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1.
Front Endocrinol (Lausanne) ; 15: 1414101, 2024.
Article in English | MEDLINE | ID: mdl-39280003

ABSTRACT

Background: Skeletal fragility is characterized by increased frequency of vertebral fractures (VFs) in acromegaly. Several trials were conducted to identify modifiable risk factors and predictors of VFs, with limited data on the prognostic role of GH receptor (GHR) isoforms. In this study, we investigated the potential role of GHR polymorphism on the occurrence of incidental VFs (i-VFs), in patients treated with second-line medical therapies. Methods: A longitudinal, retrospective, observational study was conducted on a cohort of 45 acromegalic patients not-responsive to first-generation somatostatin receptor ligands (fg-SRLs) and treated with GHR antagonist (Pegvisomant) or with the second-generation SRLs (Pasireotide long-acting release). Results: Second line treatments were Pegvisomant plus fg-SRLs in 26 patients and Pasireotide LAR in 19 patients. From the group treated with fg-SRLs+Peg-V, the fl-GHR isoform was identified in 18 patients (69.2%) and the d3-GHR isoform in 8 patients (30.8%). I-VFs arose exclusively in fl-GHR isoform carriers (p=0.039). From the group treated with Pasireotide LAR, the fl-GHR isoform was identified in 11 patients (57.9%), and the d3-GHR isoform in 8 patients (42.1%). I-VFs arose exclusively in d3-GHR isoform carriers (p=0.018). Patients with fl-GHR isoform had a higher risk for i-VFs if treated with fg-SRL+Peg-V (OR: 1.6 95%IC: 1.1-2.3, p=0.04), and a lower risk if treated with Pasi-LAR (OR: 0.26 IC95%: 0.11-0.66, p=0.038). Conclusions: Our data support a predictive role of the GHR isoforms for the occurrence of i-VFs in acromegalic patients treated with second-line drugs, tailored to the individual patient. The knowledge of the GHR polymorphism may facilitate the choice of second-line therapies, improving the therapeutic approach, in the context of personalized medicine.


Subject(s)
Acromegaly , Human Growth Hormone , Receptors, Somatotropin , Somatostatin , Humans , Acromegaly/drug therapy , Male , Female , Middle Aged , Retrospective Studies , Pilot Projects , Receptors, Somatotropin/genetics , Receptors, Somatotropin/metabolism , Receptors, Somatotropin/antagonists & inhibitors , Somatostatin/analogs & derivatives , Somatostatin/therapeutic use , Adult , Human Growth Hormone/therapeutic use , Human Growth Hormone/metabolism , Human Growth Hormone/analogs & derivatives , Aged , Polymorphism, Genetic , Longitudinal Studies
2.
J Neurosurg Case Lessons ; 8(13)2024 Sep 23.
Article in English | MEDLINE | ID: mdl-39312804

ABSTRACT

BACKGROUND: The management of recurrent Rathke's cleft cysts (RCCs) remains challenging. The off-label application of steroid-eluting bioabsorbable or nonabsorbable intracystic stents has been recently described. Early outcomes and complications of this treatment have been described as well, but long-term data are lacking. OBSERVATIONS: A 31-year-old woman who had undergone 3 prior transsphenoidal surgeries for recurrent RCC at other institutions was admitted for methicillin-susceptible Staphylococcus aureus meningitis. Five years earlier, a nonabsorbable stent had been placed in the cyst using a microsurgical transsphenoidal approach. RCC recurrence had been radiologically evident after 2 years but was clinically asymptomatic. Acute cerebrospinal fluid leakage and other causes of meningitis were ruled out; the stent was occluded. After antibiotic therapy and resolution of the meningitis, the patient underwent endoscopic removal of the catheter, excision of the cyst, and reconstruction with a nasoseptal flap. At the 18-month follow-up, she remained neurologically intact, under replacement therapy, and with no signs of recurrence. LESSONS: The authors report the first case of late meningitis due to a nonabsorbable stent positioned for the treatment of a recurrent RCC. Currently, data on the long-term outcomes, failure rates, and complications of stents for RCC are limited. https://thejns.org/doi/10.3171/CASE2477.

