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The present study reports the case of a superficial limbal dermoid surgically treated without suture by using a new technique of lamellar keratoplasty with allogenic lenticule from small incision lenticule extraction. The limbal lesion area was circumscribed by a trephine marker and lamellae were peeled off the anterior corneal stroma and sclera. After excision of the dermoid, the liquid on the bed was dried as much as possible and an appropriate stromal lenticule was attached to the implant bed without fibrin glue or suture. A bandage contact lens was applied to fix the graft. During postoperative follow-ups, the anterior segment optical coherence tomography, corneal refractive power, corneal topography and best-corrected visual acuity were examined to evaluate the surgical results. On the first day after the operation, the patient reported tearing and pain at the site. At one week after the operation, the patient reported no discomfort and conjunctival sutures were removed. At one month after the operation, the thickness of the lesion area was similar to that of the adjacent cornea, and the corneal curvature and refractive power of the lesion area were higher than that of the adjacent cornea. At three months after the operation, the patient was satisfied with the cosmetic outcome and no sign of corneal neovascularization, graft rejection or pseudo-pterygium formation was observed. The astigmatic error remained at 2.50 D. The present case provides insights into a potential treatment option for patients who need lamellar keratoplasty and may provide its benefit in contrast to conventional lamellar keratoplasty.

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Limbal dermoid is a congenital benign tumor of the limbus which is often managed by surgery if necessary. In dermoid lesions involving the deep stroma, tumor excision and reconstruction of the anterior segment with amniotic membrane transplantation or keratoplasty may be required. Herein, we present a case of deep limbal dermoid treated with surgical resection and lamellar keratoplasty using microkeratome-assisted anterior lamellar graft.

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PURPOSE: To find preoperative simple geometric parameters to predict the outcome of lamellar keratoscleroplasty in patients with corneal limbal dermoids. METHODS: We retrospectively analyzed the data of 30 patients with limbal dermoids who underwent lamellar keratoscleroplasty and were followed up for more than 6 months. Seven geometric parameters were used to analyze the relationship with postoperative visual acuity, astigmatism, and scar formation and investigated for their influence on postoperative outcome. These seven parameters included the cornea-invaded area of dermoid, dermoid area, the cornea-invaded length of dermoid, the corneal limbus invaded length of dermoid, dermoid size, bed size, graft size. Furthermore, we divided patients with corneal limbal dermoids into amblyopia group and non-amblyopia group, and analyzed the clinical characteristics of the amblyopia group. RESULTS: The mean age of the patients at surgery was 10.6 ± 5.83 years. The 7 geometric parameters, preoperative and postoperative astigmatism of the amblyopia group were higher than those of the non-amblyopia group (all P < 0.05). Among the geometric parameters analyzed, the r coefficients between the cornea-invaded length of dermoid and postoperative visual acuity and astigmatism were the highest (r = 0.854, r = 0.714). The r coefficient between the corneal limbus invaded length of dermoid and the postoperative scar was the highest (r = 0.375). The r coefficient between age and postoperative steroid-induced high intraocular pressure was the highest (r = - 0.416). In univariate regression analysis, the cornea-invaded length of dermoid was significantly correlated with postoperative visual acuity (ß = 0.270, P < 0.001) and postoperative astigmatism (ß = 1.362, P < 0.001). Among the geometric parameters analyzed, the cornea-invaded length of dermoid had best stratified patients in grouping with or without amblyopia (cutoff > 2.24). CONCLUSION: The cornea-invaded length of dermoid was the most important related factor of postoperative visual acuity, astigmatism, and amblyopia. The corneal limbus invaded length of dermoid was the most important related factor of postoperative scar formation. The geometric parameters and astigmatism in patients with amblyopia were larger than those in patients without amblyopia.

