The impact of social determinants of health on early outcomes after adult Chiari surgery.

We sought to identify social determinants of health (SDoH) for adult patients undergoing Chiari decompression surgery and to analyze their association with postoperative outcomes, including length of stay (LOS), return to the system within 30 days, and the Chicago Chiari Outcomes Score (CCOS). This is a retrospective study of adult patients who underwent Chiari decompression surgery between June 2021 and January 2023. Data was gathered through electronic medical record review and telephone surveys. Descriptive statistics were used to evaluate demographics of all patients meeting inclusion criteria. Fisher's exact tests and logistic regression were used for data analysis. A total of 37 patients underwent Chiari decompression (23 CCOS/SDoH survey respondents): 48% bony decompression only, 30% bony decompression plus intradural exploration, and 22% occipitocervical fusion. Seven patients (30%) had a LOS > 2 days, 1 patient (4%) required inpatient rehabilitation postoperatively, 4 patients (17%) returned to the system within 30 days, 10 patients (43%) had an extremely favorable CCOS (15-16), and 11 patients (48%) reported interaction with a Chiari support group. Mean follow-up was 9.5 months. Patients with occipitocervical fusion were more likely to have a LOS > 2 days (p = 0.03), patients who exercised ≥ 3 days per week were more likely to have a favorable CCOS (p = 0.04), and patients who participated in a Chiari support group were less likely to have a favorable CCOS (p = 0.03). Chiari decompression plus occipitocervical fusion may be associated with increased LOS. While more frequent exercise may be associated with better post-surgical outcomes, participation in a Chiari support group may be correlated with worse outcomes.

Bilateral cerebellopontine angle lipomas in an infant with encephalocele: illustrative case.

BACKGROUND: Bilateral cerebellopontine angle (CPA) lipomas are extremely rare. Herein the authors present a case of bilateral CPA lipomas in an infant along with a literature review of bilateral CPA lipomas. OBSERVATIONS: A newborn girl was incidentally found to have bilateral CPA lipomas during the workup for an occipital encephalocele. The encephalocele was repaired primarily on day 2 after birth. The patient demonstrated no symptoms associated with the bilateral CPA lipomas. Eight cases of bilateral CPA lipomas were identified in the literature review and are summarized. Conservative management is the consensus strategy, given minimum growth of the tumor and the high risk of surgical intervention. LESSONS: This is the first reported case of bilateral CPA lipomas in an infant as well as the first with a coexisting intracranial malformation. Intracranial lipomas share an extremely low growth rate and typically do not cause severe symptoms. The management of asymptomatic or mildly symptomatic bilateral CPA lipomas is usually conservative.

The evolution of stereoelectroencephalography: symbiotic progress in medical imaging and procedural technologies.

Stereoelectroencephalography (sEEG) was pioneered in France, at a time when cerebral anatomy was invisible to contemporaneous imaging modalities. Epilepsy surgeons relied on indirect targeting techniques to identify epileptogenic tissue. Since then, alongside the rapid rise of medical imaging technology, sEEG has experienced dramatic stepwise progress. A flurry of advancements has pushed this technique to its current-day standards, enabling neurosurgeons to access any intracranial location in a safe, highly precise, and expeditious manner. Presently, epilepsy surgeons throughout the world apply robot-assisted sEEG. Herein, the authors chronicle this incredible evolution.

Open Surgical Approaches for Meningiomas.

Meningiomas are the most common intracranial extra-axial primary tumor. Although most are low grade and slow growing, resection can be technically challenging, particularly when located at the skull base. Appropriate craniotomy and approach selection are of paramount importance to minimize brain retraction, optimize exposure, and achieve complete resection. This article summarizes various craniotomies and their approaches to meningiomas, and illustrates some nuances in performing these techniques with cadaveric dissection and operative videos.

Meningioma resection and venous thromboembolism incidence, management, and outcomes.

