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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.)
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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
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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.
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2 Handmann M. Erbliche, vermutlich angeborene, zentral
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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
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