pathogenesis and treatment of maculopathy associated with...

PERSPECTIVEPathogenesis and Treatment of Maculopathy Associated

With Cavitary Optic Disc Anomalies

NIERAJ JAIN AND MARK W. JOHNSON

� PURPOSE: To propose a unifying theory regarding thepathogenesis of maculopathy associated with cavitary op-tic disc anomalies and to describe a rational approach toachieving a permanent cure in affected eyes.� DESIGN: Interpretive essay.� METHODS: Review and synthesis of selected literature,with interpretation and perspective in relating pathoana-tomic features to pathogenesis and treatment.� RESULTS: Congenital cavitary anomalies of the opticdisc, including typical coloboma, optic pit (and other atyp-ical colobomas), morning glory anomaly, and extrapapil-lary cavitation, are associated with an enigmaticmaculopathy characterized by schisis-like thickening andserous detachment. The unifying anatomic theme of theseanomalies is the presence of a scleral (or lamina cribrosa)defect permitting anomalous communications betweenintraocular and extraocular spaces. These communica-tions enable the critical pathogenicmechanism responsiblefor the maculopathy, namely, dynamic fluctuations in thegradient between intraocular and intracranial pressuresthat direct the movement of fluid (vitreous humor or cere-brospinal fluid) into and under the retina. Vitreous trac-tion does not seem to play a significant pathogenic role.Permanent cure of the maculopathy requires either elimi-nation of the translaminar pressure gradient or closure ofthe pathway for fluid flow into the retina. We advocatecarefully titrated juxtapapillary laser photocoagulationfollowed by vitrectomy with gas tamponade for creationof a permanent intraretinal and subretinal fluid barrier.� CONCLUSIONS: The peculiar features of cavitary opticdisc maculopathy can be explained only by consideringthe pressure gradients that develop along anomalouscommunications between intraocular and extraocularspaces. A permanent cure for this condition can beachieved by closing the pathway for fluid migrationfrom the cavitary lesion into and under the retina. (AmJ Ophthalmol 2014;158:423–435. � 2014 by ElsevierInc. All rights reserved.)

Accepted for publication Jun 2, 2014.From the Department of Ophthalmology and Visual Sciences,

University of Michigan, Ann Arbor, Michigan.Inquiries to MarkW. Johnson, W. K. Kellogg Eye Center, University of

Michigan, 1000Wall Street, AnnArbor, MI 48105; e-mail: [email protected]

0002-9394/$36.00http://dx.doi.org/10.1016/j.ajo.2014.06.001

� 2014 BY ELSEVIER INC.

APECULIAR AND ENIGMATIC MACULOPATHY

commonly occurs in association with severalcongenital cavitary anomalies of the optic disc,

including typical optic nerve coloboma, optic pit (andother atypical colobomas), morning glory anomaly, andextrapapillary cavitation. Although usually described asdistinct clinical entities, these anomalies likely fall withina spectrum with variable pathoanatomic features butsimilar embryogenesis and pathologic sequelae.The pathogenesis of serous maculopathy complicating

cavitary disc anomalies is as intriguing as it is controversial,and for many decades has defied attempts to understand itclearly. This lack of clarity has spawned a diversity of treat-ment approaches with variable efficacy. Attempting to un-derstand the fluid origin and pathophysiology of thiscondition informs us about the structure of the optic discand juxtapapillary retina and the dynamic forces that influ-ence these tissues. In this Perspective, we discuss the vary-ing clinical presentations of the cavitary disc anomalies andassociated maculopathy, the probable pathophysiologicmechanisms involved, and key considerations regardingmanagement.

CLASSIFICATION OF CONGENITALCAVITARY OPTIC DISC ANOMALIES

TYPICALCOLOBOMAOFTHEOPTICNERVE IS ACONGENITAL

excavation located in the inferonasal aspect of the disc(Figure 1, Top left). It is typically a sporadic and unilateralcondition. The juxtapapillary tissues inferior to the discmay be involved. Often, the affected eye is otherwisenormal, and visual field loss is confined to the site of thedefect. Localized serous maculopathy may develop in eyeswith optic nerve coloboma,1 whereas extensive rhegmatog-enous retinal detachment more likely occurs in eyes withlarge associated chorioretinal coloboma.2

Optic disc pits are focal cavitations isolated to the discthat are typically small and temporally located, but canalso be quite large and involve other portions of thedisc. They lie on a spectrum of anomalies often referredto as atypical optic nerve colobomas.3–5 Although classicoptic pits usually are unilateral and appear as focal

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FIGURE 1. Fundus photographs showing the spectrum of cavitary disc anomalies: (Top left) typical coloboma, (Top right) classicoptic pit, (Bottom left) centrally located atypical coloboma, and (Bottom right) morning glory anomaly.

grayish cavitations (Figure 1, Top right), when atypicalcolobomas are bilateral, large, and centrally located,they can be mistaken for glaucomatous optic cupping(Figure 1, Bottom left). Optic pits may cause congenitalfield defects because of associated nerve fiber layer defects.Serous maculopathy develops in more than 50% of cases,particularly in eyes with large and temporally locatedexcavations.6,7

Morning glory disc anomaly is a unilateral markedlyenlarged optic papilla with funnel-shaped excavationinvolving the optic disc and peripapillary retina (Figure 1,Bottom right). It features a characteristic radial arrange-ment of retinal vessels emanating from the disc, glial hyper-plasia over the center of the disc, a rim of elevatedperipapillary pigmented tissue, and traction on adjacentretina.8 Affected patients typically have poor vision.

Because of its inferonasal location, typical optic disccoloboma is attributed to faulty closure of the embryonicocular fissure. However, several lines of evidence suggestthat all of the congenital cavitary disc anomalies associatedwith macular detachment may share similar embryogenicmechanisms. Because the normal embryonic fissure extendsalong the inferior aspect of the globe and completely

424 AMERICAN JOURNAL OF

surrounds the optic papilla at its most posterior extent, itis anatomically plausible that faulty closure of this fissurecould result in the diversity of cavitary disc lesions underdiscussion.9 Furthermore, histopathologic specimensdemonstrate similarities between optic pit, morning glorysyndrome, and typical coloboma.8 In each case, dysplasticretina is herniated posteriorly through a defect in the lam-ina cribrosa, juxtapapillary sclera, or both, ranging from afocal defect in optic pits to a circumpapillary defect inmorning glory anomalies. Finally, both optic pit and morn-ing glory discs frequently occur in association with typicalcoloboma in the same or contralateral eye.9 In 1 extendedfamily spanning 5 generations with an autosomal dominantinheritance pattern, 35 members had a phenotypicallydiverse range of optic disc anomalies, including disc pit,coloboma, and morning glory syndrome.4

In addition to these anomalies involving the optic nerve it-self, cavitary anomalies that lie separate from but near to theoptic nerve occasionally can cause amaculopathy identical tothat seen with cavitary disc lesions. On optical coherence to-mography (OCT) imaging, the pathoanatomic features ofsuch lesions, as with the disc anomalies, seem to involve her-niation of abnormal retinal tissue through a scleral defect.10

SEPTEMBER 2014OPHTHALMOLOGY

FIGURE 2. Images showing the fluid tract in cavitary discmaculopathy. (Top) Color fundus photograph of right eyeshowing a subtle optic pit, diffuse macular thickening, and anouter lamellar macular hole. (Bottom) Spectral-domain opticalcoherence tomography image showing that the intraretinal fluidextends to the optic pit and is breaking into the subretinal spacethrough an outer lamellar macular dehiscence.

