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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.
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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.
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AIM: To determine whether the levator palpebrae superioris (LPS)/superior rectus (SR) muscle complex, can influence the position of the upper lid and fornix in acquired anophthalmic sockets. METHODS: This comparative non-randomized and non-interventional study included retrospective data of 21 patients with unilateral acquired anophthalmic sockets repaired with spheric implants. High-resolution computed tomography (CT) measurements of the LPM/SR muscle complex and clinical topographic position of the upper lid, superior and inferior fornix depth in primary gaze position were evaluated. Demographic data were presented as frequency and percentage proportions and quantitative variables comparing the socket measurements with the normal contralateral orbit was statistically analyzed using non-parametric tests considering P<0.05. RESULTS: The anophthalmic orbits had a significantly shorter LPS length (P=0.01) and significantly thicker SR (P=0.02) than the normal orbit. Lagophthalmos was present in anophthalmic sockets but not in normal orbits (P=0.002), while levator function was normal in both (P>0.05, all comparisons). The superior fornix depth was similar in the anophthalmic socket and the contralateral normal orbit (P=0.192) as well the inferior fornix depth (P=0.351). CONCLUSION: Acquired anophthalmic sockets repaired with spheric implants have shorter LPS, thicker SR, and more lagophthalmos than normal orbits. The relationship of the LPS and SR with other orbital structures, associated with passive or active forces acting in the final position of the lids and external ocular prosthesis should be further investigated.
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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.