DJO Vol. 20, No.4, April-June, 2010 Contents
Editorial
3. The Avatar Phenomenon ...... Rohit Saxena Major Review
5. Role Of Tear Osmolarity In Dry Eye Noopur Gupta, Nidhi Gupta, Radhika Tandon 9. Dissociated Vertical Deviation(DVD) Shailesh, Rohit Saxena, Ravindranath H.M. 13. Management of Superior Oblique Palsy Archana Gupta Mahajan 19. Management of Corneal Trauma Prakashchand Agarwal, Namrata Sharma, Rajesh Sinha 24. A.V.Pattern Strabismus Jaspreet Sukhija, Balwinder Kaur, Zoram Zadeng Preferred Pratice Patterns
29. Current Managment of Retinopathy of Prematurity Parijat Chandra, Rajvardhan Azad, Yograj Sharma, Atul Kumar Cases Report
33. Goldenhar-Gorlin syndrome U.S.Tiwari, Debarati Saha 37. Crouzon Syndrome Navin Chandra 40. Bilateral Mooren’s Ulcer Uday Gajiwala, Jyotsom Ganatra, Rajesh Patel, Parin Shah, Rohan Chariwala History of Ophthalmology
45. Glaucoma Drainage Devices: A Historical Perspective Shibal Bhartiya Instruments Scan
49. Pediatric Electrophysiology Jitendra Jethani, Thakorbhai Instruction to Authors 57. Instruction to Authors
1 DJO Vol. 20, No.4, April-June, 2010
Editorial
The Avatar Phenomenon
Dear Friends,
Many Ophthalmologists must have given the ‘out of this world’ 3D movie ‘Avatar’ recently in theaters, a miss. They cannot be blamed for thinking that James %COGTQPJCFOCFGKVQPN[HQTVJG[QWPIŎ5KſŏHCP
But the truth is that he had made it as a wake – up call for the ophthalmologist. In ophthalmology if the last century was the period for ‘par excellence’ uniocular vision: from aphakia to IOLS to LASIK and beyond, this century promises to go deeper: In real 3 - D depth. Entertainment and movies have shown that they can provide television and art in 3D and it becomes imperative for us, the ophthalmologist to ensure their not one EJKNFOKUUGUVJGDGCWV[QHVJG&YQTNFYJGVJGTVJGTGCNQPGQTVJGCTVKſEKCNN[ETGCVGF ones.
Having seen so many one eyed individuals function comfortably in their surrounding and having taken our own binocular depth perception for granted, it takes a movie like Avatar to show us how different the 3D is to the 2D: like cheese is from chalk.
Movies like these remind us that we must look at our patient’s both eyes together and not separately. They remind us that great vision in either eye is not the best,
Superb binocular vision is! Appreciating the Avatar phenomenon is!
Dr.Rohit Saxena
The editorial board of the DJO solicits article from its readers on all aspects of ophthalmology. Major heads under which article are published by the DJO are given in the introduction to authors. We also welcome to article comments and advise on how to improve the DJO.
Any DOS member who has not received the previous three issue (July - Sept. 2009, Oct. - Dec. 2009 and Jan – Mar 2010) please contact DOS Secretariat [email protected], [email protected] or Editor, DJO [email protected]. Some copies have come back due to incorrect addresses, so members are requested to please provide correct addresses and contact details to DOS Secretariat.
3 DJO Vol. 20, No.4, April-June, 2010 Major Review ROLE OF TEAR OSMOLARITY IN DRY EYE
Noopur Gupta, Nidhi Gupta, Radhika Tandon Cornea & Refractive Surgery Services, Dr. R.P. Centre for Ophthalmic Sciences, AIIMS, New Delhi.
6JGſTUVGXGTRWDNKUJGFCUUQEKCVKQPQHFT[G[GYKVJ by its equivalence to a determined percentage of NaCl the occurrence of salty tears was described by Galen water solution. More recently, most authors express in the second century.1 Three centuries later, the tonicity according to the quantity of moles per litre of concept of tear salinity was based on the taste of solution (osmolarity). QXGTƀQYKPIVGCTFTQRU6JGDCUKUQHVJGHWVWTGEQPEGRV The site of tear sampling is of importance in all of osmolarity originated in 1748,when a French priest measurements of tear osmolarity. Various authors have and scientist Nollet2QDUGTXGFVJGƀWKFƀQYVJTQWIJ URGEKſGFVJGRCTVQHVJGNCETKOCNUGCHTQOYJKEJVJG[ COGODTCPGFKXKFGFD[ƀWKFUQHFKHHGTGPVQUOQNCTKV[ took the tear sample: inferior conjunctival fornix, tear However, the most common method of measuring meniscus, or cisterna lacrimalis. Tear has been taken osmotic pressure in clinics was introduced by Raoult, with absorbent paper or washed cotton and pipette. who in 1882, wrote that the freezing point of water Mishima et al introduced very thin pipettes to collect solution depends on the osmolarity.3 small quantities of tear to avoid irritative stimuli.6 Currently, the concept and importance of tear Gilbard et al introduced an L-shaped micropipette osmolarity in dry eye has been re-introduced by the under femto-biomicroscopy to avoid production International Dry Eye Workshop (DEWS), 20074 QH TGƀGZ J[RGTUETGVKQP VCMKPI XQNWOGU QH VJCVFGſPGFFT[G[GCUCOWNVKHCEVQTKCNFKUGCUGQHVJG microlitre and modifying the technique of storing tears and ocular surface that results in symptoms of the sample. The latest instrument for measuring tear FKUEQOHQTVXKUWCNFKUVWTDCPEGCPFVGCTſNOKPUVCDKNKV[ osmolarimetry is the Ocusense system, which is able with potential damage to the ocular surface. It is to measure tear sample of only 0.2 microlitre.7 CEEQORCPKGFD[KPETGCUGFQUOQNCTKV[QHVGCTſNOCPF Circadian changes in tear osmolarity have also KPƀCOOCVKQPQHQEWNCTUWTHCEG been studied. Mishima et al, in rabbits and Mandell et Ocular surface is a sensitive part of the eye, where al, in humans, observed that the cornea is 4% thicker its constituent components are closely linked together when the eyes are closed than they are in the normal and help in maintainance of adequate homeostasis in awake state, and they assumed this was because VJGTGIKQPKPYJKEJVJGVGCTſNOOWUVOCKPVCKPVJG VGCT ſNO GXCRQTCVKQP YKVJ CP QRGP G[G RTQFWEGU health of the cornea and conjunctival epithelia, and C J[RGTVQPKE ſNO 7PKCEMG GV CN TGRQTVGF VJCV VGCT at the same time contribute to the normal physiology osmolality is found to be low in mid-morning, high of the stroma. The decrease in the production of tears following lunch and throughout the afternoon, and and/or compositional qualitative changes in the tear declines in the evening. Benjamin et al found trends ſNOCUYGNNCUKPETGCUGFGXCRQTCVKQPQHVJGVGCTſNO during day towards hypertonicity. Terry et al found favour the phenomenon of hyperosmolarity. VJCVFWTKPIYCMKPIVJGVGCTſNOKUJ[RGTVQPKEYKVJCP The tonicity of normal tears is mainly determined by osmolarity of 310 mOsm/kg and 285 mOsm/kg during its content of cations (sodium, potassium, calcium, sleep, producing corneal edema on awakening. magnesium, iron and copper) and anions (chlorides, +P$CNKMYCUVJGſTUVVQUWURGEVVJCVVJG bicarbonate and phosphates). The most abundant damage of the ocular surface in sicca syndromes could component is sodium chloride. A fraction of tonicity be related to tear hyperosmolarity, but when he found is produced by macromolecules, such as proteins or PQUKIPKſECPVFKHHGTGPEGUKPVGCT0C%NEQPEGPVTCVKQP sugars. Early measurements of tear osmolarity did not in normal subjects and keratoconjunctivitis sicca directly measure the tonicity, but only expressed the (KCS) patients, he concluded that NaCl did not play content of salts and macromolecules. Thus, Frerichs a role in the pathophysiology of KCS. Mastmam et (1841) reported that tear has 0.94 to 1.3% solids, CNFGOQPUVTCVGFHQTVJGſTUVVKOGVJCVCTGNCVKQPUJKR including salts, albumin, mucus, fats and desquamated existed between osmolarity and dry eye. Mishim et epithelial cells.5 Later, the total tonicity was expressed CNEQPſTOGFVJGVGCTJ[RGTQUOQNCTKV[KPRCVKGPVUYKVJ 5 Role of Tear Osmolarity In Dry Eye DJO Vol. 20, No.4, April-June, 2010
Fig 4: Clinical picture of a patient with dry eye syndrome
(KI 6GCTſNOUVTWEVWTG COQFGTPXKGY
Fig 5: Microphotograph showing squamous metaplasia of epithelial cells on conjunctival impression cytology in a case of severe dry eye
Fig 2: Ocular surface homeostasis in a healthy eye
Fig 6: Rose Bengal stains dead, devitalized epithelial cells and mucus pink in the inter-palpebral area in dry eye syndrome
Fig 3: TearLab Osmolarity System intended to measure Fig 7: Tear break up time: Slit lamp photograph showing the osmolarity of human tears to aid in the diagnosis of DTGCMWRQHVGCTſNOCUGXKFGPEGFD[DNCEMURQVUYJGTG dry eye VJGƀWQTGUEGKPUVCKPGFVGCTſNOJCUTWRVWTGF 6 Role of Tear Osmolarity In Dry Eye DJO Vol. 20, No.4, April-June, 2010 KCS when they found that normal subjects had a tear KPƀCOOCVQT[E[VQMKPGU +.60(CNRJC CPF//2U osmolarity of 289 mOsm/l in the conjunctival fornix //2 YJKEJCTKUGHTQOQTCEVKXCVGKPƀCOOCVQT[ and 304 mOsm/l in the lid margin, while patients cells at the ocular surface. There is evidence that these with KCS had osmolarity of 329mOsm/l. Gilbard et infammatory events lead to apoptotic death of surface CNEQPſTOGFVJGſPFKPIUQH/KUJKOCGVCND[WUKPI epithelial cells, including goblet cells; thus, goblet cell samples of only 0.1-0.4ul . loss may be seen to be directly related to the effects of The designated cut-off or limit between normal EJTQPKEKPƀCOOCVKQP6JGKPETGCUGKPVJGQUOQNCTKV[ osmolarity and hyperosmolarity varies according to QHVJGVGCTſNOKPFT[G[GKUVCMGPCUCUVTGUUTGURQPUG VJGCWVJQTU6JG2KUCETKVGTKCQHFT[G[GFGſPGUOKNF by the ocular surface and this triggers the process of hyperosmolarity as about 320 mOsm/l, moderate as KPƀCOOCVKQP CPF KOOWPQNQIKECN RJGPQOGPC UWEJ 330mOsm/l and severe about 340mOsm/l.8 as the presentation of autoantígenos that enhance the Today it is accepted that there is a relationship KPƀCOOCVQT[RTQEGUU between tear hyperosmolarity and sicca syndrome. 5VWFKGU QH KPƀCOOCVQT[ OCTMGTU UWEJ CU 0( Farris et al has taken it as gold standard in diagnosing Κβ that migrates to the cytoplasm during the sicca syndrome and osmolarity of over 312 mosm/ KPƀCOOCVQT[ RTQEGUU CPF CTG FKTGEVN[ TGNCVGF VQ NJCU-%5CUGPUKVKXKV[QHCPFCURGEKſEKV[QH the phenomenon of hyperosmolarity12. The nuclear 95%.9 Laboratory studies have made it possible to translocation of the NF-Kβ is directly proportional to demonstrate that only a 1 % increase in the osmolarity VJGQUOQNCTKV[QHVGCTſNO$GTTC#GVCNEQORCTGF QHVGCTſNOKUCDNGVQRTQXQMGGRKVJGNKCNNGUKQPUCPF the nuclear translocation NF-Κβ in healthy patients, CNVGTVJGPQTOCNƀQYQHNKSWKFUVQVJGUVTQOC postmenopausal women and patients with Sjögren’s The causes of tear hyperosmolarity in dry eye were syndrome and linked them with the osmolarity of tear summarized by Gilbard,10 as follows: ſNO CPF EQPLWPEVKXCN KORTGUUKQP E[VQNQI[ QH VJGUG 1)Lacrimal gland primarily reduces its secretion ,and patients. Patients of severe dry eye demonstrated increases its salinity; JKIJ XCNWGU QH VJG ſNO QUOQNCTKV[ O1UO 2)lacrimal sea has a dcreased volume , but maintains L) and large nuclear translocation of the factor NF- the same evaporation rate; expressionκ β nd severe squamous metaplasia. NCETKOCN UGC TGFWEGU KVU ƀQY CPF VWTP QXGT DWV The effect of tear hyperosmolarity on the ocular surface maintains a similar evaporation rate; is evident. Massat (1889) wrote that evaporation of 4)physiochemical protectors against evaporation VJG VGCT ſNO RTQXQMGU J[RGTQUOQNCTKV[ YJKEJ ECP decrease. produce epithelial damage and even corneal ulcers, The hyperosmolarity11 causes epithelial injury, CPF VJCV PGTXG VGTOKPCN GPFKPIU ƀQCVKPI KP VJG VGCT complete disappearance of cell surface epithelial ſNO KPFWEG DNKPMKPI VQ TGEQXGT PQTOCN QUOQNCTKV[ layers, decreased cytoplasmic density and Other processes in which hyperosmolarity may play accumulation of mucus cells and secretions leading to a role-directly or indirectly-are alteration of corneo- epithelial desquamation and ocular surface damage. conjunctival epithelium, loss of microplicae, cell This phenomenon is generally evident between 15 membrane disruption, increased Rose-Bengal staining VQ FC[U QH VJG VGCT ſNO QUOQNCT EJCPIG 6JWU and decreased tear break up time, and decreased the hiperosmolarity triggers a series of patho- desquamation. A link between tear instability and physiological mechanisms with clear feedback hyperosmolarity has also been established that may in including effects that in turn enhance each other. VWTPRTQFWEGKPƀCOOCVKQPCPFVTKIIGTKPƀCOOCVQT[ Tear hyperosmolarity is regarded as the central neurons.13 OGEJCPKUO ECWUKPI QEWNCT UWTHCEG KPƀCOOCVKQP Without a doubt, dry eye is currently the most commonly damage, and symptoms, and the initiation of diagnosed clinical condition by ophthalmologists. A compensatory events in dry eye. Tear hyperosmolarity clear and concise knowledge of its cascading patho- arises as a result of water evaporation from the physiology and its early diagnosis, will enable us exposed ocular surface, in situations of a low to manage this condition effectively. In this regard, CSWGQWU VGCT ƀQY QT CU C TGUWNV QH GZEGUUKXG assessing the tear osmolarity these patients would not evaporation, or a combination of these events. Rapid only provide an etiological diagnosis of the disease, VGCT ſNO VJKPPKPI OC[ DG J[RQVJGUK\GF CU C TKUM but also gives us a powerful diagnostic tool for factor for tear hyperosmolarity. Hyperosmolarity evaluation of the disease, with values that are directly UVKOWNCVGU C ECUECFG QH KPƀCOOCVQT[ GXGPVU KP VJG proportional with the severity of the clinical picture epithelial surface cells, involving MAP kinases and of dry eye, and is always present in these patients. NFkB signalling pathways and the generation of
7 Role of Tear Osmolarity In Dry Eye DJO Vol. 20, No.4, April-June, 2010 REFERENCES QH C ſTUV IGPGTCVKQP NCD QP C EJKR PCPQNKVGT VGCT ſNO 1. Galen. (II Century AD). E Iatroz. Book XIV. osmometer. Ocular Surface 2005; 3:S117. 2. Nollett JA(1748). Quoted by McCaig CD, Rajnicek 8. Juan Murube J, Cortes Rodrigo MD. Eye parameters AM, Song B, Zhao M. Controlling cell behaviour for the diagnosis of xerophthalmos. Clin Exp Rheumatol electrically: Current views and future potential. Physiol 1989;7:141-50. Rev 2005;85:943-78. 9. Farris RL. Tear osmolarity. A new gold standard? Adv 3. Raoult FM. Loi de congelation des solutions aqueuses Exp Me Biol 1994; 350: 495-503. de substances organiques. CR Acad Sci Paris 1882;94:1517- )KNDCTF,26GCTſNOQUOQNCTKV[CPFMGTCVQEQPLWPEVKXKVKU 9. (French) UKEECKP*QNN[(, GF 6JGRTGQEWNCTVGCTſNOKPJGCNVJ 4. Dry Eye Working Group (No authors listed) The disease, and contact lens wear. Lubbock, TX, Dry Eye FGſPKVKQP CPF ENCUUKſECVKQP QH FT[ G[G FKUGCUG TGRQTV Institute, 1986:127-39. QH VJG &GſPKVKQP CPF %NCUUKſECVKQP 5WDEQOOKVVGG QH 11. Gilbard JP, Farris RL, Santamaria J. Osmolarity of the International Dry Eye WorkShop (2007).Ocul Surf microvolumes in keratoconjunctivitis sicca tear. Arch 2007;5[2]:75-92. Ophthalmol 1978;96:677-81. 5. Frerichs FT, in Wagner R. Handworter Physiologie 12. Berra A, Berra M. Hyperosmolarity induces nuclear 1846; 3:617. (German) translocation of NF-Κβ Bin human conjunctiva epithelial /KUJKOC 5 -WDQVC < (CTTKU 4. 6JG VGCT ƀQY cells. Invest Opthalmol Vis Sci. 2005; 46. dynamics in normal and in keratoconjunctivitis sicca 13. Liu H, Begley C, Chen M et al. A link between cases. XXI Concilium Ophthalmologic1971: 1801-5. tear instability and hyperosmolarity in dry eye. Invest 7. Sullivan BD, Angeles R, Lemp MA. Clinical results Ophthalmol Vis Sci. 2009;50(8):3671-9
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8 DJO Vol. 20, No.4, April-June, 2010 Major Review Dissociated vertical deviation (DVD)
Shailesh1, Rohit Saxena2, Ravindranath H.M.3 1Consultant, Shekar Nethralaya, Bangalore, 2Associate Prof, Dr R P Center, AIIMS, New Delhi 3Director, Drishti speciality eye Hospital, Davangere
Dissociated vertical deviation (DVD) is a poorly Diagnostic tests: understood eye motility disorder of unexplained When the eye is covered, it elevates and excycloducts; etiology. DVD is a form of cyclovertical deviation upon removal of cover, the elevated eye returns to which is known by various terms like, alternating the midline slowly along with incycloduction. These hypertropia, double hypertropia, dissociated vertical torsional movements can be observed by looking into divergence and alternating sursumduction amongst the conjunctival vessels. QVJGTU$KGNUEJQYUM[RTQXKFGFVJGſTUVEQORTGJGPUKXG Ŗ 6TCPUNWEGPVQEENWFGTVGUV 5RKGNOCPPŏU ſIWTGCE description and clinical analysis of DVD.1 In – updrift of the eye behind the occluder can be seen DVD, either eye elevates when the fellow eye is and its downward slow drifting back is observed after ſZCVKPI .CPECUVGT2 and swan called it alternating removing the occluder. sursumduction, emphasizing the monocular nature of Ŗ )TCFGFFGPUKV[ſNVGTDCT $KGNUEJQYUM[ŏU VGUVŌCU the movement. VJGFGPUKV[QHVJGſNVGTDCTKUKPETGCUGUVJGG[GFTKHVU WRCPFCUVJGFGPUKV[QHVJGſNVGTDCTKUFGETGCUGF Clinical features: the eye comes down. This is called Bielschowsky’s The characteristic feature seen in DVD is the RJGPQOGPQPſIWTG spontaneous drifting of either eye upward when the Ŗ 4GFſNVGTVGUVŌVJKUKUCFKUUQEKCVKPIVGUV6JGG[G patient is tired or after covering one eye. After the DGJKPF VJG ſNVGT FTKHVU WR CPF RCVKGPV CRRTGEKCVGU cover is removed from the elevated eye, it slowly diplopia, with the red image being lower. The amount drifts downward to settle in the primary position. of separation between the images is used to measure Other features of DVD include: the amplitude of elevation of each eye. - Excycloduction of the elevated eye. Quantitative assessment of DVD can be made by +PE[ENQFWEVKQPQHVJGſZCVKPIG[G using base-down prisms, held under the occluder - Sometimes only excycloduction is seen under cover, KP HTQPV QH VJG PQPſZCVKPI G[G WPVKN VJG FQYPYCTF when it is called as Dissociated Torsional Deviation ſZCVKQPOQXGOGPVQHVJCVG[GKUPGWVTCNK\GF (DTD). - Latent nystagmus seen in nearly half the patients - Head posture: anomalous head posture is reported in about 30% of patients.3,4 Anomalous head posture decreases the magnitude of alternating hyperphoria. Chin depression can also be seen. - DVD can occur with overaction of inferior oblique as well as superior oblique muscles. - It is rarely seen in infants, but usually presents at 2-5 years of age. &8&KUWUWCNN[DKNCVGTCNCPFCU[OOGVTKECN7PKNCVGTCN cases are commonly associated with deep amblyopia and sensory heterotropia. - DVD is associated with infantile esotropia/ exotropia, and less commonly with Duane’s retraction syndrome. Figure 1-a No deviation in primary position
9 Dissociated vertical deviation (DVD) DJO Vol. 20, No.4, April-June, 2010
Figure 1-b Left eye DVD- showing elevation of left eye Figure 1-c No DVD in the right eye when the left eye is WPFGTVTCPUNWEGPVQEENWFGTYJGPVJGTKIJVG[GKUſZCVKPI ſZCVKPI Aetiology: Numerous theories have been proposed to explain binocular stimulation9. But it does not account for this intriguing anomaly. Elastic preponderance of the DVD in patients with otherwise normal binocular elevator or the depressor muscles5, paretic factors6 functions. Several investigators have opined that the especially bilateral paresis of the depressor muscles vertical vergence movements must be predominantly and imbalances between the amount of innervation mediated by oblique muscles.10,11 Aetiology of DVD originating from each vestibular organ have been still remains obscure, but Bielschowsky’s explanation proposed earlier. Abnormal visual pathway routing of DVD as a vertical vergence eye movement appears similar to albinism or abnormal, intermittent & alternate more convincing. excitation of subcortical centers could be responsible. Recent investigations also agree with Bielschowsky’s Differential diagnosis: vertical vergence signal theory on DVD.7,8 Spielmann DVD should be differentiated from inferior oblique assumed that DVD is caused by an imbalance of overaction, (Table 1) Table 1 Differences between DVD and IOOA (Inferior oblique overaction) Features DVD IOOA 1) Hypertropia Same in primary position, Maximal in adduction, adduction & abduction never in abduction 2) Recovery movement on uncovering Slow downward drift Quick refixation
3) Superior oblique action May overact Usually underaction
4) V pattern Absent Often present
5) Pseudoparesis of Absent Present contralateral superior rectus
6) Incycloduction on Present Absent refixation
7) Latent nystagmus Often present Absent
8) Bielschowsky’s Often present Absent phenomenon
9) Red filter test Red image is always lower Red image is higher or as eye behind the filter is lower on alternation always higher
10 Dissociated vertical deviation (DVD) DJO Vol. 20, No.4, April-June, 2010 Treatment: Many surgical modalities have been tried References: with varying success rates. 1. Noorden GK Von. Binocular vision and ocular motility • Superior rectus recession – unconventional : theory and management of strabismus. St. Louis : Mosby recession of superior rectus by 7 to 9mm is required Year Book, Inc 2002; 378. in bilateral DVD. Asymmetric cases need differential 2. Lancaster WB. Factors underestimated, features recession12. QXGTGORJCUK\GFCPFEQOOGPVUQPENCUUKſECVKQP+P#NNGP • Retroequatorial myopexy of superior rectus JE, ed: Strabismus Ophthalmic Symposium. St Louis, (Faden operation) – done along with superior rectus Mosby-year book, 1958:430. recession may give better results. 3. Crone RA. Alternating hypertropia. Br J Ophthalmol, • Resection of inferior rectus – is done rarely as a 1954;38:592. single surgery. It is usually done as second surgery 4. Bechtel RT, Kushner BJ, Morton GV. The relationship when superior rectus recession fails. between DVD and head tilts. J Pediatr Ophthalmol • Anterior transpositioning of inferior obliques – is Strabismus. 1996;33:303. popular among few surgeons and effective in cases 5. Schweigger C. Die erfolge der schieloperation. Arch of DVD with inferior oblique overaction.13,14 The Augenheilkd. 30;1895:165. antielevation syndrome which is observed after this 6. Duane A. binocular movements. Arch Ophthalmol. surgery in some cases can be overcome by bunching 1933;9:579. the placement of inferior oblique adjacent to the 7. Bielschowsky A. Disturbances of the vertical motor inferior rectus. muscles of the eyes. Arch Ophthalmol.1938;20:175. Lim15 has described hypotropic DVD, which is 8. Cheeseman EW, Guyton DL. Vertical fusional mostly unilateral and commonly is associated with vergence. The key to dissociated vertical deviation. Arch OQPQEWNCTXKUWCNFGſEKVUQTJKIJO[QRKC#NVJQWIJ Ophthalmol. 1999;117:1188. the nature of the intermittent slow downward ocular 9. Spielmann A. A translucent occluder for studying deviation is similar to that of hypertropic DVD, eye position under unilateral or bilateral cover test. Am it should be considered to be a unique form of the Orthopt J. 1986; 36:65. dissociated strabismus complex. This rare condition 10. Guyton D. Dissociated vertical deviation. An can be corrected surgically by a large recession or a exaggerated normal eye movement used to damp combined recession-resection of the inferior rectus cyclovertical nystagmus. Trans Am Ophthalmol Soc. muscle. 1998;96:389. Fixating eye penalization with 1% atropine has been 11. Rijn LJ, Collewijn H. Eye torsion associated with shown by few authors to reduce DVD magnitude and disparity-induced vertical vergences in humans. Vision decompensatory phases during follow-up. Res. 1994;17:2307. 12. Esswein MB, Noorden GK von, Coburn A. Comparison of surgical methods in the treatment of dissociated vertical deviation. Am J Ophthamol. 1992;113:287. 13. Burke JP, Scott WE, Kutschke PJ. Anterior transposition of the inferior oblique muscle for dissociated vertical deviation. Ophthalmology. 1993;100:245. $KGNUEJQYUM[RJGPGOGPQP 9JGPVJGſZKPIG[GKU 14. Black BC. Results of anterior transposition of the presented with light of decreasing intensity, the eye with inferior oblique muscle in incomitant dissociated vertical DVD falls. deviation. J Am Assoc Pediatr Ophthalmol Strabismus. 1997;1:83. 15. Lim HT. Hypotropic dissociated vertical deviation: a unique form of dissociated strabismus complex. Am J Ophthalmol. 2008;146(6):948-53.
