Year : 1976 | Volume
: 24 | Issue : 2 | Page : 19--20
A case of congenital corneal anaesthesia with dextro cardia and situs inversus with ASD
S Tony Fernandez1, Mary Kuriakose1, MV Joseph2,
1 Department of Ophthalmology, Little Flower Hospital, Angamaly, Kerala, India
2 Department of Paediatrics, Little Flower Hospital, Angamaly, Kerala, India
S Tony Fernandez
Department of Ophthalmology, Little Flower Hospital, Angamaly, Kerala
|How to cite this article:|
Fernandez S T, Kuriakose M, Joseph M V. A case of congenital corneal anaesthesia with dextro cardia and situs inversus with ASD.Indian J Ophthalmol 1976;24:19-20
|How to cite this URL:|
Fernandez S T, Kuriakose M, Joseph M V. A case of congenital corneal anaesthesia with dextro cardia and situs inversus with ASD. Indian J Ophthalmol [serial online] 1976 [cited 2013 May 26 ];24:19-20
Available from: http://www.ijo.in/text.asp?1976/24/2/19/31528
Congenital corneal anaesthesia is a rare condition. A combination with dextro cardia and situs inversus has not been reported in the literature as far as we know.
A 4-year old girl was brought to the ophthalmic department with hypopyon ulcer in left eye. On examination the corneal sensation was found to be absent in both eyes. Culture and sensitivity of the conjunctival discharge was taken. No organisms were cultured.
The ulcer was treated with mydriatics, local and systemic antibiotics. Subconjunctival injections of soframycin were given without difficulty in the absence of surface anaesthesia. The ulcer did not show the expected improvement with the routine treatment. Hence lateral tarsorrhaphy was done after which the ulcer showed rapid improvement and healed completely with residual leucoma [Figure 1].
While being an in-patient, the right eye also developed a corneal ulcer. In view of the corneal anaesthesia and the condition of the left eye, lateral tarsorrhaphy was done in right eye also which helped in prompt healing of the ulcer.
On routine paediatric examination, it was found that the child was mentally normal and had dextro cardia. There was no clubbing of fingers. There was an ejection systolic murmur in second and third left intercostal spaces with fixed splitting of the pulmonary second sound. There was no other neurological deficit.
X-ray of chest showed dextro cardia with situs inversus [Figure 2]. Electrocardiographically P1 is negative, PaVL is negative, PaVR and PaVF both are positive. There is no evidence of a supraventricular arrhythmia. Thus the electrocardiogram strongly suggested atrial inversion. Rr' pattern in V2 and V3R suggested an associated atrial septal defect.
Corneal anaesthesia has been described in Riley Day Syndrome as a part of the familial autonomic dysfunction with associated excessive sweating, drooling of saliva, intermittent hypertension and cyclic vomiting etc. In our case such symptoms were absent. Corneal anaesthesia due to abnormality of cranial nerves was reported by Straub as a possible cause of congenital corneal leucomata. Our patient developed corneal opacities only after the ulcer.
Lawford described a case of bilateral (congenital) corneal and conjunctival anaesthesia in a 6-year old child in 1907 which was diagnosed because of an irregular ulcer in her left eye, but there was no mention of an associated dextro cardia.
A case of bilateral congenital corneal anaesthesia, associated with dextro cardia with situs inversus has been reported.
Our thanks are due to Dr. C. K. Eapen, M. D., Medical Superintendent, for his permission to use the hospital records.
|1||Lawford, J.B., 1907, Trans. Ophthal. Soc., U. K., 21, 80.|
|2||Riley Day, Grec'ey and Langford, 1949, Paediatrics, 9, 468.|
|3||Straubs Ophthalmologica 120, 401, 1950. [As quoted in Duke Elder (1964) System of Ophthalmology, Part 11, Congenital Deformities, 514. Henry Kimpton.]|