3.
J Neurosurg ; : 1-11, 2024 Sep 27.
Article in English | MEDLINE | ID: mdl-39332026

ABSTRACT

OBJECTIVE: The oculomotor cistern (OMC) is a meningeal cuff filled with CSF that contains the oculomotor nerve (cranial nerve [CN] III) at the level of the lateral wall of the cavernous sinus. Only a few studies have investigated the involvement of the OMC by pituitary adenomas (pituitary neuroendocrine tumors [PitNETs]), mainly with relatively small case series. The aim of this study was to perform a histomorphological description of the OMC and systematically analyze its involvement by PitNETs from radiological, clinical, and surgical perspectives. METHODS: Ten hemisellae from formalin-fixed specimens were studied with 3-µm sections. Digital image analysis software was used for morphological and quantitative assessments. Clinical, radiological, surgical, and histological data of patients undergoing endoscopic transsphenoidal surgery for PitNETs at the University of Brescia, Italy, between 2014 and 2021 were recorded. OMC involvement was graded as not compressed, compressed, and invaded. The same surgical team operated on all patients. RESULTS: The OMC had an elliptical shape with an average area of 3.1 mm2 and a length of 5.5 mm. No cisternal points of weakness were recognized in the histomorphological study. Of 315 patients, 246 had complete data: apoplexy and CN III palsy were documented in 6.9% and 8.5%, respectively. OMC compression and invasion were recorded in 106 (43.1%) and 23 (9.3%) patients. Significant associations between OMC involvement and PitNET dimensions (p < 0.001), Knosp grade (p < 0.001), preoperative oculomotor palsy (p < 0.001), Ki-67 percentage (p = 0.009), and recurrence/progression of residual tumor (p = 0.008) were found. A new postoperative CN III palsy was evident in 2%: transient in 4 cases, and persistent in 1 patient treated for a recurrent PitNET who experienced a local infection complication. Preoperative CN III palsy improved in 10 cases. CONCLUSIONS: Significant OMC involvement by PitNETs might be underrecognized, but it can be treated using the endoscopic transsphenoidal approach, and it affects patient outcomes.

5.
Article in English | MEDLINE | ID: mdl-39132990

ABSTRACT

Transbasal meningiomas1 are a rare group of tumors that grow intracranially, invade the skull base, and propagate extracranially; they are usually located at the level of the olfactory cribra and sphenoid wing.1-4 Transbasal tuberculum sellae meningiomas are a subset of these tumors, which extend into the suprasellar and sphenoid regions. The traditional transcranial approach for some transbasal meningiomas has recently evolved toward the transnasal endoscopic approach, which allows the removal of both the intranasal and intracranial components, reducing the manipulation of the optic nerves and neurovascular structures. Nevertheless, specific anatomical criteria must be present; the knowledge of specific anatomy5,6 and the surgeon's experience are fundamental for reaching the most favorable outcome.7-11 In this video, we present the case of a 50-year-old woman with a history of progressive visual impairment and scotoma in the left inferotemporal quadrant. Radiological examinations suggested a transbasal tuberculum meningioma that extended into the suprasellar region, sphenoid sinus, and optic canals. An endoscopic transtuberculum approach with intraoperative visual-evoked potentials12 was performed, allowing an early devascularization with the preservation of the superior hypophyseal arteries and the removal of the component extending in the medial portion of both optic canals. The postoperative MRI documented a gross total resection without complications. The patient reported an immediate improvement of the visual symptoms. Histopathology documented a meningioma (CNS World Health Organization grade 1) with bone invasion. The patient consented to the procedure and to the publication of her image; Institutional Review Board approval was not required for this single case report.

