Endoscopic trans-sphenoidal pituitary surgery does not impact postoperative nasal quality of life.

PURPOSE: The aim of this prospective longitudinal study was to assess both subjective quality of life using questionnaires and objective examination of nasal function with regard to olfaction, nasal air flow and mucociliary clearance in patients after minimally invasive, turbinate-preserving endoscopic transnasal trans-sphenoidal pituitary surgery. METHODS: Patients undergoing endoscopic transnasal pituitary surgery were recruited prospectively and examined during three study visits, preoperatively and 3 and 6 months postoperatively. We examined nasal function using sniffin' sticks test, rhinomanometry, saccharin transit time test, and endoscopic and radiological scores. In addition, the influence on subjective quality of life and mental health was recorded using the Sinonasal-Outcome-Test-20 (SNOT-20) and the Hospital-Anxiety-and-Depression-Scale (HADS). RESULTS: 20 patients undergoing endoscopic pituitary tumor resections were included. No significant changes in olfaction or mucociliary clearance were noted. Nasal air flow showed a tendency to increase in the postoperative course lacking significance. Both the endoscopy and the radiological scores showed a significant deterioration, especially after 3 months, with a trend towards improvement over time. However, neither the SNOT-20 nor the HADS showed significant changes compared to baseline. CONCLUSIONS: Our concept of minimally invasive endoscopic tumor resections on the pituitary gland with preservation of nasal turbinates shows low morbidity for the patient. Despite objectifiable surgery-associated changes in the nose, nasal physiology in terms of smell, airflow and mucociliary clearance can be preserved and the subjective quality of life of our patients remains stable.

Preservation of nasal turbinates in endoscopic, anterior skull base surgery-yes, we can!

OBJECTIVE: To evaluate the frequency, type and indications of nasal turbinate (NT) resection during endoscopic, anterior skull base surgery and to analyze factors that may have an impact on the need of NT removal. METHODS: In this retrospective cohort study, 306 subjects (150 males and 156 females, mean age 55.4 ± 15.3 years) who underwent multidisciplinary, transnasal, endoscopic tumor surgery of the anterior skull base using 4-handed techniques between 2011 and 2019 at the Department of Otorhinolaryngology, Medical University of Graz, were included. RESULTS: In the majority of interventions (n = 281/306; 91.8%), all NT were preserved. Significant factors influencing the need of NT resections turned out to be type of endoscopic approach (p < 0.001; V = 0.304), sagittal (p = 0.003; d = 0.481) and transversal (p = 0.017; d = 0.533) tumor diameter, tumor type (p < 0.001; V = 0.355) and tumor location (p < 0.001; V = 0.324). CONCLUSIONS: NT can be preserved in the majority of patients undergoing tumor resection in anterior, transnasal, skullbase surgery and routine resection of NT should be avoided. Variables that have an impact on the need of NT resections are types of endoscopic approaches, sagittal and transversal tumor extension and tumor type. These factors should be considered in planning of surgery and preoperative information of patients.

Vivostat®: an autologous fibrin sealant as useful adjunct in endoscopic transnasal CSF-leak repair.

The benefit of fibrin glue for reduction of postoperative CSF-leaks after endoscopic skull base surgery is not clearly evident in literature. However, its use is supposed to be beneficial in fixing grafting material. As of today there is no specific data available for otolaryngological procedures. A retrospective data analysis at a tertiary care referral center on 73 patients treated endoscopically transnasally for CSF-leaks at the ENT-department Graz between 2009 and 2012 was performed. Primary closure rate between conventional fibrin glue and autologous fibrin glue were analyzed. The Vivostat(®) system was used in 33 CSF-leak closures and in 40 cases conventional fibrin glue was used. Comparing the two methods the primary closure rate using the autologous Vivostat(®) system was 75.8 and 85.0 % with conventional fibrin glue. The secondary closure the rates were 90.9 % with Vivostat(®) 92.5 % with conventional fibrin glue. The Vivosat(®) system is a useful adjunct in endoscopic CSF-leak closure. Its advantages over conventional fibrin glue are its application system for fixation of grafting material particularly in underlay techniques. Despite this advantage it cannot replace grafting material or is a substitute for proper endoscopic closure which is reflected by the closure rates.

