The first pineoblastoma case report of a patient with Sotos syndrome harboring NSD1 germline mutation.

Germline mutations of NSD1 are associated with Sotos syndrome, characterized by distinctive facial features, overgrowth, and developmental delay. Approximately 3% of individuals with Sotos syndrome develop tumors. In this study, we describe an infant in pineoblastoma with facial anomalies, learning disability and mild autism at 1 years diagnosed as Sotos syndrome owing to carrying a novel mutation de novo germline NSD1 likely pathogenic variant. This patient expands both the mutation and phenotype spectrum of the Sotos Syndrome and provides new clinical insights into the potential mechanism of underlying pinealoblastoma pathology.

Simultaneous Single-Trajectory Endoscopic Biopsy and Third Ventriculostomy in Pineal Region Tumors: A Systematic Review and Single Arm Meta-Analysis.

OBJECTIVE: To assess the efficacy and surgical outcomes of the simultaneous single-trajectory endoscopic biopsy and third ventriculostomy (ETV) in pineal region tumors. METHODS: A systematic review and meta-analysis adhering to Cochrane Standards and PRISMA framework were conducted. PubMed, Embase, and Web Of Science databases were searched until December 2023. Outcomes included rate of histopathologic diagnosis success, ETV success, complications, required VPS, and mortality. RESULTS: Seventeen studies (N = 388) met inclusion criteria. Histopathologic diagnosis success rate was 90% for general population (95% CI: 86%-95%; I2 = 42%) and 94% for pediatric patients (95% CI: 89%-98%; I2 = 19%). ETV Success rate was 93% (95% CI: 88%-97%; I2 = 60%). An estimated risk of postoperative ETV complications was found to be 16% for the general population (95% CI: 5%-28%; I2 = 90%) and 5% for pediatric patients (95% CI: 0%-13%; I2 = 51%). The risk of requiring VPS was estimated as 2% (95% CI: 0%-4%; I2 = 39%) and for the pediatric population it was 7% (95% CI: 0%-16%; I2 = 69%). Mortality risk was found to be 1% (95% CI: 0%-3%; I2 = 0%). CONCLUSIONS: Simultaneous endoscopic biopsy and ETV demonstrated high diagnostic and therapeutic success rates. The procedure's safety profile, with low mortality and complications, supports its role in treating hydrocephalus associated to pineal region tumors. Subgroup analyses revealed higher diagnostic success rates and required VPS in the pediatric population, whilst it had lower complication rates.

Neurosurgical application of pineal region tumor resection with 3D 4K exoscopy via infratentorial approach: a retrospective cohort study.

BACKGROUND: The pineal region tumors are challenging for neurosurgeons and can lead to secondary hydrocephalus. The introduction of the exoscope has provided clinical interventions with high image quality and an ergonomic system for pineal region tumor operations. In this study, the authors describe the exoscopic approach used to facilitate the surgical resection of pineal region tumors and relieve hydrocephalus. MATERIALS AND METHODS: In this retrospective cohort study, we consecutively reviewed the clinical and radiological data of 25 patients with pineal region lesions who underwent three-dimensional exoscopic tumor resection at a single center. RESULTS: The patient cohort consisted of 16 males and 9 females, with an average age of 34.6 years (range, 6-62 years; 8 cases aged ≤18). Pathological examination confirmed eight pineal gland tumors, four gliomas, nine germ cell neoplasms, two ependymomas, and two metastatic tumors. Preoperative hydrocephalus was present in 23 patients. Prior to tumor resection, external ventricular drainage (EVD) with Ommaya reservoir implantation was performed in 17 patients. Two patients received preoperative endoscopic third ventriculostomy (ETV), and five patients received a ventriculoperitoneal (VP) shunt, including one who received both procedures. Gross total resection was achieved in 19 patients (76%) in the 'head-up' park bench position using the exoscope. Eight patients (31.6%) with third ventricle invasion received subtotal resection, mainly in glioma cases, which was higher than those without invasion (0%), but not statistically significant ( P =0.278, Fisher's exact test). No new neurological dysfunction was observed after surgery. Two patients (8%) developed intracranial and pulmonary infections, and two patients (8%) suffered from pneumothorax. Hydrocephalus was significantly relieved in all patients postoperatively, and four patients with relapse hydrocephalus were cured during the long-term follow-up. Postoperative adjuvant management was recommended for indicated patients, and a mean follow-up of 24.8±14.3 months showed a satisfied outcome. CONCLUSIONS: The exoscope is a useful tool for pineal region tumor resection and hydrocephalus relief, particularly with posterior third ventricle invasion, as total resection could be achieved without obvious complication. The special superiority of the exoscope for the indicated pineal region tumors should be highlighted.

