Primary orbital apocrine adenocarcinoma: case report and literature review.

Primary apocrine adenocarcinoma (PAA) originating from the orbit is a rare malignant neoplasm. We present the case of a 61-year-old-male with PAA of the orbit. The patient underwent a right orbital exenteration, neck dissection, and adjuvant radiotherapy. Orbital exenteration is commonly performed as the primary intervention for PAA of the orbit. The role of adjuvant radiotherapy to prevent local recurrence is unclear and may be determined on a case-by-case basis.

Asymmetric proptosis in thyroid eye disease.

PURPOSE: Patients with thyroid eye disease (TED) can present with asymmetric disease. The purpose of this study was to evaluate the prevalence of asymmetric TED in an Australian cohort and investigate clinical, biochemical, and radiological associations of asymmetric TED. METHODS: This was a retrospective study of patients with TED who underwent Hertel exophthalmometry and orbital computed tomography (CT) scans. Asymmetry was defined as a difference in the globe protrusion of ≥ 3 mm using Hertel exophthalmometry. Data was collected on patient demographics, clinical disease presentation, thyroid function tests and antibody levels. Muscles volumes were determined by manually segmenting the extraocular muscles on CT scans using a commercially available software. RESULTS: 172 orbits from 86 patients were included in the study. 28 (33%) patients had asymmetric TED. No significant differences were observed in age, gender, thyroid hormone status, anti-thyroid peroxidase levels, thyroid stimulating hormone receptor antibodies, disease activity status, or dysthyroid optic neuropathy between the asymmetric and non-asymmetric groups. The extraocular muscle volumes were significantly higher in the asymmetric orbit compared to its contralateral orbit. CONCLUSION: Asymmetric TED is a reasonably common occurrence in the course of TED. It is associated with higher extraocular muscle volumes, suggesting muscle enlargement as one of the underlying contributors to asymmetric proptosis. Thyroid eye disease should be considered in the differential of asymmetric orbital inflammatory disease.

Radiological analysis of orbital lymphoma histological subtypes.

PURPOSE: To characterise the radiological features of orbital lymphoma subtypes. METHODS: This was a multicentre, retrospective study to analyse radiological and clinical characteristics of orbital lymphoma by histological subtype across two sites within Australia and the United States. RESULTS: A total of eighty-eight patients were included. The most common subtypes were extranodal marginal zone lymphoma [EMZL] (48, 54.5%), follicular lymphoma [FL] (16, 18.2%), and diffuse large B-cell lymphoma [DLBCL] (15, 17.0%). Clinically, significant associations were found between DLBCL and vision change (p < .01), pain (p < .01), extraocular movement limitation (p = .01), and optic neuropathy (p = .01). Radiologically, there was no significant difference between the individual histopathological sub-groups with respect to bone destruction (p = .30), optic nerve involvement (p = .30) and diffuse appearance (p = .84). However, if categorised as either aggressive or indolent, aggressive lymphoma subtypes were significantly more likely to demonstrate globe indentation radiologically than indolent subtypes (Odds Ratio = 5.5, 95% CI: 1.3, 23.3; p = .02). CONCLUSIONS: DLBCL was significantly associated with vision change, pain, extraocular movement limitation and optic neuropathy clinically. Aggressive lymphoma subtypes were significantly more likely to demonstrate globe indentation radiologically. Otherwise, there were no significant differences between lymphoma subtypes and radiological findings on MRI and CT.

Dilated superior ophthalmic vein: systemic associations.

PURPOSE: To review systemic associations of patients with dilated superior ophthalmic veins (SOV) in the absence of orbital, cavernous sinus, or neurological disease. METHODS: Retrospective review of patients who had dilated SOVs with a diameter of ≥ 5.0 mm. Patients with a dilated SOV secondary to orbital, cavernous sinus or neurological disease were excluded. Patient demographics, past medical history, and SOV diameters on initial and follow up scans were collected. The maximum diameter of the SOV was taken perpendicular to the long axis of the SOV. RESULTS: Nine cases were identified. Patients ranged in age from 58 to 89 years and six out of nine were female. The dilated SOV involved both eyes in two cases, left eye in five cases and right eye in two cases. Three patients had dilated SOV likely secondary to raised venous pressures from decompensated right heart failure (n = 1), pericardial effusion (n = 1) and left ventricle dysfunction secondary to a myocardial infarction (n = 1). Five patients had a significant history of previous ischaemic heart or peripheral vascular disease. Two patients had risk factors for venous clotting disease whilst one patient had a history of giant cell arteritis and vertebral artery dissection. CONCLUSION: A dilated SOV may raise concern for life threatening conditions such as a carotid cavernous fistula and may prompt additional investigations. A dilated SOV may be reversible and secondary to raised venous pressures due to cardiac failure. Other cases may be seen in patients with significant cardiovascular risk factors, possibly due to changes in vasculature.

