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.

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.

Optic Nerve Avulsion: Pattern and Etiologies at a Tertiary Eye Care Center in Saudi Arabia: An 8-Year Retrospective Study.

Purpose: Optic nerve avulsion (ONA) is a rare but serious presentation of ocular trauma. This study investigates the presenting characteristics and etiologies of all cases of ONA over an 8-year-period at a tertiary eye care center in the Middle East. Methods: The medical records of patients diagnosed with ONA at an Ophthalmic Emergency Department between November 2014 and November 2022 were analyzed in this retrospective cohort study. Data were collected on patient age, sex, affected eye, cause of injury and imaging studies. The best-corrected visual acuity (BCVA) at presentation and at the last follow-up visit, and the duration of follow-up were documented. Results: The study sample was comprised of 44 eyes of 43 patients with ONA with median age of 16.5 (9.3-26.8) years ranging from 2 years old to 70 years old. There were (35;79.5%) males and (9; 20.5%) females. Most cases presented with an affected left eye (27; 61.4%) followed by the right eye (16; 36.4%) and only one patient (2.3%) had bilateral ONA. The most common cause of trauma resulting in ONA was a metallic object (8;18.2%). This study demonstrates the value of multi-sequence Magnetic resonance imaging (MRI) in the setting of unexplained vision loss when other modalities are inadequate or inconclusive. Conclusion: Ophthalmic morbidity resulting from ONA can be devastating. Metallic object injuries were the most prevalent cause of ONA. The presence of associated media opacities challenges the initial diagnosis of ONA. In the vast majority of cases, the vision ended as no light perception (NLP), indicating permanent vision impairment.

Acute unilateral vision loss and bilateral cerebral infarction following cosmetic filler injection.

The current case report describes acute unilateral vision loss and bilateral cerebral infarction as devastating complications following cosmetic injection of hyaluronic acid to the nasal bridge in a 34-year-old female. The patient reported the onset of sudden eye pain, headache, and vision loss in her right eye. At the initial visit to the hospital, the patient had no light perception in the right eye. Examinations revealed marked ptosis and complete ophthalmoplegia with no proptosis. At the time of the patient's hospital evaluation, computed tomography, and magnetic resonance imaging were ordered. Few mottled materials (denser than blood) were observed in the cavernous sinus with a density of 106 Hounsfield units (HU), yet no air was depicted in the cavernous sinus. MRI scan revealed multiple areas of bilateral intracranial infarcts. Although cosmetic injection of hyaluronic acid has been documented as a safe procedure, vision loss remains one of the most feared complications.

Olfactory Neuroblastoma: An Unusual Ocular Presentation.

We report a case of 51-year-old medically healthy male who presented with gradual painless diminution of vision in both eyes for 3 months. His visual acuity was hand movement perception in the right eye, and no light perception in the left eye. The intraocular pressure, external eye examination, ocular motility and anterior segment examinations were normal. Fundus examination revealed severe bilateral papilloedema and engorged tortuous veins in both eyes. Imaging exhibited a large intracranial tumour causing raised intracranial pressure. This was debulked by neurosurgery and histological examination revealed that the tumour was an olfactory neuroblastoma (ONB). This case is noteworthy since ONBs rarely present with isolated papilloedema without any accompanying ophthalmoplegia, proptosis, epistaxis, nasal, or neurological symptoms.

Acute dacryocystitis retention: a case report and literature review / Dacriocistite aguda de retenção: relato de caso e revisão da literatura

ABSTRACT Acute dacryocystitis retention (ADR) is an unusual entity that contributes to an incorrect diagnosis and treatment. We describe a case of acute dacryocystitis retention occurring in a 61-year-old diabetic male who presented with severe pain, swelling, and inflammatory signs above the left medial canthal ligament tendon. He had no previous history of epiphora. Computed tomography scan indicated acute dacryocystitis. Clinical treatment resulted in complete resolution of the condition. Syringing one month after the acute episode indicated a patent lacrimal excretory system. The temporary obstruction that evolved to an acute dacryocystitis retention was probably secondary to nasal alteration or supposed dacryoliths. Timely, conservative clinical treatment can lead to complete resolution of acute dacryocystitis retention with no further treatments.

RESUMO A dacriocistite aguda de retenção é uma entidade incomum, o que contribui para que o diagnóstico e o tratamento não sejam corretos. Estamos descrevendo um caso de dacriocistite aguda de retenção ocorrendo em um homem diabético de 61 anos que apresentou dor intensa, edema e sinais inflamatórios acima do tendão cantal medial esquerdo. Ele não tinha histórico anterior de epífora. A tomografia computadorizada indicou dacriocistite aguda. O tratamento clínico resultou na resolução completa da condição. A irrigação, um mês após o episódio agudo, indicou sistema excretor lacrimal pérvio. A obstrução temporária que evoluiu para uma dacriocistite aguda de retenção foi provavelmente secundária a alteração nasal ou supostos dacriolitos. O tratamento clínico conservador pode levar à resolução completa da dacriocistite aguda de retenção, sem necessidade de outros tratamentos.