Staged operations for a hypervascular mixed germ cell tumor with growing teratoma syndrome: a case report.

INTRODUCTION: A mixed germ cell tumor with a teratoma component can become enlarged following chemotherapy, and such an event is diagnosed as growing teratoma syndrome. Removing large, hypervascular tumors including a tumor encased by developed vasculatures from the pineal region is challenging during a single operation. CASE REPORT: A 15-year-old male underwent chemotherapy for mixed germ cell tumors according to the KSPNO G082 protocol. This case of a mixed germ cell tumor with growing teratoma syndrome was recognized very early during chemotherapy. The tumor was completely removed during the staged operations. First, the anteriorly located tumor on the third ventricle was removed via the transcallosal interforniceal approach, and 1 month later, the occipital transtentorial approach was used for the pineal tumor with decreased vascularity. CONCLUSION: Performing staged operations could be recommended for large hypervascular pineal tumors, which can be safely removed during the second operation once vascularity has decreased.

The Third Eye Sees Double: Cohort Study of Clinical Presentation, Histology, Surgical Approaches, and Ophthalmic Outcomes in Pineal Region Germ Cell Tumors.

BACKGROUND: Intracranial germ cell tumors (GCTs) predominantly occur in the adolescent and young adult population and are most frequently located at the pineal gland. Tumor masses in the pineal region may cause ophthalmic symptoms due to compression to the midbrain, frequently presenting with Parinaud syndrome and hydrocephalus due to aqueductal compression. METHODS: We conducted a single-institution cohort study of primary, pineal region GCTs to characterize the clinical presentation, as well as associated ophthalmic and hydrocephalus outcomes. RESULTS: Fifty-six primary pineal GCTs were identified. Among the 40 isolated pineal region GCTs, 15 were germinomas while 25 were nongerminomatous GCTs. Among 43 cases of hydrocephalus, endoscopic third ventriculostomy was the primary treatment in 27 cases, which was successful in 23 but failed and required additional treatment for the rest. Pineal tumor mass was significantly larger in cases with hydrocephalus compared with those without, and the 20-mm diameter of the tumor was the crucial point for obstructive hydrocephalus. Ophthalmic symptoms were commonly observed at presentation, which included diplopia (74.3%), upward-gaze palsy (69.7%), and Argyll Robertson pupil (40%). These symptoms tended to remain, and the existence of these symptoms at presentation predicted the remaining symptoms in the follow-up. CONCLUSIONS: Intracranial GCTs presenting with ophthalmic abnormalities appear to be at increased risk of residual posttreatment symptoms, while second-look surgery presents a significant risk factor for the development of new deficits. Hydrocephalus often accompanies pineal region GCTs, and in most cases both cerebrospinal fluid diversion and tissue diagnosis can be successfully achieved via endoscopic third ventriculostomy.

Resection of Symptomatic Pineal Cysts Provides Durable Clinical Improvement: A Breakdown of Presenting Symptoms and Lessons Learned.