Paediatric fronto-orbital osteoblastoma: case report.

Osteoblastoma is a rare benign bone tumour. It represents 1% of all primary bone tumours. Common locations are the vertebral column and long bones of the extremities. Although well documented in craniofacial locations, only occasional case reports describe orbital involvement. The authors present a case of a fronto-orbital osteoblastoma which posed a significant diagnostic dilemma. Despite early local recurrence, the molecular findings were supportive of a benign lesion.

Granuloma Faciale Involving the Tarsoconjunctival Surface of the Eyelid.

We present the case of a Caucasian woman with a histology-confirmed granuloma faciale of the tarsoconjunctival surface of the eyelid. A 66-year-old female patient presented with a 1-week history of painless left upper eyelid mass without history of allergy, trauma, or ocular surgery. There was an elevated well-demarcated flesh-colored lesion within the tarsus. Diagnosis was confirmed by history of wedge biopsy, which demonstrated a mixed inflammatory cell infiltrate on a background of fibrosis with subtle focal leucocytoclasis. The lesion was fully excised without evidence of recurrence at follow up. Granuloma faciale is a rare disease often misdiagnosed clinically. Our case report is the first-documented granuloma faciale on the tarsoconjunctival surface of the eyelid and could suggest alternative pathogenesis in granuloma faciale development. Awareness of granuloma faciale as a nonmalignant for differential for tarsal lesions is important for treating clinicians.

A standardized method for endoscopic necrosectomy improves complication and mortality rates.

OBJECTIVES: Endoscopic necrosectomy is effective in the treatment of walled-off necrosis (WON), and is preferred to surgical approaches, however complication and mortality rates remain high with few centers regularly employing the technique. Lack of a standardized approach may also contribute to these limitations. METHODS: Prior to the study, a multidisciplinary team applied standardized care assessment and management plan principles to develop and optimize a systematic approach for the management of WON. Preoperative, postoperative, and endoscopic management were standardized. Patient preparation, room set-up, technical features (EUS-guidance, cold-access with balloon dilation, fragmentation of necrosis on the initial procedure, antibiotic lavage, double pigtail stents), and discontinuation of PPIs to encourage auto-digestion of necrosis were included. This study employed a consecutive prospective clinical registry to assess the clinical outcomes of this standardized approach. RESULTS: 60 consecutive patients underwent 1.58 ± 0.1 necrosectomies, with debridement accomplished on the initial procedure in 98.3%. 39 patients (65%) required only one session. Clinical resolution occurred in 86.7%, with radiologic confirmation. Percutaneous drainage was required in 8 patients during follow-up, and 4 of these later required surgery. Serious adverse events occurred in 3.3% of patients, and there was no mortality. CONCLUSIONS: The standardized technique employed in this series was associated with lower rates of adverse events, morbidity, and mortality than prior large series. Use of a systematic approach, and integrating elements of this method may improve the risk profile of endoscopic necrosectomy and allow broader adoption.

Endoscopic suturing for transoral outlet reduction increases weight loss after Roux-en-Y gastric bypass surgery.

