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Acquired Glanzmann’s thrombasthenia causing prolonged bleeding following phacoemulsification
  1. S Dinakaran1,
  2. M P Edwards1,
  3. K K Hampton2
  1. 1Department of Ophthalmology, A-Floor, Royal Hallamshire Hospital, Sheffield, UK
  2. 2Department of Haematology, Royal Hallamshire Hospital, Sheffield, UK
  1. Correspondence to: S Dinakaran, Department of Ophthalmology, A-Floor, Royal Hallamshire Hospital, Sheffield, UK; sdinakaran{at}yahoo.com

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Phacoemulsification under topical anaesthesia using clear corneal incision is not a challenging procedure for the haemostatic system. In patients with known bleeding diathesis, this may be the procedure of choice to remove cataract. We report a patient who bled continuously for 36 hours following phacoemulsification under topical anaesthesia through a clear corneal incision. This was managed by using a topical haemostatic agent that has not been used in ophthalmic surgery before. Extensive haematological evaluation revealed the underlying cause to be an acquired form of Glanzmann’s thrombasthenia, a very rare condition.1,2

Case report

A 79 year old woman underwent left phacoemulsification with intraocular lens implantation under topical anaesthesia through a clear corneal temporal incision. The procedure was uneventful but she was seen to bleed from the operated eye in the recovery room. The eye was patched but the bleeding continued soaking the pads. When re-examined 2 hours later, as there was continuous bleeding, the eye was patched with gentle pressure. Examination the next day showed that the bleeding was persistent. Pressure bandage was reapplied. Examination in the operating theatre confirmed the conjunctival origin of the bleeding from the site where the left handed surgeon held the conjunctiva during surgery. Cauterisation and an attempt to suture the conjunctiva were unsuccessful. It was decided that the safest option was to use a small piece of oxidised regenerated cellulose (Surgicel, Ethicon) on the bleeding site and patch the eye.

The piece of Surgicel with clotted blood that was lying loose on the conjunctiva was removed at review 24 hours later. The conjunctival site had stopped bleeding with evidence of altered blood on the surface where Surgicel had been applied (Fig 1A). At her last review 8 weeks later, she was found to have a corrected visual acuity of 6/18 due to pre-existent macular changes secondary to retinal detachment that was reattached in 1976. The conjunctiva had healed well (Fig 1B). The patient had previously undergone an uneventful phacoemulsification and intraocular lens implantation in her right eye under sub-Tenon’s anaesthesia.

Figure 1

(A) Conjunctival site immediately after removal of Surgicel. (B) Healed conjunctival bleeding site.

The patient’s recent medical history was significant for recurrent admissions elsewhere for investigation of severe anaemia following gastrointestinal bleeding. Platelet count and clotting screen had been normal. Angiodysplasia of stomach and duodenum were treated with laser and angiodysplasia of colon was treated by hemicolectomy. Three episodes of epistaxis and an episode of vaginal bleeding were managed conservatively. She had received 60 units of blood transfusion over a period of 1 year. Interestingly, she had appendicectomy and multiple dental extractions elsewhere many years previously without any significant bleeding. She has not been on any antiplatelet agents or anticoagulants. There was no family history of bleeding disorders.

A defect in the platelet function was suspected, as her coagulation screen including the platelet count was normal. Platelet aggregation tests showed no aggregation against any agonists other than ristocetin, which is dependent on platelet glycoprotein Ib. The platelets showed normal surface levels of glycoprotein antigens IIbIIIa and Ib. The patient’s serum showed presence of inhibitory antibody against glycoprotein IIbIIIa. This led to a diagnosis of acquired Glanzmann’s syndrome, an extremely rare condition of autoimmune thrombasthenia. No underlying malignant, autoimmune, or lymphoproliferative disorder had been identified as a cause for this patient’s acquired Glanzmann’s thrombasthenia.

Comment

The patient described had uncontrollable bleeding for 36 hours following a procedure, which is generally considered safe in patients with a bleeding disorder. She developed bleeding from the conjunctival site where the surgeon grasped the conjunctiva during certain stages of the procedure. One would usually not expect any significant bleeding from this site; however, in a patient with compromised haemostasis the bleeding may be prolonged. Although the bleeding was no more than a gentle ooze at any point in time it was persistent enough for 36 hours before the topical haemostatic material Surgicel had been put to use. The consequences of an intraocular bleed may have seriously threatened her sight.3

We are not aware of any reports of the use of Surgicel in ophthalmic surgery. All reports of its use are in other fields of surgery.4,5 This material is supposed to swell up with blood and form a gelatinous mass that aids in the formation of clot. It acts as a haemostatic adjunct. The exact mode of its action in this patient with antiplatelet antibodies is unclear. Our experience shows that oxidised regenerated cellulose (Surgicel) may have a role in ophthalmic surgery especially in lacrimal and orbital surgery, when faced with bleeding that is difficult to stop. Various cautionary tales associated with use of Surgicel have been reported.6–8

Our report suggests that in the presence of a severe bleeding disorder, clear corneal phacoemulsification under topical anaesthesia may not be totally safe. When performing such a procedure in a patient with known bleeding disorder it may be safe to take all the necessary precautions in consultation with a haematologist to avoid a serious bleed that may be sight and life threatening. There may be a role for haemostatic agents like Surgicel.

References

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