Recurrent Oncocytoma of the Lacrimal Sac.

Oncocytoma of the lacrimal sac is an extremely rare tumor. In this report, we present the case of an 82-year-old woman who presented with swelling in the region of the lacrimal sac. Systemic examination and ophthalmic examination of both eyes were unremarkable. Computed tomography of the brain and orbits revealed a mass lesion involving the right lacrimal sac with expansion of the related nasolacrimal duct. Neither bone destruction nor tissue invasion was observed. Right external dacryocystectomy and debulking of the tumor were performed. Histopathological examination of the surgical specimen showed oncocytic cells arranged in an adenomatous fashion, and a diagnosis of benign oncocytoma was made. Three years later, the same patient presented with a similar complaint that was pathologically proven to be a recurrent benign oncocytoma of the lacrimal sac.

Heavy eye syndrome in a myopic patient with prior scleral buckle surgery for rhegmatogenous retinal detachment: Differential diagnosis and management.

PURPOSE: To present a challenging case of heavy eye syndrome (HES) in a 56-year-old female who previously underwent scleral buckle surgery in both eyes. OBSERVATIONS: Ophthalmic tests indicated a diagnosis of HES, confirmed using pre and postoperative magnetic resonance imaging (MRI). A silicone band loop myopexy was performed, successfully improving large angle esotropia at primary position and motility. CONCLUSIONS AND IMPORTANCE: MRI is essential to correctly identify HES, allowing a tailored surgical intervention that may lead to better outcomes for patients. Up to our knowledge, this is the first reported case of scleral fixated silicone band loop myopexy for HES in a previously scleral buckled patient.

Orbital and periorbital migration of silicone oil associated with emphysema development after retinal detachment repair - Case report and literature review.

PURPOSE: To report a very rare case of silicone oil (SO) migration and emphysema development in the orbit and periorbital tissue, including the lids and subconjunctival space, after a fourth pars plana vitrectomy (PPV) for retinal detachment (RD) treatment. OBSERVATIONS: A 53-year-old woman with a recurrent rhegmatogenous RD in the right eye underwent a fourth PPV under local anesthesia and 23-gauge vitrectomy with fluid-air exchange and SO injection. Localized choroidal detachment occurred during fluid-air exchange near the end of the surgery. High-pressure infusion of air was used as a temporary control measure prior to SO injection. In the early postoperative period, the patient developed hemifacial and periorbital swelling and the air trapped in the upper lid was associated with lid ptosis and conjunctival chemosis. The emphysema resolved with clinical management, and the mechanical ptosis subsided after partial SO removal from the lid. CONCLUSIONS AND IMPORTANCE: The SO migration and emphysema in our case were presumably related to the multiple previous sclerotomies. Periorbital emphysema can show spontaneous resolution, but the migrated SO requires surgical management.

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.

Acute dacryocystitis retention: a case report and literature review.

Acute dacryocystitis retention (ADR) is an unusual entity that contributes to an incorrect diagnosis and treatment. We describe a case of acute dacryocystitis retention occurring in a 61-year-old diabetic male who presented with severe pain, swelling, and inflammatory signs above the left medial canthal ligament tendon. He had no previous history of epiphora. Computed tomography scan indicated acute dacryocystitis. Clinical treatment resulted in complete resolution of the condition. Syringing one month after the acute episode indicated a patent lacrimal excretory system. The temporary obstruction that evolved to an acute dacryocystitis retention was probably secondary to nasal alteration or supposed dacryoliths. Timely, conservative clinical treatment can lead to complete resolution of acute dacryocystitis retention with no further treatments.

Outcomes of the use of orbital hydrogel expanders in the management of congenital anophthalmia: CT-based orbital parameter analysis.

