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CASE REPORT
Year : 1993  |  Volume : 41  |  Issue : 2  |  Page : 88-90

Pulsating enophthalmos in aplasia of sphenoid wing


Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
Vimala Menon
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


PMID: 8262612

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How to cite this article:
Menon V, Vashisht S, Gupta K K, Singh J, Prakash P. Pulsating enophthalmos in aplasia of sphenoid wing. Indian J Ophthalmol 1993;41:88-90

How to cite this URL:
Menon V, Vashisht S, Gupta K K, Singh J, Prakash P. Pulsating enophthalmos in aplasia of sphenoid wing. Indian J Ophthalmol [serial online] 1993 [cited 2020 Nov 23];41:88-90. Available from: https://www.ijo.in/text.asp?1993/41/2/88/25613

Unilateral aplasia of sphenoid wing is a rare disorder. Its association with neurofibromatosis has been stressed, [1],[2] the most impressive and common clinical feature of which is a pulsating exophthalmos. However, aplasia of sphenoid wing showing pulsating enophthalmos with other stigmata of neurofibromatosis has also been described in the literature. [3],[4],[5],[6[,]7],[8], This report also describes a case of aplasia of sphenoid wing with pulsating enophthalmos but without any other stigmata of neurofibromatosis.


  Case report Top


A 14-year old male complaining of pulsations in the left eye of two weeks duration was examined. At the time of birth his parents had noted prominence of the left eye and fullness over the left temporal fossa. Cafe-au-lait spots were not seen in the patient or in any other family member. A general physical exami­nation revealed fullness of the left temporal fossa [Figure - 1]. The fullness was bony and the soft tissue over the prominence was normal.

Visual acuity measured 6/5 in both eyes. The left eye was enophthalmic (Hertel's 15mm & 12mm at 93 base reading) and pulsatile, the pulsations being syn­chronous with the carotid pulse. The left eyeball proptosed by 4mm on Valsalva maneuver and by 2mm on head hanging. The left eyeball could also be pushed back 6-7mm into the orbit without any discomfort to the patient. No thrill or bruit was detected over the eye ball, forehead or neck. Ocular motility, fundus and visual field examination were normal.

Orbit radiography and CT scan [Figure - 2] revealed en­largement of the left orbital ring with partially defi­cient greater wing of sphenoid, elevation of the lesser wing of sphenoid and lateral displacement of the innominate line. The middle cranial fossa was en­larged anteriorly, laterally and downwards. These findings were suggestive of sphenoid wing aplasia. The carotid angiography was interpreted as normal. B-Scan ultrasonography showed the presence of a lat­erally situated, well-defined mass in the orbit [Figure - 3]a which increased in size on Valsalva maneuver [Figure - 3]b. The orbital fat was considerably reduced in thickness [Figure - 4]a and M-Scan ultrasonography showed intracranial pulse waves transmitted to the eyes and the orbital structure [Figure - 4]b.


  Discussion Top


Aplasia of sphenoidal wing is clinically character­ised by painless pulsatile exophthalmos, absence of bruit or thrill and any diplopia or neurological defi­cit. [1],[2] The diagnosis is confirmed by the characteristic radiological features of enlarged bare orbit. [2],[9],[10],[11],[12],[13]

The pulsations are due to intracranial pulse waves transmitted to the orbital contents through a major defect in the orbital wall. [1],[2],[13],[14],[15],[16] In this case M-Scan ultrasonography showed transmitted intracranial pul­sations to the eye and orbital structures.

Exophthalmos is the common presentation of apla­sia of sphenoid wirig while this case had shown eno­phthalmos in erect posture. Review of the literature has revealed only seven such reported cases of pul­sating enophthalmos. [3],[4],[5],[6],[7],[8],[10] However Savino [8] believed that two of these cases were not due to the aplasia of sphenoid wing while the other two reports gave only obscure details. [5],[10] Previous authors have suggested that the enophthalmos was due to atrophy of orbital fat.

