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Time Amplitude (uV)
33 The Clinical Electro-Oculogram
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Fig. 33.8 Best’s vitelliform macular dystrophy. Upper panel-The EOG recordings for the right and left eyes show a marked reduction in the Arden or LP:DT ratio of approximately 1.1; Middle panel-color fundus image with characteristic central vitelliform lesions; Lower panel-
of the EOG can be abnormally low, resulting in a paradoxically normal or elevated LP:DT ratio [52].
Systemic drugs affecting the outer retina or RPE are also associated with a reduced or absent light-rise of the EOG [53]. Chloroquine retinopa-
Ocular coherence tomography (OCT) images of the mac­ula show a central pigmented area (with lipofuscin) of the detached epithelium, which will eventually degenerate. Images courtesy: Srikanta Padhy, MD
thy is associated with a reduced light-rise but may not be detected through multi-focal electro­retinography or color vision testing [54]. The iron chelator, deferoxamine, can cause a reduc­tion in the light-rise and dark trough amplitudes as it can damage the RPE [55]. The anti-epileptic
458
P. A. Constable
Fig. 33.9 Images and EOG of autosomal recessive bestrophinopathy (conrmed via genetic tests) in a 25-year-old male. The rst and second panels show a montage of the fundi with a hyperpigmented ring at the equator corresponding with hyper-uorescence of the RPE.The third panel shows OCT scans of the horizontal
and vertical meridians through the macula with a hypo­reective region in the right and left eyes corresponding with a separation of the RPE from the Bruch’s membrane. The fourth panel shows reduced or absent light-rise in the EOG, implicating the BEST1 gene in the potential differ­ential diagnosis. Images courtesy: Srikanta Padhy, MD
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drug, vigabatrin, is also linked to visual eld loss and reduced EOGs in children due to its toxicity to the outer retina [56]. Other drugs that change the EOG are: digoxin (elevates EOG), cisplatin (reduces the EOG), ethambutol (elevates the LP:DT ratio), and isotretinoin (reduces LP:DT ratios) [53]. A reduced light-rise has also been reported in tamoxifen retinopathy [57]. EOGs are not routinely performed to monitor the toxic effects of drugs since electroretinograms and reti­nal imaging can identify functional or structural changes in the retina ahead of any marked reductions in the EOG.Incidentally, the LP:DT ratio is reduced in seasonal affective disorder, which is characterized by depression in the win­tertime [58], and Parkinson’s Disease [59].
33.5 Clinical Interpretation
Clinically, the main utility of an EOG is in diag­nosing and monitoring conditions caused by mutations in the BEST1 gene, which lead to a functional loss of bestrophin in the RPE. The ratio of the light-rise peak to the dark trough min­imum is the main clinical measure. The ratio is reduced where there is a disruption to the rod­RPE interface, when bestrophin function is affected, and there is a reduction in the basolat­eral chloride conductance. However, care must
be taken to observe the amplitude of the dark trough as a reduced dark trough amplitude and a relatively normal LP:DT ratio may indicate dis­ruptions in the tight junctions. The timing of the light peak is slower in those aged 55years and older, and the LP:DT ratio also reduces at a rate of 0.13units per decade; these measures must be considered when recording/interpreting the EOG in older subjects [41]. Typically, the EOG is interpreted in conjunction with additional imag­ing and visual electrophysiological tests such as the electroretinogram; these, combined with genetic information and family history, are used to make a clinical diagnosis.
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24. Marmorstein AD, Marmorstein LY, Rayborn M, etal. Bestrophin, the product of the best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium. Proc Natl Acad Sci USA. 2000;97:12758–63.
25. Yu K, Xiao Q, Cui G, etal. The best disease-linked cl− channel hBest1 regulates ca V 1 (L-type) Ca2+ chan­nels via src-homology-binding domains. J Neurosci. 2008;28:5660–70.
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33. Mergaerts F, Daems E, Van Malderen L, Spileers W. Recording of the fast oscillations in the human electro- oculogram. Doc Ophthalmol. 2001;103:63–72.
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35. Arden GB, Wolf JE. The human electro-oculogram: interaction of light and alcohol. Invest Ophthalmol Vis Sci. 2000;41:2722–9.
36. Arden GB, Wolf JE. Differential effects of light and alcohol on the electro-oculographic responses of patients with age-related macular disease. Invest Ophthalmol Vis Sci. 2003;44:3226–32.
37. Gupta LY, Marmor MF. Sequential recording of photic and nonphotic electro-oculogram responses in patients with extensive extramacular drusen. Doc Ophthalmol. 1994;88:49–55.
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39. Türksever C, Orgül S, Todorova MG. Comparing short-duration electro-oculograms with and with-
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40. Constable PA, Ngo D, Quinn S, Thompson DA. A meta-analysis of clinical electro-oculography values. Doc Ophthalmol. 2017;135:219–32.
