|Year : 1969 | Volume
| Issue : 4 | Page : 166-170
Leontiasis ossea- a case report
Department of Ophthalmology, N. R. Sircar Medical College, Calcutta, India
|Date of Web Publication||10-Jan-2008|
B K Chowdhuri
Department of Ophthalmology, N. R. Sircar Medical College, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chowdhuri B K. Leontiasis ossea- a case report. Indian J Ophthalmol 1969;17:166-70
| Introduction|| |
The term leontiasis ossea was first coined by Virchow  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|| |
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.
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.
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.
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|| |
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  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  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  ).
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  ).
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  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  ). 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|| |
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|| |
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|| |
BRITISH AUTHORS--A text Book of X-ray diagnosis Page, 457. Vol. I, III Edition Lewis, London.
CECIL LOEB - Text Book of Mcdicine, Page, 1511-12 Ilth Edition, W.B. sanders Company. Philladelphia & London, October, 1963.
DUKE-ELDER - Text Book of Ophthalmology. Page-5519. Vol. V. Henry Kimpton, London 1952.
HARRISON, J. M. -- A Text Book of Roentgenology. Page-748. London, Bailliere Tinddal & Cox, 1936.
KNAGGS. - As quoted by Duke Elder. Text Book of Ophthalmology Vol. V. Henry Kimpton, London, 1952.
VIRCHOW - Quoted from Text Book of Ophthalmology by Duke Elder Page -- 5519. Henry Kimpton, London. 1952.
[Figure - 1], [Figure - 2], [Figure - 3]