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ARTICLES
Year : 1974  |  Volume : 22  |  Issue : 2  |  Page : 27-31
 

Human cyclopia with assiciated anomalies


Department of Surgery, Medical College, Calcutta, India

Correspondence Address:
S Das Gupta
Department of Surgery, Medical College, Calcutta
India
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How to cite this article:
Gupta S D. Human cyclopia with assiciated anomalies. Indian J Ophthalmol 1974;22:27-31

How to cite this URL:
Gupta S D. Human cyclopia with assiciated anomalies. Indian J Ophthalmol [serial online] 1974 [cited 2014 Aug 20];22:27-31. Available from: http://www.ijo.in/text.asp?1974/22/2/27/31375


Cyclopia is an anatomical entity where there is partial or complete fusion of the eyes. It occurs in almost all the species of mammals and is more frequent in other classes like ayes, fishes etc.

The congenital anomaly which is designa­ted as cyclops is not limited to eye-deformities alone. There may exist various anomalous conditions of the other systems of the body, particularly of central nervous system. This condition is rare in human beings. [1],[2],[4],[8],[9],[11],[14],[15]


   Case Report Top


Mrs. A.B.C. 30 yrs, multigravida gave birth to a fullterm still born child. There was no problem in ante or post-partum period. She had two other normal children. The pedigree chart showed no abnormality in the paternal or maternal side upto second generation upwards.

The still born foetus is discussed here as a case because of rarity of cyclops in human beings.

The foetus was a full-term one. It had well deve­loped extremities and trunk. Its crown rump length was 27 cms. and weight about 1000 gms.

There was no nose. The condition is that of an ethmocephalus i. e. a nasal proboscis projects from above the median eye [Figure - 1]. A blind dimple was seen at the free end. There was a separate cavity in­side the proboscis which did not communicate exter­nally. When a stilette was passed downwards through that cavity [Figure - 3] it entered the cavity of the orbit. It did not communicate with the pharynx.

In place of two orbits there was a single orbital cavity. The orbit was diamond shaped. There were four eyelids bounding the aditus orbitae and covered the eyeballs incompletely because they were not well developed. The eye balls were lying side by side. [Figure - 1]. Each eye ball had its own cornea, iris, lens, retina etc. but the selera was fused medialy.

The extrinsic muscles showed a variable type of irregularities

Lacrimal glands : They were two in number and showed no abnormality.

Central nervous system and its coverings meninges both endosteal and meningeal layers of duramater were well developed except that the falx cerebri was totally absent. [Figure - 2]. Tertorium cerebelli was normal.

Meningeal vessels were present. Middle meningeal vessels were very prominent.

Arachnoid and piamater were fused all along as a bin single membrane and could not be differentiated.

Cerebrum : it was poorly developed and small sized. The whole cerebrum was a single sphere and there was no formation of hemispheres. The surface was com­pletely smooth because of absence of gyri and sulci [Figure - 2]. The only sulcus present was the stem of lateral sulcus. The cerebral blood vessels were few on the surface. Only the anterior part of the cerebrum was developed. The posterior part was represented by a thin-walled sac and in sagittal section it appeared to be a horse shoe shaped mass [Figure - 3]. There was no differentiation of any ventricles. Both the lateral ven­tricles and the third ventricle were fused together, dilated and opening directly into the sac. In the floor of the sac the opening of the aqueduct, with the two thalami in front and the colliculi behind were visible The lower part of the third ventricle in between two thalami was recognisable and it opened widely above in the dilated sac.

Corpus callosum, septum pellucidum and fornices were absent.

Thalami : They were fused anteriorly but posteriorly a portion of third ventricle was seen in between the two.

Cerebellum : It was poorly developed [Figure - 3]. No other abnormal feature was noticed.

Midbrain and Pons : Macroscopically no abnorma­lity was detected.

Colliculi : They were very large.

Olfactory nerves : Olfactory nerves, bulbs and tracts were absent on both sides.

Optic nerves : The two optic nerves passed through respective foramina and were quite proportionately developed. The right nerve entered the eye ball through the superomedial angle of the posterior surface.

The enterance of left nerve to the eyeball was nor­mal.

Oculomotor nerves : The nerve and all the muscles supplied by it were present.

On the left side the nerve was present and supplied the inferior oblique muscle. All other muscles were absent.

Trochlear nerves : Both the nerves were absent and so were both the superior obliques.

The remaining cranial nerves were normal.

Ethmoid vomer, nasal and lacrimal bones were absent. There was no frontal crest.

Sphenoids were normally developed. Lesser wing had cut into the brain forming as it seemed the lateral sulcus.

Occipital, temporal, parietal, zygomatic and maxillary bones were normal.

Anterior cranial fossa was undivided and steep. It was formed by only orbital plates of frontal and lesser wings of sphenoid. There was no contribution by ethmoid.

Middle cranial fossa was normal.

Posterior cranial fossa was shallow and diminished in size.

There was a fused single orbital cavity which was bounded above and laterally by orbital plates of two frontals, below and laterally by orbital surface of body of two maxillae, and laterally by zygomatic and frontals of respective sides.

Optic foramina were separate.

Superior orbital fissures were present on both sides.

Nasal Septum was absent as the bones and carti­ages were not developed at all.

