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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 2  |  Issue : 1  |  Page : 11

Juvenile glaucoma: A chance encounter in the consultation


1 Fitero Health Center, Tudela Health Area, Navarra, Spain
2 Hospital of Alcañiz, Alcañiz Health Area, Teruel, Spain
3 Los Barrios Health Center, Cadiz Health Area, Cádiz, Spain

Date of Submission12-Mar-2020
Date of Decision03-Apr-2020
Date of Acceptance09-Apr-2020
Date of Web Publication27-May-2020

Correspondence Address:
Dr Ibanez Navarro Alberto
168 Pilon's Way Street, Zaragoza 50011
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/PAJO.PAJO_12_20

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  Abstract 


Juvenile glaucoma is a rare entity, starting below the age of 35–40 years, which is discovered as a casual finding in reviews or consultations for other reasons. It is inherited from autosomal dominant penetrance. It can lead to a loss of visual acuity. The treatment is similar to conventional glaucoma, although its control, when diagnosed, is usually late and usually leads to more complex management. The differential diagnosis of glaucoma by steroids, trauma, inflammatory, or congenital late diagnosis must be made. The main difference with other types of glaucoma is that it is generally asymptomatic, and there is no corneal edema or Haag striae. Surgery is an alternative when drug treatment fails. Through a real case, we want to introduce and develop this ophthalmological pathology. This is a 25-year-old male with a history of myopia, who at a casual check-in an optician's shop is found to have increased intraocular pressure (IOP). In the campimetry was seen a ring scotoma (right eye) and an arciform scotoma (left eye [LE]). Distant vision was preserved. In optical coherence tomography there was bilateral decrease of retinal nerve fiber layer thickness , and in the right eye (RE) gonoscopy showed a highly pigmented Grade IV open angle. Initially, pharmacological management was attempted (with carbonic anhydrase inhibitors, beta-blockers and prostaglandin analogs) by achieving it in the LE. However, the RE ended up with a trabeculectomy, obtaining, in the end, a good control of the IOP.

Keywords: Congenital, glaucoma, ocular hypertension, open-angle glaucoma, trabeculectomy


How to cite this article:
Alberto IN, Julián TC, Sofía VS, Adela RP, José SG. Juvenile glaucoma: A chance encounter in the consultation. Pan Am J Ophthalmol 2020;2:11

How to cite this URL:
Alberto IN, Julián TC, Sofía VS, Adela RP, José SG. Juvenile glaucoma: A chance encounter in the consultation. Pan Am J Ophthalmol [serial online] 2020 [cited 2020 Dec 1];2:11. Available from: https://www.thepajo.org/text.asp?2020/2/1/11/285041




  Resumen Top


El glaucoma juvenil es una entidad poco frecuente, de inicio por debajo de los 35-40 años que SE descubre como hallazgo casual en revisiones o consulta por otros motivos. Es heredidatorio de penetrancia autosómica dominante. Puede llevar a una pérdida de agudeza visual. El tratamiento es similar al glaucoma convencional, aunque su control al diagnosticarse generalmente tardiamente suele llevar a un manejo más complejo. Se debe realizar el diagnóstico diferencial del glaucoma por esteroides, traumatismo, inflamatorio o congénito de diagnóstico tardío. La principal diferencia con otros tipos de glaucoma es que es generalmente asintomático y no existe edema corneal ni estrías de Haag. La cirugía es la alternativa cuando fracasa el tratamiento farmacológico.

A través de un caso real queremos introducir y desarrollar esta patología oftalmológica. Se trata de un varón de 25 años, con antecedentes de miopía, que en una revisión casual en una óptica SE evidencia un aumento de presión intraocular. En la campimetría SE aprecia un escotoma anular (OD) y un escotoma arciforme (OI). Visión lejana conservada. En el OCT una disminución de espesor generalizada bilateral y en la gonoscopia ojo derecho un ángulo abierto grado IV muy pigmentado. Inicialmente SE intentó manejar farmacológicamente (con inhibidores de anidrasa carbónica, betabloqueantes y análogos de las prostaglandinas) consiguiéndolo en el ojo izquierdo. El ojo derecho acabó con una trabeculectomía obteniendo al final un buen control de la presión intraocular.

