Acute dacryocystitis secondary to Burkholderia cepacia and Sphingomonas paucimobilis mixed infection: A novel case report.

Acute dacryocystitis caused by Burkholderia Cepacia and Sphingomonas Paucimobilis is uncommon. To the best of our knowledge, presence of mixed pathogens Burkholderia Cepacia and Sphingomonas Paucimobilis causing acute dacryocystitis in immunocompetent patients never been described. Burkholderia Cepacia and Sphingomonas Paucimobilis been reported only as a single microorganism causing other ocular infections in immunocompromised patients. Middle age, medically free female patient, presented to the emergency department at our hospital, with a history of nasolacrimal duct obstruct (NLDO) complaining of inferior preocular swelling associated with localized pain diagnosed as acute dacryocystitis. She was on oral Amoxicillin/clavulanic acid, oral Nitroimidazole antimicrobial and topical Tobramycin from elsewhere. However, no improvement had been noticed. We kept the patient on the same medications and swaps taken for culture and sensitivity. Patient presented to the first follow-up appointment with no improvement on her status. Culture and sensitivity revealed 2 pathogens: Burkholderia Cepacia and Sphingomonas Paucimobilis. We have changed the antibiotic to oral Trimethoprim/ Sulfamethoxazole as it showed positive sensitivity to the pathogens based on the sensitivity chart. Second follow-up appointment patient's condition improved.

Primary Orbital Respiratory Epithelial Cyst: A Systematic Review and Two Case Reports.

A primary orbital respiratory cyst is a congenital choristoma that presents in the orbit and with different signs and symptoms depending on the location, which might also change the surgical approach. The aim of this report is to describe 2 new cases of primary respiratory epithelial cysts and to review the literature on presentation, management, and risk factors with different surgical approaches and complications. Two cases presenting with gradually increased proptosis had a confirmed diagnosis of a respiratory epithelial cyst. CT and MRI imaging revealed a thin-walled intraconal cystic lesion. Complete surgical removal was not possible and an incisional biopsy of the wall was performed. Rarely, orbital respiratory epithelium cysts are primary lesions. The high risk of breaking the capsule during surgery and the presence of residual epithelial cells within the orbit may cause cyst's recurrence. Indeed, we recommend careful attention during dissection maneuvers and a long-term follow-up.

An advanced case of pediatric ciliary body medulloepithelioma with detailed literature review.

INTRODUCTION AND IMPORTANCE: Medulloepithelioma is the second most common primary intraocular malignant tumor in children but is often diagnosed late, which worsens the prognosis. CASE PRESENTATION: We are reporting a 6-year-old boy presenting with a ciliary body (CB) teratoid malignant medulloepithelioma (TMM), which was missed at the initial presentation. We added our case to the 97 previously reported cases in our literature review that were confirmed by cytological or histopathological examination. DISCUSSION: Medulloepithelioma has a wide age range at presentation with a mean of 5.3 ± 4.1 years, and slight male predominance (M: F ratio of 1.15). Clinically, the main symptoms/ signs are reduced visual acuity, lens changes, or a pupil "mass." Almost all reported medulloepitheliomas were unilateral (98 %). Histopathologically, non-teratoid malignant medulloepithelioma (NTMM) (35.7 %) and TMM (34.5 %) were the commonest. Enucleation was the main treatment modality because of the large tumor size of 72.7 %. A combination of chemotherapy and/or radiotherapy was used in 15.1 % and brachytherapy for small lesions in 14.1 %. Orbital exenteration was needed in 3 cases because of orbital invasion. CONCLUSION: A CB medulloepithelioma tumor can be easily missed, diagnosed late, with less chance for globe salvaging. The malignant types are more common and may result in orbital invasion, requiring even more extensive surgery. Therefore, when facing a child with lens changes, a pupil "mass," or raised intraocular pressure, pediatricians and general ophthalmologists must have an earlier suspicion and be aware of this rare entity to improve outcomes and reduce loss of vision or other disease-related morbidity.

DCR preferences among oculoplastic surgeons: Barriers and facilitators to adoption of endoscopic DCR.

