Presentation of Spontaneous Orbital Hemorrhage.

AIMS: To evaluate the presenting features of patients with nontraumatic orbital hemorrhage (NTOH) based on etiopathological factors. METHODS: A retrospective case-note review for demographics and presenting features of patients with nontraumatic orbital hemorrhage. Patients were categorized into 3 groups: group I with known systemic vascular disease, group II with known or newly revealed orbital vascular anomalies, and group III with no known underlying vasculopathy. RESULTS: One hundred and seventeen patients (68 female; 58%), with 37 (32%) in group I, 47 (40%) in group II, and 33 (28%) in group III. The average age at presentation was 70.9, 30.1, and 49.9 years, respectively, but the incidence peaked in the first decade for patients with underlying local vascular anomalies and in the eighth for those without. Of the group I patients with known cardiovascular disease, 43% were on antithrombotic agents. The most common presenting symptoms were orbital pain (59%), proptosis (56%), and diplopia (45%), while 13% had associated nausea or vomiting. Ipsilateral optic neuropathy occurred in 14% of cases, higher in group II (22%), along with nonaxial globe displacement (25%), reduced eye movements (47%), optic disc swelling (10%), and choroidal folds (9%). Imaging in patients without evident vascular anomalies showed that hemorrhages commonly occurred in the inferotemporal quadrant (32%), with about half of these having a "beached whale" configuration (46%). CONCLUSION: Nontraumatic orbital hemorrhages affect all ages, typically presenting with acute proptosis or pain (over half), various degrees of visual impairment, and reduced motility. About half of those without vascular anomalies had cardiovascular risk factors, imaging often revealing an inferotemporal mass with a "beached whale" appearance.

One-stage Intraconal Volume Modification for Exposed and Nonexposed Ball Implants.

PURPOSE: To assess long-term outcomes and reinterventions for exposed and nonexposed intraconal orbital implants after single-stage socket revisional surgery. METHODS: Retrospective case-note review of patients with exposed or nonexposed orbital ball implants, who underwent a single-stage ball exchange (Group I) or ball removal with dermis-fat graft (Group II); Group I was subdivided as having either exposed (A) or nonexposed (B) implants. Outcomes were compared with similar cohorts undergoing multistage surgery. RESULTS: Of 172 patients (54% male), 153 (89%) underwent ball exchange (Group I) and 19 (11%) had dermis-fat graft (Group II). With a mean follow up of 7.8 years, Group I patients without ball exposure (Group IB) had a slightly higher rate of reintervention for persistent volume deficit (21% vs. 9%; p = 0.163) and implant migration (6% vs. 0%; p = 0.091), while Group IA patients-having surgery for exposed implants-had more reexposures (7% vs. 3%; p = 0.270). Single-stage and 2-stage surgery had similar reintervention rates, with the exception of a trend toward fewer volume enhancements for those with nonexposed implants (17% vs. 30%; p = 0.380), and a shorter time to (re)exposure for exposed implants after 2-stage procedures (mean 0.33 vs. 3.5 years; p = 0.571). Group II patients required further volume enhancement in 32% of 1-stage procedures, and one patient needed graft removal. CONCLUSION: Single-stage volume modification is effective and the results are comparable to staged surgery for either exposed or nonexposed implants. Most patients report a stable prosthesis and satisfactory cosmesis, after a faster rehabilitation.

Long-Term Outcome for Primary Papillomas of the Lacrimal Drainage System.

