Clinicodemographic profile of orbital exenteration in a tertiary eye care center - A 20-year experience.

PURPOSE: This study aimed to determine the clinical indications for orbital exenteration, demographic profile of these patients, and clinicopathologic correlations in the current times and to compare these results with previous published data. METHODS: It was a retrospective study. All exenterations performed at a tertiary eye care center over a period of 20 years (from January 2001 to June 2020) were retrospectively evaluated. Patient records were reviewed to obtain demographic data, presenting symptoms and their duration, laterality, and clinical and histopathologic diagnosis. RESULTS: A total of 352 cases (males:females = 222:130) who underwent exenteration were identified. Patients age ranged from 11 months to 87 years (mean: 43.86 years, median: 50 years). The most common indication for exenteration was found to be eyelid malignancy in 54.36%, followed by retinoblastoma in 18.75% and primary orbital tumors in 14.49%. Out of 156 cases of eyelid malignancies requiring exenteration, squamous cell carcinoma (SCC) was the most common histologic subtype ( n = 94, 60.26%), followed by sebaceous gland carcinoma ( n = 40, 25.64%) and basal cell carcinoma ( n = 20, 12.82%). The most common primary orbital tumors requiring exenteration were adenocystic carcinoma of the lacrimal gland in adults and rhabdomyosarcoma in the pediatric age group. Benign conditions requiring exenteration included fulminant fungal orbital infections and lymphangioma among others. CONCLUSION: The number of exenterations performed have significantly increased in terms of absolute numbers. However, the ratio of exenteration to other tumor-related surgeries, mainly excision biopsy, has reduced compared to that reported from a previous study. The most common indication in our study remains eyelid malignancy followed by intraocular malignancy. However, SCC has emerged as the most common tumor histologic subtype requiring exenterations.

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.

Outcomes of canalicular trephination versus canaliculodacryocystorhinostomy in common canalicular blocks.

AIM: This study aimed to compare the outcomes and efficacy of canalicular trephination with monocanalicular stenting and canaliculodacryocystorhinostomy (canaliculoDCR) with silicone intubation. MATERIALS AND METHODS: A prospective comparative study was done in 30 patients (30 eyes) with common canalicular blocks who were randomized into two groups. Fifteen patients underwent canalicular trephination with monocanalicular stenting and 15 patients underwent canaliculoDCR with silicone intubation. Common canalicular obstruction was diagnosed by preoperative syringing and the location of block was confirmed on probing. Stents were kept in both the groups for 3 months and followed up till 6 months postoperatively. Success was defined based on both anatomical and functional outcomes. Anatomical success was defined by the free passage of fluid on syringing with the fluid felt in the throat. Functional success was defined in terms of relief from epiphora based on the subjective opinion and its categorization by Kraft and Crawford's grading and the fluorescein dye disappearance test (FDDT). RESULTS: Eighty percent of eyes in canalicular trephination group and 73.3% of eyes in canaliculoDCR group were anatomically patent on syringing at the final follow-up. Sixty-six percent of eyes in trephination group and 53.3% in canaliculoDCR group were reported to have absent epiphora (complete recovery). Ten eyes in both groups had Grade 0 and 1 FDDT indicating a functional success of 66.6% in both groups. CONCLUSIONS: Canalicular trephination can produce results comparable to canaliculoDCR with silicone intubation in cases of common canalicular blocks. The average duration of surgery is significantly less in canalicular trephination which gives this procedure an added advantage.

Metastatic Insulinoma Presenting With Postprandial Hypoglycemia.

Background/Objective: Patients with an insulinoma, a type of pancreatic neuroendocrine tumor, typically present with fasting hypoglycemia but can rarely present exclusively with postprandial hypoglycemia. Case Report: A 69-year-old man presented with episodes of postprandial blurry vision, sweating, and confusion for the last 2 years that were becoming more frequent over the last several weeks. Home blood glucose measurements revealed postprandial hypoglycemia (glucose level, 45-70 mg/dL), and symptoms were consistent with the Whipple triad. Continuous glucose monitoring revealed only postprandial hypoglycemia within 2 hours following meals. An outpatient fast was conducted with detectable insulin (6 µIU/mL) and C-peptide (2.0 ng/mL) levels with an elevated proinsulin (20.8 pmol/L) level when the serum blood glucose level dropped to 47 mg/dL (21 hours after the initiation of the fast). A computed tomography scan of the abdomen and pelvis showed a 1.6-cm hyperenhancing lesion in the distal body of the pancreas. He underwent endoscopic ultrasonography with fine-needle aspiration. Pathology revealed a low-grade, well-differentiated, neuroendocrine tumor with lymphovascular invasion and regional lymph node metastases, confirming the diagnosis of a pancreatic neuroendocrine tumor. Discussion: Exclusive postprandial hypoglycemia is estimated to occur in 6% of the insulinomas. Patients with postprandial hypoglycemia may be initially managed as those with reactive hypoglycemia; however, this case highlights the importance of evaluating for an insulinoma in a patient who has failed treatment for reactive hypoglycemia. This case also demonstrates the importance of including proinsulin levels in that evaluation. Conclusion: Pancreatic neuroendocrine tumor should be considered in postprandial hypoglycemia, even in the absence of fasting hypoglycemia. Measuring proinsulin levels is essential in the diagnostic workup of insulinoma causing hypoglycemia.

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.

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.

Evaluation of lacrimal duct probing in adults with 0.02% and 0.04% mitomycin-C in primary acquired nasolacrimal duct obstruction: A randomized comparative pilot study.

PURPOSE: The purpose of the study is to evaluate the safety and efficacy of adjunctive use of mitomycin-C (MMC) using two different concentrations 0.2 mg/ml and 0.4 mg/ml for lacrimal duct probing to treat the nasolacrimal duct obstruction (NLDO) in adults. SUBJECTS AND METHODS: Prospective, an interventional comparative randomized pilot study of lacrimal duct probing conducted in the two study groups 0.02% MMC group (n = 30) and 0.04% MMC group (n = 30) in confirmed primary acquired NLDO of <1-year duration. Patency of lacrimal duct probing confirmed by syringing was compared at 1, 3, and 6-month follow-up in the two study groups, and corresponding subjective improvement of watering was appraised according to Kraft and Crawford grading. RESULTS: Patency of lacrimal duct probing in 0.02% MMC versus 0.04% MMC group was 66.66%/73.33% (P = 0.71) at 1 month, 46.66%/66.66% (P = 0.09) at 3 months and 46.66%/66.66% (P = 0.03) at 6-month follow-up, respectively. Subjective improvement of watering (no watering and mild watering) observed in 0.02% MMC versus 0.04% MMC group was 46.66%/73.33% (P = 0.03), at 1 month, 66.66%/83.33% (P = 0.13) at 3 months and 56.66%/73.33% (P = 0.17) at 6-month follow-up. CONCLUSION: Adult lacrimal duct probing with 0.04% MMC was associated with significant higher objective success rate than adult lacrimal duct probing with 0.02% MMC, without added concurrently side effects.

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.