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Downloaded from http://bjo.bmj.com/ on May 2, 2017 - Published by group.bmj.com British Journal ofOphthalmology, 1990,74: 56-58 56 Morning glory syndrome: a histopathological study Willem A Manschot Abstract tumour, clear fluid escaped from a hole in the The clinical and histopathological findings in a new plane of section. case of morning glory syndrome are described. Microscopic examination. The cross section of Axial optic nerve retrodisplacement into a the optic nerve limit showed no evidence of peripapillary scleral ectasia (staphyloma), tumour cells. Its central part contained aggloabsence of fibrous lamina cribrosa, and optic merations of fragments of axons with varicose nerve atrophy were associated with various swellings, and there was reactive proliferation of other, non-inherent ocular developmental collagenous connective tissue. anomalies. The uninterrupted lining of the Nitrocellulose embedded serial sections of the inner wall of the staphyloma by one-layered, globe revealed an abnormal number of keratonormal retinal pigment epithelium proves that cytes with deep stromal oedema and absence of the syndrome is not due to a neuroectodermal Descemet's membrane, and endothelium in the closure defect. central cornea (Peters' anomaly of cornea). There was total loss of the anterior chamber; the chamber angle, trabecular lamellae and The term 'morning glory syndrome' (MGS) was Schlemm's canal could not be observed (Figs 1 introduced by Kindler,' because the papillary and 2A). The dislocated lens showed a hyperregion in the affected eye is reminiscent of a mature cataract and a partly calcified, anterior (withering) flower of 'morning glory'. Clinical and equatorial subcapsular cataract. A rupture in reports'-'" and four histopathological studies3 4I8I3 the posterior capsule was filled by connective gave rise to a definition of MGS as an 'unilateral tissue, originating from a fibrovascular retromalformation involving the optic disc ... associ- lental membrane, which was characteristic of ated with a peripapillary scleral defect, absence hyperplastic persistent primary vitreous. The of a lamina cribrosa and a recess formed by an membrane contained lens tissue (Fig 2B) and axial retrodisplacement of the optic nerve'.' was attached to elongated ciliary processes. Its These authors postulated that MGS should be contraction had caused the circular limbal conconsidered as a mesodermal disorder. The striction. central posterior scleral malformation may be A tent-like totally detached retina contained a associated with other intraocular anomalies, of falciform fold, which ran from the posterior stalk which non-rhegmatogenous retinal detachment of the retina to the central part of the retrolental is the most frequent. membrane. The fold showed numerous pseudoThe rarity of histopathological reports and the rosettes and defects in the nuclear layers. The rarity of anterior segment pathology, which was macular area contained a reduced but notenot described in previous microscopically worthy number ofganglion cells. The nerve fibre studied cases, warrant a report on the clinico- layer was markedly reduced. pathological data from a further case, which The posterior pole was characterised by a substantiate the postulate' that MGS is a mesodermal congenital lesion and not a coloboma of the optic nerve or disc. Case report A male infant aged 7 weeks showed unilateral microphthalmos, central corneal opacity, loss of the anterior chamber, and a mature cataract. Ultrasonography did not reveal an intraocular tumour. The ocular tension was 13 mmHg. Six weeks later, it was 30 mmHg, and 11 weeks later it was 36 mmHg. A CT scan then revealed a retrobulbar cyst connected with the sclera. Enucleation was performed, because retinoblastoma could not be excluded. Institute of Pathology, Erasmus University, Rotterdam, The Netherlands W A Manschot Correspondence to: W A Manschot, Institute of Pathology, Erasmus University, Postbox 1738, 3000 DR Rotterdam, The