Combating Airway Issues with Elective Use of an I Gel in a Child with Goldenhar Syndrome

Introduction: Goldenhar syndrome is an oculoauriculovertebral spectrum attributed to the developmental anomalies of the first and second brachial arches. Its typical presentation in children with hemifacial microsomia poses a dual challenge for the anaesthesiologist on account of difficult airway often compounded with systemic abnormalities. Case report: We describe a case report wherein a 5 year female presented to the oculoplastic clinic of our hospital for surgical removal of limbal dermoid under general anaesthesia. Airway examination revealed classical facial asymmetry with underdevelopment of jaw bone coupled with protruding incisors. A predicted difficult airway, more so in a child led us to choose an anaesthesia technique with preservation of spontaneous breathing and planned use of supraglottic device in the form of an I Gel for airway management. Conclusion: The aim of this case report is to highlight the anaesthetic implications of this not so uncommon entity presenting to ophthalmology and ENT clinics. A thorough preoperative assessment, adequate preparedness and alternative plans are keys for successful airway management in such syndromic children. Keywords: Goldenhar syndrome, hemifacial microsomia, I Gel

SURGICAL MANAGEMENT OF CORNEAL LIMBAL DERMOIDS BY SIMPLE EXCISION

Aim: To study clinical features and surgical outcomes of limbal dermoid excision in Kashmiri patients. Study Design :Interventional, Prospective Material and Methods: 20 patients with epibulbar limbal dermoid who attended the Out Patient department of Government Medical College ,Srinagar, from August 2019 to September 2020, across all age groups were included in the study. All the patients were examined for site, colour, presence of pigmentation and presence of ocular and systemic associations of the lesions and then surgically managed. Simple Excision was done with blade and scissors. Postoperative follow up was done upto 3 months Results: The age group included was 4 to 60 years with male to female ratio 3:2. All the patients had unilateral epibulbar dermoids located at infero-temporal quadrant of the limbus. Most lesions (13 cases - 65%) extended equally to cornea and sclera while a few extended more on the sclera (3 cases - 15%) or cornea (3 cases - 15%). Most (15 cases - 75%) were round. Two (10%) had Goldenhar Syndrome and both had preauricular tags and maxillary hypoplasia. Postoperatively one patient (5%) had corneal thinning and two(10%) patients had granulation tissue formation.Pigmentation of the lesion was seen in 18 cases (80%). Conclusion:Limbal dermoids in Kashmiri patients have clinical characteristics resembling those described in other parts of the world. Treatment with simple excision and supercial sclerokeratectomy gives satisfactory results.

A case of pterygium-like proliferation containing postoperative limbal dermoid remnants: a clinicopathological study

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.

Congenital central corneal dermoid: A rare entity

Background: To understand the pathogenesis of a central corneal dermoid (CD) in a 12-day-old child, a comparison of CD specimen was done with limbal dermoid (LD) and cadaveric corneal (CC) specimens by immuno-histochemical staining. Methods: The child underwent penetrating keratoplasty for visual rehabilitation. The corneal tissue was sent for histo-pathological and immunochemistry evaluation. The corneal specimen obtained was compared the origin of central CD with LD and CC based on their antigenic expression profile. Results: Clinically over a period of 75 months post operatively the child maintained a clear graft. Hematoxylin and Eosin staining of LD had a typical morphology including stratified keratinized epithelium, hair shaft with pilo sebaceous glands, eccrine sweat glands, lymphocytes, and blood vessels. Immuno-histochemical staining showed positive stain for Cytokeratin 3 epithelial marker in the epithelium of CC, LD, and CD. Smooth muscle maker (SMA) was identified in LD and CD but not in the CC as it is devoid of blood vessels. Limbal stem cell maker (P63) was detected only in LD. Vimentin, a mesenchymal stem cell marker stained positively in all three tissues of CC, LD, and CD. Conclusions: Corneal dermoid showed positive staining for mesodermal tissue components compared to both ectodermal and mesodermal components in limbal dermoid suggesting possibly a different origin of corneal dermoid