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

Purpose: Assessment of the frontalis muscle flap eyelid reanimation surgical technique for adults with severe ptosis and apraxia of eyelid opening.

Methods: A retrospective case series of 30 eyes with severe ptosis or apraxia of eyelid opening. Outcomes were assessed for margin to reflex distance 1 (MRD1), lagophthalmos, complications, and need for subsequent surgical intervention. A paired t-test was used to compare preoperative and postoperative scores of a quality-of-life questionnaire.

Results: Thirty eyes of 19 patients underwent surgery, 16 (53%) with combined frontalis + levator-Muller muscle flap and 14 (46%) with frontalis muscle flap alone. There were 14 female and 5 male patients, with an average age of 55 years (range, 18-76). Mean preoperative MRD1 was -0.6 mm (range, -5 to 2) with mean levator excursion of 7.1 mm. Seventeen eyes had a myogenic etiology, five had a paralytic etiology, six had blepharospasm with apraxia of lid opening, and two had a neurodegenerative etiology. Nineteen eyes (63%) had previously undergone ptosis repair. Mean postoperative MRD1 was 2.5 mm (range, 0.5 to 5) at mean follow-up of 63.3 days. There were no serious surgical complications; minor complications included ocular surface keratopathy and one patient who required surgical revision. Results of the QOL questionnaire indicated significant improvement in vision-related symptoms postoperatively (p = 0.02).

Conclusions: Use of the frontalis muscle flap eyelid reanimation technique, with or without a levator-Muller retractor muscle flap, was very effective in this case series and provided good upper eyelid position with acceptable corneal protection and high patient satisfaction.

Li-Fraumeni syndrome (LFS) is caused by a pathogenic germline variant at the TP53 locus and is associated with an increased predisposition to a variety of cancers. The neoplasms most frequently associated with LFS are sarcomas, breast cancer, brain tumors, and adrenocortical carcinomas. In this case report, we present a 43-year-old male diagnosed with an ocular adnexal sebaceous carcinoma of the right upper eyelid who was confirmed to have LFS with subsequent genetic testing. The mutational profile of both the patient's genetic screen and tumor sequencing were congruent, demonstrating the same pathogenic loss-of-function TP53 variant. This case report highlights the importance of pursuing genetic testing in patients with a history of multiple tumor types, particularly those with uncommon diagnoses. In this case, confirmation of LFS had important implications for personalized patient care, including identification of contraindicated treatment interventions and the imaging modalities necessary for vigilant follow-up screening.

Purpose: Outbreaks of mucormycosis were reported worldwide throughout the COVID-19 pandemic. We report clinical outcomes of a treatment protocol for COVID-19-associated rhino-orbital-cerebral mucormycosis (ROCM).

Methods: Patients with biopsy-proven mucormycosis and COVID-19 were included. All received intravenous amphotericin B deoxycholate 1 mg/kg and surgical endoscopic sinus debridement (FESS). Those with rhino-orbital or cerebral disease limited to the cavernous sinus were eligible for transcutaneous retrobulbar amphotericin B (TRAMB). Patients were followed with weekly imaging, endoscopic examinations, and serial debridement as necessary. Patients were discharged on oral posaconazole for 6 months.

Results: In total, 264 patients were followed for a mean of 2.5 months. On presentation, 163 patients (174 eyes) had eye involvement. Of these, 141 eyes (81.0%) had light perception or worse vision. By the last follow-up, 163 patients (176 eyes) were affected, and of these, 96 eyes (54.5%) had no light perception. Twenty-one patients (8%) died and 3 orbits (0.5%) were exenterated. There was no change in mortality (p = 0.38) or exenteration (p = 0.38) in the 55 patients who received TRAMB compared to patients with rhino-orbital or cerebral disease limited to the cavernous sinus who did not. Asymptomatic COVID-19 was associated with higher mortality than symptomatic COVID-19 (p = 0.025). Uncontrolled diabetes was a risk factor for death (p = 0.022). New diabetes was associated with increased mortality versus pre-existing diabetes (p = 0.005).

