CASE REPORTS AND SMALL CASE SERIES

UnoprostoneLatanoprost Unoprostone Increase of Intraocular 60 Pressure After Topical Cyclophotocoagulation Administration of 50 Prostaglandin Analogs 40 Several prostaglandins have been

demonstrated to reduce intraocular 30 pressure (IOP) in normal, hyperten- sive, and glaucomatous eyes.1-3 Two mm Hg IOP, OD 20 different prostaglandin analogs are commercially available: unopros- 10 tone (Rescula; Ciba Vision Ophthal- OS mics, Duluth, Ga) and latanoprost (Xalatan; Pharmacia Inc, Colum- 0 October 5, 1996 October 10, 1996 October 2, 1997 October 7, 1997 bus, Ohio). We observed an inverse Time reaction after topical administration Time course of intraocular pressure (IOP) for both eyes. Arrows indicate application of prostaglandin of both analogs. derivates or cyclophotocoagulation only of the left eye. Report of a Case. A 29-year-old wom- an had pigmentosa with of treatment with unoprostone, the and visual acuity increased to 6/20 typical ophthalmoscopic findings, a IOP returned to 15 mm Hg. During (Figure). ring , and a flat electro- the following weeks the IOP again There were no signs of acute retinogram. Juvenile was ranged between 1 and 35 mm Hg. anterior segment inflammation af- diagnosed at the age of 12 years. Be- Five months after this trial with uno- ter the prostaglandin applications. A cause of the characteristic malforma- prostone, another prostaglandin ana- marked atrophy of the tion of the anterior segment it was log, latanoprost, became available. At was observed with high-resolution classifiedasRiegersyndrome.Theini- this time, the IOP again was about 30 ultrasound biomicroscopy. tial IOP at the time of glaucoma de- mm Hg despite maximum tolerated tection was 50 mm Hg. Both eyes un- medical therapy without prostaglan- Comment. In the literature, we could derwent Elliot operation. The left eye din analogs. As with unoprostone, the not find any other reports of seri- required an additional cryocoagula- IOP immediately increased to 55 ous, reproducible IOP increase after tion of the ciliary body. After these mm Hg after 2 drops of latanoprost. unoprostone or latanoprost admin- operations, the IOP of the right eye This increase of IOP was again ac- istration. These prostaglandin ana- was between 8 and 14 mm Hg with- companied by corneal edema and a logs are known to be safe and effec- out further medication. The IOP of decrease in visual acuity. With intra- tive in reducing IOP.1-3 It is presumed the left eye was below 21 mm Hg un- venous 20% mannitol, the IOP rap- that they facilitate the uveoscleral out- til the patient was 26 years old. The idly dropped to 20 mm Hg and later flow, whereas trabecular outflow may IOP then began to increase, and a sec- returned to 30 mm Hg. be slightly impaired.4 One might ond cryocoagulation was performed. We now decided to perform a speculate that, in our patient, uveo- After the second cryocoagulation, the stepwise diode laser cyclophotoco- scleral outflow was considerably al- IOP varied between 0 mm Hg (with- agulation. After 4 treatments with 2 terated by the disease itself (Rieger out therapy) and 41 mm Hg OS (with burns each, the IOP ranged be- syndrome and ) maximum tolerated medical therapy tween 10 mm Hg and 20 mm Hg OS. or by the cryoprocedures. The atro- without prostaglandin analogs). At However, 5 months after the last phy of the ciliary body supports this this time visual acuity was 6/30 OD laser treatment, IOP decreased to 0 theory. Because of these alterations, and 6/12 OS. mm Hg and remained at this hypo- prostaglandins perhaps could not fur- After a 9-week period of IOP tonous level for 3 weeks. Treatment ther improve uveoscleral outflow. values between 30 and 34 mm Hg OS, with systemic and local steroids failed Thus, the slight impairment of tra- we decided to try an additional treat- to increase IOP, and visual acuity was becular outflow could have caused ment of 2 drops of unoprostone, 1 in only 6/120. This was the reason why the IOP increase. the morning and 1 in the evening. In we now tried to elevate IOP using less than 24 hours, the IOP in- prostaglandin analogs. In fact, after Thomas Ness, MD creased to 56 mm Hg, accompanied 2 drops of unoprostone, IOP in- Jens Funk, PhD, MD by corneal edema. After withdrawal creased to 55 mm Hg within 36 hours Freiburg, Germany