6.
J Clin Med ; 13(16)2024 Aug 09.
Article in English | MEDLINE | ID: mdl-39200822

ABSTRACT

Background: The aim of this paper is to evaluate the impact on the quality of life of the treatment of nasal vestibule tumors by interventional radiotherapy (IRT-brachytherapy) through a patient reported outcome questionnaire. Methods: We prospectively collected data about patients undergoing IRT according to our institutional schedule of 44 Gy delivered in 14 fractions twice a day. We recorded both acute toxicity data, using the Common Terminology Criteria for Adverse Events (CTCAE) version 5.0, and quality of life data, using the 22-item Sino-Nasal Outcome Test (SNOT-22) at baseline (T0), at 1 month (T1), at 3 months (T3), and at 6 months (T6). Results: We enrolled 10 consecutive patients treated between February 2023 and October 2023. The decrease in terms of SNOT-22 mean value was statistically significant from T0 and T6 with a p-value < 0.001. A noteworthy clinical finding is that quality of life improved regardless of the occurrence of G1-G2 side effects. Conclusions: Using SNOT-22 on patients with nasal vestibule carcinoma treated with IRT has shown an improvement in quality of life that is not strictly dependent on the occurrence of expected G1-G2 side effects.

7.
J Pers Med ; 14(7)2024 Jul 09.
Article in English | MEDLINE | ID: mdl-39063989

ABSTRACT

Recalcitrant frontal sinusitis in patients with chronic rhinosinusitis and nasal polyps (CRSwNP) has a negative impact on their quality of life due to frontal pain and a high risk of sinus occlusion, thus necessitating antibiotics, systemic corticosteroids, and multiple surgeries. The aim of this study was to assess the efficacy of dupilumab in reducing frontal pain and the need for rescue treatments for recalcitrant frontal sinusitis in patients with CRSwNP. We enrolled a cohort of 10 patients with severe uncontrolled CRSwNP and concomitant recurrent frontal sinusitis associated with severe facial pain measured by MIDAS score who were treated with dupilumab 300 mg every 2 weeks and followed for at least 12 months. The mean MIDAS score decreased from 45.6 ± 10.7 at baseline to 1.3 ± 2.3 at 6 months (p < 0.05). VAS craniofacial pain decreased from 7.3 ± 1.6 at baseline to 1.2 ± 1.5 at 6 months (p < 0.05). No patient needed oral corticosteroids during treatment with dupilumab (p < 0.05), and the use of analgesics decreased from 9.6 ± 3.1 NSAID pills/week in the last 2 months at baseline to 0.6 ± 1.3 at 1 year of follow-up (p < 0.05). Our results demonstrated that use of subcutaneous dupilumab can improve symptom control, including recurrent severe cranio-facial pain, and reduce the need for rescue medical treatments (systemic steroids and NSAID) in patients with severe uncontrolled CRSwNP and concomitant recurrent frontal sinusitis.

8.
Acta Neurochir (Wien) ; 166(1): 269, 2024 Jun 17.
Article in English | MEDLINE | ID: mdl-38880842

ABSTRACT

BACKGROUND: Velopharyngeal insufficiency (VPI) is a known complication of transoral surgery, with a reported incidence of 8.1%. The main factor related to VPI is the split of the soft palate. However, dead space resulting from transoral decompression may play a pivotal role in the pathogenesis of the dysfunction. In our experience, functionally significant dead space is almost constantly present after transoral decompression. This is probably due to malformation in children and postoperative scarring, thus configuring a nosological entity that we could define as "syndrome of the nasopharyngeal dead space." Palatal prosthesis and pharyngoplasty have been proposed, though these surgical procedures are technically tricky and with possible complications, such as OSA symptoms, snoring, and nasopharyngeal stenosis. METHODS: We proposed an effortless and minimally invasive procedure to treat this condition based on lipofilling the nasopharynx posterior wall endoscopically. To test the procedure's functional result, the submucosa of the nasopharynx posterior wall was initially filled with resorbable material, namely fibrin glue and autologous blood. The result was optimal but regressed after one month. Then, we repeated the procedure by lipofilling with autologous abdominal fat, resulting in a more stable anatomical and functional outcome at six months follow-up. RESULTS: The patient had a prompt significant improvement of his complaints (rhinolalia and oronasal regurgitation) and a correct projection of the nasopharynx posterior wall, with correct closure during phonation and absence of oronasal reflux. CONCLUSIONS: The "syndrome of the nasopharyngeal dead space" should be correctly identified after transoral surgery. It can be effectively treated with lipofilling of the posterior nasopharyngeal wall, a simple and minimally invasive procedure.


