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Table. 
Childhood Glaucomas
Childhood Glaucomas
1.
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Roy  FH Comprehensive developmental glaucoma classification. Ann Ophthalmol 2005;37 (4) 237- 244Article
3.
Ho  CLWalton  DS Primary congenital glaucoma: 2004 update. J Pediatr Ophthalmol Strabismus 2004;41 (5) 271- 288
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4.
Walton  DSKatsavounidou  G Newborn primary congenital glaucoma: 2005 update. J Pediatr Ophthalmol Strabismus 2005;42 (6) 333- 341
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5.
Yeung  HHWalton  DS Goniotomy for juvenile open-angle glaucoma. J Glaucoma 2010;19 (1) 1- 4
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6.
Ritch  RChang  BMLiebmann  JM Angle closure in younger patients. Ophthalmology 2003;110 (10) 1880- 1889
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7.
Badlani  VKQuinones  RWilensky  JTHawkins  AEdward  DP Angle-closure glaucoma in teenagers. J Glaucoma 2003;12 (3) 198- 203
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8.
Patrianakos  TDNagao  KWalton  DS Surgical management of glaucoma with the Sturge Weber syndrome. Int Ophthalmol Clin 2008;48 (2) 63- 78
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9.
Grant  WMWalton  DS Distinctive gonioscopic findings in glaucoma due to neurofibromatosis. Arch Ophthalmol 1968;79 (2) 127- 134
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10.
Castillo  MQuencer  RMGlaser  JAltman  N Congenital glaucoma and buphthalmos in a child with neurofibromatosis. J Clin Neuroophthalmol 1988;8 (1) 69- 71
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11.
Quaranta  LSemeraro  FTurano  RGandolfo  E Gonioscopic findings in patients with type I neurofibromatosis (Von Recklinghausen disease). J Glaucoma 2004;13 (2) 90- 95
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12.
Ziakas  NGRamsay  ASLynch  SAClarke  MP Stickler's syndrome associated with congenital glaucoma. Ophthalmic Genet 1998;19 (1) 55- 58
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13.
Walton  DSKatsavounidou  GLowe  CU Glaucoma with the oculocerebrorenal syndrome of Lowe. J Glaucoma 2005;14 (3) 181- 185
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14.
Shields  MBBuckley  EKlintworth  GKThresher  R Axenfeld-Rieger syndrome: a spectrum of developmental disorders. Surv Ophthalmol 1985;29 (6) 387- 409
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15.
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16.
Brodsky  MCWhiteside-Michel  JMerin  LM Rieger anomaly and congenital glaucoma in the SHORT syndrome. Arch Ophthalmol 1996;114 (9) 1146- 1147
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17.
Bowen  PLee  CSZellweger  HLindenberg  R A familial syndrome of multiple congenital defects. Bull Johns Hopkins Hosp 1964;114402- 414
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18.
Punnett  HHKirkpatrick  JA  Jr A syndrome of ocular abnormalities, calcification of cartilage, and failure to thrive. J Pediatr 1968;73 (4) 602- 606
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19.
Folz  SJTrobe  JD The peroxisome and the eye. Surv Ophthalmol 1991;35 (5) 353- 368
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20.
Izquierdo  NJTraboulsi  EIEnger  CMaumenee  IH Glaucoma in the Marfan syndrome. Trans Am Ophthalmol Soc 1992;90111- 122
PubMed
21.
Brei  TJBurke  MJRubinstein  JH Glaucoma and findings simulating glaucoma in the Rubinstein-Taybi syndrome. J Pediatr Ophthalmol Strabismus 1995;32 (4) 248- 252
PubMed
22.
Quaranta  LQuaranta  CA Congenital glaucoma associated with Rubinstein-Taybi syndrome. Acta Ophthalmol Scand 1998;76 (1) 112- 113
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23.
van Genderen  MMKinds  GFRiemslag  FCHennekam  RC Ocular features in Rubinstein-Taybi syndrome: investigation of 24 patients and review of the literature. Br J Ophthalmol 2000;84 (10) 1177- 1184