BACKGROUND: Surgical resection of symptomatic pineal cysts without hydrocephalus remains controversial because patients can present with variable symptoms. Hesitancies in surgical decision-making include determining surgical candidacy and whether results would be durable. METHODS: We performed a retrospective analysis on patients who underwent resection of their pineal cysts in our practice. We examined the presenting symptomology and investigated the radiographic changes to the morphology of the cerebral aqueduct found on follow-up imaging. We examined the clinical outcomes and complications following surgical resection of symptomatic pineal cysts. RESULTS: A total of 97 patients underwent resection of pineal cysts, with 84 patients who had adequate follow-up (mean: 30.5 months). The patient population were predominantly female (76%) presenting at a mean of 24 years of age. Almost half of the patients had headaches that were positional, with 82% being bilateral; 39% and 19% of patients presented with photophobia and sonophobia, respectively, concurrent with their headaches. Many patients presented with visual disturbance (73%) along with other non-headache symptoms. Surgery resulted in 89% of patients with clinical improvements of their headaches. CONCLUSIONS: Pineal cysts can present with variable headache symptomatology. Surgical resection of pineal cysts in carefully selected symptomatic patients after exhaustive conservative management can be performed safely and result in durable symptomatic relief.

Management of Obstructive Hydrocephalus Associated with Pineal Region Cysts and Tumors and Its Implication in Long-Term Outcome.

BACKGROUND: Different treatment options have been proposed for obstructive hydrocephalus associated with pineal lesions. We discuss the obstructive hydrocephalus management associated with pineal region tumors and cysts in Helsinki Neurosurgery. METHODS: In this article, hydrocephalus treatment by tumor-cyst removal (n = 40), shunt surgery (n = 25), and endoscopic ventriculostomies (n = 3) is evaluated in 68 patients with obstructive hydrocephalus among 136 patients with pineal region tumor and cyst. Multivariate statistical analysis was followed by univariate and multivariate regression models of last functional status, last tumor-free imaging, and disease-specific mortality of the study population. RESULTS: Preoperative hydrocephalus was linked to higher World Health Organization tumor grades, poor functional status, higher mortality, and incomplete resection of pineal region cysts and tumors. Preoperative hydrocephalus remained a predictor of poor last functional status after multivariate regression. Pineal lesion removal with the posterior third ventricle opening as primary hydrocephalus treatment resulted in better last functional status, fewer postoperative shunts, fewer hydrocephalus-related procedures, and fewer postoperative infections than in the shunt-treatment group. Multivariate regression analysis linked higher World Health Organization tumor grade, poor immediate functional status, postoperative complications, and incomplete surgical resection as independent predictors of disease mortality in patients with hydrocephalus. Same variables (except immediate modified Rankin Scale score) and higher number of shunt surgeries became independent predictors of poor last functional status at multivariate analysis. Incomplete resection was the only independent predictor of tumor-free magnetic resonance imaging at the last evaluation. CONCLUSIONS: Direct removal of pineal lesions with the opening of the posterior third ventricle could represent effective and reliable management of the associated obstructive hydrocephalus. Further research is required to generalize our inferences.

Endoscopic-assisted surgery versus microsurgery for pineal region tumors: a single-center retrospective study.

Pineal region tumors are extremely deep-seated and surgically challenging. The exposure and visualization obtained by microscopic surgery are relatively limiting. The application of high-definition endoscopes has recently provided neurosurgeons with a much more magnified and clearer view of the anatomy in the pineal region. The present study was performed to compare endoscopic-assisted surgery (ES) with microsurgery (MS) for pineal region tumors. We retrospectively analyzed patients admitted to our hospital for treatment of pineal region tumors from January 2016 to June 2019. All patients consented to undergo tumor resection with ES or MS. We compared the extent of resection, postoperative rate of hydrocephalus, complications, and outcomes between the two groups to estimate the safety and efficacy of ES. In total, 41 patients with pineal region tumors were divided into 2 groups: the ES group (n = 20) and MS group (n = 21). The rate of gross total resection was significantly higher in the ES than MS group (90.0% vs. 57.1%, p = 0.04). The rate of postoperative hydrocephalus was significantly lower in the ES than MS group (11.8% vs. 52.9%, p = 0.03). No significant differences were found in complications or the Karnofsky Performance Score between the two groups. ES can be used to safely and effectively achieve complete resection of pineal region tumors. In patients with obstructive hydrocephalus, ES provides a new way to directly open the aqueduct for cerebrospinal fluid recovery following tumor resection.

