scholarly journals Limbal Stem Cell Transplantation for Limbal Dermoid in a Case of Goldenhar Syndrome

2014 ◽  
Vol 1 (2) ◽  
pp. 97
Author(s):  
Prachi Pawar ◽  
Saurabh Shah
Keyword(s):  
Surgical Outcome ◽  
Goldenhar Syndrome ◽  
Limbal Stem Cells ◽  
Ocular Abnormalities ◽  
Branchial Arches ◽  
Skin Tags ◽  
Stem Cells Transplantation ◽  
Limbal Stem Cell Transplantation ◽  
Limbal Stem Cell ◽  
Limbal Dermoid

Goldenhar Syndrome (known as Oculoauriculovertebral Syndrome (OAVS) or facioauriculovertebral dysplasia) is a rare congenital defect classically involving eye anomalies with or without anomalous development of the ear, nose, lips, soft palate, mandible and vertebra. It is a morphogenetic anomaly associated with the 1st and 2nd branchial arches. We report a case of 9 months old male child presented with ocular abnormalities. On evaluation, he had the classical signs of this syndrome like limbal dermoids, eyelid coloboma, preauricular skin tags and hemi facial microsomia. This patient was first seen in January 2010 and has been followed and treated for the same. The patient underwent a surgery for right eye limbal dermoid at the age of 4 years. The initial presentation with course of the disease and Post-Surgical outcome is described here. The Authors performed dermoid excision with autologous conjunctiva along with limbal stem cells transplantation and achieved a satisfactory surgical outcome.

scholarly journals Do you know this syndrome?

2012 ◽  
Vol 87 (3) ◽  
pp. 495-497
Author(s):  
Luiz Maurício Costa Almeida ◽  
Michelle dos Santos Diniz ◽  
Lorena dos Santos Diniz
Keyword(s):  
Multidisciplinary Approach ◽  
Goldenhar Syndrome ◽  
Clinical Aspects ◽  
Genetic Syndrome ◽  
Live Births ◽  
Mandibular Hypoplasia ◽  
Radiologic Findings ◽  
Branchial Arches ◽  
Skin Tags ◽  
Systemic Manifestations

Goldenhar syndrome is a sporadic or inherited genetic syndrome characterized by limbal dermoids, preauricular skin tags and mandibular hypoplasia. Vertebral abnormalities may occur. The incidence of this syndrome ranges from 1 in 5,600 to 1 in >20,000 live births. It consists of abnormalities involving the first and second branchial arches. The etiology of the syndrome is heterogeneous. Diagnosis should be based principally on clinical aspects, which should be associated with the patient's systemic conditions and radiologic findings. Treatment depends on the patient's age and systemic manifestations, with a multidisciplinary approach often being required.

scholarly journals Goldenhar syndrome: a rare entity

2017 ◽  
Vol 4 (5) ◽  
pp. 1897 ◽  
Author(s):  
Tariq Harris ◽  
Mohammed Abdul Bashith ◽  
Manas M. Shanbhag ◽  
Moideen Faheem
Keyword(s):  
Multidisciplinary Approach ◽  
Systemic Treatment ◽  
Obstructive Hydrocephalus ◽  
Severe Form ◽  
Goldenhar Syndrome ◽  
Branchial Arches ◽  
Hydrocephalus Treatment ◽  
Ophthalmic Treatment ◽  
Cns Malformations ◽  
Limbal Dermoid

Goldenhar syndrome was first described by Maurice Goldenhar in 1952 it involves abnormalities in the first and second branchial arches. This syndrome appears to have varying degree of presentation from mild to severe form. Different pathophysiological mechanisms have been proposed .Oculo auriculo vertebral syndrome which includes reduction in blood flow resulting in focal haemorrhage in the first and second branchial arches. In addition there may be cardiac vertebral and CNS defects. A male baby 30-32 wks, preterm SGA was admitted in NICU for evaluation of Congenital Malformations which includes swelling of the anterior border of both eyes with b/l preauricular ear tags, malformed pinna, high arched palate and micrognatia ophthalmology examination revealed b/l limbal dermoid inferotemporal aspect. ECHO done revealed ASD 4mm.CT scan showed obstructive hydrocephalus. Treatment of the condition varies according to the severity. Ophthalmic treatment aimed first because of amblyogenic risk. Craniofacial surgery in view of severe micrognathia systemic treatment may be related to cardiac, renal and CNS malformations. Goldenhar Syndrome is a rare congenital anomaly, with cosmetic defects whose treatment may pose numerous challenges with multidisciplinary approach for optimal management.

