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Intraocular hemorrhage in a patient with hemophilia
Abstract
A 24-year-old man with severe classic hemophilia (factor VIII level less than 1%) had blunt trauma to his left eye that produced total hyphema with elevated intraocular pressure. Despite attaining sufficient hemostasis with factor VIII replacement, the anterior chamber remained completely filled with blood, which led to corneal blood-staining. Total hyphema was removed under management of replacement treatment. This consisted of sufficient cryoprecipitates to increase the calculated factor VIII level to 100%. For the 7-day post-operative period, the factor VIII level was above 15%. His surgical and post-operative course was uneventful. Blood-staining of the cornea was gradually reduced. Five months after surgery, he spontaneously had moderate hyphema with elevation of intraocular pressure. The blood in the anterior chamber was absorbed with replacement therapy. Though the visual acuity has improved, the fundus cannot be observed due to the vitreous opacity.
... [26,27] H e m a t o l o g i c a l d i s o r d e r s s u c h a s i d i o p a t h i c thrombocytopenic purpura, hypo-fibrinogenemia, disseminated intra-vascular coagulation, von Willebrands syndrome, and hemophilia may present as VH, which may be bilateral and massive in some cases. [28][29][30][31][32] Characteristically, the hematological disorders cause intra-retinal hemorrhages and vascular occlusion but may rarely present as VH. Vitreous veils in the setting of juvenile X-linked retinoschisis is also an important cause of VH. ...
Quick Response Code: Vitreous hemorrhage is associated with a myriad of conditions such as proliferative diabetic retinopathy, proliferative retinopathy following vascular occlusion and vasculitis, trauma, retinal breaks, and posterior vitreous detachment without retinal break. Multiple pathological mechanisms are associated with development of vitreous hemorrhage such as disruption of abnormal vessels, normal vessels, and extension of blood from an adjacent source. The diagnosis of vitreous hemorrhage requires a thorough history taking and clinical examination including investigations such as ultra-sonography, which help decide the appropriate time for intervention. The prognosis of vitreous hemorrhage depends on the underlying cause. Treatment options include observation, laser photo-coagulation, cryotherapy, intravitreal injections of anti-vascular endothelial growth factor, and surgery. Pars plana vitrectomy remains the cornerstone of management. Complications of vitreous hemorrhage include glaucoma (ghost cell glaucoma, hemosiderotic glaucoma), proliferative vitreoretinopathy, and hemosiderosis bulbi.
... On the other hand, ocular bleeding was also reported in patients who are known to have hemophilia, and such cases tend to be traumatic with good visual outcome as they typically seek early medical attention. [7][8][9][10][11] Wang et al. 7 presented a case of spontaneous bilateral optic disk hemorrhages in a known 13-year-old hemophilia A patient with good visual outcome (BCVA of 6/6) due to early management which prevented the extension of bleeding into the adjacent structures (ex: macula). All these cases collectively shed light on the significance of early recognition and management to prevent sight threatening, and more essentially, life-threatening bleeding events. ...
Introduction:
Suprachoroidal hemorrhage is an unexpected complication after penetrating keratoplasty during childhood. We report the case of delayed suprachoroidal hemorrhage after penetrating keratoplasty in a neonate as the first manifestation of hemophilia.
Case report:
A 4-day-old neonate was referred to our hospital because of bilateral corneal opacities present since birth. A diagnosis of bilateral Peter's anomaly type 2 with right eye corneal perforation was made after examination. Appropriate treatment was commenced, and later, right eye therapeutic penetrating keratoplasty combined with lensectomy, and anterior vitrectomy was performed. This was complicated postoperatively with suprachoroidal hemorrhage and retinal detachment. Conservative management was advised due to poor visual prognosis. Three months later, the patient was found to have high intraocular pressure in the left eye and glaucoma surgery was recommended. Proper hematological investigations before glaucoma surgery in the left eye revealed a low level of factor VIII with normal levels of factor IX and von Willebrand's factor antigen. Therefore, a diagnosis of hemophilia type A was established.
Conclusion:
This case serves as a reminder that the occurrence of suprachoroidal hemorrhage, especially in the absence of other predisposing risk factors, should warrant detailed systemic assessment to exclude underlying bleeding disorders.
... For any of these clinical ophthalmologic conditions, including hyphema, vitreous hemorrhage or even hemorrhagic glaucoma (whether diagnosed or suspected based on opthalmoscopic exam), immediate infusion to 100% activity (1 IU/mL of plasma) is essential followed by emergency assessment by an ophthalmologist [37][38][39]. Whether or not surgery is recommended, hospitalization is indicated until the clinical situation is deemed stable enough for outpatient management by both the ophthalmologist and the hematologist [40]. ...
Hematologic diseases often share clinical features within the choroid and retina because of the limited number of cell line or lines affected. No one chorioretinal change is specific for a particular hematologic anomaly; rather, it is the context and pattern that help guide clinical diagnoses. Retinal and choroidal manifestations of hematologic illness may be critical not only in identifying new or recurrent illness but also in ultimately reducing patient morbidity and mortality. Therefore, it is paramount that an open and regular dialogue be maintained between a patients hematologist and ophthalmologist.