CLINICAL FEATURES OF CAVITARY DISCMACULOPATHY

THE MACULOPATHY ASSOCIATED WITH CAVITARY OPTIC

nerve anomalies typically develops in a 2-step process,starting with fluid accumulation within the retinal stromathat results in a peculiar schisis-like retinal edema. Lincoffand associates first observed the schisis-like intraretinalfluid with biomicroscopy and noted that it seems toemanate from the disc anomaly.11 Optical coherence to-mography imaging later confirmed this observation,1,12,13

demonstrating that the intraretinal fluid accumulatesmost prominently in the outer plexiform layer.14 Morerecent high-resolution OCT imaging has shown that fluidfrom an optic pit can enter into and accumulate withinmany different retinal layers.15 The intraretinal fluidthen may percolate into the subretinal space, sometimesthrough an outer layer break that may be detectable bio-microscopically, histopathologically, or by OCT.5,11,15,16

Although the intraretinal fluid communicates with theoptic disc cavitation, the submacular fluid typically doesnot (Figure 2). Only rarely does fluid appear to enter thesubretinal space directly from the optic disc without intra-retinal fluid accumulation.15

Serous maculopathy occurs in more than 50% of eyeswith cavitary disc anomalies, with onset typically in earlyadulthood.6,7,17 However, macular detachment can beseen in early infancy as well as late in life.18 Detachmenttypically is confined to the posterior pole, but can bemore extensive in some cases, particularly in eyes with largeatypical colobomas or morning glory anomaly. Untreatedserous maculopathy associated with optic disc pits andrelated anomalies typically leads to poor visual outcomes.One natural history study of 15 untreated subjects over 9years reported that 80% lost vision to a level of 20/200 orworse.19

CAUSE OF CAVITARY DISCMACULOPATHY

THERE HAS BEEN VIGOROUS AND LONG-LASTING DEBATE

regarding the cause of the maculopathy associated withcavitary disc anomalies. Since Gass proposed in 1969that cerebrospinal fluid (CSF) from the subarachnoid spacemigrates into the subretinal space, this has been an area ofparticular curiosity.20 Much of what we know about thismaculopathy derives from careful clinical examination, his-topathologic analysis, and embryologic studies performedin the pre-OCT era. In recent years, OCT technologyhas expanded our understanding and corroborated earlierhypotheses.

� ANATOMIC CONSIDERATIONS: We endorse a mechani-cal basis for the serous maculopathy based on congenital

VOL. 158, NO. 3 MACULOPATHY ASSOCIATED WITH C

structural defects in the optic nerve head. The normal opticnerve exits the eye through a physiologic opening in theeye wall where 2 pressurized, fluid-containing compart-ments (the vitreous cavity and subarachnoid space) meeteach other and abut the retina and the potential subretinalspace. Disruption of these normal anatomic features bycongenital cavitary defects allows vitreous, CSF fluid, orboth to travel down pressure gradients, occasionally intothe retinal stroma and subretinal space.That a similar maculopathy does not occur in normal in-

dividuals is testament to the integrity provided by the intri-cate and robust architecture of the lamina cribrosa andassociated tissues of the optic disc. In normal eyes, the1.2 million retinal ganglion cell axons from the retinalnerve fiber layer converge to form the optic nerve headand exit the globe through the optic nerve. In the prelami-nar region, these axons are grouped into 1000 fascicles,separated by glial columns. The glial columns are contig-uous with the adjacent collagenous and elastic tissuesthat surround the optic nerve head and separate it fromthe adjacent retina and choroid.21 This elastic tissue seemsto provide a fluid barrier as the nerve traverses past theretina and choroid. The axons then pass through the lam-ina cribrosa to exit the eye. The lamina cribrosa is rela-tively rigid and, along with its surrounding lining of

425AVITARY OPTIC DISC ANOMALIES

astrocytic glia, provides a pressure- and water-tight seal bet-ween the intraocular and extraocular (including subarach-noid) spaces.22

Congenital cavitary anomalies of the optic disc disruptthe intricate organization of axons and connective tissuesat this critical juncture where the optic nerve exits theglobe. Histologic studies and swept-source OCT imagingreveal that cavitary disc anomalies consist of a herniationof dysplastic retina through a defect in the juxtapapillarysclera, lamina cribrosa, or both into a collagen-lined sacthat protrudes into the subarachnoid space.23–25 Adiaphanous membrane may cover the pit, but defects inthis membrane commonly are observed and provide achannel for fluid flow into the pit.5,16,26–28 As a result ofthe abnormal anatomic features in affected eyes, thevitreous, subretinal, subarachnoid, and possibly orbitalspaces may be variably interconnected through therelatively compliant and porous tissues comprising theexcavated disc anomaly.5,8,25,29

� SOURCE OF FLUID: Investigators have proposed manysources of the intraretinal and subretinal fluid in cavitarydisc maculopathy, including liquefied vitreous, CSF, andplasma leakage from retinal vasculature, choroid, or orbitaltissues. The most plausible fluid sources are the vitreouscavity and subarachnoid space, depending on the particularconformation of the congenital disc pathologic features ineach affected eye.

Vitreous cavity. Clinical and experimental studies havedemonstrated conclusively a connection between the vit-reous cavity and subretinal space in eyes with cavitarydisc anomalies. This may account for the observationthat the serous maculopathy usually begins in early adult-hood, coinciding with the onset of progressive vitreousliquefaction.

Histopathologic studies with Alcian blue have shownvitreous mucopolysaccharides within optic disc pits.24

India ink studies in Collie dogs with cavitary disc lesionssimilar to human optic disc pits demonstrated migrationof the chromophore into the subretinal space via the discanomaly.30 Numerous reports describe the passage of intra-ocular gas, silicone oil, or other vitreous substitutes into thesubretinal or sub–internal limiting membrane (ILM) spacein eyes with cavitary disc anomalies.5,31–35 Furthermore, asadditional evidence of this connection, surgeons havereported successful drainage of subretinal fluid byaspiration over the disc anomaly.5,28,36,37

Vitreous fluid accesses the disc cavitation directly or viasmall breaks in diaphanous tissue overlying the excava-tion.7,25,26,28,31 Such breaks may be visible clinicallyor may be detected with OCT imaging of excavateddisc anomalies25,38,39 as well as extrapapillary cavitaryanomalies.10 We previously reported the postoperativefinding of gas trapped within a disc cavitation, havingpassed through a small visible break in the overlying neural


membrane.5 As described below, the fact that gas bubblespass through tiny breaks overlying cavitary disc anomaliesprovides critical insights into the pathogenesis of the un-usual maculopathy in these eyes.