No requirement of perioperative glucocorticoid replacement in patients with endogenous Cushing's syndrome - a pilot study.

PURPOSE: Surgical therapy represents the first-line treatment for endogenous Cushing's syndrome (CS). While postoperative glucocorticoid replacement is mandatory after surgical remission, the role of perioperative glucocorticoid therapy is unclear. METHODS: We recruited patients with central or adrenal CS in whom curative surgery was planned and patients who underwent pituitary surgery for other reasons than CS as a control group. Patients did not receive any perioperative glucocorticoids until the morning of the first postoperative day. We performed blood samplings in the morning of surgery, immediately after surgery, in the evening of the day of surgery, and in the morning of the first and third postoperative day before any morning glucocorticoid intake. We continued clinical and biochemical monitoring during the following outpatient care. RESULTS: We recruited 12 patients with CS (seven with central CS, five with adrenal CS) and six patients without CS. In patients with CS, serum cortisol concentrations <5.0 µg/dL (<138 nmol/L) were detected in the morning of the first and third postoperative day in four (33%) and six (50%) patients, respectively. Morning serum cortisol concentrations on the third postoperative day were significantly lower when compared to preoperative measurements (8.5 ± 7.6 µg/dL vs. 19.9 ± 8.9 µg/dL [235 ± 210 nmol/L vs. 549 ± 246 nmol/L], p = 0.023). No patient developed clinical or biochemical signs associated with hypocortisolism. During follow-up, we first observed serum cortisol concentrations >5.0 µg/dL (>138 nmol/L) after 129 ± 97 days and glucocorticoids were discontinued after 402 ± 243 days. Patients without CS did not require glucocorticoid replacement at any time. CONCLUSION: Perioperative glucocorticoid replacement may be unnecessary in patients with central or adrenal CS undergoing curative surgery as first-line treatment.

Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis--a review.

PURPOSE: The aim of the study was to evaluate frequency and clinical relevance of haemorrhagic events associated with primary angiitis of the central nervous system in childhood (cPACNS), a rare but increasingly recognized disease with varying clinical presentations. METHOD: A systematic literature review from 1990 onwards was conducted to identify reported cases of cPACNS. RESULTS: A total of 110 paediatric patients met the inclusion criteria. The median age was 9.5 years. Seven children (7/110, 6.4 %) demonstrated cerebral haemorrhage. Death occurred only in children with cerebral haemorrhage (4/110, 3.6 %); both a sudden and prolonged course of disease was observed. CONCLUSION: PACNS is a rare disease and the occurrence of haemorrhage with this condition is even rarer; however, the risk of an unfavourable outcome under these circumstances seems to be increased. PACNS adds to the list of differential diagnoses of intracerebral haemorrhage in the paediatric population.

Optic nerve injury in preoperative imaging is associated with visual improvement outcome in endoscopic optic nerve decompression.

