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Year : 2021  |  Volume : 1  |  Issue : 1  |  Page : 55-57

Uveal effusion syndrome in achondroplasia

1 Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, India
2 Department of Uvea, Sankara Nethralaya, Chennai, India
3 Ocular Pathology, Sankara Nethralaya, Chennai, India

Date of Submission05-May-2020
Date of Acceptance24-Jul-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Suganeswari Ganesan
No. 41, Old 18, College Rd, Thousand Lights West, Nungambakkam, Chennai - 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1320_20

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Keywords: Achondroplasia, congenital disorder, craniofacial deformity, exudative retinal detachment, sclerectomy, sclerotomy, uveal effusion syndrome

How to cite this article:
Ganesan S, Singh P, Ganesh S, Subramanian K, Bhende M. Uveal effusion syndrome in achondroplasia. Indian J Ophthalmol Case Rep 2021;1:55-7

How to cite this URL:
Ganesan S, Singh P, Ganesh S, Subramanian K, Bhende M. Uveal effusion syndrome in achondroplasia. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Feb 26];1:55-7. Available from: https://www.ijoreports.in/text.asp?2021/1/1/55/305483

Thirty two-year-old male presented with complaints of diminution of vision in the left eye from 2 months with no significant past ocular history. He was diagnosed case of achondroplasia [Figure 1].
Figure 1: Clinical appearance of 32-year-old male having short stature and typical features of achondroplasia

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The right eye was normal with best corrected visual acuity (BCVA) of 6/6. The left eye had bullous inferior retinal detachment with no retinal or pars plana breaks [Figure 2] having BCVA of 3/60. Fundus fluorescein angiography and Indocyanine angiography showed no leakage of the dye [Figure 3]c and [Figure 3]d.
Figure 2: (a) Color fundus photograph of the left eye showing inferior exudative retinal detachment (b) OCT scan of the left eye showing neurosensory detachment with increased thickness of sclera (c) Color fundus photograph of the left eye showing resolution of exudative retinal detachment after sclerectomy with sclerotomy. (d) OCT scan of the left eye after surgery showing attached retina

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Figure 3: (a) Ultrasound B scan showing retinal detachment and thickened sclera (b) Ultrasound biomicroscopy showing supraciliary effusion (c) Fundus fluorescein angiography images in late phase showing no leak. (d) Indocyanine green angiography images in late phase no leak

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The biometric parameters measured the axial length of 22.10 mm and 21.52 mm, the peripapillary choroidal thickness of 2.0 mm and 2.1 mm, the peripapillary scleral thickness of 1.2 mm and 1.2 mm [Figure 3]a, anterior chamber depth of 2.89 mm and 2.87 mm, lens thickness of 4.20 mm and 4.78 mm in the right and left eye, respectively. The left eye showed 360° supraciliary effusion [Figure 3]b.

The patient was treated with systemic steroids, showing nil improvement. Subretinal fluid (SRF) drainage was done using the Steve Charles technique.[1] However exudative retinal detachment recurred. BCVA dropped to finger counting.

A sclerectomy with sclerotomy was performed in inferonasal and inferotemporal quadrants [Figure 4][2] Instead of all 4 quadrants, sclerectomy with sclerotomy was performed in only inferior two quadrants given thicker sclera relative to superior quadrants.[3] Histopathology of the scleral specimen revealed abnormally thickened sclera with interlacing and interwoven irregular arrangement of collagen bundles at various angles [Figure 5]b and [Figure 5]d. Sections of normal sclera shown for comparison [Figure 5]a and [Figure 5]c. SRF decreased postoperatively with complete resolution over 6 weeks and BCVA of 3/60. The patient maintained vision with no recurrence in subsequent follow-ups for 1 year.
Figure 4 (a-f): Surgical photos showing sclerectomy with sclerotomy being performed in inferonasal and inferotemporal quadrants 4 mm away from the limbus and 2 mm anterior to muscle insertion. 4 × 4 mm sclerectomy and triangular sclerotomy of 2 × 2 mm was made

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Figure 5: (a) Scleral tissue from enucleated eyeball due to intraocular tumor showing normal scleral tissue. Magnification 4X – hematoxylin and eosin stain (H and E stain). (b) Scleral tissue from achondroplasia. Magnification 4X – H and E stain. (c) Normal scleral tissue showing the regular arrangement of collagen fibers. Magnification 40X – H and E stain. (d) Scleral tissue from achondroplasia. Loss of normal arrangement of collagen fibers and gaps with the homogenisation of scleral tissue. There is no inflammation or increased vascularity magnification 40X – H and E stain

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  Discussion Top

Uveal effusion syndrome is described in nanophthalmos, eyes with the thickened sclera, and abnormal scleral tissue.[4] ultrasonography (USG) and optical coherence tomography (OCT) are noninvasive diagnostic modalities for evaluating scleral thickness.[3],[5]

The concurrence of the thickened abnormal sclera in achondroplasia could be due to collagen abnormality raising suspicion of the genetic basis of this rare disorder; however, a chance occurrence cannot be excluded.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Charles S. Controlled drainage of subretinal and choroidal fluid. Retina 1985;5:233-4.  Back to cited text no. 1
Fox SA. Simple posterior sclerotomy and sclerectomy: An adjunct to operations for glaucoma. Arch Ophthalmol 1942;28:802-13.  Back to cited text no. 2
Buckhurst HD, Gilmartin B, Cubbidge RP, Logan NS. Measurement of scleral thickness in humans using anterior segment optical coherent tomography. PLoS One 2015;10:e0132902.  Back to cited text no. 3
Elagouz M, Stanescu-Segall D, Jackson TL. Uveal effusion syndrome. Surv Ophthalmol 2010;55:134-45.  Back to cited text no. 4
Fledelius HC, Fuchs HJ, Jensen PK, Scherfig E. Uveal effusion and ultrasonic imaging: A clinical series. Acta Ophthalmol Scand 2002;80:202-10.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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