean differences or pooled odds ratio along with their 95% confidence intervals. The risk of bias was estimated, by using the ROBINS-I and RoB-II assessment tools, while the overall quality of evidence was evaluated according to the GRADE working group. RESULTS:Thirty-three studies, with 2646 patients in total, were included in the meta-analysis. Success rate was evaluated by estimating tympanic graft failure (pooled mean difference:-0.23; 95% CI: -0.61, 0.14, I2 = 33.42%), and air-bone gap improvement (pooled mean difference:-0.05; 95% CI:-0.23, 0.13, I2 = 52.69%), resulting in comparable outcomes for the two techniques. A statistically significant difference favoring the endoscopic technique was detected regarding postoperative wound infection (OR: -1.72; 95% CI: -3.39, -0.04, I2 = 0%), dysgeusia (OR: -1.47; 95% CI: -2.47, -0.47, I2 = 0%), otitis externa development (OR: -1.96; 95% CI: -3.23, -0.69, I2 = 0%), auricular numbness (OR: -2.56; 95% CI: -3.93, -1.19, I2 = 0%), as well as surgical duration (OR: -1.86; 95% CI: -2.70, -1.02, I2 = 43.95%), when compared to the postauricular microscopic approach. CONCLUSION:Endoscopic tympanoplasty is an innovative alternative to the microscopic technique, resulting in commensurate outcomes regarding success rate. Furthermore, it offers superior results concerning postoperative complications, while it presents a significant reduction in the duration of surgery, mainly when it is compared to the postauricular microscopic approach. LEVEL OF EVIDENCE:NA Laryngoscope, 134:3466-3476, 2024.

Glomus tympanicum are benign tumors of vascular origin, arising from the neural crest cells and located on promontory. The treatment of choice is surgical excision of the lesion. Traditionally, it is performed under microscopic observation. With the introduction of endoscopes in the field of ear surgeries, an endoscopic approach has also evolved. Herein, we present case reports of three patients diagnosed with glomus tympanicum tumors who were operated on using an endoscopic approach. A review of the literature is also performed. The mass was completely excised in all patients, and there were no signs of recurrence at the follow-up at least a year later. Endoscopic ear surgery is a safe and effective method of managing glomus tympanicum tumors. Its main limitation is the tumor size; however, in most cases, tumors of stages I to II as per the Glasscock-Jackson classification and types A1 to B1 according to the modified Fisch-Mattox classification can be completely removed endoscopically. Careful preoperative selection of patients warrants the best outcomes.

Objectives : Primary : To determine the rate of occult cervical metastasis in primary temporal bone squamous cell carcinomas (TBSSC). Secondary : to perform a subgroup meta-analysis of the risk of occult metastasis based on the clinical stage of the tumour and its risk based on corresponding levels of the neck Design : A systematic review and meta-analysis of papers searched through Medline, Cochrane, Embase, Scopus and Web of Science up to January 2021 to determine the pooled rate of occult lymph node/parotid metastases. Quality assessment of the included studies was assessed through the Newcastle-Ottawa scale. Setting : Centres around the world that perform surgery for TBSCC Participants : Patients with TBSCC Results : Overall, 9 out of 1034 screened studies met the inclusion criteria, for a total of 907 patients of which 388 had TBSCC. Out of the 191 patients who underwent a neck dissection, 21 had positive lymph nodes giving a pooled rate of occult metastases of 11% (95% CI: 7%-17%). When analysed using the Modified Pittsburg staging system, 21 pT2 cases had a pooled occult metastases rate of 3% (95% CI: 0%-21%), 27 pT3 cases had a pooled occult metastases rate of 12% (95% CI: 1%-60%), and 65 pT4 cases had a pooled occult metastases rate of 14% (95% CI: 7%-25%). Data available showed that most of the positive nodes were in Level II. Conclusion: The rate of occult cervical metastases in TBSCC increases based on the tumour (T) staging of the disease with majority of nodal disease found in level 2 of the neck.

