Prechiasmatic Transection of the Unilateral Dodge Class â Optic Pathway Glioma without Neurofibromatosis Type 1: Technical Description and Clinical Prognosis.

OBJECTIVE: For unilateral Dodge Class Ⅰ optic pathway glioma (OPG-uDCⅠ) without neurofibromatosis type 1, unilateral isolated optic nerve gliomas before the optic chiasm have been confirmed to possibly cause visual deterioration and poor prognosis. For this type of highly selective localized tumor, we explored surgery as the only treatment method. This article retrospectively analyzed and summarized the clinical data of this case series, with the aim of exploring the main technical details and clinical prognosis. METHODS: Included were patients with OPG-uDCⅠ without neurofibromatosis type 1 and experiencing vision loss on the affected side. The fronto-orbital approach was used, which was mainly divided into 3 parts: intraorbital, optic canal, and intracranial. All patients underwent prechiasmatic resection without any adjuvant treatments. The follow-up period was 3 months after surgery, and magnetic resonance imaging and contralateral visual acuity were reviewed annually after surgery. RESULTS: All OPG-uDCⅠ cases were completely removed without any adjuvant treatments, and there was no recurrence during the follow-up period. Pathological results showed that, except for 1 adult patient with pilomyxoid astrocytoma (World Health Organization grade Ⅱ), the others all had pilocytic astrocytoma (World Health Organization grade Ⅰ). Five patients experienced transient ptosis, and all recovered 3 months after surgery. CONCLUSIONS: For OPG-uDCⅠ without neurofibromatosis type 1, radical prechiasmatic resection of the tumor is possible, without the need for postoperative radiotherapy and chemotherapy.

Clinical description with magnetic resonance appearance of a high-grade undefined optic nerve glioma with intracranial extension.

A 4-year-old mixed-breed dog was presented for hyphema and glaucoma of the right eye. Enucleation of the right globe was carried out, and histopathology examination revealed an optic nerve glioma with incomplete surgical margins. At 8 wk after surgery, the dog had depressed mentation and a diminished pupillary light reflex of the left eye. Magnetic resonance imaging revealed an irregular, heterogeneously T2 hyperintense/T1 isointense mass in the region of the optic chiasm. Compression of the rostral thalamus was present, with effacement of the pituitary gland and involvement of the right orbit. The dog was euthanized 4.5 mo after initial presentation. An undefined glioma of the right optic nerve with extension to the diencephalon was diagnosed on necropsy. Key clinical message: Although rare, intraocular glioma is a differential diagnosis for hyphema, glaucoma, and retinal detachment. Magnetic resonance imaging should be considered in cases of intraocular neoplasia, notably in those with incomplete surgical margins of the optic nerve.

Description clinique avec aspect en résonance magnétique d'un gliome indéfini de haut grade du nerf optique avec extension intracrânienne. Un chien de race croisé âgé de 4 ans a été présenté pour un hyphéma et un glaucome de l'œil droit. Une énucléation du globe droit a été réalisée et l'examen histopathologique a révélé un gliome du nerf optique aux marges chirurgicales incomplètes. Huit semaines après la chirurgie, le chien avait une diminution du processus mental et un réflexe pupillaire à la lumière diminué de l'œil gauche. L'imagerie par résonance magnétique a révélé une masse irrégulière hétérogène hyperintense T2/T1 isointense dans la région du chiasma optique. Une compression du thalamus rostral était présente, avec effacement de l'hypophyse et atteinte de l'orbite droite. Le chien a été euthanasié 4,5 mois après la présentation initiale. Un gliome indéfini du nerf optique droit avec extension au diencéphale a été diagnostiqué à l'autopsie.Message clinique clé:Bien que rare, le gliome intraoculaire est un diagnostic différentiel pour l'hyphéma, le glaucome et le décollement de la rétine. L'imagerie par résonance magnétique doit être envisagée en cas de néoplasie intraoculaire, notamment chez ceux dont les marges chirurgicales du nerf optique sont incomplètes.(Traduit par Dr Serge Messier).