Subject(s)
Velopharyngeal Insufficiency , Humans , Male , Adipose Tissue/transplantation , Adipose Tissue/surgery , Endoscopy/methods , Nasopharynx/surgery , Palate, Soft/surgery , Postoperative Complications/etiology , Postoperative Complications/surgery , Treatment Outcome , Velopharyngeal Insufficiency/surgery , Velopharyngeal Insufficiency/etiology , Child
9.
Acta Otorhinolaryngol Ital ; 44(Suppl. 1): S28-S36, 2024 May.
Article in English | MEDLINE | ID: mdl-38745514

ABSTRACT

Objective: Management of recurrent head and neck cancer (HNC) is challenging. One option in previously irradiated patients is re-irradiation using interventional radiotherapy (IRT), the modern form of brachytherapy. Re-irradiation using IRT can be delivered as an exclusive strategy for salvage or through a postoperative or perioperative approach after salvage surgery. The aim of the present study is to analyse a bicentric Italian series focusing on the use of IRT as a re-irradiation modality and assess the resulting evidence concerning oncologic outcomes and morbidity. Methods: This is a retrospective study performed in two referral centres in Italy: Policlinico Universitario Agostino Gemelli in Rome and Azienda Ospedaliera Universitaria in Sassari. All patients who had previously received a full course of external beam RT and have been re-irradiated using high-dose-rate IRT between December 2010 and June 2023 were included. Patients were retreated either by a combination of surgery and perioperative (either endocavitary or interstitial) IRT or by exclusive interstitial IRT. Results: Thirty-four patients were included in the present series, 2 of whom underwent more than one IRT re-irradiation. Notably, no patient reported specific IRT-related toxicities. Median follow-up, excluding patients who died of HNC, was 24.5 months. Two-year local relapse-free survival was 26%, disease-specific survival 39.1%, and overall survival 36.6%. Conclusions: The present series is the largest reported experience of re-irradiation by IRT for HNC in Italy. The very low rate of toxicity confirms IRT as the safest re-irradiation modality. It is noteworthy to underline that IRT is a multidisciplinary strategy based on the close cooperation between surgeons and radiation oncologists during every phase, from the recommendation of treatment and implantation in the operating theatre, to its prescription and dose painting.


Subject(s)
Brachytherapy , Head and Neck Neoplasms , Neoplasm Recurrence, Local , Re-Irradiation , Humans , Retrospective Studies , Male , Middle Aged , Head and Neck Neoplasms/radiotherapy , Head and Neck Neoplasms/surgery , Female , Aged , Neoplasm Recurrence, Local/radiotherapy , Neoplasm Recurrence, Local/epidemiology , Brachytherapy/methods , Re-Irradiation/methods , Treatment Outcome , Adult , Italy , Aged, 80 and over
10.
Clin Neuroradiol ; 2023 Dec 08.
Article in English | MEDLINE | ID: mdl-38066106