BACKGROUND & AIMS: Weight regain or insufficient loss after Roux-en-Y gastric bypass (RYGB) is common. This is partially attributable to dilatation of the gastrojejunostomy (GJ), which diminishes the restrictive capacity of RYGB. Endoluminal interventions for GJ reduction are being explored as alternatives to revision surgery. We performed a randomized, blinded, sham-controlled trial to evaluate weight loss after sutured transoral outlet reduction (TORe). METHODS: Patients with weight regain or inadequate loss after RYGB and GJ diameter greater than 2 cm were assigned randomly to groups that underwent TORe (n = 50) or a sham procedure (controls, n = 27). Intraoperative performance, safety, weight loss, and clinical outcomes were assessed. RESULTS: Subjects who received TORe had a significantly greater mean percentage weight loss from baseline (3.5%; 95% confidence interval, 1.8%-5.3%) than controls (0.4%; 95% confidence interval, 2.3% weight gain to 3.0% weight loss) (P = .021), using a last observation carried forward intent-to-treat analysis. As-treated analysis also showed greater mean percentage weight loss in the TORe group than controls (3.9% and 0.2%, respectively; P = .014). Weight loss or stabilization was achieved in 96% subjects receiving TORe and 78% of controls (P = .019). The TORe group had reduced systolic and diastolic blood pressure (P < .001) and a trend toward improved metabolic indices. In addition, 85% of the TORe group reported compliance with the healthy lifestyle eating program, compared with 53.8% of controls; 83% of TORe subjects said they would undergo the procedure again, and 78% said they would recommend the procedure to a friend. The groups had similar frequencies of adverse events. CONCLUSIONS: A multicenter randomized trial provides Level I evidence that TORe reduces weight regain after RYGB. These results were achieved using a superficial suction-based device; greater levels of weight loss could be achieved with newer, full-thickness suturing devices. TORe is one approach to avoid weight regain; a longitudinal multidisciplinary approach with dietary counseling and behavioral changes are required for long-term results. ClinicalTrials.gov identifier: NCT00394212.

Optic nerve sheath infiltration in dysthyroid optic neuropathy.

PURPOSE: To assess the association of optic nerve sheath (ONS) infiltration, fat infiltration, and scleral enhancement with active thyroid eye disease (TED) and dysthyroid optic neuropathy (DON). METHODS: Thyroid eye disease patients who had axial and coronal fat-suppressed contrast enhanced T1-weighted magnetic resonance imaging (MRI) imaging performed were included. Optic nerve sheath infiltration was defined by the presence of thickening and circumferential enhancement of the optic nerve sheath. Clinical assessments were performed by orbital surgeons or neuro-ophthalmologists and the disease activity (active/inactive) and presence or absence of dysthyroid optic neuropathy were recorded. RESULTS: The study population consisted of 76 orbits from 38 patients with a mean age of 53 ± 15 years, with 25 (66%) being female. Optic nerve sheath infiltration was present in 28 (37%) orbits, fat infiltration in 37 (49%) and scleral enhancement in 14 (18%) orbits. ONS infiltration (OR 19.8, p < 0.01), fat infiltration (OR 5.2, p < 0.01) and scleral enhancement (OR 12.2, p = 0.01) were all significantly associated with active clinical disease. Patients with ONS infiltration had a significantly higher odds of dysthyroid optic neuropathy (OR 3.4, p < 0.05). Fat infiltration (OR 2.8, p = 0.1) and scleral enhancement (OR 2.3, p = 0.23) were not significantly associated with DON. CONCLUSIONS: Optic nerve sheath infiltration may be a predictor of dysthyroid optic neuropathy. Intraorbital fat infiltration and scleral enhancement may be used to detect active TED. These radiological findings may serve as useful diagnostic and stratification tools in evaluating TED patients.

Extraocular muscle enlargement in dysthyroid optic neuropathy.

OBJECTIVE: To investigate extraocular muscle volumes in thyroid eye disease (TED) patients with and without dysthyroid optic neuropathy (DON). DESIGN: Retrospective cohort study. PARTICIPANTS: TED patients who had computed tomography of the orbits. METHODS: The extraocular muscles were manually segmented in consecutive axial and coronal slices, and the volume was calculated by summing the areas in each slice and multiplying by the slice thickness. Data were collected on patient demographics, disease presentation, thyroid function tests, and antibody levels. RESULTS: Imaging from 200 orbits was evaluated. The medial rectus, lateral rectus, superior muscle group, inferior rectus, and superior oblique volumes were significantly greater in orbits with DON compared with TED orbits without DON (p < 0.01 for all). There was no significant difference in the inferior oblique muscle volume (p = 0.19). Increase in volume of the superior oblique muscle showed the highest odds for DON. Each 100 mm3 increase in superior oblique, lateral rectus, inferior rectus, medial rectus, and superior muscle group volume was associated with 1.58, 1.25, 1.20, 1.16, and 1.14 times increased odds of DON. CONCLUSION: All extraocular muscle volumes except for the inferior oblique were significantly greater in DON patients. Superior oblique enlargement was associated with the highest odds of DON, suggesting superior oblique enlargement to be a novel marker of DON.