[Hypofractionated stereotactic irradiation for optic nerve glioma]. / Stereotaksicheskoe obluchenie gliom zritel'nogo nerva v rezhime gipofraktsionirovaniya.

Optic nerve glioma is a rather rare tumor. It predominantly arises in pediatric patients, including those with type I neurofibromatosis. This neoplasm is accompanied by decreased visual function and exophthalmos. Treatment strategy is individualized depending on age, volume and spread of tumor, as well as severity of clinical manifestations. Possible treatment options are surgical resection, chemotherapy, radiotherapy and their combination. Radiotherapy can be recommended for patients with intact visual functions, no severe proptosis and trophic lesions. Classic fractionation mode is used as a standard. Currently, the possibility of hypofractionated irradiation is being considered. OBJECTIVE: To evaluate safety and efficacy of hypofractionated radiotherapy in patients with optic nerve glioma. MATERIAL AND METHODS: Sixteen patients with optic nerve gliomas underwent hypofractionated stereotactic irradiation (CyberKnife) between May 2014 and October 2019. Single focal dose was 5.5 Gy. There were 5 fractions up to total focal dose of 27.5 Gy. The sample enrolled 14 children with a median age of 4 years (range 23 months - 13 years) and 2 adults aged 47 and 66 years, respectively. Median of tumor volume was 2.77 cm3 (range 1.69-10.01 cm3). RESULTS: Tumor growth control was achieved in all patients, partial remission was observed in 5 (32%) patients. None patient had deterioration of visual function. Improvement of visual acuity was noted in 3 (19%) cases. Visual field enlargement occurred in 4 (67%) out of 6 patients who were preoperatively examined. After irradiation, proptosis decreased by ≥ 1 mm in 9 (60%) out of 15 patients.

Endoscopic transnasal surgery in optic pathway gliomas located in the chiasma-hypothalamic region: case series of ten patients in a single-center experience and endoscopic literature review.

OBJECTIVE: Optic pathway gliomas (OPGs) constitute approximately 3-5% of childhood intracranial tumors. In this study, the authors presented their experience of using the endoscopic endonasal approach to treat patients with OPG located in the chiasma-hypothalamic region and aimed to use the infrachiasmatic corridor in the endoscopic endonasal approach as an alternative to the transcranial approach in the surgical necessity of OPGs. METHODS: We retrospectively analyzed the data of ten patients diagnosed with OPG histopathologically among 3757 cases who underwent endoscopic endonasal surgery between August 1997 and March 2021 at Kocaeli University Faculty of Medicine Pituitary Research Center and Department of Neurosurgery. Mean follow-up period 48.5 months. During the postoperative follow-up period, 3 of these 10 patients underwent reoperation due to tumor recurrence. Combined (endoscopic endonasal approach + transcranial approach) approach was applied to 2 patients in the same session. Surgical and clinical outcomes were evaluated in detail. RESULTS: Ten patients with a mean patient age of 20.6 ± 11.4 were included in this study. The most common complaint was visual impairment. After surgery, improvement in visual impairment was observed in five patients. No increase in postoperative visual impairment was observed in any of the patients. Postoperative panhypopituitarism was not observed in any of the patients. STR resection was performed in 5 patients and NTR resection in 5 patients. No additional treatment was required during follow-up in 4 of 5 patients who underwent NTR. A total of 6 patients received postoperative radiotherapy treatment. CONCLUSIONS: In gliomas located in the chiasma-hypothalamic region, appropriate patient selection and endoscopic endonasal surgical treatment may contribute to the elimination of symptoms due to the mass effect of the tumor. It may also contribute to keeping the disease under control with targeted adjuvant therapies by clarifying the pathological diagnosis of the lesion.

Benign optic nerve gliomas in an adult: A case report.

