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   Table of Contents      
BRIEF REPORT
Year : 2001  |  Volume : 49  |  Issue : 4  |  Page : 265-6

Congenital retinoschisis : successful collapse with photocoagulation.


Vision and Medical Research Foundation, Chennai, India

Correspondence Address:
L Gopal
Vision and Medical Research Foundation, Chennai
India
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Source of Support: None, Conflict of Interest: None


PMID: 12930120

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  Abstract 

We report a case of progressive congenital retinoschis is where the schisis cavity collapsed following argon laser photocoagulation. Despite reports to the contrary, we feel that in the absence of significant vitreous or inner layer traction, photocoagulation applied as light burns in the schisis cavity may be beneficial in collapsing the cavity. Whether or not this collapse of the schisis cavity reduces the risk of rhegmatogenous retinal detachment is unclear.

Keywords: Humans, Infant, Laser Coagulation, Male, Retinoschisis, congenital, surgery, Treatment Outcome,


How to cite this article:
Gopal L, Shanmugam M P, Battu R R, Shetty N S. Congenital retinoschisis : successful collapse with photocoagulation. Indian J Ophthalmol 2001;49:265

How to cite this URL:
Gopal L, Shanmugam M P, Battu R R, Shetty N S. Congenital retinoschisis : successful collapse with photocoagulation. Indian J Ophthalmol [serial online] 2001 [cited 2019 Dec 7];49:265. Available from: http://www.ijo.in/text.asp?2001/49/4/265/14690

We report a case of progressive congenital retinoschis is where the schisis cavity collapsed following argon laser photocoagulation. Despite reports to the contrary, we feel that in the absence of significant vitreous or inner layer traction, photocoagulation applied as light burns in the schisis cavity may be beneficial in collapsing the cavity. Whether or not this collapse of the schisis cavity reduces the risk of rhegmatogenous retinal detachment is unclear.

Congenital retinoschisis is a rare bilateral disease transmitted as an X -linked recessive trait. Clinical features are varied and include inferotemporal retinoschisis, vitreous haemorrhage and foveal schisis. Management of this condition is controversial and treatment is recommended usually only when there is vitreous haemorrhage or retinal detachment. Various treatment options for retinoschisis itself include laser photocoagulation, [1,2] cryotherapy, scleral buckle[3] and parsplana vitrectomy. [3,4]


  Case report Top


A 21-month-old male child was seen in 1991 with a history of intermittent deviation of the left eye noticed from the age of 6 months. Anterior segment examination of the right eye was normal, while the left eye had a convergent squint and a yellow reflex in the pupillary region. Examination under general anaesthesia showed normal corneal diameter and intraocular pressure (IOP) in both the eyes. Fundus of the right eye showed retinoschisis in the inferotemporal quadrant and a discrete retinal cyst within the schisis area with a speck of intracystic haemorrhage and a foveal schisis. The left eye showed a large and highly elevated retinal cyst involving the entire temporal retina up to the optic disc with the elevation reaching up to the posterior lens surface. The intracystic fluid was turbid and contained cholesterol crystals. It appeared that the left eye would not benefit by treatment and hence we advised a regular check-up for the right eye.

The child was examined twice between 1991 and 1996 during which time the right fundus showed development and subsequent resolution of intracystic haemorrhage while the schisis itself was nonprogressive. His vision in 1991 was 1 /9 on Allen card testing and was 6/36; N6 in 1996.

Evaluated in July 1998, the vision in his right eye was 6/60, N8. The right fundus showed extensive progression of retinoschisis that had spread circumferentially and posteriorly, sparing only a small area enclosed within the arcades and a strip of retina around the horizontal meridian temporally. The large retinal cyst in the inferotemporal quadrant was persistent [Figure - 1]. Rhegmatogenous retinal detachment was not identified. In view of the demonstrable progression and the extensive nature of retinoschisis in a one-eyed boy; scatter argon green laser photocoagulation using the indirect laser delivery system (Coherent Novus 2000; Coherent Inc. Santa Clara, USA) was done to the entire area of schisis on an empirical basis. The laser parameters were: power - 300-400 mW; duration: 100 milliseconds, no. of burns:1767.

When examined 2 months later, the vision in his right eye was 6/36;N6 and there was a total collapse of the schisis cavity. The cyst in the inferotemporal quadrant, however, persisted [Figure - 2]. Over a 2-year follow-up his vision reduced to 3/36, N12 due to worsening of the foveal schisis. But the retina was stable with respect to the peripheral schisis. Posterior vitreous detachment did not occur following the laser.


