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Objective@#To present a rare case of Netherton Syndrome (NS) in a Filipino child. @*Case@#This is a case of an 11-year-old girl with elevated immunoglobulin E, trichorrhexis nodosa, and ichthyosis linearis circumflexa. She had dry skin with fine scaling at birth with recurrent pruritic, erythematous papules coalescing to plaques mainly on the face and extensors. The skin dryness turned to generalized redness with fine scaling, and the skin started getting tight. She could no longer completely extend both knees, but she was still able to walk. She also started having difficulty closing both eyes. She developed migratory serpiginous erythematous plaques with peripheral double-edged scaling. At six years old, she developed ulcers in the scalp, trunk, and extremities, which resulted in admission to our institution. She was managed for multiple skin infections, pneumonia, sepsis, seizure, severe malnutrition, joint contracture, atopy, and bilateral cicatricial ectropion.@*Conclusion@#Management of NS remains challenging. Common management options include emollients, topical corticosteroids, calcineurin, and protease inhibitor, and phototherapy while newer ones that need further validation include intravenous immunoglobulins and biologics such as infliximab. However, until specific recommendations are made, overall management for NS remains challenging. Regular multidisciplinary monitoring of the manifestations of NS is central to its management.
Subject(s)
Netherton Syndrome , IchthyosisABSTRACT
Upper Eye lid burns are more common among the eye injuries. Adequate acute management of eyelid burns is necessary to prevent ectropion .Ectropion of the upper eyelid is usually associated with constant danger of kerato conjunctivitis, corneal ulceration, scarring or perforation with loss of vision. The present study reviews 20 patients admitted for upper eyelid burns in the Department of Burns, Plastic and Reconstructive Surgery Govt Kilpauk Medical College & Hospital Chennai between January 2013 November 2014. our study analysis the results of early release of eyelid burns and collagen application on the day admission and Early grafting with SSG.20 Cases of upper eyelid burns were selected for release and grafting.17 cases were flame burns, 2 cases were acid burns and one case was scalds injury. Incision and collagen application on the day of admission and early split thickness skin grafting plays an important role in preventing the post burn cicatrical ectropion.
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Ectropion is characterized by eversion of the eyelid margin and exposure of the cornea and conjunctiva. This leaves the inner eyelid surface exposed and prone to irritation, and in severe cases, poor eye closure. Ectropion can occur for several reasons including congenital malformations, trauma, burns, anti-cancer medications, allergies, and inappropriate eyelid surgery. Surgical treatments usually include local flaps or skin grafts, depending on the severity of the defect. We present a case of successful cicatricial upper eyelid ectropion correction using a dermofat graft and an anti-adhesive agent (Guardix-Sol) after the wide release of tethering scar tissue.
Subject(s)
Burns , Cicatrix , Conjunctiva , Cornea , Ectropion , Eyelids , Hypersensitivity , Skin , TransplantsABSTRACT
Objective To improve the detail of the surgery for cicatricial ectropion,and the the effect was investigated. Methods Based on 46 patients withⅡ,Ⅲdegree cicatricial ectropion admitted to our department,choose the junction of normal skin and scar tissue as the incision line according to the degree of ectropion and local situation,and to reserve and recover the orbicularis oculi muscle as possible. The released areas exceeded separately the external canthus and reached the level of eye fissure,take advantage of full-thickness skin graft to correct the parallel lines and make fixation to the eyelid ectropion fusion skin graft on external canthal ligament. Results After followed up for 6 months to 2 years,46 cases of eyelid skin grafts survived completely and eyelids were in good reduction,with none case of incomplete eyelid closure. Conclusion Choose the surgical incision and the released areas in a proper way to reserve the structural integrity of orbicu-laris muscle and make skin graft fixation to inner and external canthal skin can prevent effectively the recurrence of ectropion and improve surgical progress.
ABSTRACT
Objective To investigate the method and effect of free autogenous palmaris longus tendon transplantation in the treatment of moderate and severe cicatricial ectropion.Methods The autogenous palmaris longus tendon was obtained through a small lateral proximal wrist band incision (lengths from 4 cm to 6 cm).The graft was properly sutured to the exposed tarsus and the attachments of inner and outer canthus.Local skin flap was rotated to cover the tendon and to repair the wound.Results Six man (8 eyes) with moderate and severe cicatricial ectropion were treated in our department,all case of ectropion were rectified after operation.The eyeballs were well contacted with lower eyelid and no recurrence of eetropion,epiphora and corneal exposure during 6 to 18 months' follow-up.The tension of the lower eyelid was abiding with satisfied effects.Conclusions The palmaris longus tendon is easy to obtain and there is no harm to the donor site.The cicatricial ectropion could be repaired with self-palmaris longus tendon transplantation with lasting effect and the recurrence rate is lower,which is well worthy popularizing.