ABSTRACT
ABSTRACT A patient with bilateral aniridia and progressive congenital cataract was treated at the age of 7 years through phacoaspiration with femtosecond laser capsulotomy, in-the-bag intraocular lens implantation, and pars plana vitrectomy (combined with posterior capsulotomy). The diameter of the planned anterior capsulotomy was 4.7 mm; however, due to elevated tension in the young capsular bag, the capsulotomy diameter increased (to around 6 mm) shortly after release of the tension by the laser. In addition, the patient had a very flat and small cornea, causing the formation of air bubbles in the curved laser interface. This was addressed by filling the gap by using a viscoelastic. A very similar situation was observed in both eyes. Use of the femtosecond laser facilitated a successful outcome in a complex congenital cataract case with a safe capsulotomy that ensured in-the-bag intraocular lens placement.
RESUMO Paciente com aniridia bilateral e catarata congênita progressiva foi tratado com a idade de 7 anos através de facoemulsificação com capsulotomia anterior por laser de femtosegundo, implante de lente intra-ocular e vitrectomia via pars plana (combinada com capsulotomia posterior). O diâmetro da capsulotomia anterior prevista foi de 4,7mm, porém, devido à elevada tensão do saco capsular jovem, o diâmetro da capsulotomia aumentou (para cerca de 6mm) logo após a liberação da tensão pelo laser. Além disso, o paciente tinha uma córnea muito plana e pequena, causando a formação de bolhas de ar na interface do laser. Isso foi resolvido preenchendo a lacuna usando um viscoelástico. Uma situação muito semelhante foi observada em ambos os olhos. O uso do laser de femtosegundo facilitou um resultado bem-sucedido em um caso complexo de catarata congênita com uma capsulotomia anterior segura que garantiu a colocação da lente intra-ocular dentro do saco capsular.
Subject(s)
Humans , Male , Child , Cataract/congenital , Aniridia/complications , Capsulorhexis/methods , Cataract/complications , Visual Acuity , Aniridia/surgery , Laser Therapy/methodsABSTRACT
We report a case of aniridia associated with congenital aphakia and secondary glaucoma. A 35-year-old male presented with aniridia, congenital aphakia and secondary glaucoma in both eyes. After an unsuccessful medical management, he underwent trabeculectomy with mitomycin C and anterior vitrectomy under local anesthesia in his left eye. Postoperatively, at the end of six months, intraocular pressure (IOP) in his left eye was controlled without medications. This case highlights the rare association of aniridia with congenital aphakia and secondary glaucoma.
Subject(s)
Adult , Aniridia/complications , Aniridia/surgery , Aphakia/complications , Aphakia/congenital , Glaucoma/complications , Glaucoma/etiology , Glaucoma/physiopathology , Glaucoma/surgery , Humans , Male , Optic Atrophy/etiology , Postoperative Period , Trabeculectomy , Visual AcuityABSTRACT
Aniridia (absence of iris) is a congenital, bilateral, uncommon panocular disorder. Whereas the occurrence of aniridia in the general population is 1:50000, it is present in about 1 in 70 patients with Wilm's tumor. This aniridia is sporadic and Wilm's tumor in these cases presents at an unusually early age. Aniridia was present in two cases out of 60 cases of Wilm's tumor operated at PGIMS, Rohtak. Both cases presented below two years of age. The recognition of a child with sporadic aniridia should alert to the increased risk of development of Wilm's tumor.
Subject(s)
Aniridia/complications , Humans , Infant , Kidney Neoplasms/complications , Risk Factors , Wilms Tumor/complicationsABSTRACT
O cristalino ectópico está presente em diversas doenças, que apesar de raras, relacionam-se a complicações severas. A doença mais freqüentemente encontrada no presente estudo foi a Síndrome de Marfan, na qual o deslocamento do cristalino ocorreu para a região superior e temporal. O deslocamento acarreta importante diminuição da acuidade visual e pode gerar ambliopia. Esse estudo apresenta a freqüencia e as causas de cristalino ectópico nos pacientes examinados no Setor de Genética do Departamento de Oftalmologia da Escola Paulista de Medicina no periodo de 1985 a 1993, enfatizando o diagnóstico e reabilitação ocular precoces para a prevenção da ambliopia e estrabismo.
Subject(s)
Humans , Infant , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Male , Female , Aniridia/complications , Ectopia Lentis/etiology , Homocystinuria/complications , Marfan Syndrome/complications , Ectopia Lentis/diagnosis , Visual AcuityABSTRACT
A two year old female child with bilateral wilms tumor (WT) along with multiple congenital anomalies like bilateral aniridia with congenital cataracts and nystagmus, microcephaly, mental retardation and ventricular septal defect has been described. The karyotype analysis revealed 46 xx, del 11p 13-14.1. Association of ventricular septal defect with the classical features of 'Aniridia-Wilms' tumor association' is an unusual feature in this case.
Subject(s)
Aniridia/complications , Child, Preschool , Chromosome Deletion , Chromosomes, Human, Pair 11/physiology , Female , Heart Septal Defects, Ventricular/complications , Humans , Karyotyping , Wilms Tumor/complicationsABSTRACT
El glaucoma que complica a la aniridia se debe generalmente a cambios post natales que se agregan a alteraciones determinadas congénitamente. Menos frecuente es el glaucoma congénito asociado a la aniridia. El principal cambio post natal es el cierre angular progresivo que finalmente termina adosando el muñón iridiano a la pared interna del trabéculo, alterando su función y determinando glaucoma. La patogenia que determina esta modificación morfológica y cierre angular es desconocida. El presente trabajo describe la proliferación de melanocitos en la superficie interna del trabéculo, espacios trabeculares, tejido yuxtacanalicular, pared externa del conducto de Schlemm y conductos colectores esclerales. Esta proliferación no ha sido descrita previamente en la aniridia y podría explicar las modificaciones angulares que llevan al glaucoma por cierre angular secundario en estos casos. Es probable que este mecanismo patogénico sea responsable de la refractariedad a los distintos tipos de tratamiento descritos en el glaucoma asociado a aniridia desde que el aspecto de las trabéculas, el conducto de Schiem y conductos colectores son aparentemente normales