Baseline and Post-NACT Imaging in Retinoblastoma With Optic Nerve Involvement: Can MRI Predict Prognosis?

PURPOSE: To describe a staging system for optic nerve invasion using magnetic resonance imaging (MRI) and report any correlation with survival outcome. METHODS: This was a ambispective study. Twenty-one patients with retinoblastoma who had optic nerve involvement on MRI were staged at baseline based on contrast enhancement and/or thickening and length of involvement. Response to neoadjuvant chemotherapy (NACT) was noted according to proposed response evaluation criteria and results were correlated with survival outcome. RESULTS: Baseline MRI staging was able to predict event-free survival (EFS) (P = .0015) using the log-rank test for trends. Patients with optic nerve enhancement alone showed 100% survival prognosis. Optic nerve thickening cases with complete or partial response to NACT showed better EFS (P > .90) than those with stable disease according to response evaluation criteria. CONCLUSIONS: The modified staging system for optic nerve invasion used in the current study significantly predicted EFS. The study also showed that response to NACT may be affected by baseline staging. The authors recommend that cases with optic nerve enhancement only, irrespective of the length of involvement (stage 0), may be treated with upfront enucleation. Cases with optic nerve thickening may be staged to evaluate the correlation with survival outcome in a larger cohort in future studies. [J Pediatr Ophthalmol Strabismus. 2024;61(2):98-105.].

Can Enhancement Pattern in Normal-Sized Optic Nerves on Magnetic Resonance Imaging Better Predict Tumor Invasion in Retinoblastoma Eyes?

Introduction: Optic nerve (ON) enhancement alone without ON thickening on contrast-enhanced magnetic resonance imaging (CE-MRI) can be associated with post-laminar optic nerve invasion (PLONI) in eyes with group E retinoblastoma. A few case reports and retrospective studies in the literature show a poor correlation between ON enhancement on MRI and ON invasion on histopathological examination (HPE). There is no universal consensus on the management of such cases. It is desirable that the presence and extent of a true ON invasion be reliably picked up before planning upfront enucleation in order to avoid stage II disease. Methods: In a prospective study conducted at a tertiary eye care center in North India, all retinoblastoma patients presenting with ON enhancement on imaging were evaluated. Demographic and imaging details, histopathological findings, and treatment details were recorded. The length and pattern of enhancement noted on MRI were correlated with histopathology. Follow-up was done till the end of the study period. Results: Six group E retinoblastoma eyes were evaluated. 3 eyes (50%) showed solid enhancement, 2 eyes (33.33%) had tram track pattern and 1 eye (16.66%) showed punctate enhancement pattern on CE-MRI. On histopathology, 5 (83.33%) cases showed PLONI and all 6 (100%) had ON head infiltration. The cut end of the ON was free in all cases. On correlating MRI and HPE, all eyes with solid enhancement pattern showed PLONI, of which 2/3 (66.6%) had diffuse ON infiltration. Only 50% of eyes with tram track patterns showed PLONI. The case which showed a punctate enhancement pattern showed focal infiltration by tumor cells with vacuolated cytoplasm on HPE. At the last follow-up, all patients were alive and free of disease. Conclusion: ON enhancement patterns may make it more predictive for PLONI on HPE. Solid enhancement pattern appears to correlate better with the extent of ON invasion on HPE, and longer lengths of solid ON enhancement may be considered for neoadjuvant chemotherapy rather than upfront enucleation.

Transconjunctival excision of external angular dermoid cyst: A novel approach.

PURPOSE: To excise external angular dermoid cyst (EADC) by transconjunctival approach and compare the surgical outcomes with the standard transcutaneous approach. DESIGN: This was a prospective, pilot, interventional, comparative study. METHODS: Patients with EADC with no or minimal fixity to the underlying bone on palpation and limited to eyelid were recruited. Patients were randomized into 2 groups; group 1 included patients with transcutaneous approach, and group 2 included patients with transconjunctival approach. The parameters assessed were intraoperative complications, duration and ease of surgery, postoperative complications, and overall satisfaction. RESULTS: Six children with a painless, round lesion in outer aspect of eyelid were recruited in each group. None of the patients had any intraoperative or postoperative complications, including dysfunction of eyelid contour and fold, persistence or late occurrence of lateral eyelid droop, excessive or recurrence of swelling, and ocular surface problems, especially in group 2, but a skin scar though hidden was inevitable in group 1. The duration of surgery was comparable with better ease of surgery in group 1 and a gradual learning curve in group 2. The overall satisfaction scores were significantly better in group 2 (p < 0.0001). In group 1, parents of 5 of 6 patients had to be reassured that the skin scar will fade with time. CONCLUSION: Transconjunctival excision of EADC is a viable and novel approach in patients with mobile cyst that is limited to the eyelid with no obvious bony fossa. Main limitations of the approach are that it requires surgical expertize, provides less surgical space, and has a gradual learning curve.

