A single-institution review of lacrimal gland biopsies between 1962 and 2017.

PURPOSE: To examine the clinical and histopathologic characteristics of lacrimal gland biopsies at a tertiary academic center. METHODS: A retrospective chart review of patients undergoing lacrimal gland biopsy or excision between 1962 and 2017 was performed via the ocular pathology specimen log. All cases were reviewed for demographics, clinical presentation, and histopathologic diagnoses. RESULTS: Four hundred and two eyes in 356 patients were included in the analysis. Median age was 49 (range 5-91) with a female predominance (255, 72%, p < .001). Most had unilateral involvement (308, 86.5%) and visual acuity of 20/50 or better (332 eyes, 83%). Limitation in extraocular motility was present in 71 eyes (18%), relative afferent pupillary defect in 10 eyes (2.5%), and intraocular pressure 20 mmHg or above in 80 eyes (20%). The pre-operative radiology report commented on the enlargement of the lacrimal gland in 236 eyes (58.7%), and lack thereof in 73 eyes (18.2%). The most common histopathologic diagnoses were nonspecific inflammation or orbital pseudotumor (170, 42%), lymphoma (65, 16%), pleomorphic adenoma (22, 5.5%), adenoid cystic carcinoma (19, 4.7%), granulomatous inflammation (19, 4.7%), and normal lacrimal gland (16, 4%). Three hundred and seven cases were benign (76%) and 95 malignant (24%). The biopsy specimen was diagnostic in 343 (85%), and non-diagnostic in 59 (15%). CONCLUSIONS: This is a comprehensive review of one of the largest ocular pathology databases of lacrimal gland lesions. This study confirms the wide range of inflammatory and neoplastic conditions affecting the lacrimal gland and highlights the nuances of histopathologic diagnoses and diagnostic yield of biopsies in clinical practice.

Transcutaneous endoscopic orbital surgery: a less morbid alternative to lateral orbitotomy.

BACKGROUND: Retrobulbar lesions of the orbit situated lateral to the optic nerve are difficult to access. In this article, the authors would like to present a new perspective to approach these lesions where the goals of surgery are met with minimal complications. METHODS: A retrospective analysis of patients' charts was performed. RESULTS: For approaching retrobulbar lesions an endoscopic technique was developed. Four patients (2 male and 2 female patients) presenting with retrobulbar lesions lateral to the optic nerve have been included in this study. Two patients underwent endoscopic drainage for orbital abscess and two patients were successfully biopsied endoscopically. No intra-operative complications were noted. Open procedures such as lateral orbitotomy were avoided in all the cases. CONCLUSION: Transcutaneous orbital endoscopy is a safe and a versatile technique to approach retrobulbar lesions lateral to the optic nerve. It can be used as an effective alternative to lateral orbitotomy for well selected cases. One can obtain adequate material for histopathological examination and also drain deep-seated orbital abscess using this technique. It would be possible with increasing experience to use this technique for extended applications.

Neurosarcoidosis Presenting as Longitudinally Extensive Transverse Myelitis and Orbital Mass: A Case Report.

We describe a case of neurosarcoidosis in a 64-year-old female who presented with proptosis and orbital inflammation together with bilateral lower extremity neuropathy and longitudinally extensive transverse myelitis. These two entities are not commonly associated, and the etiology of the transverse myelitis was facilitated by an orbital biopsy. The transverse myelitis caused numbness in her lower extremities and tightness in her chest and abdomen, which progressed over weeks to difficulty walking and bilateral neuromuscular weakness. Magnetic resonance imaging (MRI) revealed longitudinally extensive transverse myelitis in the cervical and thoracic spine. Computed tomography (CT) imaging of the chest revealed right hilar and mediastinal lymphadenopathy and calcified subcarinal nodes. Positron emission tomography (PET) scan revealed hypermetabolism in the mediastinum and medial left orbit. Orbital biopsy revealed non-necrotizing granulomatous inflammation suggestive of sarcoidosis. The neurologic deficits and orbital inflammation responded well to intravenous corticosteroids. Neurosarcoidosis can present with unusual clinical manifestations, as evidenced by this patient.

Accuracy between clinical and radiological diagnoses compared to surgical orbital biopsies.