Bilateral DVD. One can use a +4 dioptre lens to unmask it in the absence of a translucent occluder.
11 Dissociated vertical deviation (DVD) DJO Vol. 20, No.4, April-June, 2010
Is this dissociated deviation
Cover ʹ uncover either Slow, tonic refixation eye while watching the movement, frequently eye just uncovered associated hyper deviation and extorsion of dissociated eye
Uncovered eye Uncovered eye moves Dissociated moves ĚŽǁŶ, fellow ĚŽǁŶ, fellow eye may do exodeviation eye moves ƵƉ when same when uncovered uncovered but ŶĞǀĞƌŵŽǀĞƐƵƉ Recess LR
Dissociated Elevation may be the Vertical strabismus vertical deviation same in adduction primary and abduction
Often associated with latent nystagmus Red light always seen Red light ĂůǁĂLJƐbelow ĂďŽǀĞ white light white light regardless Eye excycloducts with one eye fixing which eye fixes when elevated and ďĞůŽǁwhite light with other eye fixing Bielschowsky phenomenon
Refiaxation movement is slow (tonic)
May be latent or manifest
ZĞĚŐůĂƐƐƚĞƐƚ May be associated with A ʹ or V - pattern s Recess SR 7 ʹ 10 mm
Diagnostic and treatment options for dissociated deviations 12 DJO Vol. 20, No.4, April-June, 2010 Major Review MANAGEMENT OF SUPERIOR OBLIQUE PALSY Archana Gupta Mahajan Shroff Charity Eye Hospital, Darya Ganj, New Delhi
For the strabismologist, superior oblique or fourth CONGENITAL nerve (IV N) palsy is the most common1,2 isolated 7PNKMGQVJGTKUQNCVGFPGTXGRCNUKGUVJGOQUVHQWTVJ cranial nerve weakness affecting motility. Possibly nerve palsies are congenital, incidence varies in because of a process of selection others, particularly different studies; out of 190 cases of superior oblique the neuro-ophthalmologist might see VI N palsy more palsy in a study3 by Helveston et al, 137 were often. congenital and 53 were acquired. 7UWCNN[VJGTGKUPQJKUVQT[QHVTCWOCQTVJGRCVKGPV Case : may erroneously date the symptoms to a trivial trauma. A twenty one year old male presented to us with a The symptoms are mostly longstanding and may be history of one month old head trauma. His chief worsening over time due to a decompensation. There complaint was intermittent vertical diplopia which KUCNCTIGJGCFVKNVCPFHCEKCNCU[OOGVT[EQPſTOGFQP got better on tilting his head to the right side. On old family photographs (the family album tomography examination, he had a left hypertropia of twenty prism or “FAT scan”). The face is fuller on the involved diopters (pd) increasing on right gaze and left head side – the ocular torticollis – a hallmark of congenital tilt. Ocular movements showed left Inferior oblique superior oblique palsy. over action and left superior oblique under action. A Characteristic Facial Asymmetry. The more subjective torsion of 8 degrees was measured on double shallow side of the face is always on the side maddox rod test and on indirect ophthalmoscopy, left of the head tilt. Ref :Plager David A. Superior excyclotorsion was seen. A diagnosis of left superior Oblique palsy and Superior Oblique myokymia in oblique palsy was made. Rosenbaum A L, Santiago A P. Clinical strabismus The question to be answered is - how do we proceed Management. Principles and surgical techniques. from here? What are the diagnostic tests required and Philadelphia; W B Saunders Company. 1999; pg what are the management options? Before determining 221. the right treatment strategy, the etiology and the muscle groups involved need to be determined, as well as the scale of discomfort to the patient. That is: • Etiology - Congenital or acquired? • Signs and symptoms – diagnosis and isolation of muscles primarily affected • Investigations • Treatment
ETIOLOGY Etiology is important as among other reasons, identifying a case as congenital helps in avoiding expensive investigations and starting with treatment Although congenital cases do not complain of without delay diplopia, intermittent vertical diplopia may occur in decompensated congenital palsy. There is no • Congenital measurable subjective torsion. • Acquired # .CZ UWRGTKQT QDNKSWG VGPFQP EQPſTOGF D[ VJG – Traumatic superior oblique traction test done at surgery. In case – Vascular of amblyopia and horizontal strabismus absence of – Tumor – very rare the superior oblique tendon should be suspected. – Iatrogenic
13 Management of Superior Oblique Palsy DJO Vol. 20, No.4, April-June, 2010 ACQUIRED EXAMINATION The most common cause of an acquired palsy is • Head tilt to the opposite side and in case of a traumatic. bilateral palsy, chin depression (V pattern) • Cover tests TRAUMATIC • Prism Bar Cover Test in all positions Unilateral Palsy • Ocular movements – versions 7UWCNN[QEEWTUCHVGTCVTKXKCNCEWVGVTCWOCGICYJCEM • Parks 3 step test on the head or a bump against furniture. The patient • Maddox rod – subjective torsion typically complains of incomitant hypertropia and • Indirect ophthalmoscopy- objective torsion vertical diplopia but there is no facial asymmetry • Associated neurological signs The superior oblique traction test is normal at the time of surgery and extorsion is typically demonstrated Cover Tests on the double Maddox rod test less than 104- 151 Are important to demonstrate the presence of degrees. hypertropia and the prism bar cover test should be done to quantify the deviation in all the gazes giving Bilateral Palsy an idea of which gaze is affected the most and thus Occurs after more severe trauma; severe closed head corresponding muscles needing surgery. trauma – associated with a period of unconsciousness. A patients having subjective complaints of torsion should be suspected of having a bilateral palsy; the objective torsion is more than 10 degrees. On examination, there is an alternating hypertropia on head tilt, a V pattern esotropia, chin down head posture and reversing Bielschowsky head tilt test.
Vascular Seen in the elderly age group, vascular palsies have an acute onset of a small angle hypertropia not more than 4-6 diopters. Mostly associated with Hypertension and diabetes, these palsies are self limiting and require a physician referral. Temporary relief can be gained with prisms; these rarely need surgery.
Tumor A rare cause of superior oblique palsy, systemic FKUGCUGKUOQUVN[EQPſTOGFD[VJGVKOGVJGU[ORVQOU of palsy appear; treatment is aimed at the underlying disease and prisms for relief of symptoms.
Latrogenic Now rarely seen, this association was seen quite commonly in the earlier days after superior oblique tenectomy or after ethmoid sinus surgery with trochlear trauma.
History As the details have mostly been covered above, the following points sum the history: • Nature of trauma Figure from: Helveston Eugene M; Superior oblique • Diplopia palsy – Etiology. The Strabismus Minute;Vol 2, no 15 • Head tilt to the opposite side • Any medical problems • Associated neurological signs i.e other cranial nerves palsies. 14 Management of Superior Oblique Palsy DJO Vol. 20, No.4, April-June, 2010 Ocular Movements Important determinant of what to operate, versions are an extremely important part of the diagnosis of UWRGTKQTQDNKSWGRCNU[#UKPVJGſIWTGVJGRCVKGPV has a left superior oblique palsy. Here, the most EJCTCEVGTKUVKE ſPFKPI KU C NGHV KPHGTKQT QDNKSWG +1 over action and to a lesser extent, superior oblique (SO) under action (- sometimes, this is slight or undetectable). The other eye has an apparent superior oblique over action. There is an ipsilateral superior rectus (SR) contracture (as seen in long standing cases).
Once the diagnosis of superior oblique palsy has been made or is suspected, it is necessary to measure the deviation - to quantify the superior oblique palsy and to classify it - then it is possible to arrive at a treatment program DIAGNOSTICS A case of left superior oblique palsy Include • Examination of other cranial nerves • Hess Charting Bielchowsky head tilt test/ Parks 3 step test • MRI scan *GNRUKPEQPſTOKPIVJGFKCIPQUKUD[VJTGGUVGRUKG Is it a left or right hypertropia? MRI Worsens in dextro or levoversion Most patients with isolated SO palsy do not need Worsens in left or right head tilt an extensive neurological workup. As most palsies The Bielchowsky head tilt test is considered positive are congenital, the characteristic facial asymmetry, for superior oblique palsy when the vertical deviation a review of old family photographs showing a head increases with the head tilted towards the higher tilt (the FAT scan) or eliciting symptoms of a long side. If the head tilt test reverses, a bilateral superior FWTCVKQP KU UWHſEKGPV VQ TWNG QWV CP CEWVG CESWKTGF oblique palsy is suspected. process. Neuroimaging may however be required in cases of: Double Maddox Rod test – Fresh traumatic The details of the procedure of this test are not – Acquired palsies not directly linked to trauma discussed here. The interpretation: – Non isolated palsies • Subjective torsion seen only in acquired palsy. Ŗ FGITGG VQTUKQP UGGP KP WPKNCVGTCN VTCWOCVKE LOCALISATION palsy – patient does not complain, seen only on The fourth nerve nucleus is in the rostral part of the dissociation. OKF DTCKP KP VJG VGEVWO 6JG PGTXG ſDTGU QTKIKPCVG • > 10 degree torsion seen in bilateral palsies, from the dorsal part of the brain stem and decussate. patient complains of tilting. &GNKECVGPGTXGſDTKNUVJGPRCUUVJTQWIJVJGVGPVQTKWO CPF RCUU KPVQ VJG UWRGTKQT QTDKVCN ſUUWTG VQ UWRRN[ Indirect ophthalmoscopy VJG UWRGTKQT QDNKSWG OWUENG 6JGUG FGNKECVG ſDTKNU Torsion can be seen objectively on examination with are vulnerable to to and fro movements of the brain an indirect ophthalmoscope. If the macula is rotated during sudden deceleration or violent head trauma. downwards or clockwise in the left eye or counter clockwise in the right eye, so the macula is below TREATMENT CNKPGFTCYPRCTCNNGNVQVJGQTDKVƀQQTCPFVGORQTCN 5WRGTKQTQDNKSWGRCNU[UGGPCUCPKPEKFGPVCNſPFKPI from the lower disc margin, torsion can be inferred. in a patient without torticollis or symptoms does not require any treatment. 15 Management of Superior Oblique Palsy DJO Vol. 20, No.4, April-June, 2010 Prisms have a limited role in the management of Knapp IV superior oblique palsy and their role is limited to Spread of hyper deviation • Very small comitant deviations, usually vascular ‘across the bottom’: • Non surgical candidates. tightness Most patients presenting to the ophthalmologist with of the ipsilateral SR Surgery SO palsy do so because of troubling symptoms and RFYGCMGPVJG+1 are usually surgical candidates. >20 pd add SO tuck if tendon lax Or contralateral KNAPPS CLASSIFICATION IR weaken -PCRR KP JKU NCPFOCTM RCRGT QP ENCUUKſECVKQP QH superior oblique palsy emphasized the importance Knapp V of the relative magnitude of hyperdeviation in the Hyper deviation ‘across XCTKQWUſGNFUQHIC\G#NVJQWIJOCP[QHVJGVTGCVOGPV the bottom’ - long-standing recommendations may not be followed at this point acquired QHVKOGVJGKORQTVCPEGQHJKUENCUUKſECVKQPNKGUKPVJG Surgery basic message that the muscle to be treated should RF54TGE 51VWEM+1TGE%.+4TGE be matched to the gaze in which the deviation is the Contralateral SO WEAKENING as originally maximum. To summarize, recommended by Knapp is a STRICT NO Ŗ %NCUUKſGF51RCNU[KPVQITQWRUFGRGPFKPIQP gaze primarily affected Knapp VI • Treatment paradigm based on the main muscle Bilateral superior oblique palsy involved • Table enumerates Knapps recommendations Table 1
Knapp I • Overaction of Antagonist IO • surgery: Weaken antagonist IO
Knapp VII Brown syndrome with superior oblique underaction Knapp II (‘CANINE TOOTH’) Ŗ 7PFGTCEVKQPQHVJGRCTGVKE SO with the deviation greater KPKVUVJGſGNFQHCEVKQP • Surgery : SO tuck, IO weakening, or yoke Inferior Rectus (IR) Recession
Surgery: • None -- if eyes are aligned around primary Knapp III • Yoke IR Recession -- if ipsilateral hypertropia • Deviation equal in the • Take down tuck, if caused by a ‘too tight’ tuck ſGNFQHVJGRCTGVKE51CPF • Free SO restriction if ipsilateral hypotropia the antagonist IO • Surgery: Present day treatment plan incorporates most of these Ŗ RF 9GCMGPVJG+1 criteria plus adds • >20 pd add SO tuck if Ō 5KIPKſECPEGQHUWRGTKQTTGEVWUEQPVTCEVWTG tendon lax – Superior oblique laxity • Or contralateral IR Ō 3WCPVKſECVKQPQHUWRGTKQTQDNKSWGNCZKV[ weakening – Management of torsion
16 Management of Superior Oblique Palsy DJO Vol. 20, No.4, April-June, 2010 Oblique traction test This test is more valuable in children; the vigorous Intra operative testing of the superior oblique is maneuver may easily tear the fragile conjunctiva in an indispensable part of evaluating patients with adults. superior oblique palsy. Traction testing is important in identifying not only the lax tendons that need to be TREATMENT ALGORITHM tucked but also the normal length tendons that cannot Although most surgeons follow the rule to operate be tucked. in the muscle involved in the position of maximum deviation, the following is a useful algorithm to Technique determine which muscle to operate. Step 1: Surgeon seated above the head of the supine, BILATERAL PALSY anaesthetized patient Management is controversial. Some suggested Two toothed forceps taken procedures: Limbus grasped at 2 and 8 o clock position in the left • BE Yoke IO weakening eye and the 4 and 10 o clock position in the right eye • BE IR weakening • Harada Ito procedure Step 2: • BE Down shift of the MR for V Eye is rotated up into an elevated, adducted position in the superior nasal quadrant while simultaneously References pushing the globe down towards the orbital apex 1. Helveston Eugene M; Superior oblique palsy – Etiology. The Strabismus Minute; Vol 2, no 15 Step 3: 2. Von Noorden G K, Campos E C. Binocular Vision Once the tendon is put on stretch, the eye is moved and ocular motility; theory and back and forth (temporally and nasally) while management of strabismus. 6; Mosby: 312-320 maintaining the tendon taut 3. Surgical treatment of superior oblique palsy.E M Helveston, J S Mora, S N Lipsky, D A Plager, F D Ellis, Step 4: D T Sprunger, and N Sondhi. Trans Am Ophthalmol Soc. Step 1 to 3 are repeated in the fellow eye 1996; 94: 315–334. The relative laxity of the tendon should be compared 4. Rosenbaum A L, Santiago A P. Clinical strabismus with the other eye. A subjective grading scale is also Management. Principles and surgical techniques. available. Philadelphia; W B Saunders Company. 1999; pg 221.