[congénito] [glaucoma] [hipertensión ocular] [glaucoma de ángulo abierto] [trabeculectomía]


  Introduction Top


Juvenile glaucoma is an unusual pathology and difficult to diagnose early since it is generally asymptomatic. This leads, at the time of diagnosis, to an already established pathology with a possible greater difficulty of management or a higher risk of complications. Through this real case, we intend to give a general overview of this pathology, develop the main characteristics that differentiate it from other types of glaucoma and raise awareness among professionals to provide the necessary tools for its early diagnosis and management.


  Case Report Top


A 25-year-old male with a history of myopia at an optical check-up showed increased intraocular pressure (IOP). Active smoking (20 cigarettes/day). There were no other chronic diseases.

The tests carried out showed an alteration: (1) IOP: Right eye (RE) 51 mmHg OI 52 mmHg (2) Fundus: RE: Good coloring papillae with edge-to-edge excavation (except in nasal). Left eye (LE): Digging 0.5 to temporary edge. (3) Campimetry: RE: Deep annular scotoma LE: Incomplete lower arciform scotoma (4) Optical coherence tomography: RE generalized retinal nerve fiber layer thickness reduction (except nasal) LE: Thickness decrease in the upper temporal and lower nasal area (5) Gonioscopy RE: A highly pigmented Grade IV open angle (6) Distant visual acuity: Normal.

He started treatment with: Acetazolamide 250 mg 1 comp/8 h; Bimatoprost 0.3 mg/ml 1 drop/24 h; timolol/dorzolamine 20 mg/ml + 5 mg/ml 1 drop/12 h.

After several weeks, and several medication adjustments, the drug treatment fails (IOP RE: 40 LE 26) so it was decided to intervene surgically: A trabeculectomy of RE, keeping the pharmacological treatment in the LE. After de surgery, he get good control just today. Last ophthalmologic checkup IOP RE 12 LE 13.


  Discussion Top


Juvenile glaucoma usually occurs between the ages of 10–35 years,[1] although the average is 18 years old at the time of diagnosis. It has an incidence of 1/40,000–60,000 individuals.[2],[3] Initially it is asymptomatic and it is usually a casual finding the ophthalmological checkup, company checkup or contact lenses replacement in opticians.[1] It is usually bilateral although it can be more pronounced in one eye. IOP progressively increases until the optic nerve is excavated, resulting in a loss of field and visual acuity.[4]

The cause of its onset is due to problems in the drainage of the aqueous humor through the transverse mesh. It is an autosomal dominant transmission disease with a high degree of penetrance. It is usually related to mutations of certain genes (CYP1B1 and MYOC).[5],[6],[7] Family members at risk for developing childhood glaucoma can be studied.

The diagnosis requires a complete ophthalmological study. It shows increased IOP and excavation of the optic nerve. Gonioscopy is usually normal and there is usually no corneal edema or Haag stria as in primary congenital glaucoma.[8] In addition, it is often the case that there is a history of myopia and/or field defects. The differential diagnosis with steroid-induced, traumatic, or inflammatory glaucoma should be made.[9],[10] Furthermore, especially in low resource populations without pediatric well child checkups, with a congenital glaucoma diagnosed late.[8]

As with other glaucomas, pharmacological treatment is the most effective and therefore, the first option.[11] By means of drugs such as carbonic anhydrase inhibitors (acetazolamide, dorzolamine, diclofenamide, brinzolamide), beta-blockers (timolol), and/or prostaglandin analogs (Latanoprost, Bimatoprost, travoprost, Tafluprost). Usually different combinations are used, individualized according to the complexity of handling. In case of failure, it is proposed for surgical treatment:[12],[13],[14] surgery in the drainage angle (goniotomy, trabeculotomy), filtration (trabeculectomy) and with laser (in the drainage angle or with cyclodiod laser) or aqueous humor derivation devices. Several studies compare some techniques with others, reaching the conclusion that, again, each case is different and should be individualized and followed until the IOP is stabilized and well controlled.


