Coexistent Rosai Dorfman disease and Langerhans cell histiocytosis in an Orbital mass: A Case Report.

INTRODUCTION: Rosai Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder of lymph node sinuses. Langerhans cell histiocytosis (LCH) is a solitary or multisystem clonal proliferation of abnormal dendritic cells (Langerhans cells) with varied presentations. The co-occurrence of these two entities is quite rare. CASE DESCRIPTION: A six-year-old boy presented with multiple mass lesions in the neck since two years and a nodular lesion in right upper eyelid for the past 4 months. He was diagnosed with tubercular lymphadenitis 2 years back, and was given a course of anti-tubercular therapy (ATT) elsewhere. No improvement was seen. Fine needle aspiration cytology (FNAC) of the cervical lymph nodes revealed reactive lymphadenitis while lymph node biopsy showed features of RDD. Excision biopsy of the orbital mass showed features of both RDD and LCH. The patient was started on tablet prednisolone. Six months later, complete resolution of lymph node enlargement and remaining orbital mass was noted. Post operative contrast enhanced Magnetic Resonance Imaging of head and neck was normal. CONCLUSION: The coexistence of RDD and LCH may be a result of divergent differentiation from a common lineage or a de novo phenotypic evolution.

Comparative Study of Bilateral Versus Unilateral Strabismus Surgery in the Management of Lateral Incomitance in Intermittent Exotropia.

PURPOSE: To evaluate and compare bilateral asymmetric lateral rectus recession versus unilateral resection-recession surgery in the management of lateral incomitance in intermittent exotropia. METHODS: A prospective randomized interventional comparative study was conducted consisting of 80 patients with intermittent exotropia (older than 7 years) having significant lateral incomitance. They were equally divided into two groups by a sealed envelope system. The bilateral group underwent bilateral asymmetric lateral rectus recession and the unilateral group underwent unilateral lateral rectus recession and medial rectus resection based on post-patch deviation. Parameters assessed were change in horizontal deviation, change in lateral incomitance, binocularity, motility limitation, and complications, if any. Surgical outcome was considered successful if the primary deviation was within ±8 prism diopters (PD) and lateral incomitance was less than 5 PD. RESULTS: The mean lateral incomitance preoperatively and postoperatively was 8.3 ± 1.6 and 2.8 ± 2.4 PD in the bilateral group and 8.9 ± 1.4 and 3.7 ± 2.5 PD in the unilateral group, respectively. There was no statistically significant difference in the postoperative lateral incomitance between the two groups (P = .25), but a statistically significant difference was observed between preoperative and postoperative lateral incomitance in each group (P < .0001). CONCLUSIONS: Both procedures are equally efficacious in achieving acceptable ocular alignment and improving significant lateral incomitance in patients with intermittent exotropia with deviations between 15 and 35 PD without causing significant motility limitation. [J Pediatr Ophthalmol Strabismus. 2023;60(4):277-281.].

Comparative Evaluation of Intraocular Lens Power Calculation Formulas in Children.

Introduction With the advent of newer microsurgical techniques, the results for cataract surgery have become quite promising. An accurate intraocular lens (IOL) power calculation is one of the most important factors in optimizing the results. The aim of this study was to evaluate the accuracy of four IOL power calculation formulas, namely, Barrett Universal II, Holladay 1, SRK/T and Hoffer Q, using optical biometry in children undergoing cataract surgery with primary IOL implantation. Methods This was a cross-sectional study. A total of 60 eyes of 42 children aged 5-16 years with congenital cataract and having undergone uneventful cataract surgery with IOL implantation were included in the study. Eyes were divided into three groups based on the axial length (AL): short (AL <22.00 mm), medium (AL 22-24.50 mm) and medium long (AL 24.50-26.00 mm). Optical biometry was used and the IOL power was calculated using the Barrett Universal II formula. The predicted postoperative refraction with the other three formulas, namely, SRK/T, Holladay 1 and Hoffer Q, using the same IOL power was estimated. This was compared with the actual postoperative refraction (spherical equivalent at 12 weeks) to give the absolute prediction error. The mean of all absolute prediction errors gave the mean absolute prediction error (MAE) values for each formula that were then compared. Results The MAE was 0.64 ± 0.73 for Barrett Universal II, 0.7 ± 0.72 for Holladay 1, 0.71 ± 0.65 for Hoffer Q and 0.8 ± 0.75 for SRK/T. Thus, Barrett Universal II had the lowest MAE across the whole group. The difference in the MAEs was not statistically significant. Conclusion Barrett Universal II had the lowest MAE and thus was predictable for the highest number of eyes in our study, although this was not statistically significant (p=0.176).

