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BRIEF REPORT
Year : 2006  |  Volume : 54  |  Issue : 4  |  Page : 275-276

Pupillary-iris-lens membrane with goniodysgenesis: A case report


Department of Glaucoma, Glaucoma services, Aravind Eye Hospital, 1, Anna Nagar, Madurai - 625020, Tamilnadu, India

Correspondence Address:
Nitin Deshpande
Glaucoma Services, Aravind Eye Hospital, 1, Anna Nagar, Madurai - 625020, Tamilnadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0301-4738.27957

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  Abstract 

We describe a rare case of pupillary-iris-lens membrane with goniodysgenesis, a unilateral neurocristopathy. The membrane represents ectopic iris on the lens with abnormal iris stroma and anterior chamber angle from aberrant induction, migration or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. This subject needed treatment for amblyopia.

Keywords: Amblyopia, goniodysgenesis, pupillary-iris-lens membrane, neurocristopathy


How to cite this article:
Deshpande N, Shetty S, Krishnadas S R. Pupillary-iris-lens membrane with goniodysgenesis: A case report. Indian J Ophthalmol 2006;54:275-6

How to cite this URL:
Deshpande N, Shetty S, Krishnadas S R. Pupillary-iris-lens membrane with goniodysgenesis: A case report. Indian J Ophthalmol [serial online] 2006 [cited 2020 May 27];54:275-6. Available from: http://www.ijo.in/text.asp?2006/54/4/275/27957

Cibis et al.[1] have reported a distinct variety of neurocristopathy different from Rieger's anomaly and persistent pupillary membrane, called congenital iris lens membrane with goniodysgenesis. It has been hypothesized that this condition is unilateral and represents ectopic iris on the lens with abnormal iris stroma and anterior chamber angle from aberrant induction, migration and regression of neural crest cells. The significance of the diagnosis of this condition lies in the fact that the membrane could result in progressive angle closure and visual loss from glaucoma or stimulus deprivation amblyopia from occlusion of the visual axis. We report here a case that presented with similar clinical features as described by Cibis et al. and needed treatment for amblyopia, although glaucoma was not a characteristic feature.


  Case Report Top


A seven-year-old female child of South Indian ethnicity presented with defective vision in the left eye. The child's mother had noticed a white spot in the left eye since birth. However, no ophthalmic opinion was sought for the condition. She was seen at the age of five years at the Aravind Eye Hospital and was advised patching and glasses. But there was poor compliance for patching schedule and follow-up.

On examination her best corrected vision in the right eye was 20/20p and in the left eye 20/80. She maintained central steady fixation in both eyes. Binocular single vision for near and distance was present with stereopsis of 240o with TNO random dot stereogram test.

On slit lamp examination, right eye was normal while left eye showed prominent Schwalbe's line. There was a thick white membrane ringing the pupil, attaching the iris to the lens and distorting the pupillary shape. The membrane extended along the pupillary margin from 10 o'clock to 4 o'clock. Clear lens was visible partly in the pupillary area not covered with membrane. Two strands of the membrane extended to the prominent Schwalbe's line at 10 o' clock and 2 o'clock [Figure - 1]a.

Gonioscopy showed broad leaves of iris stroma attached to Schwalbe's line throughout the circumference of the anterior chamber angle. Clinical appearance of anterior chamber angle [Figure - 1]b resembled that described in goniodysgenesis.[2] Intraocular pressure by applanation tonometry was 12 mmHg and 11mmHg in the right eye and left eye respectively. Horizontal corneal diameter was 10.5 mm in both eyes and the axial length was 21.96 mm in the right eye and 21.16 in the left eye. The left pupil could not be sufficiently dilated precluding view of the posterior segment, while the right eye showed a normal retina.

Ultrasound biomicroscopy [Figure - 2] showed broad iris stromal leaves inserting into Schwalbe's line with pseudo-angle formation, features suggestive of goniodysgenesis.[2] There was tenting of peripheral iris which appeared to be pulled away from the peripheral lens surface. Urine examination to rule out microscopic hematuria, was normal.

Ultrasonography and Doppler study of the renal vascular system was done which ruled out any congenital anomalies of the genitouirnary system.[3] We advised partial patching of right eye for six hours per day and regular review every four months.


  Discussion Top


Congenital pupillary iris lens membrane with goniodysgenesis is a unilateral neurocristopathy and is reported here for its rarity. To the best of our knowledge, this is the first report of the condition from India. Alkemade had referred to this disorder as Pseudo- Rieger's anomaly as he believed that it represented localized disruption in the development of the anterior chamber angle. Though the membrane was attached to prominent Schwalbe's line, unilateral involvement and absence of iris hypoplasia distinguished it from Axenfeld Rieger anomaly.[4] The iris lens membrane, unlike a persistent pupillary membrane did not originate from the iris collarette, but was attached posteriorly to the pigment layer of the pupillary margin and to the anterior iris stroma and continued onto a prominent Schwalbe's line (Duke Elders' classification of persistent pupillary membrane).

Cibis et al.[1] reported prominence of tufts of vessels along the membrane seen in a case at three weeks of age and their disappearance one year later. These were reminiscent of the neovascularization with fibrosis and resolution of such vessels which is common in diabetic retinopathy and retinopathy of prematurity. In our case since evolution of the membrane was complete, fluoroscein aniographic studies were not done.

Early recognition of this rare condition is important since Cibis et al.[1] have reported that a significant number of eyes develop pupillary block with sectoral iris bombe and angle closure glaucoma. Visual loss due to this condition is preventable by peripheral iridectomy to reverse angle closure glaucoma. Apart from glaucoma, Cibis et al. had also warned about the development of amblyopia in these individuals due to occlusion of the visual axis by the membrane.[5]

The child reported herein is being followed up on a periodic basis to exclude angle closure glaucoma, but had already developed visual deprivation amblyopia possibly due to partial occlusion of the pupillary axis by the congenital iris lens membrane. This child required no more intervention than occlusion therapy and best corrected spectacles since she had good stereopsis. Earlier recognition of dense membrane obscuring pupillary axis is important as it can be corrected by surgical excision of the membrane to prevent stimulus deprivation amblyopia, since the lens and posterior segment are usually normal behind the membrane.



 
  References Top

1.
Cibis GW, Waltermann JM, Hurst E, Tripathi RC, Richardson W. Congenital pupillary-iris-lens membrane with goniodysgenesis (A new entity). Ophthalmology 1986;93:847-52.  Back to cited text no. 1
    
2.
Jerndal T. Dominant goniodysgenesis with late congenital glaucoma. A re-examination of Berg's pedigree. Am J Ophthalmol 1972;74:28-33.  Back to cited text no. 2
[PUBMED]    
3.
Beauchamp GR, Knepper PA. Role of neural crest in anterior segment development and diseases. J Pediatr Ophthalmol Strabismus 1984;21:209-14.  Back to cited text no. 3
[PUBMED]    
4.
Shields MB. Axenfeld-Rieger syndrome: A theory of mechanism and distinctions from the iridocorneal endothelial syndrome. Trans Am Ophthalmol Soc 1983;81:736-84.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.
Cibis GW, Walton DS. Congenital pupillary-iris-lens membrane with goniodysgenesis. J AAPOS 2004;8:378-83.   Back to cited text no. 5
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure - 1], [Figure - 2]


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