• BASAL ENCEPHALOCELE ASSOCIATED WITH MORNING GLORY SYNDROME

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Arq Neuropsiquiatr 2007;65(4-A):988-991
BASAL ENCEPHALOCELE ASSOCIATED
WITH MORNING GLORY SYNDROME
Case report
Ivanete Minotto1, Nitamar Abdala2, Adriana Aparecida Siviero Miachon3,
Angela Maria Spinola e Castro4, Paulo Imamura5, Roberto Gomes Nogueira6
ABSTRACT - The basal encephaloceles refer to rare entities and they correspond to herniation of brain tissue
through defects of skull along the cribiform plate or the sphenoid bone. A rare morning glory syndrome,
with characteristic retinal defect has been reported in association with basal encephaloceles. Hypophysis
hormonal deficiencies may occur. We accounted for a pituitary dwarfism with delayed diagnosed transs-
phenoidal encephalocele associated with morning glory syndrome, showing the alterations found in retin-
ography, computed tomography and magnetic resonance imaging.
KEY WORDS: basal encephalocele, morning glory syndrome, computed tomography, magnetic resonance
imaging, pituitary dwarfism.
Encefalocele basal associada a síndrome “morning glory”: relato de caso
RESUMO - As encefaloceles basais são entidades raras e correspondem a herniações do tecido cerebral atra-
vés de um defeito do crânio, ao longo da lâmina crivosa etmoidal ou do osso esfenoidal. A rara síndrome
morning glory, com alterações de fundo de olho características pode apresentar-se associada à encefaloce-
le basal. Deficiências hormonais hipofisárias podem ocorrer. Relatamos caso de nanismo hipofisário com en-
cefalocele transesfenoidal de diagnóstico tardio associada à síndrome de morning glory, mostrando as alte-
rações na retinografia, tomografia computadorizada e ressonância magnética.
PALAVRAS-CHAVE: encefalocele basal, síndrome de “morning glory”, tomografia computadorizada, resso-
nância magnética, nanismo hipofisário.
The basal cephaloceles refer to rare entities of dif- We present a case of defect on the medium line
ficult diagnosis and correspond to herniation of the in which the TC and MRI images are fundamental to
brain tissue through a birth or acquired defect in the clarify the diagnosis.
skull along the cribiform plate or through the sphe-
noid bone. It may be associated to hormonal distur- CASE
bances or ocular malformation and, amongst, the rare A 8 years-old boy, was taken to have his retarded
morning glory syndrome1-3, which name is given due neurospychomotor evaluated. Born at term and with
to the retinal aspect similar to the tropical flower of no intercurrences, he has evolved with a low weight
same name (Fig 1A). It is believed that such anoma- and height gain since his first year of life, dispropor-
lies result from a succession of events for the medium tional to the family growth potential. He presented
line conclusion during the gestation period3. The com- converging squint, nystagmus and visual loss of the
puted tomography (CT) and magnetic resonance im- left eye. The isolated deficiency of the growth hor-
age (MRI) exams play a very important role for these mone diagnosis was confirmed. He received hormon-
anomalies since they evaluate the whole skull and al reposition presenting unsatisfactory response. The
present structures in the hernial content1,2. retinography and ophthalmological exam showed
Neuroradiology Ward, Image Diagnoses Department, Federal University of São Paulo, São Paulo SP, Brazil (UNIFESP), Endocrinology
Ward at Pediatrics Department, UNIFESP: 1Undermasters graduation at the Neuroradiology Department (DDI/UNIFESP); 2 Affiliated
Professor, DDI/UNIFESP; 3Physician at the Pediatrics Endocrinology Department, UNIFESP; 4 Assistant Professor, Pediatrics Endocri-
nology Department, UNIFESP; 5Professor of Neurophtalmology, Ophthalmology Department, UNIFESP; 6Professor-Assistant at the
Neuroradiology Department – DDI/UNIFESP
Received 27 April 2007. Accepted 17 August 2007.
