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    Ocular dermoid in Pai Syndrome: A review.
    Eur J Med Genet 2017 Jan 23. Epub 2017 Jan 23.
    National Maternity Hospital, Holles St., Dublin, D02 YH21, Ireland; Children's University Hospital, Temple Street, Dublin, D01 YC67, Ireland. Electronic address:
    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. Read More

    Preoperative evaluation and outcome of corneal transplantation for limbal dermoids: a ten-year follow-up study.
    Int J Ophthalmol 2016 18;9(12):1756-1760. Epub 2016 Dec 18.
    Shandong Eye Hospital, Shandong Eye Institute, Shandong Academy of Medical Sciences, Jinan 250021, Shandong Province, China.
    Aim: To summarize preoperative evaluation and outcome of corneal transplantation for limbal dermoids for ten years.

    Methods: Eighty-five patients diagnosed with limbal dermoids and treated with corneal transplantation were analyzed retrospectively. All patients were further divided into two groups according to absence or presence of neovascularization surrounding the dermoids in the corneal stroma. Read More

    Late Recognition of a Case of Oculo-Auriculo-Vertebral Spectrum.
    Optom Vis Sci 2016 Nov;93(11):1449-1453
    *MD, PhD †MD, FRCS, FRCOphth Department of Ophthalmology, King Faisal Specialist Hospital & Research Center, Riyadh, Kingdom of Saudi Arabia (JEM); Department of Surgery, North Bronx Health Network, Bronx, New York (JNM); and Department of Ophthalmology and Visual Sciences, Albert Einstein College of Medicine of Yeshiva University, Bronx, New York (JNM).
    Purpose: To report the late recognition of a case oculo-auriculo-vertebral spectrum (OAVS).

    Case Report: A 14-year-old male patient with a history of spinal scoliosis and anal atresia presented with a right ocular growth that was increasing in size and causing irritation. He was discovered to have a right limbal dermoid and forniceal lipodermoid. Read More

    Phenotypic characterization of epibulbar dermoids.
    Int Ophthalmol 2016 Jul 12. Epub 2016 Jul 12.
    Seattle Children's Research Institute, Seattle, WA, USA.
    The purpose of this study was to characterize the phenotypic presentation, clinical course, and outcomes of epibulbar dermoids (EpDs) which are the most common congenital eye tumor in children. Sixty-eight dermoids were identified in 58 eyes of 48 patients of Seattle Children's Hospital between 1981 and 2014 via electronic medical record search. Patients were organized into: "EpD-Only" [patients without other congenital anomalies (n = 13)], "EpD-CFM" [patients with a craniofacial microsomia (CFM) diagnosis (n = 25)], and "EpD-Other" [patients with other congenital anomalies (n = 10)]. Read More

    Sutureless Lamellar Corneoscleral Patch Graft With Fibrin Sealant for Limbal Dermoid Removal.
    J Pediatr Ophthalmol Strabismus 2016 Jun 3;53 Online:e22-5. Epub 2016 Jun 3.
    A 2-year-old boy with Goldenhar syndrome had a limbal dermoid removed and covered with a lamellar corneoscleral patch graft that was attached with fibrin glue and no sutures. The graft healed and attached well. A sutureless technique is beneficial due to decreased scarring and chance of infection. Read More

    Shave Excision With Keratopigmentation for Limbal Dermoid.
    Eye Contact Lens 2016 Apr 6. Epub 2016 Apr 6.
    Division of Cornea and Refractive Surgery (P.B.), Department of Ophthalmology, Doctor Eye Institute, Mumbai, Maharashtra, India; and Division of Cornea and Refractive Surgery (T.S., T.A.), Department of Ophthalmology, Rajendra Prasad Institute of Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India.
    Objectives: To describe a modified technique of corneal tattooing for concomitant cosmetic rehabilitation in eyes with limbal dermoid.

    Study: Case series.

