Ophthalmologic manifestations of organoid nevus syndrome: A series of 13 cases.

Background: The organoid nevus syndrome is a rare neurocutaneous syndrome typified by cutaneous sebaceous nevus, seizures and epibulbar choristomas. The condition is associated with multiple ocular abnormalities. Herein, the authors aim to study and report the ophthalmic features of cases diagnosed with organoid nevus syndrome. Methods: The authors retrospectively evaluated the records of patients with the organoid nevus syndrome who had presented to a tertiary care eye hospital in northern India. The ocular features were studied and entered in MS excel and the data were evaluated. Results: Data of 13 patients with the organoid nevus syndrome were found. All 13 patients had cutaneous features in the form of Sebaceous nevus of Jadasson, 8 patients had alopecia of the scalp area, 2 had history seizures and 10 had arachnoid cysts on neuroimaging of the head. All 13 patients had a complex choristoma involving the ocular surface. Conclusions: We conclude that the most common ophthalmologic features associated with organoid nevus are complex choristoma of the bulbar surface, scleral coat calcification and upper eyelid coloboma.

Disinsert, retract and rotate technique of plaque brachytherapy.

BACKGROUND: Plaque brachytherapy is commonly used in the management of choroidal melanomas. The surgical steps usually involve creating a conjunctival peritomy, fixing the recti muscles, with or without disinserting them based on the location of the lesion, and placing the plaque. The inferior oblique muscle is attached close to the macula, and in cases of perimacular or peripapillary lesions, the muscle needs to be sacrificed. PURPOSE: The authors here demonstrate a novel technique of placing radioactive plaque without disinserting the inferior oblique muscle in cases of perimacular or peripapillary choroidal melanomas. SYNOPSIS: The video demonstrates how the "disinsert, retract, and rotate technique" of brachytherapy plaque placement can be performed and what are the fundamentals behind this technique. The authors have performed this procedure multiple times and there has been no incidence of plaque tilt or migration. HIGHLIGHTS: In perimacular and peripapillary choroidal melanoma brachytherapy plaque placement, the inferior oblique muscle can be spared. The simple technique does not lead to any tilt or migration of the radioactive plaque. VIDEO LINK: https://youtu.be/YMIg3rYyp2o.

Small cell neuroendocrine carcinoma with primary orbital involvement - The unseen, and a review of literature.

Neuroendocrine neoplasms are derived from the epithelial lineages mainly of respiratory tract, with predominant neuroendocrine differentiation. There are only a handful of documented cases of paranasal small cell neuroendocrine carcinomas (SNEC) with primary orbital involvement. Here, the authors describe a 33-year-old male patient with rapidly progressive swelling of the right lower lid with proptosis since 4 weeks. On contrast-MRI orbit, an ill-defined multilobulated mass measuring 3.6 × 3.1 cm with intense homogenous enhancement was seen in the right retrobulbar space involving the right ethmoid sinus. On incisional biopsy, a poorly differentiated mass containing numerous small round blue cells and scanty intervening stroma with prominent necrosis and apoptosis was seen. Immunohistochemistry was strongly positive for synaptophysin. He was diagnosed as a case of SNEC and received chemotherapy, with good response till date of 9 months of follow up. The authors present a literature review and describe challenges in management of a primary orbital SNEC.

Tessier cranio-facial clefts presenting to a tertiary eye care center in Northern India: Ophthalmic features and a review of management.