Transorbital drillout to the cavernous sinus: an approach for squamous cell carcinoma with perineural spread. Illustrative cases.

BACKGROUND: This study describes a transorbital apical approach to the cavernous sinus, where the greater wing of sphenoid (GWS) and superior orbital fissure (SOF) are drilled out to access the interdural incision zone and lateral wall of the cavernous sinus. OBSERVATIONS: This was a retrospective series of 3 patients with periocular squamous cell carcinoma (SCC) and radiological evidence of perineural spread to the cavernous sinus. Following an orbital exenteration, the GWS was drilled to reach the lateral border of the SOF. The meningo-orbital band, a periosteal transition between the frontotemporal basal dura and periorbita, was incised to enter the lateral wall of the cavernous sinus. The relevant cranial nerves were biopsied to provide an accurate zonal classification of disease. LESSONS: The transorbital apical approach via the SOF provides a corridor of access to the cranial nerves within the lateral wall of the cavernous sinus. This technique was successfully performed on 3 patients with periocular SCC. One case had radiological evidence of intracavernous oculomotor nerve involvement, 1 patient demonstrated nasociliary nerve enlargement at the SOF, and another had frontal nerve involvement extending into the cavernous sinus. Cerebrospinal fluid leak occurred in 1 case addressed with fat packing and fascial closure.

The use of the paramedian forehead flap alone or in combination with other techniques in the reconstruction of periocular defects and orbital exenterations.

PURPOSE: The paramedian forehead flap (PMFF) is a reconstructive option for large eyelid defects and orbital exenterations. We report a series of cases where PMFF reconstruction was carried out at various institutions in Australia. METHODS: This study was a multi-centre, retrospective, non-comparative case series investigating the clinical outcomes of the PMFF for reconstructing periocular defects and orbital exenterations. RESULTS: This case series describes twenty-seven patients (Female = 15, Male = 12), operated between 1991 to 2019, with a median age of 81 years (range: 45-93 years). Defect locations involved combinations of the medial canthus (16/27, 59.3%), upper eyelids (7/27, 25.9%), lower eyelid (4/27, 14.8%), both upper and lower eyelids (5/27, 18.5%), and orbital (7/27, 25.9%). There were no cases of flap necrosis. Minor post-operative complications were observed in ten patients with the most common being lagophthalmos. Median duration of follow-up was 17months (Range: 2months- 23years). CONCLUSIONS: The PMFF is a versatile reconstructive tool for a range of periocular defects and orbital exenterations with minor post-operative complications.

Eosinophilic Vasculitis and Arteritic Anterior Ischemic Optic Neuropathy Associated With Anti-PD-L1 Therapy.

Immune checkpoint inhibitor therapy is frequently associated with immune-related adverse events, which occasionally manifest with visual symptoms. Here, we describe a case of unilateral and sudden-onset painless vision loss in an 82-year-old man with metastatic non-small cell lung cancer receiving immunotherapy with the anti-programmed death-ligand 1 agent atezolizumab. Examination demonstrated a right-sided relative afferent pupillary defect, diffusely swollen optic disc, and delayed choroidal and retinal arterial filling on fundus fluorescein angiography, consistent with an arteritic anterior ischemic optic neuropathy. Histology of an ipsilateral temporal artery biopsy revealed a transmural eosinophilic infiltrate without granulomas, while serology revealed the presence of antineutrophil cytoplasmic antibodies. Peripheral eosinophilia was also noted, which preceded treatment by several months. This report highlights the importance of clinician awareness of immune checkpoint inhibitors and their systemic and ophthalmic complications, which rarely appear to extend to eosinophilic temporal arteritis.