    Methods: Three patients between 12 and 20 years of age with grade I limbal dermoid underwent shave excision with corneal tattooing. Read More

    A microanatomic abnormality of the lacrimal gland associated with Goldenhar syndrome.
    Surv Ophthalmol 2016 Sep-Oct;61(5):654-63. Epub 2016 Feb 16.
    Department of Pathology, Wills Eye Institute, Jefferson Medical College, Philadelphia, Pennsylvania, USA.
    A 12-month-old male infant, noted from birth to have a diffuse right temporal epibulbar thickening that encroached on the limbus inferotemporally, was found to manifest stigmata of Goldenhar syndrome, including a limbal dermoid with vellus hairs, esotropia, astigmatism, fullness and ectropion of the lower eyelid, preauricular skin tag, agenesis of the right kidney, and a supernumerary rib. In the excised epibulbar specimen, in addition to a solid dermoid, lobules of lacrimal gland tissue were interpreted as a portion of the palpebral or orbital lobes. This tissue displayed a unique histopathologic finding. Read More

    Clinical decision upon resection or observation of ocular surface dermoid lesions with the visual axis unaffected in pediatric patients.
    Springerplus 2015 21;4:534. Epub 2015 Sep 21.
    Department of Ophthalmology, Okayama University Medical School and Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama City, 700-8558 Japan.
    Ocular surface or epibulbar dermoid lesions may present as limbal dermoids at the corneal limbus or dermolipomas in the conjunctival fornix. The purpose of this study is to review clinical features of ocular surface dermoids (grade I), with the visual axis unaffected, in pediatric patients, in order to find key features for making clinical decision, either resection or observation. The study involved 13 consecutive patients with limbal dermoids or fornix dermolipomas which did not affect the visual axis, seen in 11 years at a referral-based institution. Read More

    Transcription Factor PAX6 (Paired Box 6) Controls Limbal Stem Cell Lineage in Development and Disease.
    J Biol Chem 2015 Aug 4;290(33):20448-54. Epub 2015 Jun 4.
    the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou 510060, China, the Department of Ophthalmology, Biomaterials and Tissue Engineering Center, Institute for Engineering in Medicine.
    PAX6 is a master regulatory gene involved in neuronal cell fate specification. It also plays a critical role in early eye field and subsequent limbal stem cell (LSC) determination during eye development. Defects in Pax6 cause aniridia and LSC deficiency in humans and the Sey (Small eye) phenotype in mice (Massé, K. Read More

    Congenital complex corneal choristoma associated with unilateral bony calvarial defects, subcutaneous nodules, and alopecia.
    J AAPOS 2015 Apr;19(2):185-8
    Department of Ophthalmology and Visual Sciences, University of Utah, Salt Lake City, Utah. Electronic address:
    Complex corneal choristoma is a rare finding and its relationship with systemic disease is poorly understood. We present a 3-day-old boy with the constellation of left-sided congenital complex corneal choristoma, limbal dermoid, ipsilateral bony skull defects, and bilateral subcutaneous vascularized nodules with overlying alopecia and scalp nevi. To our knowledge this is the first case of complex choristoma involving the presence of associated bony calvarial defects. Read More

    New surgical approach for limbal dermoids in children: simple excision, corneal tattooing, and sutureless limboconjunctival autograft.
    Cornea 2015 Jun;34(6):720-3
    *Department of Ophthalmology, Jeju National University College of Medicine, Jeju, Korea; and †Department of Ophthalmology, Seonam University College of Medicine, Myongji Hospital, Goyang, Korea.
    Purpose: To evaluate the efficacy of combined surgery of simple excision, corneal tattooing, and a sutureless limboconjunctival autograft in pediatric patients with grade 1 limbal dermoids.

    Methods: We treated 4 eyes of 4 patients with combined surgery of corneal tattooing and a sutureless limboconjunctival autograft with simple excision of limbal dermoids. Preoperative and postoperative visual acuity, postoperative recovery time, cosmetic improvements, patient compliance, and complication rates were reviewed. Read More

    Corneal Fine Needle Diathermy With Adjuvant Bevacizumab to Treat Corneal Neovascularization in Children.
    Cornea 2015 Jul;34(7):773-7
    Department of Ophthalmology and Vision Sciences, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
    Purpose: To report the outcomes of corneal fine needle diathermy (FND) with adjuvant intrastromal and subconjunctival bevacizumab injection for corneal neovascularization (CN) in children.

