Part I. Definition, classification and differential diagnosis
Consensus 1: Childhood glaucoma (childhoodglaucoma) is an eye injury related to intraocular pressure. Shao Hongzhan, Ophthalmology Department, Hainan Provincial Hospital of Traditional Chinese Medicine
Note: In addition to IOP, optic disc morphology and visual field, this definition also includes the effects of IOP on other ocular tissues in infants and children.
Consensus 2: IOP measurements in infants and younger children, especially when performed under anesthesia, may be influenced by many factors.
Note: Other signs of glaucoma in infants and younger children, such as ocular enlargement, Habb pattern, and increased cup/disc (C/D) ratio, may be more important than IOP values.
Consensus 3: Glaucoma in children is divided into two main categories: primary and secondary. Secondary can be further divided into non-acquired (present at birth) and acquired (present after birth) depending on the condition. Non-acquired childhood glaucoma can also be reclassified according to signs from local or systemic in the eye.
Note: “developmental”, “congenital”, “infantile “The terms glaucoma lack a clear definition and are not recommended.
Consensus 4: Many diseases can have similar presentations to childhood glaucoma, and therefore children should not be labeled as having glaucoma until a definitive diagnosis is made and anti-glaucoma treatment is given as a result.
Diagnosis of glaucoma in children – at least 2 of the following need to be met.
1. IOP >21 mmHg (because the state of anesthesia affects all IOP measurements, the examiner should exercise caution if the IOP value is measured only under anesthesia).
2. Optic disc depression: Progressive increase in C/D; difference in C/D between eyes ≥0.2 when the optic discs are similar in size; or local disc rim thinning.
3, Corneal manifestation: Haab pattern; or corneal diameter ≥11mm (newborns), >12mm (children <1 year old), >13mm (any age).
4, Progressive progression of myopia or myopia accompanied by an eye size that exceeds normal growth.
5. Repeatable visual field defects consistent with the characteristics of glaucomatous optic nerve damage, and the visual field defects cannot be explained by other observable causes.
Suspected diagnosis of glaucoma in children – at least 1 of the following is met.
1. IOP >21 mmHg measured at two different time points.
2. Suspected glaucomatous optic disc appearance (e.g., C/D enlargement that is not proportional to the size of the optic disc).
3.Suspected glaucomatous visual field changes.
4, Increased corneal diameter or elongated eye axis in the presence of normal IOP.
Definition of children: <18 years (US), ≤16 years (UK, Europe, UNICEF) ChildhoodGlaucomaResearchNetwork/WorldGlaucomaAssociation on the classification of childhood glaucoma.
I. Primary childhood glaucoma
1. Primary congenital glaucoma (PCG)
2. Juvenile open-angle glaucoma (JOAG)
Secondary glaucoma in children
1. Glaucoma combined with non-acquired ocular abnormalities
2. Glaucoma combined with non-acquired systemic diseases or syndromes
3. Glaucoma combined with acquired disease
4. Glaucoma in children secondary to cataract surgery
Occurring after cataract surgery and meeting the definition of glaucoma, there are 3 categories.
1)congenital idiopathic cataract
2) congenital cataract combined with ocular abnormalities/systemic disease (no previous glaucoma) 3) acquired cataract (no previous glaucoma)
Differential diagnosis of primary congenital glaucoma (PCG)
A. Glaucoma combined with non-acquired ocular abnormalities related or non-acquired systemic disease/syndrome
B. Eye enlargement: (1) high myopia; (2) large corneas; (3) corneal dilatation
C. Corneal abnormalities: (1) birth injury; (2) Peters abnormality (no IOP elevation or other glaucoma signs); (3) corneal dystrophy (PPMD, CHED); (4) metabolic abnormalities (mucopolysaccharide storage disease, cystinosis).
D. Other causes of photophobia: (1) nasolacrimal duct blockage; (2) conjunctivitis; (3) corneal abrasions/keratitis.
E. Optic nerve: (1) physiological macroglossia; (2) optic nerve hypoplasia; (3) optic nerve defect; (4) optic disc microsaccades; (5) other optic nerve abnormalities.
For a short summary and discussion of the first part.
1, the new diagnosis of childhood glaucoma encompasses damage to other structures of the eye caused by intraocular pressure, not just the optic nerve
2. Glaucoma in children can be simply divided into primary and secondary
3. Glaucoma secondary to cataract surgery stands alone as a new classification, reflecting the importance now attached to this common disease.
Part II Clear diagnosis and judgment of disease progression
Consensus 1: Timely and correct diagnosis and treatment of glaucoma in children can minimize damage to visual function
Note: Examination under anesthesia or sedation can help with diagnosis and further treatment.
