Year : 1963 | Volume
: 11 | Issue : 4 | Page : 100--103
Anterior meningo-encephalocele with reports of three cases
JM Pahwa, HL Patney
Eye Hospital, Sitapur, India
J M Pahwa
Eye Hospital, Sitapur
|How to cite this article:|
Pahwa J M, Patney H L. Anterior meningo-encephalocele with reports of three cases.Indian J Ophthalmol 1963;11:100-103
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Pahwa J M, Patney H L. Anterior meningo-encephalocele with reports of three cases. Indian J Ophthalmol [serial online] 1963 [cited 2020 Feb 20 ];11:100-103
Available from: http://www.ijo.in/text.asp?1963/11/4/100/38967
Orbital meningoceles and encephaloceles are rare congenital abnormalities in which cerebral contents herniate into the orbit either through a defect in the bony walls of the orbit or at the sites of natural openings such as the optic foramen and spheniodal fissure. About 90 cases are on record since Brechet's original publication in 1831.
In the embryonic formation of the bony orbit, gaps occur through which cranio-orbital herniations take place. The herniation may be in the nature of meningoceles cephaloceles or hydro-encephaloceles. These may be anterior or posterior.
The anterior variety is by far the commonest, occurring between the junction of frontal and lacrimal bones, crebreform plate and nasal process of maxilla. The cephalocele protrudes from the inner canthus either into the lids or the base of the nose, often pushing the eyeball laterally and downwards.
The posterior type is less common, the herniations occurring either through sphenoidal fissure, optic foramen, posterior ethmoidal foramen or through a defect in the bony wall usually at the apex, sometimes in the roof or medial wall of the orbit.
Abnormalities of the skull and brain may co-exist as reported by Jaensch (1928, 1941) and Strandberg (1949). Majority of these cystic herniations contain some brain substance, although the latter may have undergone extreme atrophy and degeneration due to long contained pressure, so that the piaarachnoid become hardly recognizable and the dura is found fibrous and adherent to the skin. The fluid content of such cysts will still resemble the cerebro-spinal fluid.
In meningo-encephalocele abnormalities of the eye are unusual. It constitutes a rare cause of congenital exophthalmos. Of the 31 cases collected by Strandberg (1949) four had microphthalmos and one had coloboma of optic nerve. The other abnormalities of the eye recorded are coloboma of iris, microphthalmos, hydrophthalmos, and apparent anophthalmos. Duke-Elder (1952).
Vascular anomalies may also coexist. In Strandberg's case the carotid artery was peculiarly twisted in relation to the dura mater. Dandy (1929) found a large extra-dural vein at operation which seemed to have replaced the cavernous sinus, and the internal carotid artery was absent on the affected side.
The clinical picture is evident at birth, in early infancy or in childhood. Females are affected about twice as commonly as males. There is no evidence of any hereditary tendency. Trauma to the head may accentuate the condition, or falsely appear to be a precipitating cause. Internal hydrocephalus, orbital infections, and neoplasms are rarely exciting factors.
The essential symptom when the orbit is involved is a proptosis showing pulsations which are reduced on pressing both arteries or on withdrawal of cerebro-spinal fluid. Coughing or straining usually increases the protrusion of the eye. Pressure on the eye may produce cerebral symptoms such as slowing of pulse, giddiness, nausea and even convulsions. The exophthalmos may not pulsate at all.
In the anterior type, the cephalocoele appears at the inner angle of the eye, which progressively displaces the globe according to the situation and size of the cyst, exposing the cornea to keratitis, ulceration and its complications. Extreme displacement may cause luxation of the eyeball.
In the absence of pulsation and bony defects, the diagnosis may be difficult and may be wrongly diagnosed as an orbital cyst and so operated upon light-heatedly.
On X-Ray examination, typical bony defects opposite the cyst may be visualized. Aspiration of fluid resembling cerebro-spinal fluid from the cystic tumour will clinch the diagnosis.
Pneumoencephalography may further aid by detecting escape of air in the subarachnoid in the orbit, and demonstrating any asymmetry or deformity in the ventricular system.
Similarly angiography may reveal any associated vascular anomaly.
The treatment of this condition is unsatisfactory and carries a high mortality if excision is attempted. The surgical interference involves excision of the cyst, repair of bony defects and enucleation of the eyeball in extreme proptosis.
Case No. 1. Jakhoo, Hindu male child, 9 years old was brought to us on 17th November 1957 with the complaint of a swelling since birth on both sides of the inner angles of the orbits extending more on right side. It remained stationary for nearly 3-4 years but after that time it started increasing gradually.