Subarachnoid space. Histologically, congenital anoma-lous disc cavitations are known to extend posteriorly intothe subarachnoid space, and OCT images often show anapparent communication between the macular fluid and aperineural space associated with the disc lesion.12,13

Recent imaging with swept-source OCT has shownsubarachnoid space immediately posterior to the thinlayer of tissue (thought to be pia mater) lining thebottom of the disc excavation.25 Furthermore, there isunequivocal proof that CSF may access the subretinalspace in eyes with cavitary disc anomalies, a fact thatmay explain cases of serous maculopathy or extensiveretinal detachment that occur in infants and youngchildren before the age of significant vitreous liquefaction.Chang and associates describe a case of morning glorydisc anomaly with total retinal detachment in whichradiopaque dye injected into the subarachnoid spacemigrated into the subretinal space.40 Irvine and associatesreported a case of morning glory disc where gas in thevitreous cavity was noted to bubble out of the perineuralsubarachnoid space at the time of optic nerve sheathfenestration.8 Kuhn and associates describe a patient withbilateral optic nerve pits in whom persistent headachedeveloped as a result of intracranial migration of siliconeoil after repair of complex retinal detachment.41 Patel andassociates analyzed subretinal fluid in 2 infants withretinal detachment resulting from optic disc coloboma,finding composition consistent with CSF.29 In these 2patients with microphthalmos-coloboma syndrome, theCSF fluid was thought to derive from a retrobulbar opticnerve cyst, a congenital anomaly that occasionally also isseen in association with optic pits.42,43

Although there are relatively few such reports of directcommunication between the subretinal space and retrobul-bar spaces such as the perineural subarachnoid space andoptic nerve cysts, they provide convincing evidence thatCSF can access the subretinal space in some eyes with cavi-tary disc anomalies. In fact, as detailed below, we believethat recognition of anomalous communications betweenintraocular (vitreous cavity and intraretinal or subretinal)and extraocular (subarachnoid, cyst cavity, or both) spacesis a necessary component of any theory of pathogenesis thatpurports to explain the unique features of cavitary discmaculopathy. Appreciation of these communications andtheir anatomic variability also has critical implicationsfor therapeutic approaches to this condition.

� PATHOGENIC MECHANISMS: Vitreous traction hypo-thesis. Several authors have suggested a possible role forvitreous traction in the pathogenesis of serous maculopathyassociated with cavitary disc anomalies. Although a precise


mechanism has not been elucidated, vitreous traction isbelieved possibly to induce a small tear in diaphanous tissueoverlying the disc excavation, allowing for passage of lique-fied vitreous into the retina. Additionally or alternatively,vitreous traction on the peripapillary retina, macula, orboth is thought potentially to facilitate the accumulationof fluid in the macula. The following lines of evidencehave been offered in support of a vitreous traction mecha-nism:

1. Posterior vitreous detachment (PVD) typically is absentwhen the maculopathy first appears,6,31,44 andresolution of serous macular detachment occasionallyhas been seen after spontaneous PVD.31

2. Vitreous and glial tissue abnormalities often are presenton or near the optic disc in eyes with pits and other cavi-tary anomalies. These have been interpreted by someauthors as evidence for traction on the roof of the cavi-tation. For example, unusual vitreous strands may beobserved over the optic disc,45,46 and an anomalouscondensed Cloquet’s canal may be seen terminating atthe margin of an optic pit44 or optic nerve coloboma.47

Studies using various OCT imaging technologies havedemonstrated vitreous strands or glial tissue extendinginto pits and related anomalies.25,38,39,46

3. Pars plana vitrectomy with PVD induction results in res-olution of cavitary disc maculopathy in most eyes.Although most of the reported series included barrierlaser photocoagulation at the time of surgery, slow reso-lution of the maculopathy also has been described aftervitrectomy alone, without laser photocoagulation48 oreven gas tamponade.49 Tight vitreous adhesion to thedisc margin often is noted during surgery and has beeninterpreted as evidence for vitreous traction.49

4. Macular buckling is successful in most eyes with thiscondition, possibly by reducing vitreous traction onthe juxtapapillary retina and macula.46,50,51

Alternatively, resolution of the maculopathy mayresult from closure by the buckle of the connectionbetween the optic disc pit and adjacent retina, asoriginally suggested by the pioneers of this procedure.51

Conceptually, there are a number of serious limitationsto the vitreous traction hypothesis that can be summarizedas follows:

1. Optical coherence tomography images of eyes with def-inite vitreomacular or vitreopapillary traction show anobvious traction profile, with anteroposterior tissueelevation and sharp angulations at the point of vitreousinsertion. However, critical review of OCT images fromeyes with cavitary disc anomalies reveals no anteriortenting of the pit roof nor of the peripapillaryretina.15,27,38,39,46,48,49 In 1 study using time-domainOCT, the authors speculated that shallow posteriorvitreous separations in 10 of 16 eyes represented


vitreomacular traction, but a traction profile was seenin only 1 of these eyes.46 Other studies using OCTimaging have not shown evidence for vitreomaculartraction.15,38,39,48,49

2. All known examples of true vitreous traction are associ-ated with partial PVD states, where traction is exertedby the separating posterior hyaloid membrane on areasof residual vitreoretinal adhesion.52 However, cavitarydisc maculopathy routinely develops long before theearliest stages of partial PVD have begun (including invery young children). Optical coherence tomographyimages only rarely show partial vitreous separations ineyes with maculopathy.15,38,39,46,48,49 Furthermore,because the posterior hyaloid membrane is absent overthe optic disc, it is not anatomically feasible todevelop traction by the posterior hyaloid membraneon the tissue overlying cavitary disc anomalies.

3. Tight vitreous adhesion to the immediate peripapillaryretina (as noted during surgery in many cases of cavitarydisc maculopathy) is a routine finding in normal youngeyes and, in the absence of partial PVD, does not implyvitreous traction on the optic disc margin.

4. Surgical removal of glial or vitreous strands found inoptic pits does not seem to alter the surgical outcome,as would be expected if traction from these tissueswere important in pathogenesis.39

5. A vitreous traction model cannot explain the commonand frustrating observation of recurrent macular detach-ment after vitrectomy with peeling of the posteriorhyaloid membrane. Neither can it account for maculop-athy that develops in eyes with total PVD.46

6. Importantly, the vitreous traction hypothesis cannotaccount for the unique and enigmatic features of cavi-tary disc anomalies such as subretinal migration of vitre-ous substitutes and the intermittent sucking of vitreousstrands or debris into the pit.