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42. Johnson AA, Guziewicz KE, Lee CJ, et al. Bestrophin 1 and retinal disease. Prog Retin Eye Res. 2017;58:45–69.
43. Toto L, Boon CJ, Di Antonio L, etal. Bestrophinopathy: a spectrum of ocular abnormalities caused by the c.614T>C mutation in the BEST1 gene. Retina. 2016;36:1586–95.
44. Guziewicz KE, Sinha D, Gómez NM, et al. Bestrophinopathy: an RPE-photoreceptor interface disease. Prog Retin Eye Res. 2017;58:70–88.
45. Mullins RF, Kuehn MH, Faidley EA, etal. Differential macular and peripheral expression of bestrophin in human eyes and its implication for best disease. Invest Ophthalmol Vis Sci. 2007;48:3372–80.
46. Burgess R, Millar ID, Leroy BP, etal. Biallelic muta­tion of BEST1 causes a distinct retinopathy in humans. Am J Hum Genet. 2008;82:19–31.
47. Khojasteh H, Azarmina M, Ebrahimiadib N, et al. Autosomal recessive bestrophinopathy: clinical and genetic characteristics of twenty-four cases. J Ophthalmol. 2021;2021:6674290.
48. Fouad YA, Tawk CA, Nowara M. Autosomal recessive bestrophinopathy presenting with a mac­ular hole retinal detachment. J Vitreoretin Dis. 2022;6:312–5.
49. Kaufman SJ, Goldberg MF, Orth DH, et al. Autosomal dominant vitreoretinochoroidopathy. Arch Ophthalmol. 1982;100:272–8.
50. Yardley J, Leroy BP, Hart-Holden N, etal. Mutations of VMD2 splicing regulators cause nanophthalmos and autosomal dominant vitreoretinochoroidop­athy (ADVIRC). Invest Ophthalmol Vis Sci. 2004;45:3683–9.
51. Chen CJ, Goldberg MF. Progressive cone dysfunc­tion and geographic atrophy of the macula in late stage autosomal dominant vitreoretinochoroidopathy (ADVIRC). Ophthalmic Genet. 2016;37:81–5.
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54. Neubauer AS, Samari-Kermani K, Schaller U, et al. Detecting chloroquine retinopathy: electro­oculogram versus colour vision. Br J Ophthalmol. 2003;87:902–8.
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56. Besch D, Kurtenbach A, Apfelstedt-Sylla E, et al. Visual eld constriction and electrophysiological changes associated with vigabatrin. Doc Ophthalmol. 2002;104:151–70.
57. Wang L, Miao H, Li X.Tamoxifen retinopathy: a case report. Springerplus. 2015;4:501.
58. Lam RW, Beattie CW, Buchanan A, etal. Low elec­trooculographic ratios in patients with seasonal affec­tive disorder. Am J Psychiatry. 1991;148:1526–9.
59. Economou SG, Stefanis CN.Changes of electroocu­logram (EOG) in Parkinson's disease. Acta Neurol Scand. 1978;58:44–52.
Computed Tomography:
Dacryocystography
34
NandiniBothra andSuryasnataRath
34.1 Introduction
A century ago, in 1909, Ewing performed the rst dacryocystography (DCG) to visualize the lacrimal abscess cavity and passages using X-rays. He used bismuth subnitrate as the con­trast material. However, the surrounding bone and soft tissue details were not discernible in this DCG [1, 2]. A modication of this technique, namely, digital subtraction, made the lacrimal passage stand out but failed to show the sur­rounding details. Nevertheless, details of the common canaliculus and nasolacrimal duct could be seen, making it a useful tool in cases with ste­nosis. Using X-rays was a disadvantage due to the dangers of radiation exposure [1]. This was overcome to some extent by the advent of com­puted tomography dacryocystography (CT-DCG) in 1990. This allowed the documentation of the bone and soft tissue anatomy and the lacrimal drainage system (LDS) [3]. The most important use of CT-DCG was in complex nasolacrimal
N. Bothra Govindram Seksaria Institute of Dacryology, Kallam Anji Reddy Campus L V Prasad Eye Institute, Hyderabad, India
S. Rath (*) Mithu Tulsi Chanrai Campus, L V Prasad Eye Institute, Bhubaneswar, India e-mail: suryasnata@lvpei.org
duct obstructions (NLDO) in craniofacial trauma. It is also useful in diagnosing and monitoring tumors and masquerades involving the LDS [1]. Magnetic resonance dacryocystography (MR-DCG) was rst performed in 1993 to study the functional and morphological aspects of the lacrimal pathways. Although the adverse effects of radiation exposure were nullied, the high cost, longer acquisition time, and motion artifacts were the limitations [4]. CT-DCG is currently the most used technique because of its distinct advan­tages in delineating details of the LDS, adjacent bone and soft tissue anatomy, low acquisition time, and relatively low radiation exposure.