Family history was of no significance. There was no history of illness in the mother during pregnancy nor of the use of forceps during delivery. On examination there was found to be a big cystic swelling across the bridge of the nose between the inner angles of the eyes extending more to the right. [Figure 1] The right eyeball was pushed down and out with chemosis of conjunctiva and oedema of both lids particularly the lower one. The left eye was also slightly pushed out and somewhat down. The swelling showed some faint cerebral pulsation which became less on compression of both carotid arteries. It was slightly reducible by pressure but on firm and prolonged compression the pulse rate became reduced from 84 to 79 per minute and the child felt some giddiness. There was no other physical abnormality in any other part of the body, but mentally be appeared backward.
X-Ray examination was interesting and revealed slight enlargement of the right orbit with defective nasal wall and roof and wide orbital fissure. The diagnosis was confirmed and clinched by aspiration of a clear colour-less fluid chemically resembling cerebrospinal fluid.
Case No. 2. Sabha Pati, 1 year old Hindu male child was referred to us on 16th July 58 from our Faizabad branch. The complaint was of a swelling in the right inner angle since birth and difficulty in breathing during sleep if the child was lying on the right side. The swelling became slightly less when the child was sleeping and it increased on crying.
Family History:- Our patient is the third child while the second child died of jaundice at the age of 10-12 days. No forceps were used during delivery. There was no history of fever or illness of the mother during pregnancy.
Examination:- There was a cystic swelling on the inner side of the right orbit which was pushing the eyeball out and slightly up. The swelling was extending to the nose, and right lower lid as well [Figure 2]. It increased when the child cried and diminished during sleep. No other abnormality was seen. The eyeballs were also normal. The swelling was slightly reducible to pressure but filled up again. No cerebral effects were met with on compression of the swelling. X-Ray of the skull, showed some defect in the nasal wall of the right orbit which was also somewhat wider. The diagnosis was confirmed when on aspiration a clear fluid was taken out which on. analysis resembled cerebro-spinal fluid. The child is still coming to us off and on. The swelling is almost of the same size. He was advised to get surgically treated at Vellore but so far it has not been possible for him to go. He was cautioned against getting the cyst aspirated or operated otherwise.
Case No. 3 . Kallooprasad, 4 months old male child was brought to us on 14ht May 1960 with the complaint of a cystic swelling on the inner angle of left orbit since birth which was slowly increasing, causing oedema of both the lids so that the left eye could be opened with difficulty.
Examination:- In addition to the cystic swelling on the inner side of left orbit, which was displacing the left eye down and out, there were two cystic nodules [Figure 3] on the front of the nose below this cystic swelling. These nodules resembled the proboscis of Arhincephaly but there was no orifice at its distal end and moreover the nose was well formed.
The right eye was enophthalmic, while the left eye had microcornea with coloboma of iris. The pupil was updrawn and there was a cystoid cicatrix at 12 O'clock. As the child could only see from this eye the parents became nervous when oedema increased and covered the eye.
The cystic swelling was reducible and there was a faint cerebral pulsation on crying. X-Ray examination was not very helpful as the child could not co-operate. Aspiration of fluid through the cystic swelling and one of the nodules was done and clear fluid was taken out which resembled cerebrospinal fluid.
These three cases are reported because of the rarity of the condition and because of the interesting association of microcornea and enophthalmos in one of them.
Another interesting feature is that all throe of them were males as opposed to female preponderance in the literature.
All the three cases presented themselves in the first decade of life, two of them in the first year. In all the three, the swelling was typical of the anterior variety at the inner angles of the eye, which could be reduced by bilateral pressure on carotid arteries. Proptosis and ocular displacement were present in all the three cases. Oedema of the lids was present in two of them.
The diagnosis was confirmed in all the three by visualization of skull defects by means of X-Ray and by examination of the aspirated fluid except that in case 3, X-Ray examination could not be done satisfactorily.
One added interesting feature of the third case was the presence of multiple cystic swellings, one displacing the eyeball and the other two on the nose.
Clinical features of anterior meningo-encephaloceles are described.
Interesting features of three cases are reported.
|1||Dandy (1929), Arch. of Ophthal. 2,123.1|
|2||Duke-Elder. S. W. (1952) Tex-book of Ophthalmology Vol. V p. 4754, Kimpton. London.|
|3||Jaensch K. M. (1926) as cited in 1, p. 4756.|
|4||Jaensch K. M. (1941) as cited in 1.p. 4756.|
|5||Strandberg (1949) A. N. A. Arch.Ophthal. 42, 254.|