Orbital Apex Tumors Research Articles

Clinical Characteristics and Delayed Diagnosis of Pediatric Patients Presenting to the Emergency Department With a Newly Diagnosed Central Nervous System Tumor: A Single Institutional Experience.

Due to the varied symptomatology and inconsistent features on neurologic exam, central nervous system (CNS) tumors are difficult to diagnosis in a timely manner. To determine the clinical, neurological, and neuroimaging features of newly diagnosed CNS tumors presenting to the emergency department (ED). We evaluated a retrospective cohort of 121 consecutive patients presenting to a tertiary care pediatric ED over 7 consecutive years with newly diagnosed CNS tumors. Clinical symptomatology, neurologic findings reported by emergency room and neurology physicians, neuroimaging features, and time to diagnosis were analyzed. A total of 116 (48 female, median age 8.0 years (interquartile range, 4.4-12.6), 52% Hispanic) presented to the ED (64% self-referred) diagnosed with a brain tumor (54% posterior fossa, 24% embryonal, 24% low-grade glioma, 16% high-grade glioma) resulting in hospital admission in 92% of cases. Five were diagnosed with extradural spinal, clivus, or orbital apex tumors. Symptomatology or duration did not differ when stratified by demographics, location, or histologic subtype. Moderate degree of concordance was observed among neurologic examinations performed by ED physicians and neurologists. Delayed diagnosis (median delay = 3.5 [1-7] months) was seen in 14% of patients, 13 with primary brain tumors (11 hemispheric, 2 brain stem). Six children with delayed diagnosis of low-grade glial tumors had a nonfocal neurologic examination in comparison to 5 patients with abnormal examinations observed with primary spinal or extradural CNS tumors. Four patients with posterior fossa tumors (3 medulloblastoma, 1 ependymoma) had normal/near normal neurologic examination at presentation despite posterior fossa symptomatology related to increased intracranial pressure. Our series highlights the complexity of symptomology and neurologic findings in children presenting to the ED with newly diagnosed CNS tumors who may have a normal neurologic examination. Standardization of symptom assessment and focused neurologic examinations may lead to earlier neuroimaging and prevent delayed diagnosis.

Retrospective Case Analysis of Transnasal Endoscopic Resection of Benign Orbital Apex Tumors: Some Thoughts on Transnasal Endoscopic Surgery.

Purpose To deeply discuss the patient selection, surgical planning, surgical techniques, and the therapeutic challenge for endoscopic transnasal resection of benign orbital apex tumors (OATs). Methods We retrospectively analyzed the cases of 18 patients (18 eyes) with orbital apex cavernous hemangioma (OACH) who underwent endoscopic transnasal approach for resection of the tumor in Zhongshan Ophthalmic Center from March 2016 to May 2020. At each follow-up visit, the patients underwent measurement of their best-corrected visual acuity (BCVA), slit-lamp examination, indirect ophthalmoscopy, and visual field testing. Results There were 18 patients, 7 males and 11 females, with a mean age of 49.9 ± 12.6 years (range: 26 to 70 years). All 18 patients had unilateral tumors. Among the 18 cases, 13 were located in the right orbit and 5 were located in the left orbit. Sixteen patients underwent purely endoscopic transnasal surgery, and the other 2 patients underwent an endoscopic transnasal approach combined with a transcutaneous or transconjunctival surgical approach. Fourteen patients' OACHs were removed completely, 1 patient's OACH was partly removed, and 3 patients underwent pure decompression of the optic nerve. Fourteen patients gained improved or stable BCVA after surgery. Three patients showed postoperative vision decline, and 1 patient had no light perception after surgery. Conclusions Endoscopic surgery is an effective surgical technique for the treatment of benign tumors in the orbital apex. It is necessary to strictly select patients and fully evaluate the benefits and risks of tumor completely or partly removed.

Современные подходы к удалению новообразований вершины глазницы

Современные подходы к удалению новообразований вершины глазницы

Orbital Apex Metastasis from Adenoid Cystic Carcinoma: Acute Loss of Vision and Subsequent Recovery with the Radiation.

Orbital apex metastasis from adenoid cystic carcinoma (ACC) is rare. We present a patient with known metastatic ACC presented with a rapidly declining vision with visual acuity oculus dexter (OD) equal to counting fingers at two feet. On imaging, she was found to have a right orbital apex tumor causing compressive optic neuropathy. She received the intensity modulated radiation therapy (IMRT). After completion of the therapy, she had regained essentially a full vision with visual acuity OD of 20/30 without corrective lenses. The treatment rationale and pertinent literature are discussed in this article.

