Limbal dermoids rank among the most common tumors of the corneal limbus. They are ocular choristomas and can occur in a variety of sizes. Images in Clinical Medicine from The New England Journal of Medicine — Limbal Dermoid. Images in Clinical Medicine from The New England Journal of Medicine — The Hairy Eyeball — Limbal Dermoid.

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Indications for surgery There are recognized clinical indications for proceeding with surgical excision and anterior surface reconstruction in patients with a grade I limbal dermoid.

:CaseGoldenhar-Syndrome-Limbal-Dermoid-Coloboma

Conservative management by observation may not be a suitable option for such lesions. For the evaluation of postoperative complications, the assessed patients had a postoperative follow-up from 2 to 53 months. The data of 12 consecutive patients 14 eyeswho underwent monocentric surgery of a limbal demoid in the past 9 years, were retrospectively analyzed for intra and postoperative complications.

Right eye 2 years after removal of large limbal dermoid see Figure ddermoid. The inhibition of fibroblast growth by Mitomycin C is also reported in the treatment of a primary pterygium, a recurrent pterygium, and in filtering bleb surgery. Journal List Eye Lond v.

Management of pediatric corneal limbal dermoids

Current standard medical treatment for grade I pediatric limbal dermoids ie, with superficial corneal involvment is initially conservative. For the group overall and most individual patients, mean astigmatism, spherical equivalent, and refraction, as assessed by surgically-induced refractive change and h-vector dernoid, were not significantly changed.

He had no clefting of the lip or palate. Received May 13; Accepted Apr In the past, several different surgical techniques for the removal of dermoids have been described. In the majority of cases, only one side of the body is affected interestingly, the right side is usually the more severely affected.

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Basic and Clinical Science Course.

Goldenhar Syndrome (Oculo-Auriculo-Vertebral Spectrum):

There has been no proven association of Goldenhar syndrome with any maternal infection, medication or other insult. A thorough history should be taken from the parents, and serial in-office examinations with cycloplegic retinoscopy must be performed to monitor the size of the corneal dermoid.

A combination of surgical approaches involving excision of the dermoid from the sclera and partial keratectomy followed by reconstructive steps using a pericardial patch graft on the sclera with overlying conjunctival autologous limbal stem cell transplantation and volumetric filling of the residual corneal defect with fresh multilayered amniotic membrane rather than lamellar keratoplasty deep or superficial may allow for the best functional, refractive, and cosmetic outcomes postoperatively.

Surgery is universally indicated for grade II and III limbal dermoids, given that they generally cause refractive or occlusive amblyopia Table 1.

The rationale for using multilayered amniotic membrane transplantation is to achieve complete volumetric filling of the defective area, which should be equal in height to that of the surrounding healthy corneal tissue. Reduced inflammation with consecutive protection of the limbal stem limbaal is another commonly assumed mechanism of action.

The syndrome is almost always diagnosed early in life, before there is any complaint of symptoms by the infant patient. Excision of limbal dermoids.

Management of pediatric corneal limbal dermoids

Males are more affected than females in a 2: Full motility is evident, OU Pupils: Then the complete lamellar en bloc excision of the corneal and scleral parts of the dermoud was performed including the removal of the entire adjacent conjunctiva. J Pediatr Ophthalmol Strabismus. Seven eyes were treated by removal of the limbal dermoid by lamellar excision without further procedures.

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All surgeries were performed by the same surgeon. Vision is often impaired from astigmatic refractive errors caused by corneal astigmatism.

The transplantation of amniotic membrane following removal of a limbal dermoid cannot prevent the occurrence of a pseudopterygium. They can be autosomal dominant, recessive, X-linked, or multifactorial. The limbal dermoids did not obstruct the visual axis and the right upper eyelid coloboma did not prevent corneal protection with lid closure. Astigmatic refractive errors associated with limbal dermoids.

March 31, ; Available from: The pattern of inheritance is quite variable in epibulbar choristomas. Postoperatively, every patient showed cosmetic improvement.

Maurice Goldenhar Goldenhar, in a pair of monozygotic twins with epibulbar dermoids, auricular appendages, malformations of the auricle, and hemifacial microsomia. Squamous epithelium, hair follicles, and sebaceous glands were unanimously present in the specimen. The patient was given appropriate spectacle correction in his first months of life. It is recommended that these children undergo close clinical observation with serial examinations in the office, not only to monitor stability but also to provide reassurance for parents.

Published online May Epibulbar dermoids may present as a single lesion or as multiple lesions.