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1646

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 Corresponding author: Thomas Ness, Treatment with topical 0.5% levobu- OD and 38 cm/s OS and central reti- MD, Universita¨ts-Augenklinik nolol and 0.2% bromonidine and nal artery blood flow velocities of Freiburg, Killianstr. 5, D-79106 oral neptazane was initiated. The in- 15.26 cm/s OD and 11.89 cm/s OS. Freiburg, Germany. traocular pressure stabilized in the The intraocular pressure in the left low 20s and visual acuity remained eye at the time of the postoperative 1. Alm A, Stjernschantz J. Effects on intraocular pressure and side effects of 0.005% latanoprost counting fingers OS at 0.3 m due to color Doppler study was un- applied once daily, evening or morning. Oph- a central corneal scar. changed at 22 mm Hg. The blood thalmology. 1995;102:1743-1752. Approximately 3 months after pressure and heart rate were ap- 2. Fujimori C, Yamabayashi S, Hosoda M, et al. The clinical evaluation of UF-021, a new prostaglan- the repair, the pa- proximately the same. din-related compound, in low-tension glau- tient complained of a several-day his- coma. Nippon Ganka Gakkai Zasshi. 1993;97: 1231-1235. tory of episodic transient visual loss Comment. To our knowledge, this 3. Ziai N, Dolan JW, Kacere RD, Brubaker RF. The (to the level of bare light percep- is the first reported case of a patient effects on aqueous dynamics of PhXA41, a new tion) in her left eye that occurred who experienced episodes of tran- prostaglandin F2 alpha analogue, after topical application in normal and ocular hypertensive when she stood from a seated or su- sient visual loss associated with human eyes. Arch Ophthalmol. 1993;111:1351- pine position. She had recently re- documented orbital hemodynamic 1358. 4. Gabelt BT, Kaufman PL. The effect of prosta- sumed normal daily physical activi- changes after scleral buckling with glandin F2 alpha on trabecular outflow facility ties after being restricted in the an encircling element. Presumably, in cynomolgus monkeys. Exp Eye Res. 1990;51: postoperative period. The episodes central retinal artery blood flow, and 87-91. were reproducible and she experi- possibly ophthalmic artery blood enced up to 10 of these episodes flow, was significantly reduced as a daily, with each episode lasting 2 to result of the encircling procedure 3 minutes. and orthostatic decreases in blood Transient Visual Loss and Examination at that time re- pressure caused a further decrease Decreased Ocular Blood vealed visual acuities of 20/20 OD in ocular perfusion. The episodes of Flow Velocities Following a and finger counting at 0.3 m OS. transient visual loss resulted from Scleral Buckling Procedure Goldmann applanation tonometry the decreased ocular perfusion. Re- revealed an intraocular pressure of moval of the encircling band re- Scleral buckling procedures with en- 23 mm Hg OS that was confirmed sulted in a resolution of the tran- circling elements have been shown by the Tonopen tonometer. The an- sient visual loss episodes and a to decrease blood flow velocities in terior segment examination re- corresponding normalization of the the central retinal artery but, in most vealed a central corneal scar with a central retinal artery and other ret- cases, leave the ophthalmic artery deep and quiet anterior chamber. robulbar artery hemodynamic pa- unaffected.1 Although these hemo- The was completely attached rameters as measured with color dynamic changes are well docu- with a normal-appearing posterior Doppler imaging. mented with otherwise successful pole. A moderately high 360° scleral Decreased retinal artery blood scleral buckling procedures, they are buckle indentation effect was evi- flow rate and velocity is a recog- rarely symptomatic. We report the dent peripherally. Her blood pres- nized effect of scleral buckling pro- case of a young woman who devel- sure was 110/70 mm Hg in the right cedures with encircling elements. On oped episodes of posturally related and left arms without orthostatic average, a 50% decrease in blood transient visual loss following a changes. flow velocities with an accompa- scleral buckling procedure with an Color Doppler imaging was nied increase in resistance has been encircling element. performed on the right and left eyes demonstrated in the major tempo- and revealed ophthalmic artery ral arteries and in the central reti- Report of a Case. A 26-year-old systolic blood flow velocities of 45 nal artery following scleral buck- woman had undergone surgical re- cm/s OD and 10 cm/s OS (normal ling and encircling procedures, as pair of a 12-mm full-thickness cor- mean ± SD: 31.3 ± 4.2 cm/s). The measured by laser Doppler tech- neoscleral laceration in the left eye central retinal artery blood flow ve- niques and color Doppler imag- 6 months previously. The lacera- locities were 11.0 cm/s OD and less ing.1-4 Findings on fluorescein an- tion extended from the superior lim- than 2 cm/s OS (normal mean ± SD: giography also indicate that retinal bus to the inferior limbus. Two 10.1 ± 1.9 cm/s). During the next and choroidal circulation is dimin- months after the ruptured re- week, the episodes of transient vi- ished by scleral buckling proce- pair, she developed an inferior sual loss increased in frequency and dures and encircling elements.5 De- macula-on retinal detachment, duration and the patient elected to spite the marked hemodynamic which was treated with pars plana undergo excision of the encircling alterations, most patients seem clini- vitrectomy and scleral buckling with element. cally unaffected and do not experi- a 42-style silicone encircling ele- The episodes of transient vi- ence symptoms suggestive of poor ment (Labtician, Oakville, On- sual loss abated immediately follow- ocular perfusion.1 However, one pre- tario). The intraocular pressure was ing excision of the encircling ele- viously published report described normal until 4 weeks after the sur- ment. Color Doppler imaging was a patient who developed slowly pro- gery, when it was measured at 44 performed 2 weeks later, and dem- gressive visual field constriction that mm Hg by the Tonopen tonometer onstrated ophthalmic artery sys- reversed after the encircling band (Mentor O&O, Norwell, Mass). tolic blood flow velocities of 46 cm/s was removed.2 Other studies have