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24.
Hallenborg  CP Congenital glaucoma, cataplexy and mental retardation in a mother and son: a “new” syndrome. Birth Defects Orig Artic Ser 1971;7 (3) 207- 208
PubMed
25.
Traboulsi  EIParks  MM Glaucoma in oculo-dento-osseous dysplasia. Am J Ophthalmol 1990;109 (3) 310- 313
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26.
Vasconcellos  JPMelo  MBSchimiti  RBBressanim  NCCosta  FFCosta  VP A novel mutation in the GJA1 gene in a family with oculodentodigital dysplasia. Arch Ophthalmol 2005;123 (10) 1422- 1426
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27.
Holmes  LBWalton  DS Hereditary microcornea, glaucoma, and absent frontal sinuses: a family study. J Pediatr 1969;74 (6) 968- 972
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28.
Nowaczyk  MJClarke  JTMorin  JD Glaucoma as an early complication of Hurler's disease. Arch Dis Child 1988;63 (9) 1091- 1093
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29.
Mullaney  PAwad  AHMillar  L Glaucoma in mucopolysaccharidosis 1-H/S. J Pediatr Ophthalmol Strabismus 1996;33 (2) 127- 131
PubMed
30.
Lichter  PRSchmickel  RD Posterior vortex vein and congenital glaucoma in a patient with trisomy 13 syndrome. Am J Ophthalmol 1975;80 (5) 939- 942
PubMed
31.
Guirgis  MFWong  AMTychsen  L Infantile glaucoma and punctal atresia in a child with caudal regression syndrome. J AAPOS 2003;7 (4) 298- 299
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32.
Traboulsi  EILevine  EMets  MBParelhoff  ESO’Neill  JFGaasterland  DE Infantile glaucoma in Down's syndrome (trisomy 21). Am J Ophthalmol 1988;105 (4) 389- 394
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33.
Catalano  RA Down syndrome. Surv Ophthalmol 1990;34 (5) 385- 398
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34.
Sato  SEHerschler  JLynch  PJ  et al.  Congenital glaucoma associated with cutis marmorata telangiectatica congenita: two case reports. J Pediatr Ophthalmol Strabismus 1998;2513- 17
PubMed
35.
Murphy  CCKhong  CHWard  WJMorgan  WH Late-onset pediatric glaucoma associated with cutis marmorata telangiectatica congenita managed with Molteno implant surgery: case report and review of the literature. J AAPOS 2007;11 (5) 519- 521
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36.
Rehany  USegal  ZIRumelt  S Congenital unilateral buphthalmos in Walker-Warburg syndrome: a clinicopathological study. Eye (Lond) 1999;13 (pt 6) 778- 780
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37.
Khalaf  SSTareef  RB Walker-Warburg syndrome. J AAPOS 2006;10 (5) 486- 488
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38.
Maumenee  IHTraboulsi  EI The ocular findings in Kniest dysplasia. Am J Ophthalmol 1985;100 (1) 155- 160
PubMed
39.
Douglas  GR The ocular findings in Kniest dysplasia. Am J Ophthalmol 1985;100 (6) 860- 861
PubMed
40.
Mawn  LAO’Brien  JEHedges  TR  III Congenital glaucoma and skeletal dysplasia. J Pediatr Ophthalmol Strabismus 1990;27 (6) 322- 324
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41.
De La Paz  MALewis  RAPatrinely  JRMerin  LGreenberg  F A sibship with unusual anomalies of the eye and skeleton (Michels' syndrome). Am J Ophthalmol 1991;112 (5) 572- 580
PubMed
42.
Cohen  JS Congenital nonprogressive facial hemiatrophy with ipsilateral eye abnormalities and juvenile glaucoma. Ann Ophthalmol 1979;11 (3) 413- 416
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43.
Coats  DKPaysse  EALevy  ML PHACE: a neurocutaneous syndrome with important ophthalmologic implications: case report and literature review. Ophthalmology 1999;106 (9) 1739- 1741
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44.
Lasky  JBSandu  MBalashanmugan  A PHACE syndrome: association with persistent fetal vasculature and coloboma-like iris defect. J AAPOS 2004;8 (5) 495- 498
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45.
Yen  MTGedde  SJFlynn  JT Unilateral glaucoma in Sotos syndrome (cerebral gigantism). Am J Ophthalmol 2000;130 (6) 851- 853
