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vasculitis was reported earlier. Experimental inoculation of bacteria into the vitreous cavity revealed retinal periphlebitis to be the first clinical feature observed.[6] The pathogenesis of hemorrhages and periphlebitis is attributed to the breakdown of blood–retinal barrier, and thus perivascular exudation of plasma proteins and white blood cells.[8] Retinal vasculitis as an isolated sign of endophthalmitis has been rarely reported in literature. This could be because of the lack of awareness, rarity of this sign, more fulminant cases or delayed diagnosis or delayed presentation leading to a poor view of fundus. A Medline search revealed only eight similar cases reported so far [Table 1]. Of these, only one case was post-traumatic. These findings may mimic the picture of retinal vein occlusion, Eales’ disease, Behcet’s, sarcoidosis, pars planitis, infectious retinochoroiditis and non-specific uveitis, but a history of recent open globe trauma or surgery or systemic infection should indicate the possibility of an infectious etiology. In post-surgical eyes with increased anterior chamber reaction, dilated inferior retinal examination should be done to look for such white-centered retinal hemorrhages and vasculitis to rule out early endophthalmitis. A delay in the diagnosis and treatment of endophthalmitis is associated with poor visual recovery. All cases reviewed, including ours, had good visual outcomes with treatment.
References 1. Mamalis N. Endophthalmitis. J Cataract Refract Surg 2002;28: 729-30. 2. Kernt M, Kampik A. Endophthalmitis: Pathogenesis, clinical presentation, management, and perspectives. Clin Ophthalmol 2010;24:121-35. 3. Jeng BH, Kaiser PK, Lowder CY. Retinal vasculitis and posterior pole “hypopyons” as early signs of acute bacterial endophthalmitis. Am J Ophthalmol 2001;131:800-2. 4. CLSI. Performance Standards for Antimicrobial Disk Susceptibility Tests, Approved Standard, 10th edition, MO2-A10.Wayne, PA: Clinical and Laboratory Standards Institute. Available from: http:// www.clsi.org/source/orders/free/m02-a10.pdf.2009. [Last accessed on 2010 Dec 20]. 5. Packer AJ, Weingeist TA, Abrams GW. Retinal periphlebitis as an early sign of bacterial endophthalmitis. Am J Ophthalmol 1983;96:66-71. 6. Godley BF, Folk JC. Retinal hemorrhages as an early sign of acute bacterial endophthalmitis. Am J Ophthalmol 1993;116:247-9. 7. Chowdhury T, Jalali S, Majji AB. Successful treatment of fungal retinitis and retinal vasculitis with oral itraconazole. Retina 2002;22:800-2.
Conclusion
8. Forster RK. Endophthalmitis. In: Duane TD, editor. Clinical Ophthalmology. Hagerstown: Harper and Row; 1981. p. 1.
Awareness about retinal vasculitis being an early presenting feature could lead to early diagnosis of endophthalmitis and good visual outcome.
9. Subbiah S, McAvoy CE, Best JL. Retinal vasculitis as an early sign of bacterial post-operative endophthalmitis. Eye (Lond) 2010;24: 1410-1.
Nanophthalmos and situs inversus totalis
Key words: Dextrocardia, nanophthalmos, situs inversus totalis
Alparslan Şahin, Refik Oltulu1, Ali S Kıvrak2, Ahmet Özkağnıcı3
Cite this article as: Şahin A, Oltulu R, Kivrak AS, Özkagnici A. Nanophthalmos and situs inversus totalis. Indian J Ophthalmol 2012;60:319-21.
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Nanophthalmos is characterized by short axial length, high hypermetropia, thick sclera and a normal-sized crystalline lens. Situs inversus totalis is the mirror image of the normal morphology of the thoracic and abdominal viscera. To the best of our knowledge this is the first report of a nanophthalmic patient with situs inversus totalis. Therefore, we would like to invite the attention of our colleagues to our case and underline the importance of the systemic examination of the nanophthalmic patients to detect systemic malformations and visceral transpositions. Department of Ophthalmology, Dicle University, Diyarbakır, 1 Department of Ophthalmology, Kahramanmaraş State Hospital, Kahramanmaraş, Departments of 2Radiology and 3Ophthalmology, Selçuk University, Konya, Turkey Correspondence to: Dr. Alparslan Şahin, Department of Ophthalmology, Dicle University, Faculty of Medicine, Diyarbakır, Turkey. E-mail:
[email protected] Manuscript received: 11.02.10; Revision accepted: 23.12.10
Website: www.ijo.in DOI: 10.4103/0301-4738.98718 PMID: ***
Nanophthalmos represents an arrested development of the globe in all directions after the closure of the embryonic fissure. It is a rare clinical disorder characterized by a small eye with short axial length, high hypermetropia, thick sclera and a normal-sized crystalline lens.[1,2] The axial length in nanophthalmos measures 14-20 mm.[3] Nanophthalmic patients have a high risk for developing glaucoma and choroidal detachment. Moreover, in children, high hyperopia with resultant amblyopia and strabismus are among the more important ophthalmic considerations.[4] To the best of our knowledge, this is the first report describing the association of nanophthalmos and situs inversus totalis.
