Pediatric Orbital and Skull Base Pathology.

Pediatric orbital and skull base pathologies encompass a spectrum of inflammatory, sporadic, syndromic, and neoplastic processes that require a broad and complex clinical approach for both medical and surgical treatment. Given their complexity and often multicompartment involvement, a multidisciplinary approach for diagnosis, patient and family counseling, and ultimately treatment provides the best patient satisfaction and clinical outcomes. Advances in minimally invasive surgical approaches, including endoscopic endonasal and transorbital approaches allows for more targeted surgical approaches through smaller corridors beyond more classic transcranial or transracial approaches.

A novel classification for guiding the surgical approach for cranio-orbital lesions: a single institution case series of 45 cases and a literature review.

We aim to share our experience of the removal of cranio-orbital lesions (COLs) and propose a novel classification to guide the tailored approach selection. We retrospectively reviewed 45 consecutive patients with COLs who underwent surgery performed by the same neurosurgeon between November 2010 and November 2022. The surgical approach was selected according to the anatomical region classification of the COLs. For lesions limited to space A (lateral superior orbital fissure, SOF), the pterion or extended pterion approach (PA or EPA) was used. For lesions limited to space B (extraconal compartment medial SOF, and cavernous sinus CS) and C (intraconal compartment, medial SOF, and CS), the pretemporal transcavernous approach (PTCA) was used. For lesions limited to space D (intraconal compartment and optic canals), the subfrontal approach (SA) was used. For lesions extending into the infratemporal fossa (ITF), the cranio-orbito-zygomatic approach (COZA) was used. For lesions involving pterygopalatine fossa (PPF), the endoscopic transnasal approach (ETNA) was used. We analyzed the clinical manifestations, imaging data, surgical approaches, surgical outcomes, neurological outcomes, and follow-up data. Gross total resection was performed in 35 patients (35/45, 77.8%). SA, PA, EPA, PTCA, COZA, and ETNA were performed in 9, 9, 10, 10, 6, and 1 case(s), respectively. Progression of the residual tumor was observed in 6 cases (1 adenoid cystic carcinoma and 5 meningiomas). Surgical approach selection plays a vital role in patient prognosis. This novel classification based on the involvement of anatomic space could help surgeons select an appropriate approach to remove the COLs.

Site-based customized surgical approaches for orbital lesion and their outcomes - A case series.

INTRODUCTION AND IMPORTANCE: The orbit is a complex region of the body that is susceptible to several diseases. The term "anterior orbitotomy" describes access to the orbit through the conjunctiva or eyelid from the front. This includes transconjunctival, medial, inferior, and superior approaches. The superior and lateral regions of the posterior orbit can be adequately exposed using a frontotemporal orbitozygomatic approach. The main aim of orbital surgery is to preserve vision. We discuss numerous surgical procedures for ocular tumours in this case series, along with the results of those procedures. CASE PRESENTATION: In this case series, various surgical procedures for ocular tumours are discussed, along with their results postoperatively. Meningioma, cavernous hemangioma, dermoid, and Erdheim Chester disease were among the diagnoses. The early surgical outcome parameters in this case series were increased ocular motility and decreased proptosis. CLINICAL DISCUSSION: A medial orbitotomy was done as the lesion was in the medial orbit in our first case. In the second, third, and fourth cases, lateral orbitotomies were done because the lesion was in the lateral orbit. The frontotemporal orbitozygomatic approach was used in the fifth and sixth cases with the lesion reaching the orbital apex. Patients with symptoms should consider surgical excision, which involves completely removing the tumour while maintaining the functionality of the muscles and optic nerve. Having a good surgical view and exposure is essential to understand the anatomical relationships in a small area. CONCLUSION: The surgical strategy should be used in a manner that maintains visual acuity, limits injury to nearby objects, lowers postoperative morbidity, and is cosmetically acceptable.

Evaluation of orbital lesions with DCE-MRI: a literature review.