OBJECTIVE: To determine the preference for dacryocystorhinostomy (DCR), patient selection criteria for endoscopic DCR, endoscopic DCR technique, and barriers to adoption of endoscopic DCR. METHOD: Cross-sectional study conducted from May-December 2021. A survey was sent to oculoplastic surgeons. Questions on demographic characteristics, type of clinical practice, technique preferences, barriers and facilitators to adoption of endoscopic DCR were included. RESULTS: 245 participants completed the survey. Most respondents were located at an urban site (84%), were in private practice (66%), and had been in practice for more than 10 years (58.9%). Sixty one percent perform external DCR as the first line procedure for treating primary nasolacrimal duct obstruction. The most common factor influencing the surgeon's decision to perform endoscopic DCR was the patient's request (37%) followed by endonasal exam (32%). The most common barrier for not performing endoscopic DCR was the lack of experience and lack of training during fellowship (42%). The most worrisome complication for most respondents was failure of the procedure (48%), followed by bleeding (30.3%). Eighty one percent believe surgical mentorship and supervision during initial cases would facilitate endoscopic DCR learning. CONCLUSIONS: External Dacryocystorhinostomy is the preferred technique for treating primary acquired nasolacrimal duct obstruction. Learning endoscopic DCR early during fellowship training and high surgical volume to improve the learning curve dramatically impacts the adoption of the procedure.

Isolated bilateral orbital and paranasal Rosai-Dorfman disease affecting two brothers: a case report and a systematic literature review.

A systematic literature review revealed 88 Rosai-Dorfman Disease (RDD) cases affecting the orbit. We present a review of the literature on orbital RDD in addition to two brothers with isolated bilateral extra-nodal orbital RDD cases associated with G-6PD deficiency. The disease manifested as asymmetric extensive orbital and paranasal sinus infiltration, with orbital bone destruction. Orbital mass debulking and steroids improved the condition in both patients. This is the most extensive case series of orbital RDD reported, including clinical manifestation, imaging, pathologic results, management, and outcome. Vigilant follow-up is essential to monitor for potential malignant transformation, systemic manifestations, potential vision loss, or life-threatening recurrences.

Lacrimal Gland Lesions Biopsied in a Tertiary Eye Center in Saudi Arabia: A Clinical, Radiological, Surgical, and Histological Review.

PURPOSE: To study the demographics and clinical presentation of biopsied lacrimal gland lesions in a tertiary eye center and determine the accuracy of radiological imaging and blood investigations in reaching the diagnosis. We also studied the histopathological outcome of different lacrimal gland biopsy approaches. MATERIALS AND METHODS: A retrospective review of patients' charts from 2014 to 2021 who underwent lacrimal gland biopsy. The data collected included demographics, clinical presentations, surgical approaches used, blood workup done, and histopathological and radiological diagnoses. The accuracy of radiological imaging in diagnosing lacrimal gland pathologies was evaluated by comparing radiological interpretation to the final pathological report. RESULTS: In a total of 48 cases, the most common lacrimal gland lesion was nonspecific inflammation of the lacrimal gland (34%, n=17) followed by lymphoproliferative lesions (25%, n=12). Females (62.5%, n=30) were more affected than males (37.5%, n=18). Other lesions included pleomorphic adenoma, angiolymphoid hyperplasia with eosinophilia, and IgG4 disease. Complete blood investigations were performed in 45.8% of patients and a systemic disease was found in only 18.2%. The initial assumptive diagnosis based on MRI and CT scan images was congruous with the histopathological diagnosis in 42% and 12%, respectively. Three surgical approaches were used for incisional biopsy in this study, including sub-periosteal, trans-septal, and palpebral lobe biopsy. Lateral orbitotomy was done in all excisional biopsy cases. We found no statistical difference in the histopathological outcome between the different surgical approaches used. CONCLUSION: Nonspecific inflammation and lymphoproliferative disorders are the most common causes of lacrimal gland lesions. Blood work-up should not be used to waive off the need for lacrimal gland biopsy, and the diagnosis should preferably be based on histopathology results. The MRI is a useful tool to diagnose lacrimal gland lesions; however, histopathology remains the gold standard method.

Long-term surgical outcomes of levator resection in patients with Marcus-Gunn jaw-winking ptosis.

PURPOSE: To report long-term surgical outcomes of a series of MGJWS patients with moderate ptosis who underwent levator resection. MATERIALS AND METHODS: A retrospective review of medical records of 10 MGJWS patients with moderate ptosis who underwent levator resection was performed in two major eye centers in Riyadh, Saudi Arabia from January 1987 to December 2019. Clinical features, pre-operative and post-operative data of the 10 patients were recorded. OUTCOME MEASURES: Long-term upper eyelid position. RESULTS: The mean patient age at presentation was 5.4 years. Seven patients were female and three were male. All patients had moderate ptosis with marginal reflex distance ranged from 1 to 2 mm. The mean age at surgery was 9.5 years. After initial good ptosis correction for all patients, seven of them had a recurrence of ptosis several months after levator resection. CONCLUSIONS: Levator resection in MGJWS patients with moderate ptosis and good levator function is associated with a high rate of ptosis recurrence and less predictable surgical results.

