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Surgical Excision and Corneal Patch Graft of a Limbal Dermoid in a Pediatric Patient

Title: Surgical Excision and Corneal Patch Graft of a Limbal Dermoid in a Pediatric Patient
Author: Kenneth Han, MS4
Photographer: Dr. Amy Lin
Image or video:

Keywords/Main Subjects: Limbal Dermoid, Corneal Dermoid, Congenital Choristoma, Epibulbar Dermoid, Corneal Patch Graft
Diagnosis: Limbal Dermoid
Description of Video: Surgical excision and partial thickness corneal patch graft of a 3x3x2 mm limbal dermoid in a pediatric patient


Background:
Limbal dermoids are a form of choristoma, an ectopic growth of fully differentiated tissue, which occur on the anterior surface of the eye at the anatomical limbus. These growths usually present as yellow-white lesions and may contain a combination of connective tissue, hair, fat, skin, blood vessels, and sebaceous glands. The inheritance pattern of limbal dermoids is highly variable, with cases being reported as autosomal dominant, autosomal recessive, X-linked, or multifactorial.1 The majority (76%) of limbal dermoids occur in the inferotemporal region.2 While the exact pathophysiology of limbal dermoid development is unknown, one theory suggests that they arise from an embryological metaplastic transformation between the optic nerve and surface ectoderm.3 A majority of limbal dermoids occur sporadically, though multiple systemic conditions have been associated with limbal dermoids including Goldenhar syndrome, ring dermoid syndrome, and epidermal nevus syndrome.4,5 Importantly, limbal dermoids should not be confused with orbital dermoid cysts.


Diagnosis:
The diagnosis of limbal dermoids is usually clinical, though biopsy may be performed to confirm the diagnosis. Additionally, ultrasound biomicroscopy or anterior segment ocular coherence tomography (OCT) may be utilized to assess the depth of the dermoid, though the clinical utility of these diagnostic measures remains unclear.6,7 Patients with limbal dermoids present early in life and may be asymptomatic. However, limbal dermoids which encroach on the visual axis or produce corneal astigmatism may lead to the development of anisometropic amblyopia. The following grading system has been traditionally used to help guide management: Grade I limbal dermoids are superficial, <5mm, and are confined to the limbus. Grade II limbal dermoids are larger and may penetrate deep into the stroma without involving Descemet membrane. Grade III limbal dermoids are large, covering the entire cornea, and may extend into the iris epithelium.8


Management:
In patients with grade I limbal dermoids, mild astigmatism (<1 D), and good compliance with spectacle correction, close observation with serial examinations every 6-12 months is recommended.9 Patients in this category with suspected or proven amblyopia should be monitored more frequently and treatment with occlusion or spectacles should be attempted.10 However, indications for surgery include rapid tumor growth, visual axis occlusion, secondary corneal defect, unresponsive amblyopia, inadequate lid closure, irritation, and psychosocial factors.10 Shared decision making with parents should be made regarding the timing of surgical excision.11

Grade II and III limbal dermoids are always treated with surgical excision, as these lesions generally cause refractive or occlusive amblyopia.10 Corneal graft should be available at the time of excision, as these lesions may extend through the full thickness of the cornea. The following surgical approaches have been recommended based on a comprehensive literature review performed by Pirouzian et. al.:

Grade I (<50 μm thickness and <1 mm diameter): simple excision

Grade I (<100 μm thickness and <1 mm diameter): keratectomy + amniotic membrane transplantation (AMT) + autologous limbal stem cell allograft (ALSCA)

Grade II and deeper grade I: keratectomy + AMT + ALSCA + pericardial patch graft (PPG) versus anterior or deep anterior lamellar keratoplasty (ALK) +/- AMT


Prognosis:
In recent years, additional grading systems have been developed which include prognostic information.12 Data from these studies have revealed that, in general, lower-grade limbal dermoids are associated with better visual acuity. Thus, early diagnosis and appropriate surgical management are essential to providing the best visual outcomes for patients with limbal dermoids.


Patient Case:
We present the case of a 13-year-old female with developmental delay, cleft lip, and a history of patching in early childhood who was diagnosed with a limbal dermoid. She had experienced a gradually enlarging, smooth, white, spherical mass on the inferonasal limbus of her left eye since birth. At the time of examination, the mass measured 3x3x2 mm with elements of vasculature and overlying hair. Despite these findings, she remained asymptomatic other than an anisometromic astigmatism of the left eye. Examination revealed no syndromic features and no other ocular pathology. However, after a year of observation, the limbal dermoid continued to grow with worsening of the astigmatism. The patient and her mother elected to proceed with surgical excision and partial thickness corneal patch graft. At 1-week post-op, the patient was recovering well with stable refraction, and ophthalmic pathology confirmed the diagnosis of a limbal dermal choristoma.


References:

  1. Mansour AM, Barber JC, Reinecke RD, Wang FM. Ocular choristomas. Surv Ophthalmol. 1989;33(5):339-358. doi:10.1016/0039-6257(89)90011-8
  2. Nevares RL, Mulliken JB, Robb RM. Ocular Dermoids: Plast Reconstr Surg. 1988;82(6):959-964. doi:10.1097/00006534-198812000-00004
  3. Hameed S, Kaur I, Singh V, Mishra DC, Reddy JC. Congenital central corneal dermoid: A rare entity. Eur J Ophthalmol. 2022;32(3):NP5-NP9. doi:10.1177/1120672120986365
  4. Baum JL, Feingold M. Ocular Aspects of Goldenhar’s Syndrome. Am J Ophthalmol. 1973;75(2):250-257. doi:10.1016/0002-9394(73)91020-9
  5. Mattos J, Contreras F, O’Donnell FE. Ring dermoid syndrome. A new syndrome of autosomal dominantly inherited, bilateral, annular limbal dermoids with corneal and conjunctival extension. Arch Ophthalmol Chic Ill 1960. 1980;98(6):1059-1061. doi:10.1001/archopht.1980.01020031049007
  6. Hoops JP, Ludwig K, Boergen KP, Kampik A. Preoperative evaluation of limbal dermoids using high-resolution biomicroscopy. Graefes Arch Clin Exp Ophthalmol. 2001;239(6):459-461. doi:10.1007/s004170100300
  7. Janssens K, Mertens M, Lauwers N, De Keizer RJW, Mathysen DGP, De Groot V. To Study and Determine the Role of Anterior Segment Optical Coherence Tomography and Ultrasound Biomicroscopy in Corneal and Conjunctival Tumors. J Ophthalmol. 2016;2016:1-11. doi:10.1155/2016/1048760
  8. Mann, Ida. Developmental Abnormalities of the Eye.; 1937.
  9. Robb RM. Astigmatic Refractive Errors Associated With Limbal Dermoids. J Pediatr Ophthalmol Strabismus. 1996;33(4):241-243. doi:10.3928/0191-3913-19960701-08
  10. Pirouzian A. Management of pediatric corneal limbal dermoids. Clin Ophthalmol. Published online March 2013:607. doi:10.2147/OPTH.S38663
  11. Kaufman A, Medow N, Phillips R, Zaidman G. Treatment of Epibulbar Limbal Dermoids. J Pediatr Ophthalmol Strabismus. 1999;36(3):136-140. doi:10.3928/0191-3913-19990501-11
  12. Zhong J, Deng Y, Zhang P, et al. New Grading System for Limbal Dermoid: A Retrospective Analysis of 261 Cases Over a 10-Year Period. Cornea. 2018;37(1):66-71. doi:10.1097/ICO.0000000000001429

Faculty Approval by: Dr. Amy Lin

Copyright statement: Kenneth Han, ©2025. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/