PURPOSE: To provide a major review on the applications of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in evaluating orbital lesions. This review also outlines selected scenarios where DCE-MRI may be helpful. METHODS: A comprehensive retrospective literature review of all English language publications on PubMed, EMBASE, and Google Scholar between 1994 and 2022. This literature review examined the specific applications and clinical scenarios surrounding the utility of DCE-MRI in orbital lesions and various findings that have been presented in the current literature. RESULTS: DCE-MRI provides information on tissue physiology and permeability, beyond the anatomical features displayed on static imaging. Various measured parameters (qualitative, semi-quantitative, and quantitative) obtained by DCE-MRI have been used to differentiate between benign and malignant lesions, specific orbital lymphoproliferative diseases (OLPD), lacrimal gland lesions, and various rare orbital tumours. DCE-MRI has a limited role as an initial diagnostic imaging modality. However, DCE-MRI may prove to have benefit in predicting and monitoring treatment response in orbital lymphoma as a critical imaging study, but literature specific to orbital malignancies remains limited. CONCLUSION: The value of DCE-MRI may be in situations of diagnostic uncertainty, where it may be an additional imaging aid following conventional imaging techniques. It may also act as a critical imaging modality for monitoring of orbital tumour treatment response, but the literature remains limited. Standardisation of imaging protocol, measured parameters, and statistical analysis remain limitations of this imaging technique.

Segmentation of orbital and periorbital lesions detected in orbital magnetic resonance imaging by deep learning method.

Purpose: Magnetic resonance imaging (MRI) has a special place in the evaluation of orbital and periorbital lesions. Segmentation is one of the deep learning methods. In this study, we aimed to perform segmentation in orbital and periorbital lesions. Material and methods: Contrast-enhanced orbital MRIs performed between 2010 and 2019 were retrospectively screened, and 302 cross-sections of contrast-enhanced, fat-suppressed, T1-weighted, axial MRI images of 95 patients obtained using 3 T and 1.5 T devices were included in the study. The dataset was divided into 3: training, test, and validation. The number of training and validation data was increased 4 times by applying data augmentation (horizontal, vertical, and both). Pytorch UNet was used for training, with 100 epochs. The intersection over union (IOU) statistic (the Jaccard index) was selected as 50%, and the results were calculated. Results: The 77th epoch model provided the best results: true positives, 23; false positives, 4; and false negatives, 8. The pre-cision, sensitivity, and F1 score were determined as 0.85, 0.74, and 0.79, respectively. Conclusions: Our study proved to be successful in segmentation by deep learning method. It is one of the pioneering studies on this subject and will shed light on further segmentation studies to be performed in orbital MR images.

Gamut of Orbital Lesions in a Tertiary Neurocenter-A Clinicopathological Study of Lesions Seen Over a Period of One Decade.

Background: The orbital region is an anatomically complex area comprising crucial contiguous/adjacent structures. Since the eye has a neuroectodermal basis of embryogenesis, many of the lesions may be similar to those arising in the central nervous system. Objective: To record and describe the clinicopathological spectrum of orbital lesions presenting to a neurology center. Study Setting: The retrospective study included biopsy/resected specimens of patients with orbital/ophthalmic lesions referred to the Department of Neuropathology, between February 2007 and February 2018. Materials and Methods: : The demographic, clinical, and radiological details were retrieved from the departmental archives and the slides were reviewed. Results: There were 99 cases in the period of the study (2007-2018) with a peak in fourth and fifth decades (age range: 5 months to 68 years; mean: 37.2 years; M: F =1.06: 1). Eighty-six (86.8%) cases had epicenter in the orbit, whereas 13 (13.13%) cases were extraorbital with orbital extension. The benign neoplasms predominated (50/99, 50.5%) followed by malignant neoplasms (24/99, 24.24%), infective conditions (11/99, 11.11%) and tumor like conditions (7/99, 7.07%). The most common benign tumor was vascular tumor (17/50, 34%) followed by meningioma (12/50, 24%), while epithelial malignant tumor (6/24, 25%) was the most common malignancy. Fungal infection was the most frequent infective condition (6/11, 54.5%). Conclusion: The spectrum of ocular-orbital lesions varies with the geographic area and the nature of the institute catering to the needs of patients. The spectrum of lesions that we encountered from a neurological institute was vastly different from that reported from ophthalmic centers with very low frequency of retinoblastomas.