AIMS: To report long-term outcomes after surgery for primary benign papillomas of the lacrimal drainage system. METHODS: Retrospective review of patients with primary lacrimal drainage system papillomas, first seen between 1984 and 2021, but excluding lesions with sino-nasal origin or malignant histology. Patients were categorized by tumor histology (squamous, transitional, and mixed cell) and growth pattern (exophytic, endophytic, or mixed). RESULTS: Thirty patients (18 female; 60%) presented at a mean age of 46.1 years (12-79 years). Over an average follow-up of 10.4 years, 30% of patients experienced recurrence, with rates differing slightly by growth pattern: 25% for exophytic tumors and 40% for endophytic and mixed-pattern tumors (p = 0.431). The overall 5-year recurrence-free survival was 67% (± 9% standard error), this being slightly higher for exophytic lesions (75% ± 10%) as compared to 48% (± 19%) for tumors with an endophytic component (p = 0.370). No cases of malignant transformation were observed, and the tumor type at recurrence matched the initial diagnosis. CONCLUSION: Primary benign papillomas of the lacrimal drainage system have a long-term recurrence rate of about one-third, with a higher frequency in those exhibiting an endophytic growth pattern. The study highlights the importance of prolonged surveillance for recurrence, especially for endophytic and mixed growth pattern tumors, and for the potential to undergo malignant transformation. The results support the effectiveness of endoscopic surgical excision or laser thermos-ablation in managing recurrent lacrimal drainage system papillomas after dacryocystorhinostomy.

Repeated Lacrimal Gland Biopsies.

INTRODUCTION: To examine the indications for repeated lacrimal gland biopsies, and the rate of detection of a new diagnosis. METHODS: A single-center, retrospective review of patients who underwent more than 1 lacrimal gland biopsy, either ipsilateral or contralateral, between 2000 and 2022. RESULTS: One hundred and twenty-three patients (80 female; 65%) had repeated lacrimal gland biopsy. The commonest diagnosis on initial biopsy was chronic nonspecific dacryoadenitis (NSD) (49/123; 40%). Indications for repeated biopsy were uncertainty in making a histopathological diagnosis (16/123; 13%), poorly-responsive or recurrent ipsilateral disease (61/123; 50%), new or continued/worsening contralateral disease (30 patients; 24%), and planned tumor resection after initial biopsy (16/123; 13%). Of the 40 patients (33%) with a different histopathological diagnosis after repeated lacrimal biopsy, 4 (10%) had lymphoma, initially reported as NSD (4/49 with NSD; 8%), and 7/40 (18%) (14% of the 49 NSD patients) were reclassified as having specific inflammations (including 2 with granulomatous polyangiitis); of the 7 having reclassification as a specific dacryoadenitis, 6/7 had ipsilateral disease failing to respond to primary treatment, and 1/7 had new onset or progression of contralateral disease. All histology after the primary biopsy of 16 patients with lacrimal gland malignancies retained the same tissue diagnosis. CONCLUSION: Repeated biopsy for lacrimal gland disease in this study revealed a diagnosis of malignancy in 20%, including lymphoma in 8% of those initially diagnosed with NSD. There was a 14% rate of diagnostic progression from "non-specific" dacryoadenitis to a more specific inflammatory disease.

Orbital infarction during drug abuse: tissues of the globe do not appear to recover function.

AIM: To describe the features of an orbital infarction syndrome arising after prolonged orbital pressure during drug-induced stupor in young people. PATIENTS AND METHODS: The clinical presentation and course for drug-induced orbital infarction is described, based on a retrospective review of clinical notes and imaging. RESULTS: Two cases of orbital infarction syndrome, due to prolonged orbital compression caused by sleeping with pressure on the orbit during drug-induced stupor, are described. Both patients presented with very poor vision and mydriasis, marked periorbital swelling with some pain, and complete external ophthalmoplegia. Whilst the orbital changes and eye movements recovered, the affected eyes had persistent wide mydriasis, and remained blind with marked optic atrophy. CONCLUSION: With a mechanism analogous to prolonged orbital pressure due to improper head positioning during neurosurgical procedures, drug-users appear to risk developing an orbital infarction syndrome if they rest with prolonged pressure on the orbit during a drug-induced stupor.

Long-Term Outcome of Staged Socket Surgery for Acquired Anophthalmos.