Netherlands. Accepted for publication 30 June 1989 PATHOLOGY Macroscopic examination. The globe measured 19x18x17 mm, the cornea 11 mm. A limbal circular constriction had caused an almost spherical cornea, with a prominence of 4 mm. The optic nerve was noticeably thickened: its diameter was 6 mm, its length 5-5 mm. When its posterior limit was cut for processing through paraffin for identification of possible spread of a Figure 1: Horizontal section showing scleral peripapillary staphyloma (S) with retrodisplaced optic nerve, total retinal detachment with axial stalk, and posterior part offalciform fold (F). (Nitrocellulose section, H and E, x 4.) Downloaded from http://bjo.bmj.com/ on May 2, 2017 - Published by group.bmj.com Morning glory syndrome: a histopathological study 57 of the central vessels had occurred at its normal site adjacent to the neuroectodermal lamina cribrosa, in which no fibrous tissue was found. Anomalous blood vessels were not observed within the retinal stalk. Histopathological diagnoses. Peters's anomaly of cornea, aplasia of the chamber angle, congenital cataract, hyperplasia of persistent primary vitreous, total detachment of atrophic, dysplastic non-rhegmatogenous retina, falciform retinal fold, axial optic nerve retrodisplacement into peripapillary scleral staphyloma, absence of fibrous lamina cribrosa, and optic nerve atrophy. Figure 2: A: Total anterior synechia (A), ectopic cataractous lens, hyperplastic persistent primary vitreous (V), elongated ciliary processes, anterior part offalciform fold (F). (H and x6.) B: Posterior lens capsule with rupture (arrow), lens substance (L) in hyperplastic persistent primary vitreous (V). (H and E, xS0.) C: Nasal wall ofperipapillary staphyloa lined by retinal pigment epithelium. Note inner and outer scleral layer. Black arrow: periph part of base of staphyloma. Open arrow: pigment epithelium represented in Figure 2D. (H 4 E, x40.) D: Detail ofFigure 2C indicated by open arrow (x 260). 4 mm deep peripapillary, funnel shaped scleral ectasia (staphyloma); its diameter was 5 mm. The inner wall of the ectasia and the periphery of its base were uninterruptedly covered by one layer of normal retinal pigment epithelium (Figs 2C, 2D). Remnants of the choroid could not be recognised below the layer of pigment epithelium. The extended inner and outer scleral layer were separated by a narrow fissure. The outer layer merged into the optic nerve dura; the inner layer surrounded the retrodisplaced optic nerve head in the base of the recess. Small numbers of smooth muscle cells could be recognised within the outer scleral layer, while clusters of fat cells could be observed within the narrow fissure. The stalk of the detached, nonrhegmatogenous retina arose from the nerve head and ran completely free from the inner wall of the recess axially through the scleral aperture into the lumen of the eye. The central retinal vessels and their first branches could be recognised within the stalk. The first branching Discussion The first description of MGS is attributed to Handmann,2 who reported six cases of identical (?) hereditary anomalies of the optic disc. Heredity'4 and bilaterality of peripapillary staphylomas'4 ,s have been described exceptionally. The normal visual acuity in some of these cases justifies some doubt whether they were of MGS. The main clinical features of MGS are (1) an apparently enlarged, sometimes excavated optic disc with a central mass of whitish-grey tissue; (2) a disc surrounded by a peripapillary scleral staphyloma, which in its turn may be surrounded by an elevated peripapillary ring of white, often pigmented tissue; (3) attenuated, branching arterial and venous retinal vessels, radiating from below the epipapillary mass of whitish tissue. These radiating vessels often appeared to be more numerous than the retinal vessels in the fellow eye and showed fewer bifurcations. Visual acuity is poor. Systemic defects rarely accompany the ophthalmoscopic findings. The main histopathological features of MGS are axial retrodisplacement