Anterior segment dysgenesis and secondary glaucoma in Goldenhar syndrome

A 3-year-old girl presented with a history of watering, haze and increase in the size of the right eye for two months. The child had bilateral preauricular skin tags, limbal dermoid and dermolipoma, consistant with the diagnosis of Goldenhad syndrome. In addition, her right eye manifested enlarged cornea, flat anterior chamber, atrophic iris and elevated intraocular pressure. This case report highlights a possible association of anterior segment dysgenesis and glaucoma with Goldenhar syndrome.

Astigmatic Analysis before and after Surgical Excision of Limbal Dermoid: Functional Outcome

PURPOSE: To analyze the length and area of limbal dermoid invading the cornea and to evaluate the effects on visual acuity and astigmatism before and after surgery. METHODS: This retrospective study included 20 eyes of 20 patients who underwent surgical removal of limbal dermoid. The preoperative and postoperative visual acuity and astigmatism level were measured. In addition, we evaluated the correlations of astigmatism with the length ratio (length of limbal dermoid invading the cornea/total corneal diameter), width ratio (width of limbal dermoid invading the cornea/total corneal diameter), and area ratio (area of limbal dermoid invading the cornea/total corneal area) using preoperative anterior segment photographs. RESULTS: The mean preoperative astigmatism was 0.85 ± 0.71 D in the sound eye and 3.00 ± 3.14 D in the affected eye (p = 0.004). Length, width, and area ratio of limbal dermoid have positive correlation with astigmatism (p < 0.010 for all variables), with a larger length ratio of limbal dermoid invading the cornea producing greater reduction in postoperative astigmatism (p = 0.010, r = 0.816). The amount of astigmatism was significantly higher in patients with amblyopia in the affected eye (p = 0.030). Visual acuity gain more than 2 lines was achieved in 8 among 10 patients with amblyopia under the age of 7 years through the appropriate refractive correction and occlusion 1 year after surgery. CONCLUSIONS: Astigmatism is a major cause of amblyopia in pediatric patients with limbal dermoid. The degree of astigmatism can be predicted by the size factors of the limbal dermoid. In particular, the relative length of limbal dermoid invading the cornea has a stronger correlation with preoperative astigmatism than other factors and has correlation with postoperative reduction of astigmatism. It should be emphasized that children with limbal dermoid need appropriate refractive correction and occlusion in addition to surgical excision.

Limbal dermoid in Nager acrofacial dysostosis: A rare case report.

Nager syndrome, also called preaxial acrofacial dysostosis, comprises two groups of defects involving the limbs and craniofacial region, respectively. This syndrome is rare and only 70 cases have been reported in the literature. The exact cause of this syndrome is unknown, but there is indication that it is genetically based. Ocular manifestations of this syndrome include widely separated downward slanting eyes, absence of eyelashes, ptosis of upper eyelids and colobomas on the inner aspect of lower eyelids. We report limbal dermoid in a patient with Nager syndrome. We did not find such an association of “Limbal dermoid in Nager acrofacial dysostosis syndrome” on PubMed using Nager acrofacial dysostosis, limbal dermoid and ocular manifestations as the keywords.

Nasopalpebral lipoma coloboma syndrome.

Nasopalpebral lipoma–coloboma syndrome is characterized by nasopalpebral lipoma and eyelid coloboma. We report a case of a 16-year-old Indian girl who reported to us with this rare syndrome. Computed tomography scan showed a significantly hypodense lesion on the right side of nose which was confirmed to be a lipoma on histopathological examination. This condition should be included in differential diagnosis of conditions with congenital eyelid coloboma.

Two Cases of Cosmetic Treatment of Limbal Dermoid

PURPOSE: To report two cases of cosmetic treatment of limbal dermoid, which consist of local excision of the limbal dermoid, corneal tattooing, and amniotic membrane transplantation. METHODS: Dermoid excision, corneal tattooing, and amniotic membrane transplantation were carried out in both a girl and a woman who each had a limbal dermoid in their left eyes. Postoperatively, they were followed up for more than six months. RESULTS: No specific complications occurred. The patients were satisfied with the surgical results. After a few months the conjunctiva was found to grow into the tattooed cornea. CONCLUSIONS: In the treatment of limbal dermoid, local excision combined with corneal tattooing and amniotic membrane transplantation can be a cosmetically successful alternative choice.

The Role of Ultrasound Biomicroscopy in Operation for Limbal Dermoid

PURPOSE: To evaluate the result of excision and lamellar keratoscleroplasty using ultrasound biomicroscopy in limbal dermoid. METHODS: We retrospectively analyzed in 10 patients with limbal dermoid, who were performed preoperative ultrasound biomicroscopy to access the extent of the lesion and the depth of corneal involvement, and underwent excision and superficial keratoscleroplasty with a minimum follow-up period of 6 months RESULTS: On ultrasound biomicroscopic examination, the dermoid showed highly reflective, homogeneous echo density than surrouding tissue, leading to identifying the lateral margin of the lesion and depth of involvement. The mean maximun thickness of the lesion was 1.1 +/- 0.2mm. The six of 8 patients, who were feasible to test visual acuity, had no change in best corrected visual acuity, and 2 of 8 patients gained less than 1 line. Most patients (9 of 10), except one, had good or excellent cosmetic results with no haze (5 of 10) or minimal haze (4 of 10). CONCLUSIONS: In limbal dermoid, ultrasound biomicroscopy was helpful in accessing the extent of the lesion and the depth of involvement preoperatively, and excision and lamellar keratoscleroplasty leads to good results without complication.

Lamellar Sclerokeratoplasty for Limbal Dermoids

PURPOSE: To evalute the surgical outcome of patients with limbal dermoid choristomata treated lay lamellar sclerokeratoplasty. METHODS: We performed lamellar sclerokeratoplasty on 13 patients with limbal dermoid in 14 eyes. Lamellar corneoscleral graft was used in this operation. We evaluated cosmetic effect, refractive change and graft survival/integrity after a follow up period of at least 3 months. RESULTS: All patients who underwent lamellar sclerokeratoplasty had good or excellent cometic result with good sclerocorneal alignment. There were no significant refractive changes following the surgery. Most (13 of 14) eyes had good graft integrity and survival without graft rejection. One of 14 eyes showed graft rejection sign, but it was subsided with systemic cyclosporine therapy. CONCLUSION: Lamellar sclerokeratoplasty is thought to be a good option for the primary surgery for limbal dermoid.