Multisession Gamma Knife Radiosurgery for Orbital Apex Tumors

This study was performed to analyze the outcome of multisession gamma knife radiosurgery (GKS) in benign tumors located at the orbital apex. Medical records of 23 patients who underwent multisession GKS for benign orbital apex tumors were reviewed retrospectively. Three patients were diagnosed by histology, and the other 20 patients were given the diagnoses on the basis of clinical and radiological findings. Diagnoses included cavernous hemangioma (8 cases), meningioma (8 cases), and schwannoma (7 cases). All patients were treated with 4 sessions of GKS with 12 hours of interval. Median marginal dose in each session was 5 Gy (range, 4.5-5.5 Gy) at the 50% isodose line (range, 50%-55%). Mean clinical and imaging follow-up duration after treatment were 52.1 and 34.2 months, respectively. Tumor control was achieved in 22 patients (95.7%). Significant tumor shrinkage was observed in 17 patients (73.9%), and mean tumor volume reduction rate was 53.9%. Visual function was improved in 16 patients (69.6%) and stable in 4 patients (17.4%). Deterioration of visual acuity was reported by 3 patients (13.0%). Clinical and radiological response to multisession GKS was most excellent in cavernous hemangiomas with tumor control in all patients, and the mean tumor volume reduction rate was 68.3%. Multisession GKS proved to be an effective and safe management strategy for benign orbital apex tumors. Response to treatment was different according to the pathology, and multisession GKS may be considered as the initial treatment of choice for specific pathology such as cavernous hemangioma.

Management of the optic canal invasion and visual outcome in spheno-orbital meningiomas

ObjectiveSpheno-orbital meningiomas often present with visual deficit due to invasion of the optic canal by the tumor. This study discusses the reasons of visual impairment, the choice of the surgical approach according to the type of optic canal involvement, and the factors correlated to the visual outcome in patients harboring a spheno-orbital meningioma. Materials and methodsA surgical series of 60 spheno-orbital meningiomas is reviewed. The preoperative visual symptoms, the involvement of the optic canal in both neuroradiological studies and surgical descriptions, the different surgical approaches are reviewed. These data are correlated with the postoperative visual outcome. ResultsThe 60 spheno-orbital meningiomas were classified in 4 types according to the intraorbital tumor localization: type I, supero-lateral (18 cases); type II, inferomedial (8 cases); type III, orbital apex (22 cases); type IV, diffuse (12 cases). Thirty-six of the 60 patients (60%) had variable decrease of the visual acuity on the tumor side. Forty-three patients (71.6%) had tumor extension into the optic canal on imaging studies. On the whole, 36 patients among 43 with invasion of the optic canal (83.7%) had preoperative visual dysfunction; on the other hand, none among 17 patients without tumor invasion of the optic canal had visual dysfunction.The surgical approaches according to the tumor location were as follows. A supraorbital-pterional approach was used in the 8 inferomedial tumors, in the 22 orbital apex tumors, and in 9/12 diffuse tumors; these last two types had concentric involvement of the optic canal. Three diffuse tumors with significant extension in the infratemporal fossa were operated on via a frontotemporal-orbitozygomatic approach. A wide decompression of the optic canal was performed in all cases, excepting in two inferomedial tumors without optic canal invasion. The 18 patients with lateral tumors were approached via a lateral orbitocranial approach, including removal of the sphenoid wing and lateral orbital wall without bone flap; the resection of the lateral aspect of the optic canal was performed in the 3 cases with canal invasion.Postoperative improvement of the visual function was observed in 18 of 36 cases with visual dysfunction (50%). The rate of visual improvement was significantly higher in cases with lateral involvement (3/3 or 100%) than in those with concentric involvement of the optic canal (11/27 or 40.7%). ConclusionThe invasion of the optic canal by the tumor is the main reason of visual dysfunction in patients with spheno-orbital meningiomas. A wide opening of the optic canal must be performed routinely in patients with orbital apex and diffuse orbital tumors, where there is concentric invasion of the optic canal wall. In these cases the supraorbital-pterional approach is the technique of choice. In selected cases with lateral intraorbital tumors and invasion of the lateral aspect of the optic canal the complete tumor resection coupled with good decompression of the optic nerve may be achieved via a less invasive lateral orbitocranial approach without craniotomy.

