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Year : 1981  |  Volume : 29  |  Issue : 1  |  Page : 43-45

Acute myelomonocytic leukemia presenting as unilateral anterior cavernous sinus syndrome

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, A.I.I.M.S., Ansari Nagar, New Delhi-110029, India

Correspondence Address:
J S Saini
Dr. R. P. Centre for Ophthalmic Sciences, A.I.I.M.S. New Delhi-110029
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Source of Support: None, Conflict of Interest: None

PMID: 6945290

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How to cite this article:
Saini J S, Mukherjee G, Mohan M. Acute myelomonocytic leukemia presenting as unilateral anterior cavernous sinus syndrome. Indian J Ophthalmol 1981;29:43-5

How to cite this URL:
Saini J S, Mukherjee G, Mohan M. Acute myelomonocytic leukemia presenting as unilateral anterior cavernous sinus syndrome. Indian J Ophthalmol [serial online] 1981 [cited 2023 Dec 10];29:43-5. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1981/29/1/43/30992

Neurological and ocular involvement during the course of acute myelomonocytic leukemia (AMML) is not an uncommon entity[1],[2],[3],[4]. However, it is very rare for AMML to present either with neurological[5] or ophthalmic mani­festations[6]. A combination of unilateral total ophthalmoplegia with loss of vision without proptosis has not been reported in literature as presenting feature of AMML. The following case report describes a patient with this rare presenting manifestation.

  Case report Top

V.K., a 23 year old male was admitted in the hospital with the complaints of inability to move right eye for the last 15 days and loss of vision for 12 days.

Two weeks ago patient developed right sided temporal headache with drooping of right upper lid and inability to move the right eye. Thereafter he lost vision in right eye gradually over a period of 2 to 3 days. He received systemic prednisone (50 mg daily) for 6 days in another city hospital. However, this did not make any difference to either vision or eye movements.

Patient did not have vomiting or convul­sions. He did not have fever, cough, urinary or any bowel complaints.

General Examination: The patient was of average built but anaemic. On admission his pulse rate was 104/minute and B.P. 120/74mm Hg. There was evidence of bleeding from gums and petec­hial haemorrhages on the chest. Sternal tenderness was significant. Multiple firm, nontender lymph-nodes were palpable in the cervical and inguinal region. Firm, nontender and smooth edge of the enlarged liver could be felt 7 cm below the right costal margin. The spleen could be felt 2 cm below the left costal margin. In addition a nontender firm mass 4 x 4cm could be felt in the paraumbilical region. Cardiovas­cular and respiratory systems were clinically normal.

Neurological and Ophthalmic Examination Patient was alert, conscious and there was no motor or cerebeller dysfunction. Right sided III, IV and VI cranial nerves were paralyzed [Figure - 1] & [Figure - 2]. There was no perception of light in right eye. Pupil of the right eye was semi dilated and non reacting to light. Optic disc was ophthalmoscopically normal. There were no haemorrhages or nodular infiltration seen in the conjunctiva, iris, choroid or retina. There was no proptosis. Sensations of cornea, conjunctiva and upper lid on the right side were absent. Examination of left eye showed only petechial subconjunctival haemorr­hage near the limbus. Visual acuity was 6/6. Exophthalmometry gave a reading of 20mm for right eye and 19mm for left eye. Visual fields for left eye were normal.


1. Blood examination : Hb 9.2 gm% TLC 26300/mm 3 DLC; N 2 L 28 E 1 M 2 Blast cells 63 Stab cells 4

Platelet count 50,000/mm 3, ESR 30mm 1st hr.

Peripheral Smear :-Acute myelomonocytic leukemia [Figure - 3]

2. Bone-Marrow aspiration-consistent with acute myelomonocytic leukemia.

3. Radiographs of orbits, optic foramina, superior orbital fissures, PNS, skull and chest were normal

4. CAT Scan and Carotid Angiography normal

5. VDRL-Non-reactive.

Treatment & Progress

In the hospital, the patient received the following treatment :­

Inj. Cytosan 100 mg/sq.M. I.V. in a drip for 5 days, Inj. Daunomycin 100 mg/sq. M.I.V. on the 7th day, 2 units of fresh blood, Tab. Septran 1 b.d. and Tab. multivitamins 1 b.d.

However ocular movements did not improve in the right eye. Patient died following a bout of convulsions on the 8th day (presumably due to intracerebral haemorrhage).

  Discussion Top

Leukemic involvement of nervous system may account for the clinical findings of men­ingitis, encephalitis, monoplegia, hemiplegia, paraplegia, convulsions or involvement of cranial or peripheral nerves in about 25% of the patients. However a preceding or con­comitant involvement of nervous system at the time of diagnosis is very rare.[8],[9]

In our case the patient developed unilateral combined paralysis of oculomotor, trochlear, opthalmic division of trigeminal nerve, abdu­cens and optic nerve without proptosis. Topographically the obvious site of lesion is retro-orbital in the anterior part of the right cavernous sinus. Total ophthalmoplegia due to such a lesion in AMML has never been earlier reported as a presenting feature. Interestingly this patient had no clinically manifest meningeal or cerebral involvement. It is also very striking to note that the patient presented to the ophthalmic department with only unilateral total ophthalmoplegia without any other ocular manifestations including papil­loedema despite the loss of vision. This case emphasizes the need to keep in mind the etiological possibility of leukemia in patients presenting with unilateral anterior cavernous sinus syndrome (involvement of II, III, V, VI and ophthalmic division of V cranial nerve without proptosis) Lack of suspicion may delay the diagnosis and treatment in such a potentially fatal disease.

  Summary Top

A rare case of acute myelomonocytic leukemia presenting with unilateral involvement of II, III, IV, ophthalmic division of V & VI cranial nerves without associated proptosis is described. The patient had no meningeal or cerebral involvement. The diagnosis was confirmed on examination of blood and bone marrow smear examination.

  Acknowledgement Top

The authors express sincere thanks to the Hematology and Neurology Section of A.I.I.M.S., New Delhi, for their assistance in the management of this patient.

  References Top

Allen, R.A. and Straatsma, B.P., 1961, Arch. Ophthalmol. 6 : 490-508.  Back to cited text no. 1
Haghbin, M. and Zuelzer, W.W., 1965, J. Pediat. 67 : 23  Back to cited text no. 2
Moore, E.W., Thomas, L.B. Shaw, R.K., and Freireich, E.J., 1960, A.M.A. Arch. Int. Med, 105 451-468.  Back to cited text no. 3
Schwab, R.S. and Weiss, 1935, Amer. J. Med. Sci. 185 : 766.  Back to cited text no. 4
Hayhoe, F.G.J., 1960, Leukemia ; Research & Clinical practice, 1st Edn. p. 217-219, J and A Churchill Ltd., London  Back to cited text no. 5
Cavdar, A.O.; Arcasoy, A., Gozdasogluo, S., and Demirag, B., 1971, Lancet, 1 : 680.  Back to cited text no. 6
Gunz, F. and Bike, A.G., 1974, Leukemia, 3rd Edn. pp 182-283 Grune and Stratton, New York.  Back to cited text no. 7
Hurwitz, B.S., Sutherland, J.C. and Walker, D.M., 1970, Neurology 20 ; 771-775  Back to cited text no. 8
Henderson, E.S. 1977, Hematology. 2nd Edn. pp 830-841. Edited by Williams, W.J.; Beutler, E; Erslev, A.J. & Rundles, R.W. McGraw-Hill Book Company, New York.  Back to cited text no. 9


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


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