Background: Meningioma resection is associated with the risk of venous thromboembolism (VTE). Objectives: To determine the incidence and risk factors for VTE following meningioma resection and VTE outcomes based on the type and timing of anticoagulation. Methods: From 2011 to 2019, 901 consecutive patients underwent meningioma resection. We retrospectively evaluated the postoperative incidence of VTE and bleeding. For VTE, we determined the treatment strategy and rate of VTE complications and bleeding. Results: Pharmacologic prophylaxis was administered to 665 (73.8%) patients. The cumulative incidence for total postoperative VTE was 8.7% (95% CI: 6.9%-10.6%), and for symptomatic VTE was 6.0% (95% CI: 4.6%-7.7%). A multivariable model identified the following independent predictors of symptomatic VTE: history of VTE, obesity, and lack of pharmacologic prophylaxis. Following postoperative VTE, 58 (74.3%) patients received therapeutic anticoagulation either initially (33.3%) or after a median delay of 23.5 days (41.0%). Symptomatic recurrent VTE occurred in 13 (16.6%) patients. Following VTE, the use of subtherapeutic anticoagulation was associated with a lower rate of total VTE extension than no anticoagulation (17.5% vs 42.9%, OR 0.28, 95% CI: 0.09-0.93). In total, 14 patients (1.6%) experienced clinically relevant bleeding: 4 received therapeutic anticoagulants, 8 received prophylactic anticoagulation, and 2 received no anticoagulation. Among patients with VTE, 4 (5.1%) experienced bleeding. Conclusion: Recognition of risk factors for VTE following meningioma resection may help improve approaches to thromboprophylaxis. The management of postoperative VTE is highly variable, but most VTE patients are ultimately treated with therapeutic anticoagulants.

Specific gene expression signatures of low grade meningiomas.

Introduction: Meningiomas are the most common primary central nervous system (CNS) tumors in adults, representing approximately one-third of all primary adult CNS tumors. Although several recent publications have proposed alternative grading systems of meningiomas that incorporate genomic and/or epigenomic data to better predict meningioma recurrence and progression-free survival, our understanding of driving forces of meningioma development is still limited. Objective: To define gene expression signatures of the most common subtypes of meningiomas to better understand cellular processes and signaling pathways specific for each tumor genotype. Methods: We used RNA sequencing (RNA-seq) to determine whole transcriptome profiles of twenty meningiomas with genomic alterations including NF2 inactivation, loss of chr1p, and missense mutations in TRAF7, AKT1 and KLF4. Results: The analysis revealed that meningiomas with NF2 gene inactivation expressed higher levels of BCL2 and GLI1 compared with tumors harboring TRAF7 missense mutations. Moreover, NF2 meningiomas were subdivided into two distinct groups based on additional loss of chr1p. NF2 tumors with intact chr1p were characterized by the high expression of tumor suppressor PTCH2 compared to NF2 tumors with chr1p loss. Taken together with the high expression of BCL2 and GLI1, these results suggest that activation of Sonic Hedgehog pathway may contribute to NF2 meningioma development. In contrast, NF2 tumors with chr1p loss expressed high levels of transcription factor FOXD3 and its antisense RNA FOXD3-AS1. Examination of TRAF7 tumors demonstrated that TRAF7 regulates a number of biomechanically responsive genes (KRT6a, KRT16, IL1RL1, and AQP3 among others). Interestingly, AKT1 and KLF4 meningiomas expressed genes specific for PI3K/AKT signaling pathway, suggesting overlapping gene signatures between the two subtypes. In addition, KLF4 meningiomas had high expression of carcinoembryonic antigen family members CEACAM6 and CEACAM5. Conclusions: Each group of meningiomas displayed a unique gene expression signature suggesting signaling pathways potentially implicated in tumorigenesis. These findings will improve our understanding of meningioma tumorigenesis and prognosis.

Epidural electrical stimulation of the cervical spinal cord opposes opioid-induced respiratory depression.