PURPOSE: To evaluate the outcomes of orbital hydrogel expanders in the management of congenital anophthalmia. METHODS: In this retrospective one-armed cohort study, a chart review was performed of eight children with congenital anophthalmia who underwent orbital expansion using orbital hydrogel tissue expander from January 2006 to July 2018. Computed tomography (CT) of orbital parameters was evaluated before and after surgery. Changes in the orbital parameters were correlated with clinical factors. RESULTS: The study sample comprised 11 anophthalmic orbits of eight children (seven males, one female; median age = 12 months), with a median postoperative follow-up of 3.8 years. The anophthalmic orbital parameters after hydrogel expander implantation improved significantly compared to preoperative assessment as follows: mean orbital height improved from 21.7 mm to 25.4 mm (P < .001); width from 19.2 mm to 23.8 mm (P < .001); depth from 27.5 mm to 32.6 mm (P = .008); and volume from 3.7 cm3 to 5.3 cm3 (P = .001). Despite enlargement in all dimensions, the anophthalmic orbits with hydrogel expander had a significantly lower development than the normal orbits, mainly in height and volume. At the last postoperative visit, four (36.4%) cases had fornices deep enough to maintain the conformer. Migration and extrusion occurred in two (18.2%) cases. CONCLUSIONS: Orbital hydrogel expander can improve the orbital development in congenital anophthalmia. However, the enlargement is not as extensive as that observed in the normal orbit. Orbital expanders associated with external conformers were not sufficient to induce normal growth of lids and fornix.

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.

A Case of Escherichia coli Endogenous Panophthalmitis and Orbital Cellulitis With Normal Workup for Primary Focus.

We describe a case of a poorly controlled diabetic patient with left endogenous panophthalmitis with orbital cellulitis and positive ocular culture of Escherichia coli with negative systemic workup and rare clinical presentation. Was misdiagnosed and mismanaged as acute angle-closure glaucoma. Despite medical treatment with intravenous antibiotics, the patient required evisceration of the left eye as a result of the delay in diagnosis and treatment. A high index of suspicion for endogenous endophthalmitis and awareness of the proper workup and different clinical presentations is needed to avoid vision and life-threatening consequences.

Renal abscesses and endogenous endophthalmitis due to hypermucoviscous hypervirulent Klebsiella pneumoniae (HVKP).

We describe a diabetic patient with left eye endogenous endophthalmitis due to hypervirulent hypermucoviscous Klebsiella pneumoniae (HKVP) originating from right renal abscesses. A rare source of HVKP causing endogenous endophthalmitis. Despite treatment with intravenous ceftazidime and pars plana vitrectomy, the patient required evisceration of the left eye. A high index of suspicion for endogenous endophthalmitis and awareness of the virulence and potential antibiotic resistance of HVKP strains in the community is needed to avoid vision and life-threatening consequences.

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.

Eyelid sebaceous gland carcinoma: An assessment of the T classification of the American Joint Committee of Cancer TNM staging system 8th versus 7th edition.

PURPOSE: To assess the prognostic values of the T classification of the 8th edition of the American Joint Committee of Cancer staging system and compare it to the 7th edition. METHODS: Multicenter retrospective study of patients with eyelid sebaceous gland carcinoma. The primary outcome measure was the differences between outcomes when tumors were staged with either 7th or 8th edition. The measures evaluated included presenting features, management, histopathology, metastasis, recurrence, and mortality. RESULTS: Of the 60 patients (median age 73 years), 31 (51.7%) were females. A change in T staging occurred in 39 patients (65%) when the 8th edition was applied. Advanced categories (T3/T4) were significantly associated with nodal metastasis (p = 0.037) using the 8th edition criteria but not with the 7th edition (p = 0.066). The 8th edition T categorization significantly correlated with eye survival (p = 0.022) while the 7th edition did not (p = 0.058). Applying the 8th edition, category T4 at presentation was associated with a higher risk of nodal metastasis (p = 0.037) but not associated with local recurrence, distant metastasis, or tumor-related death (p = 0.281, p = 0.737, p = 0.319, respectively). T3/T4 category tumors were significantly associated with poor tumor differentiation (p = 0.001), and papillary histologic pattern (p = 0.024) but not with pagetoid spread (p = 0.056). CONCLUSION: The application of the 8th edition AJCC staging system for eyelid SGC may accurately predict nodal metastasis. Local recurrence and distant metastasis were not significantly associated with T classification, using either edition. Poor tumor differentiation and papillary pattern were associated with T3/T4 categories suggesting that pathological features may assist in determining prognosis.

Involvement of Multiple Trigeminal Nerve Branches in IgG4-Related Orbital Disease.