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1647

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 also demonstrated the reversibility Hospital, 900 Walnut St, Philadel- and light pigmentation was present of the hemodynamic changes after phia, PA 19107. on the skin of the trunk (Figure 1). removal of the encircling element or On examination, reasonable vi- 2,4 1. Regillo CD, Sergott RC, Brown GC. Successful scleral buckle. scleral buckling procedures decrease central reti- sual following (or fixation) behav- Although the decrease in reti- nal artery blood flow velocity. Ophthalmology. ior for age was demonstrated. There nal artery blood flow following 1993;100:1044-1049. was hypertelorism and a low hair- 2. Yoshida A, Feke GT, Green GJ, et al. Retinal cir- scleral buckling procedures with culatory changes after scleral buckling proce- line. The right had an infero- an encircling element is well docu- dures. Am J Ophthalmol. 1983;95:182-188. nasal ; the anterior seg- 3. Yoshida A, Hirokawa H, Ishiko S, Ogasawara H. mented, the mechanism by which Ocular circulatory changes following scleral ments were otherwise normal. The this occurs is unknown. Diddie buckling procedures. Br J Ophthalmol. 1992;76: fundus examination showed asym- and Ernest6 performed scleral 529-531. metric patches and streaks of darkly 4. Ogasawara H, Feke GT, Yoshida A, Milbocker buckling procedures on rabbits MT, Weiter JJ, McMeel JW. Retinal blood flow and lightly pigmented retina and found that encircling bands alterations associated with scleral buckling and (Figure 2), radiating outward from significantly reduced blood flow to encircling procedures. Br J Ophthalmol. 1992; the optic discs in a petalloid fash- 76:275-279. the retina and as measured 5. Ohkubo H. Fluorescein angiographic findings in ion. There was concern about right by strontium 85–labeled micro- the detached and reattached retina. Jpn J Oph- hypoplasia; the left op- 6 thalmol. 1988;32:423-428. spheres. This effect was not seen 6. Diddie KR, Ernest JT. Uveal blood flow after 360 tic nerve looked normal. The cy- in those eyes with segmental buck- degrees constriction in the rabbit. Arch Ophthal- cloplegic refraction was −6.25+1.00 les or sham surgery. The authors mol. 1980;98:729-730. ϫ 90 OD and +4.25S OS. A subse- speculated that the reduction of quent visit showed a 40–prism di- retinal and choroidal blood flow opter right . involved obstruction of choroidal The child was lost to ophthal- venous drainage. Effects on the Retinal Pigment Mosaicism mologic follow-up until age 2 years, retinal circulation may be the in Pallister-Killian by which time a diagnosis of PKS had result of direct or indirect increases Syndrome (Mosaic been suggested although not con- in peripheral retinal vascular resis- Tetrasomy 12p) firmed by cytogenetic testing. An ex- tance from the buckle indentation.1 amination under anesthesia was per- In this case, a severe degree of Pallister-Killian syndrome (PKS) formed, and punch biopsies of both blood flow velocity reduction was was first reported by Pallister in lightly and darkly pigmented skin seen in the central retinal artery. In 19771 and later by Teschler-Nicola were obtained. Chromosome stud- addition, the ophthalmic artery and Killian in 1981.2 This condi- ies performed on fibroblasts cul- blood flow velocity was also re- tion is caused by a mosaic tetra- tured from the darkly pigmented duced, a finding not encountered in somy of chromosome 12p, which is skin revealed a mos 47,XY,+i(12) the series of asymptomatic patients detectable in cultured fibroblastoid (q10)/46,XY karyotype in 27 of 41 reported by Regillo et al.1 It is likely cells but generally not in peripheral metaphase spreads; 14 of 41 meta- that the retinal, and possibly cho- lymphocytes. Systemic features in phase spreads showed a 46,XY roidal, perfusion was compro- childhood are numerous and vari- karyotype. Fibroblasts cultured from mised to a much greater degree than able, and include most frequently the lightly pigmented skin biopsy what usually occurs following rou- “coarse” facial features, midface specimen revealed a normal 46,XY tine scleral buckle procedures with malformations, psychomotor delay, karyotype in all 50 metaphase encircling elements. The markedly hypotonia, scalp hair sparsity, and spreads. The karyotype derived from diminished ocular perfusion pro- variegated lightly and darkly pig- the dark (hyperpigmented) skin is duced symptoms of transient vi- mented skin.1-9 We describe a consistent with PKS. sual loss precipitated by common patient with the novel ocular mani- postural changes in a young, healthy festation of retinal pigmentary adult. Fortunately, the hemody- mosaicism, and confirm for the first namic changes and symptoms were time that the fibroblast cell line reversible, even months after the from the darkly pigmented skin procedure. contains the chromosomal tetra- somy. Mitchell S. Fineman, MD Carl D. Regillo, MD Report of a Case. A41⁄2-month-old Robert C. Sergott, MD Hispanic boy was referred for evalu- George Spaeth, MD ation of an irregular . He was James Vander, MD born at 35 weeks to nonconsanguin- Philadelphia, Pa eous parents; the pregnancy was com- plicated by maternal amphetamine Supported in part by the Vitreoreti- use. The child was noted at birth to nal Research and Education Founda- have bilateral inguinal hernias and tion, Philadelphia, Pa. dislocated hips, a cleft palate, a patent Figure 1. External color photograph of the patient’s back taken at age 41⁄2 months showing Corresponding author: Carl D. ductus arteriosus, and dysmorphic stripes of whorled and streaky light and dark Regillo, MD, Retina Service, Wills Eye features. Whorled and streaky dark pigmentation.

