Giant congenital facial melanocytic nevus

Giant Congenital Melanocytic Nevus (GCMNs) is mostly reported in area of trunk followed by limbs and head. Their incidence is <1:20,000 newborns It derives attention due to its association with malignant melanoma.The risk of developing malignant melanoma is between 5 to 10%.We report a case of twelve year old boy with hyperpigmented lesion on face

Ultrasound biomicroscopy image patterns in normal upper eyelid and congenital ptosis in the Indian population

Purpose: To study the features of upper eyelid in healthy individual and different types of congenital ptosis in the Indian population using ultrasound biomicroscopy (UBM). Methods: This was a prospective observational study at a tertiary care center. Eyelid structure of healthy individuals with no eyelid abnormalities (n = 19); simple congenital ptosis (n = 33) cases; Marcus Gunn jaw-winking ptosis (MGJWP, n = 7) cases, and blepharophimosis-ptosis-epicanthus inversus syndrome (BPES, n = 20) cases were studied on a vertical UBM scan using 50-MHz probe. Lid-thickness, tarsal-thickness, orbicularis oculi and levator-Muller-orbital septum-conjunctival (LMSC) complex were measured in primary gaze. Comparison was made between four groups and results were statistically analyzed using ANOVA test. In normal individuals, LMSC measurements were repeated in down-gaze imaging. Results: Skin with subcutaneous tissue, LMSC complex and pre-aponeurotic fat-pad appeared echodense while orbicularis oculi and tarsus appeared echolucent. In primary gaze, mean thickness (± standard deviation) of the eyelid, tarsus, orbicularis oculi and LMSC, respectively, were: 1.612 ± 0.205, 0.907 ± 0.098, 0.336 ± 0.083, and 0.785 ± 0.135 mm in normal individual. LMSC showed 46.64% increase in thickness on down-gaze. The mean eyelid thickness and LMSC were thicker in MGJWP and BPES as compared to normal. In different types of congenital ptosis cases, various patterns of UBM imaging were observed. Conclusion: UBM allows noninvasive imaging of eyelid structures with good anatomical correspondence in normal eyelids and study the structural alterations of eyelids in different types of congenital ptosis. UBM can be used to highlight the anatomical difference in normal eyelids that may help modify the surgery for better cosmetic outcomes. Furthermore, it has the potential to be used in preoperative evaluation and operative planning in certain types of acquired ptosis, which needs to be evaluated

Clinicodemographic profile and treatment outcome in patients of ocular surface squamous neoplasia

Purpose: The aim is to study the clinicodemographic profile and treatment outcome of ocular surface squamous neoplasia (OSSN). Methods: This was a retrospective observational study of 57 eyes (56 cases) with clinically diagnosed OSSN, presenting in our center over the past year. Results: The median age of presentation was 55 years with male:female ratio being 4.5:1. Systemic predisposing conditions were xeroderma pigmentosa (1) postkidney transplant immunosuppression (1), and human immunodeficiency virus infection (1). Patients with predisposing conditions had a younger median age of onset (33 years). The majority of tumors were nodular (61.4%), gelatinous (61.4%), and had limbal involvement (96%). On ultrasound biomicroscopy (UBM), mean tumor height was 2.93 ± 1.02 mm, and intraocular extension was evident in seven eyes. OSSN with intraocular extension had a mean tumor height of 4.3 ± 1.32 mm. Nodal metastasis was seen in one case at presentation. As per American Joint Committee for Cancer Classification seventh edition staging-two cases were T1, one was T2, 46 were T3 and eight were T4. Treatment advised included conservative therapy for 39; wide local excision (4 mm margin clearance) with cryotherapy for seven; enucleation in four; and exenteration in four eyes. Overall, complete regression was achieved in 88% of cases during a mean follow-up of 13.5 ± 4.6 months. Recurrence was seen in three cases, which were treated with exenteration, radical neck dissection, and palliative chemo-radiotherapy, respectively. Conclusion: Although associated with old age, earlier onset of OSSN is seen in patients with systemic predisposing conditions. Thicker tumors in the setting of a previous surgery or immunocompromised status should be considered high-risk features for intraocular extension and should be evaluated on UBM

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