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Ocular Findings in Kenny's Syndrome James R

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Ocular Findings in Kenny's Syndrome James R Ocular Findings in Kenny's Syndrome James R. Boynton, MD; Thomas R. Pheasant, MD; Bruce L. Johnson, MD; Daniel B. Levin, MD; Barbara W. Streeten, MD \s=b\In 1966, Kenny described two pa- 1966, Kenny and Linarelli de- and marginally low potassium levels also tients with an unusual congenital syn- scribed an unusual congenital syn¬ were noted. Hearing and dentition were drome including dwarfism, thickened long drome.' A mother and son exhibited abnormal. Intelligence and test results of bone cortex, transient hypocalcemia, and the following characteristics: low pituitary function were normal. At age 10, normal intelligence. These and other birth clo¬ height and bone age were both retarded. weight, dwarfism, delayed Skull showed bilateral were de- roentgenograms patients previously incorrectly sure of the anterior fontanel, uniquely lobe calcifications. films of scribed as Ocular temporal X-ray "myopic." findings in thickened long bone cortex with the long bones disclosed internal cortical four subjects ranged from uncomplicated stenotic medullary cavities,2 transient thickening and stenotic medullary cavi¬ nanophthalmos with hyperopia to extreme hypocalcemia with hyperphosphate- ties. pseudopapilledema, vascular tortuosity, mia leading to tetany, and normal Physically, the patient appeared minia¬ and macular crowding. Postmortem find- mentation. To our knowledge, only ture with a prominent forehead, microph- ings from one patient showed calcium two additional reports of this syn¬ thalmos, and micrognathia (Fig 1). Height deposits demonstrable only by special drome have since this was 102 cm. The sternum was slightly appeared'·4 orig¬ bowed histochemical stains that were distributed inal The ex¬ anteriorly. description. ophthalmic At age 3 she was seen because of a large uniquely in the cornea. This distribution amination in all cases has been incom¬ right esotropia. The left eye was patched differed from the pattern seen in The incidence of greatly plete. reported "my¬ for several months, but there was no band keratopathy. Retinal calcification opia" is erroneous, and actually refers change in fixation preference. A bi medial was also an unusual feature. Because one to hypermetropia. This article de¬ recession was performed with only partial patient exhibited a pseudodoubling of the scribes the ocular findings in four cosmetic improvement, and the following optic papilla, the literature was reviewed. patients with Kenny's syndrome, in¬ year a recess-resect procedure was done on We conclude that no convincing case of cluding histologie examination results the right eye. During surgery, the choroid was rectus true doubling of the optic nerve has been from one case. exposed beneath the lateral described. Ophthalmologists should be insertion when this muscle was severed from the alert for undiagnosed electrolyte abnor- REPORT OF CASES* globe. Postoperatively, the media in the right eye were cloudy and a vitreous malities, in pa- Case 1.—The was born of normal especially hypocalcemia, patient hemorrhage was diagnosed. Two months tients with parents and exhibited slow after an Kenny's syndrome. growth later both fundi were visualized. The right uneventful She was first admit¬ (Arch Ophthalmol 97:896-900, 1979) delivery. disc was blurred and the macular area of ted to the at age 9 months with hospital each eye was described as gray. At age 5, fever, dwarfism, and retarded bone tetany, vision was recorded as counting fingers at Accepted for publication Nov 13, 1978. age. A of diagnosis hypoparathyroidism 122 cm in the right eye and 20/200 in the From the Department of Ophthalmology, was made and vitamin D insti¬ therapy left eye. The retina of the eye was Warsaw Medical Group, PC, Warsaw, NY (Drs tuted. admissions because of fever right Boynton and Levin); Department of Ophthalmol- Multiple elevated and gray inferotemporally. and convulsions followed, accom¬ ogy, University of Rochester, Rochester, NY (Dr usually The patient was not seen again until she upper disease. Boynton); Division of Ophthalmology, Retina panied by respiratory Hypo¬ was 9. Vision in the right eye was hand S. calcemia and were Service, Milton Hershey Medical Center, hyperphosphatemia motion due to a dense cataract. An early Hershey, Pa (Dr Pheasant); Department of documented electro¬ repeatedly. Sporadic band keratopathy was noted. Shortly of Pittsburgh School of abnormalities such as Pathology, University lyte hypernatremia an acute in Medicine (Dr Johnson); and State University of thereafter, glaucoma developed New York, Upstate Medical Center, Syracuse (Dr the right eye with a flat anterior chamber. Streeten). *All four of the patients reported here were A posterior sclerotomy and lens aspiration Reprint requests to Warsaw Medical Group, seen by Frederic M. Kenny, MD, who confirmed were performed. PC, Warsaw, NY 14569 (Dr Boynton). the diagnosis. We first examined the patient at age 12. Downloaded From: http://archopht.jamanetwork.com/ by a University of Pittsburgh User on 05/13/2015 Her best