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1648

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 2. Teschler-Nicola M, Killian W. Case report 72: mental retardation, unusual facial appearance, abnormal hair. Syndr Ident. 1981;7:6-7. 3. Bielanska MM, Khalifa MM, Duncan AM. Pal- lister-Killian syndrome, a mild case diagnosed by fluorescence in situ hybridization: review of the literature and expansion of the phenotype. Am J Med Genet. 1996;65:104-108. 4. Reynolds JF, Daniel A, Kelly TE, et al. Isochro- mosome 12p mosaicism (Pallister mosaic aneu- ploidy or Pallister- Killian syndrome): report of 11 cases. Am J Med Genet. 1987;27:257-274. 5. Schaefer GB, Jochar A, Muneer R, Sanger WG. Clinical variability of tetrasomy 12p. Clin Genet. 1997;51:102-108. Figure 2. Posterior (left) and peripheral (right) color fundus photographs (28-diopter ) taken at age 6. Horneff G, Majewski F, Hildebrand B, Voit T, 2 years showing streaks and patches of alternating dark and light retinal pigmentation. The demarcation Lenard HG. Pallister-Killian syndrome in older between regions is sharp. children and adolescents. Pediatr Neurol. 1993; 9:312-315. 7. Birch M, Patterson A, Fryer A. Hypopigmenta- marcations between affected and un- tion of the fundi associated with Pallister- Reported Ocular Manifestations affected RPE could not be seen. Killian syndrome. J Pediatr Ophthalmol Strabis- of Pallister-Killian Syndrome mus. 1995;32:128-131. An extensive review of the litera- 8. Horn D, Majewski F, Hildebrandt B, Korner H. ture revealed only one other diagno- Pallister-Killian syndrome: normal karyotype in Adnexae sis with similar cutaneous and fundus prenatal chorionic villi, in postnatal lympho- Hypertelorism3-5 cytes, and in slowly growing epidermal cells, but Narrow palpebral fissures3 findings: hypomelanosis of Ito. This mosaic tetrasomy 12p in skin fibroblasts. J Med Large palpebral fissures8 rareneurocutaneousdisorderconsists Genet. 1995;32:68-71. 5 9. Warburton D, Anyane-Yeboa K, Francke U. Mo- Mongoloid palpebral fissures of skin pigmentary mosaicism, patchy saic tetrasomy 12p: four new cases, and confir- Prominent epicanthal folds3,4,8 retinal hypopigmentation, and sei- mation of the chromosomal origin of the super- Lower entropion6 zures. Other ocular abnormalities numerary chromosome in one of the original 3,7 Pallister-Mosaic syndrome cases. Am J Med Genet. Table 2-4 overlap with those of PKS ( ), 1987;27:275-283. Sparse eyebrows andincludestrabismus,hypertelorism, Sparse eyelashes4 Hypoplastic supraorbital ridge3 epicanthal folds, scleralization of the , iris heterochromia, and mi- Anterior segment Recurrent Poststreptococcal Miotic pupils4-6 crophthalmia. The karyotype in hy- Sluggish pupils4 pomelanosis of Ito is normal. Iris atrophy/hypoplasia3,4,6 Our case suggests that infants Iris heterochromia4 Poststreptococcal syndrome (PSS) 1 with dysmorphic facial features, involves the development of sys- Motor other congenital abnormalities, and Nystagmus3.6.7 variegated skin pigmentation would temic nonsuppurative inflamma- Esotropia2,3 benefit from a fundus examination. tion after a streptococcal infection. Exophoria7 The presence of patchy variability in The inflammation is sterile and Exotropia3 RPE pigmentation may be consis- thought to represent an autoim- Fundus tent with PKS. In this case, stan- mune reaction between streptococ- Optic nerve atrophy5 cus-sensitized lymphocytes and host Oval optic discs6 dard karyotyping of peripheral blood 6 lymphocytes is inadequate to rule tissue because of “molecular mim- Fundus hypopigmentation 1 Other out the diagnosis. Fibroblasts cul- icry.” Common manifestations of Amblyopia5 tured from a biopsy of the abnor- PSS include acute rheumatic fever, mally hyperpigmented skin would reactive arthritis, and acute glomeru- reveal the mosaic tetrasomy 12p. lonephritis. Recently, uveitis was de- Comment. To our knowledge, we scribed as a sign of PSS.2-4 This in- are the first to describe retinal pig- Wade Graham, MD traocular inflammation also develops ment mosaicism in PKS. This find- Sandra M. Brown, MD after the bacterial infection. One re- ing is similar to the characteristic Farah Shah, MD port claims PSS uveitis can be re- variegated skin color. If we assume Vijay S. Tonk, PhD current.4 Herein, we confirm the that the retinal pigment mosaicism Lubbock, Tex findings of that single case and re- is caused by karyotype mosaicism, Mary K. Kukolich, MD port that the length of time be- this suggests that the short arm of Fort Worth, Tex tween episodes may be as long as 27 chromosome 12 contains a gene that months. affects retinal pigment epithelium (RPE) pigment production. We can- Reprints: Sandra M. Brown, MD, De- Report of a Case. A 10-year-old not explain why this striking fun- partment of Ophthalmology and Vi- white boy developed , dus feature was not noted in previ- sual Sciences, Texas Tech University ocular redness, and blurred vision ous case reports, except to postulate Health Sciences Center, Sixth and bilaterally following an episode of that the level of mosaicism in the Flint, Lubbock, TX 79430 (e-mail: streptococcal pharyngitis. Medical RPE was sufficiently low (whether [email protected]). history was otherwise noncontribu- the karyotype was predominantly 1. Pallister PD. The Pallister mosaic syndrome. Birth tory. Examination noted vision of normal or abnormal) that sharp de- Defects. 1977;13:103-110. 20/80 OU with 3+ cells in the ante-