OBJECTIVE: To evaluate potential clinical parameters having an impact on visual outcome after endoscopic optic nerve decompression in acute optic neuropathy patients. METHODS: A retrospective chart review of patients with acute optic neuropathy, who underwent endoscopic optic nerve decompression between June 2001 and November 2018 at an academic center was performed. Patients were divided into groups according to visual improvement after surgical treatment (yes/no). Following clinical parameters were compared between groups: perioperative steroid use, evidence of optic nerve affection in preoperative neuroimaging, additional optic nerve sheath incision, surgery delay and preoperative C-reactive protein (CRP) levels. Further subgroups analyses were conducted based on etiology (trauma/tumor). RESULTS: Among 32 included cases, 16 patients (50%) reported visual improvement after endoscopic optic nerve decompression. There was no significant difference in visual improvement between etiology subgroups (trauma: n = 9/20 (45%) vs. tumor: n = 7/12 (58.3%), p = 0.465). Tumor subgroup patients with visual improvement had a significantly higher prevalence of optic nerve affection in preoperative neuroimaging than those without visual improvement (p = 0.018, φ = 0.683). Perioperative steroid administration was negatively associated with visual outcome (p = 0.034, φ = 0.375). Nerve sheath incision, surgery delay and preoperative CRP levels did not have a significant impact on visual outcome (p > 0.05). CONCLUSION: Radiological findings can help as an indicator for surgical treatment since an affected optic nerve in preoperative neuroimaging resulted in better visual outcome after surgery. The use of steroids should be considered more carefully since it did not show any beneficial effect.

Endoscopic rhino-neurosurgical approach for non-adenomatous sellar and skull base lesions.

OBJECTIVE: Since endoscopic endonasal transsphenoidal surgery requires skills of both neurosurgeons and otorhinolaryngologists, and the nose is the primary corridor of approach, we favour the term `endoscopic rhino-neurosurgery` and report on our interdisciplinary experience treating non-adenomatous lesions with skull base extension. METHODS: Between 2004 and 2009, 58 patients with 21 different disease patterns underwent endoscopic rhino-neurosurgical procedures. Mean age was 39.9 years, 50% were female. Seven had undergone prior surgery. Clinically, 34.5% presented with visual field deficits and with nerve palsies. Preoperatively, 62.1% showed a normal pituitary function. RESULTS: Mean follow-up was 13.1 months. The surgical goal depended on type of lesion; the intended extent of resection was achieved in 81%. Recovery from visual field deficits occurred in 80%. Neither deteriorated nor new cranial nerve palsies were observed. A normal endocrinological function could be maintained in 94.4%. Permanent diabetes insipidus occurred in 7 patients. Surgical complications included cerebrospinal fluid (CSF) leaks in 6 patients and meningitis in 4. All complications were managed successfully. There was no surgery-related mortality. CONCLUSION: The endoscopic rhino-neurosurgical approach is applicable for a wide variety of lesions comprising sella and skull base. As our data prove, this technique can be performed with satisfying results in non-adenomatous lesions as well.

Limits of the endoscopic transnasal transtubercular approach.

INTRODUCTION: The endoscopic transnasal trans-sphenoidal transtubercular approach has become a standard alternative approach to neurosurgical transcranial routes for lesions of the anterior skull base in particular pathologies of the anterior tubercle, sphenoid plane, and midline lesions up to the interpeduncular cistern. For both the endoscopic and the transcranial approach indications must strictly be evaluated and tailored to the patients' morphology and condition. The purpose of this review was to evaluate the evidence in literature of the limitations of the endoscopic transtubercular approach. EVIDENCE ACQUISITION: A PubMed/Medline search was conducted in January 2018 entering following keywords. Upon initial screening 7 papers were included in this review. There are several other papers describing the endoscopic transtubercular approach (ETTA). We tried to list the limitation factors according to the actual existing literature as cited. EVIDENCE SYNTHESIS: The main limiting factors are laterally extending lesions in relation to the optic canal and vascular encasement and/or unfavorable tumor tissue consistency. CONCLUSIONS: The ETTA is considered as a high level transnasal endoscopic extended skull base approach and requires excellent training, skills and experience.

Establishment of clival chordoma cell line MUG-CC1 and lymphoblastoid cells as a model for potential new treatment strategies.

