Secondary Congenital Glaucoma

This article discusses developmental glaucomas with associated ocular or systemic anomalies and the most identifiable causes. Aniridia and Peters Anomaly are discussed in other articles.

Glaucoma associated with congenital ocular abnormalities includes the following:

• Aniridia
• Hypoplasia/hyperplasia of iris
• Axenfeld-Rieger syndrome
• Peters anomaly
• Congenital ectropion uvea
• Congenital corneal staphyloma
• Cornea plana
• Iridoschisis
• Megalocornea
• Microcornea
• Microphthalmos
• Morning glory syndrome
• Persistent hyperplastic primary vitreous
• Nanophthalmos
• Posterior polymorphous dystrophy

Glaucomas associated with systemic congenital abnormalities include the following:

• Weil-Marchesani syndrome
• Glaucoma in phakomatosis
• Oculocerebrorenal (Lowe) syndrome
• Hallermann-Streiff syndrome
• Cerebrohepatorenal syndrome (Zellweger) syndrome
• Stickler syndrome
• Trisomy syndromes ( Down syndrome, trisomy D, Edwards syndrome)
• Prader-Willi syndrome
• Cystinosis
• Pierre Robin syndrome
• Rubinstein-Taybi syndrome
• Oculodentodigital dysplasia
• Fetal alcohol syndrome
• Mucopolysaccharidoses
• Waardenburg syndrome
• Cockayne syndrome
• Rubella
• Stickler syndrome
• Basal cell nevus syndrome
• Nevus of Ota
• Klippel-Trenaunay-Weber syndrome
• Nail-patella syndrome
• Walker-Walburg syndrome

The main pathology is malformation of the trabecular meshwork and iris (iridotrabeculodysgenesis) or iridocorneal dysgenesis. Numerous iris processes and iridocorneal adhesions could be seen in these diseases. Neovascular glaucoma has been reported in Stickler syndrome. Isolated trabeculodysgenesis is the usual finding in primary congenital glaucoma.

Frequency

United States

Aniridia is rare, occurring in 1.8 per 100,000 live births; 50% of these patients develop glaucoma. Axenfeld-Rieger syndrome is autosomal dominant and rare; 50% of patients develop glaucoma. Glaucoma occurs in 15% of patients with posterior polymorphous dystrophy. The prevalence of neurofibromatosis-1 (NF-1) is 1 in 3000-5000 people; glaucoma occurs in 1-2% of these patients. Glaucoma occurs in one half of patients with Sturge-Weber syndrome. von Hippel-Lindau occurs in 1 in 22,500 people.

Mortality/Morbidity

See the list below:

• Medical treatment usually fails in secondary congenital glaucoma, and surgery is necessary in most cases.
• Associated disorders (eg, corneal opacity, cataract, strabismus) increase the likelihood of amblyopia, unless intervention occurs at an early age.

Race

No racial predilection exists.

Sex

See the list below:

• No sex predilection exists in aniridia, Axenfeld-Rieger syndrome, Peters anomaly, or phakomatoses.
• Lowe syndrome, one of the causes of secondary congenital glaucoma, has X-linked transmission and appears in males.

Age

Glaucoma can appear at any age depending on the underlying condition. For instance, in Peters anomaly, glaucoma is usually present at birth; on the other hand, in Axenfeld-Rieger syndrome, glaucoma may not occur until young adulthood.