of the optic nerve and a peripapillary, funnel shaped scleral staphyloma. The scleral ectasia is thought to be due to a developmental central posterior scleral anomaly. The optic nerve sheaths may be affected and may be partly replaced by fibroadipose and smooth muscle tissue. The fundamental observation in the present case of uninterrupted retinal pigment epithelium covering the entire inner wall of the staphyloma confirms that MGS is not due to a neuroectodermal closure defect. An identical continuous inner layer of pigment epithelium has been described by Cogan.8 Rack and Wright3 and Dempster et all3 reported respectively a 'broad collar of collagenous tissue in which pigmented cells are numerous' and 'nodules of metaplastic pigment epithelium which formed a ring around the neck of the recess', but not a continuous layer of pigment epithelium overlying the inner wall of the recess. A non-rhegmatogenous retinal detachment has been reported in five cases.'6'I The present sixth case with its total detachment and axial stalk within the staphyloma seems to substantiate the postulate that these non-rhegmatogenous detachments in MGS most probably are due to continuous traction exerted by the gradually increasing axial retrodisplacement of the optic nerve. A primary non-attachment of the retina Downloaded from http://bjo.bmj.com/ on May 2, 2017 - Published by group.bmj.com Manschot 58 appears unlikely. Rack and Wright3 and Cogan' depicted macroscopic and microscopic features of the radiating folds round and in the entrance of the peripapillary staphyloma. The frequently described unduly numerous attenuated branches of the central vessels, which radiate peripherally in a sometimes striking straight course from below the central white tissue, are most probably the normally more peripherally situated branches after their second ramification. In MGS they are more centrally located by the retrodisplacement of the optic nerve head, which was 4 mm (!) in the case of Rack and Wright3 and in the present case. The disc appears generally enlarged. It should, however, be realised, that in most cases the nerve head cannot be observed owing to its axial retrodisplacement and the whitish tissue which overlies and sometimes surrounds the nerve head.'- This whitish tissue consisted in all histopathologically studied cases, including the present case, of folded retina with glial, fibrous, and pigment epithelial proliferation. The depressed or elevated peripapillary, whitish often hyperpigmented ring represents the entrance of the peripapillary staphyloma. Either the retina is attached to the wall of the aperture, or retinal folds or a retinal stalk pass freely through the aperture. An elevated peripapillary ring consists of pseudofibrotic metaplastic retinal pigment epithelium, covered by a degenerated retina. It hides the aperture to the ectatic lumen. A persistent hyaloid artery has been reported twice.9'I The present case showed a thick layer of retrolental fibrovascular tissue, originating from hyperplastic persistent anterior primary vitreous. It was connected with stretched ciliary processes, and it had ruptured the posterior lens capsule. Ipsilateral Peters's anomaly of cornea has so far not been described in MGS. In avian embryos the 'mesectoderm' of the head derived from the neural crest is the source of most ocular tissues. The corneal endothelium and keratocytes are entirely so, the sclera is almost entirely of neural crest origin, while the vitreous has an unquantified derivation from the neural crest. '4 Comparative studies indicate that normal crest development is fundamentally similar in all vertebrate embryos. 'I Experimental evidence necessary to define clearly the origin of ocular tissues in mammals is not yet available, but recently experimental work has provided direct evidence that the neural crest contributes to cranifacial development in mammals. 