Orbital tumour or a retained foreign body? An uncommon case of missed diagnosis.

We report a case who presented with decreased vision, significant hypotropia, proptosis and gross limitation of extraocular motility for one year. Suspecting an orbital tumour, we asked for a computed tomography of the orbit which revealed a mass lesion in the inferior orbit. However, incisional biopsy reported inflammatory infiltration. Diagnosing it as orbital inflammatory disease, a course of oral steroids was given for four weeks. It was only after the reduction in inflammation that a foreign body was palpable in the inferior fornix. Surgical exploration revealed a large wooden foreign body measuring 3.3 × 1 × 0.3 cm. Though intraorbital foreign bodies are not rare, ambiguous history, delayed presentation and nonspecific CT findings made this case diagnostically challenging.

Comparison of fundus fluorescein angiography, optical coherence tomography and optical coherence tomography angiography features of macular changes in Eales disease: a case series.

PURPOSE: To study the macular features in Eales disease patients observed with fundus fluorescein angiography (FA), optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA). METHODS: A cross-sectional study was done on treatment naïve 31 eyes (23 patients) with Eales disease. Baseline parameters such as Best-corrected visual acuity (BCVA), slit-lamp bio microscopy (SLB), indirect ophthalmoscopy, FA, spectral-domain OCT {quantitative (central macular thickness [CMT]) and qualitative analysis on SD-OCT} and OCTA were performed. Any media opacity precluding the above investigations was excluded. RESULTS: Macular findings comprised of- epiretinal membrane, macular exudation, full thickness macular hole, sub internal limiting membrane bleed, cystoid macular oedema, neurosensory detachment and retinal thickening. Sixteen (51.6%) of our patients had macular changes as seen on all modalities together. SLB and indirect ophthalmoscopy missed macular findings in 50% patients and FA in 18.8% patients. OCT and OCTA diagnosed all macular findings. On comparison of mean BCVA in patients with macular involvement on FA, OCT and OCTA, compared to those without macular involvement, patients with macular involvement had lower BCVA (p 0.000, 0.01 and 0.001 respectively). Thus, FA missed many patients who had significant macular involvement and hence less vision. CONCLUSION: Eales disease though described in literature as classically being peripheral retina disease process, also has macular involvement. OCT and OCTA are useful guides to evaluation of macular involvement in these patients. The latter seems to be superior to FA in detecting macular abnormalities in this ailment. OCTA is non-invasive and shows deep capillary plexus changes which are not shown by any other modality.

Comparison of conventional and Hang-back methods of inferior oblique recession in V-pattern strabismus with inferior oblique overaction.

AIM: To compare outcomes of conventional and Hang-back methods of inferior oblique (IO) recession in V-pattern strabismus with inferior oblique overaction (IOOA). METHODS: Comparative randomized study was conducted consisting of 50 patients, age 6 to 35y having V-pattern strabismus [>15 prism diopter (PD)] with IOOA. They were divided equally in two groups and underwent IO recession, group A by conventional method and group B by Hang-back method. Parameters evaluated were reduction in V-pattern and IOOA, shift in torsion, improvement in binocular status. Success was a residual V-pattern of less than 10 PD at 1y of follow up. RESULTS: The mean V-pattern preoperatively and postoperatively at 1y was 24±5.4 PD and 4.2±3.51 PD in group A and 23.44±6.44 PD and 5.76±3.8 PD in group B respectively. The mean reduction in V-pattern was 20±6.78 PD in group A and 18.2±5.48 PD in group B. The mean correction of IOOA was 18.48±3.13 PD in group A and 16±2.93 PD in group B. Mean shift in extorsion was 3.08±1.8 degree in group A and 3.72±2.14 degree in group B. CONCLUSION: Both the procedures achieve a significant and comparable reduction in V-pattern and IOOA. Hang-back recession being a landmark free surgery eliminates the need for intrascleral suturing thereby reducing the risk of scleral perforation with possible postoperative adjustment of muscle. It may be considered as a good alternative for IO recession in patients of V-pattern strabismus with mild to moderate amount of IOOA.

