Journal of Postgraduate Medicine
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CASE REPORT
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Year : 1991  |  Volume : 37  |  Issue : 1  |  Page : 51-2,52A  

Iniencephaly with cyclopis (a case report).

S Ramakrishnan, D Sharma, V Ramakrishnan, PS Parihar, S Sharma, DK Kanther 
 Department of Paediatrics, R.N.T. Medical College, Udaipur, Rajasthan.

Correspondence Address:
S Ramakrishnan
Department of Paediatrics, R.N.T. Medical College, Udaipur, Rajasthan.

Abstract

Iniencephaly is a rare neural tube defect. We report a rare association of iniencephaly with cyclopia, probably the third such report in the literature.



How to cite this article:
Ramakrishnan S, Sharma D, Ramakrishnan V, Parihar P S, Sharma S, Kanther D K. Iniencephaly with cyclopis (a case report). J Postgrad Med 1991;37:51-2,52A


How to cite this URL:
Ramakrishnan S, Sharma D, Ramakrishnan V, Parihar P S, Sharma S, Kanther D K. Iniencephaly with cyclopis (a case report). J Postgrad Med [serial online] 1991 [cited 2021 Oct 23 ];37:51-2,52A
Available from: https://www.jpgmonline.com/text.asp?1991/37/1/51/801


Full Text




  ::   IntroductionTop


The diagnosis of iniencephaly rests on the following criteria set forth by Ballantyne1 i) imperfect formation of base of skull, mainly in the vicinity of foramen magnum; ii) retroflexion of the whole spine forcing the face of the foetus to look upwards and its head to pass directly on to the lumbar region and iii) rachischisis. Iniencephaly has been found to be associated with many malformations. The association of iniencephaly with cyclopia is very rare. We report a case of this association.


  ::   Case reportTop


A female aged 22 years delivered spontaneously at 32 weeks of gestation. The antenatal period was uneventful and there was no history of rash, fever, drug intake and hydramnios. 'There was no family history of any such or other fetal malformation. 'The marriage was not consanguineous. All investigations of the mother were normal.

Examination revealed a 1.5 kg stillborn female foetus. The head was small, flat and retroflexed. There was no neck and the skin over the face continued on to the thoracic skin (See [Figure:1]). There was a diamond shaped fissure on the centre of the forehead having a globe containing two cornea and pupils with single conjunctiva. 'The anterior-most structure over the face was the mouth. Behind the globe was a 3.5 cm long proboscis having a central opening (See [Figure:2]).

Skia-gram revealed no clear-cut distinction between individual cranial bones. There was overcrowding of ribs as well as cervicothoracic vertebrae with marked lordosis. The spinal canal was totally open.


  ::   DiscussionTop


Iniencephaly may be associated with other malformations like encephalocoele, absence of mandible, cleft palate, diaphragmatic hernia, single umbilical artery, low set ear and hydramnios. Cyclopia as an association with Iniencephaly has probably been reported only twice before2,4. Our case fulfilled all the criteria described by Ballantyne1. The incidence of iniencephaly in India is reported to be 1 in 65,000 deliveries3. It is much more common in female foetuses.

Antenatal radiography and ultrasonography help diagnosis in cases with hydramnios. It is believed that the failure of development of both paravertebral sclerotomes of the ventral and dorsal masses is the primary defect responsible for this malformation. Environmental factors like maternal syphilis, sedative intake etc. have also been implicated but there is no evidence that such factors influence the occurrence of iniencephaly in man.

References

1 Ballantyne JW. The embryo. In: "Manual of Antenatal Pathology and Hygiene," Edinburgh: W Green and Sons; 1904, pp 272-285.
2Bhandari B, Tak SK, Mehta R, Agrawal RK. Iniencephaly with several rare associated anomalies. Indian Pediatr 1982; 19:722-723.
3Jayant K, Mehta A, Sanghvi LD. A study of congenital malformations in Mumbai. J Obstet & Gynaecol India, 1961; 11:280-294.

 
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