Transnasal endoscopic removal of orbital apex cavernous hemangioma

To explore the strategy and indications of endoscopic transnasal resection of orbital apex cavernous hemangiomas (OACH). Eleven patients aged from 30 to 62 years-old diagnosed as OACH bypostoperative histopathology were reviewed retrospectively. Four males and 7 females were included. Both ophthalmological examination and rhinologic evaluation were adopted preoperatively. The surgeries were carried out under general anesthesia endoscopically by the same senior surgeon. After ethmoidectomy, the orbital lamina papyracea was opened, and the orbital fat and muscles were pushed back into the orbit by using the brain cotton, and then the orbital tumor was removed. The patients were kept follow-up both in ophthalmologic and rhinologic departments. As suggested by preoperative imagings, 4 lesions located in the extraconal space (nasal side), 6 between the optic nerve and the internal rectus muscle of the intraconal space, and 1 outside the optic nerve in the intraconal space. Total resection was achieved in 9 cases, and orbital decompressions were done in 2 cases. Meanwhile, orbital wall reconstruction was done in 7 cases. The follow up ranged from 6 to 47 months. Seven patients achieved visual acuity improvement and no deteriorations were found in other 4 patients. Defects of vision field in 3 patients disappeared after 2 weeks. No operative or postoperative complications occurred. The OACH located in the nasal side of extraconal space and between the optic nerve and the internal rectus muscle of the intraconal space can be accessed endoscopically by intranasal approach. Using the brain cotton to push the orbital fat and muscles back into the orbit and an experienced endoscopic surgeon are important to access a successful intranasal endoscopic removal of orbital apex tumor.

Surgical treatment of orbital apex tumors by refined transcranial orbitotomy through superolateral approaches

Objective To investigate the indications,approaches,and essentials of cranio-orbital surgeries for orbital tumors.Methods Fifty-five orbital tumors treated by refined transcranial orbitotomy through superolateral approaches,from 2003 to 2011,were reviewed.Thirty-nine tumors were excised with orbitopterional approaches,while 16 were excised with orbitofrontal approaches,orbitozygomatic approaches,or zygomatic-pterional approaches.Results Of the 55 orbital tumors,38 (69.1％) were totally resected,7 (12.7％) were subtotally resected,and 7 (12.7％) were partly resected.The other 3 (5.5％)took biopsies.The optic functions improved or retained in 45 (81.8％) patients.Nine (16.4％) patients suffered from eye-moving disfunctions or visual-acuity declines,and 1 (1.8％) patient suffered from hemiparalysis.Conclusions Tumors in the posterior and apex part of the orbit,or tumors extended from the orbit to the cranial,can be well treated through transcranial approaches.Appropriate methods may help to bring maximum convenience to exposure and excision of the lesions,and to conserve the functions.Unnecessary injuries could be avoided as well. Key words: Orbit; Orbital neoplasms ; Microsurgery; Prognosis

Benign Orbital Apex Tumors Treated with Multisession Gamma Knife Radiosurgery

The orbital apex is an important anatomic landmark that hosts numerous critical neurovascular structures. Tumor resection performed at this complex region poses a therapeutic challenge to orbital surgeons and often is associated with significant visual morbidity. This article reports the efficacy and safety of multisession gamma knife radiosurgery (GKRS) in benign, well-circumscribed tumors located at the orbital apex. Retrospective interventional case series. Five patients with visual disturbances resulting from a benign, well-circumscribed orbital apex tumor (3 cases of cavernous hemangioma and 2 cases of schwannoma). Each patient treated with GKRS with a total radiation dose of 20 Gy in 4 sessions (5 Gy in each session with an isodose line of 50%) delivered to the tumor margin. Best-corrected visual acuity, visual field changes, orbital imaging, tumor growth control, and side effects of radiation. All patients demonstrated improvement in visual acuity, pupillary responses, color vision, and visual field. Tumor shrinkage was observed in all patients and remained stable until the last follow-up. No adverse events were noted during or after the radiosurgery. None of the patients experienced any radiation-related ocular morbidity. From this experience, multisession GKRS seems to be an effective management strategy to treat solitary, benign, well-circumscribed orbital apex tumors.

Unilateral proptosis: a diagnostic clinical and neuroimaging challenge

INTRODUCTION: The differential diagnosis of unilateral proptosis includes tumors, infections often related with immunological process, trauma, vascular diseases or axial myopia. CASE REPORTS: We present two patients with unilateral proptosis. A 34-yeard old caucasian man with a 3-months history of proptosis of the left eye with diplopia and normal visual acuity; initial CT scan examination showed highly suggestive finding for orbital apex tumor or glioma of the optic nerve, but a second CT scan with complementary projections was performed and a highly swollen inferior rectus muscle simulating an orbital apex tumor was visible. A diagnosis of unilateral thyroid ophthalmopathy was made. A 56-year old caucasian woman with unilateral exophthalmus and medical history of Graves’ hyperthyroidism; previously Graves’ ophthalmopathy was suggested. Fundoscopy showed myopic fundus. The CT scan revealed normal extraocular muscles without swollen. CONCLUSION: These two cases of unilateral proptosis are described because of their clinical and radiological differential diagnosis. We suggest that the accurate diagnosis of a unilateral proptosis remains a big challenge to the neuroophthalmologist, endocrinologist, immunologist, neurosurgeon and radiologist.