The Versatility of the Free Vastus Lateralis Muscle Flap: Orbital Reconstruction After Removal of Complex Vascular Malformation in a Pediatric Patient.

Introduction: Vascular orbital lesions in pediatric population represent a demanding therapeutic challenge which requires a multidisciplinary team. In severe cases, orbital enucleation can be considered. Surgical management of enucleated orbital region in children, differently from the adults, represents a challenging procedure owing to the intrinsic relation between volume replacement and normal orbital growth. Many reconstructive options have been proposed, and many donor sites have been utilized for this purpose but each one have demonstrated potential disadvantages. Despite its well-known versatility, no report of the vastus lateralis free flap in children requiring orbital reconstruction exists in literature. Herein, we propose this surgical strategy as a valid option for the reconstruction of an extended orbital defect in a pediatric patient suffering from a mixed type of vascular malformation. Material and Methods: A patient was referred from a foreign country with an unclear medical history, presenting exorbitism and exophthalmos, proptosis of the eyeball, visus 4/10, and limited ocular motility. We made clinical-instrumental investigations with a diagnosis of complex vascular malformation. It expanded in intraorbital and retrorbital space with bulb anterior dislocation and optic nerve involvement. We performed an emptying of the orbital content via transconjunctival and via coronal incision with eyelid preservation. A free vastus lateralis muscle flap was used for reconstruction, filling the orbital cavity. We anastomosed the flap on the superficial temporal artery. An ocular conformator was then positioned. Results: We report the result at 12 months, showing a good orbital rehabilitation with an adequate prosthetic cavity, a good recovery of volume and facial symmetry, guaranteeing balanced orbital and periorbital growth. There were no major or minor complications associated with the procedure. Discussion: The reconstruction of the orbit remains a "surgical challenge" both in adults, whose goal is the restoration of volume, adequate symmetry and facial esthetics, and children, in which correcting the asymmetry has the additional objective to balance orbital growth. Many reconstructive techniques have been proposed, including the use of free flaps. The versatility of the free vastus lateralis muscle flap is well-known. It offers adequate amount of tissue with minimal morbidity to the donor site, provides a long pedicle, gives the possibility of simultaneous work in a double team, and has a constant anatomy and a safe and rapid dissection. There are no descriptions of its use for pediatric orbital reconstructions. Conclusions: In our opinion, the free vastus lateralis flap should be included as one of the best option for orbital pediatric reconstruction after enucleation.

Clinical Outcome and Technical Nuances After Resection of Orbital Cavernous Venous Malformations-A Single-Center Experience.

BACKGROUND: Cavernous venous malformations (CVMs) represent the most common benign intraorbital lesions. Enlarging or symptomatic CVMs (progressive proptosis or visual disturbances) are treated by surgical resection. For this, a variety of different surgical approaches have been described. The aim of this study was to present a contemporary series of orbital CVMs treated via open microsurgical approaches. METHODS: In this study, patients who underwent resection of orbital CVMs between 2002 and 2019 were included. Presenting symptoms were noted and neuro-ophthalmologic examinations performed pre- and postoperatively. For surgical resection, the location of the orbital CVM and its relation to the orbital anatomy led to decision-making for appropriate approaches. A comparison between anatomical location and surgical outcome was performed. RESULTS: Overall, 35 patients with orbital CVMs were included. Most common presenting symptoms were progressive proptosis (43%) and visual disturbances (34%). Most common location was the lateral quadrant (37%) followed by the superior quadrant (20%). A subfrontal craniotomy was performed in 40% of cases followed by a supraorbital craniotomy including the orbital rim in 34% of cases. For surgical excision, a cryo-probe was used in 30 patients, and complete resection was feasible in all cases. Location of a CVM within the superior quadrant was associated with improved postoperative recovery of visual acuity. No differences for clinical outcomes were observed depending on the surgical approach. CONCLUSIONS: Resection of orbital CVMs is indicated in patients with visual disturbances or progressive proptosis. In these, microsurgical approaches can be used with minimal morbidity for complete removal of these well-circumscribed lesions.