Purpose: Tessier classification is used to classify congenital facial cleft disorders utilizing the anatomical location of the cleft and its extension. The orbital and ocular morbidities associated with the birth disorder are numerous. The authors decided to perform a retrospective analysis of the clinical features of the patients who presented to a tertiary care hospital with orbito-cranial clefts. Methods: The authors retrospectively evaluated the records of patients with craniofacial clefts who had presented to a tertiary eye care hospital in northern India in the last 2 years (January 2019-December 2020). The clinical features were studied, entered in MS Excel, and the data were evaluated. Results: The data of 40 patients with Tessier cleft were found. The majority of the patients were male and presented in the pediatric age group. Unilateral involvement was more common, with maxillary hypoplasia being the most common facial anomaly associated. Eyelid coloboma and euryblepharon was the most common periocular finding; lateral epibulbar dermoid and corneal opacity were the most common ocular surface anomaly. The majority of patients had presented for cosmetic correction. The syndromic association was with Goldenhar syndrome (n = 13), Fraser (n = 2), and one each of Treacher Collins, blepharocheilodontic, organoid nevus, and oculo-dento-digital syndrome. Combined clefts were also seen. Conclusion: Tessier cleft classification is a useful tool to classify cranio-facial left anomalies. Multitudes of ocular and orbital anomalies can be associated with their different forms. Better knowledge and understanding of the classification will aid immensely in predicting the ocular defects and planning their management.

Clinical features and orbital anomalies in Fraser syndrome and a review of management options.

Purpose: Fraser syndrome is a rare congenital disorder comprising cryptophthalmos, syndactyly, and many times, urogenital anomalies. Herein, the authors aimed to study and report the clinical features and orbital anomalies in cases diagnosed with Fraser syndrome. Methods: The authors retrospectively evaluated the records of patients with Fraser syndrome who had presented to a tertiary eye care hospital in northern India in the last 2 years (from January 2019 to December 2020). The clinical features were studied, entered in MS Excel, and the data was evaluated. Results: Data of 15 patients with Fraser syndrome were found. Majority of the patients were males and presented in the pediatric age group. Bilateral involvement was more common, and the most common variant of cryptophthalmos was abortive. Complete and medial madarosis of the eyebrows was the most common periocular finding. Complete cryptophthalmos was associated with cystic globes, whereas abortive forms had superior symblepharon. Common systemic features included syndactyly, bifid nose, and urogenital anomaly. Conclusion: Fraser syndrome is an extremely rare developmental disorder; it encompasses a wide range of ocular, periocular, and orbital anomalies, along with multiple pre-existing systemic anomalies. The treating ophthalmologist should always be careful in examining these patients.

Large orbital glial heterotopia- A rare entity.

A 15-month-old child was brought to the ophthalmology outpatient department with his parents complaining of a large mass located on the nasal side of his right eye since birth. On examination, an irregular mass was seen to be located on the nasal side of his right orbit, obscuring the visual axis. On a contrast-enhanced computed tomography scan, a heterogenous lesion was seen to be located on the bridge of the nose and the medial aspect of the right orbit without any bony defect or communication with the brain or sinuses. In view of the possibility of developing amblyopia, the lesion was surgically removed. On histopathological examination, a diagnosis of extranasal glial heterotopia was made. To the best of our knowledge, this is the largest extranasal glial heterotopia in the orbit to be ever reported.

Orbital Aspergillosis, a great masquerader: a series of three unusual cases and a review of the literature.

Fungal infections of the orbit are infrequent. They typically affect immunocompromised individuals but, in some instances, can also affect an immunocompetent person. They mimic much orbital pathology clinically. Ours is a series of three patients eventually diagnosed with orbital aspergillosis but who, initially, were thought to have some other pathology.

Opening a dacryocystorhinostomy into the opposite nasal cavity in a case of hemiarhinia.

Heminasal agenesis is an exceedingly rare congenital anomaly which is frequently associated with disorders of eye, lacrimal drainage system and face. At times, a proboscis may also be noted with this condition. Herein, we present a case of a 13-year-old boy who presented to us with heminasal agenesis with concomitant microphthalmos, leucomatous corneal opacity, blepharoptosis, dacryocystocoele and maxillary hypoplasia. The child underwent a modified transeptal external dacryocystorhinostomy with placement of a lacrimal stent. There was significant improvement of epiphora and discharged following surgery. Heminasal aplasia with dacryocystocoele is surgical challenge; however, a well thought out surgical approach can yield satisfactory outcomes.

Contralateral field defect in traumatic globe luxation with optic nerve injury.