In this case, A scan ultrasonography revealed a less dense retro-orbital fat pad which in the absence of history of trauma could be due to constant intracra­nial pulsation transmitted to the orbit causing fat atrophy. The increased volume of the orbital cavity and middle cranial fossa could also contribute to the enophthalmos. Cases of aplasia of sphenoid wing may show enophthalmos or exophthalmos depending on the extent of encephalocoele, the orbital contents and the capacity or volume of the orbit and middle cra­nial fossa. Aplasia of sphenoid wing is associated with neurofibromatosis in over 50% cases. The na­ture of this association, however remains obscure [2]sub .

All the reported cases of pulsating enophthalmos had other features of neurofibromatois; the patient re­ported here did not have any other stigmata of neu­rofibromatosis.

 
  References Top

1.
In Francois J. Ocular aspects of the phakomatoses. In Vinken P1, Bruyn GW. (eds) Hand Book of Clinical Neurol­ogy. Vol. 14. Amsterdam. North Holland Publishing Com­pany. pp 619-667, 1972.  Back to cited text no. 1
    
2.
Myrianthopoulos NC. Other Skull Defects. In Vinken PJ, Bruyn GW. (eds) Handbook of clinical Neurology. Vol. 30. North Holland Publishing Company. Amsterdam. pp 269-284, 1977.  Back to cited text no. 2
    
3.
Dandy WE. An operative treatment for certain cases of meningocele (or Encephalo cele) into the orbit. Arch Oph­thalmol. 2: 123-132, 1929.  Back to cited text no. 3
    
4.
Franceschetti A and Streiff EB. Rapporti tra Neurofi­bromatosi (Rechlinghausen) Stato Disrafico (Bremer) atti XXXIV Cong. Soc. Ital. Oftal. 34: 365-369, 1937.  Back to cited text no. 4
    
5.
Kelly RE. Cited by Savino PJ, Glaser JS and Luxen­berg MN. Br J Ophthalmol. 61:482, 1977.  Back to cited text no. 5
    
6.
Lenshoek CH, Van Manen J, Notermans SLH and Penning L. Cited by Savino PJ, Glaser JS and Luxenberg MN. Br J Ophthalmol. 61: 482, 1977.  Back to cited text no. 6
    
7.
Nikiforoy BM. Pulsatile Exophthalmos in Von Rech­linghausen's disease (Russian) Vestn. Oftal. 79: 77-79, 1966.  Back to cited text no. 7
    
8.
Savino PJ, Glaser JS and Luxenberg MN. Pulsating enophthalmos and choroidal hamartomas. Two rare stigmata of neuro-fibromatosis. Br J Ophthalmol. 61: 483-488, 1977.  Back to cited text no. 8
    
9.
Binet EF, Kieffer SA, Martin SH and Peterson HO. Orbital dysplasia in neurofibromatosis. Radiology. 93: 829­833, 1969.  Back to cited text no. 9
    
10.
Burrows EH. Bone changes in orbital neurofibroma­tosis. Br J Radiol. 36: 549-561, 1963.  Back to cited text no. 10
    
11.
Holt JF and Wright EM. The radiologic features of neuro fibromatosis. Radiology. 51: 647-664, 1948.  Back to cited text no. 11
    
12.
Hunt JC and Pugh DG. Skeletal lesions in neuro­fibromatosis. Radiology. 76: 1-20, 1961.  Back to cited text no. 12
    
13.
Robertson RG. Pulsating exophthalmos due to defec­tive development of the sphenoid bone. Am J Roentgenol­ogy. 12: 44-51, 1949.  Back to cited text no. 13
    
14.
Bruwer A, and Kierland RR. Neurofibromatosis and congenital unilateral pulsating and non-pulsating exophthal­mos Arch. Ophthalmol. 53: 2-12, 1955.  Back to cited text no. 14
    
15.
Debezies OH and Walsh FB. Pulsating enophthalmos in association with neurofibromas of the eye lid. Trans Am Acad Ophthalmol. Otolol 65: 885-887, 1961.  Back to cited text no. 15
    
16.
Rovit RL and Sosman MC. Hemicranial aplasia with pulsating exophthalmos. An unusual manifestation of Von Recklinghauseri s disease. J Neurosurg 17: 104-121, 1960.  Back to cited text no. 16
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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