Abstract Background: To report surgical outcomes of transsphenoidal surgery as first-line treatment and predictors of remission in a specialist centre for patients with Cushing’s disease and to investigate long-term remission and delayed recurrence. Methods: Clinical data was obtained from a retrospective CD database while all electronic medical, laboratory and surgical patient records between 2012 and 2022 with a minimum 23 months’ follow-up, were evaluated. Result: In this single-centre study a total of 50 consecutive patients with CD (34F, 16M) underwent TSS. Median (range) age and follow-up were 37.8 (15-69) and 76.5 (23-122) months respectively. The initial remission rate was 84% (n = 42/50). Initial non-remission occurred in 8 patients (16%), with three macro- and five microadenomas. Three (2 micro, 1 macro) of the 42 patients with initial remission had late (>50 months) recurrence of hypercortisolism that required repeat TSS. Seven patients (16.6%) with non-reduced postoperative cortisol levels showed a subsequent remission. Male gender was the only factor significantly associated with lower remission rates in either short- or long-term follow-up (p = 0.003 and 0.038 respectively).Conclusions: A team-based approach, in a specialized pituitary centre, can lead to initial and durable long-term remission in patients with CD. Additionally, we found that the risk of relapse in patients with CD persisting for more than 5 years after surgery is real but low. Moreover, non-initially reduced postoperative cortisol levels do not preclude a subsequent remission. Lifelong follow-up for patients with CD appears essential, for all patients who have shown recovery of their axis relationship.

Objectives Endoscopic approaches constitute a newly introduced and promising technique in the field of stapes surgery, presenting favorable outcomes, so far. This study aims to compare endoscopic and microscopic stapes surgery based on current literature evidence, in terms of their efficacy and safety characteristics. Design We conducted a systematic literature search of three medical databases (Pubmed, Cochrane Library, and Scopus). We focused on randomized controlled studies or observational studies comparing microscopic to endoscopic stapes surgery. Data related to the efficacy and safety of each technique were extracted. Outcome data were summarized using the pooled mean differences or pooled odds ratio along with their 95% confidence intervals, according to the available data. The quality of evidence was assessed according to the GRADE recommendations. Results Thirteen studies with 705 patients were included in the meta-analysis. Success rate was evaluated by estimating air-bone gap improvement, resulting in comparable outcomes for the two techniques (mean difference: -0.20; 95% CI: -0.53, 0.14). No statistically significant difference was detected concerning postoperative complications, except for dysgeusia that was in favor of the endoscopic approach (OR: -1.46; 95% CI: -2.45, -.047). The overall quality of evidence was assessed to range from “Low” to “Very Low”. Conclusion Endoscopic stapes surgery is an innovative alternative to the microscopic technique, resulting in commensurate outcomes in terms of success rate and complications. Further high-quality studies are needed, to adequately compare the two approaches, particularly in terms of operation time, learning curve, cost-effectiveness, and otology surgical skills acquisition.

Epiphora is the first symptom of acquired nasolacrimal duct obstruction (NLDO), which may be due to various causes, including paranasal sinuses and tumours of the nasal cavity. A 28-year-old male patient presented chronic dacryocystitis with left-sided epiphora. Endoscopically, a bulging of the lateral nasal wall at the left-sided agger nasi area was noted. Imaging studies (CT and MRI) revealed a left-sided giant agger nasi mucocele. An endonasal endoscopic dacryocystorhinostomy (DCR) as well as opening of the agger nasi cell with mucocele removal and anterior ethmoidectomy was performed. Based on the literature, intranasal causes have very often occurred in patients with chronic dacryocystitis, but not reported as the cause of inflammation in the mucocele of agger nasi. Identification and recognition of this endonasal rare cause of NLDO-like agger nasi mucocele entity may facilitate timely intervention and can be treated successfully with a simultaneous endoscopic DCR procedure.

Frontal sinus mucoceles are mucous secretory lesions within the sinus, presenting a slow – growing pattern, mostly behaving like a space-occupying mass with subtle symptoms until a bone erosion of the surrounding structures takes place. Intracranial and intraorbital expansion are regarded as the most considerable complications, demanding adequate surgical management in order to avoid recurrence. Endoscopic drainage is thought to be the current treatment of choice, with external approaches or combined approaches being considered for more severe cases where anatomy and disease extension restricts adequate endoscopic visualization of the lesion. We present a case of a 31-year-old female patient with a left frontal sinus mucocele, demonstrating with an unusual lateral frontal expansion and orbit invasion, causing exophthalmos and ophthalmoplegia. She was adequately managed by a Draf III endoscopic procedure achieving wide marsupialization and enough drainage of the mucocele. No sign of recurrence was noticed during the last 6-month follow-up. Giant mucoceles of the frontal sinus are a rare pathology. To our knowledge, endoscopic endonasal approach is a treatment modality that can provide an adequate drainage pathway, thus resulting in optimal clinical outcomes, compared with external approaches. Keywords: Frontal sinus; Mucocele; Exophalmos; Pneumosinus dilatans; Pneumocele.