Opioid overdose suppresses brainstem respiratory circuits, causes apnoea and may result in death. Epidural electrical stimulation (EES) at the cervical spinal cord facilitated motor activity in rodents and humans, and we hypothesized that EES of the cervical spinal cord could antagonize opioid-induced respiratory depression in humans. Eighteen patients requiring surgical access to the dorsal surface of the spinal cord between C2 and C7 received EES or sham stimulation for up to 90 s at 5 or 30 Hz during complete (OFF-State) or partial suppression (ON-State) of respiration induced by remifentanil. During the ON-State, 30 Hz EES at C4 and 5 Hz EES at C3/4 increased tidal volume and decreased the end-tidal carbon dioxide level compared to pre-stimulation control levels. EES of 5 Hz at C5 and C7 increased respiratory frequency compared to pre-stimulation control levels. In the OFF-State, 30 Hz cervical EES at C3/4 terminated apnoea and induced rhythmic breathing. In cadaveric tissue obtained from a brain bank, more neurons expressed both the neurokinin 1 receptor (NK1R) and somatostatin (SST) in the cervical spinal levels responsive to EES (C3/4, C6 and C7) compared to a region non-responsive to EES (C2). Thus, the capacity of cervical EES to oppose opioid depression of respiration may be mediated by NK1R+/SST+ neurons in the dorsal cervical spinal cord. This study provides proof of principle that cervical EES may provide a novel therapeutic approach to augment respiratory activity when the neural function of the central respiratory circuits is compromised by opioids or other pathological conditions. KEY POINTS: Epidural electrical stimulation (EES) using an implanted spinal cord stimulator (SCS) is an FDA-approved method to manage chronic pain. We tested the hypothesis that cervical EES facilitates respiration during administration of opioids in 18 human subjects who were treated with low-dose remifentanil that suppressed respiration (ON-State) or high-dose remifentanil that completely inhibited breathing (OFF-State) during the course of cervical surgery. Dorsal cervical EES of the spinal cord augmented the respiratory tidal volume or increased the respiratory frequency, and the response to EES varied as a function of the stimulation frequency (5 or 30 Hz) and the cervical level stimulated (C2-C7). Short, continuous cervical EES restored a cyclic breathing pattern (eupnoea) in the OFF-State, suggesting that cervical EES reversed the opioid-induced respiratory depression. These findings add to our understanding of respiratory pattern modulation and suggest a novel mechanism to oppose the respiratory depression caused by opioids.

A molecularly integrated grade for meningioma.

BACKGROUND: Meningiomas are the most common primary intracranial tumor in adults. Clinical care is currently guided by the World Health Organization (WHO) grade assigned to meningiomas, a 3-tiered grading system based on histopathology features, as well as extent of surgical resection. Clinical behavior, however, often fails to conform to the WHO grade. Additional prognostic information is needed to optimize patient management. METHODS: We evaluated whether chromosomal copy-number data improved prediction of time-to-recurrence for patients with meningioma who were treated with surgery, relative to the WHO schema. The models were developed using Cox proportional hazards, random survival forest, and gradient boosting in a discovery cohort of 527 meningioma patients and validated in 2 independent cohorts of 172 meningioma patients characterized by orthogonal genomic platforms. RESULTS: We developed a 3-tiered grading scheme (Integrated Grades 1-3), which incorporated mitotic count and loss of chromosome 1p, 3p, 4, 6, 10, 14q, 18, 19, or CDKN2A. 32% of meningiomas reclassified to either a lower-risk or higher-risk Integrated Grade compared to their assigned WHO grade. The Integrated Grade more accurately identified meningioma patients at risk for recurrence, relative to the WHO grade, as determined by time-dependent area under the curve, average precision, and the Brier score. CONCLUSION: We propose a molecularly integrated grading scheme for meningiomas that significantly improves upon the current WHO grading system in prediction of progression-free survival. This framework can be broadly adopted by clinicians with relative ease using widely available genomic technologies and presents an advance in the care of meningioma patients.

Cyst Type Differentiates Rathke Cleft Cysts From Cystic Pituitary Adenomas.