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1649

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 rior chambers. Workup revealed an onstrated findings and a clinical mas. Patients typically have hear- elevated antistreptolysin O (ASO) ti- course typical for PSS uveitis.2-4 The ing loss in the second or third de- ter (760 IU/mL) (normal range, patient’s HLA-DR2 typing is inter- cade of life. About 50% of cases are 0-125 IU/mL) and an elevated eryth- esting, as this haplotype has been inherited in an autosomal domi- rocyte sedimentation rate (27 mm/h) seen in association with rheumatic nant fashion. (normal range, 0-15 mm/h). Titers fever and other PSS.1 Recurrent PSS Among the many ophthalmic for antinuclear antibodies, rheuma- uveitis is not surprising since other signs that have been described in toid factor, toxoplasmosis, Lyme dis- PSS entities may be recurrent, even NF2,1-4 has not been ease, and angiotensin-converting en- with a subclinical infection.1 highlighted. We report 2 cases in zyme were normal. Chest x-ray Confirmation that PSS uveitis which the dire consequences of sus- films, urinalysis, complete blood cell may be recurrent could be impor- tained elevated intracranial pres- count, and chemistry panel were un- tant for its diagnosis, prevention, and sures were not recognized in time to remarkable. Symptoms resolved with treatment. We agree with Leiba et al4 prevent visual loss. cycloplegics and topical steroids. that all patients with ocular signs and Twenty-seven months later he de- symptoms of PSS and active strep- Report of Cases. Case 1. A 31-year- veloped blurred vision, ocular ten- tococcal infection or a history of PSS old man reported a 2-year history of derness, and photophobia bilater- should receive eye examinations. Ex- worsening bilateral hearing loss and ally 10 days after streptococcal amining blood ASO titers may be dizziness in 1992. Bilateral sensori- pharyngitis. Medications included a useful in patients with idiopathic neural hearing was documented. Re- 10-day course of a combination uveitis and a history of a streptococ- sults of a neurological examination product consisting of amoxicillin cal infection. were otherwise normal. A mag- and clavulanate (500 mg twice netic resonance imaging (MRI) scan daily). He denied other PSS symp- William J. Wirostko, MD disclosed masses coating the cere- toms. On examination, visual acu- Thomas B. Connor, Jr, MD bral convexities (Figure 1, A), the ity was 20/50 OU. , motility, Paul F. Wagner, MD acoustic nerves, gasserian ganglia bi- visual field, and tonometry were nor- Milwaukee, Wis laterally, the right hypoglossal ca- mal. Slitlamp examination re- nal, the posterior tuberculum sella, vealed mild bulbar conjunctival hy- This study was supported in part by and planum sphenoidale. These peremia in both eyes. Both anterior the Heed Ophthalmic Foundation (Dr findings were considered diagnos- chambers had 2+ cells and flare. Fine Wirostko), and an unrestricted grant tic of sporadic NF2. A lumbar punc- white keratic precipitates were pres- from Research to Prevent Blindness ture revealed a normal opening pres- ent on the inferior cornea bilater- Inc, New York, NY. sure and the cerebrospinal fluid ally. Irides demonstrated no nod- Reprints: Thomas B. Connor, Jr, (CSF) formula was normal. Results ules or atrophy. Anterior vitreous MD, The Eye Institute, 925 N 87th St, of an ophthalmologic examination, contained mild cells in both eyes. Milwaukee, WI 53226 (e-mail: including visual acuity, visual fields, Fundus examination disclosed disc [email protected]). and ophthalmoscopy, were en- hyperemia with a few fine periph- tirely normal. eral vitreous precipitates bilater- 1. Bisno AL. Rheumatic fever. In: Kelly WN, Har- In late 1993, mild bilateral op- ris ED, Ruddy S, Sledge CB, eds. Textbook of Rheu- ally, but no “snow-banking” or vas- matology. 5th ed. Philadelphia, Pa: WB Saun- tic disc edema was noted without any cular sheathing. Blood testing ders Co; 1996:1225-1239. new symptoms or abnormalities of revealed an elevated ASO titer (753 2. Cokingtin CD, Han DP. Bilateral non-gran- visual function (Figure 2, A). An- ulomatous uveitis and a post-streptococcal syn- IU/mL), an elevated erythrocyte sedi- drome. Am J Ophthalmol. 1991;112:595-596. other MRI scan showed no changes. mentation rate (27 mm/h), and an 3. Benjamin A, Tufail A, Holland GN. Uveitis as the He was diagnosed as having pap- HLA-DR2 haplotype. Complete only clinical manifestation of post-streptococ- illedema related to impaired CSF cal syndrome. Am J Ophthalmol. 1997;123:258- blood cell count, HLA-B27, rapid 260. outflow owing to obstruction by dif- plasma reagin, and angiotensin- 4. Leiba H, Barash J, Pollack A. Post-streptococcal fuse convexity meningiomatosis. converting enzyme levels were un- uveitis. Am J Ophthalmol. 1998;126:317-318. Because he had normal visual func- remarkable. Treatment was begun tion, no further diagnostic or thera- with aggressive topical 1% predniso- peutic intervention was under- lone acetate and cycloplegics. Within Visual Loss Secondary to taken. Subsequent semiannual 6 weeks, all inflammation re- Increased Intracranial Pressure ophthalmologic examinations dur- solved, and visual acuity returned to in Neurofibromatosis Type 2 ing the next 2 years showed no new 20/20 OU. findings. Neurofibromatosis type 2 (NF2), After being lost to ophthalmo- Comment. We believe this patient previously known as central neuro- logic follow-up for 2 years, he re- had recurrent PSS uveitis. How- fibromatosis, is defined clinically by turned in September 1997 with a ever, we are aware of only 1 other the presence of bilateral acoustic 2-week history of orthostatic tran- case of recurrent PSS uveitis.4 In our nerve schwannomas and geneti- sient visual obscurations. Visual patient, both episodes of inflamma- cally by a mutation in the long arm acuity was still 20/20 OU, but auto- tion were preceded by a culture- of chromosome 22.1 Schwannomas mated visual fields showed general- positive streptococcal pharyngitis of other cranial and spinal nerves are ized depression and mildly enlarged with elevated ASO titers, and dem- often present, as well as meningio- blind spots in both eyes, with an in-