CLINICAL RELEVANCE: Traumatic globe luxation is rare vision-threatening event. Besides causing loss of vision in traumatised eye, it may also cause injury to contralateral optic nerve. Any such limitation in a one-eyed patient can make him crippled in his day-to-day activities. Here we hypothesise cause of contralateral field defect in such patients. BACKGROUND: Traumatic globe luxation is a rare event that leads to profound vision loss due to injury of the ipsilateral optic nerve and rarely a visual field defect in the contralateral eye. Through this communication, we report similar case scenarios and intend to hypothesize the mechanism that results in the occurrence of the contralateral visual field defect. METHODS: It is a retrospective, observational study. All cases with traumatic globe luxation were enrolled. Visual field analysis of the contralateral normal eye was main outcome measure. RESULTS: Four patients with traumatic globe luxation and optic nerve injury were studied. There was complete loss of vision in the traumatised eye in all the patients. One patient had complete transection of the optic nerve, whereas in three patients, the course of the optic nerve was intact, on imaging. Three patients had quadrantanopia in the contralateral normal eye for which oral steroids were given. At 1-month follow-up, there was complete recovery of the visual field defect in two patients. We noticed that all the three patients with an intact course of the ipsilateral optic nerve had quadrantanopia in the contralateral normal eye. CONCLUSION: We hypothesize that in scenarios where globe luxation is associated with incomplete transection or no transection of the optic nerve, a continuous long standing stretch on the optic nerve, transmits the pulling force to the chiasma which might result in a contralateral field defect as compared to those associated with the complete transection of the optic nerve.

Clinical Role of Swept Source Anterior Segment Optical Coherence Tomography in Strabismus Re-Operation with Lost Surgical Details.

BACKGROUND: To characterize the diagnostic role of swept source anterior segment optical coherence tomography (SS-ASOCT) in strabismus re-operations with lost surgical details. METHODS: Patients presenting to our outpatient services for strabismus care from February 2020 to March 15th 2021 were systematically evaluated. Those willing to undergo re-operation were included in the study. A total of 30 patients were identified and out of this a total of 10 eyes of 10 patients with lost surgical details were finally included in the study. Demographic details, preoperative deviation, ocular findings, orthoptic measurements, detailed SS-ASOCT findings, intraoperative measurements, and post-operative outcomes were recorded. RESULTS: The mean age of 10 patients was 23.7±5.75 years. Seven were male and three were female. The mean pre-operative SS-ASOCT measured distance from angle to insertion was 8.90±1.85 mm and the mean intraoperative distance was 9.12±2.14 mm. A mean difference of 0.21±1.31 mm was noted, and this difference was statistically insignificant (paired t test, p = 0.57). In addition, SS-ASOCT unraveled novel signs to differentiate operated eyes from the un-operated eyes, and recessed muscle from the resected muscle. CONCLUSION: In strabismus re-operation with lost surgical details, the pre-operative SS-ASOCT can provide reliable muscle details with respect to its type of surgery and amount surgery.

Bilateral globe luxation with multiple facial fractures: what next?

Luxation of the globe is a rare, vision-threatening event that can presently spontaneously or following trauma. A 35-year-old man presented with bilaterally luxated globe, postroad traffic accident. On imaging, bilateral LeFort fracture type 1, 2 and 3 with palatal split along with nasal bone and orbital floor fracture. Both condyles and left parasymphysis of mandible were fractured. There was no evidence of intracranial injury. There was pneumothorax on the right side for which intercostal drainage (ICD) tube was placed. On exploring, bilateral optic nerve avulsion was present. Both the globes were repositioned and a temporary tarsorrhaphy was placed for cosmetic rehabilitation. On follow-up visits after 6 months, both eyes were in their sockets with minimal exodeviation. It is important to reposition the globes, even with extensive periorbital fracture at the earliest. The longer the globe and orbital structures are prolapsed, the poorer are the structural and cosmetic prognosis owing to ocular ischaemia.