Chordomas are rare malignant tumors that develop from embryonic remnants of the notochord and arise only in the midline from the clivus to the sacrum. Surgery followed by radiotherapy is the standard treatment. As chordomas are resistant to standard chemotherapy, further treatment options are urgently needed. We describe the establishment of a clivus chordoma cell line, MUG-CC1. The cell line is characterized according to its morphology, immunohistochemistry, and growth kinetics. During establishment, cell culture supernatants were collected, and the growth factors HGF, SDF-1, FGF2, and PDGF analyzed using xMAP(®) technology. A spontaneous lymphoblastoid EBV-positive cell line was also developed and characterized. MUG-CC1 is strongly positive for brachyury, cytokeratin, and S100. The cell line showed gains of the entire chromosomes 7, 8, 12, 13, 16, 18, and 20, and high level gains on chromosomes 1q21-1q24 and 17q21-17q25. During cultivation, there was significant expression of HGF and SDF-1 compared to continuous chordoma cell lines. A new, well-characterized clival chordoma cell line, as well as a non-tumorigenic lymphoblastoid cell line should serve as an in vitro model for the development of potential new treatment strategies for patients suffering from this disease.

Feasibility of piezoelectric endoscopic transsphenoidal craniotomy: a cadaveric study.

OBJECTIVE: Endoscopic transsphenoidal approach has become the gold standard for surgical treatment of treating pituitary adenomas or other lesions in that area. Opening of bony skull base has been performed with burrs, chisels, and hammers or standard instruments like punches and circular top knives. The creation of primary bone flaps-as in external craniotomies-is difficult.The piezoelectric osteotomes used in the present study allows creating a bone flap for endoscopic transnasal approaches in certain areas. The aim of this study was to prove the feasibility of piezoelectric endoscopic transnasal craniotomies. Study Design. Cadaveric study. METHODS: On cadaveric specimens (N = 5), a piezoelectric system with specially designed hardware for endonasal application was applied and endoscopic transsphenoidal craniotomies at the sellar floor, tuberculum sellae, and planum sphenoidale were performed up to a size of 3-5 cm(2). RESULTS: Bone flaps could be created without fracturing with the piezoosteotome and could be reimplanted. Endoscopic handling was unproblematic and time required was not exceeding standard procedures. CONCLUSION: In a cadaveric model, the piezoelectric endoscopic transsphenoidal craniotomy (PETC) is technically feasible. This technique allows the surgeon to create a bone flap in endoscopic transnasal approaches similar to existing standard transcranial craniotomies. Future trials will focus on skull base reconstruction using this bone flap.

Intraoperative medialization of medial rectus muscle as a new endoscopic technique for approaching intraconal lesions.

BACKGROUND: Intraconal tumors of the orbit are rare entities and surgical treatment is challenging. Endoscopic transnasal approaches to the orbit offer a new perspective for surgery, although only few reports exist in literature. This study displays the Graz experience with endoscopic approaches to intraorbital tumors between 2006 and 2010 introducing a novel endoscopic technique for temporary medialization of the medial rectus muscle facilitating access to the orbital cone. METHODS: A retrospective analysis of patients' charts was performed. RESULTS: For approaches to intraconal lesions a special endoscopic temporary medialization technique of the medial rectus muscle through applying transseptal sutures was developed. Six patients (four male and two female patients) have been included in this study presenting with intraconal/intraorbital tumors. Three patients underwent endoscopic surgery for two hemangiomas and one Schwannoma, respectively, and three patients were successfully biopsied endoscopically revealing one malignant melanoma, one malignant lymphoma, and one optic glioma each. Both hemangiomas were completely resected without any deterioration of vision. The Schwannoma was partially resected with postoperative imaging showing no tumor progression within 3 months. No intraoperative complications occurred. Five cases were performed with computer assisted surgery using CT/MR fusion navigation. CONCLUSION: Although technically challenging, the endoscopic approach to the orbit, even for intraconal lesions with medialization of the medial rectus muscle, can be safe and promising for well-selected cases. Good postoperative results and sufficient material acquisition for proper histological examination can be obtained. Advantages are good visualization of the surgical field and avoidance of external scars. Limitations to endoscopic techniques are tumors in the lateral superior and lateral inferior quadrant of the orbit.