Autologous fat transfer for orbital volume augmentation in sockets with small nonseeing eyes.

OBJECTIVE: End-stage ocular diseases usually end up with atrophic bulbi or phthisis bulbi leading to orbital volume loss which needs to be addressed. We studied the use of autologous fat for volume augmentation of the orbit because it is minimally invasive and allows early rehabilitation with the use of an artificial eye. DESIGN: It was a prospective, interventional study. METHODS: A total of 14 eyes of 14 patients with atrophic bulbi with shrinkage or phthisis bulbi with no perception of light (PL) and aged older than 18 years were included for the study purpose. Painful or inflamed eyes or suspected intraocular tumors were excluded. An autologous fat graft was obtained from the lower abdomen or buttocks and injected into the retrobulbar space using a 20-gauge cannula after adequate peribulbar anesthesia. Outcomes measures were patient satisfaction, change in Hertel's exophthalmometry, vertical and horizontal palpebral aperture, and socket volume. RESULTS: Hertel exophthalmometry showed a significant improvement both with and without an artificial eye from 14.92 ± 2.3 mm to 16.71 ± 1.94 mm (p-value 0.003 without an artificial eye). The vertical palpebral aperture also showed a significant improvement from 5 ± 1.70 mm to 6.71 ± 1.58 mm (p-value < 0.001). There was a significant reduction in the socket volume from 1.22 ml to 0.39 ml (p-value < 0.001). No local or donor site complications were seen. CONCLUSIONS: Autologous fat transfer is a minimally invasive, safe, and effective procedure for orbital volume augmentation in small, nonseeing eyes. The short-term outcome of our study was good in most patients and can be considered for such patients.

Congenital Common Canalicular Lacrimal Fistula in an Asymptomatic Patient.

This case report discusses a diagnosis of congenital common canalicular lacrimal fistula in a 24-year-old asymptomatic patient.

Correlation of Levator-Muller's Complex Thickness on Ultrasound Biomicroscopy to Botulinum Toxin A Injection in Thyroid-Related Upper Eyelid Retraction.

OBJECTIVES: To study the thickness of levator palpebra superioris-Muller's muscle complex (LMC) on ultrasound biomicroscopy (UBM) and to correlate with the clinical response to botulinum toxin A (BTA) injection in patients with inactive-stage of thyroid-related upper eyelid retraction (UER). We also studied the correlation of clinical parameters, preinjection with postinjection values. METHODS: This was a prospective, interventional study. Patients with thyroid-related UER who underwent subconjunctival injection of BTA were recruited. Demographic data and clinical details were evaluated. UBM (50 MHz) was done to measure the thickness of LMC. Patient's satisfaction was graded at each follow-up. Follow-up was done at 1 week, 1 month, and 3 months' time intervals. RESULTS: A total of 13 patients were recruited and 26 eyes were divided into two groups; group 1 included eyes with UER (n = 17), and group 2 included eyes without UER (n = 9). There was a statistically significant reduction in margin reflex distance 1 (MRD1) after BTA injection at 1-week, 1-month, and 3-months follow-up with maximum reduction at 1 month. The mean LMC thickness of 26 eyes was 0.96 mm which was found to be significantly more than normal controls. On comparison of mean LMC thickness with the amount of UER and reduction in MRD1, we did not find a significant difference. CONCLUSIONS: Patients with TED have significantly thicker LMC on UBM than controls. Further studies are needed with a larger sample size on the correlation of UBM features of levator aponeurosis with response to BTA injection.

Eyelid sebaceous gland carcinoma, validation of AJCC 8th edition T staging- a retrospective study from North India.

