This report is about a 10 year old patient who was diagnosed with congenital glaucoma of both eyes. He underwent cataract surgery, filtering surgery, and diode laser ciclophotocoagulation before the insertion of Ahmed valve with mytomicine C in both eyes. The postoperative control of intraocular pressure was achieved in the right eye with topic timolol 0.5% twice a day. In the left eye encapsulated bleb, tube obstruction and endophthalmitis occurred. Authors described and discuss the management of surgical complications in the left eye. The implant of two Ahmed valve with mytomicine C, one of them at the inferior quadrant, predisposes to severe postoperative complications in refractory congenital glaucoma associated to aphaquia. To guarantee the success of IOP control and the preservation of visual functions, it is necessary a careful postoperative follow-up. Resumen El presente reporte es de un paciente masculino de 10 años de edad con diagnóstico de glaucoma congénito ambos ojos y antecedentes de afaquia bilateral, trabeculotomía asociada a trabeculectomía y ciclofotocoagulación transescleral fallidas; al que se le realizó implante valvular de Ahmed con mitomicina C en ambos ojos. Se logró control postoperatorio de la presión intraocular en ojo derecho con colirio timolol 0.5% dos veces al día. En el ojo izquierdo se presentó ampolla encapsulada, obstrucción del tubo de drenaje y endoftalmitis. Se describe y discute el manejo de estas complicaciones quirúrgicas. La colocación de dos válvulas de Ahmed con mitomicina C, una de ellas en el cuadrante inferior, predispone al desarrollo de severas complicaciones oculares en el glaucoma congénito refractario asociado a afaquia. El seguimiento postoperatorio estrecho garantiza el diagnóstico y tratamiento oportuno de las mismas.

Introduction: Congenital Ocular Melanocytosis (COM) is an infrequent disease (0,038% in Caucasian population), characterized by increase in number, size and pigmentation of melanocytes. It has been associated with ophthalmological diseases like uveal melanoma and glaucoma, and also with systemic pathologies, as Idiopathic Intracranial Hypertension, and delayed psycho-intellectual development. Clinical case: a 7-year-old female, who shows, as casual finding, iris hyperchromia in left eye, and grayish- blue multifocal pigmentation in episclera, which does not move with exploration, compatible with diagnosis of COM. The patient complains of repetitive urinary infections, so she is referred to Nephrology department, and a CUMS (Retrograde cistouretrography) is performed, establishing the diagnostic of severe vesicoureteral reflux (VUR). Discussion: There are several reports in literature related to the association between dermal hipo or hyperpigmentation and renal pathology. VUR has also been described in relation to hypomelanosis of Ito, pseudomelanosis duodeni and retinal pigment epithelium alterations. However, this is the first report in literature of Congenital Ocular Melanocytosis associated with severe Vesico-Ureteral Reflux.

Floppy eyelid syndrome is an uncommon condition characterized by rubbery and loose upper eyelid that may evert spontaneously during sleep and result in a chronic papillary conjunctivitis. Neurofibromatosis type 1 is an autosomal phacomatosis that may affect the eyelid causing mechanical blepharoptosis and lid deformation. The authors describe a case of a 58-year-old man with no previous history of neurofibromatosis who presented a plexiform neurofibroma in the eyelid simulating floppy eyelid syndrome Resumo Síndrome da frouxidão palpebral é uma condição rara caracterizada pela presença de pálpebras superiores mais elásticas que podem everter espontaneamente durante o sono e resultar em uma conjuntivite papilar crônica. A neurofibromatose tipo 1 é uma facomatose autossômica que pode afetar a pálpebra causando blefaroptose mecânica e deformações. Os autores descrevem um caso de um homem de 58 anos de idade sem historia previa de neurofibromatose que apresenta-se com neurofibroma plexiforme da pálpebra simulando a síndrome da frouxidão palpebral.

Introduction: Cockayne syndrome is an autosomal recessive disorder, with two fundamental characteristics: profound postnatal changes in physical and intellectual development and premature aging with progressive multi-organ degeneration. Retinal dystrophy, enophthalmos, optic nerve atrophy, nystagmus, dry eye, corneal infiltrates and band keratopathy are typical ophthalmological findings. Clinical cases: We describe two cases of the same family, with emphasis on ocular manifestations and their management. In the first, there was macular pigment dispersion, coroidosis, retinal vasculature attenuation, optic atrophy, and band keratopathy. The second case had cataracts, miosis, pigmentary retinopathy, retinal vascular attenuation and band keratopathy. Conclusion: The ophthalmological approach to these patients is difficult since any surgical procedure is hampered by enophthalmos, poor pupil dilation and systemic manifestations that complicate the anesthetic procedures. The intention of these cases is to alert clinicians to this rare, inherited and fatal disorder, which leads to ocular and systemic manifestations that are difficult to manage. Resumo Introdução: Síndrome de Cockayne é uma doença autossómica recessiva rara, com duas características: alterações profundas no desenvolvimento físico e intelectual pós-natal e envelhecimento prematuro com degeneração multi-orgânica progressiva. São achados oculares característicos: distrofia retiniana, enoftalmia, atrofia óptica, nistagmo, olho seco, infiltrados corneanos, queratopatia em banda, entre outros. Casos clínicos: Descrevem-se dois casos da mesma família, dando especial ênfase às manifestações oculares e sua abordagem. No primeiro, observou-se dispersão pigmentar macular, coroidose, vasculatura retiniana atenuada, atrofia óptica, miose e queratite em banda. O segundo caso apresenta cataratas, miose, retinopatia pigmentar, vasculatura retiniana atenuada e queratopatia em banda. Conclusões: A abordagem oftalmológica destes doentes é difícil uma vez que qualquer procedimento cirúrgico é dificultado pela enoftalmia, má dilatação pupilar e pelas manifestações sistémicas que complicam a anestesia. Pretende-se alertar para esta entidade rara, hereditária, que cursa com alterações oculares e sistémicas múltiplas de difícil resolução e que evoluem progressivamente para a morte.