RATIONALE: Benign optic nerve gliomas were rarely found in adults, and total resection of these lesions seems impossible. We aimed to share a rare clinical case with an unusual and instructive treatment process. PATIENT CONCERNS: A 52-year-old woman complained of a 4-month history of visual disturbance. Automated perimetry revealed visual field defect in her both eyes. DIAGNOSIS: This patient was diagnosed with optic nerve glioma. We found its pathological features consistent with the pilocytic astrocytomas (WHO grade I). INTERVENTIONS: A total resection of the tumor was smoothly performed. OUTCOMES: Repeat MRI 3 months after the surgery demonstrated no recurrence of the lesion. Two years of postoperative telephone follow-up showed a stable status of improved vision. LESSONS: We reported this interesting case to show a rare kind of condition regarding optic nerve gliomas in adults, which might help neurosurgeons like us to diagnose and treat these "invisible" tumors.

Clinical Outcome of Optic Pathway and Hypothalamic Gliomas: A 20-Year Single-Institution Retrospective Study.

BACKGROUND: Optic pathway and hypothalamic gliomas (OPHGs) are challenging to surgically remove owing to their anatomical relationship. We previously reported on surgical treatment outcomes over a 10-year time frame. The purpose of this study was to update the OPHG clinical outcomes for cases in which chemotherapy has become the primary treatment option. The role of surgery was also revisited. METHODS: Patients with a diagnosis of OPHG who underwent treatment at Seoul National University Children's Hospital from February 1999 to July 2019 were included. A multidisciplinary approach was used to determine the patients' treatment plans. Chemotherapy was the first-line treatment for all patients. When symptoms of hydrocephalus existed, debulking surgery was performed to reopen the flow of cerebrospinal fluid. RESULTS: The study included 47 patients with OPHGs. The mean age was 6.9 years. Neurofibromatosis 1 was diagnosed in 3 patients. The extent of removal was none or biopsy in 13 (28%) cases, partial resection in 23 (49%) cases, and subtotal to gross total resection in 11 (23%) cases. In 32 (68%) patients, chemotherapy was first-line treatment. Ascites after ventriculoperitoneal shunt occurred in 3 cases, and 2 cases were successfully managed with debulking surgery. Treatment outcomes showed a 5-year overall survival rate of 97.7% and a 5-year progression-free survival rate of 47.7%. CONCLUSIONS: OPHG management using less invasive operations and chemotherapy as first-line treatment is feasible. Debulking surgery in patients with OPHGs may be considered in cases with cerebrospinal fluid pathway obstruction, progression despite chemotherapy or radiation, and refractory shunt-related ascites.

The role of imaging features and resection status in the survival outcome of sporadic optic pathway glioma children receiving different adjuvant treatments.

Optic pathway glioma (OPG) is a rare brain tumor affecting children, with no standard treatment strategy. This study described the sporadic OPG survival outcomes after surgical treatment and analyzed the role of imaging features and resection status in children receiving different adjuvant treatments. This retrospective study included 165 OPG patients whose clinical information were obtained from the hospital record system. Tumor volume and residual tumor volume were calculated by delineating the lesion area. Kaplan-Meier method and Cox proportional hazards model were conducted to analyze the independent prognosis factor. A total of 165 patients were included in this study. Respectively, the 5-year overall survival (OS) and progression-free survival (PFS) were 87.58% and 77.87%. Residual tumor size and first adjuvant treatment (AT) after surgery were both associated with PFS. In patients with small-size residual tumors, there was no significant difference in PFS between the AT treatment groups. Moreover, age, exophytic cystic components, leptomeningeal metastases, and AT were associated with OS. In patients with exophytic cystic components and those with leptomeningeal metastases, there was no significant difference in OS. Our results revealed that OPG patients could avoid or defer AT by maximized resection. Age ≤ 2 years was a disadvantageous factor for OS. Patients with exophytic cystic components were more likely to benefit from primary surgery, and CT or RT was not beneficial for these patients. Patients with leptomeningeal metastases had a poor prognosis regardless of the treatment they received. Future prospective clinical studies are needed to develop more effective treatment regimens.

Cranio-Orbital Approach for Single-Stage En Bloc Resection of Optic Nerve Glioma: Technical Note.