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Case Report A five-year-old boy presented with complaint of esodeviation on his right eye. The best-corrected visual acuities in the right eye and in the left eye were 20/60 with +14.00 diopter (D) (+0.25 D cylinder α 140°) and +14.00D (+0.50 D cylinder α 50°), respectively. He had a comitant 25-D esotropia at distance and near in his right eye. Both eyes had Grade 2 overaction of inferior oblique muscles. Patient had suppression of the right eye. Ductions and versions were normal in both eyes. Horizontal and vertical corneal diameters were 11.5 mm and 10.0 mm, respectively in each eye. Results of a biomicroscopic examination of the anterior and posterior segment of the eyes were unremarkable. His intraocular pressures were 13–14 mm Hg in both eyes. The axial length measured 16.24 mm in the right eye and 15.64 mm in the left eye [Fig. 1]. The patient was consulted by a pediatrician due to growth retardation. No pathologic finding, but situs inversus totalis was found by physical examination, computed tomography and echocardiography. Computed tomography showed mirror image transposition of the abdominal and thoracic viscerae [Fig. 2]. Dextrocardia was diagnosed but anatomic appearance and the M mode measurements of the heart were normal [Fig. 3]. Family history and maternal history during pregnancy revealed no abnormal finding. Intrauterine growth retardation (IUGR) was not stated by the past medical history of the patient. Postnatal history of the case revealed that the child had been born as 2700 g in weight and 48 cm in length, which excluded IUGR. Chromosomal analysis gave no abnormality and no chronic respiratory infection episodes were reported. The study of his pedigree showed sporadic pattern. The growth retardation of the patient was suggested as related to insufficient dietary intake of nutrients.
Discussion Nanophthalmos is characterized by short axial length, thick sclera, narrow palpebral fissure, small orbit, shallow anterior chamber, normal-sized lens, and hyperopia of up to 20 D.[1,2] The small eye without ocular malformations is considered to be nanophthalmic.[5] Complications such as glaucoma, macular hypoplasia, papillomacular folds, and pigmentary retinal dystrophy have been reported.[3] But these complications were not found in our case. The etiology of nanophthalmos is still not known. The majority of the cases are sporadic, but autosomal recessive and autosomal dominant inheritance have been reported. Glycogen-like deposits, together with the abnormal fibrils, are thought to lead to scleral rigidity that causes a small intraocular volume, choroidal congestion, detachment, and exudative retinal detachment.[6,7] Karyotype analysis of the patient revealed no chromosomal anomalies. Both orbital fissure closure and visceral organ rotation occur between 5th10th gestational weeks.[8] Thus we suggest that our patient might have been affected during this time period. It was shown by histological examination that the nanophthalmic sclerae were much thicker, and the collagen fibrils showed a less orderly arrangement than the sclerae of the normal controls.[7] Nanophthalmic eyes are at risk for angle-closure glaucoma, uveal effusion, and exudative
Figure 1: The A-scan measurements of the eyes
Figure 2: The computed tomography image of the abdomen. Note the situs inversus anatomy of the abdominal organs. A: Aorta F: Fundus of the stomach L: Liver S: Spleen VCI: Vena Cava Inferior
Figure 3: Thoracic computed tomography scan shows the dextrocardia
retinal detachment. [9] Angle-closure glaucoma occurs in nanophthalmic eyes because of the large lens in the small eye. Uveal effusion is the result of choroidal congestion secondary to impaired vortex venous drainage through the thick sclera.[6]
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The high hyperopia leading to ametropic amblyopia, associated strabismus leading to strabismic amblyopia are the main complications in children.[4] Strabismus usually presents with esotropia such as in our case. The short axial length with high hyperopia is the most likely factor in the development of esotropias in nanophthalmic eyes.[4] Situs inversus totalis is a condition with left-to-right reversal of the viscera combined with dextrocardia. In addition to their abnormal position, individual organs present a symmetrical mirror image morphology. Situs inversus totalis associated with primary ciliary dyskinesia is known as Kartagener syndrome. Patients with primary ciliary dyskinesia have recurrent sinus and pulmonary infections due to immotile cilia that line their upper airways. No recurrent infections were reported in the medical history of our patient. So we did not think immotile cilia syndrome in the case. The prevalence of situs inversus totalis seems to range between 1 in 8000 and 1 in 25000.