RESUMEN
Linear Nevus Sebaceous Syndrome (LNSS) is a rare sporadic oculoneurocutaneous disorder, also classified as Organoid Nevus Syndrome. It consists of a triad of midline facial linear nevus sebaceous, central nervous system and ocular abnormalities. To the best of authors' knowledge ophthalmic features of LNSS have never been reported in Pakistani population. We report two cases of LNSS, associated with multiple cutaneous nevus sebaceous lesions, complex ocular choristomas and rare bilateral presentation in one patient. Ocular choristomas included limbal dermoids, dermolipomas at superior fornices and chroidal choristoma. Ocular surface was successfully reconstructed by excision of limbal dermoids, partial keratectomy and amniotic membrane transplant.
Asunto(s)
Coristoma/diagnóstico , Neoplasias de la Coroides/diagnóstico , Conjuntiva/patología , Anomalías del Ojo/patología , Hamartoma , Nevo Sebáceo de Jadassohn/diagnóstico , Biopsia , Niño , Coristoma/cirugía , Neoplasias de la Coroides/cirugía , Anomalías del Ojo/etiología , Femenino , Humanos , Lactante , Masculino , Nevo Sebáceo de Jadassohn/cirugía , Convulsiones/etiología , Resultado del TratamientoRESUMEN
OBJECTIVE: To evaluate the outcome of nasolacrimal intubation as a primary treatment of congenital nasolacrimal duct obstruction (NLDO) in children up to 4 years of age. METHODS: During the 3 years period from July 2008 to June 2011, in the Paediatric Ophthalmology Department. Alshifa Trust Eye Hospital, Rawalpindi, 65 eyes of 59 children, aged 12 to 48 months with congenital NLDO and no prior Nasolacrimal duct surgery were enrolled. After written informed consent, all nasolacrimal intubations using olive tip silicon tube were performed under general anaesthesia. The planned tube retention was at least 3 months. The study outcome visit was timed 1 month after tube removal and treatment success was analyzed. Intra operative and post operative complications were also noted. Data were analyzed by SPSS 16. Frequencies and percentages were calculated for categorical variables. Mean +/- SD were computed for age and duration of intubation. Chi-square test was used to compare proportion of outcomes in different age groups and duration. RESULTS: The overall success rate of Nasolacrimal intubation as a primary treatment of congenital NLDO was 89% in children between 12 to 48 months age (mean 25.8 +/- 9.8 months). The success was 92% in children under 2 years of age (P < 0.0001) and 90% in children between 2-3 years of age (P < 0.0001). The procedure remained less successful in children between 3-4 years of age (P < 0.2860) as compared to children under 3 years of age. The success rate was consistently high (92.3%) when the tube was left in situ for more than 3-6 months (P < 0.0001). CONCLUSION: Nasolacrimal intubation with Olive tip silicon intubation tube is a successful procedure as a primary treatment of Nasolacrimal duct obstruction in children under 4 years of age.