17 Management of Superior Oblique Palsy DJO Vol. 20, No.4, April-June, 2010 SUPERIOR OBLIQUE PASLSY MANAGEMENT
TRACTION TEST
Absent Tendon Markedly Lax Every one Else (Including Acquired Unilateral)
1) RECESS SR 1) TUCK SO TO MATCH INFERIOR OBLIQUE 2) WEAKEN IO ͚EKZD>͛^/ OVERACTION 2) WEAKEN IO
Yes No
,dфͬсϭϱW ,dфͬсϭϱW
Yes No Yes No
WEAKEN IO SR RESTRICTION? CONTRALAT CONTRALAT IR RECESS IR RECESS IPSILAT SR RECESS
Yes No
1) RECESS SR 1) RECESS IR (CONTRALAT) 2) WEAKEN IO 2) WEAKEN IO
18 DJO Vol. 20, No.4, April-June, 2010 Major Review Management of Corneal Trauma
Prakashchand Agarwal, Namrata Sharma, Rajesh Sinha Dr. R. P. Centre, AIIMS, New Delhi
Introduction Principles of management of ocular injury: Trauma to the eye can have various components. It 1. Rule out life threatening injury includes trauma to extraocular structures and globe 2. History and its intraocular contents. Extraocular injury 3. Examination includes injury to lids, extraocular muscles, orbital 4. Investigation OCTIKPQTDKVCNƀQQTHCEGQTOQTGUGTKQWUKPVTCETCPKCN 5. Treatment injury. Injury to globe includes injury to cornea, sclera, crystalline lens, or the posterior segment. Discussion 1. Rule out life threatening injury to brain, cardio- in this article is limited to corneal and corneo-scleral respiratory system and check vital parameters: lacerations. Ocular injury may be associated with intracranial injury. Examine the systemic condition by Glassgow A corneo-scleral laceration may be partial- or full- Coma Scale assessment, Neurological assessment thickness injury. A partial-thickness injury does not and CT scan. violate the globe of the eye (abrasion) and may or may 2. Patient presenting with acute symptoms may give not require surgical repair. A full-thickness injury history of preceding injury. Some patients have penetrates completely through the cornea, causing insidious injury and later present with secondary a ruptured globe.1 It may also be associated with lid infections or complications. Small foreign bodies, laceration (Figure 1). digital trauma, or other more subtle sources of damage may not be quickly recalled by the patient. Document information such as the setting of the injury, changes in vision, or sensation of a foreign body in the eye. Foreign body should be suspected and ruled out. Even blunt objects can cause corneal lacerations. All trauma cases are to be treated as medico-legal cases, hence all precautions such as consent, clinical photographs, X rays should be done. Obtain the patient’s pre-injury vision assessments as well as any history of previous ocular problems or ocular surgery. History of old injury in the same or other eye may be important. Patients Figure 1: Globe rupture with lid laceration usually complain of decrease in vision, watering, photophobia, bleeding and pain. Document pertinent Epidemiology medical history, current medications, allergies, and International epidemiology of penetrating eye injury tetanus immunization status. Determine information proves that most people susceptible to trauma are regarding the patient’s last oral intake if operative males, between the ages of 20 and 30 years, associated intervention is anticipated. with alcohol ingestion, injury occurring at work, home, FWTKPIſIJVCPFTQCFVTCHſECEEKFGPV$GCYCTGQHCP[ Examination local activities or traditions that may also predispose It is important to examine both the eyes. Sometimes the local population to corneal injury like bow arrow only the symptomatic eye is given due consideration. KPLWT[FWTKPI&JWUUGTCQTſTGETCEMGTKPLWT[ The physician must be meticulous in examining Cause of injury could be metal fragments, sharp the cornea and periorbital structures if there is QDLGEVUſPIGTPCKNUCKTDCIFGRNQ[OGPVGZRNQUKQPU suspicion of a corneal laceration. Typically, patients blunt force trauma and pellets.2,3 who present with this type of injury complain of an
19 Management of Corneal Trauma DJO Vol. 20, No.4, April-June, 2010 intensely painful, profusely lacrimating eye. The chamber, especially if the patient’s history suggests bulbar conjunctiva will be injected with prominent that the corneal laceration is from a small, high-speed blood vessels. object (such as from hammering metal).
6JGſTUVRTKQTKV[KPGXCNWCVKPICEQTPGCNKPLWT[KUVQ • Full-thickness corneal lacerations include or exclude a full-thickness injury and the o Do not apply pressure to the globe. resulting ruptured globe. A full-thickness injury will o Immediately place a protective shield (not a patch) allow aqueous humor to escape the anterior chamber, over the affected eye. YJKEJECPTGUWNVKPCƀCVCRRGCTKPIEQTPGCCKTDWDDNGU o Instruct the patient to avoid ocular movement under the cornea, or an asymmetric pupil secondary because extraocular muscle contraction can cause to the iris protruding through the corneal defect. extrusion of intraocular contents. o Full-thickness corneal lacerations often result in a Complete ocular examination including vision, lids, NQUU QH CSWGQWU JWOQT RTQFWEKPI C UJCNNQY QT ƀCV GZVTCQEWNCT OQXGOGPVU NKIJV TGƀGZ UYKPIKPI NKIJV anterior chamber. test, lens clarity, anterior chamber depth, intraocular o Prolapse or incarceration of the iris may produce a pressure (not in obvious globe perforation), distant teardrop distortion of the pupil. FKTGEVTGƀGZXKVTGQTGVKPCNGZCOKPCVKQPOWUVDGFQPG o Hyphema, or blood in the anterior chamber, can be another sign of anterior chamber penetration. Assess Vision Hyphema may also be seen with blunt, nonpenetrating Assessment of visual acuity must be performed, taking trauma. (Figure 2) care not to apply additional pressure to the globe. • If possible, determine visual acuity prior to examination or treatment, and separately test each eye with corrective lenses. Pinhole testing may help differentiate refractive error from uncorrectable vision when spectacles are not available. Ŗ 7UGCIGCRRTQRTKCVGXKUKQPVGUVKPIFGXKEGUUWEJCU the Snellen distance chart or a hand-held vision card. • Ask the patient to identify typed letters, the clock, or objects on the wall if the patient’s condition prevents formal (standing upright) testing. • If a patient’s vision is severely limited, determine YJGVJGT VJG RCVKGPV ECP EQWPV ſPIGTU FGVGEV movement, or identify light. Figure 2: Hyphaema
Slit lamp examination Once a diagnosis of corneal laceration is made, one • Examine the cornea using a slit beam to search must also consider the simultaneous presence of the for anterior chamber penetration. A shallow anterior following: chamber, irregularly shaped pupil (teardrop shape), hyphema (blood in the anterior chamber), bubbles in 1. Corneal foreign body VJGCPVGTKQTEJCODGTQTCƀCVEQTPGCECPDGUKIPUQH 2. Endophthalmitis corneal perforation. 3. Intraocular foreign body • Aqueous humor leaking from the anterior chamber 4. Posterior chamber injury ECPDGKFGPVKſGFD[RGTHQTOKPIC5GKFGNVGUV6JKUVGUV 5. Retinal injury KURGTHQTOGFD[FKTGEVN[CRRN[KPIƀWQTGUEGKPVQVJG 6. Orbital injury suspected corneal lesion. Visualization of diluted dye 7. Lens injury under a black light (a positive test) suggests a leak. # PGICVKXG 5GKFGN VGUV PQ FKNWVKQP QH ƀWQTGUEGKP Investigations suggests a partial-thickness injury but may be seen Laboratory Studies in small or spontaneously sealing lesions. One should • No lab studies are useful for detecting the presence avoid the temptation to press on the globe to test for a of a corneal laceration. self-sealing injury. Imaging Studies • Be sure to evaluate for a foreign body in the anterior • Radiography, CT, or MRI may be indicated to locate
20 Management of Corneal Trauma DJO Vol. 20, No.4, April-June, 2010 Antibiotics intraocular or intraorbital foreign bodies or associated Cefazolin: 500 -1000 mg IV q8h; (not to exceed 1 orbital, cranial, or facial trauma. MRI may be of some g q6h); Pediatric : 25-50 mg/kg/d IV divided TDS/ use if there is suspicion of an organic foreign body QID. (eg, wood, plant matter). However, avoid MRI if Clindamycin: 600-1200 mg/d IV divided BD/QID there is any possibility of a metallic foreign body in (not to exceed 4.8 g/d), Pediatric: 20-40 mg/kg/d IV VJGG[G7NVTCUQWPFDKQOKETQUEQR[OC[DGTGSWKTGF divided bid/qid to detect foreign body impacted in the ciliary body or 4,5 Vancomycin: Adult:2 g/d IV divided BD/QID, iris root. Pediatric: 30-40 mg/kg/d IV divided BD/TDS
Treatment Techniques of repair of Corneal Laceration: Prehospital Care Principles of repair: • Cover the patient’s eye with an eye shield or Surgical re-approximation of the ocular tissues. polystyrene/paper cup and avoid any pressure to the Re-obtain ocular integrity globe. Avoid surgical damage to lens and iris • Instruct the patient to move the eyes as little as Minimal residual astigmatism possible. • Administer tetanus toxoid injection, antiemetic and a. Lamellar injury; analgesic medication in order to reduce pressure on 'ZCOKPGEQORNGVGN[WPFGTJKIJUNKVNCOROCIPKſECVKQP the globe. 2GTHQTO 5KGFGNŏU VGUV YKVJ ƀWQTGUEGKP F[G CPF Emergency Department Care apply gentle pressure if required. Perform an examination to ascertain the extent of the Management options include: pressure patching, corneal, anterior chamber, ocular, and associated (eg, bandage contact lens, tissue adhesives6 and suturing facial, cranial) injuries. may be required in children, unstable deep wounds. • Place a protective eye shield (prefabricated or custom made) on the injured eye. This can be a commercial b. Full thickness injury; plastic eye shield or simply a polystyrene/paper cup CorneoScleral injury: taped over the eye. 6JGNKODWUUJQWNFDGUWVWTGFſTUVVQQDVCKPCNKIPOGPV • Administer antiemetics and systemic analgesic Then cornea and later the scleral is sutured with medication. exploration of the rest of globe. Scleral should be • Tetanus immunization or booster is indicated. sutured with 6-0 vicryl and conjunctiva with 8-0 • Consider use of antibiotics including route (topical vicryl sutures. 7,8 or intravenously) and frequency. • In general, topical analgesia and antibiotics should Corneal injury: DGCXQKFGFKHCEQTPGCNNCEGTCVKQPKUUWURGEVGF7UG Simple corneal full thickness injury may be tackled systemic analgesia and antibiotics. Topical anesthetics with bandage contact lens, tissue adhesives6 or may be used, if needed, to facilitate visual acuity suturing. testing and the slit lamp examination.
Medication Recommendations include a combination of a cephalosporin (eg, cefazolin) or vancomycin and an aminoglycoside (eg, gentamicin). In addition, add clindamycin if an intraocular foreign body is present or if vegetable matter has contaminated the wound. The OQUVEQOOQPQTICPKUOUKFGPVKſGFKPRQUVVTCWOCVKE endophthalmitis are Staphylococcus epidermidis, bacilli species, streptococci species, and gram- negative species. Fungal endophthalmitis is a relatively rare entity but should be considered in a patient who is recently post-surgical, immunocompromised, unresponsive to antibiotic treatment, or has a history of trauma with vegetable matter. Figure 3: Tri-radiate corneal laceration
21 Management of Corneal Trauma DJO Vol. 20, No.4, April-June, 2010 #NNEQTPGCNNCEGTCVKQPUKPFWEGƀCVVGPKPIQHEQTPGCCPF and no-touch technique of suturing should be used. its repair would lead to astigmatism. Cornea incision (Figure 5b) UJQWNFDGENQUGFYKVJOQPQſNCOGPVP[NQP7,8 All suture knots should be buried After suturing wound leak should be checked. Following factors need to be considered: Compression zone: Each suture produces a zone of compression which is triangular in shape; on either sides of the suture. The zones of compression of subsequent suture should overlap each other. The zone of compression is roughly equal to the length to the suture. Longer and deeper sutures appose deeper stroma, closing any posterior gaping and decrease the propensity for iris or vitreous to adhere to posterior corneal surface. Ideal length of suture should be approx 1.5 mm. Eversion/Inversion effects: For a single interrupted suture, inverting and everting forces are equal, so there is no tendency toward tissue depression or Figure 5: Suturing Technique elevation. A non-radial interrupted suture produces tissue torque. Corneal injury with iris incarceration: Suturing should prevent over-riding of tissues, Attempt should be made to reposit and retain the iris minimize torque and residual astigmatism and obtain tissue wherever possible without damaging it further water tight closure. ſIWTG #P[FGXKVCNK\GFKPHGEVGFVKUUWGUJQWNFDG The corneal laceration is considered as a sum up of excised by gently grasping the iris tissue. No pull multiple perpendicular segments. Each laceration UJQWNFDGCRRNKGF6JGKTKUKUEWVƀWUJYKVJVJG8CPPCU segment needs to be sutured. The suture needle scissors and the cornea sutured accordingly. 7,8 exit and entry sites should be equidistant from the incision. It is important to place the suture at equal depth on both sides of the laceration, approximately 90% depth (Figure 4).
Figure 6: Corneal laceration with iris prolapsed Corneal injury with Vitreous incarceration: Figure 4: Repaired corneal perforation Any vitreous strand should be cut with manual or automated vitrectomy cutter. The anterior chamber Order of suture placement: and wound should be free from any vitreous. Vitreous 2GTKRJGTCN UWVWTG UJQWNF DG RNCEGF ſTUV VQ ƀCVVGP in wound can cause infection, vitreous traction, the peripheral cornea and steepen the central cornea. Cystoid macular edema, Retinal detachment. (Figure 5a) The central cornea is then closed with a gentle Intraoperative bleeding: CRRQUKVKQPCNUWVWTGLWUVUWHſEKGPVVQENQUGVJGCPVGTKQT 7UG QH XKUEQGNCUVKE GRKPGRJTKPG ECWVGT[ QT surface. thrombin may help decrease bleeding. At the central area, sutures bites should be shorter
22 Management of Corneal Trauma DJO Vol. 20, No.4, April-June, 2010 Corneal injury with Lens involvement: References: Cataract extraction along with primary repair should 1.Hamill MB.Corneal and scleral trauma. Ophthalmol DG CXQKFGF 2TQDNGOU GPEQWPVGTGF CTG FKHſEWNV Clin North Am. 2002 Jun;15(2):185-94. preop assessment, biometry measurement and poor 2.Woo JH, Sundar G. Eye injuries in Singapore--don’t visualization. Second stage cataract surgery gives risk it. Do more. A prospective study. Ann Acad Med optimal results. If anterior capsule is breached and Singapore. 2006 Oct;35(10):706-18. lens matter is present in anterior chamber, it should 3.Segev F, Assia EI, Harizman N, Barequet I, Almer Z, be removed.9 Raz J, Moisseiev J. Corneal laceration by sharp objects in children seven years of age and younger. Cornea. 2007 Post operative management: Apr;26(3):319-23. Analgesics, topical and systemic antibiotics, 4.Arey ML, Mootha VV, Whittemore AR, Chason DP, cycloplegics, oral and topical steroids are given in Blomquist PH. Computed tomography in the diagnosis post operative period. of occult open-globe injuries. Ophthalmology. 2007 Aug;114(8):1448-52. Follow-up 5.Kaushik S, Ichhpujani P, Ramasubramanian A, • Follow-up care is determined at the time of the Pandav SS. Occult intraocular foreign body: ultrasound initial ophthalmologic consultation and will take biomicroscopy holds the key. Int Ophthalmol. 2008 place in the setting of the patient’s overall condition. Feb;28(1):71-3. • An isolated partial-thickness corneal laceration may 6.Velazquez AJ, Carnahan MA, Kristinsson J, Stinnett be monitored on an outpatient basis. S, Grinstaff MW, Kim T. New dendritic adhesives for • A patient who sustains a corneal laceration as part sutureless ophthalmic surgical procedures: in vitro studies of other trauma may have to be evaluated in the of corneal laceration repair. Arch Ophthalmol. 2004 hospital. Jun;122(6):867-70. 7.Maguire CJ. Suturing techniques in corneal surgical Deterrence/Prevention incisions and traumatic lacerations. Adv Ophthalmol. • Patients that engage in activities that place their 1978;37:109-14. eyes at risk for trauma should be encouraged to wear 8.Akkin C, Kayikcioglu O, Erakgun T. A novel suture protective eyewear at all times. technique in stellate corneal lacerations. Ophthalmic Surg Lasers. 2001 Sep-Oct;32(5):436-7. Complications 9.Muga R, Maul E. The management of lens damage in • Corneal lacerations frequently are complicated perforating corneal lacerations. Br J Ophthalmol. 1978 by corneal or intraocular foreign bodies, infections, Nov;62(11):784-7. traumatic cataracts, and secondary glaucoma.
23 DJO Vol. 20, No.4, April-June, 2010 Major Review ‘A’ AND ‘V’ PATTERN STRABISMUS Jaspreet Sukhija, Balwinder Kaur, Zoram Zadeng Department of Ophthalmology Post Graduate Institute of Medical Education and Research Chandigarh Introduction Pattern strabismus is vertically inconcomitant strabismus, recognized by change in the degree of horizontal deviation in upgaze and downgaze. This entity has been recognized UKPEGNQPIVKOGDWVOWEJUKIPKſECPEGYCUPQVIKXGPYJKEJTGUWNVGFKPRQQTUWTIKECNQWVEQOGKP such cases. Due to complex and varied presentation, surgical management of this phenomenon is rather complex. Definition more common followed in order of frequency by A In A-V pattern strabismus the angle of horizontal esotropia, V exotropia and A exotropia.2 UVTCDKUOWU XCTKGU UKIPKſECPVN[ YJKNG EJCPIKPI VJG ETIOPATHOGENESIS: gaze from elevation to depression or vice versa. • A pattern - horizontal strabismus (esotropia or exotropia) whose angle changes more than 10 diopters between 30 degrees upward and 30 degrees downward gaze has an. • V pattern - the variation has to be more than 15 Table showing distribution of pattern strabismus1 PD when the gaze is changed from depression to elevation. Various etiologies have been proposed but there is lack of consensus among the authors. The presumed etiopathogenesis is due to
1. Oblique muscle dysfunction Torsional imbalance as a result of sagitallization of oblique muscles leading to secondary overaction or underaction of the corresponding cyclovertical muscles. This is the most widely accepted theory 3,4. Overaction of inferior obliques can cause a V pattern by increasing its abduction action in elevation and conversely superior oblique overaction can cause A pattern .
