Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery最新文献

A 19-year-old male with a history of sickle cell disease (type SS) was seen for left upper eyelid edema during an admission for sickle cell crisis. Magnetic resonance imaging revealed T2 hyperintensity and diffusion restriction within the left greater wing of the sphenoid, corresponding to orbital bony infarction, a rare orbital manifestation of sickle cell disease. We present a case of left greater sphenoid wing infarction in the setting of sickle cell vasoocclusive crisis successfully managed with medical therapy.

Purpose: To evaluate the incidence and characteristics of menstrual changes associated with teprotumumab treatment for thyroid eye disease (TED).

Methods: A retrospective chart review of female patients receiving teprotumumab treatment was performed across three institutions between 1/2020 and 12/2023. Data collection included age, thyroid status, hormonal contraception use, pre- and post-treatment clinical activity score, adverse events, and type of menstrual changes. If menstrual changes occurred, further data collection included onset and duration of menstrual changes.

Results: Fifty-one patients were included with a mean age of 37 years (range 18-51). Changes in menstruation occurred in 28 patients (55%). Among these patients, 21 (75%) had amenorrhea, 6 (21%) had oligomenorrhea, and 1 (4%) had dysmenorrhea. The average number of days between first infusion and first change in menstruation was 58 days (range 7-150), and between last infusion and return to normal menstruation was 155 days (range 6-405). Four patients (14%) did not have a return to normal menstruation at time of last follow-up (mean 9 months, range 6-11 months). Overall mean follow-up was 16 months after last infusion.

Conclusions: Menstrual changes occurred in 55% of menstruating females receiving teprotumumab therapy. The most common changes were amenorrhea and oligomenorrhea. The majority of patients regained normal menstruation after cessation of therapy. It is important for providers to be aware of this potential adverse event when treating menstruating women with TED.

Infantile myofibromatosis is a rare fibrous tumor that typically presents during infancy or early childhood. We describe a 4-year-old boy with a history of multicentric infantile myofibromatosis who presented with new-onset right-sided proptosis. Magnetic resonance imaging (MRI) demonstrated an expansile intracranial lesion arising from the greater wing of the sphenoid bone, producing mass effect at the right orbital apex and compressing the optic nerve. Histopathologic examination of a biopsy specimen confirmed a myofibroma with associated reactive bone changes. Over a 36-month follow-up period, serial MRI studies showed gradual spontaneous regression of the orbital lesion. Although orbital involvement in infantile myofibromatosis is uncommon, prompt recognition and timely consideration of surgical or pharmacologic intervention are warranted. This case underscores the potential for spontaneous regression of orbital myofibroma following limited surgical management.

Objectives: To investigate nocturnal lagophthalmos after levator surgery for simple congenital ptosis, focusing on its correlation with office-based (clinical) lagophthalmos and factors contributing to clinical-nocturnal discrepancy.

Methods: A retrospective review was conducted on 109 children (median age, 7 years) who underwent unilateral levator surgery between 2021 and 2024. Lagophthalmos was evaluated clinically in the office and nocturnally using photographs obtained during sleep. Bell's phenomenon was assessed during daytime examination by observing upward and outward globe rotation on attempted eye closure, and nocturnal photographs were reviewed for globe position. Margin reflex distance 1 (MRD1), levator function (LF), and corneal fluorescein staining were recorded. Associations with demographic variables, ptosis severity, surgical type, and exposure keratopathy were analyzed.

Results: Sixty-five percent of patients underwent levator resection and 34.9% levator plication. At a median follow-up of 20 months (range, 5.1-58), median clinical lagophthalmos was 0 mm (range, 0-2.9) compared with 3.1 mm nocturnally (range, 0-8.3) (p < 0.001). Preoperatively, Bell's phenomenon was good in 99.1% of eyelids and remained good in 85.3% postoperatively. Nocturnal evaluation revealed scleral show in 13.8%, corneal show in 36.7%, and pupillary show in 22.9%. No significant associations were found between lagophthalmos change and demographics, ptosis severity, surgical type, MRD1, LF, or follow-up duration (p > 0.05). Greater changes were correlated with corneal staining (p < 0.001) and symptom severity (p < 0.001).

Conclusion: Significant nocturnal lagophthalmos with corneal exposure is common after levator surgery for congenital ptosis despite near-complete eyelid closure on clinical assessment. No visually threatening corneal complications were observed.

Purpose: We present a review of viral lacrimal drainage infections.

Methods: A literature review was conducted using Medline and Embase databases following PRISMA guidelines. Twenty-seven studies met the inclusion criteria and were synthesised narratively. Data obtained include demographics, clinical presentation, types of viral infection, location of the infection, investigations, medical and surgical treatment, symptom resolution, complications, and infection sequelae.