BACKGROUND AND IMPORTANCE: There is no consensus on the optimal surgical approach for managing optic nerve gliomas. For solely intraorbital tumors, a single-stage lateral orbitotomy approach for resection may be performed, but when the nerve within the optic canal is affected, two-stage cranial and orbital approaches are often used. The authors describe their technique to safely achieve aggressive nerve resection to minimize the probability of recurrence that might affect the optic tracts, optic chiasm, and contralateral optic nerve. CLINICAL PRESENTATION: A 28-yr-old woman presented with painless progressive vision loss, resulting in blindness. The second of 2 transorbital biopsies was diagnostic and consistent with low-grade glioma. The lesion continued to grow on serial imaging. The patient was offered a globe-sparing operative approach, with aggressive resection of the lesion to minimize the probability of tumor recurrence, which could possibly affect vision in her contralateral eye. The patient did well postoperatively, with clean tumor margins on pathological analysis and no evidence of residual on imaging. On postoperative examination, she had a mild ptosis, which was nearly resolved at her 6-wk outpatient follow-up. CONCLUSION: This aggressive single-stage en bloc resection of an optic nerve glioma can achieve excellent tumor margins and preservation of extraocular muscle function.

Neurosurgical experience of managing optic pathway gliomas.

BACKGROUND: Optic pathway gliomas (OPGs), also known as visual pathway gliomas, are debilitating tumors that account for 3-5% of all pediatric brain tumors. They are most commonly WHO grade 1 pilocytic astrocytomas and frequently occur in patients with neurofibromatosis type 1. The location of these tumors results in visual loss and blindness, endocrine and hypothalamic dysfunction, hydrocephalus, and premature death. Their involvement of the visual pathways and proximity to other eloquent brain structures typically precludes complete resection or optimal radiation dosing without incurring significant neurological injury. There are various surgical interventions that can be performed in relation to these lesions including biopsy, cerebrospinal fluid diversion, and partial or radical resection, but their role is a source of debate. This study catalogues our surgical experience and patient outcomes in order to support decision-making in this challenging pathology. METHODS: A retrospective review of all cases of OPGs treated in a single center from July 1990 to July 2020. Data was collected on patient demographics, radiographic findings, pathology, and management including surgical interventions. Outcome data included survival, visual function, endocrine, and hypothalamic dysfunction. RESULTS: One hundred twenty-one patients with OPG were identified, and 50 of these patients underwent a total of 104 surgical procedures. These included biopsy (31), subtotal or gross total resection (20 operations in 17 patients), cyst drainage (17), Ommaya reservoir insertion (9), or cerebrospinal fluid diversion (27). During the study period, there was 6% overall mortality, 18% hypothalamic dysfunction, 20% endocrine dysfunction, and 42% had some cognitive dysfunction. At diagnosis 75% of patients had good or moderate visual function in at least one eye, and overall, this improved to 83% at the end of the study period. In comparison the worst eye had good or moderate visual function in 56%, and this reduced to 53%. Baseline and final visual function were poorer in patients who had a surgical resection, but improvements in vision were still found-particularly in the best eye. DISCUSSION/CONCLUSION: OPG are debilitating childhood tumor that have lifelong consequences in terms of visual function and endocrinopathies/hypothalamic dysfunction; this can result in substantial patient morbidity. Decisions regarding management and the role of surgery in this condition are challenging and include cerebrospinal fluid diversion, biopsy, and in highly select cases cystic decompression or surgical resection. In this paper, we review our own experience, outcomes, and surgical philosophy.

Vision Outcomes for Pediatric Patients With Optic Pathway Gliomas Associated With Neurofibromatosis Type I: A Systematic Review of the Clinical Evidence.