    Methods: Medical records of all children who had undergone FND with adjuvant bevacizumab injection were reviewed retrospectively. Treatment efficacy was evaluated by changes in visual acuity, regression of CN, and clearing of lipid deposits with the aid of slit-lamp color images that were taken before surgical intervention and at last follow-up visit. Read More

    Encephalocraniocutaneous lipomatosis: a case report and review of the literature.
    Indian J Ophthalmol 2014 May;62(5):622-7
    Department of Ophthalmology, Shyam Shah Medical College, Rewa, Madhya Pradesh, India.
    Encephalocraniocutaneous lipomatosis (ECCL) is a rare, sporadic congenital neurocutaneous disorder that characteristically involves ectomesodermal tissues, such as skin, eyes, and central nervous system. A 3-day-old girl presented with swelling in her right eye since birth. Ocular examination of the right eye showed hypertrophy of bulbar conjunctiva with limbal dermoid, clouding of cornea, and atypical upper eyelid coloboma. Read More

    The outcomes of primary pediatric keratoplasty in Singapore.
    Am J Ophthalmol 2014 Sep 27;158(3):496-502. Epub 2014 May 27.
    Singapore National Eye Centre, Singapore; Singapore Eye Research Institute, Singapore; Duke-National University of Singapore Graduate Medical School Singapore, Singapore; Department of Ophthalmology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore. Electronic address:
    Purpose: To evaluate the long-term corneal graft survival and risk factors for graft failure in pediatric eyes.

    Design: Retrospective, interventional consecutive case series.

    Methods: Unilateral eyes of 105 patients aged 16 years and below were included from the Singapore Corneal Transplant Study between April 4, 1991 and April 4, 2011. Read More

    Surgical management of corneal limbal dermoids: retrospective study of different techniques and use of Mitomycin C.
    Eye (Lond) 2014 Jul 23;28(7):857-62. Epub 2014 May 23.
    Department of Ophthalmology, University Eye Hospital Freiburg, Freiburg im Breisgau, Germany.
    Purpose: We retrospectively studied the efficacy and safety of different surgical approaches to treating pediatric limbal dermoids with regard to intra and postoperative complications.

    Methods: The data of 12 consecutive patients (14 eyes), who underwent monocentric surgery of a limbal demoid in the past 9 years, were retrospectively analyzed for intra and postoperative complications. Group one consists of eleven eyes: seven eyes with a bare-sclera deep lamellar excision of the dermoid and four eyes with an additional amniotic membrane transplantation subsequent to excision. Read More

    Unilateral Morning Glory Disc Anomaly With Ipsilateral Limbal Dermoids.
    J Pediatr Ophthalmol Strabismus 2014 May 28;51 Online:e37-9. Epub 2014 May 28.
    The authors present the case of a child with multiple limbal dermoids and morning glory disc anomaly in the right eye. These anomalies are rarely associated and this is the first report of co-occurrence in the same eye in a patient without additional systemic anomalies. Read More

    In vivo confocal microscopy in goldenhar syndrome: a case report.
    BMC Ophthalmol 2013 Oct 16;13:55. Epub 2013 Oct 16.
    Cornea and Ocular Surface Unit, San Raffaele Scientific Institute, Milan, Italy.
    Background: Goldenhar Syndrome is characterized by malformations of multiple anatomical districts. Between these, bulbar dermoids are common and represent a significant clinical problem as they can affect both ocular function and aesthetic comfort.Histologic characterization of dermoids has been extensively performed; however, no reports exist describing in vivo confocal microscopy (IVCM) of these lesions. Read More

    Simple keratectomy and corneal tattooing for limbal dermoids: results of a 3-year study.
    Int J Ophthalmol 2013 18;6(4):463-6. Epub 2013 Aug 18.
    Department of Ophthalmology, Jeju National University College of Medicine, Jeju National University Hospital, Jeju, Korea.
    Aim: To evaluate and report the efficacy of combined surgical excision and corneal tattooing in patients with limbal dermoids.

    Methods: In a retrospective interventional case series, 9 eyes of 8 patients were treated with combined surgery of simple keratectomy and corneal tattooing for limbal dermoids. Medical records, including best-corrected visual acuity, anterior segment photography, demographic, clinical data, and follow-up information were reviewed. Read More

    Encephalocraniocutaneous Lipomatosis (Haberl and syndrome): A case report and review of literature.
    Indian J Dermatol 2013 May;58(3):232-4
    Department of Pathology, NRI Medical College and General Hospital, Guntur District, Andhra Pradesh, India.
    Encephalocraniocutaneous lipomatosis (ECCL) is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Read More