Caecal Epiploic Appendagitis Masquerading Clinically as an Acute Appendicitis: A Case Report and Brief Literature Review.

A 46-year-old female presented to our emergency department (ED) with a 2-day history of right lower abdominal pain which was associated with nausea and anorexia. Abdominal examination revealed tenderness in the right iliac fossa (RIF) with rebound tenderness and a localized guarding. Urine dipstick was normal, and the pregnancy test was negative. Her laboratory investigations were significant only for a CRP of 16.6. A presumptive clinical diagnosis of acute appendicitis was suggested based on the given history and relevant physical signs. However, an abdominal computed tomography (CT) scan revealed an epiploic appendagitis of the caecum with a normal-looking appendix. She was managed conservatively and responded well and was discharged after 2 days in good health. Though being a relatively rare case of acute localized right-sided lower abdominal pain, caecal epiploic appendagitis should be considered as one of the differential diagnoses with the final diagnosis reached usually by the radiological findings due to the nonspecific nature of clinical and laboratory features.

Improved detection and characterization of adrenal disease with PET-CT.

Positron emission tomography (PET)-computed tomography (CT) combines complementary modalities, thereby providing useful structural and functional information for the detection and characterization of a variety of conditions affecting the adrenal gland. The coregistered information provided by PET-CT is often superior to that provided by CT or PET owing to a variety of pitfalls inherent in the use of either modality alone. In addition, PET-CT can prove invaluable in the differentiation between benign and malignant adrenal disease. However, this combined modality also has certain limitations. Benign entities such as lipid-poor adenomas may demonstrate increased uptake at 2-[fluorine 18]fluoro-2-deoxy-d-glucose PET while being indeterminate at standard CT. Moreover, the combined information from PET-CT will not always obviate additional studies or biopsy. Nevertheless, radiologists and nuclear physicians should be familiar with the common as well as the atypical manifestations of adrenal disease at PET and CT. They should also be meticulous in the performance and interpretation of PET-CT, which is crucial for optimal diagnosis and treatment.

Adrenocortical carcinoma: contrast washout characteristics on CT.

OBJECTIVE: The purpose of this study was to characterize pathologically proven adrenocortical carcinoma by examination of washout attenuation characteristics on contrast-enhanced CT images. CONCLUSION: Adrenocortical carcinoma has relative contrast retention on delayed contrast-enhanced CT. All tumors in this series had a relative percentage washout less than 40%, a finding consistent with malignant disease.

Pearls and pitfalls in interpretation of abdominal and pelvic PET-CT.

The interpretation of images obtained in the abdomen and pelvis can be challenging, and the coregistration of positron emission tomographic (PET) and computed tomographic (CT) scans may be especially valuable in the evaluation of these anatomic areas. PET-CT represents a major technologic advance, consisting of generally complementary modalities whose combined strength tends to overcome their respective weaknesses. However, this combined functional-structural imaging approach raises a number of controversial questions and presents some unique interpretative challenges. Accurate PET-CT scan interpretation requires awareness of the various pitfalls associated with the imaging components, both individually and in combination. The results of recent PET-CT studies have been very encouraging, but larger prospective studies will be needed to establish optimal hybrid scanning protocols. Applying sound imaging principles, paying attention to detail, and staying abreast of advances in this exciting new modality are necessary for harnessing the full diagnostic power of abdominopelvic PET-CT.

Corynebacterium propinquum Keratitis Identified Using MALDI-TOF.

PURPOSE: To report a corneal infection due to Corynebacterium propinquum identified using the matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF) method. METHODS: A 94-year-old woman presented with suppurative keratitis. Her ocular history included Fuchs corneal dystrophy, corneal transplantation, and glaucoma. A gram-stained smear revealed coryneforms. Colonies on bacterial culture media were assayed by MALDI-TOF. RESULTS: Identification of C. propinquum was confirmed by RNA polymerase ß subunit (rpoB) gene sequencing. The patient was treated with topical cefazolin, gentamicin, and fluorometholone, and her vision improved to 20/160 unaided when last seen. CONCLUSIONS: MALDI-TOF can correctly identify isolates that are both uncommon and difficult to distinguish from related species. The ease and low running costs of this method will improve the laboratory diagnosis of ocular infections.