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| ARTICLE |
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| Year : 1966 | Volume
: 14
| Issue : 6 | Page : 260-261 |
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Congenital external ophthalmoplegia
DK Sen, A Thomas
Schell Eye Hospital, Christian Medical College, Vellore, India
| Date of Web Publication | 17-Jan-2008 |
Correspondence Address:
D K Sen
Schell Eye Hospital, Christian Medical College, Vellore
India
 Source of Support: None, Conflict of Interest: None  | Check |
How to cite this article:
Sen D K, Thomas A. Congenital external ophthalmoplegia. Indian J Ophthalmol 1966;14:260-1 |
Complete external ophthalmoplegia is very rare. Usually some movement may be elicited (partial ophthalmoplegia), sometimes very slight or only a tremor when an effort is made to follow a moving object (Cooper, 1910; Bradburne, 1912) or movement may be limited to a small excursion in one direction (Dujardin, 1894, Lawford, 1887 and others) or movement may be grossly limited in all directions (Giri, 1936). In. these cases the condition remains stationary throughout life, and frequently the vision is poor.
The anomalies are confined to the extraocular muscles but in the case described here, interesting anomalies were associated with partial external ophthalmoplegia. A case with such anomalies could not be traced in the literature available to us.
| Case Report | |  |
N., 19, male, Hindu, attended the Schell Eye Hospital, Vellore, on 2612-1964 with the complaints of pain, watering, photophobia and a foreign body sensation in the left eye following an injury with a stick 4 days back. Visual acuity was found to be in the right eye 6/18, left eye counting fingers at 3 meters.
On examination he was found to have a central corneal ulcer on the left side.
Though the patient did not complain of any other disability he was found to have a peculiar head posture with some congenital anomalies which created interest.
Head Posture:- The head was thrown back, tilted to the right a little and the face was slightly turned to the left. Eye brows were raised with a few wrinkles on the left side of the forehead. [Figure - 1].
Ptosis:- There was partial ptosis on both the sides but more on the right side, covering the upper portion of the pupil. There was practically no movement of the upper lid on both the sides when any attempt was made to look upwards.
Ocular Movements:- In the primary gaze, the right eye was divergent by 15° and a little hypotropic. Movements were grossly limited in both the eyes in all directions. Any attempt to move the eyes in any direction caused slight incycloversion and slight simultaneous divergence of both the eyes. No nystagmus was present.
Papillary Reactions were normal to light and accommodation.
Both Fundii:- Colour of the disc was normal. There was congenital deep and extensive cupping of the optic disc. (Coloboma of the disc) inferior and nasal crescents were also present. Central retinal artery was found to have divided into branches earlier than usual.
Cleft Palate and Hare Lip:- The patient gave a history of operative correction of hare lip and cleft palate in 1962. Operative scar in the upper lip on the left side was visible. When the patient was asked to open his mouth, operative scar in the palate along with two small defects were also visible. [Figure - 2].
Hydrocele :- There was a hydrocele in the funicular process on the left side which closed at the internal ring only but communicated with the tunica vaginalis.
Family History:- Patient has two elder sisters and one elder brother: none of them has any congenital deformity. Parents were found to be quite normal.
The case was treated for the corneal ulcer and when the healing was complete visual acuity had improved to 6/12 in the left eye. Visual fields were full for both white and coloured targets. No Scotoma in the fields could be detected.
Because of deep optic cupping, ocular tension was recorded repeatedly and found to be well within normal limits and all the investigations for glaucoma were found to be negative.
Though the anomalies were present from birth and were non-progressive in nature, prostigmin test was carried out to rule out any myasthenic element and was found to be negative.
| Comments | | |
Multiple congenital abnormalities in the body indicate a possible injury to the embryo before differentiation of the totipotent cells takes place by ailments like subclinical ruballa, measles and other diseases of viral and toxic origin. Though this case does not fit in with any typical symptom complex, one has to keep in mind the possibility of a genetic abnormality also.
The cause of such anomalies could not be ascertained. There was nothing particular in the antenatal history. Delivery was normal in all its stages.
| Summary | | |
A case of congenital partial external ophthalmoplegia with other congenital anomalies - a congenital coloboma of the disc, harelip, cleft palate and a hydrocele, is reported.[4]
| References | | |
| 1. | Bradburne (1912): Traps. O. Soc. xxxii, 142.  |
| 2. | Cooper (1910): Brit. Med. J., i, 917. |
| 3. | Duke-Elder. W. S. (1949), Text Book of Ophthalmology, Vol. IV, 4073: Henry Kimpton, London. |
| 4. | Giri (1936): P. R. Soc. Med., XXX, 43. |
[Figure - 1], [Figure - 2]
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