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Year : 2021  |  Volume : 59  |  Issue : 1  |  Page : 95-97

Spontaneous resolution of primary congenital glaucoma

Department of Glaucoma, Glaucoma Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Tirunelveli, Tamil Nadu, India

Date of Submission22-Mar-2020
Date of Acceptance21-Apr-2020
Date of Web Publication27-Mar-2021

Correspondence Address:
Rengappa Ramakrishnan
Department of Glaucoma, Glaucoma Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Tirunelveli, Tamil Nadu
Dr. Shylesh Dabke
Glaucoma Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Tirunelveli - 627 001, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_25_20

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Newborn primary congenital glaucoma (PCG) is an important subtype of PCG. Affected patients manifest specific signs that facilitate its recognition at birth and become important in the determination of appropriate treatment for this severe expression of PCG. Objective clinical evidence supports the occurrence of spontaneous resolution of PCG, explanation for which is unknown. Its mechanism could be related to continued postnatal development of the angle structures in eyes possessing milder angle abnormalities. The anterior segment findings are similar to those of patients with treated PCG such as normal intraocular pressures (IOPs), the optic nerve damage is stable, and filtration angles possess mild or moderate abnormalities. We describe a case of spontaneously resolved PCG in an 8 yearold female presenting with large cornea, Haab's striae, and normal IOP in one eye. Literature review shows limited data about such a case report being described from the Indian subcontinent.

Keywords: Haab's striae, primary congenital glaucoma, spontaneous resolution

How to cite this article:
Dabke S, Ramakrishnan R, Kader MA. Spontaneous resolution of primary congenital glaucoma. TNOA J Ophthalmic Sci Res 2021;59:95-7

How to cite this URL:
Dabke S, Ramakrishnan R, Kader MA. Spontaneous resolution of primary congenital glaucoma. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Oct 20];59:95-7. Available from: https://www.tnoajosr.com/text.asp?2021/59/1/95/312280

  Introduction Top

Primary congenital glaucoma (PCG), the most common hereditary childhood glaucoma, is caused by abnormality in filtration angle and is an important cause of childhood blindness.[1],[2],[3] With an incidence of 1 in 10,000 births,[4] this condition warrants surgery.[1] PCG is a rare condition, even rare is its spontaneous resolution. The rarity of this condition can be ascertained from the fact that only a handful of cases have been reported previously.[5],[6],[7],[8],[9] In this case report, we present a rare case of spontaneously arrested PCG in a 8 year old. Literature review shows limited data about such a case report being described from the Indian subcontinent.

  Case Report Top

An 8-year-old female was presented to us with the chief complaints of occasional watering and photophobia in the right eye (OD) since childhood. She was the second child born of a first-degree consanguineous marriage. There was no significant prenatal history. On further questioning, the mother gave a history of bluish discoloration of the right eye at birth for which some eye drops were prescribed at a local hospital, following which the symptoms subsided.

On examination, best-corrected visual acuity was 2/60 OD and 6/6 OS. Anterior segment evaluation of the right eye showed an enlarged cornea (horizontal diameter of 13 mm) [Figure 1] – anterior segment photograph showing larger right eye cornea] with Haab's striae [Figure 2] – anterior segment photography of the right eye showing Haab's straie] and a sluggishly reacting pupil, and the rest of the findings were within normal limits. Anterior segment evaluation of the left eye (OS) was within normal limits. Intraocular pressure in the right and left eye was 12 mmHg and 16 mmHg, respectively. Gonioscopy showed open angles with normal angle structures. Optic nerve head evaluation showed a cup–disc ratio of 0.6 in the right eye with mild sloping of neuro-retinal rim and a cup–disc ratio of 0.6 with healthy neuro-retinal rim in the left eye [Figure 3] – disc photography]. Rest of the posterior segment was normal. Central corneal thickness was 518 μ and 502 μ in the right and left eyes, respectively. Optical coherence tomography was normal in both eyes with superior and inferior quadrant retinal nerve fiber layer thickness of 107 μ and 120 μ in the right eye and 135 μ and 140 μ in the left eye. Axial length was 23.3 mm in the right eye and 22 mm in the left eye.
Figure 1: Anterior segment photograph showing an increased right eye corneal diameter

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Figure 2: Anterior segment photography of the right eye showing Haab's straie

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Figure 3: Fundus photography

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The patient was diagnosed as a case of spontaneous resolution of PCG with amblyopia in the right eye and was started on occlusion therapy. The patient has been on regular follow-up with us for 1 year, with condition of eyes being status quo, with visual acuity in the right eye showing improvement from 2/60 to 5/60, indicating responsiveness to occlusion therapy. The patient was advised further follow-up and to continue occlusion therapy.

  Discussion Top

PCG results from an abnormality of the trabecular meshwork, causing an increased resistance of aqueous humor outflow.[9],[10],[11],[12],[13],[14] Abnormality in trabecular meshwork can be due to either a developmental arrest or relative immaturity of the trabecular tissue. Initially, Barkan proposed that this defect was caused by an imperforate membrane lining the trabecular meshwork.[15] However, evidence of Barkan's membrane has never been proved by histopathologic studies. Following this initial theory, many histopathologic studies about PCG were described, which highlighted the characteristic features of the disease. In spite of various theories, the proven mechanism of the impaired outflow is still unknown. Furthermore, the penultimate question of why in some rare instances a case of congenital glaucoma with increased intraocular pressure (IOP) develops spontaneous resolution is unanswered.