Management of pineal region tumors in a pediatric case series.

Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We evaluated our surgical experience concerning procurance of a histological diagnosis, management of hydrocephalus, and choice of antitumoral treatment. We analyzed the efficacy of neuroendoscopic biopsy and endoscopic third ventriculocisternostomy (ETV) in patients with pineal region tumors between 2006 and 2019 in a single-center retrospective cross-sectional study with regard to diagnostic yield, hydrocephalus treatment, as well as impact on further antitumoral management. Out of 28 identified patients, 23 patients presented with untreated hydrocephalus and 25 without histological diagnosis. One patient underwent open biopsy, and 24 received a neuroendoscopic biopsy with concomitant hydrocephalus treatment if necessary. Eighteen primary ETVs, 2 secondary ETVs, and 2 ventriculoperitoneal shunts (VPSs) were performed. Endoscopic biopsy had a diagnostic yield of 95.8% (23/24) and complication rates of 12.5% (transient) and 4.2% (permanent), respectively. ETV for hydrocephalus management was successful in 89.5% (17/19) with a median follow-up of more than 3 years. Following histological diagnosis, 8 patients (28.6%) underwent primary resection of their tumor. Another 9 patients underwent later-stage resection after either adjuvant treatment (n = 5) or for progressive disease during observation (n = 4). Eventually, 20 patients received adjuvant treatment and 7 were observed after primary management. One patient was lost to follow-up. Heterogeneity of pineal region tumor requires histological confirmation. Primary biopsy of pineal lesions should precede surgical resection since less than a third of patients needed primary surgical resection according to the German pediatric brain tumor protocols. Interdisciplinary decision making upfront any treatment is warranted in order to adequately guide treatment.

Lateral Temporal Approach for Image-Guided Stereotactic Biopsy of Pineal Region Tumors.

BACKGROUND: Biopsy of pineal region neoplasms is frequently accomplished by way of endoscopic transventricular access or using an image-guided, computer-assisted stereotactic approach. METHODS: We evaluated a nonorthogonal lateral temporal approach for stereotactic biopsy of pineal region tumors as a variation of previously described stereotactic methods. Magnetic resonance imaging-guided frameless stereotaxy was used to plan and perform biopsies of pineal region tumors using a nonorthogonal trajectory extending from the superior or middle temporal gyri through the temporal stem, anterior to the atrium of the lateral ventricle, and posterior to the corticospinal tract. RESULTS: All patients had an uncomplicated postoperative course and remained at neurologic baseline. No parenchymal or ventricular hemorrhage was present on postoperative scans. A tissue diagnosis was obtained in all patients. CONCLUSIONS: This method appears to be a safe alternative to stereotactic biopsy using other trajectories and provides adequate tissue for definitive diagnosis.

Intracranial growing teratoma syndrome (iGTS): an international case series and review of the literature.