Children with neurofibromatosis type I (NF1) have a higher predisposition for low-grade astrocytomas of the optic pathway, commonly referred to as optic pathway gliomas (OPGs). OPGs can result in visual deterioration. Treatment outcomes in OPG-NF1 management are often reported around tumor stabilization. We sought to compare vision outcomes associated with different OPG treatment strategies to inform about this important functional metric. A meta-analysis exploring the different modalities to treat children with OPG-NF1 was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using multiple databases. Of the 113 articles identified in the search, 23 full text articles, representing 564 patients, were included for review. These articles included retrospective, prospective, and randomized controlled studies on observation (n=9), chemotherapy (n=19), radiation therapy (n=6), and surgery (n=7). Of the patients undergoing observation, 87% (60/69) demonstrated stable acuity. In the chemotherapy studies, 27.3% (72/264) demonstrated improved acuity/visual field and/or visual-evoked potential amplitudes, 39.4% (104/264) stable acuity, and 33.3% (88/264) deterioration. Both the radiation and surgical treatments reported worsening acuity at 90.9% (10/11) and 73.3% (11/15), respectively. Causal associations are not known. Indications for and timing of treatment choice warrant larger scale study to provide further understanding.

Acute angle plication of optic nerve glioma as a mechanism of rapidly progressive visual loss.

Purpose: The majority of pediatric patients with optic nerve gliomas (ONG) remain asymptomatic; however, a subset of patients suffer rapid, irreversible visual loss. The purpose of this study was to determine anatomic differences of ONG noted either by imaging or by intra-operative observation between patients with gradual visual dysfunction and those with rapid visual loss. Methods: A retrospective review was performed in patients with visual loss secondary to ONG. The clinical records, pathology, and radiographic images were reviewed for all patients. The degree of folding or plication of the optic nerve (ON) was determined by analyzing the most acute angle present in the course of the ON. Outcome measures: The primary outcome measure was the angle of plication or folding of the ON and the relationship of this to the course of visual dysfunction. Results: Six patients with ONG were included in the study. A structural difference in the ON was identified in four patients with rapid vision loss compared with two patients with more gradual visual dysfunction. In patients with rapid progressive visual loss, the ONG had a 90-degree or more acute plication of the ON. Those with more gradual visual loss had more obtuse bends in the ONG. Conclusions: We have identified that the intrinsic structure of the ONG may contribute to the rare but devastatingly rapid progression of visual dysfunction in some patients. Recognizing these changes may guide clinicians to intervene prior to the development of irreversible visual loss.

Optic pathway tumor in children: Toward a new classification for neurosurgical use.

OBJECT: Optic pathway tumors (OPT) represent a challenge for pediatric neurosurgeons. Role of surgery is debated due to the high risk of iatrogenic damage, and in lasts decades it lost its importance in favor of chemotherapy. However, in some cases surgery is necessary to make biomolecular and histological diagnosis, to manage intracranial hypertension (IH) and to cooperate with medical therapies in controlling tumor relapse. With the aim to standardize selection of surgical OPT cases, we propose a simple, practical and reproducible classification. METHODS: We retrospectively analyzed data of 38 patients with OPT treated at our institution (1990-2018). After careful analysis of MRI images, we describe a new classification system. Group 1: lesion limited to one or both optic nerve(s). Group 2: chiasmatic lesions extending minimally to hypothalamus. Group 3: hypothalamo-chiasmatic exophitic lesions invading the third ventricle; they can be further divided on the base of concomitant hydrocephalus. Group 4: hypothalamo-chiasmatic lesions extending widely in lateral direction, toward the temporal or the frontal lobes. Patients' data and adopted treatment are reported and analyzed, also depending on this classification. RESULTS: Twenty children were operated on for treatment of OPT during the study period. Permanent clinical impairment was noted in 5 (25%) of operated patients, while visual improvement was noted in 1 patient. OS rate was 100% at 5 years, with a median follow up of 9 years (ranging from 2 to 23). Prevalence of intracranial hypertension and proportion of first-line surgical treatment decision were significantly higher in groups 3-4 compared to groups 1-2 (P<0.001 for both tests). CONCLUSION: Surgery can offer a valuable therapeutic complement for OPT without major risk of iatrogenic damage. Surgery is indispensable in cases presenting with IH, as in groups 3 and 4 lesions. Eligibility of patients to surgery can be based on this new classification system.

Expanded transnasal approaches to the skull base in the Middle East: Where do we stand?