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1650

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 A B

Figure 1. Case 1. A, Magnetic resonance imaging scan in 1992 shows diffuse thickening of the convexity dura by meningioma. B, Magnetic resonance imaging scan in 1997 shows interval growth of convexity meningioma.

A

30° 30°

B

30° 30°

C

30° 30°

Figure 2. Case 1. A, In 1993, mild edema is present (left), but visual fields are normal (right). B, In September 1997, optic disc edema has become more pronounced (left), and early visual field loss has emerged (right). C, In December 1997, optic disc edema has worsened (left), and visual fields are markedly constricted (right).

ferior nerve fiber bundle defect in interval growth of the cerebral dural He was treated with acetazol- the left eye (Figure 2, B). The optic convexity masses (Figure 1, B). amide (2 g/d) and furosemide (40 disc edema was more pronounced There was no venous sinus throm- mg/d) but his visual acuity, visual in each eye. An MRI scan showed bosis or ventriculomegaly. fields, and optic disc edema wors-

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1651

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 ened during the next 2 months. By began complaining in 1997 of pro- light perception OD and 20/50 OS. December 1997, visual acuity had gressive visual loss in the left eye Pupils measured 5 mm in dim illu- fallen to 20/80 OD and 20/40 OS. Vi- during the previous year and the re- mination; the right did not react to sual fields now showed marked con- cent onset of transient visual obscu- direct light and the left was slug- striction in both eyes. Optic disc rations in that eye. His right eye had gish. A right afferent pupillary edema was even more severe (Fig- earlier been blinded by a retinal de- defect was present. Because of his ure 2, C). A lumbar puncture re- tachment. deafness, neck weakness, and quad- vealed an opening pressure of 32 cm He was totally deaf owing to bi- riplegia, visual fields could be as- H2O with a normal CSF formula. In lateral acoustic schwannomas and sessed only by confrontation, show- the month that followed, he under- had earlier undergone partial resec- ing a small temporal island in the left went optic nerve sheath fenestra- tions of a foramen magnum menin- eye. Neurotrophic in the left tion in the right eye, high-dose in- gioma, a right frontal meningioma, eye had caused partial opacification travenous methylprednisolone and a left acoustic schwannoma. The of the cornea with pannus. Ophthal- treatment, and a lumboperitoneal acoustic surgery had resulted in left moscopy of the left eye faintly dis- shunt, all of which failed to halt facial and trigeminal neuropathy closed chronic disc edema with some the downward spiral of visual loss. with resulting neurotrophic kerati- atrophy. The right fundus revealed a By February 1998, visual acuity tis in the left eye. A right acoustic chronic total retinal detatchment with had declined to hand motions OU. schwannoma had remained unop- no view of the optic nerve. The optic discs were pale and flat erated. A brainstem stimulator, A computed tomographic scan (Figure 3). A shunt tap con- placed in the medulla in 1989 to pro- of the brain showed considerable en- firmed normalization of the open- vide some hearing, precluded fur- largement of the ventricles relative ing pressure to 17 cm H2O. ther use of MRI. to an MRI in 1988 (Figure 4, A and Case 2. A 26-year-old man with Ophthalmologic examination in B). Because of the foramen mag- a remote diagnosis of sporadic NF2 1997 disclosed visual acuities of no num lesion, lumbar puncture was considered contraindicated for fear of provoking brainstem herniation. The patient’s progressive visual loss was attributed to papilledema asso- ciated with chronic obstructive hy- drocephalus from the posterior fossa masses. In preparation for a CSF shunting procedure, the patient was given acetazolamide (2 g/d) to avoid the visual loss associated with sur- gically induced sudden intracra- nial hypotension. Several weeks later, the patient’s visual acuity had fallen to 20/200 OS. Figure 3. Case 1. In 1998, 3 weeks after ventriculoperitoneal shunting, the optic disc edema has been The patient underwent ven- replaced by disc pallor. triculoperitoneal shunting that re-

A B C

Figure 4. Case 2. A, Magnetic resonance imaging scan in 1988 shows normal-sized ventricles. B, Computed tomographic scan in 1997 (before shunting operation) shows interval ventricular enlargement. C, Computed tomographic scan in 1998 (after shunting operation) shows reduction in ventricular size.

