|Year : 1980 | Volume
| Issue : 3 | Page : 161-163
IS Jain, SK Sharma, SP Dhir, RL Kaul
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
I S Jain
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jain I S, Sharma S K, Dhir S P, Kaul R L. Wyburn-Mason syndrome. Indian J Ophthalmol 1980;28:161-3
A syndrome characterised by the association of facial, retinal and intracerebral arteriovenous communication has been described under various names like Bonnet-Dechaume Blanc Syndrome,, retino-optico-mesencephalic syndrome, neuro-retinal angiomatosis and encephalo-retinal facial angioma. The occurrence of this syndrome is rare and not man\ cases have been reported in literature. Here we report a case of artereo-venous malformations involving retina, o p7 nerve conjunctiva orbit and scalp.
| Case report|| |
D.R. 17 years male (J-96821) presented with the complaints of swelling on right side of forehead accompanied by buzzing sound and redness of right eye of three months duration. He sustained injury on right side of head one year back. He also gave history of attacks of unconsciousness which started about two years back. He also feels sleepy and lethargic.
On examination there were prominent right carotid pulsations and a large A.V. fistula involving right fronto-temporal region and right upper lid [Figure - 1]. Bruit was heard all over the area. There was no other systemic abnormality found.
Ocular examination revealed visual acuity RE 6/5 and LE 6/6. Left eye was completely normal. Right eye showed mild mechanical ptosis, mild proptosis and transmitted pulsations in the lower lid area. The conjunctival vessels were markedly distended and tortuous [Figure - 2]. Cornea, iris and pupil were normal. Fundus showed that optic disc was partially covered by convolution of blood vessels which were enormously dilated, tortuous and increased in number [Figure - 3]. It was difficult to differentiate between arteries and veins. Pulsations in retinal vessels were absent. TOP was normal in both eyes. The central and peripheral fields were normal, Fluorescein angiography of right eye -bowed that dye appeared in all the vessels simultaneously in early arterial phase [Figure - 4] suggesting multiple arteriovenous communications. X-ray skull was normal. The right optic foramina was slightly larger than left but was within normal limit. Right carotid angiography [Figure - 5] revealed arteriovenous malformation with predominant feeders from superficial temporal artery and ophthalmic artery which were markedly dilated and tortuous. Vascular markings were increased in the right orbit. There was non filling of the anterior cerebral artery.
Under G.A. right external carotid artery was ligated in continuity. A large fronto temporal skin flap was reflected. The main trunk of superficial temporal artery was ligated and cut. The supraorbital and supratrochlear vessels were ligated and cut too. Arterio-venous malformations were excised and haemostasis was secured. The wound was closed in layers. The post operative period was uneventful. The lid swelling was reduced and conjunctival vessels showed some shrinkage but the proptosis and fundus picture was unchanged.
| Discussion|| |
Presence of racemose aneurysm of the retina should lead to a thorough systemic examination. The association of retinal, cerebral and facial angiomas has been well documented in the literature. Wyburn-Mason collected 27 cases of retinal arteriovenous aneurysms and found 22 cases having evidence of an arteriovenous aneurysm of the mid brain. In the two cases of racemose aneurysm of retina reported from India,, none of the case had wide speed systemic involvement. In the case reported by Seal and Kundu there was facial, retinal and suprasellar involvement in a young girl of 14 years. The case reported by Chaudhuri et a1 had only isolated involvement of the retina. The present case in addition to facial orbital and retinal involvement had conjunctival involvement.
The history of injury in the present case appears to be incidental as it appears from the details of history that trauma was of mild severity and there was no bleeding or loss of consciousness. The widespread malformations are undoubtedly of developmental origin. In the present case both internal and carotid arteries were involved in this vascular dysplasia.
Numerous neurologic maniformation varying according to the site of cerebral lesion have been described in the literature Neurological examination of the present case did not reveal any significant deficit. However the patient was dull and felt sleepy most of the time.
The diagnosis of this syndrome rests on ophthalmoscopic findings and is confirmed by radiologic examination. Carotid angiography should be carried out in every case to visualize orbital and cerebral malformations. The involvement is mostly unilateral i.e. the retinal, facial and cerebral malformation occur on th e same side of body.
Surgical removal of the facial malformation did not improve the cosmetic appearance of the patient. However the diagnosis of A.V. malformations was confirmed histopathologically.
| Summary|| |
A rare case of Wyburn-Mason Syndrome involving retina optic nerve, conjunctiva, orbit and scalp in a young male of 17 years is reported.
| References|| |
Wyburn R.M., 1943, Brain 66: 163.
Archer D.B., 1979, A.V. Amer, J. Ophthalmol.25:104.
Gutlalloude, 1979, Canadian J. Ophthalmol 14:47,
Seal, G.N. and Kundu. T.R., 1979, Ind. J.Ophthalmol. 25:52.
Chaudari, P.L., 1979, Ind. J. Ophthalmol. 2:467.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]