Dr. Roberto G. Nogueira - Rua Pará 126 / 52 - 01243-020 São Paulo SP - Brasil.
988
Basal encephalocele: morning glory syndrome
Arq Neuropsiquiatr 2007;65(4-A) Minotto et al.
Fig 1. (A) The flower morning glory. (B) Left eye retinography with a morning glory aspect:
optical disk enlarged choanoid and cupped aspect, pink coloration with a central white mass
which hides the vessels ways at the back of the disk. It’s is surrounded by a grey ring, a little
lifted, with irregular borders and mixed with some colored areas. The vessels are multiple,
thin and radiated. The yellow membrane of the remaining vitreous over the superior tempo-
ral area of the disk.
Fig 2. (A) A 3D reconstructed sella turcica CT image showing the main damage of the floor
(arrow). (B) CT image reconstruction in the sagittal plane, in soft window showing the dam-
age on the sellar floor and the encephalocele (arrow). (C) Coronal CT image in bone window
showing the main flaw on the sellar floor (arrow).
characteristic alterations of the morning glory syn- was observed (Fig 2). On the MRI (Phillips Gyroscan
drome in the left eye, observing an optical disk with 1,5T), sagittal and coronal images from the sellar re-
an enlarged choanoid and cupped aspect, with a pink gion were obtained on the T1 weighted spin echo
pigmentation and a central white mass which hid the (T1WSE) sequence before and after the paramagnetic
way of the vessels at the bottom of the disk. The disk contrast medium intravenous administered, and in T2
was surrounded by a little elevated grey ring, with weighted spin echo sequence. A sellar content consti-
tuted by the pituitary stalk, optical chiasm, adenohy-
irregular borders and mixed with colored areas. The
pophysis and neurohypophysis occurring on the right
vessels were multiple, thin and radiated (Fig 1B).
side was observed. It was observed the extension of
At the age of 20, he was submitted to imaging ex- the anterior portion of the third ventricle into the in-
ams of the sellar region. On the CT of the sella, with terior of the sella, and the hernial content with tissue
volumetric acquisition and three-dimensional recon- characteristics not defined on the inner side of the
struction, a defect at the main area of the sellar floor sellar floor and of the sphenoidal sinus (Fig 3).
989
Basal encephalocele: morning glory syndrome
Minotto et al.
Arq Neuropsiquiatr 2007;65(4-A)
Fig 3. MRI images of the sagittal plane on
T1 without iodine contrast (A), sagittal with
iodine contrast (B) and coronal with iodine
contrast (C) showing the hypophysis in the
inner sella (black arrow). Inferior retrac-
tion of the pituitary stalk (white arrow),
the optic chiasm and anterior portion of
the third ventricle to the inner sella (dou-
ble white cross). The bone flaw on the sell-
ar floor with the hernial content of undis-
tinguished anatomic aspect, characterized
as gliocele (black arrow with white border),
is located into the sphenoidal sinus.
DISCUSSION the passage of several portions of the intracranial
Encephalocele is a congenital defect of the skull structure such as the hypophysis, the anterior por-
bone and of the dura-mater with extracranial hernia- tion of the third ventricle floor, optic chiasm and op-
tion of any intracranial structure. It is found with a tic nerves1,2,4. The symptoms may be developed at the
geographic variation and with different occurrences neonatal period or at the first childhood, and they
when related to sex and race, and in different asso- consist of expansive processes of the epipharynx and
ciation to the neural tube malformations, suggesting pituitary dwarfism3,6,7, and the hypertelorism is almost
that some of them may present genetic origin, and constant. When these symptoms do not manifest
that the several types of encephalocele may corre- themselves, visual disturbances and hypothalamohy-
spond to distinct genetic defects4. It can be divided pophysial dysfunction1 may lead to the diagnostic, as
into four groups. The cephalic meningoceles, which occurred in our case.