Dynamic pressure gradients. The observation that vitreoussubstitutes such as gas,5,31,33 silicone oil,5,33–35 and perfluo-rocarbon liquid32,35 can migrate into the subretinal spaceafter vitrectomy in eyes with cavitary disc maculopathyprovides critical insight into the pathogenesis of thisdisorder. As noted above, such migrations clearly provea communication between the vitreous cavity andsubretinal space through the anomalous disc excavation.However, this phenomenon also implies an unusual andcomplex pathogenesis, because surface tension physicsdictate that passage of intravitreal gas through a smallopening is not possible without a substantial pressuregradient across the opening. We previously reviewed therelevant surface tension considerations and calculatedthat the pressure gradient required to push a gas bubblethrough a generous 200-mm defect in the roof of anoptic pit is at least 11 mm Hg.5

It is critical to point out that within the closed system ofthe normal eye, significant pressure differences between


various compartments (eg, vitreous cavity and subretinalspace) do not exist. For a pressure gradient to be presentacross the roof of a cavitary disc anomaly, there must becommunication with a space outside the eye. Given itsclose anatomic proximity to optic disc cavitations, the peri-neural subarachnoid space likely is involved in most cases.

Measured with lumbar puncture in the lateral recumbentposition, normal intracranial pressure ranges from 5 to15 mm Hg, with a mean of 12 mm Hg.53 Given a meanintraocular pressure of 16 mm Hg, the average pressuregradient across the lamina cribrosa in normal individualsis 4 mm Hg.54 However, intracranial pressure varies withfactors such as body position and venous pressure, and largefluctuations in CSF pressure have been measured in bothnormal and pathologic situations.55 Thus, the translaminar(intraocular–intracranial) pressure gradient fluctuatesthroughout the day and is likely sometimes to be largeenough to force gas (or liquid) into a cavitary disc anom-aly.5

As shown in Figure 3,5 intracranial pressure fluctuationscan be transmitted to the pit by direct CSF flow if the con-nective tissue capsule is porous. In cases where the pitcapsule is impermeable, pressure alterations may be trans-mitted via small pressure-induced excursions of thecapsule. In this case, the pit may behave as a bulb syringe,drawing fluid (or a vitreous substitute) into the pit sac whenintracranial pressure drops, and ejecting it again when CSFpressure rises. Given the variable anatomic features andanomalous interconnections that characterize cavitarydisc lesions, the fluid ejected from the pit sac in a giveneye could be liquid vitreous, CSF, or even a mixture ofthe 2 fluids. Repeated small aliquots of fluid driven intothe retinal stroma would be expected to cause progressiveschisis-like retinal edema and eventually to dissect intothe subretinal space.

We believe that transient alterations in the translaminarpressure gradient are necessary to explain the peculiarbehavior of cavitary disc anomalies and their associatedmaculopathy. Clinical evidence for the occurrence of thesetransient gradients comes from several observations. First,subretinal migration of vitreous substitutes through cavi-tary lesions almost always occurs after surgery and oftensuddenly, suggesting abrupt changes in the pressuregradient associated with everyday activities.5,17,31,34,35

Second, several authors have observed vitreous strands ordebris being sucked intermittently into optic pits andthen dislodged again with either ocular movement or asudden rise in intraocular pressure.43,45 This suggests thatin addition to CSF pressure alterations caused by theValsalva maneuver and positional changes, fluctuationsin intraocular pressure induced by ocular saccades andeye rubbing are important in the production of dynamictranslaminar pressure gradients.

Third, with newer OCT technologies, clinicians increas-ingly have observed the phenomenon of vitreous incarcer-ation into cavitary disc anomalies (Figure 4). Similar


herniations of vitreous into pit-like disc craters in Colliedogs previously were documented histologically.30 ThisOCT finding is common38,39,46 and is sometimesinterpreted as evidence for vitreous traction on the pit.However, this configuration more plausibly suggestsvitreous collagen being sucked into the cavitation andprovides compelling evidence for the existence oftranslaminar pressure gradients. An interesting relatedobservation is that of detached retina herniating into acolobomatous disc cavitation.29 Incarcerations of vitreousor retina can develop only along pressure gradients (at scle-rotomies, corneoscleral lacerations, etc) and do not occurwithin the closed system of a normal eye.Finally, the observation of transient cavitary disc macul-

opathy, sometimes associated with corresponding transientchanges in the appearance of the optic disc excavation, isfurther evidence for dynamic fluctuations in the pressuregradient between the subarachnoid space and subretinalspace in these eyes.5,34,56

Unifying theory of pathogenesis. In summary, the peculiarfeatures of the maculopathy associated with the variouscongenital cavitary disc anomalies and extrapapillary cavi-tary lesions10 cannot be explained by considering only thevitreous and subretinal compartments within a closed eye.Based on the histologic findings and imaging studiesreviewed above, the unifying anatomic theme of theselesions is the presence of a scleral defect permittinganomalous communications between intraocular(vitreous cavity and retinal or subretinal) and extraocular(subarachnoid, cyst cavity, or both) spaces. Thesecommunications enable the critical pathogenicmechanism responsible for the maculopathy, namely,fluctuations in the gradient between intraocular andintracranial pressures that direct the movement of fluidthrough the cavitation into (and under) the retina.

MANAGEMENT

GIVEN THE NATURAL HISTORY OF PROGRESSIVE VISUAL

decline in untreated cavitary disc maculopathy, clinicianshave used numerous strategies to treat this condition,including pharmacologic therapy with corticosteroids oracetazolamide, optic nerve sheath fenestration,8 macularbuckling,50,51 inner retinal fenestration,57 laser photocoag-ulation alone,20,26,58,59 and pars plana vitrectomy with orwithout laser photocoagulation and gas tamponade. Thelarge number of proposed treatments and their variableefficacy reflect the unusual and variable pathoanatomicfeatures of this condition and the resulting collectiveuncertainty regarding its management.

� NONSURGICALAPPROACHES: Pharmacologic agents. Thereis anecdotal evidence that some cases of cavitary disc


FIGURE 3. Schematic illustration showing the anatomic features of an optic pit and the associated maculopathy. (Top) The herni-ated dysplastic tissue and pit capsule vary in permeability from one eye to another. In eyes with an impermeable capsule, the pit func-tions like a bulb syringe, (Bottom left) ‘‘sucking’’ vitreous fluid into the pit sac during a drop in intracranial pressure (ICP), and then,(Bottom center) during a rise in pressure, expelling it from the sac. (Bottom right) In eyes with a permeable capsule, fluctuations inICP are transmitted to the pit by cerebrospinal fluid migration across the capsule. (Reprinted with permission from: Johnson TM,Johnson MW. Pathogenic implications of subretinal gas migration through pits and atypical colobomas of the optic nerve. ArchOphthalmol 2004;122(12):1793–1800. Copyright � 2004 American Medical Association. All rights reserved.)

maculopathy may respond to treatment with oral aceta-zolamide60 or corticosteroids.61 A possible therapeuticmechanism of action of these agents is alteration ofthe translaminar pressure gradient by reducing intracra-nial pressure.62 The limited experience with these drugssuggests that the macular fluid tends to reaccumulate on


discontinuing treatment.60 Empiric use of a pharmacologicagent could be considered early in the course of cavitarydisc maculopathy or in patients who are poor surgicalcandidates. In eyes that respond with resolution of macularfluid, laser photocoagulation may offer a way to preventmaculopathy recurrence on tapering the drug.61,62


FIGURE 4. Optical coherence tomography image showing vit-reous incarceration in an optic pit. Incarcerated vitreous tissue(arrows) provides compelling evidence for the existence of a pres-sure gradient across the optic pit (image courtesy of Barbara A.Blodi, MD).

