Glaucoma, Secondary Congenital
- Author: Inci Irak-Dersu, MD; Chief Editor: Hampton Roy Sr, MD more...
Background
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
- 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
- Hallermann-Streiff syndrome
- Cerebrohepatorenal syndrome (Zellweger) syndrome
- Stickler syndrome
- Trisomy syndromes (Down syndrome, trisomy D, Edwards syndrome)
- Cystinosis
- Pierre Robin syndrome
- Oculodentodigital dysplasia
- Mucopolysaccharidoses
- Waardenburg syndrome
- Stickler syndrome
- Basal cell nevus syndrome
- Nevus of Ota
- Walker-Walburg syndrome
Pathophysiology
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.
Epidemiology
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
- 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
- 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.
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