AIM: To assess the concordance between diagnosing orbital lesions by clinical examination, orbital imaging, and histological evaluation, in order to help guide future research and clinical practice. METHODS: A retrospective analysis was undertaken at a large regional tertiary referral centre of all surgical orbital biopsies performed over a 5-year period, from 1st January 2015 until 31st December 2019. Accuracy and concordance between clinical, radiological and histological diagnoses are reported as percentage sensitivity and positive predictive value. RESULTS: A total of 128 operations involving 111 patients were identified. Overall, sensitivities of 47.7% for clinical and 37.3% for radiological diagnoses were found when compared to the histological gold standard. Vascular lesions that have characteristic clinical and radiological features had the highest sensitivity at 71.4% and 57.1%, respectively. Inflammatory conditions showed the lowest sensitivity in both clinical (30.3%) and radiological (18.2%) diagnoses. The PPV for inflammatory conditions were 47.6% for clinical and 30.0% for radiological diagnoses. CONCLUSION: Accurate diagnoses are difficult to reach by relying on clinical examination and imaging alone. Surgical orbital biopsy with histological diagnosis should remain the gold standard approach for definitively identifying orbital lesions. Although larger scale prospective studies would help further refine concordance and guide future research avenues.

Computerized Tomography-Guided Core-Needle Biopsy of Orbital Space-Occupying Lesions: A Case Series.

PURPOSE: To describe our experience in performing biopsy of post-septal orbital masses with core needle under computerized tomography guidance (CT-CNB). METHODS: The medical records of all patients who underwent this procedure were reviewed. The procedure was performed under local anesthesia on a day case basis under a peribulbar block. A planning non-contrast computerized tomography (CT) scan of the orbits was performed to localise the mass. A 6-cm 18-G Temno Evolution® semi-automated biopsy needle was inserted through the skin into the orbit. Prior to further advancement of the needle, a low-dose CT limited to the previously determined plane was performed to confirm its position. The needle was then advanced, and the cutting needle was deployed to obtain the biopsy. RESULTS: Five patients who underwent CT-CNB were identified. The CNB was successful in 4 patients and revealed a metastatic prostate adenocarcinoma, diffuse large B-cell lymphoma, a metastatic neuroendocrine tumour, and orbital inflammatory disease. The biopsy failed in the fifth patient when the needle failed to penetrate the tumour despite good localisation on CT. He was eventually diagnosed with fibrous meningioma of the greater wing of sphenoid on open biopsy. None of the patients had any complications other than peri-ocular bruising which was present in all of them. CONCLUSION: CT-CNB of mass lesions located in the lateral aspect of the orbit can be an alternative to open biopsy in selected cases. It avoids major surgery and allows the use of radiotherapy, if required, without any delay.

[Excisional surgery of orbital tumors]. / Exzisionale Chirurgie orbitaler Tumoren.

The indications for orbital tumor surgery are an incisional biopsy to confirm the diagnosis or in malignant operable tumors a complete excision or a debulking to avoid complications in large invasively infiltrating tumors. In the case of benign tumors, the indications for surgery depend mostly on the clinical symptoms and cosmetic esthetic disfigurement. In the present article the preoperative examinations as well as surgical access approaches to different orbital regions, endoscopic procedures and methods of intraoperative navigation are presented. Magnetic resonance imaging is the instrument of choice, whereby in many cases computed tomography (CT) adds further information. Depending on the indications, diffusion-weighted sequences, CT angiography and digital subtraction angiography (DSA, catheter angiography) are added to the preoperative diagnostics. For space-occupying lesions located anterior to the bulbar equator, an anterior orbitotomy can be performed transconjunctivally or transpalpebrally. A lateral orbitotomy is used to reach lateral, laterocranial, and lateroinferior orbital segments, whereas transcranial approaches are suitable for processes located far posterior and for those with retro-orbital intracranial extension as well as for processes in the optic foramen/superior orbital fissure. The indications for an endonasal access approach are processes medial to the bulb or optic nerve and up to the orbital apex. A transantral access can be chosen for caudal, mediolateral, and medioinferior space-occupying lesions. Modern orbital surgery is complemented by endoscopic procedures and intraoperative navigation. Orbital tumors belong to the interdisciplinary relevant diseases. Therefore, an optimal management takes place at specialized multidisciplinary centers.

Lateral rectus muscle biopsy as diagnosis of unknown metastatic breast cancer. / Biopsia del músculo recto lateral orbitario como diagnóstico de cáncer de mama metastásico no conocido.

