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An Approach to Visual Loss in a Child
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
Muhammad Hassaan Ali, Stacy L. Pineles
Any child who presents with photophobia, lacrimation and blepharospasm should be strongly suspected to have congenital glaucoma unless proved otherwise. Glaucoma in children usually occurs due to angle dysgenesis and is often associated with other congenital ocular abnormalities like Peter's and Rieger's anomaly (37,38). All children with congenital glaucoma should undergo regular testing for IOP, corneal diameter, anterior segment examination, cup to disc ratio and axial length measurement. Out of all these parameters, the corneal diameter is the most reliable indicator of severity of the glaucoma in children. A corneal diameter of greater than 14 mm indicates advanced buphthalmos and an advanced stage of the disease. Another characteristic feature of infantile glaucoma is the possibility of reversal of cup to disc ratio once pressure of the eye is controlled. Infantile glaucoma if not treated early may cause complete blindness in children. The treatment of choice is mostly surgical with goniotomy performed in eyes with clear corneas and angle filtration surgery preferred in eyes with hazy corneas (39). Complicated cases with failed filtration surgeries are usually treated with glaucoma shunt devices (40). Lastly, clinicians should always offer genetic counseling to the parents if more than one sibling gets affected.
Answers
Published in Samar Razaq, Difficult Cases in Primary Care, 2021
The canal of Schlemm is a circular canal responsible for draining the aqueous humour from the anterior chamber into the ciliary veins of the eye. Resistance of flow through the canal results in the build-up of pressure in the eye, leading to damage to the optic nerve. Congenital absence or abnormality of the canal (or any other congenital abnormality of the angle of the anterior chamber) can lead to primary buphthalmos or infantile glaucoma. The child will present with progressive enlargement of the eye as the intraocular pressure increases. The anterior chamber deepens and a progressive myopia may develop. Oedema, inflammation, photophobia and epiphoria usually ensue. Delayed diagnosis will invariably result in damage to the optic nerve head, leading to an irreversible reduction in vision. Treatment is by surgery and involves a trabeculectomy to allow increased drainage in the angle of the anterior chamber.
Glaucoma
Published in Mary E. Shaw, Agnes Lee, Ophthalmic Nursing, 2018
Buphthalmos is a rare congenital condition affecting one in 10,000 births and resulting in increased intraocular pressure caused by a defect or blockage of the drainage angle by an embryonic membrane. Occasionally, the canal of Schlemm is absent. Forty percent have raised intraocular pressures in utero, 50% manifest in the first year of life, and 10% manifest between the first and third year of life.
A case of neurofibromatosis type 1 and unilateral glaucoma with ectropion uveae
Published in Ophthalmic Genetics, 2022
Akshaya L. Thananjeyan, Tanya Karaconji, Maree Flaherty, Sophia Zagora, Robyn V. Jamieson, John R. B. Grigg
Our patient, on initial presentation, was conservatively managed until developing unilateral right-sided ocular hypertension. It has been previously proposed that globe enlargement in NF1 may not solely be attributed to ocular hypertension or glaucoma, as in cases with regional gigantism, ocular overgrowth can play a significant role in its manifestation (6). Hoyt et al. (6) documented a case of enlarging buphthalmos in a patient with NF1 and hemifacial hypertrophy with only moderately elevated IOP, attributing globe enlargement to additional factors including ocular gigantism and generalized hyperplasia of the orbit while drawing parallels to how regional gigantism affected surrounding structures. Similar to our patient, clinically evident buphthalmos preceded facial hemihypertrophy.
Incidence of Sturge–Weber syndrome and associated ocular involvement in Olmsted County, Minnesota, United States
Published in Ophthalmic Genetics, 2020
Heba T. Rihani, Lauren A. Dalvin, David O. Hodge, Jose S. Pulido
Management options for glaucoma include topical medications, filtering surgery (goniotomy, trabeculotomy, trabeculectomy, tube shunt), and cyclodestructive procedures (24,51,52). Glaucoma secondary to SWS remains a challenging disease with low success rate due to severe surgical complications, including choroidal effusion, expulsive hemorrhage, and exudative retinal detachment, with intraocular pressure remaining refractory to multiple treatments in some cases (53,54). In our series, (n = 2, 15%) developed retinal detachment in the setting of previous multiple glaucoma surgeries. In another case (n = 1, 7.7%), the patient developed hypotonous maculopathy with ERM post trabeculectomy, but vision was maintained at 20/50 at last follow-up visit. Moreover, cases with early onset glaucoma and buphthalmos had worse visual acuity outcome (noting that subjects 4 and 5 with poor final visual outcomes were treated decades ago when modern surgical techniques in treating glaucoma might have not been applied). Therefore, long-term follow-up is essential to facilitate early intervention and management of complications in these patients, such a cataract, retinal detachment, and glaucoma, to prevent vision-threatening disease progression.
Retinal detachment and infantile-onset glaucoma in Stickler syndrome associated with known and novel COL2A1 mutations
Published in Ophthalmic Genetics, 2018
Thomas J. Wubben, Kari H. Branham, Cagri G. Besirli, Brenda L. Bohnsack
Although rarely reported, five eyes of three patients were diagnosed with infantile-onset glaucoma in this series (Table 3). The age of diagnosis in these patients was 3 weeks, 3 months, and 1 year. All eyes with infantile-onset glaucoma presented with buphthalmos and corneal edema; the latter of which prevented adequate gonioscopic examination. The mean axial length of these eyes was 30.1 mm, and the average intraocular pressure (IOP) prior to first glaucoma procedure was 28.9 ± 4.1 mmHg. The spherical equivalent of those eyes diagnosed with infantile-onset glaucoma was significantly greater than those eyes without infantile-onset glaucoma (−19.6 ± 2.5 versus −8.3 ± 4.9, p < 0.001). All of these eyes required surgical intervention beyond angle surgery (trabeculotomy) with a mean number of 5.4 glaucoma procedures. Three of the eyes diagnosed with infantile-onset glaucoma also experienced a RD and became phthisical during follow-up. The two eyes that did not become phthisical at last follow-up had visual acuities of 20/70 and 20/100, with IOP in the mid-teens on multiple ocular antihypertensive medications (Table 1 in the Supplement).