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| PHOTO ESSAY |
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| Year : 2023 | Volume
: 3
| Issue : 2 | Page : 601-602 |
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Kranenburg syndrome
Shrinkhal
Department of Ophthalmology, AIIMS, Raebareli, Uttar Pradesh, India
| Date of Submission | 01-Nov-2022 |
| Date of Acceptance | 10-Feb-2023 |
| Date of Web Publication | 28-Apr-2023 |
Correspondence Address:
Shrinkhal
Department of Ophthalmology, AIIMS, Raebareli, Uttar Pradesh
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/IJO.IJO_2897_22
Keywords: Kranenburg syndrome, optic disc pit, optic disc pit with maculopathy
How to cite this article:
Shrinkhal. Kranenburg syndrome. Indian J Ophthalmol Case Rep 2023;3:601-2 |
A 46 years old female presented with a chief complaint of decreased vision in the right eye for the last 6 years and in the left eye for the last 20 years. On examination, her distant visual acuity (VA) was 6/9, and hand movement close to face in her right and left eyes [Figure 1]a and optical coherence tomography (OCT) [Figure 1]b and [Figure 1]c picture, respectively. She had undergone laser photocoagulation twice in the left eye for the pathology but did not show any visual improvement. Fundus examination of the left eye revealed a temporal optic disc pit (ODP) with associated large maculopathy (ODP-M) (subretinal and intraretinal fluid accumulation) and temporal retinal detachment. The right eye was normal. Diagnosis of left eye Kranenburg syndrome was made. The patient was prescribed refractive correction for the right eye and was explained about different treatment options for the left eye and the poor visual prognosis. The patient denied any intervention for the left eye. | Figure 1: (a) Red free fundus photograph of the left eye with ODP-M. A temporal ODP is noted (red arrow) with a large area of retinal detachment (green demarcation line). (b) A horizontal OCT scan through the optic disc pit (red arrow) and fovea with en-face analysis, showing an intraretinal and subretinal fluid, extending towards the ODP. (c) Optic disc cube analysis with 3D visualization showing optic pit with elevated neurosensory retina (red arrow) from the retinal pigment epithelium
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| Discussion | |  |
ODP is an uncommon developmental anomaly of the disc. It occurs equally in men and women with an overall estimated incidence of 1 in 11,000 people.[1],[2] It is characterized by the presence of a defect in the disc, and is considered an entity of the spectrum of several cavitary anomalies of the optic disc. It is usually sporadic and unilateral in incidence. However, it can be bilateral in around 15% of the cases.[3] It may be associated with intraretinal or subretinal accumulation of the fluid at the macula, known as Kranenburg syndrome, and subsequently, lead to decreased VA. These are usually single, oval-shaped grayish depressions in the optic disc, and are most commonly found at the inferotemporal region of the optic disc, but may also be found elsewhere. ODP as for itself is an asymptomatic entity and is usually found incidentally. However, association with maculopathy leads to significant visual loss. There is no known and established cause of maculopathy along with ODP, however as it is usually seen in the third to fourth decade of life; so is postulated to develop posterior vitreous detachment (PVD).[4] Although spontaneous resolution with improvement in vision has been reported in some cases, however, the majority of cases have a poor prognosis, with a natural history of gradual worsening and a final VA of 20/200 or worse.[5] Various treatment options include the use of corticosteroids, laser photocoagulation and the temporal disc margin, intravitreal gas injection, macular buckling surgery, and a combination of these.
In our patient, taking into consideration the long duration of the entity (20 years), very poor VA in the affected eye (hand movement close to face), and two failed attempts of laser photocoagulation at the temporal disc margin, poor visual prognosis in the left eye was explained to the patient.
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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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| References | | |
| 1. | Kranenburg EW. Crater-like holes in the optic disc and central serous retinopathy. Arch Ophthalmol 1960;64:912-24.  |
| 2. | Georgalas I, Ladas I, Georgopoulos G, Petrou P. Optic disc pit: A review. Graefes Arch Clin Exp Ophthalmol 2011;249:1113-22. |
| 3. | Brodsky MC. Congenital optic disk anomalies. Surv Ophthalmol 1994;39:89-112. |
| 4. | Brockhurst RJ. Optic pits and posterior retinal detachment. Trans Am Ophthalmol Soc 1975;73:264-91. |
| 5. | Apple DJ, Rabb MF, Walsh PM. Congenital anomalies of the optic disc. Surv Ophthalmol 1982;27:3-41. |
[Figure 1]
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