Müller muscle-Conjunctival Resection (MMCR) Surgery: A Comprehensive Literature Review.

PURPOSE: This literature review aims to provide a comprehensive overview of the current understanding, indications, techniques, outcomes, and complications of Müller muscle-conjunctival resection (MMCR) surgery. It also addresses areas of debate with MMCR such as predictability concerning Phenylephrine response as well as the amount of muscle resection required. METHOD: This literature review was compiled based on the available evidence from PubMed from 1975 to August 2023. RESULTS: The success rate of MMCR ranges from 72% to 95%, with high patient satisfaction reported in most studies. The exact range of eyelid elevation after MMCR is variable, with a reported average of 2.1 mm MRD1 elevation. CONCLUSION: MMCR is a well-established surgical technique used to correct upper eyelid ptosis that results from dysfunction or attenuation of the levator aponeurosis. MMCR proved to be a safe and effective procedure with a high patient satisfaction rate and low risk of complications.

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.

Orbital abscess with white eye in patients treated with oral antibiotics for orbital cellulitis.

PURPOSE: To report a case series of seven patients with late presentation of orbital/subperiosteal abscess following oral treatment of orbital cellulitis. METHODS: A retrospective case series of all patients presented with orbital abscess following oral treatment of orbital cellulitis from two tertiary-care eye centres in Riyadh, Saudi Arabia, was conducted. Demographic profiles, risk factors, initial clinical presentation, management regimens, and final outcome were analysed. RESULTS: Patients presented mainly with proptosis and limitation of extraocular motility without external ophthalmic inflammatory signs. Most patients needed surgical evacuation despite the appropriate initiation of intravenous antibiotics following presentation to our hospitals. CONCLUSION: Treating orbital cellulitis with oral antibiotics may lead to delayed presentation of orbital abscess without external ophthalmic inflammatory signs.

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.

Invasive sino-orbital aspergillosis with brain invasion in an immunocompetent pregnant patient.

PURPOSE: Invasive Fungal Sinusitis (IFS) is a potentially life-threatening condition that can progress rapidly to the orbit and the brain, especially if it goes on undetected for a long period. We report a case of a 28-year-old pregnant woman in her second trimester with sino-orbital A spergillosis and subsequent brain involvement who tragically developed deterioration of her neurological status and a spontaneous abortion. OBSERVATIONS: The patient presented to the ophthalmology emergency department, King Khaled Eye Specialist Hospital, Riyadh, complaining of left upper eyelid fullness with a palpable eyelid mass and chronic relapsing remitting dull pain for 4 months. Clinical examination was significant for reduced colour vision in the left eye, limited left supraduction, left upper eyelid firm palpable mass, inferior dystopia and proptosis of 4 mm. Magnetic Resonance Imaging (MRI) done without contrast-enhancement due to her pregnancy revealed aggressive infiltrative sinonasal, nasal septum, cribriform plate, orbital, intracranial infiltration with extensive brain edema and midline falcine herniation patterns. Endoscopic endonasal biopsy of the lesions showed septate hyphae branching at acute angles, suggestive of A spergillosis. Her neurological status deteriorated with a spontaneous abortion during admission. CONCLUSIONS AND IMPORTANCE: This case demonstrates that IFS could present only with proptosis, eyelid fullness, chronic pain without external inflammatory signs and should be considered in such presentation even in immunocompetent patients. Early detection and management are crucial. Whether pregnancy presents a relative immune susceptibility to IFS is an issue that needs further in-depth investigation.

Lacrimo-auriculo-dento-digital syndrome: A case report and literature review.

We report a healthy 18-year-old male Saudi with bilateral agenesis of the lacrimal puncta and canaliculi associated with large dacryocystocele on the right side without tearing or inflammation, detected in conjunction with other characteristic features of lacrimo-auriculo-dento-digital dysplasia syndrome. Computed tomography scan indicated that dacryocystoceles were bilateral and asymmetrical, with large dimensions at the right side and associated to a right side maxillary sinus mucocele. The right dacryocystocele was surgically removed, and the histology indicated characteristics of the lacrimal sac. The liquid content of the dacryocystocele was negative for microbes. The atypical mucocele in the maxillary sinus disappeared after dacryocystocele removal probably, due to recovery of sinus drainage.

Microbiology and outcome of pediatric orbital cellulitis in a Tertiary Eye Care Center in Saudi Arabia after the routine administration of Haemophilus influenzae Type B vaccine.