PURPOSE: A classification system for cystic sellar lesions does not exist. We propose a novel classification scheme for these lesions based on the heterogeneity of the cyst wall/contents and the presence of a solid component on imaging. METHODS: We retrospectively reviewed 205 patients' medical records (2008-2020) who underwent primary surgery for a cystic sellar lesion. Cysts were classified a priori into 1 of 4 cyst types based on the heterogeneity of the cyst wall/contents and the presence of a solid component imaging. There was high interrater reliability. Univariable and multivariable models were used to estimate the ability of cyst type to predict the two most common diagnoses: Rathke cleft cyst (RCC) and cystic pituitary adenoma. RESULTS: The frequencies of RCC and cystic pituitary adenoma in our cohort were 45.4% and 36.4%, respectively. Non-neoplastic lesions (e.g., arachnoid cysts and RCC) were more likely to be Type 1 or 2, whereas cystic neoplasms (e.g., pituitary adenomas and craniopharyngiomas) were more likely to be Type 3 or 4 (p<0.0001). Higher cyst types, compared to Type 1, had higher odds of being cystic pituitary adenomas compared to RCCs (OR: 23.7, p=0.033, and 342.6, p <0.0001, for Types 2 and 4, respectively). Lesions with a fluid-fluid level on preoperative MRI also had higher odds of being pituitary adenomas (OR: 12.7; p=0.023). Cystic pituitary adenomas were more common in patients with obesity (OR: 5.0, p=0.003) or symptomatic hyperprolactinemia (OR: 11.5; p<0.001, respectively). The multivariable model had a positive predictive value of 82.2% and negative predictive value of 86.4%. CONCLUSION: When applied to the diagnosis of RCC versus cystic pituitary adenoma, higher cystic lesion types (Type 2 & 4), presence of fluid-fluid level, symptomatic hyperprolactinemia, and obesity were predictors of cystic pituitary adenoma. Further validation is needed, but this classification scheme may prove to be a useful tool for the management of patients with common sellar pathology.

Silent corticotroph pituitary adenomas: clinical characteristics, long-term outcomes, and management of disease recurrence.

OBJECTIVE: Silent corticotroph adenomas (SCAs) are a distinct subtype of nonfunctioning pituitary adenomas (NFAs) that demonstrate positive immunohistochemistry for adrenocorticotropic hormone (ACTH) without causing Cushing's disease. SCAs are hypothesized to exhibit more aggressive behavior than standard NFAs. The authors analyzed their institution's surgical experience with SCAs in an effort to characterize rates of invasion, postoperative clinical outcomes, and patterns of disease recurrence and progression. The secondary objectives were to define the best treatment strategies in the event of tumor recurrence and progression. METHODS: A retrospective analysis of patients treated at the authors' institution identified 100 patients with SCAs and 841 patients with NFAs of other subtypes who were treated surgically from 2000 to 2019. Patient demographics, tumor characteristics, surgical and neuroimaging data, rates of endocrinopathy, and neurological outcomes were recorded. Cohorts of patients with SCAs and patients with standard NFAs were compared with regard to these characteristics and outcomes. RESULTS: The SCA cohort presented with cranial neuropathy (13% vs 5.7%, p = 0.0051) and headache (53% vs 42.3%, p = 0.042) compared to the NFA cohort, despite similar rates of apoplexy. The SCA cohort included a higher proportion of women (SCA 60% vs NFA 45.8%, p = 0.0071) and younger age at presentation (SCA 50.5 ± 13.3 vs NFA 54.6 ± 14.9 years of age, p = 0.0082). Reoperations were comparable between the cohorts (SCA 16% vs NFA 15.7%, p = 0.98). Preoperative pituitary function was comparable between the cohorts with the exception of higher rates of preoperative panhypopituitarism in NFA patients (2% vs 6.1%, respectively; p = 0.0033). The mean tumor diameter in SCA patients was 24 ± 10.8 mm compared to 26 ± 11.3 mm in NFA patients (p = 0.05). Rates of cavernous sinus invasion were higher in the SCA group (56% vs 49.7%), although this result did not reach statistical significance. There were no significant differences in extent of resection, intraoperative CSF leak rates, endocrine or neurological outcomes, or postoperative complications. Ki-67 rates were significantly increased in the SCA cohort (2.88 ± 2.79) compared to the NFA cohort (1.94 ± 1.99) (p = 0.015). Although no differences in overall rates of progression or recurrence were noted, SCAs had a significantly lower progression-free survival (24.5 vs 51.1 months, p = 0.0011). Among the SCA cohort, progression was noted despite the use of adjuvant radiosurgery in 33% (n = 4/12) of treated tumors. Adequate tumor control was not achieved in half (n = 6) of the SCA progression cohort despite radiosurgery or multiple resections. CONCLUSIONS: In this study, to the authors' knowledge the largest surgical series to assess outcomes in SCAs to date, the findings suggest that SCAs are more biologically aggressive tumors than standard NFAs. The progression-free survival duration of patients with SCAs is only about half that of patients with other NFAs. Therefore, close neuroimaging and clinical follow-up are warranted in patients with SCAs, and residual disease should be considered for early postoperative adjuvant radiosurgery, particularly in younger patients.

