A 9-month-old girl is brought to the clinic for left eye redness and tearing that began last evening. Temperature is 37 C (98.6 F), pulse is 130/min, and respirations are 30/min. Examination of the left eye shows clear tearing on the eyelashes with conjunctival erythema. The left cornea and globe are larger than the right. The patient blinks frequently and turns away when light is shined in the left eye. Extraocular muscle movements are intact and appear nonpainful. The patient has a port-wine stain overlying the left forehead, eyelids, and cheek. Her mother says the lesion is more erythematous than normal, but there is no tenderness, warmth, or pruritus on examination. Which of the following is the best next step in management of this patient?
Glaucoma in children | |
Pathophysiology |
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Key features |
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Management |
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IOP = intraocular pressure. | |
This patient has an enlarged cornea and globe due to glaucoma. Glaucoma is characterized by optic neuropathy most commonly caused by increased intraocular pressure (IOP) from impaired drainage of intraocular fluid. Presentation in infancy typically involves signs of corneal edema, including tearing (epiphora), photophobia, blepharospasm, and conjunctival erythema. Increased IOP also causes stretching of the sclera (ie, enlarged globe) and cornea (ie, cloudy, large cornea) as well as cupping of the optic nerve.
Glaucoma in children may be due to anatomic abnormalities of the eye (eg, angle dysgenesis) or secondary to infection, tumor, trauma, or an underlying disorder that affects intraocular fluid. This patient likely has underlying Sturge-Weber syndrome (SWS), a neurocutaneous disorder characterized by the triad of a facial port-wine stain, leptomeningeal capillary-venous malformations, and glaucoma. Glaucoma in SWS is due to a congenital anterior chamber angle anomaly and increased episcleral venous pressure (from episcleral hemangioma). The eye ipsilateral to the cutaneous lesion is usually affected. The increased pressure may cause the lesion to appear more erythematous. Onset is typically in infancy (often at birth) but can occur into adulthood.
Clinical diagnosis of glaucoma is supported by elevated IOP on tonometry, and surgery is the primary treatment for pediatric glaucoma to preserve vision.
(Choices A and B) Antibiotic eye drops are indicated for bacterial conjunctivitis, which causes conjunctival injection with purulent eye drainage. Corneal/globe enlargement are not seen. Culture of the drainage is rarely indicated but can be considered in the neonatal period for suspicion of conjunctivitis caused by Chlamydia trachomatis or Neisseria gonorrhoeae.
(Choice C) Nasolacrimal duct (NLD) massage is the treatment for NLD obstruction, a common cause of isolated eye tearing in infants. This patient's photophobia and enlarged cornea and globe make this diagnosis unlikely.
(Choice D) Periorbital cellulitis presents with eyelid swelling, erythema, warmth, and tenderness and is treated with oral antibiotics. Orbital cellulitis causes similar symptoms plus proptosis and pain with eye movements; treatment is typically intravenous antibiotics. Neither condition causes corneal enlargement, and this patient has no signs of infection (ie, fever, periorbital warmth/tenderness).
(Choice F) Topical antihistamines treat allergic conjunctivitis, which presents with eye tearing, itchiness, and conjunctival erythema. An enlarged cornea and globe would not be expected.
Educational objective:
Glaucoma in infants typically presents with tearing, photophobia, blepharospasm, and an enlarged cornea and globe. Patients with Sturge-Weber syndrome are at increased risk due to a congenital anterior chamber angle anomaly. Evaluation of glaucoma includes tonometry to measure intraocular pressure.