2. Horizontal muscle dysfunction: anomalous insertion of horizontal recti, medial recti A) A pattern esotropia showing increase in angle in higher and lateral recti lower than normal, causing V upgaze pattern and reverse in A pattern have been reported 5,6. B) A pattern exotropia wherby the Xt increases on For example underaction of both lateral recti leads to downgaze a weakening of divergence on looking up, causing A C) V pattern esotropia with increase in angle on pattern esotropia and whereas overaction of the lateral downgaze recti causes V pattern esotropia. D) V pattern exotropia showing more XT in upgaze 3. Vertical muscle dysfunction: Prevalance: primary anomalies in the function of the vertical Due to absence of uniform criteria there is wide muscles has also been proposed. The tertiary action variation in the incidence reported. The incidence of the vertical recti is adduction. If superior rectus is reported is 12.5 % - 87.7%.1 V esotropia pattern is underacting then in upgaze then adduction will also be diminished resulting in divergence in upgaze. 24 ‘A’ And ‘V’ Pattern Strabismus DJO Vol. 20, No.4, April-June, 2010 4. Anatomical anomalies of orbits, the angle of deviation) from direct elevation (25-30 e.g., mongoloid or antimongoloid features and torsion degrees) to direct depression (25-30 degrees) must be of orbits taken. • Actions of the extraocular muscles should be 5. Anomalies of muscle insertions investigated thoroughly through examination of ocular motility so that an overaction or underaction of 6. Anomalies of muscle pulley a muscle is detected. Success of surgery may depend QPVJKU7PNGUUVJGUGRTQDNGOUCTGEQTTGEVGFTGURQPUG EXAMINATION: to treatment may not be as anticipated Symptoms • The results of the surgery may also be unpredictable Asthenopia and diplopia are the usual complaints. because of anomalies of the muscle pulleys and The increase in deviation in looking down is tolerated abnormalities of the orbits. well as it does not interfere with the routine functions • Oblique muscles should only be operated on if they like reading or other near work. The increase in the UJQYCUKIPKſECPVFGITGGQHF[UHWPEVKQP WPFGTCEVKQP deviation in downgaze may cause visual discomfort. or overaction). • Vertical muscles should only be selected for Work up surgery if other options like surgery on horizontal or The usual assessment routine to any case having oblique muscles have failed or are contraindicated problems of ocular motility to be carried out in all the Ŗ +P VJG CDUGPEG QH UKIPKſECPV F[UHWPEVKQP QH cases. Angle of deviation should be measured in at QDNKSWG OWUENGU UWTIGT[ UJQWNF DG EQPſPGF VQ VJG least 3 vertical directions as follows: horizontal recti (vertical displacement with recession / resection according to the need). 1) In 30 degrees elevation • The action of a rectus is weakened in the direction 2) In primary position (PP) it’s insertion is transposed, e.g., if the insertion of 3) In 30 degrees depression medial rectus is displaced downwards as is required in V pattern strabismus, adduction is weakened in MANAGEMENT: depression Management includes treatment • Medial rectus insertion is always transposed of refractive error and amblyopia if present. Surgical towards the apex of A or V to abolish the convergence management is done when indicated. The indications of the arms of the letters. for the surgery are • Lateral rectus insertion is always displaced 1. Deviation manifesting 50 % of time or more and/ towards the base of A or V to straighten the divergent or arms of the letters. 2. Presence of sensory problems, e.g., suppression, • As regards the surgery on oblique muscles it should defective binocular functions be kept in mind that weakening of an overacting oblique 3. When it poses a cosmetic problem muscle gives better results than does strengthening of 4. Requirement of normal binocular functions and an underacting oblique muscle. ocular motility for certain professions • While planning surgery of multiple muscles specially recti one should remember that there is a Aims of surgery: possibility of anterior segment ischemia. Oblique (a) To achieve orthotropia in more practical and muscles do not contribute to the circulation of the useful positions of gaze, i.e., in primary position and anterior segment. infraversion (looking directly downwards). The latter • Moderate degrees of A or V patterns require only is the position in which most of the daily activities symmetrical surgery on the obliques or surgery on like walking, eating, reading and writing etc. are horizontal recti with vertical repositioning of their performed. insertions as mentioned above. (b) To obtain comfortable binocular single vision • If the degree of A or V pattern is more than 30 (also in primary position and looking down). PD 4-6 muscles may have to be operated on but it (c) To correct abnormal (compensatory) head posture. is wiser not to do too much at one sitting as there This will automatically result if above two have been are so many factors affecting the outcome. And a achieved. UKIPKſECPVQXGTEQTTGEVKQPNGCFKPIVQFKRNQRKCKUSWKVG Planning the line of management of pattern strabismus an uncomfortable condition. • Exact measurements of the pattern (the change in • As far as possible both horizontal and vertical
25 ‘A’ And ‘V’ Pattern Strabismus DJO Vol. 20, No.4, April-June, 2010 deviations should be corrected in one sitting. Choice of surgical procedures Surgical treatment on horizontal recti, vertical recti and oblique muscles has been advocated according to the muscle involved. (1) Horizontal muscle surgery with vertical displacement of insertions and /or slanting the insertion of horizontal recti 1DNKSWG OWUENG UWTIGT[ KP ECUGU QH UKIPKſECPV oblique muscle dysfunction, e.g., inferior oblique overaction in V-pattern and superior oblique overaction in A-pattern. Surgery of horizontal recti in the form of recession and resection procedures along with their upward or downward displacement. 7-10 Whenever the oblique muscle dysfunction is incriminated11,12 oblique muscles are operated. Vertical recti have also been operated upon to correct this pattern.13
1) HORIZONTAL MUSCLE SURGERY : combined with vertical transposition of their insertion. The Principle of the surgery is to weakened the action Algorithm for management of A pattern strabismus of the muscle in the direction in which its insertion (From Von Noorden) is shifted. Eg : if the insertion of the lateral rectus is shifted downwards, its action i.e abduction will be weakened in downgaze. V- pattern : lateral rectus insertion shifted upwards and medial rectus insertion downwards A- pattern : medial rectus insertion shifted upward and lateral rectus insertion downward. The amount of shift depends on the difference in the angle of deviation in midline vertical gaze. But usually a moderate amount of shift i.e half the length of the muscle insertion is done.
• Slanting the newly transposed muscle insertion for an added effect in case of a larger pattern so that Algorithm for management of v pattern strabismus (From selective weakening can be achieved in elevation or Von Noorden) depression as required. eg,. A pattern ET- The medial rectus has to be weakened more in up-gaze than in 2) OBLIQUE MUSCLE SURGERY : done in down-gaze to abolish the convergence of the arms of adjunct to the horizontal muscle recession or resection A. This can be achieved by transposing its insertion surgery as indicated. It should be done only when upwards and slanting the new insertion so that there is a presence of oblique muscle overaction its upper end is further away from limbus than the V- pattern : weakening of overacting inferior oblique lower end. Putting the upper end further back further muscle weakens the adduction action of the medial rectus in A- pattern : weakening of overacting superior oblique elevation. muscle
26 ‘A’ And ‘V’ Pattern Strabismus DJO Vol. 20, No.4, April-June, 2010 Inferior Oblique weakening can be done by : • Myectomy –corrects about 20 prism dioptres of pattern • Recession of the muscle insertion depending upon the amount of vertical incomitance • Anterior transposition if assosciated with DVD Superior Oblique weakening • Posterior tenectomy of the superior oblique(PTSO) for mild overaction (15 prism dioptres) • Tenectomy for moderate overaction (20 prism dioptres) • Translational recession for 25 prism dioptres of A pattern
COMPLICATIONS OF A-V PATTERN STRABISMUS SURGERY : 1) Iatrogenic vertical and torsional strabismus can occur when a normal oblique muscle is operated upon 2) Vertical and torsional diplopia 3) Superior oblique palsy 4) Consecutive overaction of the direct antagonist muscle 5) Compensatory head posture following the above complications 6) Anterior segment ischemia following vertical recti surgery 7) Persistence of oblique muscle overaction
27 ‘A’ And ‘V’ Pattern Strabismus DJO Vol. 20, No.4, April-June, 2010 SUMMARY : Treatment of pattern strabismus is more complicated CPFFKHſEWNVVJCPVJGJQTK\QPVCNEQOKVCPVUVTCDKUOWU The aim of the surgery is to reduce the pattern and to achieve fusion in primary and downgazes. The results of surgery are rather unpredictable, especially in case of combined surgery ( recti and oblique). Hence, careful planning of surgery is to be done , most important is to determine preoperatively whether there is an abnormality in the action of the oblique muscles.
References: 1. Costenbader FD . Symposium : The A and V patterns in strabismus . Trans Am Acad Ophthalmol otolaryngol ; 1964; 68:354 2. Breinin G. The pathophysiology of A and V patterns. In symposium . The A and V patterns in strabismus. Trans Am Acad Ophthalmol otolaryngol; 1964; 68:354 3. Bagolini B, Campos E and Chiesi C. Plagiocephaly ECWUKPIUWRGTKQTQDNKSWGFGſEKGPE[CPFQEWNCTVQTVKEQNNKU Arch Ophthalmol.1982 ; 100 : 1093-1096. 4. France T. Strabismus in hydrocephalus . Am Orthopt J 1975 ; 25: 101-105 . 5. Postic G. Etiopthogenie des syndromes A. et V. Bull Mem Soc. Fr. Ophthalmol 1965; 78 : 240 6. Nakamura T. Awaya S. Miyama E: Insertion anomalies of horizontal muscles in A & V pattern: Nippom Ganka- Gakka-Zazzhi 1991; 95 : 698 – 703. 7TKUV/,*QTK\QPVCNUSWKPVYKVJXGTVKECNFGXKCVKQPU Arch. Ophthalmol.1951; 46 : 245 – 267. 8. Costenbader FD. Strabismus Ophthalmic Symposium 11 (ed. Allen) St. Louis, 325, (1958). 9. Knapp P. Vertically Incomitant horizontal strabismus: The So-called A and V syndromes Trans. Amer. Ophthalmol soc.1959; 57: 666 – 699. 10. Boyd TAS., Leitch, G.T. and Budd, G.E. A new treatment for ‘A’ and ‘V’ patterns in strabismus by slanting muscle insertion . Canadian J. Ophthal 1971; 6: 170. 11. Jampolsky A. Bilateral Anomalies of the oblique muscles. Trans. Amer. Acad. Ophthal 1957 ; 61: 689. 12. Gobin MH. Anteroposittion of the inferior oblique muscle in A-V esotropia. Ophthalmologica1964 ;148.: 325 - 341. 13. Miller JE. Vertical recti transplantation in the A and V syndromes. Arch. Ophthal 1960 ; 64:175.
28 DJO Vol. 20, No.4, April-June, 2010 Preffered Practice Pattern CURRENT MANAGEMENT OF RETINOPATHY OF PREMATURITY Parijat Chandra, Rajvardhan Azad, Yograj Sharma, Atul Kumar Dr. R. P. Centre for Ophthalmic Sciences, AIIMS, New Delhi
INTRODUCTION Early Treatment For Retinopathy Of Prematurity Retinopathy of Prematurity (ROP) is a vasoproliferative Cooperative Group (ETROP)4 study. ETROP group disorder of the retina affecting premature babies. As divides these eyes into 2 categories – more advanced neonatal centers continue to save Ŗ 6[RG412FGſPGFCU smaller premature babies, the incidence of ROP is o zone I, any stage ROP with plus disease or on the rise, and has become an important cause of o zone I, stage 3 ROP without plus disease or childhood blindness across the world. o zone II, stage 2 or 3 ROP with plus disease. However, in developing countries there is a major lack Ŗ 6[RG412FGſPGFCU of awareness about ROP among ophthalmologists o zone I, stage 1 or 2 ROP without plus disease and neonatologists, due to which a large number of or these children are not screened and left untreated; to o zone II, stage 3 ROP without plus disease. be later detected with advanced ROP, when prognosis becomes poor. As awareness is spreading about ROP, Type I ROP requires prompt treatment, while Type more centers have started establishing ROP screening 2 ROP is regularly followed up and considered for programs to detect and treat this blinding disease treatment only if it progresses to Type 1 or threshold in a timely manner and thereby reduce childhood ROP. blindness. It is essential here to be aware of the newly 6JG KPVGTPCVKQPCN ENCUUKſECVKQP QH TGVKPQRCVJ[ QH FGſPGF VGTOKPQNQI[ ECNNGF #IITGUUKXG 2QUVGTKQT prematurity (ICROP)1 ITQWR JCU ENGCTN[ ENCUUKſGF 412 #2412 YJKEJUKIPKſGUCTCRKFN[RTQITGUUKXG ROP into zones and stages to enable both beginners severe form of ROP in zone I or zone 2 posterior, and experts managing ROP to communicate easily which is characterized by prominent plus disease, COQPI VJGOUGNXGU &GſPKVKXG 412 UETGGPKPI KNNFGſPGF TGVKPQRCVJ[ YKVJ ƀCV PGQXCUEWNCTK\CVKQP guidelines have been established to detect treatable deceptively featureless junction between vascularized ROP in a timely manner. and non vascularized retina, and can rapidly progress The American screening guidelines2 recommend to stage 5 ROP. It is essential to be aware of AP-ROP, UETGGPKPIDCDKGU IDKTVJYGKIJVQT YMU)# and treat it promptly when present. or higher in case of unstable babies considered high 4GVECO CUUKUVGF ƀWQTGUEGKP CPIKQITCRJ[ KP 4125 risk to develop ROP by neonatologist. They suggest has emerged as a useful investigative modality which VJG ſTUV UETGGPKPI DG FQPG CV YMU 2%# QT YMU helps to distinguish vascular from avascular retina, after birth, whichever is later. Indian expert group3 identify causes of persisting plus disease, identify skip UWIIGUVU UETGGPKPI DCDKGU I DKTVJ YGKIJV QT areas of treatment and detect early neovascularization YMU)#QTJKIJGTKPECUGQHWPUVCDNGDCDKGU in AP-ROP enabling timely treatment of these cases. considered high risk to develop ROP by neonatologist. Another interesting area of development has been the 6JG[ TGEQOOGPF ſTUV UETGGPKPI DG FQPG CV YMU computerized analysis of vascular tortuosity6. This PCA or 4wks after birth, whichever is earlier (even at helps in a better understanding of plus disease and 2-3 weeks in VLBW babies). detects changes suggestive of severity or progression of ROP. TREATMENT OF ROP Timely screening and prompt treatment is the key Laser Photocoagulation to success in ROP. If treated in time, ROP mostly Laser Photocoagulation has become the treatment regresses uneventfully and has good visual outcome. of choice for treatable ROP when indicated. The The current treatment strategy is guided by The basic principle of laser treatment is the ablation
29 Current Management of Retinopathy of Prematurity DJO Vol. 20, No.4, April-June, 2010 of the avascular retina to remove the ischemic Scleral buckling is performed in stages 4A and 4B stimulus stimulating neovascularization. Laser with the aim to relieve vitreoretinal traction and help photocoagulation in eyes of premature babies is KP CDUQTRVKQP QH UWDTGVKPCN ƀWKF # GPEKTENKPI done using a laser indirect ophthalmoscopic delivery band / buckle is passed (2.5mm wide) and anchored system with preferably Green or Diode lasers. It is a to the sclera with anchoring sutures. This encirclage time intensive procedure and requires trained experts requires removal after a few months to allow the eye to to perform it. grow normally. Scleral buckling has led to favorable The treatment aims to laser the entire avascular results and is widely used7. retina, while sparing the ridge, and completing the With advent of modern vitreoretinal surgical procedure in one sitting in both eyes. Since laser techniques, lens sparing pars plana vitrectomy helps treatment is a stressful procedure, it requires regular to relieve the vitreoretinal traction better internally, vitals monitoring by a neonatologist. and is proving more effective than scleral buckling in Regular weekly follow up is required till the selected cases with good results.8,9 Now three port 25- disease regresses. If laser skip areas are noted, laser gauge transconjunctival sutureless vitrectomy is also augmentation of these patches may be needed. emerging as an effective technique to attach the retina Sometimes the ROP may progress to advanced stages in patients with stage 4 retinal detachment in ROP.10 and then require surgical intervention. Vitreoretinal surgery Advanced stage 5 ROP requires vitreoretinal surgery by experienced vitreoretinal surgeons and is available only at few tertiary centers. Surgery basically involves lensectomy, dissection and separation of retrolental membranes, removal of vitreoretinal traction from center to periphery to allow the retina to settle. The aim of surgery is an attempt to attain ambulatory vision for the child so they can perform their activities of daily living. Various diagnostic modalities like ultrasonography ECP JGNR CUUGUU VJG TGVKPCN HWPPGN EQPſIWTCVKQP KP Lasered regressed ROP the anterior and posterior parts. Open-open funnel Cryotherapy EQPſIWTCVKQPUJCXGVJGDGUVUWTIKECNQWVEQOGYJKNG Before laser photocoagulation gained popularity, PCTTQYPCTTQYEQPſIWTCVKQPUHCTGRQQTN[7$/JCU Cryotherapy was the preferred treatment and was used also been found to have a useful role in detecting to ablate the avascular retina. The main disadvantage anterior surgical space and determining the site of of Cryotherapy was it required general anesthesia, instrument entry. which carried a high risk in premature babies. However, in spite of best surgical efforts, the Though cryotherapy allowed faster treatment, yet anatomical and visual outcomes are relatively poor.11 the treatment parameters were less controlled and it The poor prognosis and bad surgical outcomes are led to extensive chemosis, congestion, cataract, pain often complicated by associated ocular disorders like and prolonged morbidity and was often associated glaucoma, uveitis, posterior synechiae etc. with more iatrogenic ocular complications. However, cryotherapy is still used at some centers because cryo machine is much more economical compared to the laser equipment, but laser is the treatment of choice now.