Predictors of postoperative biochemical remission in acromegaly.

PURPOSE: Acromegaly is a rare neuroendocrine condition that can lead to significant morbidity. Despite China's vast population size, studies on acromegaly remain sparse. This study aimed to investigate the clinical characteristics and predictors of biochemical remission after surgery for acromegaly using the China Acromegaly Patient Association (CAPA) database. METHODS: A retrospective nationwide study was conducted using patient-reported data from CAPA database between 1998 and 2018. The principal component analysis (PCA) and logistic regression analysis were employed to determine independent predictors of biochemical remission at 3 months in patients after surgery. RESULTS: Of the 546 surgical cases (mean age: 36.8 years; 59.5% females), macroadenomas and invasive tumors (Knosp score 3-4) were 83.9% and 64.1%, respectively. Ninety-five percent of patients were treated with endonasal surgery and 36.8% exhibited biochemical remission at 3-months postoperatively. The following independent predictors of biochemical remission were identified: preoperative growth hormone (GH) levels between 12 and 28 µg/L [odds ratio (OR) = 0.58; 95% confidence interval (CI), 0.37-0.92; p = 0.021], preoperative GH levels > 28 µg/L (OR = 0.55; 95% CI, 0.34-0.88; p = 0.013), macroadenoma (OR = 0.56; 95% CI, 0.32-0.96; p = 0.034), giant adenomas (OR = 0.14; 95% CI, 0.05-0.38; p < 0.001), Knosp score 3-4 (OR = 0.37; 95% CI, 0.24-0.57; p < 0.001), and preoperative medication usage (OR = 2.32; 95% CI, 1.46-3.70; p < 0.001). CONCLUSIONS: In this nationwide study spanning over two decades, we highlight that higher preoperative GH levels, large tumor size, and greater extent of tumor invasiveness are associated with a lower likelihood of biochemical remission at 3-months after surgery, while preoperative medical therapy increases the chance of remission.

Utilizing Technology for Dermatology Care in Tajikistan: A Health Systems Perspective.

In Tajikistan, dermatologic services are available across the country. Yet, the most experienced dermatologists work at the National Republic Center for Dermatology and Venereology (NRCDV). Patients from across the country bypass local dermatologists and self-refer to NRCDV. Furthermore, no formal mechanisms exist for dermatologists in different cities to consult with experts at NRCDV. The authors designed a teledermatology program linking dermatologists across Tajikistan to NRCDV. They used the World Health Organization health systems framework to plan this program and define objectives. To date, 228 teledermatology consultations have taken place. The authors find that good governance is key to program implementation.

Corticotroph hyperplasia and Cushing disease: diagnostic features and surgical management.

OBJECTIVE: This study was done to compare corticotroph hyperplasia and histopathologically proven adenomas in patients with Cushing disease by analyzing diagnostic features, surgical management, and clinical outcomes. METHODS: Patients with suspected pituitary Cushing disease were included in a retrospective cohort study and were excluded if results of pathological analysis of the surgical specimen were nondiagnostic or normal. Cases were reviewed by two experienced neuropathologists. Total lesion removal was used as a dichotomized surgical variable; it was defined as an extracapsular resection (including a rim of normal gland) in patients with an adenoma, and for hyperplasia patients it was defined as removal of the presumed lesion plus a rim of surrounding normal gland. Bivariate and multivariate analyses were performed. Recurrence-free survival was compared between the two groups. RESULTS: The final cohort consisted of 63 patients (15 with hyperplasia and 48 with adenoma). Normal pituitary acinar architecture was highly variable. Corticotroph hyperplasia was diagnosed based on the presence of expanded acini showing retained reticulin architecture and predominant staining for adrenocorticotropic hormone. Crooke's hyaline change was seen in 46.7% of specimens, and its frequency was equal in nonlesional tissue of both groups. The two groups differed only by MRI findings (equivocal/diffuse lesion in 46% of hyperplasia and 17% of adenoma; p = 0.03). Diagnostic uncertainty in the hyperplasia group resulted in additional confirmatory testing by 24-hour urinary free cortisol. Total lesion removal was infrequent in patients with hyperplasia compared to those with adenoma (33% vs 65%; p = 0.03). Initial biochemical remission was similar (67% in hyperplasia and 85% in adenoma; p = 0.11). There was no difference in hypothalamic-pituitary-adrenal axis recovery or disease recurrence. The median follow-up was 1.9 years (IQR 0.7-7.6 years) for the hyperplasia group and 1.2 years (IQR 0.4-2.4 years) for the adenoma group. Lack of a discrete lesion and diagnostic uncertainty were the only significant predictors of hyperplasia (sensitivity 53.3%, specificity 97.7%, positive predictive value 88.9%, negative predictive value 85.7%). An adjusted Cox proportional hazards model showed similar recurrence-free survival in the two groups. CONCLUSIONS: This study suggests an association between biochemically proven Cushing disease and histopathologically proven corticotroph hyperplasia. Imaging and operative findings can be ambiguous, and, compared to typical adenomas with a pseudocapsule, the surgical approach is more nuanced. Nevertheless, if treated appropriately, biochemical outcomes may be similar.