ABSTRACT

PURPOSE: This retrospective study aimed to assess the safety and technical efficacy of preoperative direct puncture embolization using a nonadhesive ethylene vinyl alcohol (EVOH) copolymer-based liquid embolic agent (LEA) combined with balloon occlusion at the origin of the external carotid artery for head and neck paragangliomas (HNP). METHODS: We conducted a review of all consecutive cases of HNPs treated with direct puncture embolization using EVOH-based LEAs between 2017 and 2022 in 2 tertiary high-volume hospitals. RESULTS: A total of 25 cases of HNPs in 24 patients underwent direct puncture embolization (12 males, 12 females, mean age 50.9 ± 15.6 years). The average lesion volume was 299.95 mm3. The mean procedure time was 139.11 min, and the mean volume of EVOHs used per case was 19.38 mL. Successful complete devascularization was achieved in all cases, with a homogeneous and deep penetration of the embolic agent into the tumor vessel bed regardless of the LEA type. CONCLUSION: Preoperative embolization of HNPs using a direct puncture technique and EVOHs is a safe, efficient, and feasible treatment option with a low risk of complications. This procedure facilitates surgery by transforming tumors into avascular masses that are well-delineated against the surrounding normal tissue.

11.
J Clin Med ; 12(19)2023 Sep 24.
Article in English | MEDLINE | ID: mdl-37834798

ABSTRACT

Interventional radiotherapy (brachytherapy) has become the new therapeutic standard in the management of early stages nasal vestibule tumors; in fact it allows for high local control rates and low toxicity profiles. However, since more and more patients will receive interventional radiotherapy (brachytherapy) as primary treatment, it is desirable to implement novel strategies to reduce the dose to organs at risk with the future aim to result in further lowering long-term side effects. MATERIALS AND METHODS: We were able to identify two different strategies to reduce dose to the treatment volume, including the implantation technique (the implant can be interstitial, endocavitary or mixed and the catheters may be placed either using the Paris system rules or the anatomical approach) and the dose distribution within the implant (the most commonly used parameter to consider is the dose non-uniformity ratio). We subsequently propose two novel strategies to reduce dose to organs at risk, including the use of metal shields for fixed organs as in the case of the eyes and the use of a mouth swab to push away mobile organs, such in the case of the mandible. We used two different algorithms to verify the values namely the TG-43 and the TG-186. RESULTS: We provided an accurate literature review regarding strategies to reduce toxicity to the treatment volume, underlining the pros and cons of all implantation techniques and about the use dose non-uniformity ratio. Regarding the innovative strategies to reduce the dose to organs at risk, we investigated the use of eye shielding and the use of swabs to push away the mandible by performing an innovative calculation using two different algorithms in a series of three consecutive patients. Our results show that the dose reduction, both in the case of the mandible and in the case of eye shielding, was statistically significant. CONCLUSION: Proper knowledge of the best implantation technique and dose non-uniformity ratio as highlighted by existing literature is mandatory in order to reduce toxicity within the treatment volume. With regard to the dose reduction to the organs at risk we have demonstrated that the use of eye shielding and mouth swab could play a pivotal role in clinical practice; in fact, they are effective at lowering the doses to the surrounding organs and do not require any change to the current clinical workflow.

12.
J Clin Med ; 12(17)2023 Aug 23.
Article in English | MEDLINE | ID: mdl-37685541

ABSTRACT

PURPOSE: the aim of this multicenter study is to preliminarily assess the role of the Endoscopic Endonasal Approach (EEA) in ultra-early (i.e., within 12 h) management of selected neurosurgical emergencies in terms of clinical and radiological outcomes. METHODS: 26 patients affected by sellar/parasellar pathologies with rapid progression of symptoms were managed with EEA within 12 h from diagnosis in three Italian tertiary referral Centers from January 2016 to December 2019. Both clinical and radiological data have been collected preoperatively as well as post-operatively in order to perform retrospective analysis. RESULTS: The average time from admission to the operating room was 5.5 h (±2.3). The extent of resection was gross-total in 20 (76.9%), subtotal in 6 (23.1%) patients. One patient experienced re-bleeding after a subtotal removal of a hemorrhagic lesion. Patients with a longer time from admission (>4 h) to the operatory room (OR) experienced stable impairment of the visual acuity (p = 0.033) and visual field (p = 0.029) in the post-operative setting. CONCLUSIONS: The Endoscopic Endonasal Approach represents a safe, effective technique that can be efficiently used with good results in the management of selected neurosurgical emergencies in centers with adequate experience.