    Limbal dermoid in Nager acrofacial dysostosis: a rare case report.
    Indian J Ophthalmol 2014 Mar;62(3):339-41
    Department of Oral Medicine and Radiology, Subharti Dental College and Hospital, Meerut, Uttar Pradesh, India.
    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. Read More

    Limbal dermoid in Nager syndrome acrofacial dysostosis: A rare case report.
    Indian J Ophthalmol 2013 Apr 26. Epub 2013 Apr 26.
    Department of Oral Medicine and Radiology, Subharti Dental College and Hospital, Meerut, Uttar Pradesh, India.
    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. Read More

    Management of pediatric corneal limbal dermoids.
    Clin Ophthalmol 2013 28;7:607-14. Epub 2013 Mar 28.
    Tayani Institute, Division of Ophthalmology and Cornea, Mission Viejo in affiliation with Children's Hospital of Orange County at Mission Hospital, CA, USA ; Rady's Children's Hospital of San Diego, San Diego, CA, USA.
    This paper reviews the data in the published literature (PubMed from 1937 to 2011) concerning the medical and surgical management of pediatric limbal dermoids. Current standard medical treatment for grade I pediatric limbal dermoids (ie, with superficial corneal involvment) is initially conservative. In stages II (ie, affecting the full thickness of the cornea with/without endothelial involvement) and III (ie, involvement of entire cornea and anterior chamber), a combination of excision, lamellar keratoplasty, and amniotic membrane and limbal stem cell tranplantation are advocated. Read More

    Limbal dermoid epithelium shares phenotypic characteristics common to both hair epidermal and limbal epithelial stem cells.
    Curr Eye Res 2013 Aug 10;38(8):835-42. Epub 2013 Apr 10.
    Save Sight Institute, University of Sydney, NSW, Australia.
    Purpose: To determine putative limbal epithelial stem cell marker expression in human limbal dermoids compared to stem cell niches in normal limbus and hair follicles of normal human dermis.

    Methods: Human limbal dermoids (n = 7), normal skin (n = 2) and normal limbal (n = 7) tissue were examined. Immunohistochemistry was performed on paraffin embedded specimens using automated and manual immunostaining with primary antibodies to CK15, CK14, Cadherin-P (CDH3), Wnt-3, Wnt-4, Wnt-5a, Dickkopf (DKK)-3, Sox-2, Sox-10, Sox-13, PEDF, NGFR p75 and β-catenin. Read More

    Deep anterior lamellar keratoplasty in children.
    Am J Ophthalmol 2013 Mar 4;155(3):570-574.e1. Epub 2012 Dec 4.
    L.V. Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, India.
    Purpose: To evaluate the outcomes of deep anterior lamellar keratoplasty (DALK) in children.

    Design: Retrospective interventional case series.

    Methods: setting: Institutional, L. Read More

    Studies of a pedigree with limbal dermoid cyst.
    Int J Ophthalmol 2012 18;5(5):641-3. Epub 2012 Oct 18.
    Ophthalmic Laboratories and Department of Ophthalmology, Translational neuroscience Center, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
    Aim: To study clinical features and gene mutations within the paired-like homeodomain transcription factor 2 (PITX2) gene in a pedigree of bilateral limbal dermoids.

    Methods: Complete eye examinations have been performed on each individual of the family. Exons of paired-like homeodomain transcription factor 2 (PITX2) were amplified by polymerase chain reaction, sequenced, and compared with a reference database. Read More

    An unusual association of Goldenhar syndrome.
    Int Ophthalmol 2013 Feb 29;33(1):91-4. Epub 2012 Sep 29.
    Department of Ophthalmology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey.
    Goldenhar syndrome is well known for its classical triad of epibulbar dermoids or lipodermoids, auricular appendages and pretragal fistulas. Here we report a case of Goldenhar syndrome with an unusual association of a fibroepithelial polyp attached to a limbal dermoid. A case of Goldenhar syndrome in a 5-month-old male infant presented with the features of a fibroepithelial polyp attached to a limbal dermoid, right-sided polydactylia with hypoplastic thumb, and accessory preauricular appendages on the left side. Read More