PURPOSE: Intracranial growing teratoma syndrome (iGTS) is a rare phenomenon of paradoxical growth of a germ cell tumor (GCT) during treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of iGTS in Western countries. METHODS: Pediatric patients from 22 North American and Australian institutions diagnosed with iGTS between 2000 and 2017 were retrospectively evaluated. RESULTS: From a total of 777 cases of central nervous system (CNS) GCT, 39 cases of iGTS were identified for an overall frequency of 5%. Pineal region was a more frequent location for iGTS as compared to cases of GCT without iGTS (p < 0.00001). In patients with an initial tissue diagnosis of GCT, immature teratoma was present in 50%. Serum AFP or ßhCG was detectable in 87% of patients (median values 66 ng/mL and 44 IU/L, respectively). iGTS occurred at a median of 2 months (range 0.5-32) from diagnosis, in the majority of patients. All patients underwent surgical resection, leading to gross total resection in 79%. Following surgery, all patients resumed adjuvant therapy or post treatment follow-up for GCT. At a median follow-up of 5.3 years (range 0.2-11.8), 37 (95%) of patients are alive, including 5 with stable residual mass. CONCLUSION: iGTS occurs in 5% of patients with GCT in Western countries. Tumors of the pineal region and GCT containing immature teratoma appear to be associated with a higher risk of developing iGTS. Complete surgical resection is the mainstay of treatment. Overall survival of patients developing iGTS remains favorable.

Second Window Indocyanine Green (SWIG) Near Infrared Fluorescent Transventricular Biopsy of Pineal Tumor.

BACKGROUND: Second Window Indocyanine Green (SWIG) is a novel technique for real-time, intraoperative tumor visualization using a high-dose infusion of indocyanine green (ICG) 24 hours before surgery. Due to pathologic diversity found in the pineal region, tissue diagnosis in patients with pineal region mass is essential to optimize further clinical management. CASE DESCRIPTION: We present the case of a 75-year-old woman with known pineal region mass for 18 years, who presented with progressive classic signs and symptoms of obstructive hydrocephalus over the past 6 months. Preoperative imaging confirmed a contrast-enhancing pineal region tumor, which appeared to be obstructing the aqueduct of Sylvius, causing proximal obstructive hydrocephalus. We delivered 5 mg/kg of ICG intravenously 24 hours before the surgery. The patient underwent an endoscopic third ventriculostomy and a biopsy of the pineal lesion. The tumor demonstrated clear near-infrared fluorescence, which was distinct from surrounding third ventricle floor and ependyma. The signal-to-background ratio was 2.9. The final pathology report revealed a World Health Organization grade I pineocytoma. CONCLUSIONS: We report on a novel application of near-infrared fluorescence for tumor identification of pineal region tumors, using the "SWIG technique."

Increased VEGF levels in a case of papillary tumor of the pineal region with intracranial hemorrhage: a potential surrogate indicator of tumor angiogenesis and aggressiveness?

Pineal tumors are rare, about 1% of all intracranial tumors. At variance with pineocytomas, usually characterized by a good prognosis, papillary tumors behave more aggressively. Owing to their rarity, little is known about their biology and clinical behavior, moreover conflicting data on prognosis have been reported. Here we present an unusual case of papillary neuroepithelial tumor of the pineal region in a 40-year-old man who was admitted in a state of unconsciousness due to the presence of intracranial hemorrhage. After 21 days from admission, he underwent third ventriculostomy for hydrocephalus and biopsy of the lesion. Since bleeding manifestations are uncommonly associated with this kind of tumors, we performed some additional non routine laboratory tests in order to identify biological indicators of disease course and abnormal angiogenesis. Coagulation screening tests were performed to rule out the presence of coagulopathy and vascular endothelial growth factor (VEGF ) levels were measured in plasma as marker of tumor angiogenic potential. Histologic evaluation confirmed the diagnosis of a papillary tumor of the pineal region with the presence of tiny vessel lumens that may account for increased angiogenesis Coagulation screening was normal and VEGF levels were extremely high if compared to healthy individuals. After 20 months of follow-up the tumor mass, radiotherapy treated, appeared dramatically reduced at MRI evaluation, and, interestingly, VEGF levels, although still higher than in healthy individuals, resulted significantly decreased as compared to those measured at time of first hospital admission suggesting a role for VEGF as indicator of tumor aggressiveness. In conclusion, measurement of angiogenesis circulating soluble markers could have an additional feedback in the diagnosis, therapy and monitoring the disease in patients with very rare CNS tumors as papillary tumors of pineal region that have non univocal clinical behavior and prognosis.