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1652

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 vealed an opening CSF pressure of a pressure gradient between the ven- pressure, demonstrating unequivo- 33 cm H2O. Four weeks after shunt- tricles and subarachnoid space, lead- cally the acquired nature and pos- ing, visual acuity had improved to ing to ventriculomegaly. We specu- sible etiologic association with nega- 20/70 OS. Confrontation visual fields late that this may be the more tive maxillary sinus pressure in at least showed persistent nasal loss with a common cause of papilledema in some cases of silent sinus syndrome. preserved temporal island. Fundus NF2, referred to previously by other examination disclosed signifi- authors.3,4 Report of a Case. A 27-year-old cantly less optic disc edema in the The early recognition and treat- woman with painless, progressive left eye with mild pallor. A postop- ment of papilledema in patients with sinking of her right eye over a erative computed tomographic scan NF2 can be difficult. As in our sec- 3-month period demonstrated 8 mm revealed normalization of ventricu- ond patient, media opacification of and 4 mm of hy- lar size (Figure 4, C). from coexistent neurotrophic kera- poglobus. Computed tomography titis can inhibit the detection of disc showed a small, opacified right Comment. Our cases highlight edema. Once recognized, it may be maxillary sinus with a depressed or- chronic papilledema as a cause of se- difficult to decide at what point the bital floor (Figure 1), a new find- vere visual loss in NF2. This mani- potential for developing chronic ing, as a magnetic resonance imag- festation has been mentioned in pre- atrophic papilledema outweighs the ing study performed 3 years earlier vious reports, but without emphasis risks of intervention. Whether early (for new-onset seizures) was nor- or explanation of mechanism. For surgery in these patients is war- mal (Figure 2). example, Bouzas et al2 mentioned ranted is unknown. Regular oph- The patient underwent right or- that 4 (7.4%) of 54 patients with NF2 thalmologic follow-up is essential in bital floor reconstruction with max- had progressive visual field constric- preventing visual morbidity. Vi- illary antrostomy. Before surgical tion from long-standing papille- sual loss can be particularly devas- manipulation, the maxillary os was dema, one of whom had loss of cen- tating to these patients, as they of- found to be occluded, and an 18- tral acuity in one eye to 20/160. Kaye ten have hearing impairment and gauge needle attached to a pressure et al4 and Landau and Yasargil3 sepa- other neurological disabilities. transducer (model 90602A; Space- rately made passing references to 2 Labs Inc, Redmond, Wash) was in- patients with NF2 and papilledema Dilip A. Thomas, MD serted into the sinus. A pressure of secondary to intracranial tumors. Jonathan D. Trobe, MD −23 mm Hg was recorded. Furthermore, the National Insti- Wayne T. Cornblath, MD tutes of Health guidelines2 include Ann Arbor, Mich Comment. Silent sinus syndrome an initial ophthalmic assessment for is spontaneous enophthalmos and detecting the presence of juvenile Reprints: Jonathan D. Trobe, MD, W. hypoglobus associated with a small, cataracts and for the “photography K. Kellogg Eye Center, 1000 Wall St, ipsilateral maxillary sinus.1 It de- of papilledema.” No explanation ac- Ann Arbor, MI 48105 (e-mail: velops over a course of days to years companies this recommendation. [email protected]). and is not associated with trauma. In our first patient, increased in- At presentation, the maxillary os may 1. Mulvihilll JJ, Parry DM, Sherman JL, Pikus A, tracranial pressure was likely caused Kaiser-Kupfer MI, Elridge R. NIH conference: be patent or occluded, and the si- by diffuse convexity meningiomato- neurofibromatosis 1 (Recklinghausen disease) nus may be partially or completely sis that interfered with CSF outflow and neurofibromatosis 2 (bilateral acoustic neu- opacified. rofibromatosis): an update. Ann Intern Med. 1990; across the arachnoid granulations. In 113:39-52. One theory for the develop- this communicating hydrocepha- 2. Bouzas EA, Parry DM, Elridge R, Kaiser-Kupfer ment of silent sinus syndrome is as lus, the fact that there was no pres- MI. in patients with neuro- follows. Occlusion of the maxillary fibromatosis 2. Neurology. 