    Thrombophilia gene mutations in oculoauriculovertebral spectrum.
    Genet Couns 2012 ;23(1):65-72
    Department of Medical Genetics, Gazi University, Faculty of Medicine, Ankara, Turkey.
    Oculoauriculovertebral spectrum or Goldenhar syndrome is a phenotypically and probably genetically heterogeneous disorder characterized by hemifacial microsomia (unilateral ear abnormalities and ipsilateral mandibular hypoplasia) as well as vertebral anomalies and epibulbar dermoid/lipodermoids. Although most cases of the Goldenhar syndrome are sporadic, both autosomal recessive and dominant inheritance have been reported so far. In this report, we describe the clinical aspects of two familial cases with evidence of autosomal dominant inheritance and a non-familial case, and compare them with the reports in the literature. Read More

    Sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus syndrome.
    Pediatr Dermatol 2012 May-Jun;29(3):365-7. Epub 2011 Nov 28.
    Department of Dermatology and Mackay Memorial Hospital, Taipei, Taiwan.
    SCALP syndrome is an acronym describing the coincidence of sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus). We present a fourth case of this syndrome. Read More

    Surgical management of pediatric limbal dermoids with sutureless amniotic membrane transplantation and augmentation.
    J Pediatr Ophthalmol Strabismus 2012 Mar-Apr;49(2):114-9. Epub 2011 Aug 30.
    Gavin Herbert Eye Institute, Department of Ophthalmology, University of California, Irvine, Med Surge I, Irvine, CA 92697, USA.
    Purpose: To further evaluate the efficacy of a new surgical technique for removal of pediatric corneal-limbal dermoids and ocular surface reconstruction using multilayered amniotic membrane.

    Methods: Three pediatric patients with corneal-limbal dermoid (grade I) in one practice were identified in a retrospective fashion. All patients underwent deep lamellar excision followed by sutureless multilayered amniotic membrane transplantation by a single surgeon (AP). Read More

    [A case of neurocutaneous melanosis associated with focal cortical dysplasia].
    No To Hattatsu 2011 Jul;43(4):300-3
    Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo.
    A newborn baby boy presented with giant melanocytic nevi on the face, trunk and extremities, and focal cortical dysplasia on MRI. At 3 months of age, he developed intractable epilepsy, and MRI at 2 years of age revealed a high-intensity area in the bilateral cerebellum on T1-weighted images, indicative of melanosis. Based on the findings of the skin and MRI, we diagnosed the boy with neurocutaneous melanosis. Read More

    Ophthalmic pterygium: a stem cell disorder with premalignant features.
    Am J Pathol 2011 Feb;178(2):817-27
    Inflammation and Infection Research Centre, School of Medical Sciences, University of New South Wales, Sydney, Australia.
    Pterygia are common ocular surface lesions thought to originate from limbal stem cells altered by chronic UV exposure. Traditionally regarded as a degenerative condition, pterygia also display tumor-like features, such as a propensity to invade normal tissue and high recurrence rates following resection, and may coexist with secondary premalignant lesions. This study was initiated to determine the rate of concurrent ocular surface diseases in patients with pterygia recruited from the practice of a single surgeon operating in a Sydney metropolitan hospital. Read More

    Fibrin-glue assisted multilayered amniotic membrane transplantation in surgical management of pediatric corneal limbal dermoid: a novel approach.
    Graefes Arch Clin Exp Ophthalmol 2011 Feb 7;249(2):261-5. Epub 2010 Sep 7.
    Department Ophthalmology, Kaiser Permanente Medical Group, 710 Lawrence Expressway, #490, Santa Clara, CA 95051, USA.
    Purpose: To report a new surgical technique for excising pediatric corneal limbal dermoid and the post-resection ocular surface reconstruction.

    Methods: We describe a method of deep lamellar excision followed by sutureless multilayered amniotic membrane transplantation in surgical management of corneal limbal dermoid.

    Result: This technique achieves a rapid corneal re-epithelization, reduces post-operative pain, and will diminish post-operative scarring. Read More

    Repair of limbal dermoid with excision and placement of a circumlimbal pericardial graft.
    Eye Contact Lens 2010 Jul;36(4):228-9
    Department of Ophthalmology, SUNY Downstate Medical Center, Brooklyn, NY 11203, USA.
    Objectives: To report the treatment of a limbal dermoid tumor with excision of the lesion combined with the placement of a processed pericardial graft.

    Methods: A case report of a new approach to treat large limbal dermoids after excision of the lesion from the cornea and limbus.