Clinical correlation recommended: accuracy of clinician versus radiologic interpretation of the imaging of orbital lesions.

Purpose: To assess the accuracy of radiographic interpretation between the clinician and radiologist when compared to histopathology of orbital lesions. Methods: A retrospective chart review of patients at the University of California Davis Eye Center who underwent orbitotomy from 1/1/2000 to 5/22/2019 was performed. Charts with a preoperative imaging report, preoperative clinical assessment including the clinician's interpretation of imaging, and histopathologic diagnosis were included. The specific diagnoses were grouped into related classes of pathology for the analysis. The clinical and radiologic assessments were compared against the final histopathologic diagnosis for concordance. A concordance analysis was performed. Results: 242 patients (mean age 49 years, 53.5% female) were reviewed. Of these records 185 documented the clinician's clinical impression, the radiology report, as well as the histopathology report. The clinician's preoperative assessment had substantial agreement [kappa = 0.72 (0.65,0.79)] with the final histopathologic result and was correct in 75.7% (140/185) of cases whereas the radiology report was correct in 52.4% (97/185) with a moderate level of agreement [kappa = 0.47 (0.39, 0.55)]. In 49.2% (91/185) of cases the final histopathology correlated with both the clinical impression and radiology report [kappa = 0.58 (0.55, 0.61)]. Conclusions: The accurate interpretation of orbital imaging is a challenge and histopathologic examination remains the gold standard for diagnosis. While orbital imaging is a valuable diagnostic tool the interpretation of these studies is most accurate when conducted in the context of the patient's medical history, clinical exam, and with the physician most familiar with various orbital lesions.

The role of ultrasonography in differential diagnosis of orbital lesions.

AIMS: The aim of this study is to assess the value of ultrasound in the differential diagnosis of orbital lesions. METHODS: One hundred and thirty-eight patients with clinical features of an orbital mass were examined by orbital ultrasound prior to undergoing surgery, from January 2000 to January 2017. All patients underwent excisional or incisional orbital biopsy. The results of orbital ultrasonography were compared with the final histological diagnosis. RESULTS: Orbital lesions were localized by ultrasonography in 133/138 cases (96.4%); the false-negative rate of orbital echography was 3.6% (5/138). The nature of the orbital lesions was correctly determined by ultrasonography in 54.3% of the cases (75/138) preoperatively (true positives). In 58/138 (42%) patients, there was no correspondence between the ultrasound diagnosis and the histological diagnosis (false positives). The sensitivity of orbital ultrasonography for the detection of an orbital mass was 93.75% (CI 87.87-99.63%), while the specificity yielded no meaningful result (CI 0.00-60.24%). Moreover, the specificity of orbital ultrasonography to identify a malignant tumor falls within a CI of 0-62.72%. CONCLUSIONS: Orbital ultrasonography is a rapid and noninvasive test that is highly sensitive in displaying an orbital mass; however, the specificity in the differential diagnosis of orbital lesions is not meaningful, particularly if malignancy is suspected. The assessment of orbital diseases requires multiple diagnostic approaches to balance the strengths and weaknesses of each method.

Endoscopic orbital decompression for proptosis in non-thyroid eye disease.