Nasal air sinuses frontal and mastoid air sinuses were present. Maxillary and ethmodial air sinuses were absent.

Premaxilla, palate, eustochian tube, upper and lower jaws were present and normal.

Upper and lower incisor teeth were rudimentary and were lying hurried.

Vertebral column was normal.

Other associated malformations : External genitalia were ill developed. Penis was very rudimentary. Prepuce extended only upto the root. There was no differentiation between glans penis and body. Corporacavernosa and spongiosum were poorly developed. External urethral orifice was present. Scrotum was small.

Endocrines : Adrenals and thyroid were normal but pituitary was absent.


   Discussion Top


One of the explanations for this congenital disorder is that the primordia arises separately and undergoes a secondary fusion because of the non-development of frontonasal process and bones of nose. According to some others malformation of brain is primary for the exis­tence of a single anlage which may remain single or may undergo partial division.

Experimental work by some workers [7],[9],[19] showed that primary defect is in the anterior end of the notochord and the mesoderm surro­unding it, which results in defective induction of forebrain tissue from the overlying ectoderm.

It has now been concluded that parts are deficient from the very beginning. The defi­cient part corresponds to a wedge shaped area of anterior end of forebrain and adjacent mes­oderm at a very early presomite or even pre­neurular stage. During the course of develop­ment the eyes and neighbouring structures develop in a closed or coalesced relation from the beginning.

If defective induction of forebrain tissue is the cause of a cyclops it is not clear why in this foetus the anterior part of cerebral hemisphere had developed whereas the post­erior part did not develop at all. Further the cerebellum was also poorly developed.

Edmonds [6] concluded from his studies on the endocrines in cyclopia that pitutary­adrenal interrelationship demonstrated in post-natal life are also active in the foetus but the nondevelopment of pituitary with presence of thyroid and adrenal in this case is very striking. The absence of pituitary­adrenal axis or pituitary-thyroid interrela­tionship and their feed back mechanism should have reflection on the development of adrenal and thyroid.

The ill development of external genitalia may be due to lack of endocrine hormones of pituitary and adrenals. These two glands in the foetus control growth and differentiation of sexual development. As the pituitary is absent and adrenal is hypoplastic it may be that these have resulted in hypoplasia and ill­-development of external genitalia.


   Summary Top


A case of cyclops with details of anatomi­cal abnormal features has been presented and discussed. A review of etiogenesis has been discussed.


   Acknowledgement Top


I am thankful to prof. M. Sen, Gupta, prof. S. Mitra, prof. H. Banerjee, for his guidance in preparing this article.

 
   References Top

1.Adelmann, H. B., 1936, Quart. Rev. Biol. 11 161, 284.  Back to cited text no. 1    
2.Ballantyne, J. W., 1904, Manual of Antenatal Pathology and Hygiene. The embryo, Edin­burgh.  Back to cited text no. 2    
3.Barber, A. N. and Muelling R. J., 1950, Arch. Ophthal. 43, 989.  Back to cited text no. 3    
4.Birnbaum, R. and Blacker, G., 1912, A Clinical manual of the Malformation and congenital dis­eases of the Foetus-J. & A. Churchill, London.  Back to cited text no. 4    
5.Chapman, K., 1936, Arch, Ophthal. 16, 40.   Back to cited text no. 5    
6.Edmonds, H. W., 1950, Arch. Path. 50, 727.   Back to cited text no. 6    
7.Gartner, S., 1947, Arch. Ophthal. 37 : 220.  Back to cited text no. 7    
8.Gladstone, R. J. & Wakeley, C.P.G., 1920, J. Anat. 54, 196.  Back to cited text no. 8    
9.Gruenwald, P., 1947, Arch. Path. 44, 398, 495 and 648.  Back to cited text no. 9    
10. Meeker, L. H. and Aebli, R., 1947, Arch. Ophthal. 38, 159.  Back to cited text no. 10    
11. Naegeli, O ., 1897, Arch. Entwick, 5, 169. As quoted by R. A. Willis in-The Borderland of Embryology and Pathology-Butterworth & Co. London, 1958  Back to cited text no. 11    
12.Potter, E., 1962, Pathology of the Faetus and the new born. The year book publishers. INC. Chicago.  Back to cited text no. 12    
13.Smith, S. & Boulgakow, B., 1927 J. of An­atomy, 61 ,105.  Back to cited text no. 13    
14.Spemann, H., 1901-1903, Arch. Entwick, 12, 224: 15, 448 and 16, 551-As quoted by Willis, R. A. in 'The Borderland of Embryology and Pathology. Butterworth and Co. (Publishers) Ltd. London 1958.  Back to cited text no. 14    
15.Sperling, F., 1943, Anat. Rec. 85, 413.  Back to cited text no. 15    
16.Stockward, C. R., 1909, Anat. Rec. 3, 167.  Back to cited text no. 16    
17.Wilber, I. E., 1939, Amer. J. Ophthal. 22, 1120.  Back to cited text no. 17    
18. Willis, R. A., 1958, The Borderland of Embryo­logy and Pathology, Butterworth and Co. (Pub­lishers) Ltd., London.  Back to cited text no. 18    
19. Wright, S. & Wagner, K., 1934, Amer, J. Anat., 54, 383.  Back to cited text no. 19    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]
 
 
    Tables

  [Table - 1]



 

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