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Goldenhar syndrome is a rare congenital disorder. Limbal dermoid associated with this syndrome can cause high astigmatism and amblyopia in children. We report significant reduction in astigmatism, after limbal dermoid excision with lamellar keratoplasty, in a rare case of Goldenhar syndrome. A three-year-old female patient, with left-sided limbal dermoid with high astigmatism and amblyopia, was referred to us for visual rehabilitation. The patient had left-sided limbal dermoid and preauricular appendages. Oral examination revealed bifid labial frenum, a deep antegonial notch on the left side of the mandible and missing left upper central incisor teeth. She was diagnosed as a case of Goldenhar syndrome. Her best-corrected visual acuity was 20 of 20 in the right eye and 20 of 200 in the left eye. Refraction showed astigmatism of 10 diopters in her left eye. She underwent limbal dermoid excision with lamellar keratoplasty in her left eye, after which her astigmatism reduced considerably. Early surgical intervention is recommended in cases of limbal dermoid with high astigmatism as it provides not only good cosmetic but also good visual benefits by early institution of treatment for amblyopia.

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In this report we illustrate the ophthalmologic assessment of two patients affected by Proteus Syndrome (PS), an extremely rare genetic disorder. Case #1 describes a 26 year old male patient followed for multiple ophthalmic anomalies: a limbal dermoid cyst, a unilateral cataract, bilateral nystagmus, severe myopia and unilateral optic nerve head drusen. Case #2 describes a 20 year old female patient referred to our Ophthalmology Department for a routine ophthalmologic evaluation after being treated for 3 years with Miransertib (an experimental AKT-pathway inhibitor). Both patients underwent a complete ophthalmologic examination and a multimodal imaging evaluation. The multimodal imaging approach has revealed useful to evaluate both cases in detail and to keep track of disease evolution over time, moreover providing helpful features to further characterize this rare syndrome.

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We present a case of SCALP syndrome, which was diagnosed in a male infant with the characteristic findings of sebaceous nevi, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and giant congenital melanocytic nevi, or pigmented nevi. We identified a germline compound heterozygous DOCK6 mutation and a somatic mosaic NRAS Q61R mutation in the giant congenital melanocytic nevus. This report will increase clinician awareness of SCALP syndrome and augment the literature in characterizing this rare syndrome, including its genetic background.

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Limbal dermoid (LD) is a congenital ocular tumor that causes amblyopia and damages visual acuity (VA) and visual function. This study evaluated the therapeutic efficacy of perceptual learning (PL) toward improving contrast sensitivity function (CSF) and VA. A total of 25 children with LD and 25 normal children were compared in terms of CSF and VA. The LD group was further randomly allocated into two arms: nine underwent PL combined with patching and eight underwent patching only; eight patients quit the amblyopia treatment. The primary outcome was the area under log CSF (AULCSF), and the secondary outcome was the best corrected VA (BCVA). The CSF was obviously reduced in the LD group compared with that in the normal group. Moreover, the difference in the changes in the AULCSF between the PL and patching groups after 6 months of training was 0.59 (95% CI: 0.32, 0.86, p < 0.001), and the between-group difference in VA at 6 months was −0.30 (95% CI: −0.46, −0.14, p < 0.001). Children suffering from LD with amblyopia exhibited CSF deficits and VA loss simultaneously. PL could improve CSF and VA in the amblyopic eye better than patching.

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BACKGROUND: This study reports a case of pterygium-like proliferation containing postoperative limbal dermoid remnants and its clinicopathological features. CASE PRESENTATION: A 79-year-old Japanese woman, with a history of congenital limbal dermoid resection at age 12, presented with a pterygium-like tissue growth in the left eye. Its temporal location and marked thickness with abundant fatty droplets were atypical of primary pterygium. We performed pterygium surgery and ocular surface reconstruction. Pathological findings included squamous metaplasia, neovessels, and elastic degeneration, as well as prominent subepithelial and stromal accumulation of collagen fibers, adipose tissue formation, and presence of a peripheral nerve corresponded with the frequent findings of limbal dermoid. Ki67, a marker for cell proliferation, was immunopositive in pterygial epithelial cells and neovascular endothelial cells, but not in dermoid components. CONCLUSIONS: Although the pathological finding of degenerative elastic fibers indicated the common feature of ultraviolet-induced pterygium, clinical appearances were atypical possibly due to modification with dermoid remnants.