1993;43:622-623. sure gradient across the ventricular 3. Landau K, Yasargil GM. Ocular fundus in neu- sinus os forms an enclosed muco- outflow routes explains why the ven- rofibromatosis type 2. Br J Ophthalmol. 1993; sal space where resorption of air cre- 77:646-649. tricles were not enlarged. A similar 4. Kaye LD, Rothner AD, Beauchamp GR, Meyers ates negative pressure. Such nega- mechanism has been described in a SM, Estes ML. Ocular findings associated with tive pressure has been recorded in case of childhood meningiomato- neurofibromatosis type II. Ophthalmology. 1992; occluded rabbit maxillary sinuses2 5 99:1424-1429. 3 sis. Dural sinus drainage may also 5. Avellino AM, Hair LS, Symanns WF, et al. Men- and in humans. Negative sinus pres- have been compromised by the me- ingeal meningiomatosis in a child: case report. sure may cause thinning and in- ningiomas, but was not seen on MRI Clin Neuropathol. 1994;13:82-87. ward bowing of the sinus walls, in- or documented by angiography. To cluding the orbital floor, resulting in the best of our knowledge, these hypoglobus. If the maxillary os re- mechanisms of increased intracra- Negative Sinus Pressure opens, the sinus fluid, which ini- nial pressure in NF2 have not been and Normal Predisease tially may have provided some sup- previously described. Imaging in Silent Sinus port for the thin orbital floor, may In our second patient, papille- Syndrome drain, allowing further depression of dema was due to compression of the the orbital floor and globe. This may brainstem by tumor. This led to an We present the first case of silent si- account for the rapid presentation obstruction of CSF outflow through nus syndrome with both normal pre- of some patients, the variable pat- the fourth ventricle. In this noncom- disease imaging findings and docu- ency of the os, and the variability of municating hydrocephalus, there is mented negative maxillary sinus sinus fluid.

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1653

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 Figure 1. Computed tomographic scan (axial [left] and coronal [right]) at time of presentation shows marked depression of the right orbital floor with opacification of the right maxillary sinus.

Figure 2. Magnetic resonance imaging study 3 years before presentation shows normal orbits and maxillary sinuses bilaterally (left, anterior; right, posterior).

In the more than 25 cases in our nature as well as likely etiologic as- Girardeau, MO 63702-2018 (e-mail: experience, and a nearly equal num- sociation with negative maxillary si- [email protected]). ber in the literature, we are aware of nus pressure in at least some cases 1. Soparkar CNS, Patrinely JR, Cuaycong MJ, et al. only one case of silent sinus syn- of silent sinus syndrome. The silent sinus syndrome. Ophthalmology. 1994; drome with normal predisease neu- 101:772-778. roimaging findings4 and only one re- Joseph K. Davidson, MD 2. Scharf KE, Lawson W, Shapiro JM, Gannon PJ. Pressure measurements in the normal and oc- port of documented negative sinus Charles N. S. Soparkar, MD, PhD cluded rabbit maxillary sinus. Laryngoscope. pressure.3 Our patient represents the Jack B. Williams, MD 1995;105:570-574. 3. Kass ES, Salman S, Montgomery WW. Mano- first case of silent sinus syndrome James R. Patrinely, MD metric study of complete ostial occlusion in with both normal predisease imag- Houston, Tex chronic maxillary atelectasis. Laryngoscope. 1996; ing and documented negative max- 106:1255-1258. 4. Eto RT, House JM. Enophthalmos: a sequela of illary sinus pressure, demonstrat- Corresponding author: Joseph David- maxillary sinusitis. AJNR Am J Neuroradiol. 1995; ing unequivocally the acquired son, MD, 360 S Mt Auburn Rd, Cape 16:939-941.

ARCH OPHTHALMOL / VOL 117, DEC 1999 WWW.ARCHOPHTHALMOL.COM 1654

©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021