Calcified Prolactinoma of the Pituitary Gland: Illustrative Case Reports Highlighting Medical versus Surgical Intervention.

The presence of calcification is uncommon in pituitary adenomas, and often lends support to other diagnoses including craniopharyngioma. The majority of calcified pituitary adenomas are prolactin-secreting tumors. We report two patients with calcified macroprolactinomas, one that was treated medically with a biochemical response and partial tumor response, and one that was treated successfully via an endoscopic endonasal transsphenoidal approach. Suspected calcified prolactinomas can be initially managed medically as per standard treatment for typical prolactinomas; however, the presence of diffuse calcification may hinder tumor shrinkage. Tumors that are refractory to medical treatment can be safely managed with surgery.

Effectiveness of Gamma Knife Radiosurgery in the Treatment of Refractory Trigeminal Neuralgia: A Case Series.

BACKGROUND: Medical management is the first line of treatment for trigeminal neuralgia (TN). Patients with medically refractory TN may undergo a variety of invasive surgical interventions with varying success rates. Management of TN refractory to both medical and surgical intervention remains somewhat controversial. OBJECTIVE: To assess the effectiveness of Gamma Knife radiosurgery (GKRS; Elekta Instruments AB) for medically refractory TN. METHODS: A retrospective review was conducted for 57 cases (47 patients) who underwent GKRS for refractory TN at our institution between 2005 and 2018. TN pain outcomes were evaluated using the Barrow Neurological Institute (BNI) Pain Scale. A good outcome was defined by post-GKRS BNI score of I-III, whereas treatment failure was defined BNI score IV-V. RESULTS: Of the total 57 GKRS procedures, 47 (82.5%) had good outcomes. A total of 22 patients (46.8%) experienced complete pain relief off medications (BNI I). The average time to pain relief was 30 d (range 1-120 d). Prior invasive surgical treatment for TN was not found to have a significant impact on GKRS outcomes (P = .32). Target and treatment volumes were not found to correlate significantly with GKRS outcomes (.47 and .47, respectively). Complications included 2 cases (4.2%) of facial numbness. A total of 37 patients (78.7%) did not have any additional invasive surgical interventions following GKRS treatment. CONCLUSION: GKRS is a safe and effective treatment modality for both medically and surgically refractory TN. Complete symptom relief was possible in patients with prior surgical or GKRS treatments. Recurrent symptoms following surgery or GKRS should not exclude a patient from future GKRS consideration.

Clinical utility of routine postoperative morning cortisol monitoring in detecting new hypothalamic-pituitary-adrenal axis insufficiency following endoscopic transsphenoidal surgery for sellar lesions.