corrected vision was 20/800 in the dilated tortuous arterial and venous sys¬ patient (Fig 5). He was 135 cm tall. Best right eye and 20/200 in the left eye. The tems. Perivascular hyperfluorescence corrected vision was 20/30 in each eye with refractive error in the left eye was + 24.00 proved to be tortuous vessels viewed end on a +9.25 sphere in the right eye and a +9.75 diopters. The fundus of the right eye was and out of focus. As the study progressed sphere in the left eye. Corneas measured 9 obscured by a membrane secondary to the there was no increase in perivascular fluo¬ mm in diameter. Optic discs appeared lens extraction. Corneas measured 8 mm in rescence to imply leakage or loss of compe¬ slightly elevated with blurred margins and diameter and exhibited band keratopathy, tence of the retinal vasculature. The retina tortuous retinal vessels (Fig 6). Findings greater on the right. The left fundus was appeared crowded, as if confined to a from the remainder of the ocular examina¬ filled with multiple, dilated, tortuous smaller than normal space. The fovea was tion were normal. vessels. The entire retina appeared swollen surrounded by a capillary free zone smaller Case 3.-The 50-year-old mother of and abnormal, especially in the region than expected, an estimated 250 µ rather patient 2 was also examined. She had a about the optic nerve. The vascular pattern than 500 µ. There was no late hyperfluores¬ history of mild diabetes and was under 117 combined with these highlights presented cence or dye leakage. cm in height. Vision was 20/25 with a the deceptive picture of an accessory optic B-scan ultrasonography showed a single + 5.75, +1.75 x 130° in the right eye and nerve head (Fig 2). nerve pattern and a globe 13 mm in length 20/40 with a +6.50, +1.25 x 60° in the left Photographs, however, disclosed a single (Fig 4). The nerve pattern appeared slight¬ eye. Corneas measured 10 mm in diameter. elevated optic nerve head with tortuous, ly enlarged relative to the size of the globe. The fundi were unremarkable. redundant retinal circulation simulating Roentgenograms of the optic foramina Case 4.—The patient enjoyed good health an accessory optic disc above. The retinal were unremarkable. until age 5, at which time he experienced pigment epithelium was prominent in the Case 2.—The mother and son originally an episode of tetany that was treated posterior pole with pigment clumping in described by Kenny in 1966 recently were successfully with orally administered cal¬ the parafoveal region. Fluorescein angiog¬ examined. Their systemic findings are cium. Subsequently, he was begun on vi¬ raphy confirmed a single retinal circula¬ detailed in that report. The 13-year-old boy tamin D therapy. At age 8, growth re¬ tion (Fig 3). Dye transit demonstrated had a facial appearance similar to the first tardation was apparent. Extensive clinical Fig 1.—Facial appearance of a 13-year-old Fig 2.—Left eye: retinal vessels are Fig 3.—Left eye: fluorescein angiogram, girl, characteristic of Kenny's syndrome tortuous and dilated; disc is blurred and arterial phase. Single tortuous vascular (case 1) elevated. Confluence of vessels combined system is present. There was no leakage with unusual retinal highlights simulates of fluorescein in later photographs (case "accessory disc" superotemporal to nerve 1)- head. Macular area is deeply pigmented and poorly defined (case 1). Fig 4.—Left eye: B-scan ultrasonogram Fig 5.—Characteristic facial appearance of Fig 6.—Left eye: disc is slightly elevated demonstrates microphthalmos, globe a 13-year-old boy with Kenny's syndrome and vasculature moderately tortuous measuring 13 mm anteroposteriorly. Optic (case 2). (case 2). nerve pattern (large arrow) is slightly larg¬ er than expected for eye this size. Distance between range lines (small arrows) is 10 mm (case 1). Downloaded From: http://archopht.jamanetwork.com/ by a University of Pittsburgh User on 05/13/2015 Fig 7.—Basal ganglia contains numerous irregular calcium depos¬ Fig 8.—Optic disc is elevated, lacks physiologic cup, but is not its and mineralized thick-walled vessel (hematoxylin-eosin, origi¬ edematous. Scierai aperture is slightly narrow (hematoxylin- nal magnification 60). eosin, original magnification 35). Fig 9.—Low-power view of central cornea. Superficial stroma is uninvolved while deeper stroma, which is darker staining, contains abundant calcium (Von Kossa, original magnification X35). Fig 10—Termination of corneal calcification zone. Black-staining keratocytic nuclei are heavily calcified and abundant fine gran¬ ules of calcium are present in collagen lamellae. In contrast, uninvolved upper left portion of field contains pale-staining keratocytic nuclei and no calcium deposits in collagen (Von Kossa, original magnification 350). Fig 11.—Periphery of zone of corneal calcification shows abrupt, curved termination. In this area, mineralization involves superfi¬ cial stroma but Bowman's layer is spared throughout its length (Von Kossa, original magnification 60). Fig 12.—Peripheral retina shows heavy calcium impregnation (black) of cytoplasm of Müller cells beneath internal limiting membrane (small arrows).
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