RNA-sequencing highlights differential regulated pathways involved in cell cycle and inflammation in orbitofacial neurofibromas.

Although most commonly benign, neurofibromas (NFs) can have devastating functional and cosmetic effects in addition to the possibility of malignant transformation. Orbitofacial NFs, in particular, may cause progressive, disfiguring tumors of the lid, brow, temple, face, and orbit, and clinical evidence suggests that they may have increased local aggressiveness compared to NFs developing at other sites. The purpose of this study was to identify biological differences between orbitofacial NFs and those occurring at other anatomic sites. We performed RNA-sequencing in orbitofacial (n = 10) and non-orbitofacial (n = 9) NFs. Differential gene expression analysis demonstrated that a variety of gene sets including genes involved in cell proliferation, interferon, and immune-related pathways were enriched in orbitofacial NF. Comparisons with publicly available databases of various Schwann cell tumors and malignant peripheral nerve sheath tumor (MPNST) revealed a significant overlap of differentially expressed genes between orbitofacial versus non-orbitofacial NF and plexiform NF versus MPNST. In summary, we identified gene expression differences between orbitofacial NF and NFs occurring at other locations. Further investigation may be warranted, given that orbitofacial NF are notoriously difficult to treat and associated with disproportionate morbidity.

Assessing public awareness of daily eyelid hygiene habits in Saudi Arabia: An online survey study.

PURPOSE: Incidences of ocular conditions, including meibomian gland dysfunction (MGD), blepharitis, and dry eye have been increasing globally. Eyelid hygiene is key to maintaining ocular surface health and improving ocular symptoms. This study investigated the awareness of eyelid hygiene among the Saudi population. METHODS: This cross-sectional survey was conducted between April 2020 and May 2020 across all regions of Saudi Arabia through the distribution of an electronic self-administered questionnaire among the Saudi population. RESULTS: A total of 1102 responses were received (women, 76.3%; men, 23.7%). Only 33.2% respondents reported consciously washing their eyelids, while 70.1% had never heard about MGD; however, most of the respondents (93.2%) had heard about dry eye. Based on the questionnaire results, the Saudi population had suboptimal (18.4%) level of awareness of eyelid hygiene. At least one ocular symptom was reported by 98.1% of the respondents. CONCLUSION: The level of awareness of eyelid hygiene in Saudi Arabia was found to be suboptimal, particularly among patients with MGD and dry eyes. Poor knowledge about the benefits of daily eyelid hygiene was the primary barrier to its practice. We recommend that ophthalmologists raise awareness of good eyelid hygiene among patients with MGD and eye dryness. Despite the current findings, further studies and evidence are required before recommending daily eyelid hygiene practices in the general population, including those without clinical symptoms of MGD or eye dryness.

Marcus Gunn Jaw-Winking Synkinesis With Ipsilateral Eyelid Myokymia.

PURPOSE: To describe a novel observation of ipsilateral eyelid myokymia in the context of Marcus Gunn jaw-winking synkinesis (MGJWS). METHODS: A retrospective case series of 5 patients observed to have myokymia in the context of MGJWS in 2 tertiary hospitals in Riyadh, Saudi Arabia was conducted. Demographic profile including age and gender, and clinical features were analyzed. RESULTS: Five patients (3 males and 2 females) with MGJWS were noted to demonstrate the phenomenon of ipsilateral eyelid myokymia. All but 1 had right-sided MGJWS. The myokymia was seen as upper eyelid twitching in a vertical fashion along the levator palpebrae superioris muscle field of action. All subjects also had ipsilateral Monocular elevation deficiency. CONCLUSION: Ipsilateral upper eyelid myokymia is a potential feature of MGJWS. Monocular elevation seems to be a constant feature among MGJWS patients with levator muscle myokymia.Marcus Gunn jaw-winking synkinesis (MGJWS) is not well understood. Ipsilateral eyelid myokymia is a potential feature of MGJWS. This finding suggests that peripheral dysinnervation is likely to be a part of MGJWS.Supplemental Digital Content is available in the text.

Marcus Gunn jaw-winking synkinesis (MGJWS) is not well understood. Ipsilateral eyelid myokymia is a potential feature of MGJWS. This finding suggests that peripheral dysinnervation is likely to be a part of MGJWS. Supplemental Digital Content is available in the text.