S. Tonya Stefko

A case of bilateral silent sinus syndrome presenting with chronic ocular surface disease.

The silent sinus syndrome is characterized by atelectasis of the maxillary sinus in the setting of subclinical maxillary sinusitis and obstruction of the osteomeatal unit. The resultant expansion of orbital volume causes enophthalmos and hypoglobus. A review of the literature reveals only unilateral cases. The authors present a case of bilateral silent sinus syndrome presenting with chronic ocular surface disease. A 56-year-old man was referred for tarsorrhaphy to treat a chronic recurrent corneal ulcer. Exam revealed an area of corneal thinning in the left eye. The patient appeared cachectic and there was enophthalmos, hypoglobus, and lagophthalmos on both sides, left worse than right. Due to the patients appearance and a history of sinonasal malignancy, recurrent malignancy was suspected. A systemic workup was unrevealing, and radiographic studies showed the features of silent sinus syndrome bilaterally. The patient underwent sinus surgery and placement of a left orbital floor implant.

Linkage analysis of X-linked cone-rod dystrophy : Localization to Xp11.4 and definition of a locus distinct from RP2 and RP3

Progressive X-linked cone-rod dystrophy (COD1) is a retinal disease affecting primarily the cone photoreceptors. The COD1 locus originally was localized, by the study of three independent families, to a region between Xp11.3 and Xp21.1, encompassing the retinitis pigmentosa (RP) 3 locus. We have refined the COD1 locus to a limited region of Xp11.4, using two families reported elsewhere and a new extended family. Genotype analysis was performed by use of eight microsatellite markers (tel-M6CA, DXS1068, DXS1058, DXS993, DXS228, DXS1201, DXS1003, and DXS1055-cent), spanning a distance of 20 cM. Nine-point linkage analysis, by use of the VITESSE program for X-linked disorders, established a maximum LOD score (17.5) between markers DXS1058 and DXS993, spanning 4.0 cM. Two additional markers, DXS977 and DXS556, which map between DXS1058 and DXS993, were used to further narrow the critical region. The RP3 gene, RPGR, was excluded on the basis of two obligate recombinants, observed in two independent families. In a third family, linkage analysis did not exclude the RPGR locus. The entire coding region of the RPGR gene from two affected males from family 2 was sequenced and was found to be normal. Haplotype analysis of two family branches, containing three obligate recombinants, two affected and one unaffected, defined the COD1 locus as distal to DXS993 and proximal to DXS556, a distance of approximately 1.0 Mb. This study excludes COD1 as an allelic variant of RP3 and establishes a novel locus that is sufficiently defined for positional cloning.

Endoscopic endonasal orbital cavernous hemangioma resection: global experience in techniques and outcomes.

Endoscopic orbital surgery represents the next frontier in endonasal surgery. The current literature is largely composed of small, heterogeneous, case series with little consensus regarding optimal techniques. The purpose of this study was to combine the experience of multiple international centers to create a composite of the global experience on the endoscopic management of a single type of tumor, the orbital cavernous hemangioma (OCH).

Orbital surgery: state of the art.

Much has been written about the repair of orbital fractures, yet some debate still exists among surgeons with regard to indications for and timing of fracture repair and various surgical techniques. Controversies regarding the surgical maneuvers include the incision, surgical approach, and methods of wound closure. More detailed imaging modalities have allowed clinicians to understand the injuries more completely and plan for and execute more ideal reconstructions. Recent advances in orbital implant materials and the role of endoscopy in orbital fracture repair add to this debate about which techniques would be best for particular injuries. This article discusses these issues and provides the most current literature review regarding the management of various orbital fractures.

Lateral Orbitotomy Approach for Lesions Involving the Middle Fossa: A Retrospective Review of Thirteen Patients

Background Classically used for treatment of orbital lesions, the lateral orbitotomy with cantholysis can be combined with a temporal craniectomy for lesions involving the middle cranial fossa. Objective To present a single-center experience with the lateral orbitotomy approach for lesions involving the middle fossa. Metuods Twenty-five patients underwent lateral orbitotomies from April 2012 to July 2015. Excluding patients with solely intraorbital pathologies, 13 patients’ clinical and radiographic records were retrospectively reviewed. Results Signs/symptoms in the 13 patients (ages 28-81) included proptosis (69%), decreased visual acuity (31%), diplopia (54%), and afferent pupillary defect (69%). Pathologies were meningioma (8), esthesioneuroblastoma, lymphoma, chordoma, Ewings sarcoma, and squamous cell carcinoma. Surgical goals were maximal safe resection in 8 patients, palliative debulking in 3 patients, and cavernous sinus biopsy in 2 patients. In 8 patients for whom maximal resection was the goal, 2 had gross total resection, while 6 had near-total resection. All patients (3) for whom palliation was the goal had symptomatic improvement. Both cavernous sinus biopsies obtained diagnostic tissue without complications. All patients with proptosis (n = 9) and diplopia (n = 7), and 2 of 4 patients with decreased visual acuity had improvement in their symptoms. No patient reported worsening of their symptoms. Mean follow-up was 12 mo (2-30 mo). Complications included oculorrhea (1), pseudomeningocele (2), transient ptosis (2), and forehead numbness (1). Conclusion The lateral orbitotomy is a promising approach for carefully selected lesions with involvement of both the lateral orbit and middle cranial fossa. It provides minimally invasive access for biopsy, decompression, or resection.