Ocular surface response to fibrin sealant versus vicryl suture for conjunctival closure after strabismus surgery: An objective assessment.

PURPOPSE: This study aims to evaluate the ocular surface response after strabismus surgery, using two different materials for conjunctival closure. METHODS: Randomized prospective comparative study was done. After performing strabismus surgery, conjunctival flap was apposed with fibrin sealant in Group 1 and 8-0 vicryl suture with buried knots in Group 2. Preoperative and postoperative measurement of tear film break up time and Schirmer test for tear secretion was done in both the groups of 30 patients each at regular follow-up visit up to 2 months. Postoperatively, both the groups were also compared for the resolution of discomfort and redness. RESULTS: Conjunctival recession was not seen in any group. Mean period was 5.8 days for resolution of discomfort (pain, lacrimation, and irritation) and 7.2 days for redness in Group 1 compared to 12 and 16 days in Group 2 (P < 0.05). During the study, we noticed significant decrease in tear film stability and increase in tear secretion in both the groups. However, in Group 2, the parameters were significantly more altered and even changes persisted for longer duration. CONCLUSION: Early rehabilitation of ocular surface might be related to unaltered healing process with the use of a biological substance (fibrin sealant) as compared to suture.

Comparative study of 25- versus 20-gauge pars plana capsulotomy and vitrectomy in pediatric cataract surgery.

PURPOSE: To compare 25- and 20-gauge pars plana vitrectomy (PPV) for the management of pediatric cataract. METHODS: 20 eyes of 15 patients were randomly divided into two groups to undergo pars plana capsulotomy and vitrectomy by either 25-gauge (group A) or 20-gauge (group B) PPV after lens aspiration and IOL implantation. The two groups were compared for total surgical time, time taken in doing pars plana capsulotomy and vitrectomy, and the size of posterior capsulotomy. Post-operative astigmatism was compared at 3 months. RESULTS: The mean total surgical time in group A was 49.2 ± 6.7 min, while mean total surgical time in group B was 62.5 + 5.48 min (p = 0.001). The mean time taken for pars plana capsulotomy and vitrectomy was 4.1 ± 1.19 min in group A and 5.0 ± 0.73 min (p = 0.03) in group B. The mean size of the PCCC in group A was 3.3 ± 0.34 mm, while in group B it was 4.0 ± 0.33 mm (p = 0.001). The mean astigmatism at 3 months in group A was 0.65 ± 0.31 diopters, while in group B it was 1.45 ± 0.92 diopters (p = 0.019). CONCLUSIONS: 25-gauge transconjunctival sutureless PPV can be an attractive alternative to 20-gauge system in the management of pediatric cataracts.

Comparison of adjustable sutures versus nonadjustable sutures in intermittent exotropia.

PURPOSE: To compare adjustable sutures versus nonadjustable sutures for intermittent exotropia. METHODS: In this randomized prospective interventional study, 40 adult patients with intermittent exotropia were randomly divided into 2 equal groups. Both groups underwent bilateral lateral rectus recession. In group A, adjustable suture recession was performed, and in group B, nonadjustable suture recession was performed. Patients were followed up for 6 months and outcome measures were residual deviation, binocular status, and need for resurgery. RESULTS: Success was defined as alignment of 2 eyes <10 prism diopters (PD) of deviation at the end of 12 weeks. Need for resurgery in a 12-week follow-up period was considered to be failure. At the end of the study, 90% of the patients in group A and 85% of the patients were within 10 prism diopters of orthophoria (p = 0.316). At the end of 6 months, mean deviation in group A was 6.20 PD and in group B it was 5.60 PD (p = 0.31). No patient underwent resurgery. CONCLUSIONS: Adjustable hang-back recession has no definite added advantage over nonadjustable hang-back recession in intermittent exotropia.