FIGURE5. Optical coherence tomography image showing opticpit maculopathy after failed laser photocoagulation. The macul-opathy persists unchanged after 2 sessions of laser photocoagu-lation alone (treated area shown with arrowheads) failed tocreate an intraretinal barrier to fluid migration in this patient,who was referred for further management.

Laser photocoagulation alone. In theory, a laser-inducedjuxtapapillary chorioretinal scar that functions as abarrier to fluid migration from the optic nerve should bean effective treatment for cavitary disc maculopathy,regardless of the specific anatomic features of thecongenital anomaly. A complete barrier interrupts thefinal common pathway for serous maculopathy whetherthe fluid derives from the vitreous cavity or thesubarachnoid space in a given eye.

However, both the published literature and commonclinical experience suggest that barricade laser applied inisolation yields low success rates.26,31,56,59 Opticalcoherence tomography imaging has clarified that laserphotocoagulation alone typically is ineffective because itfails to produce a barrier to intraretinal fluid migration.Although such treatment may stimulate an adhesionbetween the photoreceptors and retinal pigmentepithelium, the most common route for fluid migrationout of the cavitary disc anomaly (ie, through the retinalstroma) is unaffected by this adhesion (Figure 5). Perma-nent blockade of intraretinal fluid movement requires anintraretinal scar. In eyes with existing maculopathy, thisend point is not readily achievable without the assistanceof a vitreous cavity gas bubble. As discussed below, wehave found that titrated laser photocoagulation given asan adjunct to vitrectomy is highly effective in producinga barrier to both intraretinal and subretinal fluid migration.

It is not unreasonable to consider the use of juxtapapil-lary laser photocoagulation prophylactically before thereis significant accumulation of intraretinal fluid. In sucheyes, it may be possible to achieve an intraretinal barrierwithout gas tamponade. However, these eyes, which lackintraretinal or subretinal fluid, or both, to buffer the nervefiber layer, are also at greater risk of visual loss attributableto papillomacular bundle injury.31 In light of this risk and


the variable natural history of cavitary disc anomalies, wegenerally do not recommend prophylactic laser photocoag-ulation for our patients without existing maculopathy.

� SURGICAL APPROACHES: Macular buckling. The macu-lar buckling procedure involves fixing a scleral sponge tothe posterior globe in the region of the macula. Surgicalsuccess depends on adequate indentation and accuratepositioning of the sponge. Although experienced groupshave reported excellent anatomic and visual outcomeswith this approach,50,51 it has not been adopted widelybecause most vitreoretinal surgeons are unfamiliar anduncomfortable with placing buckling elements in thislocation. The therapeutic mechanism of macularbuckling in this setting is not entirely clear. It is likelythat the force of indentation itself may close theintraretinal communication between the disc anomalyand the macula, creating a barrier to fluid flow.50,51

Alternatively, some believe that the buckle modifies thedirection of traction forces associated with the posteriorhyaloid, converting an inward centripetal vector into anoutward vector.46

Vitreous surgery. For patients with cavitary disc macul-opathy causing significant visual loss, our preferred treat-ment consists of carefully titrated juxtapapillary laserphotocoagulation combined with pars plana vitrectomyand gas tamponade. The primary objective of this approachis to create a permanent barrier to intraretinal (as well assubretinal) fluid migration from the optic disc cavitation.We prefer this procedure because it is relatively simple,uses techniques familiar to all vitreoretinal surgeons, pro-vides reliable and long-lasting results, and can be appliedto all eyes regardless of variations in anatomic features orfluid source.


FIGURE 6. Schematic illustration showing a juxtapapillary fluid barrier. Laser-induced intraretinal and subretinal scarring blocksfluid flow (arrow) out of the optic pit. The scarring spares the nerve fiber layer to avoid central visual field loss. Intraretinal and subre-tinal fluid in the macula gradually resolve after the barrier is established.

FIGURE7. Fundus photograph showing acute retinal opacifica-tion after juxtapapillary laser photocoagulation for disc pitmaculopathy. Immediately after carefully titrated slit-lampdelivery of juxtapapillary red laser photocoagulation, there ismoderate whitening of the outer and middle retinal layers,sparing the inner retina. The patient then underwent vitrectomywith gas tamponade to promote formation of an intraretinal andsubretinal adhesion.

Careful titration of the laser applications is critical tosafely produce a permanent barrier to fluid migration outof the optic pit and into the retinal stroma. To block fluidflow into or under the retina effectively, the laser-inducedscar must extend from the retinal pigment epitheliumthrough the middle retinal layers (Figure 6). Importantly,it must spare the retinal nerve fiber layer so as to avoid cen-tral vision loss. To maximize our ability to control andtitrate the thermal reaction, we perform the juxtapapillarylaser photocoagulation with slit-lamp delivery through acontact lens just before prepping the patient for the vitrec-tomy procedure. We typically use red (647-nm) laser lightwith a 200-mm spot size, titrating the power and duration toachieve a moderate-intensity end point. Carefully moni-toring the laser treatment with contact lens biomicroscopygreatly enhances the ability to achieve a thermal end pointthat is just right: hot enough to involve the outer and mid-dle retinal layers but not so intense as to injure the nervefiber layer (Figure 7). Treatment is placed in 4 to 5confluent rows in the temporal juxtapapillary area, over acircumferential extent corresponding to the presence ofintraretinal or subretinal fluid (as determined by OCTand biomicroscopy). The intraretinal fluid helps to bufferthe nerve fiber layer from thermal injury.59

Vitrectomy is performed within 1 or 2 hours of laserphotocoagulation on the assumption that a fresh laser reac-tion has the best chance of becoming an intraretinal scar.The primary purpose of the vitrectomy, in our view, is topermit the placement of a large vitreous cavity gas bubblethat will dry and compress the retinal layers in the juxtapa-pillary area and facilitate formation of an intraretinal scarduring 7 to 10 days of postoperative face-down positioning.Although intravitreal gas injection without vitrectomytheoretically may achieve the same purpose, this approachlikely is less effective.63 After core vitrectomy and peelingof the posterior vitreous cortex, a total fluid–gas exchange is


performed. It is typically not necessary to drain submacularfluid, because this usually will be displaced out ofthe macula after surgery by the gas bubble. However, itis reasonable during fluid–air exchange to drain asmuch intraretinal or subretinal fluid, or both, as possibleby careful aspiration over the optic disc cavitation.5,28,36

