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   Table of Contents      
ARTICLE
Year : 1969  |  Volume : 17  |  Issue : 4  |  Page : 166-170

Leontiasis ossea- a case report


Department of Ophthalmology, N. R. Sircar Medical College, Calcutta, India

Date of Web Publication10-Jan-2008

Correspondence Address:
B K Chowdhuri
Department of Ophthalmology, N. R. Sircar Medical College, Calcutta
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Chowdhuri B K. Leontiasis ossea- a case report. Indian J Ophthalmol 1969;17:166-70

How to cite this URL:
Chowdhuri B K. Leontiasis ossea- a case report. Indian J Ophthalmol [serial online] 1969 [cited 2020 Nov 23];17:166-70. Available from: https://www.ijo.in/text.asp?1969/17/4/166/38539


  Introduction Top


The term leontiasis ossea was first coined by Virchow [6] in 1896 to describe the leonine appearance of the facial deformity in this rare condition of un­known etiology. Subsequently, many other conditions deforming the bones of the skeleton have been included un­der the same general descriptive term.


  Case Report Top


P.K.M., aged 27 years, Hindu male student, visited the ophthalmic out­patients' department of the N. R. Sircar Medical College Hospital on 1-11-67 with the following complaints :­

1. Swelling of the left frontal re­gion of the head and of the left side of the face since 16 years.

2. Gradual loss of vision of the left eye following headache and fever about 3 years ago and loss of vision in the right eye following headache and fever about 2 years ago. Ultimately both eyes became totally blind.

3. Gradual forward bulging of the right eye ball for the last 1½ months.

4. Bleeding and difficulty of breath­ing through the left nostril for 1½ months.

5. Fever and mild pain in the right eye for the last 2-3 days.

Family History:- His parents and their three daughters were alive and healthy, having no ocular complaint or swelling on any part of the body.

Personal History:- He was quite healthy with normal habits before the onset of ocular symptoms. He had suffered from typhoid fever about 16 years ago and was once admitted in this hospital and was operated on the right hip joint by an orthopedic sur­geon.

General Examination : - The pa­tient was undernourished with average height (5' 4"). The head and face were much broader than the average. The left half of the face was more pro­minent than the right half on account of swelling of the zygomatic and max­illary region [Figure - 1]. There was a bony swelling on the left frontal region which according to him was present since birth. Another bony swelling was present on the lateral aspect of the left humerus, giving it a curved shape which had been developed following a fall. [Figure - 1]. The patient was anaemic.

Central Nervous System - General intelligence was good and he was men­tally sound. Power, tone, nutrition and coordination of muscles were with­in normal limits. No abnormal move­ments or jerks were detected.

Cranial Nerves

I - Olfactory function was slightly impaired.

II -There was no light perception in both eyes because of optic atrophy.

III, IV & VI-These nerves were affected and resulted in restriction of ocular movements and dilatation of the pupils which were insensitive to light.

VIII -The patient was deaf.

V, VII, IX - XII- These were nor­mal.

Cardio-vascular respiratory and uro­genital systems were quite normal.

Alimentary system The liver was just palpable and slightly tender.

Local Examination

Both the eyeballs were proptosed with widening of the palpebral fis­sures. The right eye-ball was pushed down and out and the left deviated outwards only. [Figure - 1]. There was a mild degree of lagophthalmos on the right side. The lids were swollen, with prominent veins in the upper lids. The inner halves of the lids were reddened.

Over the right lacrimal area there was a bulging which was tender and which was extending over the nasal halves of the superior and inferior or­bital margins. The consistency of the swelling was hard but it was soft in some places. The swelling was tender.

The conjunctivae on both sides were chemosed.

The cornea on the right side showed exposure keratitis and mild circumcorneal injection. On the left side it was normal.

Both the pupils were dilated and non-reacting to light and accommoda­tion.

The tension was normal on both the sides.