scholarly journals Hyaluronan-Based Hydrogel Scaffolds for Limbal Stem Cell Transplantation: A Review

Cells ◽  
2019 ◽  
Vol 8 (3) ◽  
pp. 245 ◽  
Author(s):  
Mazyar Yazdani ◽  
Aboulghassem Shahdadfar ◽  
Catherine Joan Jackson ◽  
Tor Paaske Utheim
Keyword(s):  
Extracellular Matrix ◽  
Stem Cell ◽  
Limbal Stem Cells ◽  
Receptor Proteins ◽  
Hydrogel Scaffolds ◽  
Cell Based Therapy ◽  
Limbal Stem Cell Transplantation ◽  
Limbal Stem Cell ◽  
Cell Niche ◽  
Synthesis And Degradation

Hyaluronan (HA), also termed hyaluronic acid or hyaluronate, is a major component of the extracellular matrix. This non-sulfated glycosaminoglycan plays a key role in cell proliferation, growth, survival, polarization, and differentiation. The diverse biological roles of HA are linked to the combination of HA’s physicochemical properties and HA-binding proteins. These unique characteristics have encouraged the application of HA-based hydrogel scaffolds for stem cell-based therapy, a successful method in the treatment of limbal stem cell deficiency (LSCD). This condition occurs following direct damage to limbal stem cells and/or changes in the limbal stem cell niche microenvironment due to intrinsic and extrinsic insults. This paper reviews the physical properties, synthesis, and degradation of HA. In addition, the interaction of HA with other extracellular matrix (ECM) components and receptor proteins are discussed. Finally, studies employing HA-based hydrogel scaffolds in the treatment of LSCD are reviewed.

scholarly journals A patient with multiple skin tags, preauricular pit, limbal dermoid: Atypic Goldenhar syndrome

TURKDERM ◽  
2014 ◽  
Vol 48 (3) ◽  
pp. 166-168
Author(s):  
Kıymet Handan Kelekci ◽  
Nihal Olgaç Dündar ◽  
Şemsettin Karaca ◽  
Ali Karakuzu ◽  
Onur Er
Keyword(s):  
Goldenhar Syndrome ◽  
Skin Tags ◽  
Limbal Dermoid

scholarly journals Limbal Stem Cell Transplantation: Clinical Results, Limits, and Perspectives

2018 ◽  
Vol 2018 ◽  
pp. 1-12 ◽  
Author(s):  
Marta Sacchetti ◽  
Paolo Rama ◽  
Alice Bruscolini ◽  
Alessandro Lambiase
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Corneal Epithelium ◽  
Clinical Results ◽  
Limbal Stem Cells ◽  
Fellow Eye ◽  
Ocular Surface Reconstruction ◽  
Limbal Stem Cell Transplantation ◽  
Limbal Stem Cell

Limbal stem cell deficiency (LSCD) is a clinical condition characterized by damage of cornea limbal stem cells, which results in an impairment of corneal epithelium turnover and in an invasion of the cornea by the conjunctival epithelium. In these patients, the conjunctivalization of the cornea is associated with visual impairment and cornea transplantation has poor prognosis for recurrence of the conjunctivalization. Current treatments of LSCD are aimed at replacing the damaged corneal stem cells in order to restore a healthy corneal epithelium. The autotransplantation of limbal tissue from the healthy, fellow eye is effective in unilateral LSCD but leads to depauperation of the stem cell reservoir. In the last decades, novel techniques such as cultivated limbal epithelial transplantation (CLET) have been proposed in order to reduce the damage of the healthy fellow eye. Clinical and experimental evidence showed that CLET is effective in inducing long-term regeneration of a healthy corneal epithelium in patients with LSCD with a success rate of 70%–80%. Current limitations for the treatment of LSCD are represented by the lack of a marker able to unequivocally identify limbal stem cells and the treatment of total, bilateral LSCD which requires other sources of stem cells for ocular surface reconstruction.