Results: Four hundred and fifty-five cases of viral lacrimal drainage infections have been reported so far.  The majority of viral lacrimal drainage infections were secondary to herpes simplex virus (HSV) (83.1%, 378/455), followed by adenovirus (11.9%,54/455), varicella zoster virus (VZV) (2.2%, 10/455), and Epstein-Barr virus (EBV) (2.0%, 9/455). The majority [47.5% (216/455)] involved the canaliculus only. Of the 16 patients with nasolacrimal ductobstruction, all cases were related to adenovirus, EBV or VZV infections, not HSV. Where specified, 100% (10/10) cases of EBV-related lacrimal drainage infections and 75% (3/4) VZV-related lacrimal drainage infections completely resolved following conservative treatment alone. In contrast, only 1.1% (2/186) cases of HSV-related lacrimal drainage infection, and 5% (1/20) of adenovirallacrimal drainage infections, improved following conservative treatment alone.

Conclusion: Viral lacrimal drainage infections are relatively uncommon but should be suspected in patients presenting with new onset epiphora in the context of recent viral infections. Of these, the majority are likely to be related to HSV followed by adenovirus, which seem to be associated with a higher risk of permanent epiphora from scarring, compared with VZV and EBV infections, where the symptoms may be more likely to resolve through conservative treatment alone.

Sweet syndrome (SS), or acute neutrophilic dermatosis, is a dermatologic condition characterized by fever and neutrophilia with tender papular or plaque-like eruptions; diagnosis is confirmed histologically and the disease typically responds to systemic corticosteroids. Ocular associations include conjunctivitis, peripheral ulcerative keratitis, scleritis, uveitis, glaucoma, and retinal detachment, with occasional periocular involvement. A rare clinical variant, necrotizing Sweet's syndrome (NSS), closely mimics necrotizing fasciitis (NF). While NF requires urgent, extensive surgical debridement, NSS requires tissue biopsy, systemic corticosteroid therapy, and systemic evaluation to rule out underlying malignancy; misdiagnosis can therefore lead to inappropriate surgery and delayed treatment. We present three patients with isolated periocular SS. One had the classical form, while two were subsequently diagnosed with hematologic disease. All three exhibited features of NSS and underwent limited surgical debridement with microbiological and histopathological work-up, which confirmed SS. Collagen sheet grafting was used for wound coverage; two patients achieved rapid and cosmetically favourable recovery, while in one patient with full-thickness margin necrosis collagen alone was insufficient and an acquired eyelid coloboma remained. These cases underscore the need to distinguish NSS from NF: despite similar clinical appearance, the management is diametrically opposite.

Ocular and orbital symptoms are among the most common presenting signs in granulomatosis with polyangiitis (GPA). This report describes a patient with bilateral anophthalmos presenting with several weeks of left socket pain that was unresponsive to analgesics and low-dose oral steroid, without periorbital inflammation or orbital lesions on imaging. She was initially diagnosed with orbital neuralgia secondary to extensive prior orbital fractures and hardware and was scheduled for hardware removal. While awaiting surgery, she was admitted 1 month later for pneumonia and was diagnosed with GPA following an extensive systemic work-up. After commencing systemic treatment for the GPA, her left socket pain resolved. Therefore, GPA should be considered in the differential diagnosis of anophthalmic socket pain, even without periorbital inflammation or orbital lesions.

Purpose: To report the presentation, management, and outcomes of paediatric frontoethmoidal mucoceles causing epiphora.

Methods: Retrospective, interventional study involving six paediatric patients with paranasal sinus mucoceles presenting with epiphora over 13 years at a tertiary dacryology centre. Demographics, meticulous history, visual acuity, ophthalmic evaluation, lacrimal irrigation, and endoscopic evaluation of the nostril were documented. Computed tomography scans were performed in all patients. All patients underwent an endoscopic endonasal marsupialization of the mucocele. Functional improvements in epiphora and external signs were analysed.

Results: Six paediatric patients with a mean age of 12.83 years and equal sex distribution were included. The main presenting complaint was epiphora in all children and proptosis in one over an average period of 2.4 years. One child had history of trauma. External examination showed a telecanthus in all children and proptosis or dystopia in four. One child with prior trauma had a secondary acquired nasolacrimal duct obstruction (SALDO), while the others had freely patent irrigation. Four patients had ethmoidal and two had fronto-ethmoidal mucoceles, compressing the lacrimal drainage passage. After the endoscopic endonasal marsupialization of the mucocele, five children had relief from the epiphora with reduction in the proptosis and telecanthus. The patient with traumatic SALDO underwent additional endoscopic dacryocystorhinostomy and did well.

Conclusions: Paediatric sinus mucoceles are rare cases with very subtle signs, often presenting first to an ophthalmologist. Thus, vigilance and knowledge about the entity is necessary to identify and manage it in early stages.