[10] No racial predilection exists and the male-to-female incidence is 1:1. Situs inversus totalis is usually discovered incidentally as in our case. Dextrocardia is easily diagnosed by clinical examination. Although chest roentgenogram, ultrasound, scintigraphy and angiography are used in the diagnosis of visceral organ transpositions, computed tomography and magnetic resonance imaging are the preferred examinations for the definitive diagnosis of situs inversus totalis. The recognition of situs inversus totalis may help avoid mishaps at surgery or other interventions that result from the failure to recognize reversed anatomy or an atypical history. Both nanophthalmos and situs inversus totalis are rare clinical conditions. To the best of our knowledge, this is the first
Isolated homonymous hemianopsia due to presumptive cerebral tubercular abscess as the initial manifestation of human immunodeficiency virus infection Sujit Gharai, Pradeep Venkatesh, Anindita Sinha1, Satpal Garg, Prapti Ghosh2 We report a case of isolated homonymous hemianopsia due to presumptive cerebral tubercular abscess as the initial manifestation of human immunodeficiency virus (HIV) infection. Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, 1Department of Radio-diagnosis, All India Institute of Medical Sciences, New Delhi, 2Department of Ophthalmology, Patna Medical College, Bihar, India Correspondence to: Dr. Sujit Gharai, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India. E-mail:
[email protected] Manuscript received: 02.05.10; Revision accepted: 30.12.10
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report of a nanophthalmic patient with situs inversus totalis. The clinicians should keep in mind that nanophthalmos may accompany situs inversus totalis.
References 1. Singh OS, Simmons RJ, Brockhurst RJ, Trempe CL. Nanophthalmos: A perspective on identification and therapy. Ophthalmology 1982;89:1006-12. 2. Wu W, Dawson DG, Sugar A, Elner SG, Meyer KA, McKey JB, et al. Cataract surgery in patients with nanophthalmos: Results and complications. J Cataract Refract Surg 2004;30:584-90. 3. Goldstein DA, Tessler HH. Episcleritis, scleritis, and other scleral disorders. In: Yanoff M, Duker JS, Augsburger JJ, Azar DT, editors. Yanoff: Ophthalmology. 2nd ed. Philadelphia, Pa: Mosby; 2004. chap. 64. 4. Sener EC, Mocan MC, Saraç OI, Gedik S, Sanaç AS. Management of strabismus in nanophthalmic patients: A long-term follow-up report. Ophthalmology 2003;110:1230-6. 5. Auffarth GU, Blum M, Faller U, Tetz MR, Völcker HE. Relative anterior microphthalmos: Morphometric analysis and its implications for cataract surgery. Ophthalmology 2000;107:1555-60. 6. Brockhurst RJ. Vortex vein decompression for nanophthalmic uveal effusion. Arch Ophthalmol 1980;98:1987-90. 7. Fukuchi T, Sawada H, Seki M, Oyama T, Cho H, Abe H. Changes of scleral sulfated proteoglycans in three cases of nanophthalmos. Jpn J Ophthalmol 2009;53:171-5. 8. Nischal KK. Developmental anomalies of the anterior segment and globe. In: Wright KW, Spiegel PH, editors. Pediatric ophthalmology and strabismus. 2nd ed. New York: Springer; 2002. chap. 24. 9. Krohn J, Seland JH. Exudative retinal detachment in nanophthalmos. Acta Ophthalmol Scand 1998;76:499-502. 10. Splitt MP, Burn J, Goodship J. Defects in determination of left-right asymmetry. J Med Genet 1996;33:498-503.
A 30-year-old man presented to our outpatient department with sudden loss of visibility in his left visual field. He had no other systemic symptoms. Perimetry showed left-sided incongruous homonymous hemianopsia denser above the horizontal meridian. Magnetic resonance imaging revealed irregular wellmarginated lobulated lesions right temporo-occipital cerebral hemisphere and left high fronto-parietal cerebral hemisphere suggestive of brain tubercular abscess. Serological tests for HIV were reactive, and the patient was started only on anti-tubercular drugs with the presumptive diagnosis of cerebral tubercular abscess. Therapeutic response confirmed the diagnosis. Atypical ophthalmic manifestations may be the initial presenting feature in patients with HIV infection. This highlights the need for increased index of suspicion for HIV infection in young patients with atypical ophthalmic manifestations. Key words: Acquired immunodeficiency syndrome, cerebral abscess, human immunodeficiency virus, homonymous hemianopsia, initial ophthalmic manifestation, tubercular abscess, tuberculosis Cite this article as: Gharai S, Venkatesh P, Sinha A, Garg S, Ghosh P. Isolated homonymous hemianopsia due to presumptive cerebral tubercular abscess as the initial manifestation of human immunodeficiency virus infection. Indian J Ophthalmol 2012;60:321-4.