PURPOSE: Orbital decompression is an established surgical treatment option for a range of orbital conditions. We report the outcomes of endonasal decompression to recess the globe for conditions and pathologies other than thyroid eye disease. METHODS: This was a retrospective case series of patients who underwent endoscopic orbital decompression for proptosis secondary to non-thyroid eye disease orbital pathologies. The procedures were carried out by oculoplastic surgeons across two hospital sites between January 2011 and July 2018. Information collected includes patient demographics, diagnosis, surgical details, pre- and postoperative clinical findings (including visual acuity, exophthalmometry readings, intraocular pressure, ocular motility and diplopia), complications and further treatment. RESULTS: There were seven cases of endoscopic decompression, each due to a different pathology. The reasons for decompression were proptosis secondary to optic nerve sheath meningioma (1); sphenoid wing meningioma (1); idiopathic myositis (1); axial myopia (1); chronic third nerve palsy (1); to protuberant keratoprosthesis (1); and Crouzon syndrome with corneal exposure (1). Visual acuity remained stable or improved in all patients postoperatively. There was an average reduction in proptosis of 3.5 ± 1.4 mm (standard deviation - SD). Ocular motility remained stable in 100% (7/7). There were no intraoperative or postoperative complications, including no new cases of postoperative diplopia. CONCLUSIONS: Endoscopic orbital decompression can be performed for patients with proptosis associated with a large globe, facial dysplasia or medial and infero-medial orbital lesions.

Outcomes After Pterional and Supraorbital Eyebrow Approach for Cranio-Orbital Lesions Communicated via the Supraorbital Fissure-A Retrospective Comparison.

OBJECTIVE: The pterional approach (PA) has been used to remove cranio-orbital lesions that have communicated via the supraorbital fissure (SOF). The supraorbital eyebrow approach (SEA) has become increasingly popular as a minimally invasive approach. However, few reports have examined the SEA for cranio-orbital lesions. We assessed the relative advantages, disadvantages, and selection criteria for these 2 approaches. METHODS: The data from all consecutive patients who had undergone removal of a cranio-orbital lesion that communicated via the SOF using the PA or SEA were analyzed. RESULTS: Of the 25 patients, 13 had undergone surgical resection using the SEA and 12 had undergone resection using the PA. The SEA provided better cosmetic satisfaction, resulted in a shorter surgical time, and required a shorter incision (P < 0.05). Proptosis had improved significantly in all the patients. No significant differences were found in the total resection rates, visual outcomes, recovery of ptosis, or other new surgically related complications between the 2 groups (P > 0.05). All the patients were followed up for an average of 21.9 months. One patient in each group had developed recurrence or progression that required radiotherapy. The disease of the other patients with subtotal resection was stable or atrophic. CONCLUSION: The SEA provided better cosmetic satisfaction, resulted in a shorter surgical time, and required a shorter incision. The SEA might be a more minimally invasive option for lesions communicating via the SOF without obvious hyperostosis. The PA might be more reasonable for lesions with obvious hyperostosis and more extensive lesions in the cavernous sinus.

Efficacy and Tolerability of CyberKnife Stereotactic Robotic Radiotherapy for Primary or Secondary Orbital Lesions: A Single-Center Retrospective Experience.

INTRODUCTION: Orbital lesions are rare, but are likely to become symptomatic and can impact on patients' quality of life. Local control is often difficult to obtain, because of proximity to critical structures. CyberKnife stereotactic robotic radiotherapy could represent a viable treatment option. MATERIALS AND METHODS: Data on patients treated for intraorbital lesions from solid malignancies were retrospectively collected. All patients underwent treatment with CyberKnife system. We analyzed local control, response rate, symptoms control, progression-free survival and overall survival, acute and late toxicity. RESULTS: From January 2012 to May 2017, 20 treatments on 19 patients were performed, with dose ranging from 24 to 35 Gy in 1 to 5 fractions, prescribed at an average isodose line of 79.5% (range: 78-81). After a mean follow-up of 14.26 months (range: 0-58), overall response rate was 75%, with 2 and 4 patients presenting a partial and complete response, respectively. Mean time to best measured response was 15.16 months (range: 2-58). Thirteen patients were alive, with a local control rate of 79%. Mean time to local progression was 5 months (range: 3-7). Three patients reported improvement in symptoms after treatment. Mean planning target volume dose coverage was 97.2% (range: 93.5-99.7). Mean maximum dose (D max) to eye globe, optic nerve, optic chiasm, and lens was 2380.8 cGy (range: 290-3921), 1982.82 cGy (range: 777.3-2897.8), 713.14 cGy (range: 219.5-2273), and 867.9 cGy (range: 38-3118.5). Four patients presented acute toxicity. CONCLUSION: This current retrospective series demonstrated that CyberKnife robotic stereotactic radiotherapy is a feasible and tolerable approach for intraorbital lesions.