16 The association of Peters's anomaly of the cornea and hyperplasia of persistent primary vitreous with the posterior scleral malformation in MGS may be purely coincidental, but it might also be a manifestation of a neural-crest-derived 'mesoectodermal' disturbance: a 'neurocristopathy'. 7 Some contractile peripapillary staphylomas have been reported.' 120 Their movements can be explained by the presence of heterotopic smooth muscle tissue in the posterior sclera or choroid.2' This smooth muscle tissue is often accompanied by heterotopic adipose tissue in the choroid and/ or in the enlarged distal region of the optic nerve and its sheaths. It occurred unilaterally in 24 of 26 cases.2' Smooth muscle cells and fat cells were also observed in the ectatic scleral tissue of the peripapillary staphyloma in the present case. The author thanks Professor W R Lee for his help in the preparation of the manuscript. 1 Kindler P. Morning glory syndrome: unusual congenital optic disc anomaly. Am j Ophthalmol 1970; 69: 376-84. 2 Handmann M. Erbliche, vermutlich angeborene, zentral gliose Entartung des Sehnerven mit besonderer Beteiligung der Zentralgefasse. Klin Monatsbl Augenheilkd 1929; 83: 145-52. 3 Rack JH, Wright GF. Coloboma of the optic nerve entrance. BrJ Ophthalmol 1%6; 50: 705-9. 4 Pedler C. Unusual coloboma of the optic nerve entrance. BrJ Ophthalmol 1%1; 45: 803-7. 5 Krause U. Three cases of the morning glory syndrome. Acta Ophthalmol (Kbh) 1972; 50: 188-98. 6 Jensen PE, Kalina RE. Congenital anomalies of the optic disk. Amj Ophthalmol 1976; 82: 27-31. 7 Rieger G. Zum Krankheitsbild der Handmannschen Sehnervenanomalie 'Windenbluten' ('morning glory') Syndrom?. Klin Monatsbl Augenheilkd 1977; 170: 697-706. 8 Cogan DG. Coloboma of optic nerve with overlay of peripapillary retina. BrJ Ophthalmol 1978; 62: 347-50. 9 Pau H. Handmannsche Sehnervenanomalie und Morning Glpry Syndrom ('Windenbluten-Syndrom'). Klin Monatsbl Augenheilkd 1980; 176: 745-5 1. 10 Brini A, Charton MN, Baumgartner M. Staphylome scleral peripapillaire avec ectopie posterieure de la papille (morning glory syndrome). J Fr Ophtalmol 1980; 3: 323-6. 11 Koenig SB, Naidlich TP, Lissner G. The morning glory syndrome associated with sphenoidal encephalocele. Ophthalmology 1989; 89: 1368-73. 12 Amalric P. Que faut-il penser des syndromes de morning glory? Bull Ophtalmol Fr 1982; 82: 793-9. 13 Dempster AG, Lee WR, Forrester JV, McCreath GTM. The morning glory syndrome - a mesodermal defect? Ophthalmologica 1983; 187: 222-30. 14 Johnston MC, Noden DM, Hazelton RD, Coulombre JL, Coulombre AJ. Origin of avian ocular and periocular tissues. Exp Eye Res 1979; 29: 27-43. 15 Johnston MC. The neural crest in abnormalities of the face and brain. In: Bergsma D, ed. Birth defects. Original article series XI No 7. New York: Liss, 1975: 1-18. 16 Tan SS, Morriss-Kay GM. Analysis of cranial neural crest cell migration and early fates in post-implantation rat chimaeras. J Embryol Exp Morphol 1986; 98: 21-58. 17 Bolande RP. The neurocristopathies: a unifying concept of disease arising in neural crest maldevelopment. Hum Pathol 1974; 5:409-29. 18 Wise JB, McLean AL, Gass JDM. Contractile peripapillary staphyloma. Arch Ophthalmol 1966; 75: 628-30. 19 Sugar HS, Beckman H. Peripapillary staphyloma with respiratory pulsation. Radiol Neuro-ophthalmol 1969; 68: 895-7. 20 Kral K, Svarc D. Contractile peripapillary staphyloma. AmJ Ophthalmol 1971; 71: 1090-2. 21 Willis R, Zimmerman LE, O'Grady R. Smith RS, Crawford B. Heterotopic adipose tissue and smooth muscle in the optic disc. Arch Ophthalmol 1972; 88: 139-46. Downloaded from http://bjo.bmj.com/ on May 2, 2017 - Published by group.bmj.com Morning glory syndrome: a histopathological study. W A Manschot Br J Ophthalmol 1990 74: 56-58 doi: 10.1136/bjo.74.1.56 Updated information and services can be found at: http://bjo.bmj.com/content/74/1/56 These include: Email alerting service Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article. Notes To request permissions go to: http://group.bmj.com/group/rights-licensing/permissions To order reprints go to: http://journals.bmj.com/cgi/reprintform To subscribe to BMJ go to: http://group.bmj.com/subscribe/