INTRODUCTION:Burkholderia cepacia complex (Bcc) is a group of gram-negative bacilli that have rarely been isolated in the ear, nose and throat region in immunocompetent patients. Bcc show resistance to most available antibacterial drugs.CASE REPORT:We present the case of an immunocompetent 31-year-old male reporting a pulsating headache with right supraorbital swelling associated with exophthalmos. A brain CT scan showed an expansive giant cystic lesion occupying the right frontal sinus, extending to the anterior cranial fossa. Management and outcome: drainage with the resecting of the floor of the frontal sinus from the orbital plate of the ethmoid bone to the nasal septum (Draf IIb) was performed with wide marsupialization of the mucopyocele. Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) analysis was used to identify the isolate. MRI 1 and 12 months after surgery showed complete lesion removal. The patient was followed for 12 months with complete recovery of symptoms.CONCLUSION:Paranasal sinuses disease with cranial expansion and orbital complications constitutes an emergency. For the first time in the literature, Bcc was isolated in the frontal sinus, extending into the anterior cranial fossa, in an immunocompetent patient. An endoscopic surgical approach with microbiological identification and management by appropriate antibacterial drug treatment seems to be the key to success.

Bilateral choanal atresia (CA) is a rare congenital malformation frequently associated with other anomalies. CHARGE association is closely linked to bilateral CA. The aim of this study was to describe the outcomes of the endoscopic repair in bilateral CA, and to assess the role of postoperative nasal stenting in two cohorts of CHARGE-associated and non-syndromic CA. Thirty-nine children were retrospectively analyzed (16 patients had CHARGE-associated CA). The rate of postoperative neochoanal restenosis was 31.3% in the CHARGE population, and 47.8% in the non-syndromic CA cohort. Data on postoperative synechiae and granulation tissue formation, need for endonasal toilette and dilation procedures, and number of procedures per patient were presented. Stent positioning led to a higher number of postoperative dilation procedures per patient in the non-syndromic cohort (p = 0.018), and to a higher rate of restenosis both in the CHARGE-associated, and non-syndromic CA populations. Children with CHARGE-associated and non-syndromic bilateral CA benefitted from endonasal endoscopic CA correction. The postoperative application of an endonasal stent should be carefully evaluated.

BACKGROUND AND AIMS:Anti-Ro52 antibody (Ab) reactivity is highly prevalent in autoimmune rheumatic diseases (ARDs), mainly Sjögren's syndrome (SjS) and systemic lupus erythematosus (SLE), but also in other inflammatory disorders. Thorough assessment of the prevalence, clinical significance and epitope specificity of Ro52-autoAbs in cancerous diseases is still lacking. MATERIAL AND METHODS:Anti-Ro52 Ab reactivity was tested in a large cohort of 490 patients with various malignant diseases. Ro52-autoAb epitope mapping by an in house line immunoassay was carried out using 5 recombinant Ro52 polypeptides spanning Ro52. RESULTS:Anti-Ro52 abs were significantly more prevalent in patients with ovarian cancer (30%) compared to patients with 6 other malignant diseases (median 8.1%, range 5.9-15.8%). The presence of anti-Ro52 abs in patients with ovarian cancer was strongly associated with better overall survival. Ro52 epitope mapping of patients with ovarian cancer was dissimilar to that of SLE and SjS ARDs, less frequently recognizing Ro52-1 and Ro52-4 fragments compared to patients with SLE and SjS. CONCLUSION:We demonstrate for first time an unexpectedly high frequency of anti-Ro52 abs in patients with ovarian cancer, their presence indicating better overall survival. Their distinguishing epitope profile may suggest a non-SLE or SjS-related stimulus for autoAb production.

Our primary goal was to evaluate the effect of stimulus duration on electrogustometry (EGM) thresholds. Additionally, we sought to evaluate any sex‐related influences and compare the above results to those of taste strips.

INTRODUCTION:Paediatric tumours in the sellar and parasellar regions present clinical and surgical challenges due to anatomical position and behaviour. We illustrate a rare case which caused obstructive hydrocephalus.CASE PRESENTATION:The study included a 14-year-old girl with a glioneuronal tumour (40 mm) originating from the optic chiasm, obliterating the aqueduct, with consequent triventricular hydrocephalus. The patient underwent extended endoscopic endonasal surgery and repair of the skull-base deficiency using a multi-layer technique with fascia lata. The 12-month follow-up showed no complications or recurrences, with recovery in visual acuity.CONCLUSION:The immediate placement of external ventricular drainage, in combination with an extended trans-sphenoidal approach, is a safe and feasible option to treat suprasellar paediatric lesions with hydrocephalus.