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PURPOSE: To report the surgical management of extensive epibulbar dermoids with autologous oral mucous membrane transplantation. OBSERVATIONS: While rare, extensive dermoids that encroach upon the visual axis carry a poor prognosis. We report the case of a 7-week old premature male infant who presented with large bilateral epibulbar dermoids obscuring the visual axis. He was treated first with sequential bilateral optical iridectomies under the clearest corneal areas, followed several months later by sequential dermoid excision and amniotic membrane transplantation in each eye. He subsequently underwent autologous "simple" oral mucosal epithelial transplantation (SOMET) as well as strabismus surgery. Conclusions and Importance: Here we present the first case, to the best of our knowledge, of the use of SOMET in managing post-operative pseudopterygium following dermoid excision. To our knowledge it is the also the first application of this technique in a young pediatric patient. A good clinical outcome may be achieved with SOMET, which may offer a minimally invasive alternative to other traditional modalities.

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PURPOSE: To study the ophthalmic clinical profile and the management outcomes of children having Goldenhar syndrome (GS). METHODS: Retrospective review of included children fulfilling the diagnostic criteria of GS was performed. The demography, ophthalmic features, systemic anomalies, and treatment outcomes were recorded manually from patient files. An ENT, pediatrics, cardiology, and orthopedics consultation was sought for all GS patients before taking up for any ophthalmic surgical procedure. The anatomical (ocular surface and eyelid) and functional {vision and extraocular movements (EOM)} factors were exclusively studied. We ensured a minimum postoperative follow-up of 12 months, and our data were compared with the major studies featuring 'ophthalmic features' of GS. RESULTS: Totally 30 children (females = 18, 60%) were included with a median presenting age of 48 months. Twenty-seven (90%) had unilateral ophthalmic involvement with major features being upper eyelid coloboma (n = 25, 75.76%), lipodermoid (n = 18, 54.55%), and limbal dermoid (n = 10, 30.3%). Diminution of visual acuity was recorded in 22 (73.3%), while five (16.7%) had limitation of EOM. Systemically, the prominent features included hemifacial hypotrophy (100%), auricular anomalies (80%), cardiac anomalies (10%), and scoliosis (6.67%). The eyelid colobomas were repaired with the direct closure ± cantholysis technique or using a Tenzel's flap. All children had satisfactory anatomical and functional outcomes after ophthalmic surgical interventions without any significant complications. CONCLUSION: The tailored ophthalmic surgical intervention(s) provides satisfactory restoration of anatomy and functionality of the eye. These children need specific multi-discipline consultations for the holistic management and complete care.

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PURPOSE: To report a novel technique to treat superficial limbal dermoid by using the corneal intrastromal lenticules obtained from SMILE and to evaluate the initial clinical outcomes of lenticule patch graft for treatment of limbal dermoid. METHODS: In this single-center case series, lenticules were obtained from patients undergoing SMILE for the correction of myopia and the lenticule patch graft was performed in three patients with limbal dermoid. Patients were postoperatively followed at first, second weeks and first, third months after surgery. The main measured outcome parameters were included the best-corrected visual acuity, transparency of graft, tectonic integrity and restoration of optical transparency. RESULTS: All surgeries were successful, and all patients recovered well. Obviously, improving tendency in visual acuity and astigmatism was seen at the final follow-up in all eyes. No statistically significant difference has found in the thickness of the cornea between the surgical field and the corresponding normal field. Globe integrity was achieved in all cases, and no immune rejection or perforation was detected until the last follow-up visit in all eyes treated. CONCLUSIONS: Lenticule keratoplasty may be a safe, feasible and inexpensive surgical option for the treatment of corneal dermoid. It retains globe integrity and decreases the risk of complications such as postoperative limbal ectasia and visible corneal scarring and should be widely promoted in the areas with shortage of donor corneas.

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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.

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PURPOSE: To evaluate the safety and efficacy of lamellar keratoplasty using preserved donor corneas to treat limbal dermoids. STUDY DESIGN: Retrospective study. METHODS: The clinical records of 19 patients with limbal dermoids, who underwent lamellar keratoplasty using preserved corneas that were observed for more than 6 months at the Keio University School of Medicine between January, 2000 and December, 2017, were retrospectively reviewed. We retrospectively analyzed demographics, surgical outcomes, the occurrence of any surgically induced changes in refraction, and intra and postoperative complications. RESULTS: Patient age at surgery showed 2 peaks, the first ranged from 0 to 6 years, and the second from 13 to 20 years. All patients except one had good cosmetic results. Preoperative astigmatism was more than 2 diopters in 12 of 16 eyes for which refractive data were recorded. The refractive cylinder in 8 of the 16 eyes differed after surgery by less than 2 diopters. Treatment of amblyopia by occlusion of the fellow eye and spectacle prescription was done either prior to or following surgery, and resulted in improved visual acuity in 7 patients. Intraoperative complications did not occur in any of the patients. Postoperatively, all patients except one showed corneal re-epithelialization within a week. CONCLUSION: Lamellar keratoplasty using preserved corneas for limbal dermoid yields good cosmetic results. However, improvements in astigmatism and visual acuity are not guaranteed. Preoperative treatment of amblyopia gives a better prognosis for improved visual acuity postoperatively. Long-term observation including amblyopia treatment is required before and after surgery.

