|Year : 1981 | Volume
| Issue : 2 | Page : 121-123
Human cyclopia with associated microstoma and and anencephaly
RC Gupta, VK Gupta, Sneh Gupta
Department of Ophthalmology, General Hospital, Ambala City, India
R C Gupta
Civil Hospital Ambala City, Haryana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta R C, Gupta V K, Gupta S. Human cyclopia with associated microstoma and and anencephaly. Indian J Ophthalmol 1981;29:121-3
Cyclopia is a condition where elements of the two eyes are fused completely or partially to form a single midline eye in the forehead. Complete fusion of two globes constitutes a cyclop whereas partial fusion wherein some or all the ocular structures are paired within a single globe is called a synophthalmos. The sclerae may be fused and all the other structures duplicated except the optic nerve. The optic nerve may be single, divided or duplicated.
The palpebral aperature is usually large and elliptical or diamond shaped. In many cases a single arched fold of skin represents the lids., In some rare cases fused upper lids are composed of two parts which meet in the midline in a notch. The lower lids on the other hand are, usually divided by a notch in the midline. The so divided two halves have an orally directed angle and in the angle a single caruncle may be present as a rarity. The lids are usually, rudimentary and do not have a tarsal plate. These have fine hairs at the margins instead of cilia.
Cyclop has a grossely deformed orbit. Here the nasal cavity is absent and a single central cavity - the pseudo-orbit is present. Nose is usually represented by a single snout like proboscis which is the rudimentary fronto nasal process. This proboscis is usually seen in the midline just above the orbit.
Cyclop is often associated with anencephaly wherein the head is imperfectly developed, particularly the vault of the skull and the brain. A few may be born alive but die within a very short time. Hydramnios is frequently associated with this condition.
| Case report|| |
A twenty year old lady was admitted in this hospital as a case of full term pregnancy. There was no history of any venereal disease, diabetes mellitus, pre-eclamptic toxaemia, renal disorders, rubella or any other viral disease, exposure to X-rays. However, there was history of having taken some indigenous medicine during first trimester of pregnancy in the hope that the medicine would help continue her pregnancy as she had already aborted once after having conceived after six years of marriage. She had also been exposed to total solar eclipse during first trimester of pregnancy.
At the time of admission she had overdistended abdomen. Foetal parts were not clearly palpable and foetal heart sounds not heard.
She went into labour and she delivered an anencephalic, synophtha lmic gasping female baby [Figure - 1]. This abnormal baby died half a minute after birth.
The baby had a single fused eye in the centre of the forehead [Figure - 1]. There was present a single snout like proboscis overhanging the large diamond shaped palpebral fissure. The palpebral fissure measured 2.50 cm. x 2.50 cm. The lids though rudimentary, contained almost all the elements. Eye lashes were present in all the lids but were scanty in the lower lids. Tarsal ducts were visible through the thin hyperaemic conjunctiva. Conjunctival fornices were present. There were no puncta and no lacrimal sacs. The upper lids were continuous whereas the lower lids were separated by a clear cut gap of 2 mm. Symblephron could be seen on each side in the midline.
Paired ocular structures were seen in a single globe. The corneae were elliptical in shape and each measured 5 mm. x 2 mm. There was conjunctival encroachment over the medial side of both the corneae. Pupils were eccentric and each measured 1.5 mm. in diameter. Iris was brownish in colour. Anterior chambers were well formed and the lenses were clear. Ophthalmoscopically the media were clear and part of disc was visible in both the eyes. There was a soft emminence measuring 10 mm. into 10 mm. in the upper and a hard elevation in the lower angle of the palpebral fissure.
The proboscis measured 30 mm. in length and 12.5 mm. in diameter. The free end of this tube had a depression of 5 mm. diameter and in the depression was present a hole 1mm. in diameter. The tube was patent throughout its whole length, though it did not communicate with the lungs. The oral fissure measured 15 mm. only.
The limbs and feet were symmetric and were of normal shape. There was no other abnormality. Necropsy was not permitted and hence the internal anatomy could not be studied.
| Discussion|| |
Cyclopia or synophthalmia has, since long, been a subject of curosity because of its typical appearance. It is a congenital anomaly but it is not hereditary because life is impossible in them. Here the child is born dead or dies soon after birth, so that no cyclop has ever reproduced. No familial incidence has ever been reported except by Klopstock.
There are different views expressed in literative about the causation of this human monstrosity.
The present case in fact is a case of synophthalmos. Here almost all the structures are double. Even the optic disc is double and is visible ophthalmoscopically and there may possibly, be two separate optic nerves. The striking feature in this case is the visibility of fundus apart from completeness of each unit of synophthalmos. Another added feature is the association of microstoma which in itself is very rare.
| Summary|| |
A case of cyclop associated with anencephaly and microstoma is reported.
| Acknowledgements|| |
We thankfully acknowledge the help of Dr. Pushpa Garg, Dr. Abha Kulshrestha and Dr. Gurmeet Saini of Deptt. of Gynaecology and Obstetrics of this hospital.
| References|| |
Gartner, S., 1947, Arch. Ophthalmol., 37:220.
Kindred. J.E., 1945, Arch. Ophthalmol, 33:217.
Wilber, I.E., 1939, Amer. J. Ophthalmol. 22:1120.
Chapman, K.H., 1936, Arch. Ophthalmol., 16: 40.
Klopstock, A., 1921, Monatschr. F. Geburtsh. U. Gynak., 56: 59.
[Figure - 1], [Figure - 2], [Figure - 3]