Aggressive inverted papilloma with intracranial invasion and short malignization time.

Inverted papillomas (IP) are considered benign lesions with a prevalence up to 4% among all sinunasal tumors; however, invasive growth and varying tendency for malignization are reported in literature. We report the case of a 69-year-old woman suffering from a large, aggressively growing IP invading the orbit, skull base, and frontal lobe of the brain. Within only 3 months' time the papilloma showed transformation into an invasive carcinoma, leaving surgical therapy in vain due to explosive recurrence. Intracranial and intraorbital expansion by IP is possible despite histology not showing signs of malignancy initially. In "regular" IP close endoscopic follow-up is mandatory to not overlook recurrence harboring malignancy.

European position paper on endoscopic management of tumours of the nose, paranasal sinuses and skull base.

Tumours affecting the nose, paranasal sinuses and adjacent skull base are fortunately rare. However, they pose significant problems of management due their late presentation and juxtaposition to important anatomical structures such eye and brain. The increasing application of endonasal endoscopic techniques to their excision offers potentially similar scales of resection but with reduced morbidity. The present document is intended to be a state-of-the art review for any specialist with an interest in this area 1. to update their knowledge of neoplasia affecting the nose, paranasal sinuses and adjacent skull base; 2. to provide an evidence-based review of the diagnostic methods; 3. to provide an evidence-based review of endoscopic techniques in the context of other available treatments; 4. to propose algorithms for the management of the disease; 5. to propose guidance for outcome measurements for research and encourage prospective collection of data. The importance of a multidisciplinary approach, adherence to oncologic principles with intent to cure and need for long-term follow-up is emphasised.

Lymphocytic hypophysitis in the pediatric population.

BACKGROUND: Lymphocytic hypophysitis (LYH) is a rare inflammatory disease of the pituitary gland that usually affects women in their ante- or immediate postpartum period; males are affected less frequently than females. An autoimmune pathogenesis is suggested. Symptoms comprise anterior and/or posterior pituitary insufficiency of varying degrees. So far, specific characteristics of this rare disease in childhood are not well described. CASE HISTORY: We report the case of a 12-year-and-11-month-old boy with histologically confirmed LYH clinically presenting with diabetes insipidus. A high-dose steroid therapy was administered, in which the therapeutic effect was uncertain. His 6-year follow-up is presented. DISCUSSION: The literature is reviewed for children presenting with LYH; their characteristics, pituitary involvement, and clinical follow-up are listed and discussed. CONCLUSION: Even though magnetic resonance imaging can be highly suspicious for LYH, only surgical exploration can confirm the diagnosis. The efficacy of medical treatment is still controversial; a close follow-up is necessary to control and correct the endocrinological function, if required.

Chiari I malformation and intra-cranial hypertension:a case-based review.

OBJECTIVE: To present clinical and morphological findings before and after surgery in a child with Chairi I malformation (CMI) and intra-cranial hypertension (IH). The literature is reviewed and pathophysiologic factors are discussed. CLINICAL PRESENTATION: A 13-year-old obese boy with a 3-week history of headaches, neck pain, torticollis and progressive visual deterioration was admitted. Bi-lateral chronic papilloedema and decrease in visual acuity were found in the presence of a previously diagnosed CMI. INTERVENTION AND FOLLOW-UP: Intra-cranial pressure monitoring demonstrating increased pressure levels was followed by a sub-occipital decompression, C1 laminectomy and duroplasty. Post-operatively, the boy improved markedly, the 6 months follow-up opthalmological examination demonstrated resolution of papilloedema, but consecutive bi-lateral optic nerve atrophy. CONCLUSION: IH with progressive visual deterioration represents one of the varying clinical presentations of CMI and may be classified as a secondary form of idiopathic IH. Neuro-ophthalmological examination in all patients with CMI is recommended to identify the real incidence of this presentation. Altered CSF dynamics, venous hypertension and obesity as co-factors may be causative pathophysiologic factors.