|Year : 2018 | Volume
| Issue : 3 | Page : 161-162
Retinal detachment after cyclophotocoagulation in a child with knobloch syndrome
Ali S Al-Beshri1, Igor Kozak2, Earl Randy Craven3
1 Glaucoma Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
2 Moorfields Eye Hospital Centre, Abu Dhabi, United Arab Emirates
3 Glaucoma Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, USA
|Date of Web Publication||4-Jan-2019|
Dr. Igor Kozak
Moorfields Eye Hospital Centre, Abu Dhabi
United Arab Emirates
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Knobloch syndrome (KS) is typically characterized by high myopia, vitreoretinal degeneration, retinal detachment, and macular abnormalities. We report a case of glaucoma in KS, which represents the fourth reported case and the first description of the retinal events after the glaucoma procedure. Retinal detachment followed standard cyclophotocoagulation procedure for glaucoma in a 2-month-old boy. Ophthalmologists should be aware of the possibility of retinal detachment from any ocular intervention in patients with KS.
Keywords: Cyclophotocoagulation, glaucoma, Knobloch syndrome, retinal detachment
|How to cite this article:|
Al-Beshri AS, Kozak I, Craven ER. Retinal detachment after cyclophotocoagulation in a child with knobloch syndrome. Middle East Afr J Ophthalmol 2018;25:161-2
|How to cite this URL:|
Al-Beshri AS, Kozak I, Craven ER. Retinal detachment after cyclophotocoagulation in a child with knobloch syndrome. Middle East Afr J Ophthalmol [serial online] 2018 [cited 2019 Apr 26];25:161-2. Available from: http://www.meajo.org/text.asp?2018/25/3/161/249324
| Introduction|| |
Knobloch syndrome (KS) is typically characterized by high myopia, vitreoretinal degeneration, retinal detachment, and macular abnormalities.,, Hull et al. recently reported new features in KS patients: pigment dispersion syndrome, cone-rod dysfunction seen with electroretinography, and glaucoma (two patients). Glaucoma is not a typical feature of KS and no description as to what is seen after interventions has been reported. We report a case of glaucoma in KS, which represents the fourth reported case and the first description of the retinal events after the glaucoma procedure.
| Case Report|| |
A 2-month-old male was referred to King Khaled Eye Specialist Hospital (KKESH) in Riyadh, Saudi Arabia, with diagnosis of proliferative vitreoretinopathy (PVR), light perception, and retinal detachment in the left eye. Ocular history was positive for epiphora and buphthalmos. He was the product of a first-degree consanguineous marriage and of a normal full-term pregnancy. There was severe head lag, occipital bone defect, and generalized hypotonia. Axial length was 32 mm in the right eye and 20 mm in the left eye. The corneal diameter was 13.5 mm and 10.5 mm in the right and left eyes, respectively. Lenses were clear with a persistent retrolental membrane in the left eye. The posterior segment in the right eye showed a myopic fundus with no breaks, chorioretinal atrophy, and a tilted disc with no visible cup [Figure 1]a. In the left eye, the retina was detached with PVR involving the macula. The management plan at age 2 months was retinal surgery with endotamponade, which was cancelled because of bronchiolitis and rescheduled 2 weeks later. At that time, examination under anesthesia found the left eye inoperable [Figure 1]b. Retinoscopy in the right eye revealed – 20D refraction, and retina was as described before. The right eye had intraocular pressure (IOP) of 26 mmHg for which he was discharged on dorzolamide hydrochloride–timolol maleate ophthalmic fixed combination solution.
|Figure 1: RetCam image of the right eye before cyclophotocoagulation procedure showing flat retina, attenuated retinal vessels, and tessellated fundus (a). RetCam image of the left eye showing detached retina with proliferative vitreoretinopathy (b)|
Click here to view
He was again referred at 21 months of age. Examination under anesthesia revealed an IOP of 31 mmHg and corneal thickness of 480 microns. The seeing eye was at high risk of RD even without surgical intervention including goniotomy or trabeculotomy. These kinds of eyes in the literature have been reported to have many complications with any incisional surgeries. The assumption was that doing only gentle laser may be the least invasive and benign, and cyclophotocoagulation was recommended for the right eye. One hundred and ninety degrees of treatment was performed inferiorly with 1300–1500 MW power and 2000-ms duration after transillumination to identify the ciliary body. On the 1st postoperative day, the eye had a formed anterior chamber, and IOP was digitally in the midteens. The family presented before the next scheduled postoperative visit; ocular examination revealed a soft eye with +2 conjunctival injection. B-scan revealed a funnel-shaped retinal detachment and choroidal detachment [Figure 2]. Visual evoked potential showed flat response.
|Figure 2: Ultrasound B-scan image of the right eye showing funnel-shaped total retinal detachment (white arrow) in axial (a) and longitudinal (b) sections|
Click here to view
| Discussion|| |
Knobloch and Layer reported a nonrelated family in which 5 of 10 siblings with high myopia were found to have vitreoretinal degeneration, retinal detachment, and occipital encephalocele. Apart from typical features in KS, there may be some variability, especially in central nervous system findings. Khaliq et al. reported a consanguineous Pakistani family in which multiple individuals had high myopia, vitreoretinal degeneration, and occipital scalp defect. Other findings included choroidal sclerosis, atrophy, pigmentary abnormalities, subluxation of the lens, cataract, one glaucomatous eye, phthisis bulbi, and nystagmus. Increased IOP is not a part of typical KS, and only three cases of glaucoma have been described in patients with KS up to date.,
Herein, we report a monocular KS infant with glaucoma (fourth reported case) and poor vision. The blinding retinal detachment following a limited cyclophotocoagulation is the first report of any glaucoma treatment results in KS. Retinal detachment is a very rare complication after cyclophotocoagulation in glaucoma patients. Given the history of the fellow eye in this case, it is possible that retinal detachment might have happened coincidentally following a natural history of the disease. The mechanism of retinal detachment following cyclophotocoagulation is not well understood. We presume that cyclophotocoagulation by shrinking the ciliary body retracts the already fragile vitreous base causing a retinal break and detaching the retina which is usually weak and thin in such eyes.
Ophthalmologists should be aware of possibility of retinal detachment from any ocular intervention in patients with KS.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Knobloch WH, Layer JM. Retinal detachment and encephalocele. J Pediatr Ophthalmol 1971;8:181-4.
Sertié AL, Sossi V, Camargo AA, Zatz M, Brahe C, Passos-Bueno MR, et al.
Collagen XVIII, containing an endogenous inhibitor of angiogenesis and tumor growth, plays a critical role in the maintenance of retinal structure and in neural tube closure (Knobloch syndrome). Hum Mol Genet 2000;9:2051-8.
Khan AO, Aldahmesh MA, Mohamed JY, Al-Mesfer S, Alkuraya FS. The distinct ophthalmic phenotype of Knobloch syndrome in children. Br J Ophthalmol 2012;96:890-5.
Hull S, Arno G, Ku CA, Ge Z, Waseem N, Chandra A, et al.
Molecular and clinical findings in patients with Knobloch syndrome. JAMA Ophthalmol 2016;134:753-62.
Khaliq S, Abid A, White DR, Johnson CA, Ismail M, Khan A, et al.
Mapping of a novel type III variant of Knobloch syndrome (KNO3) to chromosome 17q11.2. Am J Med Genet A 2007;143A: 2768-74.
Ishida K. Update on results and complications of cyclophotocoagulation. Curr Opin Ophthalmol 2013;24:102-10.
[Figure 1], [Figure 2]