Conclusion: A multidisciplinary approach is crucial to manage COVID-19-ROCM. In our cohort, TRAMB therapy did not increase mortality or exenteration rates. While poor vision on presentation was profound, some vision recovery was noted with treatment. COVID-19 immune dysregulation may predispose patients to ROCM, particularly those with asymptomatic disease.

Purpose: To assess the utility of pig head model as an oculoplastic surgical training tool.

Methods: Fresh pig head was availed for surgical workshop for entropion correction, eyelid margin repair, and evisceration with implant for oculoplastic surgery-naïve participants. Skill improvement, surgical understanding, and performance of the participants were evaluated both subjectively and objectively by trained oculoplastic surgeons. Subjective assessment was done by a standardized questionnaire based on Likert scale shared with the participants post training. Objective evaluation was done by the faculty based on a three-point scale and a competency-based surgical assessment rubric.

Results: There were 18 surgery-naïve participants in the workshop which comprised ophthalmology residents and comprehensive ophthalmologists. About 88.88% of the participants were able to perform the lid margin and sub-tarsal dissection in entropion surgery. While performing lid tear repair, 94.44% were correctly able to identify the grey line and anterior and posterior lamellae. About 83.33% of the participants were able to place an implant in the scleral shell or intraconal space. About 83.33% of the participants noted that texture and tissue maneuvering were similar to the human eye while performing surgical steps. About 94.44% of the participants reported better understanding, development of skill and additional confidence after training. The median score on objective assessment was 3. The performance on real patients resulted in a median score of 4 for entropion correction.

Conclusion: Porcine orbital dissection is an available, affordable, and useful tool for oculoplastic surgical training. Training on porcine model can improve anatomical understanding, clinical judgement, and surgical efficiency in trainees.

The present study reports a case of solitary neurofibroma attached to the Inferior Rectus (IR) muscle tendon in a 24-year-old healthy woman and reviews the relevant literature regarding the clinical presentation, diagnosis, and management of this uncommon tumor. The patient underwent successful surgical resection of the tumor, leading to the resolution of associated symptoms (left lower eyelid protrusion and redness). Pathological examination confirmed the diagnosis of neurofibroma based on characteristic histopathological and immunohistochemical markers. This case report underscores the rarity of solitary neurofibromas and primary neoplasms of orbit and ocular adnexa. We also discuss the background of solitary neurofibromas originating from orbit and ocular adnexa. The successful management of this case through surgical resection highlights the importance of accurate diagnosis and tailored treatment strategies. To the best of our knowledge, this is the first reported solitary neurofibroma confined solely to the IR tendon.

This case report highlights a unique presentation of Erdheim-Chester Disease (ECD) with bilateral scleral lesions, choroidal infiltration, and extensive sinus involvement. It is the first case report where the diagnosis was confirmed through a scleral biopsy after an initial presentation of a unilateral nodular scleritis. There was a gradual disease progression and ocular examination later revealed bilateral subconjunctival hyperemic lesions and mild exophthalmos, ophthalmoplegia, and extensive choroidal infiltration. Infiltration of the frontal and maxillary sinus was present and extended into the nasal cavity and both orbits. The diagnostic work-up is described in detail. Current treatment options are analyzed. It is emphasized that the ophthalmologist can play a crucial role in the diagnosis of ECD, given the substantial prevalence of orbital and ocular symptoms. The overall prognosis for ECD remains unfavorable, particularly in cases with orbital involvement. This case underscores the complexity and importance of a multidisciplinary approach in managing ECD.

A 23-year-old female symptomatic with unilateral proptosis with superior globe displacement and hypertropia was found to have a multiloculated cystic lesion with bony lining arising from the inferomedial orbit. The adjacent maxillary sinus was markedly smaller on the affected side, with no defect of the wall on radiographic or intraoperative examination. Surgical excision and histological analysis of the lesion demonstrated an orbital respiratory epithelial cyst. A literature review of congenital orbital respiratory epithelial cysts is presented. To our knowledge, this is the first published case of choristomatous orbital respiratory epithelial cyst associated with ossification, and a primary or secondary anomaly of the adjacent sinus without bony defect.