Simple Identification of Cerebrospinal Fluid Turbulent Motion Using a Dynamic Improved Motion-sensitized Driven-equilibrium Steady-state Free Precession Method Applied to Various Types of Cerebrospinal Fluid Motion Disturbance.

The motion of cerebrospinal fluid (CSF) within the subarachnoid space and ventricles is greatly modulated when propagating synchronously with the cardiac pulse and respiratory cycle and path through the nerves, blood vessels, and arachnoid trabeculae. Water molecule movement that propagates between two spaces via a stoma, foramen, or duct presents increased acceleration when passing through a narrow area and can exhibit "turbulence." Recently, neurosurgeons have started to perform fenestration procedures using neuroendoscopy to treat hydrocephalus and cystic lesions. As part of the postoperative evaluation, a noninvasive diagnostic technique to visualize the water molecules at the fenestrated site is necessary. Because turbulence is observed at this fenestrated site, an imaging technique appropriate for observing this turbulence is essential. We therefore investigated the usefulness of a dynamic improved motion-sensitized driven-equilibrium steady-state free precession (Dynamic iMSDE SSFP) sequence of magnetic resonance imaging that is superior for ascertaining turbulent motions in healthy volunteers and patients. Images of Dynamic iMSDE SSFP from volunteers revealed that CSF motion at the ventral surface of the brainstem and the third ventricle is augmented and turbulent. Moreover, our findings confirmed that this technique is useful for evaluating treatments that utilize neuroendoscopy. As a result, Dynamic iMSDE SSFP, a simple sequence for visualizing CSF motion, entails a short imaging time, can extensively visualize CSF motion, does not require additional processes such as labeling or trigger setting, and is anticipated to have wide-ranging clinical applications in the future.

An anatomical study of the foramen of Monro: implications in management of pineal tumors presenting with hydrocephalus.

BACKGROUND: For pineal tumors presenting with hydrocephalus, simultaneous endoscopic third ventriculostomy (ETV) and tumor biopsy is commonly used as the initial step in management. To analyze the restriction which the foramen of Monro poses to this procedure, one must start with a detailed description of the microsurgical anatomy of the foramen in living subjects. However, the orientation and shape of the foramen of Monro make this description difficult with conventional imaging techniques. METHOD: Virtual reality technology was applied on MRIs on living subject without hydrocephalus, as well as patients with hydrocephalus, to generate precise anatomical models with sub-millimeter accuracy. The morphometry of the foramen of Monro was studied in each group. In addition, displacement of the margins of the foramen was studied in detail for simultaneous ETV and pineal tumor biopsy through a single burr hole. RESULTS: In 30 normal subjects, the foramen of Monro had oval-shaped openings averaging 5.23 mm2. The foramen was larger in people above age 55 (p = 0.007) and on the left side compared to the right (p = 0.002). For patients with clinical presentation of hydrocephalus, the average opening was 32.6 mm2. Simulated single burr hole simultaneous ETV and pineal tumor biopsy was performed in 10 specimens. Average displacement of the posterior and anterior margins of the foramen was 5.71 mm and 5.76 mm, respectively. However, maximum displacement reached 9.3 mm posteriorly and 10 mm anteriorly. CONCLUSIONS: The foramen of Monro is an oval-shaped cylinder that changes in size and orientation in the hydrocephalic patient. If universally applied to all patients regardless of foramen and tumor size, ETV/biopsy can displace structures around the Foramen of Monro up to 1 cm, which can potentially lead to neurological damage. Careful pre-operative assessment is critical to determine if a single burr hole approach is safe.

The Purely Endoscopic Supracerebellar Infratentorial Approach for Resecting Pineal Region Tumors with Preservation of Cerebellomesencephalic Vein: Technical Note and Preliminary Clinical Outcomes.