are constituted by the leptomeninges and the cere- The basal encephaloceles associate themselves to
brospinal fluid (CSF), and the glioceles, which are glial optic malformation and retinal defect1, observing an
cells cysts containing CSF. The meningoencephaloce- increase on the prevailing (67.7%) of the morning
les, which consist of leptomeninges, CSF and brain pa- glory syndrome3,7-10, which is an unusual congenital
renchyma, recognizes the meningoencephaloventric- anomaly on the optic nerve. It was described by Reis
ulocele when parts of the ventricles and of portions and by Handman3. Kindler called it morning glory3,11.
of the choroid plexus participate on the herniation. Its frequency on the population is unknown5 and it
And the atresic changes on the encephalocele, char- is transmitted hereditarily with an autossomal domi-
acterized by tuberous lesions situated on the medium nant pattern. Most cases are unilateral3,4 but there
line of the scalp, either in the vertex (parietal form) or are rare cases of bilaterality6,7 with twice as much fre-
in the occipital protuberance (occipital form)4. quency on females3. It is featured by the increase and
The basal encephalocele is the rarest and of the copping of the optic nerve on the optic disk region
most difficult diagnostic and it corresponds to 1% to with the persistence of a glial tissue with a yellow
10% of all of them1,2. The basal encephalocele fre- color in the middle constituted by hyaloid remains.
quently escapes the diagnosis and may be detected The vessels follow a radial pattern to the periphery.
at adult age2,4,5. On the transsphenoidal encephalo- The coloboma is surrounded by a lifted ring of reti-
cele, the bone defect occur as a result of the chondri- nal pigmentation, which resembles the morning glory
fication of the intersphenoidal synchondrosis defect flower. Clinically, there is a decrease of the unilateral
on the sphenoid body, causing the persistence of the visual accuracy frequently associated to the displace-
craniopharyngeal canal, which normally closes itself ment of the retina, which occurs in 30% to 38% of
by the fiftieth gestation day. Its persistence allows the cases12-14.
990
Basal encephalocele: morning glory syndrome
Arq Neuropsiquiatr 2007;65(4-A) Minotto et al.
Some theories have been proposed to explain the normally deformed inside the herniation. The usage
malformation. Itakura and coworkers8 described a suc- of intravenous paramagnetic contrast media helps to
cession of events which culminated in the bottom of identify vascular structures and it might be important
the eye anomaly. According to these authors, the scir- to evaluate surgical risks17. The images must also be
rhous palate would have embryological origin on the obtained in larger areas for a complete evaluation of
first brachial arch, the palatine process, which would the brain parenchyma to disclose malformations. In
originate itself from the maxillary process, melting our case, the structures in the interior of the sphenoi-
completely with the septonasal, which in turn derives dal sinus were not characterized as brain tissue and so
from a frontal saliency around the sixtieth gestational being classified as gliocele5. The CT exam can better
day. In this stage, no internal layers of the retina and show the bone defect and it must be considered as a
of the optic nerve are well differentiated. During the complementary method of imaging diagnostic, and
seventh gestational week, the axons of the ganglionic the 3-D reconstruction facilitates this process.
cells of the retina start to form the optic nerve reach-
The skull image study is fundamental in the di-
ing its full development around the twenty-seventh
agnostic process of the ocular congenital alteration
gestational week. If a transsphenoidal encephalocele
related to the retarded growth due to a possible as-
blocks the palate fusion, and as this phenomenon
sociation to the basal encephalocele. The chosen pro-
precedes the optic nerve formation, there could be
cedure is MRI using CT as a complementary method.
an abnormal development of the nerve with a white
The association of basal encephaloceles to endochri-
glial tissue formation in its centre8,13,15. These abnor-
nal disorders and visual alteration suggests that a
malities may present themselves in many combina-
brain imaging study must be performed and com-
tions and in different degrees of severity and, there-
pleted with an exam directed to the hypothalamo-
fore, the possibility of association cannot be discard
hypophysial region.
even on the absence of exuberating clinical evidence,
but being always indicated to a detailed evaluation
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