Clinicopathological Profile of Limbal Dermoid: A Case Series with Review of Immunohistochemical Findings

Background: Limbal dermoid is a rare, congenital, benign lesion occurring at the corneoscleral junction. Although usually harmless, it can cause cosmetic issues, astigmatism, and occasionally affect vision. Some cases are associated with Goldenhar syndrome, a congenital condition involving craniofacial, auricular, and vertebral anomalies. Histopathology and immunohistochemistry (IHC) help confirm the diagnosis and identify tissue components. This case series describes four Grade I limbal dermoids - two isolated and two linked with Goldenhar syndrome—to highlight their clinical, systemic, and histopathological features. Methodology: This retrospective case series included four clinically diagnosed limbal dermoid patients evaluated at a tertiary care hospital. All underwent detailed ocular examination, slit-lamp assessment, and photographic documentation. Cases 1 and 4 had isolated limbal dermoids, while Cases 2 and 3 showed features of Goldenhar syndrome. Data collected included demographics, presenting complaints, lesion grade and location, refractive error, systemic findings, and surgical details. All excised specimens were processed and analyzed in our hospital for routine staining and immunohistochemical evaluation. Discussion: All four patients were 10–13 years old, and all lesions were Grade I, mostly located inferotemporally. Two patients had systemic features of Goldenhar syndrome, such as facial asymmetry and preauricular tags. All children had astigmatism, which improved with refractive correction, and none required urgent surgery. Histopathology showed keratinized epithelium, collagenous stroma, adnexal structures, and adipose tissue in all cases, while syndromic cases also contained cartilage. IHC findings further confirmed the choristomatous nature of the lesions. Conclusion: This case series highlights the benign nature of Grade I limbal dermoids and their occasional association with Goldenhar syndrome. Most lesions respond well to conservative management, while syndromic cases need thorough systemic evaluation. Histopathology and IHC play an important role in distinguishing isolated lesions from syndromic ones and help guide appropriate treatment planning.