    Results: The surgical result was very reasonable from a cosmetic result, and the patient recovered quite well. Read More

    The group of epidermal nevus syndromes Part II. Less well defined phenotypes.
    J Am Acad Dermatol 2010 Jul;63(1):25-30; quiz 31-2
    Department of Dermatology, University of Marburg, Marburg, Germany.
    Unlabelled: In addition to the well established epidermal nevus syndromes, such as Schimmelpenning, Becker, Proteus, CHILD, or nevus comedonicus syndromes, the group of less well defined phenotypes associated with epidermal nevi presently includes nevus trichilemmocysticus syndrome, didymosis aplasticosebacea, SCALP syndrome (sebaceous nevus, central nervous system malformations, aplasia cutis congenita, limbal dermoid,and pigmented nevus), Gobello syndrome, Bäfverstedt syndrome, NEVADA syndrome (nevus epidermicus verrucosus with angio-dysplasia and aneurysms), and CLOVE syndrome (congenital lipomatous overgrowth, vascular malformations, and epidermal nevus). These syndromes can thus far be regarded as being in limbo. Future clinical and molecular research may show which of these disorders can be added to the list of well defined nosologic entities. Read More

    Limbal Stem Cell Transplantation for Limbal Dermoid.
    Ophthalmic Surg Lasers Imaging 2010 Mar 9:1-2. Epub 2010 Mar 9.
    Limbal dermoids are benign peripheral corneal neoplasms, which cause cosmetic problems and visual impairment. Deep dermoids may require corneal and scleral dissections such as a lamellar or even a penetrating keratoscleroplasty. In these 2 cases, the authors used an autologous limbal stem cell transplantation and achieved good results. Read More

    Epicorneal polypoidal lipodermoid: lack of association of central corneal lesions with goldenhar syndrome verified with a review of the literature.
    Surv Ophthalmol 2010 Jan-Feb;55(1):78-84
    Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA.
    It is remarkable to uncover a new aspect of congenital epibulbar solid dermoids and lipodermoids. We describe a dramatic central epicorneal polypoidal lipodermoid coloboma accompanied by an upper eyelid coloboma that was not associated with Goldenhar syndrome. Histopathologically the excised lesion displayed superficial layers of epidermis and a thin dermis with eccrine glands, vestigial hair structures, and bundles of arrector pili smooth muscle that extended from the undersurface of the epidermis to the bulge area of the primitive hairs. Read More

    Visual outcome and corneal topography after eccentric "shaped" corneal grafts.
    Cornea 2009 May;28(4):379-84
    Institute of Ophthalmology, Joseph Eye Hospital, Tiruchirapalli 620 001, Tamil Nadu, India.
    Purpose: To study the clinical and visual outcome and corneal topography in eyes that underwent full-thickness or lamellar eccentric-"shaped" (biconvex/crescentic) corneal grafts.

    Setting: Tertiary care center in south India.

    Methods: In this retrospective, interventional, noncomparative case series, eccentric-shaped corneal grafts were performed in 10 eyes (10 patients). Read More

    Transplanted corneal graft with metastatic cholangiocarcinoma to the donor eye.
    Eye Contact Lens 2008 Nov;34(6):340-2
    Department of Ocular Surface Disorders, Shenzhen Ophthalmic Center Affiliated Ji'nan University, Shenzhen, China.
    Objectives: To report a case of corneal transplantation from a donor died of cholangiocarcinoma with metastasis to the eye.

    Methods: A patient with limbal dermoid received corneal transplant from a donor died of cholangiocarcinoma. Pathologic examination of the remaining donor limbal tissue revealed metastasis of tumor to the limbal vessels. Read More

    Congenital eye and adnexial anomalies in Kano, a five year review.
    Niger J Med 2008 Jan-Mar;17(1):37-9
    Department of Ophthalmology, Bayero University Kano, Nigeria.
    Background: Knowledge of prevalence and types of congenital eye and adnexial anomalies is important as some of these anomalies can lead to childhood blindness. The aim of the study is to determine the types of congenital eye and adnexial anomalies seen in the eye clinic of Aminu Kano Teaching Hospital Kano, Nigeria between the years 2001 to 2005. To determine the proportion of children below the age of 10 years who presented with congenital eye and adnexial anomalies, the proportion of eye surgeries due to such anomalies, and proportion of these anomalies amenable to (surgical) intervention. Read More