PURPOSE: This study compares the 8th edition staging of AJCC for prognosis of eyelid Sebaceous Gland Carcinoma (SGC) patients with respect to the 7th edition. METHODS: A retrospective study was undertaken over a period of 100 months. Ninety-nine histopathologically proven cases of eyelid SGC available for follow-up were recruited. Patients were staged by both the 7th and 8th editions of AJCC and followed up at six monthly intervals after surgery. RESULTS: Of the 99 eyelid SGC patients recruited, recurrence and orbital invasion developed in 22%, lymph node metastasis in 21% and 4% had distant metastasis and also died. High-risk features include tumour size>20 mm, orbital invasion, exenteration and staging by both the 7th and 8th editions of AJCC. Cox regression analysis revealed that staging by AJCC 8th edition was associated with greater risk for local recurrence (HR 3.01,95% CI-1.65-5.51%, p < 0.01) lymph node metastasis (HR 8.26, 95% CI 3.96-17.19%, p < 0.01) and disease-free survival (HR 4.61, 95% CI 2.81-7.54). The Kaplan-Meir survival curves of eyelid SGC patients by the 8th edition AJCC staging were also significantly associated with lymph node metastasis (p < 0.01), tumour-related deaths (p < 0.01) and reduced disease-free survival (p = 0.07). The higher Harrell's values by the 8th edition signify better predictive value for lymph node metastasis and DFS (disease-free survival). The lower AIC values indicate a better monotonicity of gradients for lymph node metastasis, recurrence and DFS. CONCLUSION: Staging by the 8th AJCC edition is, therefore, recommended for eyelid SGC as it gives a better perspective about disease outcome. The orbital extension was the single most important predictor of lymph node metastasis, recurrence and death.

Psychosocial factors among patients undergoing orbital exenteration.

CLINICAL RELEVANCE: Patients undergoing exenteration have significant anxiety and depression, and lower quality of life that worsens after surgery. Therefore, counselling by an eye health-care provider along with the team of psychologist and psychiatrist could be provided pre-operatively to assess baseline psychosocial status and be continued post-operatively to look for any deterioration. BACKGROUND: Orbital exenteration comes with psychological trauma of disfigurement of face. The study aimed to evaluate the quality of life, anxiety and depression in patients undergoing orbital exenteration, before and after the procedure; and also compared the same with patients previously rehabilitated by spectacle-mounted prosthesis after exenteration. METHODS: It was a prospective cohort study. Group I included patients who were planned to undergo orbital exenteration, and group II were those already using spectacle-mounted prosthesis post orbital exenteration for at least 3 months. Level of depression was assessed using Patient Health Questionnaire-9(PHQ-9), anxiety using Generalised Anxiety and Depression scale-7(GAD-7), and quality of life was assessed using Functional Assessment of Cancer Therapy- General (FACT-G) score. RESULTS: A total of 19 patients were recruited in group I and 10 patients in group II. The baseline demographic factors were comparable. The scores for PHQ-9 and GAD-7 were significantly higher at 3-month post-exenteration follow-up, indicating significantly higher levels of anxiety and depression after orbital exenteration in group I. Both these scores were significantly lower in group II compared to group I (3 months follow-up), indicating lower levels of anxiety and depression after rehabilitation with prosthesis. The FACT-G and its subscores were all significantly higher in group II patients compared to post-operative scores of group I patients, indicating higher overall quality of life after rehabilitation with prosthesis. CONCLUSIONS: Patients undergoing exenteration have a significant level of anxiety and depression, and lower quality of life that worsens after orbital exenteration.

Autologous fat grafting for the correction of cicatricial ectropion.

BACKGROUND: Cicatricial ectropion can be corrected by invasive surgical modalities such as full-thickness skin graft, lateral tarsal strip procedure, etc., which are associated with donor site morbidity and additional scars. Use of autologous fat graft (AFG) in treatment of burn scars has previously shown encouraging clinical results as to skin color, texture, and softness. OBJECTIVE: We aim to introduce the use of AFG for the correction of mild to moderate grades of cicatricial ectropion of the lower eyelid in adult patients. METHODS: This was a prospective, interventional, non-comparative, pilot study including 5 patients with a cicatricial ectropion of grade I-III. All these patients underwent a pre-injection workup to assess anatomical outcomes (grade of ectropion, vertical palpebral aperture (VPA), lagophthalmos, and eyelid retraction), functional outcomes (dry eye status) and aesthetic outcome (change of scar color, thickness, pain, and consistency). RESULTS: At 6-months follow-up assessment, all patients had improvement in grade of ectropion, significant improvement in VPA and eyelid retraction, and almost complete resolution of lagophthalmos in all the three patients who had pre-injection lagophthalmos. There was a significant improvement in scar color, thickness and consistency on Visual Analogue Score (VAS) with no donor site or systemic complications after the procedure but mild pain on day 1 post-injection at the donor site CONCLUSION: AFG transfer of cicatricial ectropion not only eludes the need of a invasive surgery but provides satisfactory correction with stable outcome with an overall improvement in texture of the scar and has low risk.

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.

Experience on rhino-orbital mucormycosis from a tertiary care hospital in the first wave of COVID-19: An Indian perspective.

