Secondary childhood glaucoma

As noted in Table 12, pediatric glaucoma may occur as a result of a multitude of ophthalmic conditions, many of which have significant systemic implications for the primary care provider.

Trauma

ETIOLOGY AND DIAGNOSIS

The most common form of traumatic glaucoma in childhood occurs as a result of anterior chamber hemorrhage (hyphema). This typically occurs 1–3 days following a blunt, nonpenetrating injury to the globe. Rebleeding in hyphema typically occurs 3–5 days after the initial injury and can cause a significant pressure spike which can result in nausea, vomiting, and severe visual loss if not treated promptly.

The diagnosis of traumatic glaucoma is made when the IOP is found to be elevated following an injury, or suspected injury, consistent with the development of glaucoma. An assessment of IOP is important in any child who sustains a significant ocular injury.

MANAGEMENT/TREATMENT AND PROGNOSIS

Although bedrest and hospitalization are no longer considered mandatory, eye protection, cycloplegia, and avoidance of further injury are paramount. Topical therapy for elevated IOP is sometimes indicated. Anterior chamber washout is necessary in some cases of hyphema associated with glaucoma to prevent permanent blood-staining of the cornea or optic nerve damage. Children with sickle cell hemoglobinopathies are at special risk for this complication and may require earlier surgical intervention.

Most cases can be treated if identified in a timely manner. However, even in cases with spontaneous resolution of the hyphema, patients remain at long-term risk of developing glaucoma later in life secondary to damage to the anterior chamber angle structures as a result of the initial injury. All patients with hyphema should be examined not only during the acute postinjury phase but also throughout their life for signs of glaucoma.

Neoplasia

The most common cause of glaucoma secondary to ocular tumor in childhood is retinoblastoma. This may occur due to iris neovascularization (rubeosis) with consequent angle closure, infiltration of the angle with tumor cells, or secondary to a large tumor pushing the iris forward and closing off the angle. Other causes include leukemia, lymphoma, and juvenile xanthogranuloma. Treatment is usually medical along with treatment of the underlying cause as indicated. Glaucoma surgery is contraindicated in patients with retinoblastoma.

Uveitis (iritis) (161)

ETIOLOGY AND DIAGNOSIS

The most common example of uveitis causing glaucoma is seen in juvenile rheumatoid arthritis. The uveitis in the setting of HLA-B27 disease (ankylosing spondylitis) generally occurs in adolescents and young adults. Most patients with uveitis that develop glaucoma do so as a complication of chronic disease. Many of these patients are being followed by an ophthalmologist for their underlying disorder. However, some patients with uveitis initially present with coexistent glaucoma. In a smaller group of patients, the glaucoma may be responsible for symptoms leading to their presentation. Assessment of IOP is an important part of the care of the child with uveitis.

161

161 Ciliary flush and corneal edema in acute anterior uveitis.(Courtesy of Hasan M. Bahrani, MD.)

Chronic iritis can cause glaucoma in childhood as a result of anterior segment inflammation. This inflammation may lead to inflammation and abnormal function of the trabecular meshwork (trabeculitis), or cause scarring of the angle drainage structures (angle closure). In addition, glaucoma is a common side-effect of long-term topical corticosteroid use. For this reason, the chronic treatment of uveitis with topical corticosteroids should be avoided if possible.

MANAGEMENT/TREATMENT AND PROGNOSIS

Medical treatment is the first line of treatment in patients with glaucoma and uveitis. If possible, corticosteroids should be discontinued. Topical glaucoma medication may be helpful. Surgery is useful in those patients who fail to respond to medical management.

Early and more aggressive treatment can help to avoid the changes in the anterior segment that lead to glaucoma. Systemic therapy with corticosteroid-sparing agents will also help to decrease the incidence of glaucoma. Patients who present with glaucoma at the time of diagnosis are especially challenging.

Glaucoma following pediatric cataract surgery

ETIOLOGY AND DIAGNOSIS

Glaucoma is common following pediatric cataract surgery, developing in up to 25% or more of patients undergoing lensectomy in one study. During the immediate postoperative period, pupillary block leading to angle closure glaucoma can occur either with or without intraocular lens placement. Open-angle glaucoma is more common and may occur months to years after lensectomy. The underlying cause of this form of glaucoma is unknown.

In young infants, the signs and symptoms typical of primary infantile glaucoma may occur. Older children behave more like those with juvenile glaucoma. Because of the high risk and the fact that the signs and symptoms may be subtle, an especially high level of surveillance for the development of glaucoma must be maintained in any child undergoing cataract surgery.

MANAGEMENT/TREATMENT AND PROGNOSIS

Laser or surgical management of the iris is needed in cases that have developed pupillary block glaucoma. Medical therapy with topical agents are the preferred initial form of therapy unless rapid IOP lowering is required, in which case oral agents (i.e. acetazolamide) may be needed. Surgical intervention including Seton implantation and cycloablation (destruction of the ciliary processes which produce aqueous humor) are often required in these cases to control elevated IOP.

Glaucoma following cataract surgery in children is difficult to treat and often requires both medical and surgical treatment. Lifelong follow-up is mandatory to optimize the opportunity for early diagnosis and treatment in these patients.

Other causes of secondary glaucoma in children

There are a multitude of additional causes of secondary childhood glaucoma as noted in Table 12. These include lens-induced glaucoma (Marfan’s and homocystinuria), secondary angle closure (retinopathy of prematurity, microphthalmos, and persistent hyperplastic primary vitreous), glaucoma associated with increased venous pressure (cavernous or dural sinus fistula), and intraocular infection (toxoplasmosis, herpetic iritis, and endogenous endophthalmitis).