Scleral Buckling / Lens sparing vitrectomy Advanced ROP progressing to Stage IV and beyond requires surgical intervention, and the results and prognosis becomes poorer with disease progression. ROP progression is often due to lack of screening, delayed treatment or progression despite laser, especially in cases of AP-ROP. Demarcation Line 30 Current Management of Retinopathy of Prematurity DJO Vol. 20, No.4, April-June, 2010
Preoperative and Postoperative picture of an eye with stage 5 ROP
Subtotal retinal detachment Total retinal detachment
Anti-VEGF drugs Often cases of aggressive posterior ROP or those with delayed presentation / treatment may continue to progress, and there has been a need for an adjunct to laser which can help reduce the vascularity and control progression of ROP. Due to the success of Anti-VEGF agents like Bevacizumab, Pegabtanib sodium in various neovascular ocular pathologies like wet AMD, diabetic retinopathy, Eales diseases, venous occlusions etc. – many ophthalmologists started trying these agents Demarcation Ridge in ROP with some success. Preliminary studies have demonstrated useful effect of bevacizumab and Pegabtanib in pilot trials since these agents have helped to reduce vascularity, neovascular proliferation and help control the disease process. 12,14 It is important to realize that these drugs are not FDA approved for treatment of ROP, and may have long term side effects following systemic drug absorption which may block normal vasculogenesis of vital organs. Moreover, the complications of repeated intravitreal injections are also of major concern. Thus, there is a need for large scale multi-centric VTKCNU VQ VGUV VJGKT GHſECE[ KP VTGCVOGPV HQT 412 Some notable studies underway investigating into Extraretinal Fibrovascular proliferation their role include Pan-VEGF Blockade for the 31 Current Management of Retinopathy of Prematurity DJO Vol. 20, No.4, April-June, 2010 Treatment of Retinopathy of Prematurity (BLOCK of retinopathy of prematurity: results of the early treatment ROP) trial in which premature newborn infants for retinopathy of prematurity randomized trial. Arch with bilateral progressive APROP despite complete Ophthalmol. 2003 Dec;121(12):1684-94. peripheral retinal ablation will be given Bevacizumab 5. Azad R, Chandra P, Khan MA, Darswal A. Role administered in a single dose; and will assess its safety, QH KPVTCXGPQWU ƀWQTGUEGKP CPIKQITCRJ[ KP GCTN[ FGVGEVKQP GHſECE[ KP VTGCVOGPV QH 412 #PQVJGT PQVCDNG VTKCN and regression of retinopathy of prematurity. J Pediatr is Bevacizumab Eliminates the Angiogenic Threat Ophthalmol Strabismus. 2008 Jan-Feb;45(1):36-9. of Retinopathy of Prematurity (BEAT-ROP) trial, a 6. Wallace DK, Freedman SF, Zhao Z. Evolution of prospective, randomized, controlled, multi-centric RNWU FKUGCUG KP TGVKPQRCVJ[ QH RTGOCVWTKV[ SWCPVKſECVKQP study where intravitreal Bevacizumab injections by ROPtool. Trans Am Ophthalmol Soc. 2009 Dec;107:47- versus conventional laser will be compared for 52. vision-threatening ROP (acute stage 3 ROP in zone I 7. Beyrau K, Danis R. Outcomes of primary scleral or posterior zone II with plus disease). buckling for stage 4 retinopathy of prematurity. Can J These study results will give clearer guidelines for Ophthalmol. 2003 Jun;38(4):267-71. the role of anti-VEGF agents in ROP. Till then its 8. El Rayes EN, Vinekar A, Capone A Jr. Three-year usage must be restricted to select progressive APROP anatomic and visual outcomes after vitrectomy for stage 4B cases as an adjunct to laser, after ethics clearance and retinopathy of prematurity. Retina. 2008 Apr;28(4):568-72. detailed informed consent. 9. Bhende P, Gopal L, Sharma T, Verma A, Biswas RK. Functional and anatomical outcomes after primary lens- In conclusion, it is best to run effective screening sparing pars plana vitrectomy for Stage 4 retinopathy of programs to ensure early detection and timely prematurity. Indian J Ophthalmol. 2009 Jul-Aug;57(4):267- treatment in children with ROP. As ROP progresses, 71. the treatment outcomes get poor. Therefore, spreading 10. Kychenthal A, Dorta P. 25-gauge lens-sparing awareness is vital to tackle this preventable cause of vitrectomy for stage 4A retinopathy of prematurity. Retina. childhood blindness. 2008 Mar;28(3 Suppl): S65-8. Erratum in: Retina. 2009 Jan;29(1):127. REFERENCES: 11. Gopal L, Sharma T, Shanmugam M, Badrinath SS +PVGTPCVKQPCN %QOOKVVGG HQT VJG %NCUUKſECVKQP QH et al. Surgery for stage 5 retinopathy of prematurity: the 4GVKPQRCVJ[QH2TGOCVWTKV[6JG+PVGTPCVKQPCN%NCUUKſECVKQP learning curve and evolving technique. Indian J Ophthalmol of Retinopathy of Prematurity revisited. Arch Ophthalmol. 2000 Jun;48(2):101-6 2005 Jul;123(7):991-9. Review. 12. Mintz-Hittner HA, Kuffel RR Jr. Intravitreal injection 2. American Academy of Pediatrics, American Academy of bevacizumab (avastin) for treatment of stage 3 retinopathy of Ophthalmology American Association for Pediatric of prematurity in zone I or posterior zone II. Retina. 2008 Ophthalmology and Strabismus. Screening Examination of Jun;28(6):831-8. Premature Infants for Retinopathy of Prematurity. Pediatrics 13. Guttman C. Pioneering use of pegaptanib sodium to 2006; 117: 572-576 treat ROP unveils many issues for future studies. EuroTimes. 3. Jalali S, Anand R, Kumar H, Dogra MR, Azad R, September 2006. Pg 34. Gopal L. Programme planning and screening strategy in 14. Micieli JA, Surkont M, Smith AF. A systematic retinopathy of prematurity. Indian J Ophthalmol. 2003 analysis of the off-label use of bevacizumab for severe Mar;51(1):89-99. retinopathy of prematurity. Am J Ophthalmol. 2009 4. Early Treatment For Retinopathy Of Prematurity Oct;148(4):536-543. Cooperative Group. Revised indications for the treatment
32 DJO Vol. 20, No.4, April-June, 2010 Case Report Goldenhar-Gorlin syndrome U.S. Tiwari, Debarati Saha Department of Ophthalmology, G.R.Medical College, Gwalior
)QNFGPJCTU[PFTQOGYCUſTUVFGUETKDGFD[ preauricular skin tags, there was no sign suggestive Maurice Goldenhar in 1952 in a subject having of external, middle or internal ear abnormality. Nose triad of accessory tragi, mandibular hypoplasia and and oropharyngeal examinations were normal. epibulbar dermoid present since birth1. Subsequently it was named as oculo auriculo vertebral dysplasia Investigation: (OAVD) by R.J.Gorlin (1963) due to the presence of i) X-ray spine showed hemi vertebra of S1 and additional vertebral anomalies2. lumbosacral scoliosis, but cervical spine was absolutely normal. Case report: ii) X-ray mastoid (Schullars view) showed hypoplastic A six months old male child was brought to the eye mastoid air cells bilaterally [normal at this age]. OPD by his parents with the complaints of yellowish KKK 75)CDFQOGPHQWPFPQQTICPQOCN[ white elevated mass in left eye with a gap in the upper The clinico-radiological triad of epibulbar dermoid, lid since birth. preauricular skin tags and vertebral anomalies were The parents (nonconsanguinous marriage) gave consistent with the diagnosis of oculo-auriculo- no positive family history of such condition. They vertebral dysplasia (OAVD), which is a subtype of had two elder daughters having no abnormality. No oculo-auriculo-vertebral spectrum (OAVS) or facio- history of fever or any other health ailment of the auricular-vertebral sequence (FAV) or Goldenhar mother during pregnancy period was reported. There sequence or Goldenhar syndrome3,4,5. This reported was no history of maternal smoking, alcoholism or case of OAVD also has unusual features of lid any toxic drug intake. However the mother had some coloboma and congenital scoliosis due to sacral hemi facial characteristics of prominent and bossed frontal vertebra. bone, telecanthus, prominent eyes with antimongoloid slant, small sharp nose and pointed chin but no dental Discussion: abnormality. Many authors consider OAVD and Goldenhar On ocular examination of the baby, there was syndrome to be synonymous while others believe that upper lid coloboma of the left eye on medial 1/3rd that the syndrome encompasses two broad subgroups and a epibulbar dermoid at 3 to 6 O’ clock position namely OAVD and hemi facial microsomia5. This (8.5 mm diameter, hemispherical) encroaching up view is based on developmental similarities which to half of the pupillary area. The dermoid had no CTGCF[URNCUKCQHſTUVCPFUGEQPFDTCPEJKCNCTEJGU UWRGTſEKCN JCKT ITQYVJ QP KV 6JG DCD[ YCU VCMKPI CPFKPVGTXGPKPIſTUVRJCT[PIGCNRQWEJCPFDTCPEJKCN ſZCVKQP RTGHGTGPVKCNN[ YKVJ JKU TKIJV G[G 1EWNCT cleft in both the subgroups. That is why Goldenhar examination including pupillary light reaction and U[PFTQOGKUCNUQMPQYPCUſTUVCPFUGEQPFDTCPEJKCN fundus examination was normal in both the eyes. arch syndrome. The dysplasia is believed to occur There were three preauricular skin tags on the either due to the teratogen exposure or a vascular left side and one on right side. One more skin tag with disruption in intrauterine life or maternal diabetes3,4. DNKPFUMKPſUVWNCYCURTGUGPVQPNGHVEJGGMQXGTVJG A new theory put forward for this developmental line joining angle of the mouth and left tragus. anomaly states that OAVS occurs as a result of On general and systemic examination the child ectodermal non disjunction early in embryonic showed no abnormality except a bluish grey colored development during 4th to 8th weeks of intrauterine naevus at the nape of the neck. life with subsequent mesodermal tethering. This No abnormality was found on cardiovascular theory explains multisystem involvement in OAVS6. and central nervous system examination. The baby The incidence of Goldenhar syndrome has was playful and of normal developmental milestones. been reported to be between 1:3500 and 1:5600, With Otorhinolaryngological evaluation, other than with a male: female ratio of 3:27. The incidence is
33 Goldenhar-Gorlin Syndrome DJO Vol. 20, No.4, April-June, 2010 higher, about 1:1000, in children with congenital cells. Inner ear abnormality includes conductive &/or deafness1. It mostly occurs as sporadic case but sensorineural deafness11. familial incidences are also noted suggestive of autosomal dominant or recessive inheritance. In c) Vertebral: addition, some researchers suggest that the disorder Involvement of axial skeleton (vertebrae & ribs) may be caused by the interaction of many genes, has been observed in 24% of patients in the largest possibly in combination with environmental factors series reported by Rollnic et al125RKPCDKſFCKUVJG - multifactorial inheritance8. Few other authors have commonest and least severe of all anomalies10. The reported its association with trisomy of chromosome vertebral anomalies are known to range from hypoplasia 22 and chromosome 94,5. Variable phenotypic & occipitalisation of Atlas, fusion of posterior expression is the characteristic of this syndrome & element of cervical vertebrae, block vertebra, spina the spectrum of phenotypic abnormalities can range DKſFCQEEWNVCJGOKXGTVGDTCCPFDWVVGTƀ[XGTVGDTCG from mild to severe even within the same affected to complete sacral agenesis3. Vertebral anomalies are family. There are cases of identical (monozygotic) more commonly seen in cervical region & cervical twins in which only one has the syndrome, even lordosis has been reported. Congenital scoliosis may though they received the same genetic blueprint; occur along with hemi vertebra. Rare case report of whilst other researchers describe examples of kyphosis & scoliosis with hemi vertebra in Goldenhar siblings having Goldenhar Syndrome. There are a syndrome has also been reported13. few families with an affected person having a 50% chance of passing on the syndrome. Family history d) Facio-dental & skull anomalies: may include cleft lip or palate, unusually shaped ears, Hyploplasia of malar bones, zygomatic arch, maxilla asymmetry of face, small chin, skeletal problems, eye and mandible may be present; characteristic of hemi abnormalities, internal organ anomalies or speech facial microsomia. Some rare associations recorded and dental problems. The abnormalities are found to in the literature are macrostomia, micro¬gnathia, high be mostly unilateral in 85% of cases and bilateral in XCWNVGFRCNCVGDKſFVQPIWGENGHVRCNCVGOCNQEENWUKQP 10-33% cases9. Kulkarni VV, Shah MD & Parikh AA and other dental anomalies and hydro¬cephalus14. reported, in Goldenhar syndrome, ocular anomalies especially bilateral dermoids are seen in 60% of the e) Other organ anomalies: cases, vertebral anomalies in 40% of the cases and ear Goldenhar syndrome may present as a complex of anomalies also in 40% of the cases8. craniofacial anomalies associated with vertebral, cardiac, neural, pul¬monary and genitourinary The various features of Goldenhar syndrome or abnormalities15. Although not well reported, up to 70% OAVD: of cases have urogenital anomalies that include fused MKFPG[UTGPCNCIGPGUKUXGUKEQWTGVGTKETGƀWZ 874 a) Ocular: RGNXKWTGVGTKE LWPEVKQP 27, QDoUVTWEVKQP WTGVGTCN Epibulbar dermoids are the commonest abnormality duplication, multicystic kidney and ret¬rocaval found in about 75% cases10. In rare cases they may be ureter16,17. bilateral. They are classically located at infero temporal The presence of heart disease in such patients quadrant. These are choriostomas; i.e. Nest of normal accounts for 50% incidence18. Tetralogy of Fallot tissue at abnormal places. Lipodermoids are present (TOF) and ventricular septal defects (VSD) are the in less than 50% of cases10. Dacryocystitis has also most common cardiovascular anomalies associated been reported. The other rarer ocular associations are with OAVS2. Multiple cardiovascular malformations upper lid coloboma, microphthalmos, cataract, iris & including Wolff-Parkinson-White (WPW) syndrome, choroid coloboma, tilted disc, optic nerve hypoplasia, a partial anomalous pulmonary venous connection, squint, ocular motility problems and anophthalmos10. patent ductus arteriosus (PDA), an anomalous origin of the coronary arteries, and a right sided descending b) Auricular: aorta were revealed by electrocardiography, Preauricular skin tags, pits/sinuses and accessory echocardiography and cardiac catheterization. auricles are most common features; usually unilateral, Goldenhar syndrome is quite rare, but the frequency but can be bilateral in 33% cases10. Other external ear of cardiovascular malformations in this syndrome is abnormalities range from dysmorphic ear to microtia 5-58%. It is necessary to perform a careful evaluation to anotia (rare, reported by Jaison and Batra11). Middle of general malformations, especially cardiovascular ear may be narrow or atretic with hypoplasia of air malformations19.Anophthalmos, facial palsy,
34 Goldenhar-Gorlin Syndrome DJO Vol. 20, No.4, April-June, 2010 ECNEKſECVKQP QH HCNZ EGTGDTK WPFGUEGPFGF VGUVGU natal screening and genetic advice may be offered for Turner’s syndrome and glaucoma are some rarer future pregnancies. reported associations20,11.In approximately 5 to 15 per cent of affected individuals, mild mental retardation may also be present21,22,23. A case has been reported with ocular motility disturbances and congenital heart disease as rare association of Goldenhar syndrome24. Non axial skeletal involvement in the form of thumb hypoplasia along with Goldenhar-Gorlin syndrome has also been reported25.
The syndrome may require to be differentiated from Left eye upperlid coloboma , epibulbar dermoid & the following conditions: preauricular skin tags Other syndromes associated with multiple preauricular tragi include Treacher-Collins syndrome, Wolf-Hirschhorn syndrome, Nager’s acrofacial dysostosis, Wildervanck syndrome (cervico-oculo- acoustic syndrome), Townes-Brocks syndrome and Delleman syndrome26,27. Treacher Collins syndrome is associated with maxillary and mandibular hypoplasia but associated with less common occurrence of eye and ear anomalies8. In this syndrome coloboma affect lower eyelids rather than upper eyelids28. Ear Left sided accessory tragi & skin tag with blind skin anomalies may also occur in Pierre Robin ſUVWNCQPEJGEM syndrome29, Moebius syndrome30 and thalidomide embryopathy. However, these conditions have their own distinguishing features (e.g. marked mandibular hypoplasia in Pierre Robin syndrome, sixth and seventh nerve palsies in Moebius syndrome and phocomelias and amelias in thalidomide embryopathy).
Prognosis and management: Children with Goldenhar Syndrome usually look forward to a long life and normal intelligence. #P[U[UVGOKECPQOCN[UJQWNFDGRTQORVN[KFGPVKſGF Right sided preauricular skin tag & managed appropriately. Special tests such as ultrasound, x-ray, CT scan or MRI may be required in order to obtain complete information about a given case. Hearing aids, Speech therapy, Orthodental treatment and Physiotherapy may be the integral parts of management. The structural anomalies of the eyes and ears in Goldenhar syndrome can be corrected by plastic surgery. Surgery is usually postponed until the growth of the structure is completed. The epibulbar dermoids should be removed.
Pre-natal diagnosis: Scanning may identify the condition in certain cases where facial or skeletal anomalies are present. Antenatal sonographic detection of OAVS has also 5 X-Ray spine showing hemivertebra of S1 and to been reported by Witters et al and other workers . Pre- lumbosacral scoliosis 35 Goldenhar-Gorlin Syndrome DJO Vol. 20, No.4, April-June, 2010 Consent: Recognizable patterns of human malformation. Genetic, Written consent was taken from the parents of embryologic and clini¬cal aspects. Philadelphia. WB the patient to publish the report and photographs. Saunders 1982; 497-500. 16 Ritchey ML, Nurheck J, Huang C. Keating MA, Bloom References: &# 7TQNQIKECN OCPKHGUVCVKQPU QH )QNFGPJCT U[PFTQOG 4GFF[/84GFF[227UJC4CPK2*GOC$KPFW. 7TQNQI[ Facio-auricular vertebral syndrome-a case report. Indian J 17 lshitoya S. Arai Y, Waki K. Okubo K, Suzuki Y. Left Hum Genet 2005; 11: 156-8. retrocaval ureter associated with the Goldenhar syndrome 2 Nakajima H, Goto G, Tanaka N, Ashiya H, Ibukiyama $TCPEJKCNCTEJU[PFTQOG ,7TQN C. Goldenhar syndrome associated with various 18 Corey JC: Malformations and Syndromes involving cardiovascular malformations. Jpn Circ J 1998; 62: 617- craniofacies, Paediatrics (Editor - Rudolph. Abraham M), 20. #RRNGVQPEGPVWT[ETCHVU%QPPGEVKEWV75# R 3 Gibson JNA, Sillence DO, Taylor TKF. Abnormalities 19 Jpn Circ J 1998; 62: 617-620. of spine in Goldenhar’s syndrome. J Pediatr Orthop 1996; 20 Rao VA, Kaliaperumal S, Subramanyan T, Rao KR, 16: 344-349. Bhargavan R. Goldenhar’s sequence with associated 4 Kobrynski L, Chitayat D, Zahed L et al. Trisomy 22 juvenile glaucoma in Turner’s syndrome. Indian J and Facioauriculovertebral (Goldenhar) Sequence. Am J Ophthalmol 2005; 53: 267-8. Med Genet 1993; 46: 68-71. -QTNKP4,WG-,CEQJUGP7GVCN1EWNQCWTKEWNQXGTVGDTCN 5 Witters I, Schreurs J, Wing JV, Wouters W, Fryns JP. dysplasia, J Pediatr 1963; 63: 991. Prenatal Diagnosis of facial clefting as part of the oculo 22 Sugas HS. The Oculoauriculo vertebral ysplasia auriculo-vertebral spectrum. Prenat Diagn 2001; 21: 62- syndrome of Goldnhar. Am J Ophthalmol 1966; 62: 678- 64. 82. 6 Lam CH. A theory on the embryogenesis of 23 Ziauras E, Farbew MCC. Diamond CCA.pedunculated oculoauriculo-vertebral (Goldenhar syndrome). J lipodermoid in auriculovertebral dysplasia. Arch Opthal. Craniofac Surg 2000; 11: 547-552. 1990; 108: 2032-33. )TCDD 9% 6JG ſTUV CPF UGEQPF DTCEJKCN CTEJ 24 Verma MJ, Faridi M. Ocular motility disturbances syndrome. Plast and Reconstruct Surg. 1965; 36: 485-50. (Duane retraction syndrome and double elevator palsy) 8 Kulkarni VV, Shah MD, Parikh AA. Goldenhar with congenital heart disease, a rare association with syndrome: A case report. J Postgrad Med 1985; 31: 177- Goldenhar syndrome-A case report. Indian J Ophthalmol 9. 1992; 40: 61-2 9 Sharma JK, Pippal SK, Raghuvanshi SK, Shitij A. 25 Das A, Ray B, Das D, Das S. A case of Goldenhar- Goldenhar-Gorlin’s syndrome: A case report. Indian J Gorlin syndrome with unusual association of hypoplastic Otolaryngol Head Neck Surg. 2006; 58: 97-101. thumb. Indian J Ophthalmol 2008; 56: 150-2. 10 Maj V Mathur, Lt. Col. R.K.Mishra; MJAFI 2000; 56: 26 Miller TD, Metry D. Multiple accessory tragi as 231-232. a clue to the diagnosis of the oculo-auriculo-vertebral 11 Jaison SG, Batra N. Goldenhar syndrome with multiple (Goldenhar) syndrome. J Am Acad Dermatol 2004; 50:1 additional anomalies. Indian J Ophthalmol. 1996; 44: 42- 1-3. 44. 27 Schaffer JV, Antaya R. Genodermatoses. In: Bolognia 12 Rollnic BR, Kaye CI, Nagotoshi K. et al. Oculo- JL, Jorizzo JL, Rapini RP, editors. Dermatology. 1 st ed. auriculo-vertebral dysplasia and variant: Phenotypic Elsevier Ltd; 2003; p. 926. characteristics of 294 patients. Am. J Med Genet 1987; 28 Collins, E. T.: Cases with symmetrical congenital 26: 361-75. notches in the outer part of each lid and defective 13 Das A, Majumdar S, Chatterjee SS. Atypical case of development of the malar bones. Trans. Ophthalmol. Soc. oculo-facio-auriculo-vertebral dysplasia (goldenhar-gorlin 7- 3WQVGFD[5CNOQPCPF.KPFGPDCWO syndrome). Indian J Ophthalmol 1999; 47: 131-3. (1978). 14 Gorlin, R.J. and Pindborg J.J. Syndromes of head and 29 Dennison, W. M.: The Pierre-Robin syndrome. neck. McGraw-Hill Book company Lon¬don, Page 422. Paediatrics, 36: 336-341, 1965. 15 Smith DW. Facio-auriculo-vertebral spectrum. In 30 Summit R.: Familial Goldenhar Syndrome: Birth Defects, 5: 106-109. 1969.
36 DJO Vol. 20, No.4, April-June, 2010 Case Report CROUZON SYNDROME Navin Chandra Department of Ophthalmology, Assam Medical College & Hospital, Dibrugarh
A 27 year old male patient presented with sudden Ocular Examination: onset of pain, redness, photophobia and diminution of vision in left eye since 10 days, no past history of RIGHT EYE LEFT EYE Visual Acuity 6/9 HM +ve ocular injury. Eye Lid Normal Normal At presentation visual acuity in right eye was 6/9 and Conjunctiva Normal Normal VJCVQHNGHVG[GYCU*/ XGQPUNKVNCORGZCOKPCVKQP Cornea Normal Cornea Lesion corneal ulcer with hypopyon was diagnosed. (Ulcer) 1 x 2 mm, oval with On general examination maxillary hypoplasia, parrot irregular margin DGCMPQUGDTQCFJCPFUYKVJUJQTVſPIGTUCECPVJQUKU in the center of nigricans were present. X-ray cervical spine, skull, cornea hands and foot showed typical picture of Crouzon Anterior Normal Hypopyon Chamber present syndrome. Pupil NSNR Cannot be seen Because of rarity of presentation case is being Fundus Normal Cannot beseen reported. Vision 6/9 HM +ve
Key Words: Crouzon Syndrome
Introduction It is a part of a group of genetic disorders known CU C DTCPEJKCN CTEJ U[PFTQOGU OQTG URGEKſECNN[ VJGſTUVDTCPEJKCN QTRJCT[PIGCN CTEJYJKEJKUVJG precursor of maxilla and mandible. This syndrome is named after Octave Crouzon, a French physician. Photograph–2 Parrot Beak Nose with B/L Exophthalmos It is also known as craniofacial dysostosis and acrocephalosyndactylyType II. General Examination It is caused primarily by premature fusion of the Brachycephaly coronal and sagittal sutures. (transverse diameter > AP diameter) ICD–10 á Q75.1 Psittichorhina (Parrot beak nose) 1Postgraduate Trainee, Exophthalmos Hypertelorism History Maxilla Hypoplastic A 27-years old male patient presented with sudden Mandibular Prognathism onset of pain, redness, photophobia and diminution Concave Face (frog like face) of vision in Left Eye since 10 days; No Past History Acanthosis Nigricans of Ocular Injury. Dental Malocclusion
Photograph–3 Frog-like Face with Maxillary Hypoplasia Photograph–1 General Appearance at Presentation and Mandibular Prognathism 37 Crouzon Syndrome DJO Vol. 20, No.4, April-June, 2010
Photograph–7 X-ray Cervical Spine Lateral View
X-ray of Both Hand AP and Lateral View: – Tubular bones of hand are short – Distal phalanx of all digits are short
Photograph–4 & 5 Shortening of Distal Phalanx
Investigations Blood Routine Examination: Haemoglobin: 11.3 gm% (78%) Total WBC Count : 7,500/mm3 Differential Leukocyte Count : N68E2B0L29M1 Random Blood Sugar : 75 mg% X-rays: X-ray Chest PA View : Normal X-ray Cervical Spine and Lateral View : Normal Photograph–8 X-ray of Both Hand AP View
Photograph–6 X-ray Chest PA View Photograph–9 X-ray of Both Feet Lateral View
38 Crouzon Syndrome DJO Vol. 20, No.4, April-June, 2010 X-ray of Both Feet AP and Lateral View: Diagnosis – Tubular bones of feet are short On the basis of clinical features and x-rays report the – Distal phalanx of all digits are short case showed typical picture of Crouzon syndrome.