Because of the previously referenced reports of subretinal,intraretinal, and intracranial migration of vitreoussubstitutes, we avoid the use of perfluorocarbon liquid or


FIGURE 8. Optical coherence tomography (OCT) images showing 2 examples of laser barricade leading to resolution of cavitary discmaculopathy. In the first case (Left column), the (Top left) preoperative OCT image demonstrates that intraretinal fluid tracts extendboth temporally and nasally from the optic pit. (Middle left) A subretinal and intraretinal scar (arrowheads) is present 6 weeks afterlaser photocoagulation (along with vitrectomy and gas tamponade) along the temporal juxtapapillary retina. Intraretinal and subre-tinal fluid persist in the macula at this time. (Bottom left) The macula is dry 18 months after inducing the laser scar (arrowheads),but a patent fluid tract remains in untreated retina nasally. In the second case (Right column), the (Top right) preoperative time-domain OCT image demonstrates extensive fluid migrating from the cavitary lesion into the macula. (Middle right) At 9 months aftersurgery, a laser-induced intraretinal and subretinal scar (arrowheads) is blocking fluid migration (arrow) out of the disc cavitation.The macular fluid is resolving. (Bottom right) The macula is completely dry on this image obtained 15 months after induction ofthe laser scar (arrowheads).

silicone oil in these cases. Liquids are more likely to migratethan gas because of their lower surface tension.Furthermore, migration of silicone oil or heavy liquidinto the brain or subretinal space is significantly moreproblematic and difficult to manage than is gas migration.

After resolution of the gas bubble after surgery, OCT im-aging is helpful in determining whether an intraretinal fluidbarrier has been achieved. Successful cases show no subre-tinal or intraretinal fluid in the area of juxtapapillary lasertreatment, although intraretinal and sometimes subretinalfluid persists in the macular area initially. The retinal tissuecomprising the laser-induced fluid barrier may be slightlythinned, with less definition of its cell layers (Figure 8).After a barrier has been achieved, slow resolution of themacular fluid follows over the subsequent 3 to 12 months.If macular fluid reaccumulates, OCT can delineate thepersistent channels of communication between the opticdisc and macula, which can be addressed with additionallaser and gas tamponade.


Our experience using this approach in 10 consecutivepatients (unpublished data) suggests a high rate of successwith no cases of recurrent maculopathy after the barrierhas been achieved. The barrier was formed after a singlesurgery in 8 cases and required a second procedure (laserand fluid–gas exchange) in 2 eyes. To date, we haveobserved no cases of central vision loss resulting from nervefiber layer injury. Published studies using vitrectomy, laser,and gas have reported promising yet variable outcomes,probably because of variability in achieving an adequatelaser barrier.28,38,64 As an intriguing alternative to laserphotocoagulation in select cases, Travassos and associatesrecently described the successful use of a small plug ofautologous sclera to create a barrier to the passage ofvitreous fluid into large optic pits in 3 eyes.65

In light of the pathogenic considerations outlined above,we believe that a long-term cure of cavitary disc maculop-athy is most likely to be achieved by the establishment of apermanent barrier to intraretinal and subretinal fluid flow


out of the cavitary anomaly. However, Hirakata and asso-ciates reported that vitrectomy alone, without barrier laserphotocoagulation and with or without gas tamponade, ledto resolution of maculopathy in approximately 90% of eyesin a small series.48,49 Data are not available to determinethe true long-term recurrence rate after this procedure.However, this observation suggests that the vitrectomyitself has a beneficial effect in this condition, whether byaltering pressure gradients, releasing unidentifiable tractionforces, stimulating scarring within the cavitary anomaly,removing incarcerated vitreous that is maintaining patencyof a fluid channel, or other unknown mechanisms. In arecent small series, 50% of subjects demonstrated sustainedresolution of maculopathy after 1 or 2 intravitreal gas injec-tions without vitrectomy,66 but it is unclear why gas alonewould have a sustained effect. An earlier study demon-strated no sustained effect in any subject undergoing pneu-matic displacement for optic pit maculopathy.67

Other vitrectomy adjuncts. Several adjuncts to vitreoussurgery for cavitary disc maculopathy have been pro-posed that seem to be unnecessary and may increase sur-gical morbidity. Although several investigators haveadvocated peeling the ILM in these cases, there is no


plausible pathogenic role for the ILM in these eyes, whichare young and have no PVD, vitreoschisis, or epiretinalmembranes. Given that numerous surgical series showexcellent results without ILM peeling38,39,48,49 and thatILM peeling may result in a high incidence of macularhole creation in these eyes,68 we believe this maneuver isunwarranted. Creation of an inner retinal fenestrationduring vitrectomy has been proposed as a way to redirectthe flow of fluid from the cavitary anomaly away from themacula.57 However, such fenestrations have been shownto close spontaneously in the early postoperativeperiod,69 suggesting that any benefit seen after thisprocedure likely derives from the vitrectomy itself.Although authors have recommended peeling condensedvitreous or glial tissue from the disc cavitation,27,39 thereis no evidence that this procedure, which involves risk ofoptic nerve injury, increases the surgical success rate.Finally, there is anecdotal, unpublished experience withthe use of various tissue adhesives placed over thecavitary disc lesion at the time of vitrectomy. Thisadjuvant has no theoretical role in eyes with a CSF fluidsource and it remains unclear whether adhesives provideadded benefit beyond the vitrectomy procedure regardlessof the fluid source.

ALL AUTHORS HAVE COMPLETED AND SUBMITTED THE ICMJE FORM FOR DISCLOSURE OF POTENTIAL CONFLICTS OF INTERESTand the following were reported. Dr Johnson serves as a Data and Safety Monitoring Committee member for clinical trials sponsored by GlaxoSmithKlineand Oraya. Supported by the Heed Ophthalmic Foundation, San Francisco, California. Involved in Design and conduct of study (N.J., M.W.J.); Collec-tion, management, analysis, and interpretation of data and literature (N.J., M.W.J.); and Preparation, review, and approval of manuscript (N.J., M.W.J.).

REFERENCES

1. Hotta K, Hirakata A, Hida T. Retinoschisis associated withdisc coloboma. Br J Ophthalmol 1999;83(1):124.

2. Jesberg DO, Schepens CL. Retinal detachment associatedwith coloboma of the choroid. Arch Ophthalmol 1961;65(2):163–173.

3. Brodsky MC. Congenital optic disk anomalies. Surv Ophthal-mol 1994;39(2):89–112.

4. Slusher MM, Weaver RG Jr, Greven CM, Mundorf TK,Cashwell LF. The spectrum of cavitary optic disc anomaliesin a family. Ophthalmology 1989;96(3):342–347.

5. JohnsonTM, JohnsonMW. Pathogenic implications of subre-tinal gas migration through pits and atypical colobomas of theoptic nerve. Arch Ophthalmol 2004;122(12):1793–1800.

6. Gordon R, Chatfield RK. Pits in the optic disc associated withmacular degeneration. Br J Ophthalmol 1969;53(7):481–489.