34.2 Indications
ofDacryocystography
It delineates the following in the lacrimal drain­age system:
1. Position and size of the lacrimal sac with
respect to the bony landmarks.
2. Abnormalities like lacrimal sac diverticula.
3. Filling defects caused by mucopeptide
concretions.
4. Functional passage of tears in the lacrimal
system.
5. Lacrimal sac tumours.
6. Partial nasolacrimal duct obstructions.
7. Differentiate masquerading conditions from
LDS abnormalities.
© The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd. 2024 T. Das, P. Satgunam (eds.), Ophthalmic Diagnostics, https://doi.org/10.1007/978-981-97-0138-4_34
463
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N. Bothra and S. Rath
34.3 Dyes Used inDacryocystography
There are oil-soluble and water-soluble dyes available for conventional DCG and CT-DCG. The ideal dye must be radio-opaque, non-irritant, non-toxic, homogenous, with opti­mal viscosity, and should not cause adverse reac­tions [1]. Oil-soluble dyes like lipiodol provide signicantly better images than water-soluble dyes like iohexol, iopamidol, sinogran, and omnipaque; however, these are also very viscous, immiscible with tears, and carry a risk of tissue reaction or granuloma formation [57]. This makes water-soluble dyes preferable over oil­soluble counterparts. Water-soluble dyes can have a high (iohexol, iopamidol 300) or low (omnipaque) iodine content. Those with high iodine content provide better images but may cause more patient discomfort [57]. For patients allergic to iodinated substances, gadobutrol can be used [8]. MR-DCG uses a gadolinium solution as a contrast medium [4]. The authors use iohexol dye in a 50:50 dilution with normal saline for CT-DCG.
34.4 CT-DCG Scan Requisition
A request for CT-DCG should ideally full a few prerequisites for optimal information. These include:
1. Region of interest: computed tomography
scans of the orbit + dacryocystography.
2. Slice thickness: typically, 2 mm cuts (sec-
tions) are preferred for the orbit.
3. Imaging plane: Coronal, axial, three-
dimensional volumetric reconstruction (3-DR), and parasagittal views are requested. Coronal and axial views demonstrate the pas­sage of the LDS in the lacrimal sac fossa and a small portion of the NLD (nasolacrimal duct) and surrounding bony canal. The 3-DR scans provide a view of the LDS in three dimensions and determine the position of the LDS with respect to the bony structures. The parasagittal view shows the bony segment of NLD in its entirety.
4. Tissue windows: The bone windows help delineate the relationship of a dye-lled LDS with the surrounding bony structures, and the soft tissue windows show the position of the dye-lled LDS within the surrounding soft tissues.
34.5 Methods
1. Drop method: The dye is instilled in the infe­rior cul-de-sac, and the passage of the dye is monitored on serial scans. This technique is mainly used to assess the functional capacity of the LDS.
2. Injection method: The dye is directly injected into the system using an irrigation cannula. This method is faster and serial scans are not needed.
34.5.1 Patient Stories
Patient 1
A 27-year-old male presented with complaints of watering from his left eye and double vision for 2 months. He had sustained a road trafc acci­dent 4months before, followed by a maxillofa­cial repair of facial fractures. The irrigation of the left LDS showed complete regurgitation of mucoid discharge from the opposite punctum, and the right side was patent. CT-DCG 3-DR scans showed a large dilated sac on the left side with a block at the junction of the lacrimal sac and nasolacrimal duct with minimal spillover of the dye and patent system on the left side. The sac was fairly in position on the left side. There was an inferior rim fracture with plates on the maxillary bone just short of the expected area of surgery. Coronal CT scans of the orbit showed an associated large oor fracture on the left side involving the strut. The nasal anatomy and cribri­form plate position appeared normal (Fig.34.1).
Inference: CT-DCG helped delineate the anat­omy of the bones in relation to the lacrimal sys­tem, which was relatively preserved. This allowed the surgical team to decide on an external or endoscopic dacryocystorhinostomy. An orbital
34 Computed Tomography: Dacryocystography
abc
465
Fig. 34.1 Clinical photograph showing minimal pigmen­tation of the medial canthal region with mild telecan­thus on the left side (Panel A). Computed tomography scans with dacryocystography and 3D reconstructed images demonstrate a dilated sac on the left side with obstruction at the sac-duct junction; the right side shows
fracture repair could be planned during the same sitting.