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46.
Stone  RAScheie  HG Periorbital scleroderma associated with heterochromia iridis. Am J Ophthalmol 1980;90 (6) 858- 861
PubMed
47.
Mullaney  PBJacquemin  Cal-Rashed  WSmith  W Growth retardation, alopecia, pseudoanodontia, and optic atrophy (GAPO syndrome) with congenital glaucoma. Arch Ophthalmol 1997;115 (7) 940- 941
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48.
Ilker  SSOztürk  FKurt  ETemel  MGül  DSayli  BS Ophthalmic findings in GAPO syndrome. Jpn J Ophthalmol 1999;43 (1) 48- 52
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49.
Mandal  AKSingh  APRao  LSingh  LHornby  SJones  B Roberts pseudothalidomide syndrome. Arch Ophthalmol 2000;118 (10) 1462- 1463
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50.
Finzi  SPinto  CFWiggs  JL Molecular and clinical characterization of a patient with a chromosome 4p deletion, Wolf-Hirschhorn syndrome, and congenital glaucoma. Ophthalmic Genet 2001;22 (1) 35- 41
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51.
Saal  HMGreenstein  RMWeinbaum  PJPoole  AE Autosomal recessive Robinow-like syndrome with anterior chamber cleavage anomalies. Am J Med Genet 1988;30 (3) 709- 718
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53.
Mimiwati  ZMackey  DACraig  JE  et al.  Nail-patella syndrome and its association with glaucoma: a review of eight families. Br J Ophthalmol 2006;90 (12) 1505- 1509
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54.
Mayatepek  EKurczynski  TWRuppert  ESHennessy  JRBrinker  RAFrench  BN Expanding the phenotype of the Proteus syndrome: a severely affected patient with new findings. Am J Med Genet 1989;32 (3) 402- 406
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55.
Tunnessen  WW  JrLowenstein  EH Glaucoma associated with the fetal hydantoin syndrome [letter]. J Pediatr 1976;89 (1) 154- 155
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56.
Saraiva  JMGama  EPires  MMSequeira  JF First report of glaucoma as a feature of the 3C syndrome. Clin Dysmorphol 1995;4 (2) 156- 160
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57.
Gupta  GWalton  DS Infantile glaucoma associated with cranio-cerebello-cardiac syndrome. J Pediatr Ophthalmol Strabismus 2006;43 (4) 244- 245
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58.
Lee  WBBrandt  JDMannis  MJHuang  CQRabin  GJ Aniridia and Brachmann-de Lange syndrome: a review of ocular surface and anterior segment findings. Cornea 2003;22 (2) 178- 180
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59.
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60.
Turaçli  METekeli  O Infantile glaucoma in a patient with Rothmund-Thomson syndrome. Can J Ophthalmol 2004;39 (6) 674- 678
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61.
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62.
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63.
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64.
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65.
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66.
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69.
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70.
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71.
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73.
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74.
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75.
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76.
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77.
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78.
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81.
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82.
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84.
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85.
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Clinical Sciences
June 2010