The earliest description of this unusual presentation of spontaneous resolution was reported in two articles.[6],[16] The first report in 1975 describes a 23-year-old male with amblyopia in the left eye who in the first months of his life presented with a history of photophobia and tearing. Both eyes showed enlargement with break in the Descement's membrane. IOP measurement and optic nerve head appearance were normal.[16] The second report by Lockie and Elder described four cases of spontaneous resolution of PCG. All cases had large corneas, presence of Haab's striae, normal IOP, and cupping.[6] Nagao et al. reported a series of 14 eyes with spontaneously resolved PCG.[5] At the time of diagnosis, large corneas with normal IOP were observed in all the affected eyes and Haab's striae in 10 of the 14 eyes. Mastropasqua et al. reported novel confocal microscopic changes in an adult eye with megalocornea and regressed congenital glaucoma.[9] In a more recent case report by Sanghi et al., a 37-year-old male patient was reported to have spontaneous resolved PCG in the right eye with enlarged cornea and Haab's striae with normal IOP in both eyes.[17]

The mechanism of spontaneous resolution of PCG is unknown. It is documented, however, that the angle continues to develop after birth,[11] and this may result in the disappearance of the abnormal, embryological tissue that is thought to cause precanalicular obstruction. Shaffer and Weiss[18] have also described partial expression of the infantile glaucoma angle anomaly in the “uninvolved” eye of patients with unilateral glaucoma, but without other signs of the disease.

From the above-mentioned reports, spontaneous resolution of congenital glaucoma appears to be a definite entity, though the mechanism is not understood. Recognition of the spontaneous resolution of PCG is important to prevent the performance of unnecessary glaucoma surgery. Prognosis of patients with spontaneous resolution is still not fully understood and warrants further study to recognize recurrent glaucoma. Such cases, however, necessitate a close monitoring for the development of active glaucoma. Parents of these patients must be warned of the symptoms of buphthalmos and told to return at the earliest development of such symptoms.

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 his/her/their consent for his/her/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


Conflicts of interest

There are no conflicts of interest.

  References Top

Ho CL, Walton DS. Primary congenital glaucoma: 2004 update. J Pediatr Ophthalmol Strabismus 2004;41:271-88.  Back to cited text no. 1
Miller SJ. Genetic aspects of glaucoma. Trans Ophthalmol Soc UK 1966;86:425-34.  Back to cited text no. 2
Taylor RH, Ainsworth JR, Evans AR, Levin AV. The epidemiology of pediatric glaucoma: The toronto experience. J AAPOS 1999;3:308-15.  Back to cited text no. 3
Walton DS. Primary congenital open angle glaucoma: A study of the anterior segment abnormalities. Trans Am Ophthalmol Soc 1979;77:746-68.  Back to cited text no. 4
Nagao K, Noël LP, Noël ME, Walton DS. The spontaneous resolution of primary congenital glaucoma. J Pediatr Ophthalmol Strabismus 2009;46:139-43.  Back to cited text no. 5
Lockie P, Elder J. Spontaneous resolution of primary congenital glaucoma. Aust N Z J Ophthalmol 1989;17:75-7.  Back to cited text no. 6
Shaw M, Handley S, Porooshani H, Papadopoulos M. A case of arrested primary congenital glaucoma. Eye (Lond) 2013;27:100.  Back to cited text no. 7
Pollack A, Oliver M. Congenital glaucoma and incomplete congenital glaucoma in two siblings. Acta Ophthalmol (Copenh) 1984;62:359-63.  Back to cited text no. 8
Mastropasqua L, Carpineto P, Ciancaglini M, Nubile M, Doronzo E. In vivo confocal microscopy in primary congenital glaucoma with megalocornea. J Glaucoma 2002;11:83-9.  Back to cited text no. 9
Tawara A, Inomata H. Developmental immaturity of the trabecular meshwork in congenital glaucoma. Am J Ophthalmol 1981;92:508-25.  Back to cited text no. 10
Anderson DR. The development of the trabecular meshwork and its abnormality in primary infantile glaucoma. Trans Am Ophthalmol Soc 1981;79:458-85.  Back to cited text no. 11
Shaffer RN. Pathogenesis of congenital glaucoma; gonioscopic and microscopic anatomy. Trans Am Acad Ophthalmol Otolaryngol 1955;59:297-308.  Back to cited text no. 12
Maul E, Strozzi L, Muñoz C, Reyes C. The outflow pathway in congenital glaucoma. Am J Ophthalmol 1980;89:667-73.  Back to cited text no. 13
Maumenee AE. The pathogenesis of congenital glaucoma: A new theory. Trans Am Ophthalmol Soc 1958;56:507-70.  Back to cited text no. 14
Barkan O. Pathogenesis of congenital glaucoma: Gonioscopic and anatomic observation of the angle of the anterior chamber in the normal eye and in congenital glaucoma. Am J Ophthalmol 1955;40:1-1.  Back to cited text no. 15
Le Rebeller MJ, Lagoutte F. Spontaneous resolution of a case of congenital glaucoma. Bull Soc Ophtalmol Fr 1975;75:555-9.  Back to cited text no. 16
Sanghi G, Brar GS, Gupta R, Ahuja A. A case of spontaneously resolved primary congenital glaucoma. Indian J Ophthalmol 2015;63:618-20.  Back to cited text no. 17
[PUBMED]  [Full text]  
Shaffer RN, Weiss DI. Congenital and Paediatric Glaucoma. St Louis: C.V. Mosby Co.; 1970. p. 115-6.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3]


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