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Small incision refractive lenticule extraction (SMILE) is a femtosecond laser technique to correct myopia and myopic astigmatism. Herein, we report a technique where intrastromal lenticule obtained from the SMILE procedure served as a graft for lamellar keratoplasty in the management of a limbal dermoid. An 18-year-old woman presented to the clinic with a corneal-limbal mass in the right eye. Slit-lamp examination revealed a vascularized circular mass of approximately 6 mm × 5 mm, which was attached at 7 o'clock in the inferotemporal region of the corneal limbus; this suggested limbal dermoid. Anterior segment optical coherence tomography revealed superficial involvement of the cornea. The patient was treated with excision and lamellar keratoplasty by using femtosecond intrastromal lenticule. The lenticule was sutured over the cornea with 10-0 interrupted nylon sutures. On postoperative follow-up, best-corrected visual acuity was 20/20; there was no corneal neovascularization and no sign of rejection. This case of limbal dermoid was managed by simple surgical excision and lamellar keratoplasty with a SMILE-extracted lenticule. This method may serve as an alternative surgical approach for management of limbal dermoid.

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PURPOSE: To present the ophthalmic manifestations of a 3-month old female with SCALP syndrome. OBSERVATIONS: The patient presented with multiple ocular anomalies including bilateral limbal dermoids, esotropia and left optic nerve hypoplasia. CONCLUSIONS: We describe systemic and ocular anomalies in a rare case of SCALP syndrome. This report provides additional information on the ocular anomalies not previously described that may be associated with this clinical entity.

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To assess the postoperative outcomes of limbal dermoid excision with corneoscleral graft transplantation. The charts of 8 consecutive patients (mean age: 13.0y) who had undergone limbal dermoid excision with lamellar corneoscleral graft transplantation by a single surgeon were retrospectively reviewed. Mean dermoid size was 7.75 mm (6.0-12.0 mm). Mean visual acuities (in logMAR units) before and after surgery were 1.8 and 1.7, respectively (P=0.29). Spherical equivalents were 1.3 diopter (D) before surgery and 0.7 D after surgery (P=0.40). The mean astigmatism measurements before and after surgery were 2.4 D and 1.5 D, respectively (P=0.17). Vector analysis revealed a mild change in astigmatism with a mean "d" of 3.2 (0.56-6.89). No intra- or post-operative complications occurred. Lamellar keratoplasty for limbal dermoids is safe and offers good cosmesis and tectonic stability. A significant decrease in the amount of astigmatism is not expected following surgery.

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Pai Syndrome is a rare congenital malformation syndrome of unknown cause with hypertelorism, midline cleft lip, nasal and facial polyps, ocular anomalies and the presence of distinctive lipomas adjacent to the corpus callosum. Herein, we present an infant girl with Pai Syndrome diagnosed in the first week of life with typical facial findings and associated pericallosal lipoma identified on cranial ultrasound and brain MRI. These typical features identified included median cleft of the upper lip (in her case as a forme fruste) with a cleft alveolus and a mid-anterior alveolar process congenital polyp. In addition to these findings there was mild hypertelorism and an ocular abnormality on the right eye. An ophthalmology assessment on day 5 identified the ocular lesion as a limbal dermoid. Several ocular anomalies have been reported in association with Pai Syndrome, however, dermoids have not been frequently described in this Syndrome and not before in a limbal location. Increasing identification of previously unreported ocular abnormalities in Pai Syndrome may improve diagnosis and may prove useful in future work attempting to elucidate the aetiology of this rare syndrome.

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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.

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