Ocular movements were much res­tricted in all the directions of gaze.

The media were clear.

The fundi oculi, presented a picture of primary type of optic atrophy on both the sides. No other abnorma­lity could be detected in both the fundi.

Investigations

Skull-measurements: The measure­ments of the skull denoted it to be one of brachycephaly with a cephalic in­dex of 82.5.

Blood-cytology : It gave a total W.B.C. count of 8500/ cmm, with a differential count of polym. 68%, lymphocytes 30%, eosinophils 2%, haemoglobin was 50%.

Blood chemistry : which covered es­timation of prothrombin time, fasting blood-sugar level, blood-urea, total proteins, non-protein nitrogen, serum calcium, cholestrol and alkaline phosphatase were within normal limits.

E.S.R. was high 68 mm 1st hour.

Urine Calcium: was 22.0/mgm/100 cc.

V.D.R.L.
was negative.

Electro-cardiogram : The conclu­sion from the examination was one of left ventricular hypertrophy.

X-ray of skull: A.P. and Lateral views showed diffuse hyperostosis of bone with loss of differentiation be­tween outer and inner tables [Figure - 2]. This hyperostosis of bones extended into the facial bones.

Base of skull showed also irregular formation of bones and the anatomy of the skull was very much distorted.

Chest including scapular region showed diffuse ground glass appear­ance of bone matrix and there was marked expansion of left clavicle due to cyst like cavities. The same cystic change was also noticed in the left scapular region resulting in polyostotic fibrous dysplasia.

Both hip bones showed multiple cystic changes. [Figure - 3]. Multiple cys­tic expansions were also noticed in the neck and upper portion of both fe­murs.

Nose and Throat: Report: Sepsis was detected clinically in the nose and throat. Blood stained discharge through the left nostril was noticed and foul smell was complained of.


  Discussion Top


Virchow in 1896 had described the term 'Leontiasis ossea' as a rare condition occurring at any age, being a very chronic disease of unknown etio­logy, characterised by a slowly spread­ing osteitis usually commencing on the floor of the anterior cranial fossa and gradually spreading to the facial bones and the walls of the sinuses. British Authors [1] on the other hand, with­out using, the term in a specific sense have included a number of conditions like osteitis fibrosa, osteitis deformans and chronic periostitis under the ter­minology - leontiasis ossea.

Knaggs [5] as quoted by Duke Elder has divided the manifestations of leon­tiasis ossea into two types - a creep­ing periostitis and a diffuse osteitis. The periostitic type usually affects the bones of the lower jaw and may take its origin from an infected tooth. Some­times it may originate from suppura­tion of the lacrimal sac. The disease spreads over the bones of the face and cranial vault, producing ivory hard exuberant masses which are densely opaque to X-rays. In the osteitic form there may be general affections of the skull bones which may become several inches thick so that the head becomes enlarged. The usual involvement is of the frontal bone causing a downward displacement of the orbit and propto­sis. In both the types, the optic nerves may undergo atrophy due to pressure of the bony overgrowths (Duke Elder [3] ).

The reported case: in the absence of any infection in the neighbourhood of the jaw belongs to the osteitic group of Knaggs.

Microscopically, the majority of re­ported biopsies show that this disease process is characterised by fibrosis of marrow with proliferation of osteo­blasts and osteoclasts and marked overgrowth of normal appearing bone with wide vascular channels (Cecil­Loeb [2] ).

Occasionally leontiasis ossea may develop polyostotic fibrous dysplasia characterised by sclerosis and malfor­mations of different bones and cyst like lesions in the bones of the extre­mities. In fact Cecil-Loeb [2] has consi­dered leontiasis ossea as a manifesta­tion of polyostotic dysplasia.