PURPOSE: To evaluate long-term outcomes of staged volume rehabilitation for acquired anophthalmos. METHODS: Case-note review of patients who had preceding i) eye removal without implant, ii) eye removal with an intraconal implant, but ball-related problems, or iii) removal of exposed implant. Secondary interventions were a) a first-time ball implant, b) dermis-fat graft, c) ball repositioning, d) ball replacement after prior removal, or e) orbital floor implantation. RESULTS: Secondary volume-augmenting surgery was performed in 175 sockets at a mean age of 42.7 years (range 2-91), with 62% secondary ball implants, 3% dermis-fat grafts, 6% ball repositioning, 19% ball replacement after prior removal for exposure, and 10% having orbital floor implantation. After this surgery, further volume enhancement was required in 21% of sockets, this being 40% for spheres ≤18 mm diameter, in contrast to 6% for those ≥20 mm ( p < 0.001). Exposure or malposition of the secondary implant occurred in 8% (12/151) and was unrelated to implant type, size, wrapping, or prior irradiation. Tertiary surgery addressed lining deficiency (18%) or eyelid malposition (25%). Overall, 92/175 (53%) had tertiary surgery to improve cosmesis and comfort, with 49% (36/92) being related to small implants. At a mean follow-up of 9.1 years, 82% of sockets had adequate volume, 79% had excellent lining, and 93% were comfortable. Prosthetic fit was satisfactory in 96% of cases, and 97% reported improved cosmesis. CONCLUSION: Over half of the sockets having planned 2-stage volume enhancement may need further procedures, especially after small-volume secondary implants, but, with meticulous surgery, reasonable long-term results can be achieved with few complications.

Horizontal eyelid shortening alone versus combined procedures for the correction of involutional lower eyelid entropion.

BACKGROUND: Conventionally, management of involutional entropion involves correcting both vertical and horizontal laxity, however the optimal surgical approach is debated. OBJECTIVE: To compare the recurrence rate of horizontal lower eyelid shortening alone with combined surgical approaches for correction of involutional lower eyelid entropion. METHODS AND MATERIAL: A retrospective, comparative, consecutive case series of patients undergoing surgery for involutional lower eyelid entropion with both horizontal and vertical laxity. Patients were categorized based on the procedure as: Group 1, horizontal shortening alone, Group 2, horizontal shortening with everting sutures), and Group 3, horizontal shortening with retractor plication. RESULTS: Of the 249 procedures (31 bilateral) performed on 218 patients, 54 (22%) involved horizontal eyelid shortening alone (Group 1), while 80 (32%) had this combined with everting sutures (Group 2), and 115 (46%) with retractor plication (Group 3). The anatomical success rates for Groups 1, 2, and 3 were 93, 94, and 90% (P = 0.69), respectively. Similarly, reported symptom improvements were 94, 93, and 93% for these groups (P = 0.91). After an average follow-up of 15.7 months (median 10; range 6-81), Group 1 (eyelid shortening alone) had a 7% (4/54) recurrence, compared to 8% (16/195) in Groups 2 and 3 (combined procedures) (P = 1.0). Complications were minor, with no significant difference between groups. CONCLUSION: Eyelid shortening alone appears to be as effective as combined techniques for involutional entropion with both horizontal and vertical eyelid laxity. Nevertheless, procedure selection should consider primary pathogenic factors.

Recurrent superior orbital fissure syndrome associated with VEXAS syndrome.

PURPOSE: To describe a case of recurrent orbital inflammation and superior orbital fissure syndrome associated with VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome. CASE PRESENTATION: VEXAS syndrome is a recently identified multi-system inflammatory disease of late adult onset. The authors describe the case of a 76-year-old man who presented with recurrent episodes of orbital inflammation, with superior orbital fissure syndrome, dacryoadenitis and orbital myositis. He had a constellation of systemic disorders including recurrent chest infections, congestive cardiac failure, pulmonary emboli and skin rashes. The underlying diagnosis of VEXAS syndrome was confirmed by genetic testing, which revealed the UBA1 mutation. CONCLUSION: VEXAS syndrome should be considered in the differential diagnosis of orbital inflammatory disease associated with multi-system inflammatory disorders.

Long-Term Surgical Outcome for Orbital Solitary Fibrous Tumors.