    SCALP syndrome: sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: a distinct syndromic entity.
    J Am Acad Dermatol 2008 May 30;58(5):884-8. Epub 2008 Jan 30.
    Rady Children's Hospital, San Diego, California, USA.
    Nevus sebaceus syndrome (SNS) is a constellation of nevus sebaceus with extracutaneous findings, including the ophthalmologic nervous, and musculoskeletal systems. Didymosis aplasticosebacea is a recently described entity consisting of aplasia cutis congenita and nevus sebaceus, implying twin spotting (didymosis). We describe a neonate with a nevus sebaceus on the scalp and a limbal dermoid on her left eye. Read More

    [Two cases of dermolipoma].
    Nippon Ganka Gakkai Zasshi 2007 Dec;111(12):965-9
    Department of Ophthalmology, Aichi Medical University, Japan.
    Background: Dermolipoma is an uncommon benign tumor, congenitally occurring on the conjunctiva, and may be present at other sites. The appearance of dermolipoma closely resembles orbital fat prolapse and limbal dermoid, and therefore it is necessary to take this into account in diagnosis. Postoperative complications such as blepharoptosis, diplopia, or keratoconjunctivitis sicca can develop. Read More

    Atypical oculo-orbital complex choristoma in organoid nevus syndrome.
    J Pediatr Ophthalmol Strabismus 2006 Mar-Apr;43(2):119-22
    Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi.
    We report organoid nevus with left oculo-orbital choristoma fixing the globe in hypotropia, precanthal skin tag, and upper eyelid coloboma and right limbal dermoid. Computed tomography revealed a dilated lateral ventricle and cerebral atrophy. No neurologic deficit existed. Read More

    Full-thickness central corneal grafts in lamellar keratoscleroplasty to treat limbal dermoids.
    Ophthalmology 2005 Nov 15;112(11):1955. Epub 2005 Sep 15.
    Department of Ophthalmology, National Taiwan University Hospital, Taipei, Taiwan.
    Purpose: To assess the surgical outcomes in patients who underwent lamellar keratoscleroplasty with full-thickness central corneal grafts for limbal dermoids.

    Design: Retrospective, noncomparative, analysis of a consecutive case series with chart review.

    Participants: Ten patients (6 male, 4 female; mean age, 12. Read More

    Corneal keloid mimicking a recurrent limbal dermoid.
    J Pediatr Ophthalmol Strabismus 2005 May-Jun;42(3):189-90
    Department of Ophthalmology, University of Texas Health Science Center at San Antonio, San Antonio, Texas 78229, USA.
    We report a 12-year-old boy who presented with the clinical appearance of a limbal dermoid recurrence. Histologic study of the lesion revealed it to be a corneal keloid. In the event of a recurrent mass following dermoid excision, keloid should be considered in the differential diagnosis. Read More

    [Epibulbar dermoids--clinical features and therapeutic methods].
    Klin Monbl Augenheilkd 2004 Oct;221(10):872-7
    Klinik und Poliklinik für Augenheilkunde, Medizinische Fakultät der Technischen Universität Dresden.
    Background: In a strict sense epibulbar dermoids are defined as congenital changes of mesodermal and ectodermal origin. Usually they are located at the limbus of the cornea in the inferotemporal quadrant, they can only rarely be observed in more central regions of the cornea. Intraocular structures are rarely involved. Read More

    [Solid limbal dermoid in an 80-year-old patient].
    Klin Monbl Augenheilkd 2004 Sep;221(9):776-80
    Augenklinik mit Poliklinik der Universität Erlangen-Nürnberg, Erlangen.
    Background: Typically solid limbal dermoids are excised in pre-school age unless a high irregular astigmatism and its risk for amblyopia lead to an earlier intervention.

    Case Report: An 80-year-old lady from a rural area complained about a burning, tearing and foreign body sensation of the left eye for two months. In the past two years she had recognized that an extraocular prominence which had been present since birth had shown a tendency to grow. Read More

    Juvenile xanthogranuloma as an isolated corneoscleral limbal mass: a case report.
    Korean J Ophthalmol 2003 Jun;17(1):63-6
    Department of Ophthalmology, Wonju Christian Hospital, Yonsei University Wonju College of Medicine, Kangwon-do, Korea.
    A case of a juvenile xanthogranuloma of the corneoscleral limbus was encountered in a 5-year-old oriental boy, who presented with a 5-month history of a lump in the right eye. The lesion extended from the inferior limbus. This yellow-orange mass was vascular and firmly fixed to the underlying tissue. Read More

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