Background: To highlight the clinical presentations and management outcomes of rhino-orbital mucormycosis during first wave of COVID-19 pandemic in North India. Methods: A retrospective observational study. 15 patients with mucormycosis (orbital disease) who presented during short span of 3 months (October-December 2020) in a tertiary-care referral institution were analysed. Results: At presentation, 13 of 15 patients had uncontrolled diabetes. Four had history of COVID-19 infection. All patients had advanced orbital disease with sinusitis; cavernous sinus involvement was in nine and intracranial spread in three patients. Liposomal amphotericin-B was started and prompt orbital exenteration with sinus surgery was performed in 12 patients. All 12 patients survived with an average follow-up of 4.8 months. Conclusion: In the present series, cases with orbital spread of mucormycosis were mostly found in non-COVID uncontrolled diabetics. Exenteration was done in 80% of cases with advanced orbital disease. Prevention and early detection of infection at the stage of sino-nasal involvement might help to prevent spread and/or halt the orbital disease.

Cysticercosis in ophthalmology.

Cysticercosis is caused by Taenia solium, a cestode or tapeworm that preferentially affects the subcutaneous tissue, brain, muscle, and the eye. It is traditionally a disease of low socioeconomic regions, but large-scale population migration has made it a matter of global concern. Its ocular invasion is a potentially blinding disease. In the last two decades, there has been considerable discussion of cysticercosis; however, most comes from a limited number of case observations. Thus, to overcome this limitation, we summarize and analyze twenty years of medical literature (from 2000 to 2020) on cysticercosis in ophthalmology.

Clinical Significance of Cyclin Expression Profiling in Ocular Surface Squamous Neoplasia.

Ocular surface squamous neoplasia (OSSN) can recur, metastasize, and even cause death. Cyclins regulate the cell cycle progression at different phases and its dysregulation is associated with uncontrollable cell growth and malignant transformation of the cell. Overexpression of cyclin has been reported in various malignancies and is associated with poor prognosis. However, the role of cyclins in OSSN remains unexplored. This study has been designed to assess the prognostic significance of cyclin (cyclin B1, E1, and D1) immunoexpression in 100 OSSN patients. The targeted proteins demonstrated overexpression of cyclin B1, cyclin E1, and cyclin D1 in 55%, 37%, and 56% OSSN cases prospectively. A gradual and significant increase in the cyclin B1 (P=0.01) and cyclin D1 (P=0.005) expression was seen from Tis to the T4 category. Overexpression of cyclin B1 was associated with poor disease-free survival and worst prognosis in both early (P=0.03) as well as advanced T staged (P=0.038) OSSN patients. Overexpression of cyclin E1 was associated with worst disease-free survival (P=0.01) and poor prognosis in advanced stage OSSN patients. Our findings suggest that cyclin B1 and cyclin E1 have prognostic relevance in OSSN patients, and therefore are recommended for detecting high-risk category cases. A significant increase in the expression of cyclins from early to advanced stage indicates that cyclins play an important role in the pathogenesis of OSSN patients.

Recurrent Ptosis in a Case of Dubowitz Syndrome.

Dubowitz syndrome is a relatively rare genetic and developmental disorder. An eight-year-old female presented with a complaint of drooping in her left eye since birth. She had undergone ptosis surgery two years back. There was a history of delayed speech and delayed dentition. She was of moderate built appropriate to her age. There was microcephaly, sparse hair, flat bridge of the nose with a prominent rounded tip, short stature, low-set ears, and micrognathia with subsequent protrusion of upper two incisors. Based on the clinical features a diagnosis of Dubowitz syndrome with left recurrent ptosis was made. She underwent frontalis sling surgery and had a satisfactory outcome.

Eyelid Dirofilaria During COVID-19 Pandemic: A Telemedicine Diagnosis.

Telemedicine is an important tool improving the delivery of health care services for diagnosis, treatment and prevention of diseases. A 22-year-old female consulted over video teleconsultation for a swelling in the left upper eyelid, which also moved at certain times for eight days. The video revealed a vermiform swelling on her left upper eyelid with occasional movements. A provisional diagnosis of subcutaneous eyelid Dirofilaria infection was made, and with the help of a surgeon practising locally, it was removed in total. The worm was 40 mm in length and had a slender white body, identified as a sexually immature female Dirofilaria repens. Herein, we share this exciting experience of diagnosing subcutaneous eyelid dirofilariasis at a telemedicine video conference.

An Eye Capturing Clutch - An Orbital Foreign Body.