BACKGROUND: Endoscopic transnasal surgery has gained rapid global acceptance over the last two decades. The growing literature and understanding of anterior skull base endoscopic anatomy, in addition to new dedicated endoscopic instruments and tools, have helped to expand the use of the transnasal route in skull base surgery. OBJECTIVE: Report our early experience in expanded endoscopic transnasal surgery (EETS) and approach to skull base neoplasms. DESIGN: Descriptive, retrospective case series. SETTING: Major tertiary care center. PATIENTS AND METHODS: A retrospective case review was conducted at King Saud University Medical City between December 2014 and August 2019. Cases with skull base neoplasms that underwent EETS were included. EETS was defined as endoscopic surgical exposure that extended beyond the sellar margins (prechiasmatic sulcus superiorly, clival recess inferiorly, cavernous carotid lines laterally). Routine transsphenoidal pituitary neoplasms, neoplasms of sinonasal origin and meningoencephaloceles were excluded. MAIN OUTCOME MEASURES: Preoperative clinical assessment, imaging results, surgical approach, and hospital course were all retrieved from the patient electronic charts. Clinical follow-up, perioperative complications, and gross residual tumor rates were documented and reviewed. SAMPLE SIZE AND CHARACTERISTICS: 45 cases of EETS, 13 males and 32 females with mean age of 39.0 (17.7) years (range 2-70 years). RESULTS: The series comprised a wide range of pathologies, including giant pituitary adenoma (8 cases), meningioma (23 cases), craniopharyngioma (4 cases), chordoma (4 cases), optic pathway glioma (2 cases), epidermoid neoplasms (2 cases), astrocytoma (1 case), and teratoma (1 case). For the entire series, gross total resection was achieved in 25/45 operations (55.5%). Postoperative cerebrospinal fluid leak was the most common complication observed in 9 patients (20%) which were all managed endoscopically. Major vascular complications occurred in 2 patients (4.4%) and are described. Other complications are outlined as well. No mortality was observed. CONCLUSIONS: EETS to the skull base can be done with results comparable to traditional approaches. More work is needed to expand our experience, improve outcomes, and educate the public and medical community in our region about the usefulness of this approach. LIMITATIONS: Sample size and study design. CONFLICT OF INTEREST: None.

En-Bloc Resection Versus Resection After Evacuation and Suction of the Content for Orbital Optic Nerve Glioma Causing Visual Loss and Disfiguring Proptosis.

PURPOSE: To evaluate the surgical outcomes of two different techniques of resection for optic nerve gliomas confined to the intra-orbital segment. METHODS: This prospective, comparable, clinical interventional case series was conducted at the orbital clinic of Assiut University Hospital, the referral centre of Upper Egypt in the period between 2006 and 2018. The study included 10 children with optic nerve gliomas confined to the intra-orbital part without intracanalicular or intracranial extension and causing severe visual loss and disfiguring proptosis. In all cases, lateral orbitotomy was performed to expose the mass. In 5 cases (group A), the glioma was resected en-bloc. In 5 cases (group B), a new technique of resection was introduced. The wall of the glioma was incised, the content was evacuated and suctioned and followed by resection under good visualization of the markedly reduced mass in size. RESULTS: In the 2 groups, no tumour regrowth was reported during the follow-up period of 3-12 years. In group A, the 5 cases developed postoperative third nerve damage with paralytic ptosis and one case had severe neurotrophic keratitis ended by dense corneal opacity. In group B, a healthy ipsilateral eye was preserved in all cases and no one case developed postoperative paralytic ptosis. CONCLUSIONS: Reduction of size of an intra-orbital optic nerve glioma by evacuation and suction of the content before resection is highly recommended. This makes the surgical field during resection more visible and minimizes the possibility of third or other nerves damage.

Craniofacial Approaches to Pediatric Orbital Tumors.