  Conclusions Top


  • Juvenile glaucoma is usually asymptomatic, and that is where the danger lies, since diagnosis is usually late and management more complicated than with other types of glaucoma
  • A complete ophthalmological study should be performed, where unlike other types of glaucoma, there is usually no corneal edema or Haag stria
  • Combined drug treatment is the therapy of choice, reserving in resistant cases surgery in one or both eyes.


Acknowledgments

We would like to thank the protagonist of our article who from the first moment was willing and satisfied that we all learned from his illness. We also thank all the primary care professionals whose main role in prevention is vital to the early fight against disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflict of interest.



 
  References Top

1.
Kwun Y, Lee EJ, Han JC, Kee C. Clinical characteristics of juvenile-onset open angle glaucoma. Korean J Ophthalmol 2016;30:127-33.  Back to cited text no. 1
    
2.
Clark L, Taubman S, Stahlman S. Update: Incidence of glaucoma diagnoses, active component, U.S. Armed Forces, 2013-2017. MSMR 2019;26:15-9.  Back to cited text no. 2
    
3.
Marx-Gross S, Laubert-Reh D, Schneider A, Höhn R, Mirshahi A, Münzel T, et al. The Prevalence of Glaucoma in Young People. Dtsch Arztebl Int 2017;114:204-10.  Back to cited text no. 3
    
4.
Dietze J, Blair K, Havens SJ. Glaucoma. StatPearls; 2020. Available from: https://www.ncbi.nlm.nih.gov/books/nbk538217/. [last updated on 2019 Nov 22].  Back to cited text no. 4
    
5.
Reis LM, Tyler RC, Weh E, Hendee KE, Kariminejad A, Abdul-Rahman O, et al. Analysis of CYP1B1 in pediatric and adult glaucoma and other ocular phenotypes. Mol Vis 2016;22:1229-38.  Back to cited text no. 5
    
6.
Criscione J, Ji W, Jeffries L, McGrath JM, Soloway S, Pusztai L, et al. Identification of a novel MYOC variant in a Hispanic family with earlyonset primary openangle glaucoma with elevated intraocular pressure. Cold Spring Harb Mol Case Stud 2019;13:5.  Back to cited text no. 6
    
7.
Kaur A, Vanita V, Singh J. Screening of CYP1B1 Arg368His as predominant mutation in North Indian primary open angle glaucoma and juvenile onset glaucoma patients. Mol Biol Res Commun 2018;7:181-6.  Back to cited text no. 7
    
8.
Abu-Amero KK, Edward DP. Primary Congenital Glaucoma. GeneReviews®; 30 September, 2004. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1135/. [Last updated on 2017 Aug 17].  Back to cited text no. 8
    
9.
Kaur S, Kaushik S, Singh Pandav S. Traumatic Glaucoma in Children. J Curr Glaucoma Pract 2014;8:58-62.  Back to cited text no. 9
    
10.
El Afrit MA, Mazlout H, Trojet S, Larguech L, Megaieth K, Belhaj S, et al. Cortisone glaucoma: Epidemiological, clinical, and therapeutic study. J Fr Ophtalmol 2007;30:49-52.  Back to cited text no. 10
    
11.
Thygesen J. Glaucoma therapy: Preservative-free for all? Clin Ophthalmol 2018;12:707-17.  Back to cited text no. 11
    
12.
Grover DS, Smith O, Fellman RL, Godfrey DG, Butler MR, Montes de Oca I, et al. Gonioscopy assisted transluminal trabeculotomy: An ab interno circumferential trabeculotomy for the treatment of primary congenital glaucoma and juvenile open angle glaucoma. Br J Ophthalmol 2015;99:1092-6.  Back to cited text no. 12
    
13.
Young JW, Caprioli J. Laser trabeculoplasty as first-line glaucoma treatment. Lancet 2019;393:1479-80.  Back to cited text no. 13
    
14.
Khalil DH, Abdelhakim MA. Primary trabeculotomy compared to combined trabeculectomy-trabeculotomy in congenital glaucoma: 3-year study. Acta Ophthalmol 2016;94:e550-4.  Back to cited text no. 14
    




 

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