Eyebrow Incision for Combination Supraorbital Minicraniotomy with Orbital Osteotomy: Application to Cranio-Orbital Lesions.

BACKGROUND: A pterional-orbital or subfrontal-orbital approach is recommended as a surgical treatment in cranio-orbital lesions. We describe a less invasive approach through an eyebrow incision combined supraorbital minicraniotomy and orbital osteotomy for treating some selected cranio-orbital lesions. METHODS: Sixteen patients with different cranio-orbital lesions were treated using this less invasive approach. Postoperative outcomes were evaluated to shed light on specific parameters related to this approach. RESULTS: The 16 patients with cranio-orbital lesions underwent 17 operations. A total resection was achieved in 11 lesions. All the patients were followed up for 3-54 months. Postoperative proptosis improved in all cases. Five cases of visual impairment were improved, but 4 patients with blindness did not recover. One patient with bitemporal hemianopia recovered. Three patients with ocular dyskinesia did not recover. Two patients had transient cranial nerve III palsy, and 2 patients had cranial nerve VI palsy. One had delayed hydrocephalus. One died 1 year later as a result of pulmonary metastases. One recurred and the patient underwent a second operation. All the patients had a modified Rankin Scale score ≤1 at 12 weeks follow-up. CONCLUSIONS: Some selected cranio-orbital lesions can be treated through a supraorbital eyebrow approach with orbital osteotomy. The presence of retro-ocular fat allows the orbital lesions to be classified as a lesion of the intraretro-ocular or extraretro-ocular fat. It is safe to resect the lesion of extraretro-ocular fat from the retro-ocular fat interface. However, the lesion with optic nerve and extraocular muscles involved should be removed from the intermuscular septae.

Role of Fine Needle Aspiration Cytology as a Diagnostic Tool in Orbital and Adnexal Lesions.

PURPOSE: To evaluate the role of fine needle aspiration (FNAC) as a diagnostic tool in cases of orbital and ocular adnexal masses. Cytological findings were correlated with histopathological diagnosis wherever possible. METHODS: FNAC was performed in 29 patients of different age groups presenting with orbital and ocular adnexal masses. Patients were evaluated clinically and investigated by non-invasive techniques before fine needle aspiration of the masses. Smears were analyzed by a cytologist in all cases. Further, results of cytology were compared with the histopathological diagnosis. RESULTS: The age of patients ranged from 1 to 68 years (mean: 29.79±19.29). There were 14 males and 15 females with a male to female ratio of 0.93:1. Out of 29 cases, 26 aspirates were cellular. Cellularity was insufficient in three (10.34%) aspirates. Out of 26 cellular aspirates, 11 were non-neoplastic while 15 were neoplastic on cytology. Subsequent histopathologic examination was done in 21/26 cases. Concordance rate of FNAC in orbital and ocular adnexal mass lesions with respect to the precise histologic diagnosis was 90%. CONCLUSION: When properly used in well-indicated patients (in cases where a diagnosis cannot be made by clinical and imaging findings alone), FNAC of orbital and periorbital lesions is an invaluable and suitable adjunct diagnostic technique that necessitates close cooperation between the ophthalmologist and cytologist. However, nondiagnostic aspirates may sometimes be obtained, and an inconclusive FNAC should not always be ignored.