  Discussion Top


Congenital retinoschisis is predominantly transmitted as an X-linked recessive trait and occurs almost exclusively in males. Since the disease frequently affects the macula, or causes vitreous haemorrhage, the presenting features are poor vision, strabismus and nystagmus. The diagnosis is usually made in childhood.

Bilateral inferotemporal retinoschisis is the classic presentation. Foveal schisis is present in nearly 100% of cases and may be the only presenting feature in half the instances. Blood-filled retinal cysts as seen in our patient have been described[5] and are known to clear spontaneously. Unsupported retinal vessels can give rise to recurrent vitreous haemorrhage. Retinoschisis-related retinal detachment occurs in 11- 16% of patients. [1,6] Congenital retinoschisis is typically stable and treatment is indicated only when there is a complication which threatens or impairs vision.

The appropriate management of progressive macula-threatening retinoschisis has been debated. Parsplana vitrectomy with inner wall excision and photocoagulation to the outer wall has been recommended by some authors. [3,4] Prophylactic treatment with laser photocoagulation has had varied success. Brockhurst et al[2] reported that laser photocoagulation in 5 eyes with congenital retinoschisis not only failed to ameliorate the disease, but also caused holes in the external, or both layers, thus leading to retinal detachment. Kellner et al[1] reported that prophylactic photocoagulation was of no use since it was complicated by retinal detachment in 43% of their series. Ambler et al[7] reported success in one of three cases of posteriorly situated retinoschisis-retinal detachment following treatment with laser photocoagulation.

The occurrence of retinal detachment due to holes in the outer layer seems to be a potentially high risk factor and could result from a combination of relatively heavy burns, thin outer layer and retinal traction caused by the taut inner layer. The present case suggests that light burns of laser photocoagulation to the schisis area, in absence of significant traction by the inner layer, is probably safe and may not induce iatrogenic retinal breaks. The mechanism for the successful collapse of schisis cavity is not clear. Obviously, the burns at the outer later of the schisis facilitated the absorption of the intracystic fluid. Although the schisis cavity collapsed in the present case, the value of the treatment in stopping the progression of the schisis remains questionable. Possibly, the risk of rhegmatogenous retinal detachment is reduced.

Barrage laser photocoagulation beyond the limits of schisis does not seem to have much scientific basis. Pathologically, there is nothing to suggest that spread of retinoschisis is only by continuity as evidenced by the occurrence of foveal schisis away from peripheral lesion. The plane of split is in the nerve fibre layer. Light photocoagulation in the normal retina beyond is likely to cause adhesion between outer retinal layers and retinal pigment epithelium and does not involve the nerve fibre layer. Hence, it is difficult to believe that this burn can stop the split in the nerve fibre layer from progression.

In conclusion, this case indicates that laser may be effective in collapsing a retinoschisis cavity. However, we advise caution and add that in view of past reports, the benefit of treatment should be weighed against the potential risk of precipitating new complications. Evaluation of the amount of traction on the outer retinal layer may be crucial in case selection, and light laser burns may also reduce the risk of retinal break formation.

 
  References Top

1.
Kellner U, Brummer S, Foerster MH, Wessing A. X-linked congenital retinoschisis. Graefes Arch Clin Exp Ophthalmol 1990;228:432-37.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.
Brockhurst RJ. Photocoagulation in congenital retinoschisis. Arch Ophthalmol 1970;84:158-65.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.
Rosenfeld PJ, Flynn HW Jr, McDonald HR,Rubsamen PE, Smiddy WE, Sipperley JO, et al. Outcomes of vitreoretinal surgery in patients with X-linked retinoschisis. Ophthalmic Surg Lasers 1998;29:190-97.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.
Ferrone PJ, Trese MT, Lewis H. Vitreoretinal surgery for complications of congenital retinoschisis. Am J Ophthalmol 1997;123:742-47.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.
Conway BP, Welch RB. X-chromosome-linked juvenile retinoschisis with hemorrhagic retinal cyst. Am J Ophthalmol 1977;83:853-55.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.
George ND, Yates JR, Moore AT. Clinical features in affected males with X-linked retinoschisis. Arch Ophthalmol 1996;114:274-80.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.
Ambler JS, Gass JD, Gutman FA. Symptomatic retinoschisis-detachment involving the macula. Am J Ophthalmol 1991;112:8-14.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure - 1], [Figure - 2]



 

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