Patient 2
A 30-year-old gentleman with a history of blunt trauma to the forehead and upper part of his nose with a cricket bat 4years ago complained of right eye watering after trauma. A CT-DCG scan and 3-DR images showed a small shrunken lacrimal sac on the rightside and an obstruction at the lac­rimal sac-nasolacrimal duct junction. On rotating the 3-DR images, the right-side sac position could be conrmed with respect to the underly­ing lacrimal sac fossa. The coronal images, bone windows depicted a normal relationship between the dye-lled sac with the lacrimal sac fossa and a shrunken sac compared to the left side. The parasagittal cuts showed no evidence of any bony fractures in the bony NLD canal. The remaining orbital and nasal anatomy appears normal (Fig.34.2).
Inference: External or endoscopic approach could be attempted. Expect the sac to be small, brosed, and slightly higher in position.
Patient 3
A 38-year-old male sustained multiple facial fractures after a road trafc accident. He com­plained of epiphora on both sides. He underwent a maxillofacial repair after the accident. On look­ing at the CT-DCG scans, gross lacrimal sac mal­position was noted (left, more than right). The right lacrimal sac was almost completely scarred. The left sac was displaced postero-superiorly in
drainage of the dye to the inferior meatus; left inferior orbital rim fracture, screws and plates on the maxillary and zygomatic bones on both sides (Panel B) and coronal scans showing left-sided orbital oor and medial wall fracture involving the strut, with the cribriform plate hav­ing a normal position and normal nasal anatomy (Panel C)
orbit beneath the ipsilateral frontal sinus. Damage to the horizontal plate of the cribriform and other fractures of the orbital walls were also noted (Fig.34.3).
Inference: An osteotomy at this location could lead to a signicant risk of injury to the skull base. In addition, maneuvering the canalicu­lar-lacrimalsac anatomy in such a malposition would be challenging. In this scenario, DCT (dacryocystectomy) may be a safer option. Alternatively, if attempted, a staged approach with the reconstruction of the naso-ethmoid frac­ture and correction of the medial canthal dystopia may need navigation guidance to decrease the risk of CSF (cerebrospinal uid) leak. Subsequently, a conjunctival DCR with a Jone’s tube may be considered for intractable epiphora.
Patient 4
After a road trafc accident, a young male pre­sented with a red, inamed, and tender swelling below the right lower eyelid medially with com­plaints of epiphora. Irrigation revealed total regurgitation of the uid from the opposite punc­tum, with the swelling becoming more tense and pronounced. The CT-DCG scans showed a mini­mally displaced sac with a large postero-lateral diverticulum, partially lled with diluted dye (Fig.34.4).
Inference: While trying to do a dacryocysto­rhinostomy surgery, one has to ensure that the diverticula are either completely excised or inter­nalized into the ostium created to prevent recur­rences of the problem and failure of the procedure.
466
ab
N. Bothra and S. Rath
c
d
e
Fig. 34.2 Clinical photograph demonstrating a raised tear meniscus height and minimal discharge medially in the right eye (Panel A). The CT-DCG and 3DR images show a small shrunken sac with obstruction at the sac-duct junction (Panel B). The 3-DR image, right-sided view, and
maximum intensity projection sequence (MIPS) show the sac is within the lacrimal sac fossawith theobstruction at the sac-duct junction (Panels C and D). The parasagittal cuts demonstrate a normal bony nasolacrimal duct with no drainage (Panel E)
The position of the diverticula noted on the scans will help take the appropriate surgical steps. A posterior diverticulum can be managed to a large extent during an endoscopic DCR, but a large anterolateral diverticulum is best approached via an external approach.
Patient 5
A 10-year-old boy was scratched by a dog in the medial part of the right eye about a year ago. He has had complaints of epiphora for the past 10 months. There was scarring in the mid­canalicular area. On probing and irrigation, the
ab
34 Computed Tomography: Dacryocystography
467
c
d
e
Fig. 34.3 Clinical photograph showing traumatic tele­canthus on the left side (Panel A). The 3D CT-DCG showed a shrunken, brosed sac on the right side and a postero-superiorly displaced sac on the left side (Panel B). The CT-DCG scans, coronal cuts demonstrate the orbital fractures on the right side a displaced, shrunken sac and a
damaged cribriform plate (Panel C) and naso-orbito­ethmoid fracture, postero-superior dislocation of the sac, and damaged cribriform plate on the left side (Panel D). The CT scan orbit, coronal cuts, bone windows demon­strate the presence of orbital fractures and cribriform plate damage on both sides (Panel E)
upper and lower canaliculus on the right side showed a mid-canalicular obstruction, but the child was certain he could appreciate minimal uid in the nasal cavity. The CT-DCG scans conrmed the mid-canalicular obstruction with no evidence of any trace of dye in the remaining LDS (Fig.34.5).
Inference: Considering the scarred tissue on the conjunctival side of the medial eyelid and the obstruction precisely at the same spot, one can explore the area with a guarded prognosis or con­vince the patient to undergo a conjunctival­dacryocystorhinostomy since the remaining anatomy is unaffected.