To assess the efficacy of vertical muscle surgery for management of hypotropia in monocular elevation deficiency type II.

AIM: To assess the efficacy of vertical muscle surgery for management of hypotropia in monocular elevation deficiency (MED) type II. Knapp's is described as standard procedure for management of MED type II. However, it is not graded and has unpredictable amount of correction. Besides this, there is drift towards overcorrection with time and limitation of movements in extreme adduction and abduction. MED is a vertical misalignment for which vertical muscle surgery is also described but limited literature is available. METHODS: Thirteen fresh cases of MED type II with hypotropia >20 PD and age >4 years were included in our interventional study. All cases underwent superior rectus resection and inferior rectus recession (vertical R&R) depending upon amount of preoperative deviation. Success was defined as hypotropia <5 PD at 1-year follow-up. RESULTS: Twelve patients (92.30 %) were aligned to within 5 PD. Six patients (46.15 %) had gain in elevation. Bell's phenomenon was improved in six patients (46.15 %). There was no limitation in down gaze in any patient. None gained stereopsis. CONCLUSION: Vertical R&R is a good alternative for MED type II with predictable amount of correction especially in patients with higher preoperative deviation. It spares horizontal muscles for correction of any associated horizontal deviation.

Optic nerve hypoplasia.

Optic nerve hypoplasia (ONH) is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65%) than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED).

Congenital accessory palpebral aperture--an addition to the spectrum of Delleman syndrome.

Delleman syndrome (oculocerebrocutaneous syndrome, MIM 164180) is characterized by orbital cysts, microphthalmia/anophthalmia, focal skin defects, skin appendages and multiple cerebral malformations. We herein describe a case of an 8-month-old male child with features suggestive of Delleman syndrome along with a rare congenital lid anomaly - an accessory palpebral aperture, not reported so far to the best of our knowledge.

Fine needle aspiration biopsy in orbital lesions.

PURPOSE. The study was performed to evaluate the efficacy of fine needle aspiration biopsy (FNAB) in orbital lesions. METHODS. Seventeen patients with orbital masses who had been fully investigated by non-invasive techniques participated in this study. FNAB was performed by standard technique, as an outpatient procedure, with ultrasound guidance in lesions posterior to the equator. A trained cytologist analysed all the smears. RESULTS. Specific results were obtained in 14 of the 17 (82%) patients studied by FNAB. Ten cases were neoplastic (8 malignant and 2 benign), 3 were inflammatory and 1 was a case of histiocytosis X. In 3/17 cases the results were non-specific. These were treated as pseudotumours and responded well to systemic steroids. In 7 cases the clinical and radiological diagnosis was confirmed by FNAB. Non-invasive investigations like USG, CT and MRI, however, failed to provide accurate diagnosis in the other 7 (41%) cases. In these patients, FNAB yielded a pathological diagnosis (histiocytosis X, cryptococcosis, non Hodgkin's lymphoma, adenocarcinoma, pleomorphic adenoma, Schwannoma and cysticercosis), helping us to modify treatment with an excellent response. No significant complications were encountered following the aspiration biopsies. CONCLUSION. FNAB proved to be a reliable method for distinguishing between malignant and non-malignant lesions. It was found to be rapid, accurate, cost-effective, safe and a valuable addition to ultrasound, CT scan and MRI in the diagnosis of orbital lesions. This tool may help in avoiding a traumatic surgical intervention.

Orbital hydatid cyst: an unusual presentation.

PURPOSE. To describe an unusually large and long-standing hydatid cyst of the orbit causing gross disfigurement of the eyeball. METHODS. Detailed investigations, consisting of radiological and hematological studies, were carried out to determine the cause of the unusually severe and long-standing proptosis. The patient underwent orbital exploration for the presumed diagnosis of orbital echinococcosis.The entire cyst with the globe was removed and sent for histopathological examination. RESULTS. The gross and histopathological findings of the specimen removed surgically confirmed the diagnosis of orbital hydatid cyst. CONCLUSION. The diagnosis of orbital hydatid cyst should be considered in unilateral severe proptosis of long duration with gross disfigurement of the globe and orbital walls.