PURPOSE: To evaluate the incidence of pediatric Haemophilus influenzae related orbital cellulitis after the routine administration of the H. influenzae vaccine in Saudi Arabia and to identify the most common pathogens, predisposing factors, related complications, and the need for surgical intervention. METHODS: A retrospective chart review was performed of patients with a discharge diagnosis of orbital cellulitis who presented to the King Abdul Aziz University Hospital, Riyadh, Saudi Arabia. Saudi children born between 2000 and 2016 were included. RESULTS: Thirty-one cases were included and 8 had positive cultures. No cases of H. influenzae were found. Of all the positive culture, the most common pathogens isolated were Staphylococcus aureus, Streptococcus pneumoniae and group A Streptococcus in 3 cases (37.5%), 2 cases (25%) and 2 cases (25%). The most common predisposing factor was sinusitis in 90.3% of cases. Sixteen patients (51.6%) developed complications. Fifteen patients (48.3%) developed subperiosteal abscesses, 1 patient (3.22%) developed a lid abscess, 2 patients (6.45%) developed recurrent orbital cellulitis, and 1 patient (3.22%) developed persistent strabismus. Only 10 (31.25%) patients underwent surgical intervention during admission. CONCLUSION: The vaccine seems to be effective at eliminating the orbital cellulitis potentially related to H. influenzae. The most common current pathogens were Gram-positive Staphylococcus and Streptococcus species. Sinusitis remains the most common cause of orbital cellulitis in children. In select cases, medical treatment can resolve the infections however, close observation is warranted.

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.

Adams Oliver syndrome: A mimicker of familial exudative vitreoretinopathy.

PURPOSE: To describe an infant with Adams Oliver syndrome (AOS) with ocular signs similar to familial exudative vitreoretinopathy. OBSERVATIONS: A full-term female infant presented with a congenital scalp defect, hypoplasia of the fingers and toes along with a radial retinal fold in the right eye and tractional retinal detachment in the left eye. Fluorescein angiography findings included peripheral retinal nonperfusion, irregular vascular sprouting beyond the vascular-avascular junction, pinpoint areas of hyperfluorescence as well as late peripheral and posterior vascular leakage. The patient was clinically diagnosed with Adams Oliver syndrome based on the collective findings. Laser photocoagulation to the avascular retina was performed in both eyes which resulted in stabilization of the condition after 2 years of follow up. CONCLUSION AND IMPORTANCE: The ocular phenotype in AOS may be similar to familial exudative vitreoretinopathy. Therefore, suspicion of the diagnosis should prompt ophthalmic evaluation including fluorescein angiography to detect and possibly treat the ischemic retinopathy.

Krause's accessory lacrimal gland dacryops - case report and literature review.

The authors are describing two rare cases of dacryops involving the Krause accessory lacrimal glands and affecting the orbit. In both cases, the lesion was located in the inferior fornix, with no preceding trauma, surgery or trachomatous conjunctival scarring. The first case involved a clear orbital extension with evidence of optic nerve compression. The second case extended from the anterior orbit to the limbus. Both had surgical removal with good outcome. As Krause gland dacryops are similar to other orbital cysts, clinical, imaging and histopathologic evaluation are essential to confirm the final diagnosis.

Congenital bilateral upper lid eversion.

We report two healthy Saudi newborns with congenital bilateral upper lid eversion evolving with good outcome using conservative treatment. The current literature including epidemiology, clinical characteristics, possible etiologic factors, and treatment was reviewed.

DIFFUSE RETINAL VASCULAR LEAKAGE AND CONE-ROD DYSTROPHY IN A FAMILY WITH THE HOMOZYGOUS MISSENSE C.1429G>A (P.GLY477ARG) MUTATION IN CRB1.

PURPOSE: To describe a specific cone-rod dystrophy phenotype in a family with the homozygous c.1429G>A; p.Gly477Arg mutation in CRB1. The detailed phenotype of subjects with this specific mutation has not been described previously. METHODS: Clinical examination included full-field electroretinography and high-resolution and widefield retinal imaging and uveitis workup. Molecular genetic analysis included next-generation sequencing of known retinal dystrophy genes and Sanger sequencing for segregation analysis. RESULTS: Three affected male siblings (26, 16, and 8 years old) were diagnosed with cone-rod dystrophy, featuring bilateral macular hypoautofluorescent lesions. In addition, the eldest brother was found to have retinal vascular leakage throughout the retina without telangiectasia. Uveitis laboratory workup was unremarkable. The homozygous c.1429G>A; p.Gly477Arg mutation in CRB1 was found to segregate with disease in this family. CONCLUSION: To the best of our knowledge, diffuse vascular leakage without telangiectasia or exudation, with bull's eye maculopathy, has not been reported previously in CRB1-cone rod dystrophy. This expands the phenotype complexity associated with CRB1 mutations and confirms that dystrophies associated with mutations in this gene may appear with features of uveitis.