Clinical Classification of Childhood Glaucomas

Author Affiliations

Author Affiliations: Department of Pediatrics, Massachusetts General Hospital (Dr Yeung), and Department of Ophthalmology, Massachusetts Eye and Ear Infirmary (Dr Walton), Harvard Medical School, Boston, Massachusetts.

Arch Ophthalmol. 2010;128(6):680-684. doi:10.1001/archophthalmol.2010.96
Abstract

Objective  An updated classification of the primary and secondary childhood glaucomas is offered for clinical use, and associated systemic diseases are included to enable their early recognition in children with known glaucoma.

Methods  Approximately 650 clinical records of patients with pediatric glaucoma were reviewed for type of glaucoma and associated systemic disease. A literature search was done for additional reported causes of childhood glaucoma. Previous classifications of pediatric glaucomas were also reviewed. Pertinent references to support inclusion of each clinical entity in the updated classification are included.

Results  A comprehensive and referenced classification of the pediatric glaucomas was enabled by this review.

Conclusion  A comprehensive, etiologically based classification of the pediatric glaucomas is now available to assist with the recognition of the many causes of primary and secondary glaucoma in childhood and to support the selection of specific treatment choices.

The childhood glaucomas have been classified by the age of onset, inheritance, associated systemic findings, and anatomy, according to the associated and responsible anterior segment anomalies.1,2 In this article, we offer a comprehensive classification of the childhood glaucomas to assist in the recognition and differential diagnosis of the reported clinically recognizable causes of primary and secondary pediatric glaucomas (Table).

TERMINOLOGY

Historically, the childhood glaucomas have been labeled developmental glaucomas based on the associated presence of developmental defects of the eye.1Primary childhood glaucomas will be classified as those caused by anomalies of the filtration angle. These glaucomas are often of genetic origin and may be associated with systemic diseases and other ocular defects. We have identified the systemic diseases that have been described in association with childhood glaucoma.

Congenital glaucoma denotes a glaucoma that occurs early in life related to a congenital anomaly. Newborn primary congenital glaucoma defines a glaucoma entity that is recognized immediately at birth with the presence of profound defects of the anterior segment. Infantile primary congenital glaucoma includes patients with evidence of glaucoma most often recognized in the first year of life. Late recognized primary congenital glaucoma indicates an entity diagnosed significantly after an age when ophthalmologic examination of the patient would have recognized the presence of abnormalities related to glaucoma.

Juvenile glaucoma has been used to describe glaucoma in childhood. We have continued its use specifically with juvenile open-angle glaucoma that characteristically develops during childhood.

The secondary childhood glaucomas are those that occur as the result of independent disease mechanisms that secondarily impair the function of the filtration angle.

COMMENT

All classifications of the childhood glaucomas have revealed the impressive number of clinical entities that may feature or be complicated by childhood glaucoma. Previously these diseases have been variably identified and classified.2 The term association in reference to glaucoma with systemic diseases does not require that the glaucoma is an essential aspect of the disease. In our classification, we list these entities together; however, the strength of the glaucoma relationship might be different. Glaucoma may be coincidental or strongly genetically related to the systemic disease. In the future, additional clinical experience and genetic testing may establish the relative importance of these relationships.

The use of this classification as a clinical aid can facilitate early recognition of glaucoma and identification of the specific glaucoma diagnosis as well as meaningfully influence the choice of treatment. Without recognition of specific types of childhood glaucoma, it is more difficult for the clinician to appropriately select treatment that has been found to be most beneficial. The secondary glaucomas are an important and large group of childhood glaucomas. When confronted with a child with glaucoma and atypical clinical findings, the clinician may be assisted in making an accurate etiologic diagnosis by reviewing the tabulated causes of both the primary and secondary glaucomas. Finally, this childhood glaucoma classification can enable improved communication between those who care for these particular patients.

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Article Information

Correspondence: David S. Walton, MD, 2 Longfellow Place, Ste 201, Boston, MA 02114 (walton.blackeye@gmail.com).

Submitted for Publication: August 21, 2009; final revision received August 21, 2009; accepted November 12, 2009.

Author Contributions: The authors had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the analysis and presentation.

Financial Disclosure: None reported.

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