PURPOSE: To describe the occurrence of multiple trigeminal nerves (TGNs) enlargement in patients with orbital IgG4-related disease. METHODS: Retrospective review of MRI findings and medical records of 6 patients (10 orbits) with orbital IgG4-related disease and enlargement of more than 1 TGN. Orbital biopsies were performed in all cases revealing the typical lymphoplasmacytic infiltrate with significant plasma cell positivity for IgG4 (IgG4+/IgG ratio ≥ 40%). Three experienced neuroradiologists reviewed the MRI sequences using a digital imaging viewer system (Horos, https://horosproject.org/). RESULTS: Bilateral involvement of at least 2 TGNs divisions was detected in all 6 patients. Enlargement of both V1 and V2 nerves was diagnosed in 5 patients, and in 3 cases, all TGN divisions were involved. V2 nerves were the most affected. In this division, all 12 infraorbital nerves were enlarged, followed by lesser palatines (10/83.3%), superior alveolar (10/83.3%), and zygomatic (6/50%). V1 and V3 nerves were less affected albeit 9 (75%) frontal branches (V1), and 50% of the inferior alveolar (V3) nerves were also enlarged. CONCLUSIONS: Widespread involvement of the TGN is an important feature of IgG4-related disease.

Histopathological assessment of optic nerve invasion guided by radiological findings in enucleated globes with retinoblastoma.

BACKGROUND: Optic nerve (ON) invasion is an important high-risk feature, and an indicator for neoadjuvant chemotherapy and prognosis. We aim through this study to correlate the detected-ON invasion by Magnetic resonance imaging (MRI) with the corresponding confirmed histopathological level of invasion. METHODS: A retrospective study of enucleated globes with the diagnosis of retinoblastoma received in the histopathology department(s) from January 2015 to December 2016 (2 years). Slides were reviewed for ON invasion assessment, charts were reviewed for basic demographic data. All patients underwent MRI under sedation upon diagnosis and MRI findings were collected for the above correlation. RESULTS: A total of 38 patients were included: 21 males and 17 females. 29 (77.3%) had unilateral involvement, 7 (18.4%) had bilateral involvement and 2 cases had trilateral disease. The overall mean age at diagnosis was 22.63 ± 15.15 months. Histopathological examination revealed ON invasion in 28 cases (74%) distributed as follows: prelaminar (31.6%), laminar (18.4%), and post-laminar (23.7%). MRI confirmed post-laminar ON invasion in 8 cases (true positive) but failed to detect this in 1 case. Additionally, MRI detected another 8 cases of ON invasion that were false positive on histopathology (accuracy: 63.3%; sensitivity: 88.9%; specificity: 72.4%; Positive predictive value (PPV): 50%; Negative predictive value (NPV): 95.5%). CONCLUSIONS: MRI is found to be less sensitive in evaluating prelaminar and laminar ON invasion (0.0 and 42.9%) compared to post-laminar invasion (88.9%). MRI has generally better specificity in detecting ON invasion irrespective of the invasion level. In our study, obtaining deeper and/or additional histologic sections from the other surface of the tissue block in cases where a post-laminar ON invasion by MRI is found but not confirmed histopathologically in routine sections is essential to avoid missing such an important high-risk feature.

Orbital solitary fibrous tumors: a multi-centered histopathological and immunohistochemical analysis with radiological description.

BACKGROUND: Solitary fibrous tumors (SFT), formerly called hemangiopericytoma, are rare tumors derived from mesenchymal cells originally described in the pleura, but these tumors may affect extraserosal tissues including the lacrimal gland and orbit. OBJECTIVE: Conduct a multi-centered clinical, radiological and histopathological analysis of 17 orbital SFT cases. DESIGN: A retrospective case series. SETTING: Three eye centers in two countries. PATIENTS AND METHODS: The data collected from the charts of 17 adult patients presenting with tissue diagnosis of orbital hemangiopericytoma or SFT from January 2003 to December 2018 included demographics, clinical imaging and histopathological information including immunohistochemical (IHC) characteristics. MAIN OUTCOME MEASURES: The demographic characteristics, clinical presentation, and histopathological patterns or variants of SFT were analyzed. SAMPLE SIZE: 17 adult patients. RESULTS: Mean age was 45 years (range 23-80 years). Male to female ratio was 3:1. The right eye was affected in 12 (70.5%) patients. Commonest presentation was proptosis in 13/17 (76% of patients). Other symptoms were impaired motility (29%) and ptosis (11%). Lesions mostly affected the medial orbit (35%), then orbital apex in 11%. The histopathological classic pattern-less variant was the commonest. One case with aggressive behavior, multiple recurrences and atypical features was encountered. Immunohistochemical (IHC) markers used included CD34 expression in all cases, Bcl-2 expression in 10/11, CD99 in 9/9 and Vimentin in 4/4. STAT6 was used in 2 cases. CONCLUSIONS: SFTs are rare tumors affecting the orbit in both genders equally in their mid-forties, but showed male predominance in our analysis with a predominant classic histopathological pattern. Tissue diagnosis is essential and requires IHC studies for confirmation. LIMITATIONS: Sample size is relatively small owing to the rarity of this tumor in the orbit. CONFLICT OF INTEREST: None.