Introduction 1.1 Terminology, epidemiology and heredity Congenital glaucoma is a major cause of blindness in children, despite its low incidence (1:10,000 births) 1. This category includes isolated congenital glaucoma (also called primary congenital glaucoma) and glaucomas associated with other developmental anomalies, either systemic or ocular. Juvenile glaucoma is the term used to designate cases in which the pressure rise develops after the third birthday but before the age of 16 years. 2 The enlargement of the eye (buphthalmos) is least common, despite the elevated intraocular pressure. Gonioscopy is normal or reveals trabeculodysgenesis. This condition may simulate the primary open-angle glaucoma. The eyes with primary congenital glaucoma have an isolated maldevelopment of the trabecular meshwork not associated with others developmental ocular anomalies or ocular diseases that can raise intraocular pressure. It's the most common glaucoma of infancy, occurring in about 1: 30,000 live births 1. Primary congenital glaucoma is a bilateral disease in about 75% of cases, with males accounting for approximately 65% of cases. 2 Most cases are sporadic in occurrence, with no evident hereditary pattern. In approximately 10% in which a hereditary pattern is evident, it generally is believed to be autosomal recessive. Many authors believe the inheritance pattern is polygenic. 1 1.2 Pathogenesis Clinical evidence supports the theory that the obstruction to aqueous flow, with a resultant increase in intraocular pressure, is located at the trabecular sheets. This obstruction is caused by maldevelopment of the anterior chamber angle, unassociated with any other major ocular anomalies (isolated trabeculodysgenesis). 2 Clinically, trabeculodysgenesis is characterized by absence of the angle recess, with the iris inserted into the surface of the trabeculum in one of two configurations: 2 a. Flat iris insertion: the iris inserts flatly into the thickened trabeculum at or anterior to the scleral spur. b. Concave iris insertion: is less common. The plane of the iris is well posterior to the normal position of the scleral spur. However, the anterior iris stroma continues upward and over the trabecular meshwork, obscuring the scleral spur and the others angular structures. www.intechopen.com Glaucoma-Basic and Clinical Concepts 484 1.3 Clinical presentation Frequently, the first symptoms of primary congenital glaucoma are epiphora, photophobia and blepharospasm. These symptoms occur secondary to the corneal epithelial edema caused by elevated intraocular pressure. The elevated intraocular pressure also causes an enlargement of the eye (buphthalmos) (Figure 1), mainly at the corneoscleral junction. Stretching of the zonules can cause lens subluxation. 3
Nessa difícil jornada acadêmica, alguns nos incentivaram, abriram portas, mostraram possibilidades, sempre no sentido de caminhar adiante. Estes são verdadeiros professores, mestres no ensinamento e na dedicação. Ao Prof. Dr. Alberto Jorge Betinjane, minha eterna gratidão e admiração pelos seus ensinamentos, pelo carinho e, principalmente, pelo exemplo de retidão. A Profa. Dra. Angela Maggio da Fonseca, exemplo de sabedoria, não somente científica, como também na doação às pessoas que a cercam. Ao Prof. Dr. Remo Susanna Júnior, minha eterna admiração. Não poderia deixar de agradecê-lo em especial por sua generosidade, bondade, e luta por um mundo mais justo. Quero agradecê-lo por me adotar em vários momentos de minha vida. vi Agradecimentos vii Aos meus sogros Jair e Aparecida, fontes incessantes de auxílio, apoio e amor. Obrigada por terem criado um filho tão maravilhoso. Aos queridos avós Olga, Nino e Lucíria pelos exemplos de retidão, honestidade, humildade e amor ao próximo. À querida amiga Marilci, por ter a certeza de que a verdadeira amizade existe. À querida Roberta Giampani, pelo incentivo e apoio em todas as horas. Ao amigo Fernando Fusari Bento de Lima, por toda ajuda e amizade. Às tias Cleusa, Cléa e Wilma, pelo constante incentivo. À tia Cleide, pelo incentivo e pela revisão gramatical desta tese. À Dra. Sônia Regina Lenharo Penteado, querida amiga e exemplo de companheirismo, pelo apoio nesta jornada. Ao Prof. Dr. Arnaldo Zanotto, pela amizade,pela atenção e todo carinho na análise estatística desta tese. Ao amigo Dr. José Eudes Carani, homem de palavras doces, que com um simples gesto consegue nos fazer sorrir. Ao Prof. Dr. Samir Jacob Bechara, que esteve presente em minha vida em vários momentos diferentes, em todos eles sempre disposto a ajudar.
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