Consensus 2: Children should not be labeled as having glaucoma or started on treatment unless the diagnosis has been confirmed and other glaucoma-like conditions have been ruled out.
Note: If the diagnosis is in doubt, or if progression of the disease is uncertain, appropriate periodic follow-up or examination under anesthesia/sedation is recommended.
Consensus 3: Glaucoma in children is characterized by elevated intraocular pressure and a characteristic optic disc depression. In addition to this, glaucoma in infants and children is often accompanied by an enlarged, bulls-eye.
Note: For pediatric glaucoma patients, IOP measurements as well as optic disc examinations should be performed throughout life. Because the structure of the eye in infants with glaucoma is susceptible to high intraocular pressure, if the pressure continues to rise, it will lead to larger corneas, longer eye axes, and the progressive development of myopia. These factors should be taken into account and evaluated regularly.
Consensus 4: The effect of anesthetic drugs on IOP is not yet predictable. All inhaled anesthetics will lower IOP, sometimes rapidly and significantly.
Note: Chloral hydrate, ketamine, and midazolam have not been found to lower IOP and it is recommended that the same anesthetic be used at each examination
Consensus 5: Corneal enlargement is a common feature of all types of glaucoma in infants and young children as well as in younger children.
Note: Corneal enlargement due to elevated intraocular pressure usually occurs before the age of 3 years. Serial measurements of corneal diameter can be helpful in making a definitive diagnosis and monitoring progression before age 3 years. Central corneal thickness (CCT) should not be used to correct IOP values, as its role in children is not yet clear.
Consensus 6: Anterior chamber angioscopy is essential for the correct diagnosis of glaucoma in children and for the development of surgical plans. It should be performed at least once when possible.
Consensus 7: Optic disc appearance is an important and sensitive indicator for the diagnosis of glaucoma in children and for determining disease progression.
Note: Optic disc size, cup/disc ratio, localized defects along the disc and defects in the retinal nerve fiber layer should be recorded in the dilated state. Recording the appearance of the optic disc at baseline and during follow-up is helpful in determining the diagnosis and the response to treatment. Reversal of optic disc depression is common in pediatric glaucoma patients who are effective with treatment. Automated optic nerve imaging systems (e.g., OCT, etc.) are limited in their application due to the lack of standard values for normal populations and the inconvenience of portability.
Consensus 8: When the sclera remains susceptible to IOP, rapid changes in refractive status and eye axis can help in the diagnosis of the disease and in determining whether it is effective for treatment.
Note: An eye axis length above the normal range is highly suggestive of the possibility of glaucoma. Continued growth of the eye axis outside of the normal range suggests that treatment for glaucoma is not yet optimal.
Consensus 9: Visual field examination is valuable but challenging in the evaluation of glaucoma in children.
Note: The “raw” sensitivity of current automated visual field meters and the associated grayscale plots reflect only mean defect (MD), graphical standard deviation (PSD), and graphical and overall deviation plots derived from age-matched data that are not included in current commercial automated visual field meters for children.
Part III Genetics component
Consensus 1: Genetic evaluation is important in children with glaucoma, especially for those glaucoma types for which a correlation between genotype and phenotype has been established.
Note: Examples of genetic diagnoses.
—Absent iris: PAX6
—Primary congenital glaucoma: CYP1B1
— Juvenile open-angle glaucoma (JOAG): MYOC
—Axenfeld-Rieger: PITX2, FOXC1
—Peter anomaly: PAX6, CYP1B1, PITX2, FOXC1
Consensus 2: CYP1B1 gene mutations are more common and are more common in familial cases than in disseminated cases.
Consensus 3: MYOC mutations are found in families with autosomal dominant POAG (JOAG).
Consensus 4: Axenfeld-Rieger anomalies and syndromes are associated with mutations in the PITX2 and FOXC1 genes.
Consensus 5: Absence of iris and PAX6 mutation
Note: Sporadic aniridia: PAX6 mutations are often combined with WT1 gene deletions. Therefore, regular renal ultrasound should be performed in patients with aniridia (to exclude Wilms’ tumor) until WT1 gene deletion is excluded.
Consensus 6: Mutations in the LTBP2 gene can lead to a variety of phenotypes, including lens ectasia, large corneas, small spherical lenses, and secondary glaucoma.
Consensus 7: Accurate clinical diagnosis is essential for the need to provide genetic counseling to families.
Note: Because of differences in epiphenomenon and expression, parents and siblings of affected children need to undergo eye examinations to obtain accurate genetic information.
Consensus 8: Systemic pediatric examination is important for the treatment of children with glaucoma, largely helping to detect systemic abnormalities and prompting early treatment.
Consensus 9: Genetic analysis has an important role in genetic diagnosis, genetic data analysis, and risk of recurrence assessment.