Primary pediatric orbital tumors requiring surgery are uncommon and often require multidisciplinary management. Commonly used surgical approaches to the orbit include transconjunctival, transcutaneous (eyelid), transcranial, or extracranial osteotomies. This paper reviews a 10-year experience of cases that required a transcranial or extracranial surgical approach at the Birmingham Children's Hospital. A total of 9 patients were identified between the years 2008 to 2017. Pathologies included rhabdomyosarcoma, juvenile ossifying fibroma, optic nerve glioma, and retinoblastoma. Surgical approaches to the orbit included supraorbital bar osteotomy (transcranial) or lateral orbitotomy (extracranial). Surgical team members included neurosurgery, craniofacial surgery, and ophthalmology. This study aims to review the role of surgery in management as well as the specific indications for performing transcranial or extracranial osteotomies. It also highlights the excellent access achieved with the use of these osteotomies in certain cases, especially when compared with transconjunctival or transcutaneous approaches.

Newly Diagnosed Optic Pathway Glioma During Pregnancy.

BACKGROUND: Optic pathway gliomas (OPGs) are relatively rare, and their presentation after the first decade of life is even less common. Although many treatment options exist, surgery is typically reserved for tumors significantly compressing surrounding structures. Pregnancy can complicate the management of these tumors, as fetal developmental considerations limit the ways in which they are imaged and treated. CASE DESCRIPTION: In this report we detail the case of a 27-year-old pregnant woman who was found to have an OPG during her third trimester. After a decline in this patient's vision and clinical status, a decision was made to induce labor at 31 weeks so that her disease could be more thoroughly addressed. CONCLUSIONS: While OPGs are typically benign tumors, pregnancy complicates their management significantly. Contrast media and anesthesia pose significant risks to the fetus, while pregnancy may contribute to increased rates of tumor growth and clinical deterioration. Managing OPGs in pregnant patients thus requires balancing the risks to the fetus and patient.

Endoscopic transnasal resection of optic pathway pilocytic astrocytoma.

PURPOSE: Optic pathway gliomas (OPGs) are low-grade neoplasms that primarily affect children. The management of OPGs remains controversial. Reports on the use of the endoscopic endonasal approach (EEA) in OPGs are extremely limited, and no such reports exist on its utility for pediatric OPGs. Here, we report our results and experience with OPGs treated with the EEA. METHODS: We retrospectively reviewed the medical records of OPG patients who were treated surgically via the EEA at our institutions from 2015 to 2017. Data on the demographics, clinical presentation, surgical complications, clinical outcomes, radiological imaging, and visual outcomes were recorded for each patient. RESULTS: Four cases were identified, with visual disturbances being the predominant complaint. The mean patient age was 15.5 years. Three cases showed normal preoperative hormonal profiles, but one patient had hypothyroidism. All tumors identified in this study were World Health Organization grade I pilocytic astrocytomas. Surgical complications included hypopituitarism in two patients, meningitis in two patients, cerebrospinal fluid leak in one patient, and transient diabetes insipidus in one patient. No patient experienced worsening neurological or visual symptoms postoperatively. CONCLUSIONS: Although our data are preliminary, the EEA provides a direct corridor to OPG with acceptable results in terms of tumor resection and visual outcomes. Hypothalamic-pituitary axis dysfunction remains a limitation of any treatment modality for OPGs and should be considered whenever possible. Definitive conclusions are pending as the learning curve of this approach is steep. Further work is needed to understand patient selection for such an approach.

Optic Nerve Glioma in Two Sisters with Family History of Neurofibromatosis Type 1.

Optic nerve glioma (ONG) is associated in 10% of patients with neurofibromatosis (NF) type 1. To date no consensus has been reached regarding the therapeutic approach and prevention of visual impairment in these patients. Reports in the literature vary from a conservative approach (observation) to the use of single treatment modalities or multimodality protocols of surgical removal, radiotherapy, and/or chemotherapy. We present our experience with two siblings with ONG whose mother carries cutaneous stigmata of NF type 1. The younger sister was diagnosed 3 years after the treatment of the older sibling following recommended imaging for screening. Postoperative follow-up for 11 and 15 years, respectively, demonstrated lack of tumor regrowth and preserved vision in the contralateral eye. We discuss the treatment strategy in pediatric patients with orbital ONG associated with NF type 1.