X–ray Skull AP and Lateral View: Treatment – Brachycephaly Conservative Treatment: – The lateral ridge of bilateral orbits appears Antibiotic Eye Drops elevated giving bilateral #TVKſEKCN6GCT'[G&TQRU ő*CTTGSWKPŒEQPſIWTCVKQPQHQTDKV Antibiotic Eye Ointment – B/L orbit are shallow with exorbitism Tarsorrhaphy was done in the Left Eye . – Profound maxillary hypoplasia – High arched palate Discussion – Calvaria is thinned out Crouzon Syndrome is a genetic disorder. with focal lucencies giving It is an autosomal dominant disorder. “copper seater appearance” Incidence 1 : 25,000 (suggesting possibly of hydrocephalous) Associated with FGFR2 and FGFR3 gene – B/L Dental malocclusion Sex and race not associated Ō %CNEKſECVKQPQH$.UV[NQJQKFNKICOGPV Conclusion Because of rarity of presentation, case is being reported.
References (1) L.E.O. Crouzon. Dysostose cranio-faciale hereditaire. Bulletin de la societe des medecins des Hospitausc de Paris. 1912; 33: 545—555. (2) Reardon W, Winter RM, Rutland P, Pulleyn LJ, Jones BM, Malcolm S (September 1994). “Mutations in the ſDTQDNCUV ITQYVJ HCEVQT TGEGRVQT IGPG ECWUG %TQW\QP syndrome”. Nat Genet. 8(1); 98—103; doi 10, 1038/ngo 994—98. Photograph–9 X–ray Skull AP View (3) Meyers GA, Orlow SJ, Munro IR, Przylepa KA, Jabs EW (December 1995). “Fibroblast growth factor receptor 3 (FGFR3) transmembrane mutation in Crouzon syndrome with Acanthosis nigricans”. Nat Genet 11(4): 462—4. doi 10, 1038/ng 1295—462. (4) James, William; Berger, Timothy; Elston, Dirk (2005). Andrews Disease of the Skin; Clinical Dermatology (10th Edition). Saunders. ISBN 0721629210. (5) Nuckolls GH, Shum L, Slankin HC. Progress towards understanding craniofascial malformations. Cleft Palate- Craniofac J 1999; 36: 12—26. (6) Carlson BM. Human Embryology and Development Biology, 2nd Edition, St. Louis: CV Mosby, 1999. (7) Nardich TP, Zimmerman RA, Bauer BS, Altman NR, Bailaniuk IT. Mid Face; Embryology and congenital Photograph–9 X–ray Skull Lateral View lesions. In: Som PS, Curtin HD. Head and Neck Imaging, Differential Diagnosis Vol. St. Louis: CV Mosby, 1996; 3—60. Crouzon Syndrome (Type–II) (8) Wilkie AOM. “Craniosynostosis”, Genes and Apert Syndrome (Type–I) Mechanism. Human Mol Genet 1997; 6: 1647—1656. Saethre–Chjotzen Syndrome (Type–III) (9) Reardon W, Wintel RM, Rutland P et al. Mutations KP VJG ſDTQDNCUV ITQYVJ TGEGRVQT IGPG ECWUGU ETQW\QP syndrome. Nature Genet 1994; 8: 98—103.
39 DJO Vol. 20, No.4, April-June, 2010 Case Report Bilateral Mooren’s Ulcer 1 Uday Gajiwala, 2 Jyotsom Ganatra, 1Rajesh Patel, 1Parin Shah,1 Rohan Chariwala 1. SEWA Rural, Jhagadia-393110, Dist.-Bharuch, Gujarat, INDIA, Phone-(02645)220021,
#DUVTCEV /QQTGPŏU7NEGTKUCPKFKQRCVJKETCRKFN[RTQITGUUKXGRCKPHWNRGTKRJGTCNWNEGTCVKXG keratitis with no associated scleritis. It’s a diagnosis of exclusion which means all other diagnosable systemic disorders that could be responsible for the progressive destruction of EQTPGCOWUVDGTWNGFQWV6JGGVKQNQI[QH/QQTGPŏU7NEGTTGOCKPUWPEGTVCKP*QYGXGTTGEGPV UVWFKGUKPFKECVGVJCVKVKUCPCWVQKOOWPGFKUGCUGFKTGEVGFCICKPUVCURGEKſEVCTIGVOQNGEWNGKP the corneal stroma, triggered in genetically susceptible individuals by one of several possible mechanisms. A 40 years old female presented to us on 25th January with white cells and spreading towards centrally and 2008 from rural tribal area of Sagbara with bilateral EKTEWOHGTGPVKCNN[ +V YCU RQUKVKXG HQT ƀWQTGUEGKP peripheral ulcerative keratitis with perforation and stain indicating overlying epithelial defect. The pupil iris prolapse in right eye. She was a known case of was peaked inferotemporally in right eye due to diabetes mellitus diagnosed seven years ago. This case perforation and in left eye due to impending report aims to highlight the diagnosis and treatment of Mooren’s ulcer. Presentation:
Diagnosis of Mooren’s ulcer was made after she underwent extensive medical and laboratory testing to rule out an infectious or systemic cause of corneal melt.
5JG DGPGſVGF D[ TKIJV G[G RCVEJ ITCHV NGHV G[G tissue adhesive with BCL application, Both eyes conjunctival peritomy and immunosuppressive drugs along with control of diabetes.
Case Report: A 40 year old female from rural tribal area of Sagbara with low socio economical status presented to us on Right Eye 25th January 2008 with complaint of decreased vision, redness, watering, ocular pain and photophobia in both eyes for last 2 months more so in right eye for last one week. She was having diabetes mellitus for last seven years which was controlled by medication. There was no history of trauma or any associated joint pain.
On examination, best corrected visual acuity in right eye was 6/36 and in left eye, it was 6/24. There was inferior crescent of corneal thinning involving 6 clock hours (3-9 o’clock) with Limbal involvement but no associated scleritis. In right eye, there was a perforation with Iris prolapse at 6-7 o’clock position. +PDQVJG[GUVJGTGYCUQXGTJCPIKPIGFIGKPſNVTCVGF Left Eye
40 $KNCVGTCN/QQTGPŏU7NEGT DJO Vol. 20, No.4, April-June, 2010 perforation. There were no signs of secondary infection and/or Iritis on examination. Dilated Indirect ophthalmoscopy was normal in both eyes. (No diabetic retinopathy)
She was investigated to rule out systemic disease causing peripheral ulcerative keratitis. RBS was 301 mg% showing uncontrolled diabetes mellitus on the day of presentation.
LE-After 6 Months Follow Up +PXGUVKICVKQPU FQPG Ō *GOQITCO YKVJ '54 7TKPG routine and microscopy, VDRL, RA factor, HCV, X- ray chest and Joints, SGPT, ANCA, ANA, HBsAg, Scraping of the ulcer was done, and did not reveal any causative organisms on smear or culture. She was referred to her physician to control diabetes LE-Just Before Tissue Adhesive mellitus as well as to rule out systemic disorders causing peripheral ulcerative keratitis. She was placed on Insulin injections to control DM.
After excluding systemic diseases associated with peripheral ulcerative keratitis, A diagnosis of Bilateral Mooren’s ulcer was made and systemic immunosuppressive therapy was started with oral methotrexate 10 mg once a week and oral systemic steroids at a dose of 1.5 mg / kg / day.
Right eye iris abscission (of prolapsed part) with patch graft (free Hand) was done after controlling diabetes mellitus. Tissue adhesive with Bandage Contact Lens Application with conjunctival peritomy was done in After Tissue Adhesive left eye.
Locally she was started on Prednisolone eye drops JQWTN[ 1ƀQZCEKP G[G FTQRU VKOGU C FC[ VKOQNQN maleate eye drops twice a day, 2 % HPMC eye ointment three times a day.
Tissue Adhesive and Bandage Contact Lens were removed after 6 weeks in left eye. It was epithelialized and scarred without any overhanging edge. Right eye patch graft is doing well after 6 months. Best corrected visual acuity in right eye 6/18 and left eye 6/18 after 6 months follow up.
Discussion: RE-After 6 Months Follow Up Although the diagnosis of Mooren’s ulceration may 41 $KNCVGTCN/QQTGPŏU7NEGT DJO Vol. 20, No.4, April-June, 2010 DG FKHſEWNV YJGP C RCVKGPV ſTUV RTGUGPVU YKVJ 27- form of Mooren’s ulcer has a predilection for young the clinical appearance is characteristic. However, men of African origin. a thorough medial history, physical examination and appropriate laboratory investigations must be Lewallen and courtright7, in their published series of performed to rule out underlying systemic conditions Mooren’s ulcer, suggest that younger patients had ECWUKPI27-UKPEG/QQTGPŏU7NEGTKUCFKCIPQUKUQH bilateral disease less frequently than older patients exclusion. (1.5:1) regardless of race. Although they found that men were 1.6 times more likely to have Mooren’s /QQTGPŏU WNEGT YCU ſTUV FGUETKDGF D[ $QYOCP KP ulcer than were women. 18491 and then by McKenzie in 1854 as “Chronic serpiginous ulcer of cornea or ulcus roden”2. Mooren’s Different entities have been associated with Mooren’s name, however, became attached to this rare disorder ulcer, often leading to conjecture that there may be a because of his publication of cases in 1863 and 18673. causal relationship. *G YCU VJG ſTUV VQ ENGCTN[ FGUETKDG VJKU KPUKFKQWU EQTPGCN RTQDNGO CPF FGſPG KV CU C ENKPKECN GPVKV[ An association with helminthiasis has been suggested Nettleship4 summarized the accumulated reported in Nigeria8, Schanzlin9 speculated that the antigen experience with the disorder in a classic article. antibody reaction to helminth toxin deposited in RGTKRJGTCN EQTPGC RTQXQMGF VJG KPƀCOOCVKQP CPF /QQTGPŏUWNEGTKUKFKQRCVJKED[FGſPKVKQPQEEWTTKPI ulceration. Recently in 2 patients with bilateral in complete absence of any diagnosable systematic Mooren’s ulcers chronic hepatitis with infection C was disorder that could be responsible for progressive documented10,11 and they improved after treatment of destruction of the cornea with no associated scleritis. the hepatitis C with interferon. The authors proposed that molecular mimicry may be involved, with the Its exact pathophysiology remains uncertain, hepatitis C virus stimulating an autoimmune response although a growing body of evidence indicates that it to corneal antigens through cross reacting epitopes. KUCPCWVQKOOWPGFKUGCUGFKTGEVGFCICKPUVCURGEKſE target molecule in the corneal stroma resulting in Based on the clinical presentation20 and the low dose its destruction by degradative enzymes, which are CPVGTKQT UGIOGPV ƀWQTGUEGKP CPIKQITCRJKE ſPFKPIU released primarily by neurotrophils attracted into there seem to be three distinct varieties of Mooren’s the area by diverse stimuli,12,13 probably triggered in ulceration: genetically susceptible individual by one or several mechanisms. 7PKNCVGTCN /QQTGPŏU WNEGTCVKQP 7/ characterized by an excessively painful progressive Wood and Kaufman having reported 9 cases concluded corneal ulceration in one eye in elderly patients, that there were two clinical types of Mooren’s ulcer5. CUUQEKCVGF YKVJ PQP RGTHWUKQP QH VJG UWRGTſEKCN 6JGſTUVNKOKVGFV[RGKUWUWCNN[WPKNCVGTCNYKVJOKNF vascular plexus of the anterior segment. to moderate symptoms, generally responds well to 2. Bilateral aggressive Mooren’s ulceration medical and surgical treatment. This type is believed (BAM), which occurs in young patients, progresses to occur in older patients and has become known as circumferential and only later, centrally in the cornea. typical or benign Mooren’s ulcer. In contrast, the Angiography shows vascular leakage and new vessel second type is bilateral, with relatively more pain formation which extends into the base ulcer. and generally a poor response to therapy in younger 3. Bilateral indolent Mooren’s ulceration (BIM), patients, became known as atypical or malignant, which usually occurs in middle aged patients presenting Mooren’s ulcer. The benign type is bilateral in 25% with progressive peripheral corneal guttering in both of patients and the malignant type is bilateral in 75 % G[GUYKVJNKVVNGKPƀCOOCVQT[TGURQPUG6JGTGKUPQ of Patients. change from the normal vascular architecture on angiography except an extension of new vessels into Keitzman6 published a series of 37 cases of progressive the ulcer. Mooren’s ulcer in Nigeria affecting primarily healthy men between age of 20 and 30 yrs and the clinical DD: course was very rapid. Perforation occurred in 36% • Rheumatoid arthritis of the patients. As a result, the generalized belief has • Wegener’s Granulomatosis developed that the progressive and relentless atypical • Polyarteritis Nodosa
42 $KNCVGTCN/QQTGPŏU7NEGT DJO Vol. 20, No.4, April-June, 2010 • Other collagen vascular diseases properties. Ŗ +PƀCOOCVQT[DQYGFFKUGCUG • Giant cell Arteritis A therapeutic soft contact lens or patching of eye may • Staphylococcal Marginal Keratitis DGDGPGſEKCNYJGPWNEGTKUFGGR • Local infections causes • Terrien’s degeneration Conjunctival Resection: • Pellucid degeneration • If ulcer progresses despite steroid regimen, • Ocular Rosacea conjunctival resection should be performed.15-18 Ŗ 7PFGT VQRKECN QT UWD EQPLWPEVKXGN[ CPGUVJGUKC Treatment: this consists of conjunctival excision to bare sclera Today, most experts agree on stepwise approach in extending at least 2 clock hours to either side of management of Mooren’s ulcer19. peripheral ulcer and approximately 4 mm posterior to the corneoscleral limbus and parallel to the ulcer.23 1. Topical Steroids The overhanging lip of ulcerating corneas may also 2. Conjunctival Resection DGTGOQXGF2QUVQRGTCVKXGN[CſTORTGUUWTGFTGUUKPI 3. Systemic immuno suppressive should be used. 4. Additional Surgical Procedure 5. Rehabilitation The rationale of this procedure: Conjunctiva adjacent to the ulcer contains Goals: KPƀCOOCVQT[EGNNUVJCVOC[DGRTQFWEKPICPVKDQFKGU To arrest the destructive process and to promote against the cornea and cytokines which amplify the healing and re-epithelialization of the corneal KPƀCOOCVKQP CPF TGETWKV CFFKVKQPCN KPƀCOOCVQT[ surface.15-18 cells.