PURPOSE: To report long-term outcomes after surgery for orbital solitary fibrous tumors. METHODS: Retrospective review of patients with orbital solitary fibrous tumor, first seen between 1971 and 2022. Primary excisions were grouped as (A) surgically intact, (B) macroscopic, but with cell spillage, or (C) known incomplete excision. RESULTS: Fifty-nine patients (31 female; 53%) presented at a mean age of 43.0 years (19-82 years), with 5 patients (8.5%) having malignant solitary fibrous tumors. Average follow up was 11.4 years (median 7.8; range 1-43 years). There were 28 of 59 (47%) group A patients with 1 of 28 (3%) having recurrence, 20 of 59 (34%) group B having 6 of 20 (30%) recurrences, and 11 of 59 (19%) group C with 9 of 11 (82%) recurrence ( p < 0.001 for recurrence rate). At a mean of 8.9 (range 1-23.6) years after initial treatment, continued local tumor growth was evident in 16 (27%) patients, with higher-grade recurrence in 3 of 14 (21%) cases. No patient had systemic disease at presentation, but 2 of 59 patients (3%) developed metastases at 22 and 30 years after first treatment. The 10-year progression-free survival was 94% (group A), 60% (group B), and 36% (group C). Tumor disruption or incomplete excision (groups B + C) carries the highest risk of tumor recurrence (hazard ratio 15.0; 95% confidence interval, 1.98-114; p = 0.009), with no correlation to tumor size or histology. CONCLUSIONS: Orbital solitary fibrous tumors have a low recurrence rate with surgically intact excision; piecemeal excision, capsular disruption, or known incomplete resection have a high recurrence rate, which can occur decades later. Baseline postoperative scans is recommended, together with long-term clinical and interval imaging.

Presentation of orbital solitary fibrous tumours.

AIMS: To evaluate presenting features of patients with orbital solitary fibrous tumours (SFTs), based on histological phenotype. METHODS: A retrospective case-note review was performed for demographics and presenting features for patients with orbital SFTs. The tumours were classified as "Group IA" hypocellular SFT phenotype, "Group IB" haemangiopericytoma phenotype and low mitotic activity, and high-grade "Group II" haemangiopericytoma phenotype with high mitotic activity. RESULTS: Sixty-four patients (34 female; 53%) presented at a mean age of 42.2 years (median 38; range 19-82), with Group II patients presenting at an older age (mean 53 years). Median symptom duration was 12 months for Groups IA and IB, compared to 4 months for Group II, the commonest symptoms being proptosis (53%), diplopia (41%), periorbital swelling (31%), and altered vision (19%). Mean LogMAR was 0.17 (median 0.0; range -0.2-4), and 14% had ipsilateral optic neuropathy, with no significant difference between the three groups. Non-axial displacement was noted in 69%, a palpable mass in 45%, and reduced eye movements in 59%; choroidal folds and optic disc swelling were recorded in 12% and 9%. SFTs were mostly extraconal (59%), within the superior and superonasal quadrants (44%), with an average estimated tumour volume of 4.9 ml (median 3.6; range 0.31-14.5 ml). CONCLUSION: SFTs may present with impaired visual function (∼15%), fundal abnormalities (a fifth), globe displacement (two-thirds), and reduced ocular motility (over a half). High-grade tumours tend to present more than a decade later, with a shorter duration of symptoms.

Incidence and Risk Factors for Poor Postoperative Visual Outcome After Excision of Orbital Cavernous Venous Malformations.

AIMS: To determine the incidence and risk factors for poor postoperative visual outcome (PPVO) after removal of orbital cavernous venous malformations (OCVMs). METHODS: Retrospective case-note and imaging review for patients undergoing excision of OCVMs, with estimation of odds-ratios (ORs) and relative risks (RRs) for visual loss in relation to the position of the mass, surgical approach, and patient factors. RESULTS: The 290 patients (179 female; 62%) presented at a mean age of 46.4 years: 243/287 (85%) OCVMs were intraconal, with 213/243 (88%) located freely in the posterior two-thirds of the orbit, and 30/243 (12%) wedged tightly in the apex. PPVO was observed in 6.9% (20/290) patients, solely after removal of intraconal lesions, Univariate analysis showed increased risk with preoperative relative afferent pupillary defect (RAPD) (14/107 [13%]; RR 2.9; P = 0.011), apical lesions (9/30 [30%]; RR 5.8; P < 0.001), situated below optic nerve (15/115 [13%]; RR 3.3; P = 0.007), fibrous masses (14/78 [18%]; RR 6.7; P = 0.005), or intraoperative diastolic blood pressure below 50mmHg (10/64 [16%]; RR 2.8; P = 0.007). Multivariate analysis found apical extension (OR 4.9; P = 0.036) and fibrous lesions (OR 10.0; P = 0.035) as strongest predictors for PPVO. The incidence of complete visual loss (no light perception) was 4.1% (12/290); half of these patients had preoperative acuity of counting fingers or worse, 8 (67%) had RAPD, 7 (58%) wedged apical lesions, and 8 (67%) were below the optic nerve. CONCLUSION: PPVO after excision of OCVMs can occur in up to 5% of "free" retrobulbar intraconal lesions and in approximately one-third of apical lesions.