The commonest condition which can simulate leontiasis ossea is Paget's dis­ease. Snapper considers that most cases of Leontiasis ossea are nothing but manifestations of Paget's disease. In Paget's disease however, there is usually thickening of the inner table of the skull bones with calcification of the diploe but no exuberant localised growth of bone. Moreover, Paget's disease is never generalised and y the peripheral skeleton is usually unaffect­ed. Bio-chemical tests are also helpful in differentiating Paget's disease from leontiasis ossea. In Paget's disease serum alkaline phosphatase is increas­ed which may be 20-30 times the nor­mal with normal serum calcium, al­though urinary calcium excretion is increased. But in leontiasis ossea, serum alkaline phosphatase, serum calcium and calcium excretion in the urine are all normal.

Multiple osteomas of the skull may sometimes be confused with leontiasis ossea, but osteoma is definitely circum­scribed and connected to the base by a definite pedicle better seen by X-ray (Harrison [4] ). Besides these, epileptic fits and cerebrospinal rhinorrhoea may be present.

Recklinghausen's bone disease may be differentiated by the presence of hypercalcaemia, hypophosphatemia, hypercalcuria and nephrosclerosis and sometimes X-rays show giant cell tumour (osteoclastoma).

In the reported case, radiological and clinical examinations have estab­lished the bony overgrowth on the bones of the skull, face and orbit pro­ducing marked deformity of skull, face and orbits along with proptosis and optic atrophy. In addition, the radio­logical examination of the rest of the skeleton has shown cystic expansion of left clavicle and left scapula along with cystic changes in both hip bones and the upper ends of both femurs and left humerus. This proved that there is as­sociated polyostotic fibrous dysplasia. The biochemical tests are normal which have differentiated it from Pa­get's disease. The difficulty to breathe through the left nostril and the at­tack of acute dacryocystitis can be ex­plained by pressure exerted by the bony growth in the nasal passage and the nasolacrimal duct.

As reported by Snapper unilateral involvement of the skull and marked facial asymmetry as seen in this case may be present in about 1/3 of the leontiasis ossea cases.


  Summary Top


A rare case of Leontiasis Ossea is reported with typical signs in the form of hyperostosis of the bones of face and cranium with the loss of differ­entiation between outer and inner tables, proptosis, optic atrophy with polyostotic changes in the bones like humerus, clavicle, scapula, hip-bones and femur etc. The serum alkaline phosphatase was normal without any hypercalcemia of hypercalcinuria.


  Acknowledgement Top


I am indebted to Professor B. N. Chatterjee, F.R.C.S. (Edin.), F.R.C.S. (Lond.), D.O. (Lond.), Department of Ophthalmology, Nilratan Sircar Medi­cal College for his constant guidance. My thanks are also due to Dr. S. Basu, Professor of Radiology of this hospital for taking repeated X-ray plates of the patient.

 
  References Top

1.
BRITISH AUTHORS--A text Book of X-ray diagnosis Page, 457. Vol. I, III Edition Lewis, London.  Back to cited text no. 1
    
2.
CECIL LOEB - Text Book of Mc­dicine, Page, 1511-12 Ilth Edition, W.B. sanders Company. Philladelphia & London, October, 1963.  Back to cited text no. 2
    
3.
DUKE-ELDER - Text Book of Ophthalmology. Page-5519. Vol. V. Henry Kimpton, London 1952.  Back to cited text no. 3
    
4.
HARRISON, J. M. -- A Text Book of Roentgenology. Page-748. London, Bailliere Tinddal & Cox, 1936.  Back to cited text no. 4
    
5.
KNAGGS. - As quoted by Duke Elder. Text Book of Ophthalmology­ Vol. V. Henry Kimpton, London, 1952.  Back to cited text no. 5
    
6.
VIRCHOW - Quoted from Text Book of Ophthalmology by Duke Elder Page -- 5519. Henry Kimpton, Lon­don. 1952.  Back to cited text no. 6
    


    Figures

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



 

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  In this article
Introduction
Case Report
Discussion
Summary
Acknowledgement
References
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