A 6-month-old male infant was referred by his pediatrician for evaluation of a “large right pupil” associated with suspected corneal enlargement. His mother reported him seeing well and with no history of epiphora, photophobia, or suspected corneal cloudiness. He was born by caesarian section at 35 weeks with a birth weight of 8 lbs, and until 6 weeks of age showed a much narrower palpebral fissure in the left eye. Review of photographs from 1 month of age confirmed the constant presence of both anisocoria and an associated larger right cornea. On examination, he appeared healthy, happy, and without digital anomalies or hyperextensible joints. Good fixation, following, and interest in toys was demonstrated equally with each eye. No nystagmus was present. The eye movements were full with no eye deviation. No evidence of ptosis was present. The anterior segment examination revealed clear corneas, without breaks in Descemet’s membrane and with estimated diameters of 13 mm in the right eye and 12 mm in the left eye. The anterior chamber depths were 5 corneal thicknesses in the right eye and 3 corneal thicknesses in the left eye. The irides were normal and in character and color, and the lenses were clear. Inspection of the pupils confirmed anisocoria with the right pupil larger than the left but with equal reactivity to light. The applanation intraocular pressures were 12 mm Hg in both eyes. The corneal thickness measurements were 553 μm in the right eye and 570 μm in the left eye. The posterior segment was normal with each optic disc well vascularized without cupping. Cycloplegic refraction found +1.00 diopters of hyperopia in both eyes. Ultrasonography determined axial lengths of 17 mm in both eyes. Gonioscopy demonstrated normal and symmetric findings with normal trabecular meshwork and ciliary body components. Repeat assessment done at 7 months found no changes associated with intraocular pressures of 13 mm Hg in both eyes.
What’s Your Diagnosis?
For the correct answer, see page 124.
The correct answer to What’s Your Diagnosis? is primary relative anterior macrophthalmos.
References
- Ho CL, Walton DS. Primary Megalocornea: clinical features for differentiation from infantile glaucoma. J Pediatr Ophthalmol Strabismus. 2004;41:11–17.
- Auffarth GU, Blum M, Faller U, Tetz MR, Volcker HE. Relative anterior microphthalmos. Ophthalmology. 2000;107:1555–1560. doi:10.1016/S0161-6420(00)00240-2 [CrossRef]
- Nagao K, Noel NP, Noel ME, Walton DS. The spontaneous resolution of primary congenital glaucoma. J Pediatr Ophthalmol Strabismus. 2009;46:139–143. doi:10.3928/01913913-20090505-04 [CrossRef]