Chorio-Retinal Folds Associated With Orbital Cavernous Venous Malformations.

PURPOSE: To determine the incidence of clinically detectable chorio-retinal folds (CRFs) with orbital cavernous venous malformations (OCVMs) and their recovery. METHODS: Retrospective case-note and imaging review, with estimation of odds ratios in relation to the position and volume of the mass. RESULTS: Records for 402 patients were reviewed, 83 (21%) having CRFs. The mean logarithm of minimum angle of resolution acuity was similar with or without CRFs (0.37 and 0.31, respectively; p = 0.46), but induced hyperopia was commoner with CRFs (76% vs. 12%; p < 0.001), exophthalmos greater (4.52 mm vs. 2.97 mm; p < 0.001), eye movement restriction commoner (37% vs. 21%; p = 0.004), and disc swelling more prevalent (42%, vs. 17%; p < 0.001). Orbital cavernous venous malformations with CRFs were almost all intraconal (98%; odds ratio 9.96; p = 0.002), and 93% (77/83) midorbital (odds ratio 6.02; p < 0.001). The median size with CRFs was twice that of those without (3.85 ml vs. 1.92 ml; p < 0.001), and two-thirds OCVMs with folds had volumes >2.5 ml ( p < 0.001). The OCVM was excised in 76 of 83 (92%) of CRF group and 213 of 319 (67%) of those without ( p < 0.001). The postoperative acuity was improved or unchanged in 67 of 76 (88%) eyes with folds, and 184 of 213 (83%) without CRFs ( p = 0.84). The proportion recovering an acuity within 1 Snellen line (or better than) the unaffected side was 80% with CRFs and 77% in their absence ( p = 0.63). Induced hyperopia persisted in 39% of all patients, with the mean being higher with CRFs (2.22D vs. 1.02D; p = 0.017). CONCLUSIONS: CRFs occur in ~25% of mid-intraconal OCVMs. Despite OCVM excision, 39% of operated patients retain significant residual hyperopia (54% if CRFs present before surgery), and 41% of such CRFs remain clinically detectable after surgery (with variable visual impairment). Earlier surgery might, therefore, be advisable in patients with CRFs and/or induced hyperopia.

Necrotizing Blepharoconjunctivitis and Keratitis in Human Monkeypox.

Importance: Ophthalmic manifestations occur in less than 5% of patients with human mpox (monkeypox), most commonly presenting with self-limiting conjunctivitis and keratitis. Cases with severe ophthalmic complication are uncommon. Objective: To present a case of human mpox with sight-threatening necrotizing blepharoconjunctivitis. Design, Setting, and Participants: This is a report of a patient who developed necrotizing conjunctivitis due to the monkepox virus at a large university hospital. Data were collected from July to October 2022. Main Outcomes and Measures: Description of the progression and clinical evaluation of the ocular condition and the management. Results: A 63-year-old HIV-positive man presented initially with conjunctivitis and eyelid swelling and developed skin lesions from monkeypox virus 2 days later. Despite remaining stable systemically, after 4 days, his ophthalmic condition evolved to necrotizing blepharoconjunctivitis for which systemic antiviral treatment with tecovirimat was given along with topical trifluoridine, 1%, eye drops. In addition, he required repeated tissue debridement with amniotic membrane grafting to preserve the eye integrity. Conclusions and Relevance: The severity of this observation was associated with a coexisting immunocompromised state and appeared similar to findings associated with other orthopoxviruses. Ophthalmic manifestations could be the initial presentation of human mpox and could also be severe. Early recognition and intervention may limit the likelihood of substantial ocular morbidity.

