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Year : 1976  |  Volume : 24  |  Issue : 4  |  Page : 38-40

Empty sella syndrome with neurofibromatosis


R. N. T. Medical College, Udaipur, India

Correspondence Address:
R L Agrawal
Reader in Ophthalmology, R. N. T. Medical College, Udaipur
India
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Source of Support: None, Conflict of Interest: None


PMID: 411749

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How to cite this article:
Agrawal R L, Bhargava S, Samma A H, Kothari A K, Bedi H K, Shrimali R L. Empty sella syndrome with neurofibromatosis. Indian J Ophthalmol 1976;24:38-40

How to cite this URL:
Agrawal R L, Bhargava S, Samma A H, Kothari A K, Bedi H K, Shrimali R L. Empty sella syndrome with neurofibromatosis. Indian J Ophthalmol [serial online] 1976 [cited 2021 Apr 19];24:38-40. Available from: https://www.ijo.in/text.asp?1976/24/4/38/31285

A rare case of Neurofibromatosis with empty sella syndrome was discovered acciden­tly during a search for bony involvement of skull.

Neurofibromatosis is a hereditary congeni­tal disease characterized by widespread hyperplasia and neoplasm of supportive tissue of the whole nervous system.

Isolated lid tumours are insidious, seen in children who have similar lesions of other ocu­lar tissues, involving the temporal portion of the upper lid first, with dark and hairy skin. Destruction of orbital bone is frequently seen. Exophthalmos is less common.


  Case Report Top


A 18-year, female, admitted complaining of gradually increasing painless swelling of right upper lid since birth and was unable to see while looking straight without lifting the lid, for last five years due to rapid growth of swelling. Her mother also had multiple nodules of varying size over the body, more marked on the face.

She was well built and well nourished, and had multiple nodules with cafe-au-lait spots varying from pin head to pea size all over the body.

Other Systems : Other systems were normal.

Local Examination : Upper Lid of the right eye. There was a large nodular firm swelling of 3½" x 2½" x 1" size extending from orbital margin and hanging due to its own weight covering the zygomatic region [Figure - 1]. Lid was stretched dragging eyebrow downwards having ectropion. There were two more swellings of 1¼"x 1" ½" size laterally. The skin over them was pigmented and adherent to deeper tissues at places.

Lower Lid : Had a firm mobile non-pulsatile swelling of 1½"x l" x½" size on the lateral half and no bruit could be heard.

Palpebral fissure was slit like with restricted move­ments of eyeball due to symblepharon. [Figure - 2].

Conjunctiva was congested and hypertrophic. Ad­enexa, anterior-chamber, pupil, iris and lens were found to be normal.

Vision was counting fingeral 2½ ft. on lifting the growth.

Fundus : Details could not be seen due to lid growth. Left eye was normal.

Investigations :
Nothing abnormal detected in C.S.F., urine and blood except ESR which was 20 mm. in 1hr.

X-Ray Skull: Sella turcica enlarged with no evidence of raised intracranial tension [Figure - 3].

Measurements of Pituitary fossa

Normal Patient

Anterior to posterior edge 6-8 mm 12 mm

From highest concave point

anterior to posterior. 8-10 mm 21 mm

Depth from Centre 8-10 mm 13 mm

Pneurnoencephalography: Pituitary fossa was enlarged with air seen in the pituitary fossa, temporal, occipital and supra sellar regions. The rest of skull bones normal [Figure - 4].

Biopsy : Histopathology shawe the hemour to be lifical case of neuro fibroma [Figure - 5]


  Discussion Top


The term empty sella syndrome was des­cribed as a specific gross anatomical variation where an incomplete diaphragm sellae encom­passed a large opening above the flattened hy­pophysis which lined the sella floor and incom­pletely filled the sellar cavity, hence the im­pression of empty sella[3], which may be (1) Idiopathic or (2) Secondary.

In idiopathic type the incomplete diaph­ragma sella apparently allows the arachnoid to herniate through the diaphragmatic opening. This pouch may enlarge because of hydrostatic pressure of CSF and compress the pituitary tissue against the sella and the floor of the sella becomes relatively -&-mineralized. The empty sella syndrome is a distinct anatomical radiographic entity characterised by remodell­ing and nontumorous enlargement of the sella due to incompleteness of diaphragma sella, where the cerebi o-spinal fluid pressure may be raised. The changes range from a normal to balloon shaped sella and to an enlarged globu­lar sella.

Secondary type occurs following surgery, irradiation of an intraseller tumour which may cause visual impairement.

The case of idiopathic type was diagnosed on the basis of (1) Skiagram Skull showing ballooned pituitary fossa with no evidence of increased intra cranial tension, (2) Phenumoe­nephalography revealed empty sellar space filled with air. (3) No clinical evidence of pituitary or thyroid involvement. (4) no history of operation or irradiation.

Many cases previously reported by Kaufmn[1], and Stephen et a1[2], were discovered casually during the search for conditions unrelated to pituitary and same happened with this case. The literature shows only 33 cases and in 4 cases only pituitary was involved. As there is no involvment of pituitary in majority of cases of empty sella syndrome, a routine radiogra­phic examination is not done, but there may be many more cases of idiopathic empty sella, which can be proved by radiographic examina­tion including pneumo-encephalography.

It is important to bear this entity in mind to avoid suspicion for a pituitary tumour which may create ugly misgivings and disturbing anxieties in the patients as well as the unwary treating clinician.


  Summary Top


A case report of 18 years young girl who presented a large swelling of both the lids of right eye and generalised neurofibromatosis, X-Ray skull revealed a large sella, the patient did not show any pituitary and thyroid disfun­ction. Pneumo-encephalography confirmed the presence of an empty sella.

 
  References Top

1.
Kaufman, B. 1968. Radiology, 90, 931.  Back to cited text no. 1
    
2.
Stephen, F. H., Reymond, R., Colon, B.H. and Collen, S. M., 1972, The Medical Clinic of North America 56 897.  Back to cited text no. 2
    
3.
Walsh, F. B., 1969. Clinical Neuroophthalmo­logy, published by the Williams & Wilkins Company, 3, (3).  Back to cited text no. 3
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]



 

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