7. Brown GC, Shields JA, Goldberg RE. Congenital pits of theoptic nerve head. II. Clinical studies in humans. Ophthal-

mology 1980;87(1):51–65.8. Irvine AR, Crawford JB, Sullivan JH. The pathogenesis of

retinal detachment with morning glory disc and optic pit.Trans Am Ophthalmol Soc 1986;84:280–292.

9. Apple DJ. New aspects of colobomas and optic nerve anom-alies. Int Ophthalmol Clin 1984;24(1):109–121.

10. Aggio FB, Farah ME, Meirelles RL, Costa RA, Monteiro ML.New Stratus OCT findings in extrapapillary excavated anom-aly and secondary macular complications. Retina 2007;27(1):113–115.

11. Lincoff H, Lopez R, Kreissig I, Yannuzzi L, Cox M, Burton T.Retinoschisis associated with optic nerve pits. Arch Ophthal-mol 1988;106(1):61–67.

12. Krivoy D, Gentile R, Liebmann JM, et al. Imaging congenitaloptic disc pits and associatedmaculopathy using optical coher-ence tomography. Arch Ophthalmol 1996;114(2):165–170.

13. Rutledge BK, Puliafito CA, Duker JS, Hee MR, Cox MS. Op-tical coherence tomography of macular lesions associated withoptic nerve head pits.Ophthalmology 1996;103(7):1047–1053.

14. Roy R, Waanbah AD, Mathur G, Raman R, Sharma T. Op-tical coherence tomography characteristics in eyes with opticpit maculopathy. Retina 2013;33(4):771–775.

15. Imamura Y, Zweifel SA, Fujiwara T, Freund KB, Spaide RF.High-resolution optical coherence tomography findings inoptic pit maculopathy. Retina 2010;30(7):1104–1112.

16. Christoforidis JB, Terrell W, Davidorf FH. Histopathology ofoptic nerve pit-associated maculopathy. Clin Ophthalmol2012;6:1169–1174.

17. Haik BG, Greenstein SH, Smith ME, Abramson DH,Ellsworth RM. Retinal detachment in the morning gloryanomaly. Ophthalmology 1984;91(12):1638–1647.


http://refhub.elsevier.com/S0002-9394(14)00333-X/sref1
















































18. Sugar HS. Congenital pits in the optic disc and their equiva-lents (congenital colobomas and colobomalike excavations)associated with submacular fluid. Am J Ophthalmol 1967;63(2):298–307.

19. Sobol WM, Blodi CF, Folk JC, Weingeist TA. Long-termvisual outcome in patients with optic nerve pit and serousretinal detachment of the macula. Ophthalmology 1990;97(11):1539–1542.

20. Gass JD. Serous detachment of the macula. Secondary tocongenital pit of the optic nervehead. Am J Ophthalmol

1969;67(6):821–841.21. Sadun AA. Optic disc pits and associated serous macular

detachment. In: Ryan SJ, ed. Retina. Vol 2. 4th ed. Philadel-phia: Mosby; 2006:1883–1887.

22. Sadun AA, Currie JN, Lessell S. Transient visual obscura-tions with elevated optic discs. Ann Neurol 1984;16(4):489–494.

23. Irvine AR, Crawford JB, Sullivan JH. The pathogenesis ofretinal detachment with morning glory disc and optic pit.Retina 1986;6(3):146–150.

24. Ferry AP. Macular detachment associated with congenital pitof the optic nerve head. Pathologic findings in two cases simu-lating malignant melanoma of the choroid. Arch Ophthalmol

1963;70(3):346–357.25. Ohno-Matsui K, Hirakata A, Inoue M, Akiba M, Ishibashi T.

Evaluation of congenital optic disc pits and optic disc colobo-mas by swept-source optical coherence tomography. InvestOphthalmol Vis Sci 2013;54(12):7769–7778.

26. Brockhurst RJ. Optic pits and posterior retinal detachment.Trans Am Ophthalmol Soc 1975;73:264–291.

27. Doyle E, Trivedi D, Good P, Scott RA, Kirkby GR. High-res-olution optical coherence tomography demonstration ofmembranes spanning optic disc pits and colobomas. Br J

Ophthalmol 2009;93(3):360–365.28. Postel EA, Pulido JS, McNamara JA, Johnson MW. The eti-

ology and treatment of macular detachment associated withoptic nerve pits and related anomalies. Trans Am OphthalmolSoc 1998;96:73–88. discussion 88–93.

29. Patel CK, Berg SJ, Quaghebeur G, et al. Ectopiccerebrospinal-like fluid from retrobulbar cysts as a possiblecause of pediatric retinal detachment associated with opticdisc coloboma: new implications for management. ArchOphthalmol 2012;130(8):1065–1067.

30. Brown GC, Shields JA, Patty BE, Goldberg RE. Congenitalpits of the optic nerve head. I. Experimental studies in colliedogs. Arch Ophthalmol 1979;97(7):1341–1344.

31. Bonnet M. Serous macular detachment associated with opticnerve pits. Graefes Arch Clin Exp Ophthalmol 1991;229(6):526–532.

32. Bartz-Schmidt KU, Heimann K. Pathogenesis of retinaldetachment associated with morning glory disc. Int Ophthal-mol 1995;19(1):35–38.

33. Coll GE, Chang S, Flynn TE, Brown GC. Communicationbetween the subretinal space and the vitreous cavity in themorning glory syndrome. Graefes Arch Clin Exp Ophthalmol1995;233(7):441–443.

34. Salam AK-LD, Luff AJ. Superior retinal detachment in anoil-filled eye with a colobomatous optic disc. Retinal Cases& Brief Reports 2008;2(2):124–125.

35. Dithmar S, Schuett F, Voelcker HE, Holz FG. Delayedsequential occurrence of perfluorodecalin and silicone oil in


the subretinal space following retinal detachment surgery inthe presence of an optic disc pit. Arch Ophthalmol 2004;122(3):409–411.

36. Ehlers JP, Kernstine K, Farsiu S, Sarin N, Maldonado R,Toth CA. Analysis of pars plana vitrectomy for optic pit-related maculopathy with intraoperative optical coherencetomography: a possible connection with the vitreous cavity.Arch Ophthalmol 2011;129(11):1483–1486.

37. Harris MJ, de Bustros S, Michels RG, Joondeph HC. Treat-ment of combined traction-rhegmatogenous retinal detach-ment in the morning glory syndrome. Retina 1984;4(4):249–252.

38. Chang S, Gregory-Roberts E, Chen R. Retinal detachmentassociated with optic disc colobomas and morning glory syn-drome. Eye (Lond) 2012;26(4):494–500.

39. Gregory-Roberts EM, Mateo C, Corcostegui B, et al. Opticdisk pit morphology and retinal detachment: optical coher-ence tomography with intraoperative correlation. Retina2013;33(2):363–370.

40. Chang S, Haik BG, Ellsworth RM, St Louis L, Berrocal JA.Treatment of total retinal detachment in morning glory syn-drome. Am J Ophthalmol 1984;97(5):596–600.