Persistent orbital inflammation following complete excision of deep dermoid cysts.

Piecemeal excision of dermoid cysts carries the risk of implanting epithelial fragments into orbital fat, which is well recognized to continue secreting oily debris, inciting chronic, often granulomatous inflammation. The authors present the clinical and histological details for two patients with persistent lipogranulomatous inflammation for years after piecemeal excision of deep orbital dermoid cysts, in the absence of any residual epithelium. The importance of copious saline lavage - to 'float-out" and reduce microscopic lipid droplets - is also emphasised.

Presenting features for developmental cysts of the orbit.

PURPOSE: To evaluate the clinical features of developmental cysts of the orbit. PATIENTS AND METHODS: Retrospective study of patients who had excision of cysts between 1992 and 2020. RESULTS: Three hundred and 58 patients (189 male; 53%) with orbital developmental cysts were identified, all being unilateral. Age at surgery varied from birth to 77 years (mean 17, median 18 years) and the average symptom duration was 5 years (median 18 months; range 1 day-50 years). The commonest presenting features were a peribulbar lump or upper lid swelling, followed by proptosis, pain, diplopia and reduced vision. Most patients (82%) had a palpable mass, with epidermoids, sebaceous dermoids and keratinised dermoids commonly affecting the superotemporal quadrant, and conjunctiva-containing cysts usually being biased to a medial location. Cysts were lined by keratinised epithelium with dermal structures (224/358; 63%), non-keratinised epithelium with dermal structures (69/358; 19%), epidermis without identifiable dermal structures (19/358 'epidermoids'; 5%), conjunctiva (12/358; 3%), respiratory epithelium (4/358; 1%), or mixed dermal and conjunctival epithelia (30/358 'dermo-conjunctival' cysts; 8%). Overall, two-thirds (242/358; 66%) had histological evidence of chronic intramural inflammation, and a half of cysts showed granuloma formation (178/358 cysts). Chronic inflammation was less common with conjunctival cysts (54%, 7/12 patients) and none showed granuloma formation. CONCLUSION: Developmental cysts of the orbit vary from the relatively common dermoid cysts to the extremely rare respiratory epithelial-lined cysts. Respiratory cysts, being deeper, may present late in life and cysts containing conjunctival epithelium tend to be less inflamed and typically favour the superonasal quadrant.

Pupillary Abnormalities After Excision of Orbital Cavernous Venous Malformations.

AIMS: The aim of this study was to determine the incidence and recovery rate for pupillary abnormalities after excision of orbital cavernous venous malformations (OCVMs). METHODS: Retrospective case-note and imaging review for patients with OCVMs, with an estimation of odds ratios (ORs) in relation to the position of the mass and surgical approach. RESULTS: The 287 included patients (176 female; 61%) presented at a mean age of 46.5 years: 243/287 (85%) OCVMs were intraconal, 253/287 (88%) located in the posterior two-thirds of the orbit, and 29/287 (11%) wedged tightly in the apex. Pupillary changes-varying from minor sectoral paresis (only evident on slit-lamp examination) to a marked mydriasis-were noted in 21% (60/287) of patients and were strongly associated with removal of intraconal (60/243 [25%]; p < 0.001), apical (14/29 [48%]; OR, 4.3; p < 0.001), inferior or inferotemporal (41/105 [39%]; OR, 5.5; p < 0.001) masses. Lateral approaches-either with (23/129; 18%) or without (30/71; 42%) osteotomy-were associated with 88% (53/60) of all abnormalities (OR, 4.1; p < 0.001). Where known, the abnormalities improved in 43/50 (86%), although 15/43 (35%) had a persistent tonic pupil. CONCLUSION: Pupillary abnormalities after excision of OCVMs can occur in up to a quarter of intraconal lesions-particularly those located inferotemporally or inferiorly-and in about a half of apical lesions. Lateral or inferolateral orbital approaches that disrupt the inferior intraconal fat appear to be associated with a higher risk. Most changes resolve or improve markedly, although a third of those affected may have a persistent tonic pupil.