Primary congenital glaucoma: Spontaneous resolution

A 7-year-old boy referred from a school screening camp for defective vision in the left eye (LE). He was the first child born of a nonconsanguineous marriage. He was born via a normal vaginal delivery without the use of forceps. Mother gave a history of corneal haze in the LE immediately after birth, which cleared on its own without treatment. There was no family history of glaucoma, and the child was not having any systemic disease. His best-corrected visual acuity (BCVA) was 6/6 in the right eye (RE) and 6/36 with −3 Dsph −1.25 Dcyl × 40° in the LE. Slit-lamp examination of the RE was normal with a corneal diameter of 11.5 mm, and LE showed megalocornea with a diameter of 13mm, Haab’s striae, deep anterior chamber, normal color and pattern of iris, and clear lens [Fig. 1a-c]. His intraocular pressure (IOP) was 15 and 20 mmHg in the RE and LE, respectively. Gonioscopy showed open angles in both eyes. Posterior segment examination was within normal limits in both eyes, with a cup-to-disc ratio of 0.3 in the RE and 0.5 in the LE, with a healthy neuroretinal rim [Fig. 1d and e]. Axial length was 22.79 and 24.27 mm in the RE and LE, respectively. Central corneal thickness was 582µ in the RE and 601µ in the LE.Figure 1: Profile photograph showing a bigger left eye than right (a), slit-lamp photograph of the right eye showing a normal anterior segment (b) and the left eye showing Haab’s striae (arrowhead) (c), and fundus photograph showing a cup-to-disc ratio of 0.3 in the right eye (d) and 0.5 in the left eye (e) with a healthy neuroretinal rimHe was diagnosed with spontaneous resolution of primary congenital glaucoma (PCG) in the LE. He was advised to patch the RE for 5 h per day and to come for regular follow-up. At the last follow-up visit, BCVA remained the same, and IOP was 16 in the RE and 20 mmHg in the LE without treatment. PCG is the most common childhood glaucoma with characteristic signs of corneal cloudiness, Haab’s striae, ocular enlargement, and optic nerve head cupping due to increased IOP. At 5 months of gestation, the iris is in contact with the corneal endothelium, and neural crest cells destined to form the trabecular meshwork (TM) are buried beneath the uveal tissue. In the last trimester, the corneoscleral coat grows faster than the uveal tract, resulting in posterior migration of the uveal tissue, exposing the TM. Failure of posterior migration of the uveal tissue results in PCG. The mechanism for spontaneous resolution was probably due to posterior migration of the iris in the postnatal period resulting in exposure of the TM.[1] These eyes will have normal IOP without treatment, but there will be signs of increased IOP during infancy such as megalocornea, Haab’s striae, optic disc cupping, and increased axial length. The importance of knowing this rare condition is to avoid unnecessary treatment as well as these eyes need lifelong follow-up as they can develop high IOP later.[2,3] Authors’ contributions Premanand Chandran: Concepts, design, definition of intellectual content, literature search, data acquisition, manuscript preparation, manuscript editing, manuscript review, and guarantor; Praveena S. Kumar: Concepts, definition of intellectual content, literature search, and manuscript preparation; Vishnu Subramanian: Concepts, literature search, and manuscript preparation; Nishita Pilli: Concepts, literature search, and manuscript preparation. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.