Foreign bodies inside the orbital cavity are rare. They may lead to serious complications, depending on their nature, size and mechanism of injury. A 29-year-old male presented with a motorcycle handle embedded in his left orbit, with the vision unaffected. Active wound bleeding, increasing hematoma, a low haemoglobin level, signs of hypovolemic shock, ocular acuity and mobility were investigated. A computed tomography scan revealed a long bent metallic object lodged between the globe and floor of the left orbit with fracture of the medial orbital wall and ethmoidal hemosinus with an intact cribriform plate of the ethmoid. An interdepartmental collaborative effort of Oculoplasty, Oro-Maxillofacial and Neurosurgery were utilized in the removal of the foreign body. The patient recovered well after the surgery and a course of antibiotic therapy. A single large round-tipped foreign body in orbit composed of both metal and plastic is an extremely rare incident and fortunately in our case, was relatively harmless despite its large size. The diagnosis and management of intraorbital foreign bodies must be tailored according to their type and a proper localisation by all possible means, blunt dissection, careful haemostasis aided with good lighting, and exposure helps in their atraumatic removal.

Stratifin in ocular surface squamous neoplasia and its association with p53.

PURPOSE: Sunlight-induced p53 mutations are known to contribute towards increased risk of ocular surface squamous neoplasia (OSSN). Stratifin (14-3-3σ)/HEM (human epithelial marker) is a p53-mediated inhibitor of cell cycle progression and has been shown to be a target of epigenetic deregulation in various carcinomas. In the present study, Stratifin expression, its promoter methylation status as well as expression of mutant p53 in early and advanced AJCC stages (8th edition) of OSSN, was evaluated. METHODS: Sixty-four OSSN [20 conjunctival intraepithelial neoplasia (CIN) and 44 squamous cell carcinoma (SCC)] patients were registered for this study, and they were followed up for 36-58 months (mean 48 ± 3.6). Immunoexpression of Stratifin and mutant p53 protein, mRNA expression of Stratifin by reverse transcription polymerase chain reaction (PCR) and methylation status of Stratifin by methylation-specific PCR, was undertaken. RESULTS: Hypermethylation of Stratifin promoter in 63% (40/64), loss of Stratifin expression in 75% (48/64) and downregulation of Stratifin mRNA in 61% (39/64) were observed. Stratifin hypermethylation was significantly associated with reduced disease-free survival in both early and advanced T stage SCC cases. Expression of mutant p53 expression was seen in 48% (31/64) OSSN cases. Of the 31 patients with mutant p53 expression, 87% (27/31) also demonstrated loss of Stratifin immunoexpression. A significant association was seen between mutant p53 expression and Stratifin loss (p = 0.01) in advanced T stage SCC cases. CONCLUSIONS: Hypermethylation of Stratifin gene and its reduced mRNA expression both are potential biomarkers for identifying high-risk OSSN patients. Aberrant methylation of Stratifin and simultaneous mutant p53 expression implicates involvement of p53-Stratifin mediated signalling pathway in the pathogenesis of OSSN.

Mucous membrane grafting (fibrin glue vs. suture) for lid margin pathologies in Stevens-Johnson syndrome: randomized comparative study.

OBJECTIVE: To compare fibrin glue (with three cardinal sutures) (FG) and polygalactin suture (PS) for mucous membrane grafting (MMG) in terms of graft apposition and recurrence of lid margin keratinization (LMK) and metaplastic lashes (ML) in patients with Stevens-Johnson syndrome (SJS). DESIGN: Prospective randomized comparative interventional study. METHODS: Twenty patients diagnosed with SJS and lid margin abnormalities including LMK with or without ML were randomized to undergo either fibrin glue (FG)-assisted MMG (n = 10) or continuous 8-0 polygalactin suture (PS)-assisted MMG (n = 10). They were evaluated preoperatively and during follow-up at 1 week and 1, 2, 3, and 6 months. The parameters assessed were best-corrected visual acuity (BCVA), tear break-up time (TBUT), Schirmer-1 test, corneal and conjunctival complications, graft apposition and width (GW), LMK, ML, impression cytology, and operative time. The primary outcome measures are incidence of graft displacement and recurrence of LMK and ML. RESULTS: None of the eyelids in FG group (0/40) and 1 eyelid in PS group (1/40) had graft displacement. Recurrence of LMK occurred in 7.5% of eyelids (3/40) in both the study groups. Recurrence of ML occurred in 2.5% (1/40) in FG group and 5% (2/40) in PS group. The mean operative time for MMG in FG group was 39.5 ± 2.40 min and in PS group was 56 ± 1.63 min (p = 0.001). CONCLUSIONS: As graft apposition with suture involves significantly longer intraoperative time, if cost is not a limiting factor then fibrin glue is a viable option for the MMG for lid margin pathologies.