13.
Neurosurg Rev ; 46(1): 248, 2023 Sep 19.
Article in English | MEDLINE | ID: mdl-37725193

ABSTRACT

Endoscopic transsphenoidal surgery is a novel surgical technique requiring specific training. Different models and simulators have been recently suggested for it, but no systematic review is available. To provide a systematic and critical literature review and up-to-date description of the training models or simulators dedicated to endoscopic transsphenoidal surgery. A search was performed on PubMed and Scopus databases for articles published until February 2023; Google was also searched to document commercially available. For each model, the following features were recorded: training performed, tumor/arachnoid reproduction, assessment and validation, and cost. Of the 1199 retrieved articles, 101 were included in the final analysis. The described models can be subdivided into 5 major categories: (1) enhanced cadaveric heads; (2) animal models; (3) training artificial solutions, with increasing complexity (from "box-trainers" to multi-material, ct-based models); (4) training simulators, based on virtual or augmented reality; (5) Pre-operative planning models and simulators. Each available training model has specific advantages and limitations. Costs are high for cadaver-based solutions and vary significantly for the other solutions. Cheaper solutions seem useful only for the first stages of training. Most models do not provide a simulation of the sellar tumor, and a realistic simulation of the suprasellar arachnoid. Most artificial models do not provide a realistic and cost-efficient simulation of the most delicate and relatively common phase of surgery, i.e., tumor removal with arachnoid preservation; current research should optimize this to train future neurosurgical generations efficiently and safely.


Subject(s)
Endoscopy , Skull Base Neoplasms , Humans , Animals , Cadaver , Computer Simulation , Databases, Factual
14.
Cancers (Basel) ; 15(18)2023 Sep 09.
Article in English | MEDLINE | ID: mdl-37760463

ABSTRACT

Clival chordomas are rare but aggressive skull base tumors that pose significant treatment challenges and portend dismal prognosis. The aim of this study was to highlight the advantages and limitations of available treatments, to furnish prognostic indicators, and to shed light on novel therapeutic strategies. We conducted a retrospective study of clival chordomas that were surgically treated at our institution from 2003 to 2022; for comparison purposes, we provided a systematic review of published surgical series and, finally, we reviewed the most recent advancements in molecular research. A total of 42 patients underwent 85 surgeries; median follow-up was 15.8 years, overall survival rate was 49.9% at 10 years; meanwhile, progression-free survival was 26.6% at 10 years. A significantly improved survival was observed in younger patients (<50 years), in tumors with Ki67 ≤ 5% and when adjuvant radiotherapy was performed. To conclude, clival chordomas are aggressive tumors in which surgery and radiotherapy play a fundamental role while molecular targeted drugs still have an ancillary position. Recognizing risk factors for recurrence and performing a molecular characterization of more aggressive lesions may be the key to future effective treatment.

15.
Acta Otorhinolaryngol Ital ; 43(Suppl. 1): S3-S13, 2023 Apr.
Article in English | MEDLINE | ID: mdl-37698095

ABSTRACT

Objective: This narrative review analyses factors affecting recurrence of Chronic rhinosinusitis with nasal polyps (CRSwNP) after surgery, such as type, extension and completeness of endoscopic sinus surgery (ESS). We also described new implications in the management of recurrences after the advent of biologics. Methods: We identified four topics: definition of disease state; factors linked to recurrence of polyps; evaluation and management of recurrence in clinical practice. Results: We analysed the differences between exacerbation and recurrence, as well as the concept of "controlled disease". We focused on potential predictors of recurrence after ESS, such as type 2 inflammation, asthma, aspirin-exacerbated respiratory disease, incomplete initial surgery and lack of adherence to long-term post-operative local corticosteroids. We discussed the new aspects of diagnosis and treatment of recurrences after surgery, summarising our suggestions in a detailed algorithm for practical management of patients with recurrent disease. Conclusions: The results emphasised the importance of accurate evaluation of patients with CRSwNP recurrence, focusing on the reasons of failure and risk of disease progression, in order to guide personalised interventions. It is crucial to define the concept of appropriate surgery, which affects the choice between starting a biologic or repeating surgery.