OBJECTIVE: Hypothalamic-pituitary-adrenal (HPA) axis dysfunction is a well-documented complication of transsphenoidal craniotomy (TSC) for sellar lesions. The authors aimed to assess their multidisciplinary approach to the diagnosis and treatment of postoperative hypocortisolemia utilizing conservative screening methods. METHODS: The authors performed a retrospective review of 257 patients who underwent TSC for pituitary adenoma (PA) or Rathke cleft cyst (RCC) at the University of Southern California between 2012 and 2017. Patients with preoperative adrenal insufficiency, Cushing's disease, or < 3 months of postoperative follow-up were excluded. Patient demographics, pathology, tumor characteristics, and complications were recorded. Postoperative day 1 (POD1) morning serum cortisol was assessed in all patients. Hypocortisolemia on POD1 (serum cortisol < 5 µg/dl) prompted a 7 am cortisol level measurement on POD 2 (POD2). Clinical signs and symptoms of hypocortisolemia were consistently monitored. After two serum cortisol levels < 5 µg/dl, or one serum level < 5 µg/dl plus a high clinical suspicion for HPA dysfunction, high-risk patients received glucocorticoid supplementation. RESULTS: Data on 165 patients were included in the analysis; there were 101 women (61.2%) and 64 men (38.7%). Preoperative diagnoses included nonfunctional adenoma (n = 97, 58.7%), growth hormone-secreting adenoma (n = 37, 22.4%), RCC (n = 18, 10.9%), prolactinoma (n = 8, 4.8%), and other (n = 5, 3.0%). One hundred thirty-eight patients (63.0%) had either suprasellar extension or cavernous sinus invasion. POD1 hypocortisolemia was diagnosed in 8 patients (4.8%). Of these patients, 2 (1.2%) were clinically asymptomatic and had normalized POD2 cortisol levels. Six patients (3.6%) had clinical symptoms and POD2 cortisol levels confirming HPA axis deficiency. Of these 6 patients treated with early glucocorticoid replacement, 2 patients recovered HPA axis function during follow-up, making the incidence of new, permanent HPA axis deficiency 2.5%. CONCLUSIONS: In the authors' institutional review, all patients warranting postoperative glucocorticoid replacement had both complicated surgical courses and associated clinical symptoms of hypocortisolemia. The authors' algorithm of withholding steroids until patients demonstrate clear evidence of postoperative hypocortisolemia is safe and clinically efficacious. Their data further suggest that routine postoperative cortisol screening may not be necessary following an uncomplicated operative resection, with gland preservation and the absence of clinical symptoms indicative of HPA dysfunction.

Congenital Vomer Agenesis: Report of Two Cases.

INTRODUCTION: Congenital vomer agenesis is an extremely rare condition in which the vomer bone does not fully develop, which can lead to septal perforation. CASE REPORT: We report two cases with a defect in the vomer bone in the posteroinferior portion of the septum, found accidentally while performing a pre-operative CT scan for nasal obstruction evaluation. They were diagnosed with congenital vomer agenesis. CONCLUSION: There are afew reports of vomer agenesis in literatures. By increasing usage of sinonasal endoscopic examination,we expect to address more cases in the future.

Acute invasive fungal rhinosinusitis: our experience with 18 cases.

Acute invasive fungal rhinosinusitis (AIFRS) is a rapidly progressive life threatening infection that is seen most commonly among immunocompromised patients. We present a case series of 18 patients clinically and histopathologically diagnosed with AIFRS with a mean follow-up of 9.11 ± 2.51 months (range 6-17). Demographic data, apparent symptoms and signs, underlying disorders, and outcomes are discussed. The mean age was 39.56 ± 20.66 years (range 2-75). The most common underlying diseases were diabetes mellitus (50 %) and leukemia (44.44 %). Mucosal biopsy confirmed fungal invasion of the nasal mucosa in all cases. The main fungi were Rhizopus oryzae (55.56 %), Absidia mucor (16.67 %), and Aspergillus fumigatus (27.78 %). Headache and facial pain (77.8 %), facial paresthesia (55.6 %), and ophthalmoplegia (33.3 %) were the most common symptoms and signs. Computed tomography and endoscopic findings showed various stages of sinonasal (100 %), pterygopalatine fossa (55.56 %), orbital (44.45 %), and cerebral (5.56 %) involvement. All patients underwent serial surgical debridement (3.78 ± 1.80 times; range 2-8) simultaneously with systemic antifungal therapy and proper management of the underlying disease. The most extreme case with brain involvement survived and recovered with no evidence of recurrent disease following treatment. All patients were considered cured after two endoscopic negative histopathologic evaluations. Three patients (16.67 %) died, one from uncontrolled leukemia and two due to renal failure. AIFRS is a potentially fatal condition, however, early diagnosis and management of the underlying disease accompanied with systemic antifungal and aggressive serial surgical intervention appears to be effective in reducing mortality in most patients.