|Year : 1983 | Volume
| Issue : 2 | Page : 93-94
SPT Sambandam, NR Prithivirajan
154 D.B. Road, R.S. Puram, Coimbatore-641 002
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sambandam S, Prithivirajan N R. Arhinencephaly unilateralis. Indian J Ophthalmol 1983;31:93-4
Arhinencephaly unilateralis is a rare congenital deformity which has the same fundamental disturbance as cyclopia, limited however to oneside. Here the lateral nasal process develops anomalously, so that the nostril on one side is represented by a proboscis which projects outwards from the region of the upper and inner angle of the orbit and hangs penduloulsly on the face, resembling the central proboscis in cyclopia.
| Cast report|| |
A six year old boy was brought with a complaint of left lower lid abcess by his mother. On examination, his right eye was normal. In the left eye there was an abcess below the lower lid: which was tender and fluctuant. There was a coloboma of the lower lid at the junction of medial 1/3 with the lateral 2/3 [Figure - 1]. Both lacrimal puncta were seen and there was a lacrimal fistula with a mucus discharge. Fluorecine drops instilled into the conjunctival sac came through the fistulous opening. In left eye fundus, there was a coloboma of the disc with congenital pigmentation on the temporal side of the disc. Both eyes were hypermetropic R.E. +1.5 D.Sph. L.E.+3.0 D.Sph.
The nostril on the left side was absent and there was a proboscis which was projecting outwards from the region of the upper and inner angle of the left orbit. It was hanging pendulously down on the face. It was covered with normal skin but had no lumen. It was slightly shorter than the right nostril. On the right side the nostril was normal with a bony cartilagenous septum normally placed. Skull, other parts of the face and palate were normally developed. No other congenital anomalies were detected in any other system of the body.
X-Ray of the left orbit revealed a round radio-opaque shadow at the root of the proboscis. X-Ray Skull did not reveal any other abnormality. Blood VDRL for both the mother and child was negative.
| Family history|| |
The child was the first child of the family and was the product of full term uneventful pregnancy. The parents were not consanguinously related and there had been no history of ocular and other congenital deformities in the family. Other younger siblings were normal in all respects. Surgery was advised but refused by the parents.
| Discussion|| |
Unlike cyclopia, Arhinencephaly Unilateralis is compatible with life as evidenced in this case where the boy has grown up to six years with normal health. On the affected side the eye may be grossly malformed. Duke Elder cautions that in certain cases the central canal of the proboscis can be traced in the depths orbital cavity to the region of the cribriform plate and the olfactory lobes-a point to be remembered in undertaking any operation for its excision and plastic repair, which may involve the danger of fistula and meningial infection.
In Arhinencephaly Unilateralis, the lateral nasal process develops anomolously, so that one side of the future nose has separated completely except at the top, where it remains attached to the paraxial mesoderm covering the forebrain with the result that maxillary process runs to the mid line. The development of lacrimal apparatus is also affected. As in this case the nasolacrimal duct may open outside.
| Summary|| |
A case of Arhinencephaly Unilateralis, which is a rare congenital anomaly is reported. The eye on the affected side had lacrimal fistula, coloboma of the lower lid, coloboma of the disc and congenital pigmentation of the fundus.
| References|| |
Duke Elder. S. 1964, System of ophthalmology. Vol. HE Part.2.p. 442-44. Henry Kimpton London.
[Figure - 1]