Steroids: Multiple resections may be necessary. Initially: Intensive topical steroids Prednisolone acetate 1% eye drop Systemic immuno suppressive therapy: One hourly Indication: In association with: Bilateral or progressive Mooren’s ulcer that fails to Topical Cycloplegics preceding therapeutic attempts will require systemic Prophylactic Antibiotics cytotoxic theory to bring a half to the progressive corneal destruction If epithelial healing does not occur within 2-3 days, the frequency of tropical steroids can be increased to Commonly used agents: every half hour. Cyclophosphamide – 2 mg / kg / day Methotrexate (7.5 – 15 mg once a week) Once healing occurs, the frequency can be reduced Azathioprine (2 mg / kg body weight / day) and tapered slowly over a period of several months. The degree of fall in white blood cell count is considered Such management, especially in unilateral, benign as the most reliable indicator of immunosuppressant form has met with good results. produced by cyclophosphamide. Topical cyclosporine-A therapy (0.5% solution) also found useful in some studies. /QUVCWVJQTUDGNKGXGVJCVVJGGXKFGPEGHQTVJGGHſECE[ Systemic steroids: of systematic immune suppressive chemotherapy for progressive bilateral Mooren’s ulcer is quite strong, Oral pulse therapy: and further believe that such treatment should be 60-100 mg daily of oral Prednisolone is indicated employed sooner rather than later in the care of such when topical therapy is ineffective after 7-40 days. patients, before the corneal destruction, has become too extensive to need for surgery. When topical steroids may be dangerous because of RTGECTKQWUN[FGGRWNEGTQTKPſNVTCVG14, 22 Adverse effects of these cytotoxic and Topical tetracycline may be used for anticollagenolytic immunosuppressive medications, such as anemia,
43 $KNCVGTCN/QQTGPŏU7NEGT DJO Vol. 20, No.4, April-June, 2010 alopecia, nausea, nephrotoxicity, are likely and it /QQTGP # 7NEWU 4QFGPU 1RJVJCNOKCVTKUEJG must be administer in close observation of physician. Beobachtungen. Berlin, A. Hirschwald:107-110, 1867 4. Nettleship A, Brkic S, Vackonic S: Chronic Serpiginous Additional surgical procedures: 7NEGTQHVJG%QTPGC /QQTGPŏUWNEGT 6TCPU1RJVJCNOQN Small perforations / Impending perforation may be 5QE7- treated with application of tissue adhesive – isobutyl 5. Wood T, Kaufman H: Mooren’s ulcer. Am J cyanoacrylate and placement of a soft contact lens Ophthalmol 71:417-422, 1971 to provide comfort and to prevent dislodging of the 6. Keitzman B. Mooren’s ulcer in Nigeria. Am J glue. Ophthalmol 65:679-685, 1968 7. Lewallen S, Courtright P: Problems with current When a perforation is too large for tissue adhesive concepts of the epidemiology of Mooren’s corneal ulcer. to seal the leak, some type of patch graft will be Ann Ophthalmol 22:52-55, 1990 necessary. This may range from a small tapered plug 8. Majekodunmi AA. Ecology of Mooren’s ulcer in of corneal tissue to a penetrating keratoplasty. Nigeria. Doc Ophthalmol 49:211-219, 1980 5EJCP\NKP & /QQTGPŏU 7NEGTCVKQP +P 5OQNKP ) Rehabilitation: Thoft R, eds. The cornea. Boston. Little brown 1994, pp Once the active ulceration has stopped and the 408-414. TGOCKPKPI EQTPGC JCU DGGP EQORNGVGN[ QRCEKſGF KV 10. Wilson S, Lee W, Murakami C et al. Mooren’s is possible perform PK on these patients, even in the type Hepatitis C virus-associated corneal ulceration. face of a thinned and vascularized cornea. Ophthalmology 101:736-745, 1994 11. Moazami G, Auran J, Florakis G et al: Interferon Because of the immune systems remarkable memory, treatment of Mooren’s ulcers associated with hepatitis C. surgical attempts at rehabilitation in Mooren’s Am J Ophthalmol 119:365-366, 1995 ulceration, should be done only with concurrent 12. Brown S. Mooren’s ulcer: Histopathology and immunosuppressant, even when the active disease proteolytic enzymes of adjacent conjunctiva. Br J has been arrested, or is burnt out because attempts Ophthalmol 59:670-674, 1975 at penetrating keratoplasty often are associated with 13. Young R, Watson P: Light and electron microscopy recurrence and graft failure. QH EQTPGCN OGNVKPI U[PFTQOG /QQTGPŏU 7NEGT $T , Ophthalmol 66:341-356,1982 Conclusion: 14. Ferguson E III, Carreno O: Mooren’s ulcer and Bilateral Mooren’s ulcer in young patients can progress delimiting keratotomy. South Med J 62:1170-1175, 1969 rapidly in a circumferential fashion towards the 15. Chow C, Foster CS: Mooren’s ulcer. Int Ophthalmol center of the cornea and can present with perforation Clin 36:1-13, 1996 and Iris prolapse early in a course of disease. Though 16. Dinzis P, Mondino B: Management of non-infectious ENKPKECNCRRGCTCPEGKUEJCTCEVGTKUVKEU/QQTGPŏU7NEGT corneal ulcers. Surv Ophthalmol 32:94-110, 1987 remains a diagnosis of exclusion and systemic diseases /QPFKPQ$ +PƀCOOCVQT[FKUGCUGUQHVJGRGTKRJGTCN CUUQEKCVGF YKVJ 27- OWUV DG TWNGF QWV %WTTGPV cornea. Ophthalmology 95:463-472, 1988 treatment options and work up have resulted in a 18. Robin G, Schanzlin D, Verity S et al. Peripheral UKIPKſECPVKORTQXGOGPVKPVJGRTQIPQUKUQHRCVKGPVU corneal disorders. Surv Ophthalmol 31:1-36, 1986 YKVJ/QQTGPŏU7NEGT9KVJCRRTQRTKCVGOCPCIGOGPV 5CPIYCP85 44 DJO Vol. 20, No.4, April-June, 2010 History of Ophthalmology Glaucoma Drainage Devices: A Historical Perspective Shibal Bhartiya Cornea and Glaucoma Services, Dr R P Centre for Ophthalmic Sciences, AIIMS, New Delhi, The history of glaucoma drainage devices (GDD) is Molteno tube remains the yardstick against which one of triumph of the human imagination spirit and other tube devices are compared. KPIGPWKV[ +VŏU C UVQT[ QH VJG KPFQOKVCDNG UEKGPVKſE Generation 3: Posterior Drainage spirit of inquiry, and of sheer perseverance and the 6QCXQKFRTQDNGOUNKMGFGNNGPHQTOCVKQPRQQTſNVTCVKQP resilience of the clinicians’ spirit in not succumbing associated with pre-existing anterior conjunctival to a disease. scarring, and extrusion, posterior placement beneath The earliest references of a seton (literally, hair) is a Tenon’s capsule soon came into vogue. Subsequent rather scary attempt in 1906, to drain a hypopyon by tube and plate GDDs share the essential design placing a horse hair through a corneal paracentesis. EQPEGRV QH RQUVGTKQT ſNVTCVKQP XKC C VWDG KP VJG The same technique was later applied to treat two anterior chamber to a plate element secured beneath patients with painful absolute glaucoma. Tenon’s capsule. These include the Molteno, Krupin, Anecdotal reports of attempts to shunt aqueous to a Baerveldt, Ahmed, and OptiMed GDDs. variety of unconventional sites, including the vortex Generation 4: External Resistance mechanisms veins and the nasolacrimal duct were instrumental in The next landmark innovation is also attributable to the evolution of the current generation of implants. the genius of Molteno who recognized that resistance What these did succeed in doing was to direct the VQƀQYFKUVCNVQVJGUENGTQUVQO[IGPGTCNN[TGOCKPU clinician to the fact that the optimal site of drainage low until limited subconjunctival wound healing was the subconjunctival space. JCU QEEWTTGF CPF VJG KPKVKCNN[ CIITGUUKXG ſNVTCVKQP Generation 1: Translimbal GDDs becomes more limited. 6JGſTUVVTCPUNKODCN)&&TGRQTVGFD[ 0GYGT/QFKſECVKQPU The current GDDs have undergone major metamorphosis in terms of the materials used for manufacture of the plates. The tubes that shunt aqueous from the AC to the plate are almost always made of silicon. The materials used for manufacture of the plates include elastomeric silicone (polydimethylsiloxane), silicone, PMMA, and other hydrophobic polymers. The tubes placement has also undergone innovations with an alternate site of placement in the posterior chamber, or the vitreous cavity. The size of the plates of the implant are seen to vary from 134 and 268 sq mm for the single and double plate Molteno to 250, 350, 425, 500 sq mm for Figure 2 Ahmed Glaucoma Valve Baerveldt’s implants. Newer Implants SOLX Gold Shunt GMS Plus is a 24 karat gold 3x6 mm rectangle which contains numerous micro- channels that bridge the anterior chamber and the suprachoroidal space, thus controlling aqueous QWVƀQY The express minishunt is a stainless steel device which consists of a Shaft with spur and external plate with a scleral slot. Its size is 400 microns, with an external diameter 27G. It acts by shunting aqueous out of the aqueous into the subcojunctival space The T Flux implant is a T shaped poly-MEGMA, J[FTQRJKNKE CET[NKE VJCV FTCKPU ƀWKF D[ OGCPU QH Figure 3 Gold SOLX implant capillarity and osmosis. It has been designed for use with non-penetrating glaucoma surgery Figure 4 Express Glaucoma Shunt Fig 1 Baerveldt and Molteno Implants 46 Glaucoma Drainage Devices: A Historical Perspective DJO Vol. 20, No.4, April-June, 2010 Table 1: Landmark Dates in the history of GDD: 1907 Rollet and Moreaui implanted horse hair head to drain fluid out of anterior chamber 1912 Silk thread as first translimbal GDD implanted by Zorab 1959 Epstein unsuccessfully inserted a polythene tube in an attempt to drain fluid, failed because of excessive scar formation near the limbus , seton migration, and conjunctival erosion. 1969 Molteno introduced the concept of large surface area required to disperse the aqueous (Acrylic tube with thin plate at limbus) 1976 Molteno implant with a long silicone tube attached to a large end plate placed 9-10 mm posterior to the limbus . 1976 Krupin introduced the first pressure sensitive unidirectional valve (opening-11mm Hg, closing-9 mm Hg) provided resistance to the flow of aqueous and prevented early postoperative hypotony 1981-Double plate valve by Molteno 1992 George Baerveldt recognises that increase in the surface area of the end plate(s) results in lower IOPs . Introduced a nonvalved silicone tube attached to a large barium impregnated silicone plate with a surface area of 250 mm², 350 mm², or 500 mm ² 1993 Ahmed introduced AGV 1997 Introduction of the Helies drainage device which uses an artificial meshwork of PTFE fibers 1998 Glaucoma Drainage Devices have been implanted in 2,980 patients 2001 FDA approved the AquaFlow Collagen Glaucoma Drainage Device as an alternative treatment for open-angle glaucoma 47 Glaucoma Drainage Devices: A Historical Perspective DJO Vol. 20, No.4, April-June, 2010 References chamber tube shunt to an encircling band in the treatment 1. Rollett M, Moreau M. Traitement de hypopyon par of neovascular glaucoma. Ophthalmology 1982;89:1188– le drainage capillary de la chambre anterieure. Rev Gen 94. Ophtalmol 1906;25:481–9. 17. White TC. A new implantable ocular pressure relief 2. Rollett M, Moreau M. Le drainage au crin de la device. A preliminary report. Glaucoma 1985;7:289–94. chambre anterieure contre l’hypertonie et la douleur. Rev 18. Joseph NJ, Sherwood MB, Trantas G, et al. A one piece Gen Ophtalmol 1907;26:289–92. drainage system for glaucoma surgery. Trans Ophthalmol 3. Lee P-F, Wong W-T. Aqueous-venous shunt 5QE7- Ō for glaucoma: report on 15 cases. Ann Ophthalmol 19. Fellenbaum PS, Almeida AR, Minckler DS, et al. 1974;6:1083–8. Krupin disk implantation for complicated glaucoma. 4. Mascati NT. A new surgical approach for the control Ophthalmology 1994;101:1178–82. of a class of glaucomas. Int Surg 1967;47:10–15. 20. Lloyd MA, Baerveldt G, Heuer DK, et al. The Baerveldt 5. Zorab A. The reduction of tension in chronic glaucoma. glaucoma implant—long-term histologic studies in rabbits Ophthalmoscope 1912;10:258–61. and clinical experience in humans. Invest Ophthalmol Vis 6. Stefansson J. An operation for glaucoma. Am J Sci 1991;746. Ophthalmol 1925;8:681–93. 21. Jacob J, Stalmans I, Zeyen T. Ahmed and Baerveldt $KEM/97UGQHVCPVCNWOHQTQEWNCTFTCKPCIG#TEJ glaucoma drainage implants: long-term results and Ophthalmol 1949;42:373–88. HCEVQTUKPƀWGPEKPIQWVEQOG$WNN5QE$GNIG1RJVCNOQN 8. Muldoon WE, Ripple PH, Wilder HC. Platinum implant 2009;313:19-29. in glaucoma surgery. Arch Ophthalmol 1951;45:666–72. 22. Reinthal EK, Rohrbach JM, Grisanti S. Glaucoma 9. Row H. Operation to control glaucoma: preliminary drainage implants. Klin Monbl Augenheilkd. 2010 report. Arch Ophthalmol 1934;12:325–9. ;227:49-55. 6TCPEQUEQ/77UGQHVCPVCNWOKORNCPVUHQTKPFWEKPI 23. Thieme H.Glaucoma drainage devices. Ophthalmologe. a permanent hypotony in rabbits’ eye. Am J Ophthalmol 2009 ;106:1135-46. 1949;32:499–508. 24. de Jong LA.The Ex-PRESS glaucoma shunt versus 11. Gibson G. Transcleral lacrimal canaliculus transplants. trabeculectomy in open-angle glaucoma: a prospective Trans Am Ophthalmol Soc 1942;40:499–515. randomized study. Adv Ther. 2009 ;26:336-45. 12. Losche W. Vorschlage zur Verbesserung der 25. Melamed S, Ben Simon GJ, Goldenfeld M, Simon Zyklodialyse. Klin Monatsbl Augenheilkd 1952;121:715– )P'HſECE[CPFUCHGV[QHIQNFOKETQUJWPVKORNCPVCVKQP 16. to the supraciliary space in patients with glaucoma: a pilot 13. Bietti GB. The present state of the use of plastics in study. Arch Ophthalmol. 2009 ;127:264-9. eye surgery. Acta Ophthalmol 1955;33:337–70. 26. Barton K, Heuer DK. Modern aqueous shunt 14. Molteno ACB. New implant for drainage in glaucoma. implantation: future challenges. Prog Brain Res. Animal trial. Br J Ophthalmol 1969;53:161–8. 2008;173:263-76. 15. Krupin T, Podos SM, Becker B, et al. Valve implants 27. Minckler DS, Francis BA, Hodapp EA, Jampel HD, KPſNVGTKPIUWTIGT[#RTGNKOKPCT[TGRQTV#O,1RJVJCNOQN Lin SC, Samples JR, Smith SD, Singh K. Aqueous shunts 1976;81:232–5. in glaucoma: a report by the American Academy of 16. Schocket SS, Lakhanpal V, Richards RD. Anterior Ophthalmology. Ophthalmology. 2008;115:1089-98. 48 DJO Vol. 20, No.4, April-June, 2010 Instruments Scan Pediatric Electrophysiology Dr. Jitendra Jethani, Dr. Thakorbhai Pediatric Ophthalmology V Patel Eye Institute Haribhakti Complex, Salatwada Vadodara Introduction ERG (Electroretinogram) Electrophysiology, especially in children, can The ERG is the mass electrical response of the retina EQPVTKDWVG C UKIPKſECPV NQV +V KU QPG QH VJG to luminance stimulation and is recorded using corneal most underutilized diagnostic test in pediatric electrodes. The stimuli are delivered by a Ganzfeld ophthalmology in our country. The tests are objective, (Fig 1) bowl, an integrating sphere enabling uniform safe, noninvasive, swift and easy to administer. Also, YJQNG ſGNF KNNWOKPCVKQP 6JG )CP\HGNF RTQXKFGU they can give unique insight into the functional DQVJƀCUJUVKOWNCVKQPCPFCFKHHWUGDCEMITQWPFHQT integrity of different levels of the visual pathway. photopic adaptation. The Krubsfeld is the hand held It is really advantageous to use visual electrodiagnostic bowl used for ERG under general anesthesia. The tests in preliminary disease stages in young infants. It reference electrodes are positioned at the ipsilateral enhances the chances of successful surgical or clinical outer canthi if a bipolar contact lens electrode is not intervention and allows genetic counseling when it used. is most pertinent to young parents. It also helps in emotional acceptance and allows for more easier implementation of educational programs. Despite advances in imaging methods, its application in young children is still questionable. What does electrophysiology encompass? The electroretinogram (ERG) indicates the massed TGVKPCN TGURQPUGU VQ VJG HWNN ſGNF NWOKPCPEG UVKOWNCVKQP YJKEJ TGƀGEVU VJG HWPEVKQP QH VJG photoreceptors and the inner nuclear layers of the retina, the electro-oculogram (EOG) expresses retinal pigment epithelium (RPE) function and the interaction between the RPE and the (rod) photoreceptors and the XKUWCNGXQMGFRQVGPVKCN 8'2 TGƀGEVUQRVKERCVJYC[ function beyond the eye to the visual cortex. These tests complement, and supplement, other visual methods of assessment. Thus, an abnormal ERG may suggest the necessity for metabolic screening and an abnormal VEP in association with a normal ERG can indicate the need for structural imaging studies. The results of visual electrophysiological tests are usually displayed as graphs of voltage (in microvolts) plotted against time (in milliseconds). These graphs have characteristic waveforms and the constituent Figure 1 Ganzfeld bowl RQUKVKXG CPF PGICVKXG RGCMU CTG SWCPVKſGF D[ VJGKT latency (called implicit time by some workers), relative to the onset of stimulus delivery, and their Various types size (amplitude) relative to the previous peak or an In response to retinal illumination, some retinal cells estimated baseline. hyperpolarize and other depolarize. The gross effect 49 Pediatric Electrophysiology DJO Vol. 20, No.4, April-June, 2010 of these summed biopotentials is recordable at the photocurrent response is determined by the upper front surface of the eye as a series of negative and limit of dark, or circulating current available. (Figure RQUKVKXGXQNVCIGEJCPIGU RGCMU KPVJGſTUVOU 2) after the light stimulation. These series of peaks are the ERG. The form and timing of the ERG is related to the eye’s state of light adaptation and to the intensity, spatial, chromatic and temporal characteristics of the stimulus. 6JGƀCUJGNGEVTQTGVKPQITCOKUIGPGTCVGFD[CWPKHQTO ƀCUJQHNKIJVGIHTQOVJGNKIJVƀCUJGFKPC)CP\HGNF QT D[ .'&U VJCV TGƀGEVU GNGEVTKECN CEVKXKV[ HTQO most of retina. A pattern ERG (pERG) is recorded when a structured stimuli are used; for example, checkerboards or ITCVKPIU7UWCNN[R'4)UCTGTGEQTFGFWUKPIUVKOWNK localized to the macular and paramacular areas. There is no overall change in mean retinal luminance, and light scatter within the eye is minimized. Multifocal ERG (mERG) is obtained by stimulating the central 300 to 500 of the retina with a contiguous CTTC[QHƀKEMGTKPIJGZCIQPU/WNVKRNGJGZCIQPUCTG simultaneously alternated, but the m-sequence determines that no pattern of simultaneous hexagon Figure 2 Normal ERG wave is repeated twice in the sequence. There are important clinical advantages in separately The b wave is a corneal positive component; b assessing the rod and cone activity. Rods can detect wave activity is associated predominantly with the single light quanta in dark backgrounds. Rod ERGs depolarisation of on centre bipolar cells. are slower responders than cone ERGs, as they are TGƀGEVKPI C NQPIGT RCVJYC[ VJTQWIJ VJG TGVKPC 6JG rod pathway is preferentially activated by dim, short wavelength light stimuli or by a very dim white ƀCUJGU RTGUGPVGF CV NGUU VJCP U WPFGT FCTMGPGF (scotopic) laboratory conditions. The cone pathways are preferentially stimulated by high intensity white NKIJVCPFNQPIGTYCXGNGPIVJƀCUJGURTGUGPVGFWPFGT RJQVQRKE EQPFKVKQPU CU YGNN CU D[ HCUV ƀCUJ TCVGU delivered above 15/s. Origins of waves Light stimulation of both rods and cones causes transient and sustained changes in the extracellular ion Figure 3 Oscillatory potentials composition. The movement of these ionic currents through the membrane and interstitial resistance of Oscillatory potentials (OPs) (Figure 3) are a series Muller cells give rise to an associated voltage change of high frequency positive wavelets. OPs overlap and this mass dipolar response is detectable at the with the rising phase of b wave. Early wavelets of front surface of the eye as ERG. 12UTGƀGEVTQFHWPEVKQPCPFőQPŒRCVJYC[UCPFNCVGT Granit, in 1933, suggested that three processes are in wavelets are linked with the cone system and “off ” KPXQNXGFKPIGPGTCVKPIVJGƀCUJ'4) RTQEGUU+ 2+ KU pathway activity. the main contributor to the c wave (EOG), PII to the The c wave is a slow positive going wave, shows b wave and P III to the a wave. variable presence among healthy controls, mainly as The a wave is the main corneal negative wave. The it represents the interaction of two retinal potentials C YCXG KU C TGƀGEVKQP QH OCUU J[RGTRQNCTKUCVKQP QH with opposite polarity and similar time course. photoreceptors. The maximum amplitude of the The d wave is a positive wave that is closely associated 50 Pediatric Electrophysiology DJO Vol. 20, No.4, April-June, 2010 with a reduction in light under photopic conditions. master the technical requirements of their chosen Flicker ERGs elicited by stimuli rates greater than 20/ electrode, to ensure good ocular contact, to ensure UTGƀGEVEQPGRJQVQTGEGRVQTCEVKXKV[ proper electrode impedance, to ensure that waveforms 6JG2'4)KUV[RKECNN[CDKRJCUKEEQPſIWTCVKQPYKVJC CTGEQORCTCDNGVQUVCPFCTF'4)UCPFVQFGſPGDQVJ positivity at 50ms (P 50) and a negativity at 95 (N95). normal values and variability (which may be different 2 KPFKECVGU OCEWNCT HWPEVKQP CPF 0 TGƀGEVU with different electrodes) for their own lab. Skin ganglion cell function electrodes are not generally recommended as active recording electrodes. The machine: parameters & equipment Recording electrodes Electrodes that contact the cornea or nearby bulbar conjunctiva are strongly recommended for basic HWNNſGNF TGEQTFKPI 6JGUG KPENWFG EQPVCEV NGPU GNGEVTQFGU EQPFWEVKXG ſDGTU CPF HQKNU EQPLWPEVKXCN loop electrodes and corneal wicks. For most users, contact lens electrodes (Figure 4) will provide the highest amplitude and most stable recordings; such electrodes should be centrally transparent with an optical opening as large as possible, and incorporate a device to hold the lids apart. The corneal surface should be protected during use with a non-irritating and non-allergenic ionic conductive solution that is relatively non-viscous (e.g., no more viscous than 0.5% methyl cellulose). Figure 5 Reference electrodes Reference electrodes may be incorporated into the contact lens-speculum assembly to make contact with the conjunctiva (‘bipolar electrodes’). This is the OQUVUVCDNGEQPſIWTCVKQPGNGEVTKECNN[#NVGTPCVKXGN[ electrodes can be placed near each orbital rim temporally as a reference for the corresponding eye. Figure 4 Contact lens electrode More viscous solutions can attenuate signal amplitude. Other types of corneal and conjunctival electrodes require more skill to use but may have EGTVCKPCFXCPVCIGU7UGTUUJQWNFDGCYCTGVJCVUKIPCN amplitude is reduced as the point of ocular contact moves away from the corneal apex. Topical anesthesia is necessary for contact lens electrodes but may not be required for other types of corneal and conjunctival electrodes. It is necessary that all electrophysiologists Figure 5 Ground electrodes 51 Pediatric Electrophysiology DJO Vol. 20, No.4, April-June, 2010 A separate skin electrode should be attached to an electrode position and compliance in order to avoid indifferent point and connected to ground. Typical artifactual recordings. Burian Allen electrodes are locations are on the forehead or ear. good for children, although they are more preferable Skin reference electrode characteristics in children