From the first days of life, a female newborn suffered from nasal congestion and a runny nose. The first MRI, at 42 days of life, revealed a solid and expansive mass of 1 cm in the left nasal fossa. The radiological examination repeated at 4 months of life showed an increase in volume, up to 2 cm (

Orbital metastases represent a rare condition and only 33 cases of metastases from hepatocellular carcinoma (HCC), usually revealed by slowly progressive exophthalmos, have been described in the literature. A 62-year-old man affected by HCC reported a sudden left exophthalmos and amaurosis. A cranial CT scan revealed pathological tissue occupying the superior portion of the orbit. During endoscopic decompression of the optic nerve, an active venous intraorbital haemorrhage was noted. Following angiography, permanent occlusion was performed on a neoforming circulus fed by ramification of the anterior deep temporal artery, anterior ramification of the superficial temporal artery and sphenoidal ramification of the medial meningeal artery. A percutaneous incisional orbital biopsy showed features of metastatic foci of HCC. This is the first description of orbital metastasis from HCC revealed by a sudden exophthalmos.

Objectives: Nodal metastasis is an important prognosticator in primary parotid cancers. The management of the clinically node-negative neck is an area lacking consensus. This study investigates the occult nodal metastasis rate, and prognostic indicators in primary parotid cancers. Materials and methods: We performed a multicentre retrospective case note review of patients diagnosed and treated surgically with curative intent between 1997 and 2020. Demographic, clinic-pathological and follow-up data was recorded. Results: After exclusions, 334 patients were included for analysis, with a median follow-up of 48 months. The overall rate of occult lymph node metastasis amongst patients undergoing elective neck dissection was 22.4%, with older age, high-grade and more advanced primary tumours being associated with higher rates. On multi variable analysis, age >= 60 years (HR = 2.69, p = 0.004), high-grade tumours (HR = 2.70, p = 0.005) and advanced primary tumours (pT3-4, HR = 2.06, p = 0.038) were associated with worse overall survival. Occult nodal metastasis on final pathology was associated with a close-to-significant reduction in regional recurrence free survival (HR = 3.18, p = 0.076). Conclusion: This large series confirms the significant occult lymph node metastasis rate in primary parotid cancer, and demonstrates the importance of primary histology, tumour grade and stage in predicting survival outcome. This data supports the use of elective neck dissection in patients with high-risk tumours.

AbstractWhile the so-called pseudoaneurysms can result from arterial injury during trans-sphenoidal surgery or after a trauma, spontaneous aneurysms of cavernous–internal carotid artery (CICA) are rare. Symptoms vary and the differential diagnosis with other, more frequent, sellar lesions is difficult. We describe three cases of misdiagnosed CICA spontaneous aneurysm. In two cases the onset was with neuro-ophthalmological manifestations, classifiable as “cavernous sinus syndrome.” The emergency computed tomography scan did not show CICA aneurysm and the diagnosis was made by surgical exploration. The third patient came to our attention with a sudden severe unilateral epistaxis; endonasal surgery revealed also in this case a CICA aneurysm, eroding the wall and protruding into the sphenoidal sinus. When the onset was with a cavernous sinus syndrome, misdiagnosis exposed two patients to potential serious risk of bleeding, while the patient with epistaxis was treated with embolization, using coils and two balloons. Intracavernous nontraumatic aneurysms are both a diagnostic and therapeutic challenge, because of their heterogeneous onset and risk of rupture, potentially lethal. Intracavernous aneurysms can be managed with radiological follow-up, if asymptomatic or clinically stable, or can be surgically treated with endovascular or microsurgical techniques.

A 58-year-old immunocompetent woman gave a 2-week history of headache, facial pain and fever, without photophobia, nausea or vomiting. Initially, the pain was left-sided retrobulbar and radiated to the vertex. It was dull and intermittent without postural variation but progressively intensified, becoming more constant with nocturnal interference. The pain gradually extended bilaterally to the cheeks, maxillae and frontoparietal regions while remaining predominantly left-sided. Two days before assessment, she had developed horizontal diplopia on left lateral gaze. On examination, her temperature was 38.5 ° C, and there was limited left eye abduction consistent with left sixth nerve palsy. Her other cranial and peripheral nerve function, ears, nose and throat were normal with no signs of meningeal irritation. Blood tests (including blood cultures) were negative. CT scan of the head identified left sphenoid sinus opacification (figure 1A,B; black arrows) with …