Subject(s)
Biological Products , Nasal Polyps , Sinusitis , Humans , Nasal Polyps/complications , Nasal Polyps/surgery , Chronic Disease , Sinusitis/complications , Sinusitis/surgery , Recurrence , Biological Products/therapeutic use
16.
Acta Neurochir (Wien) ; 165(11): 3421-3429, 2023 11.
Article in English | MEDLINE | ID: mdl-37733080

ABSTRACT

OBJECTIVE: To refine a reliable and reproducible intraoperative visual evoked potentials (iVEPs) monitoring protocol during endoscopic transsphenoidal surgery. To assess the reliability of baseline iVEPs in predicting preoperative visual status and perioperative iVEP variation in predicting postoperative visual outcome. METHODS: Sixty-four patients harboring tumors of the pituitary region were included. All patients underwent endoscopic endonasal approach (EEA) with iVEPs monitoring, using a totally intravenous anesthetic protocol. Ophthalmological evaluation included visual acuity and visual field studies. RESULTS: Preoperatively, visual acuity was reduced in 86% and visual field in 76.5% of cases. Baseline iVEPs amplitude was significantly correlated with preoperative visual acuity and visual field (p = 0.001 and p = 0.0004, respectively), confirming the reliability of the neurophysiological/anesthetic protocol implemented. Importantly, perioperatively the variation in iVEPs amplitude was significantly correlated with the changes in visual acuity (p < 0.0001) and visual field (p = 0.0013). ROC analysis confirmed that iVEPs are an accurate predictor of perioperiative visual acuity improvement, with a 100% positive predictive value in patients with preoperative vision loss. CONCLUSIONS: iVEPs during EEA is highly reliable in describing preoperative visual function and can accurately predict postoperative vision improvement. SIGNIFICANCE: iVEPs represent a promising resource for carrying out a more effective and safe endoscopic transsphenoidal surgery.


Subject(s)
Evoked Potentials, Visual , Pituitary Neoplasms , Humans , Reproducibility of Results , Pituitary Neoplasms/diagnosis , Pituitary Neoplasms/surgery , Pituitary Neoplasms/pathology , Vision, Ocular , Endoscopy/methods , Retrospective Studies , Treatment Outcome
18.
Ital J Pediatr ; 49(1): 85, 2023 Jul 16.
Article in English | MEDLINE | ID: mdl-37455311

ABSTRACT

BACKGROUND: Cornelia de Lange syndrome is a rare genetic disease with otolaryngological involvement. The classic phenotype is characterized by distinctive facial features, intellectual disability, growth delay, hirsutism, and upper-limb reduction. Nasal polyposis was previously reported in association with chronic rhinosinusitis, however data about prevalence, diagnosis, treatment and prognosis are lacking for this cohort of patients, affected by rare disease. CASE PRESENTATION: We describe the whole diagnostic and therapeutic workflow of nasal polyps in two pediatric patients with Cornelia de Lange, successfully diagnosed and treated by nasal endoscopy. CONCLUSION: Our report confirm that nasal endoscopy is a safe and useful tool in the diagnosis, treatment and follow-up of nasal polyps, even in Cornelia de Lange syndrome pediatric patients. We want to increase the alert for the detection of nasal polyps in patients with Cornelia de Lange syndrome since pediatric age. We recommend endoscopy in all patients with Cornelia de Lange syndrome and symptoms of chronic nasal obstruction and/or OSAS. Multidisciplinary team and sedation service could be useful in the management of Cornelia de Lange syndrome patients with airway obstruction symptoms and sleep disturbance when severe intellectual disability, autism or psychiatric findings are present.


