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

We report a case of unilateral Morbihan's disease (MD) in a 55-year-old male presenting to a private oculofacial practice in San Francisco. Successful treatment with a series of triamcinolone injections to the upper and lower eyelids was achieved after several unsuccessful therapies. The authors provide a comprehensive review of the literature from 2022 to 2025 on MD.

Sphenoid wing dysplasia and plexiform neurofibromas are among the diagnostic criteria for neurofibromatosis 1 (NF1), an autosomal dominant neurocutaneous disorder. It has been called by different names based on the involved anatomic areas such as orbito-palpebral, orbito-temporal, and orbito-facial plexiform neurofibromatosis. Management of the deformity in plexiform neurofibromatosis is challenging given the ill-defined, infiltrative, and vascular nature of the lesion, associated with a defect in the greater wing of sphenoid, resulting in pulsatile proptosis. A staged multidisciplinary approach has been described in literature to correct the bony dysplasia followed by correction of the soft tissue components. Craniofacial bony deformities have been mostly described to be managed via craniotomy approach by neurosurgeons whereas the periorbital debulking of tumor and correction of canthal dystopia and blepharoptosis is done by ophthalmic plastic surgeons at a later stage. Bony defects have been traditionally managed with the help of bone grafts or titanium mesh or a combination of both, but, more recently, the use of patient specific implants has also been described which offer bespoke reconstruction. A single staged transorbital approach to reconstruct the skull base defect and orbital aperture along with palpebral debulking and levator resection has not been described till date to the best of the authors' knowledge. Here, the authors describe a case of orbitopalpebral plexiform neurofibromatosis with pulsatile proptosis managed via a single staged transorbital approach using computer-assisted 3 dimensional (3D) virtual surgical planning.

Purpose: This systematic review and meta-analysis compares the effectiveness of bone grafts versus titanium mesh implants in managing orbital fractures, focusing on diplopia, enophthalmos, postoperative complications, and aesthetic outcomes.

Methods: A systematic search of MEDLINE, EMBASE, CINAHL, and CENTRAL databases was conducted as per PRISMA guidelines. Studies directly comparing bone grafts and titanium mesh in orbital fracture management were included. Primary outcomes included diplopia, enophthalmos, complications. Aesthetic outcomes were qualitatively assessed. Meta-analyses were performed using fixed and random-effects models and risk of bias was evaluated using the ROBINS-I tool.

Results: Eight studies involving 291 patients were included. No significant difference in diplopia incidence was observed between groups (OR: 1.87; 95% CI: 0.52-6.71; p = 0.34). Titanium mesh was associated with a significantly lower risk of enophthalmos (OR: 2.83; 95% CI: 1.19-6.75; p = 0.02). Complication rates were comparable (OR: 2.39; 95% CI: 0.55-10.35; p = 0.25). Aesthetic outcomes were favourable with both materials but insufficiently reported for meta-analysis.

Conclusion: Both bone grafts and titanium mesh implants are effective options for orbital fracture repair. Titanium mesh offers advantages in restoring orbital volume and preventing enophthalmos, supporting its preferential use in high-risk cases. Material selection should consider patient factors, defect characteristics, surgeon expertise and material availability.

Systemic sarcoidosis rarely presents as tattoo granulomas. We present a case of an eyelid tattoo granuloma as a prodrome to the diagnosis of sarcoidosis; this case is unique in its onset being several decades following the initial tattooing event, in addition to its resolution with intralesional triamcinolone. A 63-year-old female presented with ipsilateral upper and lower lid margin tattoo granulomas, 6 months post bilateral conjunctivitis. A punch biopsy of the granuloma revealed dermal non-caseating granulomatous inflammation. Serum angiotensin-converting enzyme levels were elevated, and a computed tomography scan revealed pulmonary lymphadenopathy, in keeping with a diagnosis of sarcoidosis. The granulomas resolved with a combination of intralesional triamcinolone and topical hydrocortisone ointment. The differential diagnosis for tattoo-associated granulomatous lesions is broad. This case report highlights the importance of biopsy and appropriate investigations for tattoo granulomas, as it may be the only sign of a systemic condition such as sarcoidosis.

Purpose: To evaluate diagnostic tests and patient characteristics associated with specific diagnoses of orbital inflammation to optimize cost-effectiveness.

Methods: A retrospective, observational case series, including 121 adult patients who underwent work-up for a clinically inflamed orbit. Chart review collected demographic and clinical information. Cost of testing was estimated based on publicly available data. Logistic regression was used to identify factors associated with a specific diagnosis.

Results: The median patient age was 49 years, and a majority were female (63%) and White (51%). A mean of five laboratory tests was ordered per patient. Two-thirds of the expenditure on diagnostic testing did not provide key diagnostic information. A specific diagnosis was reached for 31% of patients. Specific final diagnosis was significantly associated (p < 0.05) with older age (OR 1.6 per decade), male gender (OR 2.9), bilateral disease (OR 4.8), diffuse inflammation (OR 11.6), and bone involvement (OR 20.9).

Conclusions: Orbital inflammation was associated with a broad diagnostic work-up that led to a nonspecific diagnosis in the majority of cases. Cost savings may be achieved by judicious use of tests that provide limited diagnostic information (ANA, anti-dsDNA, ACE, anti-CCP, ESR, CRP, platelets) and reserving comprehensive work-up for patients with risk factors.

Epstein-Barr Virus-associated smooth muscle tumors (EBV-SMTs) are rare neoplasms that primarily occur in immunocompromised individuals. Orbital EBV-SMTs are an exceptionally rare subset of these tumors which may cause mass effect that leads to functional impairment. We report a case of a patient with systemic lupus erythematosus on long-term corticosteroids who was later found to have T cell deficiency and markedly reduced CD4 and CD8 counts. She presented with a rapidly enlarging right orbital mass causing proptosis, optic nerve compression, and visual dysfunction. Imaging revealed an extraconal lesion with bony erosion and sinus extension. Histopathology confirmed the diagnosis of EBV-SMT. The patient was managed by a multidisciplinary team including Ophthalmology (Oculoplastic Surgery) and Otorhinolaryngology (Rhinology). Pre-operative 3D-printed models were used for surgical planning. Tumor debulking was then performed using a combined transorbital and endoscopic endonasal approach, together with intraoperative CT and MRI-based image guidance to maximize safety and preserve optic nerve function. Systemic screening identified additional EBV-SMT lesions requiring surgical intervention, and sirolimus therapy was initiated as a possible immunomodulatory adjunct to slow the growth of these multifocal tumors. There is currently no standardized treatment for orbital EBV-SMTs. Surgical resection or debulking remains the mainstay, but complete excision may be limited by anatomical constraints. Immune reconstitution, together with targeted therapies such as sirolimus and bevacizumab offer additional options. This case highlights the importance of considering EBV-SMT in immunocompromised patients with rapidly enlarging orbital masses. Multidisciplinary management, including surgery, systemic evaluation, and immune modulation, is crucial to optimize outcomes.

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.