|Year : 1969 | Volume
| Issue : 4 | Page : 158-159
Marchesani's syndrome- case report
Department of Ophthalmology, Government Hospital, Masulipatam, (A. P.), India
|Date of Web Publication||10-Jan-2008|
C M Sarma
Department of Ophthalmology, Government Hospital, Masulipatam, (A. P.)
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sarma C M. Marchesani's syndrome- case report. Indian J Ophthalmol 1969;17:158-9
Marchesani's syndrome is otherwise known as Dystrophia Mesodermalis Hyperplastica. This rare condition was first described in 1939 by Marchesani. In this condition, there is hyperplasia of the mesodermal tissues. Probably both mesodermal and ectoderrnal elements play a part in the etiology of this syndrome.
Marchesani's syndrome is familial and congenital. It is recessive in character. It is characterised by short stature, stocky figure, short limbs, well developed musculature, thick skin and subcutaneous fat, short squat fingers and toes, with broad spade like hands and feet, and broad and square head-There is delayed carpal ossification.
Ocular defects include spherophakia, microphakia, ectopia lentis, lenticular myopia and secondary glaucoma. Chorioretinal changes are rare.
| Case Report|| |
H.B., a Muslim girl aged 12 years, was brought to the ophthalmic out patient department of the Government Hospital, Masulipatam on 2-11-1968 with the complaint of defective vision both eyes, more in the left eye.
The present complaint was noticed when the child went to school at the age of six years. She was not able to see what was written on the black board. She was advised to sit on the front bench by her teacher so that she could see well. She was taken to many native doctors who treated her with eye drops and some injections.
She has one brother and sister and they do not have similar complaints. They are healthy, and well built. History of consanguinity was not present.
| General Examination|| |
Height : 40 inches
Span with extended upper extremities -38 inches
Upper body segment-20 inches
Lower body segment-20 inches
Ratio of the segments-1 : 1
| Skeletal Features|| |
She was short, with thick and broad face and short and thick extremities. Hands and feet were spade like. The fingers and digits were small, thick and broad.
| Ocular Features|| |
Ocular Features: -
Cornea. Both cornea were clear but had keratoglobus like enlargement.
The anterior chambers were deep.
The iris was tremulous in both the eyes.
Pupils: The right pupil was reacting well to light and accommodation, whereas in the left eye the reaction was weak.
Both the lenses were subluxated.
Fundi oculi : There was temporal pallor of the optic disc on the right side whereas there was marked glaucomatous optic atrophy with cupping of the disc in the left eye.
The tension was 20 mm Hg. right eye, 50 mm Hg. left eye.
In the right eye, vision was counting fingers at a distance of four meters, improving upto 6/24 with -3.0 D sph.
In the left eye it was only perception of light with no projection.
TREATMENT: The patient was not willing to undergo any surgical treatment. For the right eye 3.0.D. Sphere and plane glass for the left eye were prescribed as there was improvement of vision in the right eye. Patient was asked to report back whenever she developed pain and redness in the eyes.
| References|| |
Dhanda. R. P.:
Marchesani's syndrome -J. All India Ophthal. Soc. 15, 193-196 (1967).
Duke Elder. W. S.: System of ophthalmology--Vol. 111. Part 2. Pp. 1107-9. Henry Kimpton, London (1964).
F.: Marchesani's syndrome- British J. Ophth. 45,
[Figure - 1], [Figure - 2], [Figure - 3]