Autologous Cultivated Limbal Stem Cell Transplantation after Failed Previous Limbal Graft

2017 ◽  
Vol 27 (5) ◽  
pp. e137-e139
Author(s):  
Karl A. Knutsson ◽  
Stanislav Matuska ◽  
Paolo Rama
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Ocular Surface ◽  
Ex Vivo ◽  
Limbal Stem Cells ◽  
Limbal Stem Cell Transplantation ◽  
Limbal Stem Cell ◽  
Contralateral Eye

Purpose To describe a case of unilateral limbal stem cell deficiency (LSCD) with previously failed autologous graft, resolved by ocular surface reconstruction using cultured autologous limbal stem cells from the contralateral eye. Case Report A 35-year-old patient presented to our clinic with LSCD due to a unilateral alkali burn. The patient had received a previous limbal graft from the contralateral eye that had failed to impede corneal conjunctivalization. We decided to repeat limbal stem cell transplantation using an ex vivo cultivation procedure to reduce the risk of tissue harvesting on the healthy fellow eye. A small limbal biopsy (1.5 × 1.5 mm) near the previously excised limbus was performed. Stem cells were then isolated and cultured on fibrin and a 3T3 feeder cell layer using a standard protocol. Four months later, the cultivated cells on fibrin were grafted after pannus removal. In the subsequent months, the ocular surface stabilized and inflammation decreased. Two years later, the patient underwent large tectonic lamellar keratoplasty for severe corneal thinning involving the entire cornea, and 6 months later central penetrating keratoplasty and extracapsular cataract extraction with intraocular lens implantation and pupilloplasty was performed. Following reconstruction, the patient showed improved best-corrected vision from count fingers to 20/200 due to amblyopia, and the ocular surface was stable with a transparent corneal graft. Conclusions Ex vivo limbal stem cell transplantation is a valid technique for treating LSCD and can be utilized for treating patients who have had previous failed limbal grafts.

scholarly journals Effect of Cross-Linking Density on the Structures and Properties of Carbodiimide-Treated Gelatin Matrices as Limbal Stem Cell Niches

2018 ◽  
Vol 19 (11) ◽  
pp. 3294 ◽  
Author(s):  
Jui-Yang Lai ◽  
Li-Jyuan Luo ◽  
David Ma
Keyword(s):  
Surface Roughness ◽  
Stem Cell ◽  
Cross Linking ◽  
Epithelial Tissue ◽  
Human Amniotic Membrane ◽  
Limbal Stem Cells ◽  
Structures And Properties ◽  
Cross Linker ◽  
Limbal Stem Cell ◽  
Stem Cell Niches

Given that human amniotic membrane is a valuable biological material not readily available for corneal epithelial tissue engineering, gelatin is considered as a potential alternative to construct a cellular microenvironment. This study investigates, for the first time, the influence of cross-linking density of carbodiimide-treated gelatin matrices on the structures and properties of artificial limbal stem cell niches. Our results showed that an increase in the carbodiimide concentration from 1.5 to 15 mM leads to an upward trend in the structural and suture strength of biopolymers. Furthermore, increasing number of cross-linking bridges capable of linking protein molecules together may reduce their crystallinity. For the samples treated with 50 mM of cross-linker (i.e., the presence of excess N-substituted carbodiimide), abundant N-acylurea was detected, which was detrimental to the in vitro and in vivo ocular biocompatibility of gelatin matrices. Surface roughness and stiffness of biopolymer substrates were found to be positively correlated with carbodiimide-induced cross-link formation. Significant increases of integrin β1 expression, metabolic activity, and ABCG2 expression were noted as the cross-linker concentration increased, suggesting that the bulk crystalline structure and surface roughness/stiffness of niche attributed to the number of cross-linking bridges may have profound effects on a variety of limbal epithelial cell behaviors, including adhesion, proliferation, and stemness maintenance. In summary, taking the advantages of carbodiimide cross-linking-mediated development of gelatin matrices, new niches with tunable cross-linking densities can provide a significant boost to maintain the limbal stem cells during ex vivo expansion.

scholarly journals A Review of Limbal Stem Cell Deficiency

2019 ◽  
Vol 2 (1) ◽  
pp. e4-e11
Author(s):  
John E. Conto
Keyword(s):  
Stem Cell ◽  
Contact Lenses ◽  
Systemic Disease ◽  
Anterior Segment ◽  
Treatment Strategies ◽  
Limbal Stem Cell Deficiency ◽  
Contact Lens Wear ◽  
Limbal Stem Cell Transplantation ◽  
Limbal Stem Cell ◽  
Amniotic Membranes

Limbal stem cell deficiency (LSCD) can be secondary to multiple etiologies including contact lens wear, chemical or thermal trauma, and systemic disease, any of which can result in the reduction of the number of stem cells or their decreased functionality. Primary LSCD is seen with a variety of congenital anterior segment disorders. Often LSCD can be stabilized and timely diagnosis is the key. The use of topical corticosteroids and artificial tear lubricants, along with treatment of any underlying conditions, and discontinuation of contact lenses are important initial treatment strategies. Advance treatments include amniotic membranes, limbal stem cell transplantation and keratoprostheses.

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