under anesthesia. The skin should be prepared by cleaning, and a suitable conductive paste or gel used to insure good Normal values and measurement electrical connection. The ERG matures during infancy, and newborn and infant signals must be interpreted with great caution. Stimulus duration Later infantile and young childhood ERGs approach The standard is based on stimuli of duration adult waveform and size. Pediatric ERGs should considerably shorter than the integration time of any ideally be compared to those from normal subjects photoreceptor. Thus, the light stimulus should consist of the same age, even though there may be little QH ƀCUJGU JCXKPI C OCZKOWO FWTCVKQP QH CDQWV normative data available. Because movement and ms. RQQTſZCVKQPECPOCMGRGFKCVTKETGEQTFUXCTKCDNGKP amplitude and waveform, several repetitions of each Clinical Protocol ERG be recorded in order to recognize reproducible Pupillary dilation waveforms and choose the best examples. Pupils should be maximally dilated for all ERG EOG (Electro oculogram) recordings and pupil size should be noted. The EOG is a measure of the function of the RPE Pre-adaptation to light or dark and the interaction between the RPE and the The recording conditions outlined specify 20 min of RJQVQTGEGRVQTU6JGRCVKGPVOCMGUCſZGFFGITGG dark adaptation before recording rod ERGs, and 10 lateral eye movement during a period of 20 min dark min of light adaptation before recording cone ERGs. adaptation and then during a 12-15 min period of light If contact lens electrodes are used, the wearing time adaptation. the eye movements are made every 1-2 ECPDGOKPKOK\GFD[FCTMCFCRVKPIſTUVCPFKPUGTVKPI seconds for approximately 10 seconds every min. the electrodes under dim red light at the end of the The amplitude of signal recorded between electrodes adaptation period. However, care should be used to positioned at medial and lateral canthi reaches a avoid too bright a red light, and an additional 5 min minimum during dark adaptation, the dark trough, and of dark adaptation may be needed for recovery after a maximum during light adaptation, the light peak. lens insertion. The development of a normal light peak requires Fixation normally functioning photoreceptors in contact with a #ſZCVKQPRQKPVUJQWNFDGKPEQTRQTCVGFKPVQUVKOWNWU PQTOCNN[HWPEVKQPKPI42'6JG'1)KUSWCPVKſGFD[ domes. A stable eye is important so that eye movements calculating the size of the light peak in relation to the do not alter the optimal corneal electrode position, dark trough as a percentage, the Arden index. A normal produce electrical artifacts, or allow blockage of light EOG light rise is >175% for most laboratories. by the electrode or eyelid. VEP (Visual Evoked potentials) Sedation or anesthesia The VEP is normally largest in the midline, around 3 7PEQORNKCPVEJKNFTGP GURGEKCNN[CIGUŌHQTYJQO to 5 cm above the inion. VEPs are elicited to stimulate EQPVCKPOGPVECPDGFKHſEWNV OC[DGEQOGEQORNKCPV C YKFG EGPVTCN CTGC QH VJG XKUWCN ſGNF (QT ENKPKECN with oral sedation or anxiolysis. Medical guidelines RWTRQUGU WPKHQTO NKIJV ƀCUJGU CPF EJGEMGT DQCTF should be followed with respect to indications, risks, pattern stimuli are usually used. medical monitoring requirements and the choice of a sedative / relaxant versus general anesthesia. Considering the variability of pediatric records, there will generally be little effect on ERG amplitude or waveform with sedation although full anesthesia may modify the ERG. Electrodes Contact lens electrodes are applicable to infants and young children, but pediatric sizes will be required with speculum-containing models, and care must be used to minimize corneal and psychological trauma. Special care is required with children to monitor Figure 7 Cortical origins of VEP 52 Pediatric Electrophysiology DJO Vol. 20, No.4, April-June, 2010 8'2U TGƀGEV UWTHCEG CEVKXKV[ QH EQTKVECN I[TK CPF intersubject variability than the pattern reversal VEP VJGTGHQTG OCKPN[ TGƀGEV CEVKXKV[ QH CTGCU QH VJG but shows less sensitivity to confounding factors such XKUWCNſGNFTGRTGUGPVGFCVVJGUWTHCEGQHI[TK2 CURQQTſZCVKQPQTG[GOQXGOGPVU EQORQPGPV QH VJG ƀCUJ 8'2 CTKUGU HTQO EQTVKECN activation by the retino geniculate pathway. DIAGNOSTIC APPLICATIONS Flash VEP is used mainly when visual acuity is poor ERG or when cooperation if limited. This is a complex 6JGƀCUJ'4)RNC[UKVUOQUVXKVCNTQNGKPEQPFKVKQPU waveform and shows great variability. where ophthalmoscopy shows a normal or questionably Pattern reversal VEP is usually elicited by checks that normal, fundus and optic disc appearance and yet the alternate from black to white and vice versa. child seems to have poor vision. Pattern onset VEPs are elicited by the brusque Both rods and cones are affected appearance of a pattern usually lasting between 100 In Leber’s congential amaurosis, both rod and cone and 300 ms. It is used more widely in clinical practice ERGs are most usually non-detectable. Concurrent as its waveform is maintained across the life span. In 8'2 JGNRU CU VJG RTGUGPEG QH ƀCUJ 8'2 CEVKXKV[ older children, it is useful in identifying the abnormal indicates cases who are likely to have some degree of pathway projection associated with albinism. rudimentary vision as opposed to complete blindness Predominantly rods are affected How are the waves recorded? various parameters Rod dysfunction can occur in isolation as in retinitis pigmentosa (RP) or linked with a wide variety of Electrodes EQPFKVKQPUKORQTVCPVN[XKVCOKP#FGſEKGPE[ Standard silver-silver chloride or gold In the early stage, ERGs show a partial preservation disc surface electrodes are recommended for recording of cone ERG activity and sometimes mixed rod/ 8'2U6JGGNGEVTQFGUUJQWNFDGſZGFVQVJGUECNRCPF cone activity attenuation. In early and middle maintained using procedures recommended by the EJKNFJQQF8'2UVQƀCUJCPFRCVVGTPUVKOWNCVKQPCTG manufacturer. commonly of normal size and latency which as the disease progresses P VEP is delayed and reduced in Electrode placement amplitude. The scalp electrodes should be placed relative to Predominantly cones are affected bony landmarks, in proportion to the size of the head, In children with rod monochromatism according to the International 10/20 system. The (achromatopsia), the rod mediated ERG to dim white anterior/posterior midline measurements are based QTDNWGƀCUJKUWUWCNN[PQTOCNDWVVJGYJKVGƀCUJEQPG on the distance between the nasion and the inion mediated ERG elicited under photopic conditions and over the vertex. The active electrode is placed on the *\ƀKEMGTTGURQPUGKUPQVFGVGEVCDNG6JG8'2KU scalp over the visual cortex at Oz with the reference KPXCTKCDN[ UKIPKſECPVN[ CVVGPWCVGF FGITCFGF CPF electrode at Fz. Commonly used ground electrode delayed. positions include the forehead, vertex (Cz), mastoid, Inner retinal dysfunction earlobe (A1 or A2) or linked earlobes. 6JGPQTOCNDTKIJVƀCUJOKZGFTQFEQPG'4)JCUCD wave that is 1.5 to 2 fold the size of the a wave. When Clinical protocol the b wave amplitude is markedly attenuated or not Preparation of the patient detectable, the mixed ERG has a larger than average Pattern stimuli for VEPs should be presented when the a wave. The negative ERG morphology indicates pupils of the eyes are unaltered by mydriatic or miotic preserved photoreceptor activity and dysfunction of drugs. For pattern stimulation, the visual acuity of the the inner retina layers, usually affecting the bipolar patient should be recorded and the patient should be and Muller cells. The negative ERG is associated optimally refracted for the viewing distance of the with X linked congenital stationary night blindness, X screen. Monocular stimulation should be performed. linked retinoschisis, Bull’s eye maculopathy, infantile In infants or some other special populations, when and juvenile neuronal ceroid lipofuschinosis. binocular stimulation may be used to assess whether any afferent signals are reaching primary visual Visual Evoked potential (VEP) EQTVGZ9JGPCƀCUJUVKOWNWUKUWUGFYKVJOQPQEWNCT stimulation, care should be taken to ensure that no ERG and VEPs recorded together can give valuable light enters the unstimulated eye. information in pediatric population regarding the The pattern onset /offset response shows a greater macular and extramacular areas and the quality of 53 Pediatric Electrophysiology DJO Vol. 20, No.4, April-June, 2010 macular pathway function. benchmark and are particularly useful when comparing Ocular opacity the two eyes that is the interocular differences in the $TKIJV ƀCUJGU WUWCNN[ RGPGVTCVG CNN DWV VJG FGPUGUV acuity QEWNCT QRCEKVKGU CPF ƀCUJ 8'2U CPF '4)U CTG What is pattern and multifocal? particularly valuable when ocular opacities prevent adequate visualization of the perimacular fundus PERG The PERG is the response of central retina to and Optic nerve disorders iso-luminant stimulus, usually a reversing black Monocular stimulation and transoccipital multichannel and white checkerboard. It allows both a measure of VEP recording will help to indicate whether a disease central retinal function and and evaluation of retinal process is affecting the optic nerve, chiasm or pathway ganglion cell function. beyond the chiasm. The transient PERG has two components: P 50 Optic Neuritis. In optic nerve demyelination related to at approx 50 ms and N95 at 95 ms. Measurement optic neuritis with visual involvement, the P VEP is concentrates on the amplitude of P50 from the trough almost invariably markedly delayed to non detectable. of the early negative N35 component. N 95 is a Once the acuity has improved P VEP levels rise to contrast related component generated in the retinal moderate levels which is unlike adulthood cases ganglion cells. (Figure 8) YJGTGVJG[OCKPVCKPUKIPKſECPVFGNC[U Optic nerve hypoplasia. In moderate hypoplasia cases the VEP is variably attenuated probably related to the degree of hypoplasia. In severe cases the VEP are markedly attenuated In Optic nerve compression, the PVEP is usually degraded, attenuated and mildly delayed in optic atrophy caused by compressive ischemic or degenerative conditions. (QT ETCPKQHCEKCN F[UQUVQUKU ƀCUJ CPF 2 8'2U can provide an early indication of visual pathway dysfunction in syndromic and non syndromic craniosynostonosis. Optic nerve glioma usually leads to a broadening, attenuation and mild delay of the P VEP from the affected eye. Albinism The crossed asymmetry VEP distribution in albinism Figure 8 PERG wave is opposite to that of chiasmal compression or achiasma. In infancy, the “albino” crossed asymmetry The P50 component of the PERG is abnormal in ECPDGUJQYPD[WUKPICƀCUJUVKOWNCVKQP disorders of macular function with preservation of N95:P50 ratio. The N95 component (ganglion cell) of Perinatal hypoxia the PERG is usually selectively affected if the PERG 6JG ƀCUJ 8'2 FWTKPI VJG CEWVG UVCIG ECP IKXG CP is abnormal in optic nerve disease but the PERG may indication of the degree of visual function and be normal. eventual outcome. Amblyopia: Visual testing? Sweep VEP Objective estimates of visual acuity can be inferred by P VEP amplitude tends to fall of linearly with spatial noting the smallest pattern size to consistently elicit a frequency near the limit of acuity. The extrapolation P VEP when refractive error is corrected. of high frequency limb of the spatial tuning function A strabismic amblyope may give higher resolution to zero amplitude produces an intercept that correlates acuity than recognition acuity secondary to spatial with subjective visual resolution; this has been the distortion. VEP acuity is also a different measure and basis of the sweep VEP techniques. it is unrealistic to expect a direct relationship between Sweep techniques can progressively and rapidly test objective and behovioral acuity measures. (usually in 8-16 s) a range of spatial frequencies *QYGXGT VJG 2 8'2 ſPFKPIU FQ RTQXKFG C IQQF (checks or gratings) to determine a VEP threshold. 54 Pediatric Electrophysiology DJO Vol. 20, No.4, April-June, 2010 Each spatial frequency is presented for a discrete Reporting an ERG interval (usually for 5-10 s) and the change in pattern The standards for reporting an ERG (by ISCEV) is to size can be continuous or sampled. QDVCKPſXG'4)U (KIWTG '4)VQCYGCMƀCUJ CTKUKPIHTQOVJGTQFU KPVJG Multifocal ERG (MERG) dark-adapted eye 6JG /'4) RTQXKFGU CP ő'4) XKUWCN ſGNFŒ QH VJG '4)VQCUVTQPIƀCUJKPVJGFCTMCFCRVGFG[G electrical response of the retina in different locations (3) Oscillatory potentials extending out to about 250 radius. It can be used to '4)VQCUVTQPIƀCUJ CTKUKPIHTQOVJGEQPGU KP FKHHGTGPVKCVG YJGVJGT C XKUWCN ſGNF FGHGEV KU FWG VQ the light-adapted eye retinal or optic nerve disease. '4)UVQCTCRKFN[TGRGCVGFUVKOWNWU ƀKEMGT 5KPEG VJG ſTUV QTFGT UKIPCNU CTG IGPGTCVGF HTQO 6JGſTUVCTGVJGUEQVQRKE FCTMCFCRVGF TGURQPUGU the photreceptor and bipolar cells in the retina, the and the last 2 are the photopic (light adapted) standard MERG will pick up diseases of the outer responses. retina and inner retina but will be normal in cases where the ganglion cells or optic nerve is injured. ERG and VEP in clinical practice When ERG and VEP are used together, they help KPſPFKPIQWVYJGVJGTVJGP[UVCIOWUQTCHCKNWTGQH DCD[VQſZCPFHQNNQYKUUGEQPFCT[VQCPFF[UHWPVKQP in the anterior visual pathway or it is otherwise. 6JG[ECPJGNRKPſPFKPIQWVVJGNGXGNQHVJGENPKECN problem in the sense that it determines the level like at photoreceptor level (rods or cones), the inner retinal layer, optic nerve or at the chiasm or postchiasmal pathway. Figure 9 Standard ERG pathway. Reporting a VEP A minimum of two recordings of each VEP condition should be acquired, measured and displayed. Reports of the standardized conditions should specify the normal normal P50 normal P50 P50 UVKOWNWURCTCOGVGT VJGſGNFUK\GQHVJGUVKOWNWUVJG N95 abnormal abnormal abnormal UVTGPIVJ VKOGKPVGITCVGFNWOKPCPEG QHVJGƀCUJQT mean luminance of the pattern, the pattern element size and contrast of pattern stimuli, the frequency of stimulation, the eye tested and the recording PVEP PVEP PVEP ERG ERG RCTCOGVGTU VJGſNVGTUGVVKPIUCPFVJGNQECVKQPUQHVJG positive (i.e., active) and negative (i.e., reference) and indifferent (i.e., ground) electrodes. normal abnormal abnormal normal abnormal non- optic nerve optic nerve maculopathy generalised organic dysfunction dysfunction retinal dysfunction (from Holder GE. Prog Ret Research 2001; 20: 531-61) +PQNFGTKPHCPVUVJGUGVGUVUECPJGNRVQſPFKPIQWVYJ[ the patching is not responding, it becomes an objective indicator of posterior hemisphere dysfunction with XKUWCNſGNFNQUU It also helps in older children with complaints of headache or possible functional visual loss. 55 Pediatric Electrophysiology DJO Vol. 20, No.4, April-June, 2010 Exercises The ERG waveforms of left eye are shown in the Case 1 (Figure 10) picture. The scotopic responses are good. The The 1st wave form shows response which is less than RJQVQRKETGURQPUGUCTGXGT[UOCNN6JG*\ƀKEMGT normal. The 2nd waveform shows a good response revealed a increased latency and a reduced amplitude. for full threshold scotopic waveforms. Oscillatory The diagnosis of ametropic amblyopia was changed RQVGPVKCN CPF RJQVQRKE YCXGHQTOU CTG ƀCV 6JG NCUV to cone dysfunction YCXGHQTO YJKEJ KU VJG *\ ƀKEMGT TGURQPUG KP RJQVQRKE EQPFKVKQPU UJQYU ƀCV TGURQPUG UWIIGUVKXG Case 4 (Figure 13) of a cone dysfunction. Case 2 (Figure 11) A 5 year old child presented with complains of loss of vision in RE. There was a history of pars First waveform that is scotopic –25dB response, plana vitrectomy with lensectomy for traumatic which shows delayed response and low amplitude. endophthalmitis and child had 3/60 vision. He was However, full threshold scotopic response which referred for amblyopia treatment. An ERG was shows a negative ERG. The a wave is prominent and FQPG CPF CNN VJG YCXGHQTOU YGTG HQWPF VQ DG ƀCV almost as big as b wave. Also, the cone responses suggestive of nil photoreceptor function and therefore are also normal with a sharp b wave (cone shaped the chances of improvement of vision and amblyopia pointed). This was suggestive of congenital stationary treatment were severely reduced which was informed night blindness to the parents Case 3 (Figure 12) Conclusion Electrophysiology is a wonderful diagnostic tool especially for children where the subjective responses CTGFKHſEWNVVQQDVCKPCTGUNQYCPFUQOGVKOGUCDUGPV and unreliable. References 1. Holder GE. The pattern electroretinogram and an intergrated approach to visual pathway diagnosis. Prog Ret eye research 2001; 20: 531 2. Sutter E. The interpretation of multifocal binary Kernels. Documenta Ophthalmologica. 2000; 100: 49-75 3. Marmor MF, Holder GE, Seeliger MW, Yamamoto S. Standard for clinical electroretinography (2004 update). A 8 year old child presented with complains of Documenta Ophthalmologica 108: 107–114, 2004. reduced vision in both eyes. He was using glasses 4. Odom JV, Bach M, Barber C, Brigell M, Marmor both eyes -2.5 D cycl 180 improving to 6/24 since MF, Tormene AP, Holder GE, Vaegan. Visual evoked 2 years. Stereoacuity was 1800 sec and retina was potentials standard (2004)Documenta Ophthalmologica 108: 115–123, 2004. 115 normal. 5. Kriss A, Thompson. Pediatric visual electrophysiology. The child was subjected to ERG and the waveforms Pediatric Ophthalmology and Strabismus, Second Edition. show a subnormal response for photopic thresholds. Wright KW, Spiegel PH, Springer, 2003. 56 DJO Vol. 20, No.4, April-June, 2010 Instruction to Authors Instruction to Authors Aims of the Journal .1675':%'. 5RTGCFUJGGV ſNGU 2+%6%*#46 Delhi Journal of Ophthalmology (DJO) is the quarterly ſNGU QT *CTXCTF ITCRJKE &Q PQV UGPF ITCRJU CPF journal published by Delhi Ophthalmological diagrams in freehand. The disk should be labeled Society. The DJO aims to become an easily readable YKVJVJGVKVNGQHVJGCTVKENGCWVJQTŏUPCOGVJGſNG fully referenced journal which will provide the name, and software used including version. 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Any may not be published elsewhere without permission RTQRTKGVCT[ QT ſPCPEKCN KPVGTGUV KP CP[ RTQFWEV mentioned in the manuscript should be stated on the from the Editor. title page. 59 Instruction to Author DJO Vol. 20, No.4, April-June, 2010 Revision of Manuscripts author(s) of that article will be given an opportunity Manuscripts sent for revision must be returned within to reply. The general instructions for authors should the time stipulated in the Editor’s letter. Failing this, be followed. The letters should be accompanied by the disclosure and copyright transfer statement. the manuscript must be resubmitted. Authorship is limited to three, and signatures of all Rejected Manuscript authors are required. The manuscripts of rejected articles are not returned due to high postal expenses. Book Reviews Book reviews should be accompanied by Miscellaneous photocopies (one set) of the title page (citing page Patients should have there identity concealed numbers, indexing, illustrations, year of publication, (including names and hospital numbers) or their publishing company), and contents page(s). photographs should be accompanied by the Journal Abstracts RCVKGPVŏUYTKVVGPRGTOKUUKQPVQRWDNKUJ#P[ſIWTG that has been published elsewhere should have an Abstracts of interesting articles published in other acknowledgment to the original source and proof journals may be submitted. Those contribution of permission to use from the holder of copyright. journal abstracts should include a photocopy of the Categories of Manuscripts published article. Review Articles Electronic Manuscript Reviewers write short articles in which they present This should accompany the paper text at the time developments in their topic, emphasizing the aspects QHUWDOKUUKQP7RQPCEEGRVCPEGHQTRWDNKECVKQPQT that, in their opinion are the most important. In at the time of revision when a manuscript is likely addition, they provide short annotations to the papers to be accepted for publication, the corresponding published in their topic during period reviewed. CWVJQTYKNNJCXGVQUWDOKVCPGNGEVTQPKEſNGQPFKUM This selected bibliography is printed at the end of in addition to the original manuscript. Disks that each review. Your review should be 10-14 typed are IBM PC compatible (non Macintosh) will be pages. The article should highlight and discuss all accepted. Floppy disks should be MS-DOS based in KPVGTGUVKPIFGXGNQROGPVUKP[QWTUWDLGEVCUTGƀGEVGF word perfect 5.1 of MS Word for windows. Files in in the recent literature. In addition to describing formats other than these should be converted to MS- recent trends, you are encouraged to give your own Word DOS text format (ASCII) before submission. opinions of the topics discussed. The disk should be labeled with the title of the article, However, be careful of expressing conclusions in CWVJQTŏU PCOG VJG ſNG PCOG CPF UQHVYCTG WUGF a way that might be construed as biased against a including version. The disk must contain exactly the particular researcher, product or manufacturer. same material as the revised manuscript including the tables, legends, and graphs. Graphs and line drawing/ Original Articles diagrams must be sent in graphic format, that is, EPS, Original article should generally not exceed 3,000 .1675':%'. 5RTGCFUJGGV ſNGU 2+%6%*#46 words or 12 double-spaced pages. ſNGU QT *CTXCTF ITCRJKE &Q PQV UGPF ITCRJU CPF diagrams in freehand. The disk should be sent in Brief Reports proper packaging to avoid damage and corruption These should not exceed 1000 words with a maximum QHVJGKPHQTOCVKQPFWTKPIVTCPUKV7PTGCFCDNGFKUMU of 4 illustrations. They should follow the following will be returned to the author substitution. Disk with format: introduction, case(s), and discussion. No their packaging will be returned to the author after more than 8 references should cited. Each brief use by the journal on request. report must begin with a 75-100 words summary Complimentary Copies VJCVJKIJNKIJVUVJGUKIPKſECPEGQHVJGCTVKENG$GUKFGU these requirements, the general instructions for Complimentary copies are sent to authors of author should be followed. published articles even if they are neither DOS members nor subscribers. This request should be Letter to the Editor made in the reprint order form. Comments about an article published in the journal, or topics of ophthalmic interest are considered. Errat Comments regarding articles in the journal should Substantial errors in published material are corrected be submitted within 3 months of publication, and the at the earliest possible after being brought to the notice of the Editorial Board. 60