41. Kuhn F, Kover F, Szabo I, Mester V. Intracranial migration ofsilicone oil from an eye with optic pit. Graefes Arch Clin ExpOphthalmol 2006;244(10):1360–1362.

42. Lee PY, HM, Wang TJ. Optic disc pit combining with anoptic nerve cyst. A case report. Taiwan J Ophthalmol 2012;2(1):31.

43. Friberg TR, McLellan TG. Vitreous pulsations, relativehypotony, and retrobulbar cyst associated with a congenitaloptic pit. Am J Ophthalmol 1992;114(6):767–769.

44. Akiba J, Kakehashi A, Hikichi T, Trempe CL. Vitreous find-ings in cases of optic nerve pits and serous macular detach-ment. Am J Ophthalmol 1993;116(1):38–41.

45. Hirakata A, Hida T, Wakabayashi T, Fukuda M. Unusualposterior hyaloid strand in a young child with optic disc pitmaculopathy: intraoperative and histopathological findings.Jpn J Ophthalmol 2005;49(3):264–266.

46. Theodossiadis PG, Grigoropoulos VG, Emfietzoglou J,Theodossiadis GP. Vitreous findings in optic disc pit macul-opathy based on optical coherence tomography. GraefesArch Clin Exp Ophthalmol 2007;245(9):1311–1318.

47. Akiba J, Yoshida A, Ohta I, Igarashi H, Kakehashi A. Anom-alous Cloquet’s canal in a case of optic nervehead colobomaassociated with extensive retinal detachment. Br J Ophthalmol1993;77(6):381–382.

48. Hirakata A, Okada AA, Hida T. Long-term results of vitrec-tomy without laser treatment for macular detachment associ-ated with an optic disc pit. Ophthalmology 2005;112(8):1430–1435.

49. Hirakata A, Inoue M, Hiraoka T, McCuen BW 2nd. Vitrec-tomy without laser treatment or gas tamponade for maculardetachment associated with an optic disc pit. Ophthalmology2012;119(4):810–818.

50. Theodossiadis GP. Treatment of maculopathy associatedwith optic disk pit by sponge explant. Am J Ophthalmol

1996;121(6):630–637.51. Theodossiadis GP, Theodossiadis PG. Optical coherence to-

mography in optic disk pit maculopathy treated by the mac-ular buckling procedure. Am J Ophthalmol 2001;132(2):184–190.

























































































































52. Johnson MW. Posterior vitreous detachment: evolution andcomplications of its early stages. Am J Ophthalmol 2010;149(3):371–382.

53. Gilland O. Normal cerebrospinal-fluid pressure.N Engl J Med1969;280(16):904–905.

54. Berdahl JP, Allingham RR. Intracranial pressure and glau-coma. Curr Opin Ophthalmol 2010;21(2):106–111.

55. Bradbury M. Anatomy and physiology of the cerebrospinalfluid. In: Schurr PHPC, ed. Hydrocephalus. Oxford: OxfordUniversity Press; 1993:19–47.

56. Perkins SL, Han DP, Gonder JR, Beaumont PE, Colev G. Dy-namic atypical optic nerve coloboma associated with tran-sient macular detachment. Arch Ophthalmol 2005;123(12):1750–1754.

57. Spaide RF, Fisher Y, Ober M, Stoller G. Surgical hypothesis:inner retinal fenestration as a treatment for optic disc pitmaculopathy. Retina 2006;26(1):89–91.

58. Sugar HS. An explanation for the acquired macular pathol-ogy associated with congenital pits of the optic disc. Am JOphthalmol 1964;57:833–835.

59. Annesley W, Brown G, Bolling J, Goldberg R, Fischer D.Treatment of retinal detachment with congenital optic pitby krypton laser photocoagulation. Graefes Arch Clin Exp

Ophthalmol 1987;225(5):311–314.60. Prakash P, De Salvo G, Lotery AJ. Morning glory with serous

macular detachment responds to oral acetazolamide. Eye

(Lond) 2010;24(11):1732–1733.

LXXI Edward Jackson

The American Journal of Ophthalmology and ElsevierInc. will jointly recognize Hans Grossniklaus, MD,MBA, at this year’s American Academy of

Ophthalmology meeting in Chicago as the 71st EdwardJackson Memorial Lecturer. Dr Grossniklaus of Emory Uni-versity in Atlanta, GA, will present his lecture, entitled‘‘Retinoblastoma: Fifty Years of Progress,’’ on October 19thduring the opening session scheduled from 8:30 AM to10 AM at Hyatt McCormick Place. Dr Grossniklaus is thefounding director of the Ocular Oncology and Pathology


61. Mustonen E, Varonen T. Congenital pit of the optic nervehead associated with serous detachment of the macula. ActaOphthalmol (Copenh) 1972;50(5):689–698.

62. Ridings B, Saracco JB, Escoffier P, Denis D, Fany A. [Treat-ment of a case of retinal detachment in morning glory syn-drome]. J Fr Ophtalmol 1988;11(11):743–746.

63. Georgalas I, Ladas I, Georgopoulos G, Petrou P. Optic discpit: a review. Graefes Arch Clin Exp Ophthalmol 2011;249(8):1113–1122.

64. Cox MS, Witherspoon CD, Morris RE, Flynn HW. Evolvingtechniques in the treatment of macular detachment caused byoptic nerve pits. Ophthalmology 1988;95(7):889–896.

65. Travassos AS, Regadas I, Alfaiate M, Silva ED, Proenca R,Travassos A. Optic pit: novel surgical management ofcomplicated cases. Retina 2013;33(8):1708–1714.

66. Akiyama H, Shimoda Y, Fukuchi M, et al. Intravitreal gas in-jection without vitrectomy for macular detachment associ-ated with an optic disk pit. Retina 2014;34(2):222–227.

67. Lincoff H, Kreissig I. Optical coherence tomography of pneu-matic displacement of optic disc pit maculopathy. Br J

Ophthalmol 1998;82(4):367–372.68. Shukla D, Kalliath J, Tandon M, Vijayakumar B. Vitrectomy

for optic disk pit with macular schisis and outer retinal dehis-cence. Retina 2012;32(7):1337–1342.

69. Slocumb RW, Johnson MW. Premature closure of innerretinal fenestration in the treatment of optic disk pit macul-opathy. Retinal Cases & Brief Reports 2010;4(1):37–39.

Memorial Lecture

service, director of the L.F. Montgomery Laboratory, andthe F. Phinizy Calhoun Jr. Professor of Ophthalmology atEmory Eye Center. He has served on the board of the Amer-ican Journal of Ophthalmology in a variety of capacities for 20years, served as president of the American Ophthalmolog-ical Society last year, and is currently president of theAmer-ican Association of Ophthalmic Oncologists andPathologists. Dr Grossniklaus’ areas of expertise includediagnostic ophthalmic pathology, ocular oncology,ophthalmic pathology research, and translational research.
























































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