Combined Endoscopic Endonasal Transorbital Approach with Transconjunctival-Medial Orbitotomy for Excisional Biopsy of the Optic Nerve: Technical Note

Background Access to the intraorbital optic nerve segment can be facilitated via a transcranial approach that allows access to the entire orbital cavity. The endoscopic endonasal approach (EEA) combined with a transconjunctival-medial orbitotomy represents an alternative technique to achieve the same goal. Objective Report a surgical technique that allows total resection of the intraorbital optic nerve with minimal trauma and excellent results. Further extend and define the limits and indications of the EEA to orbital surgery. Methods A patient with rapidly progressive, but asymmetric, vision loss underwent EEA for optic nerve biopsy. Due to the undetermined histopathological diagnosis and complete unilateral vision loss, diagnostic total optic nerve resection was indicated. The entire intraorbital length of the nerve was resected via an endoscopic endonasal transorbital approach combined with transconjunctival-medial orbitotomy. Results A 2-cm intraorbital nerve segment was sent for pathological examination. The patient maintained normal extraocular movements and experienced no complications. The postoperative course was uneventful and the patient was discharged the next day. Conclusion The EEA provides another option for access to the entire optic nerve. It is a safe and effective technique lacking cosmetic defects and providing an alternative corridor to traditional transcranial approaches to the orbit.

Endoscopic Endonasal Optic Nerve Decompression for Fibrous Dysplasia

Objective To evaluate visual outcomes and potential complications for optic nerve decompression using an endoscopic endonasal approach (EEA) for fibrous dysplasia. Design Retrospective chart review of patients with fibrous dysplasia causing extrinsic compression of the canalicular segment of the optic nerve that underwent an endoscopic endonasal optic nerve decompression at the University of Pittsburgh Medical Center from 2010 to 2013. Main Outcome Measures The primary outcome measure assessed was best‐corrected visual acuity (BCVA) with secondary outcomes, including visual field testing, color vision, and complications associated with the intervention. Results A total of four patients and five optic nerves were decompressed via an EEA. All patients were symptomatic preoperatively and had objective findings compatible with compressive optic neuropathy: decreased visual acuity was noted preoperatively in three patients while the remaining patient demonstrated an afferent pupillary defect. BCVA improved in all patients postoperatively. No major complications were identified. Conclusion EEA for optic nerve decompression appears to be a safe and effective treatment for patients with compressive optic neuropathy secondary to fibrous dysplasia. Further studies are required to identify selection criteria for an open versus an endoscopic approach.

Minimally Invasive Approaches for Anterior Skull Base Meningiomas: Supraorbital Eyebrow, Endoscopic Endonasal, or a Combination of Both? Anatomic Study, Limitations, and Surgical Application

BACKGROUND Minimally invasive accesses to the anterior skull base include the endoscopic endonasal approach (EEA) and the supraorbital eyebrow approach. These 2 are often seen as competing approaches, not alternative or combinatory approaches. In this study, we evaluated the anatomic limitations of each approach and the combined approach for accessing the anterior skull base. METHODS Ten neurovascular injected cadaver heads were used for the study. The supraorbital approach to the anterior skull base was performed on 5 heads, and EEA was done on the other 5 heads. Then, the supraorbital approach was added to the 5 heads receiving EEA. Visualization and surgical limitations were recorded by the ability to perform resection of the crista galli, anterior clinoid, cribriform plate, and planum sellae. RESULTS The maximal lateral extension of EEA for anterior skull base was the midorbit line anteriorly but narrowing down toward the orbital apex. The limitation of the supraorbital approach was found mostly medial and anterior. Drilling of anterior skull base was impossible medially between the sphenoethmoidal suture and the posterior aspect of the crista galli. The combined approach showed complementary areas of visualization and surgical maneuverability. Three clinical cases were presented to illustrate the indications for the stand-alone supraorbital approach, EEA, and combined approach. CONCLUSION The limitations of the EEA when dealing with lateral extension of anterior skull base meningiomas, and the limitations of the supraorbital eyebrow approach for medial skull base drilling and reconstruction, can be overcome by a judicious, anatomically based combination of both approaches.

Nonhealing orbital floor fracture in a pediatric patient: A unique presentation of pseudo-silent sinus syndrome

ABSTRACT Silent sinus syndrome was first described as spontaneous enophthalmos and hypoglobus associated with subclinical maxillary sinusitis without prior trauma or surgery. This clinical entity has later been described after trauma in which damage to the ostiomeatal complex leads to atelectasis of the maxillary sinus. We report a case of a 14-year-old boy who presented 4 years after sustaining a non-operative orbital floor fracture with enophthalmos and transient diplopia. Computed tomography (CT) demonstrated enlargement in size of the original orbital floor fracture and bilateral maxillary sinus disease. Bilateral chronic sinusitis suggested an anatomical predisposition to sinusitis unrelated to the prior trauma. The authors propose that, in this case, negative pressure in the maxillary sinus and chronic inflammation led to bone resorption and failure of the orbital fracture to heal. This differs from prior reports of silent sinus syndrome in that there was complete resorption of bone of the orbital floor and no decrease in volume of the maxillary sinus given the open communication of the sinus and the orbit, making this a unique presentation of pseudo-silent sinus syndrome in a pediatric patient.

Congenital entropion and hypotropia secondary to duplication of the inferior rectus muscle.

Congenital anomalies of the extraocular muscles apart from congenital fibrosis are relatively rare. Duplication of extraocular muscles is a particularly rare congenital anomaly that has been rarely reported in the literature. We present a case of unilateral congenital entropion and hypotropia resulting from duplication of the inferior rectus muscle.