The Egyptian Journal of Haematology

: 2015  |  Volume : 40  |  Issue : 1  |  Page : 1--2

Acute retrobulbar haemorrhage in haemophilia B

Jothi Saravanan, Alangudi Achari Rajendraprasad, Begum Zaibunissa, Sundaram Priyadharshni 
 Department of Ophthalmology, Coimbatore Medical College Hospital, Coimbatore, Tamil Nadu, India

Correspondence Address:
Jothi Saravanan
Department of Ophthalmology, Coimbatore Medical College Hospital, Coimbatore - 641 036, Tamil Nadu


Acute retrobulbar haemorrhage is a very rare complication of haemophilia. We present a 16-year-old boy with haemophilia B with spontaneous retrobulbar haemorrhage of the left orbit. He showed a favourable response to treatment with factor IX replacement with no recurrence until 8 months.

How to cite this article:
Saravanan J, Rajendraprasad AA, Zaibunissa B, Priyadharshni S. Acute retrobulbar haemorrhage in haemophilia B.Egypt J Haematol 2015;40:1-2

How to cite this URL:
Saravanan J, Rajendraprasad AA, Zaibunissa B, Priyadharshni S. Acute retrobulbar haemorrhage in haemophilia B. Egypt J Haematol [serial online] 2015 [cited 2022 May 22 ];40:1-2
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Full Text


Haemophilia A and B are disorders of blood coagulation caused by defective synthesis or synthesis of dysfunctional factors VIII and IX, respectively. The incidence of haemophilia is one in 25 000 male births [1] , out of which 14% are due to haemophilia B [1] . The hallmark of haemophilia is haemorrhage following minor trauma. Spontaneous haemorrhage is seen in patients with less than 1% of normal factors VIII or IX activity [1] .

Haemorrhage commonly occurs in the musculoskeletal system followed by gastrointestinal, genitourinary and central nervous system bleeding [1] . Ocular complications of haemophilia are very rare [2],[3] .

 Case report

A 16-year-old boy with recurrent bleeding episodes since 1½ years of age was diagnosed as severe haemophilia B. He presented with a painful proptosis of the left eye with periorbital ecchymosis. Ocular examination revealed axial proptosis (23 mm Hertel in left eye), subconjunctival haemorrhage, restricted extra ocular movements (EOM) and visual acuity of 6/24. Pupillary reaction was normal. Fundus examination was normal. Intraocular pressure was 14 mmHg. There was no history of ocular trauma. Computed tomographic scan and ultrasonography of the orbit were suggestive of a retrobulbar haemorrhage. These characteristic findings led to the diagnosis of acute retrobulbar haemorrhage of the left orbit.

As per the Haemato-oncologist advice, we administered 2000 IU of factor IX intravenously once daily for 5 days, along with topical antibiotics and lubricants and oral acetazolamide 250 mg twice daily. Proptosis started decreasing gradually from the second day of treatment and completely disappeared in 2 weeks with restoration of EOM. At the end of treatment, left eye exophthalmometry was 18 mm Hertels, intraocular pressure was 12 mmHg and the best-corrected visual acuity was 6/6, N 6 . Radioimaging after recovery was not performed because of financial constraints on the patient's part and to avoid unnecessary radiation exposure.

The patient has been under follow-up for 8 months following treatment. No recurrence was observed.


Haemophilia is a less common cause of spontaneous retrobulbar haemorrhage [4] , more common cause being orbital vascular anomalies [4],[5],[6] . Spontaneous retrobulbar haemorrhage due to uncontrolled hypertension [7],[8] and anticoagulant therapy [9] has also been reported.

Retrobulbar haemorrhage following trauma in haemophilia has been reported in the literature.

Zolog et al. [10] described a case of acute unilateral retrobulbar haemorrhage in an 11-year-old boy with mild haemophilia A following a local minor trauma. Conservative management led to complete remission of the ocular phenomena in 6 weeks except for the decrease in visual acuity caused by the optic nerve involvement due to mechanical compression.

White and Mundis [11] described a case of orbital haematoma after retrobulbar anaesthesia in a patient with haemophilia A, haematoma that occurred 12 h after cataract surgery; the case ended with loss of vision at the involved eye, despite the ophthalmological and haematological treatment provided.

Other ocular complications of haemophilia are subconjunctival haemorrhage, hyphaema and palpebral haematoma [12],[13],[14],[15] .


The characteristic feature of our case is its rarity that the retrobulbar haemorrhage was spontaneous with no history of trauma, and there was a very good response to conservative management with no loss of vision. Thus, we conclude that conservative management can be a safe and effective mode of treatment of acute retrobulbar haemorrhage in haemophilia.


Conflicts of interest

There are no conflicts of interest.


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