The case concerns a 58 year-old female with no medical history of interest who consulted due to binocular diplopia of one week onset. It was associated with costal pain, dyspnoea, intense asthenia and weight loss of 2 months onset. In the blood analysis in the Emergency Department it showed hypercalcaemia, renal failure, and bicytopenia. The chest x-ray showed lytic bone lesions that initially lead to multiple myeloma with extra-osseous involvement. In addition to the corresponding study, in case of horizontal diplopia, a cranial CT scan without contrast was performed where an adjacent lesion to the lateral wall of the left orbit is observed. This was of soft tissue density, and included the external rectus muscle that exerts a mass effect on the optic nerve by displacing it medially. Many lytic bone diffuse lesions with salt and pepper pattern were found in the calotte. A rectus lateral muscle and bone biopsy of the sacral wing was performed, resulting in metastasis of carcinoma compatible with mammary origin. An atypical case is presented of horizontal diplopia in the context of a patient with a severe constitutional picture with no established diagnosis, in which the biopsy of the lateral rectum was key to the confirmation diagnosis.

Orbital Metastases: When to Suspect? When to biopsy?

Incidental orbital masses that are asymptomatic and appear benign are often observed without surgical intervention unless there is a clinical or radiographic change in the mass. There is a burgeoning population of cancer patients with incidental masses that have been detected while under surveillance for metastasis. This population of patients is growing due to a number of reasons, including more extensive imaging, an aging population, and more effective cancer treatments. Closer scrutiny should be applied to these patients, due to the possibility of the mass being an orbital metastasis. In addition, the approach to these patients may have implications regarding the adult patient without a cancer history who presents with a symptomatic orbital mass. The purpose of this paper is to explore the approach to the patient with and without a cancer history who presents with an orbital mass.

Masquerading Orbital Sarcoidosis with Isolated Extraocular Muscle Involvement.

Two patients, previously diagnosed and treated for euthyroid, autoantibody-negative thyroid eye disease, presented with active orbitopathy. An atypical disease course and presentation prompted orbital biopsy. Extraocular muscle histopathology demonstrated noncaseating granulomatous inflammation consistent with presumed orbital sarcoidosis involving multiple extraocular muscles, including the inferior oblique in one of the cases. These two cases emphasize the importance of a broad differential diagnosis and the utility of an orbital biopsy in the context of an unusual disease presentation or poor treatment response. The patients' clinical course is discussed alongside important clinical signs, imaging findings, and biopsy results that support a diagnosis of isolated orbital sarcoidosis.

[Rationale for a diagnostic approach in non-Graves' orbital inflammation--Report of 61 patients]. / Établissement d'un arbre décisionnel diagnostique dans les orbitopathies inflammatoires non basedowiennes chroniques--à propos de 61 patients.

INTRODUCTION: Orbital inflammatory syndromes include a wide variety of inflammatory intraorbital processes which are very different in terms of clinical presentation and prognosis. We currently prefer to differentiate so-called "specific" inflammations, for which an etiology is able to be identified, from idiopathic orbital inflammatory syndromes (IOIS), for which the etiology remains unknown and the histology is nonspecific. PURPOSE: To propose an efficient diagnostic approach for clinicians managing patients with non-Graves' orbital inflammations. MATERIALS AND METHODS: This is a retrospective and prospective study concerning 61 patients managed by the medical team for non-Graves' orbital inflammations between May, 1999 and May, 2013 in the ophthalmology departments of Nice and Limoges university hospitals in France. Seventeen specific inflammations, 19 orbital lymphomas and 25 idiopathic orbital inflammatory syndromes were included. Patients were divided into two groups. Thirty-six patients (group 1) underwent primary biopsy, while for the other 25 (group 2), therapy was begun empirically without biopsy. We could therefore compare both approaches in terms of diagnostic efficiency and time until identification of a specific etiology. RESULTS: Our statistical results show that an approach without primary biopsy leads to a number of specific diagnoses statistically much lower than that obtained by the approach with primary biopsy. Also, the risk of missing a specific inflammation (with as a consequence an inappropriate treatment and a risk of functional sequelae as well as a fatal risk of missing a lymphoproliferative pathology) is very clearly higher in the case of not performing primary biopsy. Finally, the average time elapsed between the initial consultation with the ophthalmologist and a specific diagnosis was one month in the case of the first approach, while this delay was almost three times higher with the second approach, with a mean of 2.91 months (P<0.01). DISCUSSION: Our study shows that biopsy should be the mainstay of diagnostic management. A trial of empiric treatment is only performed first in myositis or in locations where biopsy could jeopardize functional prognosis. It should only be done after biopsy in all other cases. Of course, in all cases of relapse or recurrence after treatment, biopsy should be performed or repeated. CONCLUSION: The diagnostic work-up of a patient with an orbital inflammatory process must of course include blood testing and orbital imaging, but also a systematic primary biopsy for histological examination in the vast majority of cases. It must be repeated at least in the case of any doubt about the diagnosis or in the case of any recurrence or resistance to treatment.