Cellular angiofibroma of the orbit

Cellular angiofibroma is a benign mesenchymal tumor most commonly located in the distal genital tract of both men and women. Although extragenital locations have been reported rarely, this is the first report of cellular angiofibroma of the orbit. A 58-year-old man presented with a mass in the left superomedial orbit since 2 years. Magnetic resonance imaging showed a well-demarcated lesion with a homogeneous intermediate signal intensity on both T1- and T2-weighted images, homogeneous contrast enhancement and high signal intensity on diffusion-weighted images. Complete excision was performed through a medial upper eyelid crease incision. Histopathology showed a vascular CD34-positive and STAT6-negative spindle cell tumor with monoallelic loss of FOXO1, indicating cellular angiofibroma

Accelerated growth of orbital schwannomas during pregnancy does not correlate with sex hormone- or growth factor receptor status

Purpose: Until now, three cases of growth of an orbital schwannoma during pregnancy have been published. We aim to provide additional insight in the effect of pregnancy on orbital schwannomas. Methods: We present two additional cases of accelerated growth of orbital schwannomas during pregnancy and investigate receptor expression profiles for estrogen, progesterone, androgen, VEGF, EGF, FGF, PDGF-Rβ and ki-67 in the two pregnant cases and six non-pregnant cases. Results: Case 1: A 26-year-old woman developed unilateral exophthalmos during pregnancy, with normal visual acuity and ocular motility. During a subsequent pregnancy, again the exophthalmos progressed. MRI showed a mass suggestive of schwannoma. After delivery, resection of the lesion was performed through an anterior approach. Pathology confirmed schwannoma. The expression profile was positive for estrogen- and FG

Post-operative Refractive Prediction Error After Phacovitrectomy: A Retrospective Study

Introduction Many authors have reported on a myopic post-operative refractive prediction error when combining phacoemulsification with pars plana vitrectomy (phacovitrectomy). In this study we evaluate the amount of this error in our facility and try to elucidate the various factors involved. Methods This was a retrospective study which included 140 patients who underwent phacovitrectomy (39 with macular holes, 88 with puckers, and 13 with floaters). Post-operative refractive error was defined as the difference between the actual spherical equivalent (SEQ) and expected SEQ based on the SRK/T and Holladay-II formulas. Both univariate (paired t test, independent t test, one-way analysis of variance, or Mann–Whitney test) and multivariate (regression analysis) statistical analyses were performed. Results Overall, a refractive error of − 0.13 dpt (p = 0.033) and − 0.26 dpt (p < 0.01) were found in the SRK/T and Holladay-II formulas, respectively. For the independent diagnoses, only macular holes showed a myopic error with the SRK/T (− 0.31 dpt; p < 0.01) and Holladay-II (− 0.44 dpt; p < 0.01) formulas. In univariate analysis, significant factors involved in myopic refractive error were macular hole as diagnosis (p < 0.01 for SRK/T and Holladay-II), gas tamponade (SRK/T p = 0.024; Holladay-II p = 0.025), pre-operative myopia (p < 0.01 for SRK/T), and optical technique for axial length measurement (SRK/T and Holladay-II p < 0.01). In the multivariate analysis, pre-operative axial length (p = 0.026), optical technique for axial length measurement (p < 0.01), and pre-operative SEQ (p < 0.01) were independent predictors for myopic refractive error in the SRK/T formula. For the Holladay-II formula, optical technique for axial length measurement (p < 0.01) and pre-operative SEQ (p = 0.04) were predictive. Conclusion Various factors are involved in determining the myopic refractive error after phacovitrectomy. Not every factor seems to be as important in each individual patient, suggesting a more tailored approach is warranted to overcome this problem

Adult orbital xanthogranuloma: long-term follow-up of treated cases

Background: Adult orbital xanthogranulomatous disease (AOXGD) is a group of rare disorders. Four subtypes are identified: adult-onset xanthogranuloma (AOX), adult-onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim-Chester disease (ECD). Therapy options vary and little is known about the long-term effect of the treatment. In this study, we will describe the clinical behaviour, effect of treatment, and long-term outcome in a consecutive series of patients with AOXGD. Methods: This is a descriptive, retrospective study with a long follow-up term of 21 patients with histologically proven AOXGD, treated between 1989 and 2021 in the Rotterdam Eye Hospital and Erasmus MC University Medical Center. Results: Twenty-one patients with histologically proven AOXGD were included. The follow-up ranged from 2-260 months (median of 67 months). Six of the nine patients with AOX were treated with surgery alone, with recurrence in two. Three received systemic therapy, with recurrence in one. All four patients with AAPOX received systemic treatment, the disease recurred in two. Two patients with NBX were treated with surgery alone, with recurrence in one. Four required additional therapy with recurrence in two. Both patients with ECD required systemic therapy. Conclusions: Recognition of AOXGD is important, in particular, because of the potential severe systemic locations in the different subtypes. Surgical excision might be a sufficient therapy for patients with AOX. Patients with AAPOX, NBX, and ECD warrant systemic therapy. Currently, there is no conclusive evidence for a superior treatment strategy, but further studies are necessary to investigate treatment options.MTG4Molecular tumour pathology - and tumour genetic

Intracranial actinomycosis of odontogenic origin masquerading as auto-immune orbital myositis: a fatal case and review of the literature

Background: Actinomycetes can rarely cause intracranial infection and may cause a variety of complications. We describe a fatal case of intracranial and intra-orbital actinomycosis of odontogenic origin with a unique presentation and route of dissemination. Also, we provide a review of the current literature. Case presentation: A 58-year-old man presented with diplopia and progressive pain behind his left eye. Six weeks earlier he had undergone a dental extraction, followed by clindamycin treatment for a presumed maxillary infection. The diplopia responded to steroids but recurred after cessation. The diplopia was thought to result from myositis of the left medial rectus muscle, possibly related to a defect in the lamina papyracea. During exploration there was no abnormal tissue for biopsy. The medial wall was reconstructed and the myositis responded again to steroids. Within weeks a myositis on the right side occurred, with CT evidence of muscle swelling. Several months later he presented with right hemiparesis and dysarthria. Despite treatment the patient deteriorated, developed extensive intracranial hemorrhage, and died. Autopsy showed bacterial aggregates suggestive of actinomycotic meningoencephalitis with septic thromboembolism. Retrospectively, imaging studies showed abnormalities in the left infratemporal fossa and skull base and bilateral cavernous sinus. Conclusions: In conclusion, intracranial actinomycosis is difficult to diagnose, with potentially fatal outcome. An accurate diagnosis can often only be established by means of histology and biopsy should be performed whenever feasible. This is the first report of actinomycotic orbital involvement of odontogenic origin, presenting initially as bilateral orbital myositis rather than as orbital abscess. Infection from the upper left jaw extended to the left infratemporal fossa, skull base and meninges and subsequently to the cavernous sinus and the orbits