BACKGROUND: The cerebellomesencephalic vein (CMV) was frequently sacrificed in surgery approached via the supracerebellar infratentorial (SCIT) route for resecting pineal region tumors, which resulted in potential risk of neurologic deficit. Preserving the CMV in the SCIT approach could enhance the safety and effectiveness of this natural corridor surgery. The aim of this article was to identify the probability and safety of preserving the CMV through the application of neuroendoscopy in the SCIT approach. METHODS: Clinical data of patients who underwent pineal region tumor resection through a purely endoscopic SCIT approach were retrospectively analyzed, focusing on surgical techniques and clinical outcomes. RESULTS: The study included 8 patients with pineal region tumors. The CMV was preserved intact in all patients. Total tumor removal was achieved in 7 of 8 patients. In 1 patient with 2 tumors in the pineal region and roof of the third ventricle, the tumor in the pineal region was resected completely, followed by subsequent chemotherapy combined with radiotherapy, after which the other tumor disappeared totally. All patients recovered normally with uneventful postoperative outcomes. CONCLUSIONS: The advantage of close observation and panoramic view provided by neuroendoscopy combined with meticulous manipulation improved the ability to preserve the CMV in resecting pineal region tumors via the SCIT approach. The neuroendoscopic technique enhances the safety and efficacy of the SCIT approach.

An Active Duty Sailor With Worsening Headaches and Blurry Vision.

Headaches are a typical presentation to a military medical department or emergency room. Having a broad differential diagnosis and utilizing a thorough physical exam can assist providers in honing down the list of pathology and in identifying potentially life-threatening causes of cephalgia such as intracranial tumors. In this case, a 27-year-old man presented with progressive headaches along with vision changes for the preceding 2-3 months. On initial physical exam, he was found to have bilateral papilledema using a panoptic ophthalmoscope, confirmed after sending the patient to optometry. Neurology evaluated him and found a pineoblastoma on MRI. He underwent eventual neurosurgical debulking and radiation. The highlight of this case is the critical history components along with physical exam techniques that can assist providers in the identification of life-threatening causes of a headache. Direct ophthalmoscopy was vital in this case to encourage referral for further management. In selected studies, emergency medicine providers performed ophthalmoscopy in 14% of patients, with roughly 10% of those patients having erroneous findings. Another study showed that 13% of all cases presenting to a large academic center had fundoscopic findings that were important to the final diagnosis.

Pineal Cyst-Related Aqueductal Stenosis as Cause of Intractable Headaches in Nonhydrocephalic Patients.

BACKGROUND: Pineal cysts (PCs) are histologically benign lesions of the pineal gland. Although the majority of PCs are asymptomatic, some cases are ambiguous and accompanied by nonspecific symptoms of variable severity. We suggested that disabling headache in nonhydrocephalic patients with PCs is associated with cerebral aqueduct (CAq) stenosis. METHODS: A retrospective analysis was conducted in patients with PCs suffering from headache without secondary hydrocephalus who underwent surgical resection at Burdenko Neurosurgery Center between 1995 and 2016. All available medical records and radiographic images were retrospectively assessed in these patients. The comparison groups included 22 patients with nonoperated PCs and 25 healthy individuals. Specific magnetic resonance imaging measures were selected to assess the morphometry of the CAq and degree of the stenosis. RESULTS: In 25 patients (82%) we observed clinical improvement after surgery in a follow-up period. Among those with improvement, 10 of them (40%) experienced total relief and 15 of them (60%) had marked headache diminishment. In 5 patients the headache remained persistent. The preoperative rostral CAq diameter appeared to be significantly narrower (P = 0.0011045), and the preoperative rostral/caudal diameter ratio (Rd/Cd) was found to be lower (P = 0.004391) in patients who recovered from headache versus those who did not. CONCLUSION: The results indicate a statistically significant relationship between the changes in the CAq morphometrics and the clinical outcome in postoperative period. Surgical removal of symptomatic pineal cysts in patients without hydrocephalus can be considered as an effective treatment. However, a thorough preoperative examination and patient selection should be conducted in every case.