Subject(s)
De Lange Syndrome , Intellectual Disability , Nasal Polyps , Humans , De Lange Syndrome/complications , De Lange Syndrome/diagnosis , De Lange Syndrome/therapy , Follow-Up Studies , Nasal Polyps/complications , Nasal Polyps/diagnosis , Nasal Polyps/therapy , Endoscopy
19.
Front Endocrinol (Lausanne) ; 14: 1123267, 2023.
Article in English | MEDLINE | ID: mdl-37206441

ABSTRACT

Growth Hormone-secreting adenomas exhibits variable biological behavior and heterogeneous natural history, ranging from small adenomas and mild disease, to invasive and aggressive neoplasms with more severe clinical picture. Patients not cured or controlled after neurosurgical and first-generation somatostatin receptor ligands (SRL) therapy could require multiple surgical, medical and/or radiation treatments to achieve disease control. To date, no clinical, laboratory, histopathological, or neuroradiological markers are able to define the aggressiveness or predict the disease prognosis in patients with acromegaly. Therefore, the management of these patients requires careful evaluation of laboratory assessments, diagnostic criteria, neuroradiology examinations, and neurosurgical approaches to choose an effective and patient-tailored medical therapy. A multidisciplinary approach is particularly useful in difficult/aggressive acromegaly to schedule multimodal treatment, which includes radiation therapy, chemotherapy with temozolomide and other, recent emerging treatments. Herein, we describe the role of the different members of the multidisciplinary team according to our personal experience; a flow-chart for the therapeutic approach of difficult/aggressive acromegaly patients is proposed.


Subject(s)
Acromegaly , Adenoma , Human Growth Hormone , Pituitary Neoplasms , Humans , Acromegaly/etiology , Acromegaly/therapy , Acromegaly/pathology , Growth Hormone , Pituitary Neoplasms/drug therapy , Human Growth Hormone/therapeutic use , Adenoma/pathology
20.
World Neurosurg ; 172: 12-19, 2023 Apr.
Article in English | MEDLINE | ID: mdl-36657712

ABSTRACT

BACKGROUND: The oculomotor cistern (OMC) is a cerebrospinal fluid space bound by meningeal layers that surrounds the oculomotor nerve as it crosses the oculomotor triangle to reach the lateral wall of the cavernous sinus at the level of the anterior clinoid process. Although several anatomical and radiological studies are available, its anatomy and relationship with pituitary adenomas (PAs) are still matter of discussion. OBJECTIVE: The aim of the study is to provide an updated and focused overview of the OMC, highlighting the different perspectives and descriptions from anatomical, radiological, and clinical points of view. METHODS: A scoping review was conducted up to 29th October 2022, according to PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews) criteria. PubMed, Web of Science, Scopus databases, and correlated citations were investigated. RESULTS: Of the 562 records identified, 22 were included in the present analysis. There were 13, 5, and 4 anatomo-surgical, radiological, and clinical studies, respectively. Though there is general consensus on its definition, data are variable on different features of OMC. Defects or absence of dural layers adjacent to the oculomotor nerve were described in only 4 papers. The transition from meningeal to neural layers is still unclear. PAs with OMC involvement are poorly studied and have unique clinical characteristics. To date, 21 patients have been described; the reported prevalence of OMC involvement by PAs ranges from 4.1% to 14.6%. CONCLUSIONS: Clarifying the OMC features with further systematic studies may not only broaden theoretical knowledge but also have implications on endoscopic transnasal pituitary surgery.


Subject(s)
Adenoma , Pituitary Neoplasms , Humans , Adenoma/diagnostic imaging , Adenoma/surgery , Endoscopy , Oculomotor Nerve/diagnostic imaging , Oculomotor Nerve/surgery , Pituitary Gland/diagnostic imaging , Pituitary Gland/surgery , Pituitary Neoplasms/diagnostic imaging , Pituitary Neoplasms/surgery
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