Transnasal Endoscopic Image-Guided Orbital Apex Surgery

Image guidance has emboldened surgeons while at the same time has allowed safer surgery. Transnasal endoscopic orbital surgery has been described in the treatment of nasolacrimal duct obstruction and Graves' ophthalmopathy and as an approach to optic canal decompression in cases of traumatic optic neuropathy. To expand the utility of this technology, we have utilized a transnasal endoscopic image-guided approach to biopsy or excise medial orbital apex tumors since 2001.

Primary Adenoid Cystic Carcinoma Presenting as an Orbital Apex Tumor

Primary adenoid cystic carcinoma occurring in the orbital apex is rare. We present the clinical features of a patient who initially presented with the clinical and radiologic features of an orbital pseudotumor. He developed features of orbital apex syndrome and repeat imaging showed a tumor of the orbital apex with intracranial invasion. He underwent radical skull base surgery and pathologic examination revealed adenoid cystic carcinoma in the orbital apex with a normal lacrimal apparatus. He received post operative radiation and the outcome in the light of a review of available literature is being discussed.

Orbital Decompression as an Alternative Management Strategy for Patients with Benign Tumors Located at the Orbital Apex

Tumors located in the intraconal portion of the orbital apex, especially those inferior to the optic nerve, can be difficult to access surgically, carrying a significant risk of ocular morbidity. The purpose of this study was to investigate outcomes in 5 patients with benign-appearing but symptomatic tumors located in the intraconal portion of the orbital apex in which orbital decompression was performed as an alternative management strategy to resection. Retrospective interventional case series. Five patients were diagnosed with a compressive optic neuropathy secondary to a benign-appearing tumor at the orbital apex. Each patient underwent surgical decompression of the affected orbit. None of the patients had the tumor biopsied or resected. Best-corrected visual acuity (VA), pupillary responses, visual fields (VFs), color vision, and orbital imaging. Each of the patients demonstrated improvement in visual function, as measured by VA, VFs, and, in some cases, color vision. One patient required a second orbital decompression for recurrent optic neuropathy 4 years after the initial decompression. Complications included ptosis and enophthalmos in 2 patients and diplopia in the extreme right gaze in 1 patient. Orbital decompression is a therapeutic option for patients with compressive optic neuropathies from benign orbital apex tumors, offering potential improvement in optic nerve function while sparing morbidity from attempts at surgical resection.

Interactive image guidance for surgical localization of orbital apical tumors

The surgical management of orbital apex tumors is difficult. Intervention in this small posterior space is fraught with complications. We have used an interactive image guidance system to successfully biopsy and resect three tumors at the orbital apex without significant surgical morbidity. These three cases are presented. These interactive image guidance systems have revolutionized skull-base surgery and have a role in the surgical management of orbital apex tumors.

Spontaneous Visual Improvement in Orbital Apex Tumor

Spontaneous Visual Improvement in Orbital Apex Tumor

Orbital apex: correlative anatomic and CT study

A detailed analysis of the coronal anatomic and CT appearances of the orbital apex is presented. In cadavers, coronal CT 9800 scans of the orbital apex and precisely corresponding cryomicrotomic sections were obtained. The CT appearance of the optic nerve, anulus of Zinn, and cranial nerves III-VI at the superior orbital fissure and orbital apex were determined. These anatomic structures were also demonstrated in clinical CT studies. Practical applications of the anatomic landmarks in evaluating orbital apex tumors are illustrated.

False diagnosis of orbital apex tumor by CAT scan in thyroid eye disease: Susac JO, Martins AN, Robinson B, Corrigan DF, Ann Neurol 1(4):397, 1977

False diagnosis of orbital apex tumor by CAT scan in thyroid eye disease: Susac JO, Martins AN, Robinson B, Corrigan DF, Ann Neurol 1(4):397, 1977

False diagnosis of orbital apex tumor by CAT scan in thyroid eye disease: by J.O. Susac, A.N. Martins, B. Robinson, and D.F. Corrigan. Ann Neurol 1: 397–398, 1977

False diagnosis of orbital apex tumor by CAT scan in thyroid eye disease: by J.O. Susac, A.N. Martins, B. Robinson, and D.F. Corrigan. Ann Neurol 1: 397–398, 1977

Unilateral endocrine exophthalmos. Diagnostic problems in association with computed tomography.

Swollen orbital extraocular muscles may mimic an orbital apex tumor on computed tomography. In five of our patients, in spite of highly suggestive findings on CT scanning, indicating the presence of an orbital apex lesion, the correct diagnosis proved to be endocrine exophthalmos. The value of complementary CT projections and of orbital ultrasonography to assist in the differential diagnosis is discussed, and the additional contribution of orbital phlebography for the confirmation of lesions in the orbital apex is emphasized.