Central nervous system bleedingIntracranial hemorrhageSpinal hematomaClotting factor replacement: recommendations for the treatment of central nervous system bleedsNon-central nervous system-emergent eventsBleeding from organ rupture or hematoma of an abdominal viscusSymptoms of nerve compression or compartment syndromeOphthalmologic emergenciesRare clinical emergenciesRupture of a pseudotumorConclusion
References
In the hemophilias or other congenital clotting deficiencies, either acute or chronic hemorrhage in vulnerable anatomic locations can produce a hematologic emergency. In virtually every instance, replacement of the relevant missing clotting factor or cells (platelets) should follow immediately critical initial cardiovascular or respiratory resuscitation measures. A history of recent trauma should increase the hematologists' index of suspicion but the absence of such a history should not eliminate the possibility of injury-induced bleeding in these populations. Surgical management may need to follow resuscitation and factor replacement if organ compromise cannot be prevented by these initial therapeutic interventions. In the latter circumstances, clotting factor replacement strategies will likely need to be continued until homeostasis is achieved and requisite healing is well underway. Occult hemorrhage that progresses may produce emergent circumstances particularly in the central nervous system and nearby to the airway. Therefore, mainstays of emergent management for inherited bleeding disorders include a cautious expectancy that the status quo may not assure adequate management and vigilance that bleeding may return insidiously.
Hyphema (blood in the anterior chamber) can occur after blunt or lacerating trauma, after intraocular surgery, spontaneously (e.g., in conditions such as rubeosis iridis, juvenile xanthogranuloma, iris melanoma, myotonic dystrophy, keratouveitis (e.g., herpes zoster), leukemia, hemophilia, von Willebrand disease, and in association with the use of substances that alter platelet or thrombin function (e.g., ethanol, aspirin, warfarin). The purpose of this review is to consider the management of hyphemas that occur after closed globe trauma. Complications of traumatic hyphema include increased intraocular pressure, peripheral anterior synechiae, optic atrophy, corneal bloodstaining, secondary hemorrhage, and accommodative impairment. The reported incidence of secondary anterior chamber hemorrhage, that is, rebleeding, in the setting of traumatic hyphema ranges from 0% to 38%. The risk of secondary hemorrhage may be higher in African-Americans than in whites. Secondary hemorrhage is generally thought to convey a worse visual prognosis, although the outcome may depend more directly on the size of the hyphema and the severity of associated ocular injuries. Some issues involved in managing a patient with hyphema are: use of various medications (e.g., cycloplegics, systemic or topical steroids, antifibrinolytic agents, analgesics, and antiglaucoma medications); the patient's activity level; use of a patch and shield; outpatient vs. inpatient management; and medical vs. surgical management. Special considerations obtain in managing children, patients with hemoglobin S, and patients with hemophilia. It is important to identify and treat associated ocular injuries, which often accompany traumatic hyphema. We consider each of these management issues and refer to the pertinent literature in formulating the following recommendations. We advise routine use of topical cycloplegics and corticosteroids, systemic antifibrinolytic agents or corticosteroids, and a rigid shield. We recommend activity restriction (quiet ambulation) and interdiction of non-steroidal anti-inflammatory agents. If there is no concern regarding compliance (with medication use or activity restrictions), follow-up, or increased risk for complications (e.g., history of sickle cell disease, hemophilia), outpatient management can be offered. Indications for surgical intervention include the presence of corneal blood staining or dangerously increased intraocular pressure despite maximum tolerated medical therapy, among others.
To describe the clinical course of a 52-year-old white man with previously undiagnosed hemophilia who suffered blunt trauma to the right eye with consequent delayed, spontaneous suprachoroidal hemorrhage.
Observational case report.
The patient's clinical course from the time of trauma to postenucleation is discussed and correlated with B-scan ultrasonography and histopathology.
The patient developed recurrent hyphemas, delayed choroidal hemorrhage with detachment and eventually loss of the eye. Hematologic evaluation established a diagnosis of mild factor VIII deficiency.
Despite mild hemophilia, this patient had not experienced any clinically important morbidity until his eye injury. Ophthalmologists should be aware that bleeding disorders such as hemophilia predispose patients to spontaneous intraocular hemorrhage.
We report a rare case of idiopathic acquired factor VIII inhibitor in an 80-year old Chinese man presented as sudden onset of monocular blindness because of macular haemorrhage. This was complicated by painful glaucoma that did not respond to medical treatment. The patient died of cerebral haemorrhage shortly afterwards. Most cases of reported intraocular bleeding in acquired haemophiliacs are iatrogenic because of intraocular operations in undiagnosed cases, and spontaneous intraocular haemorrhage has never been reported. The literature reports of intraocular bleeding in hereditary and acquired haemophilia cases are summarized.
Vitreous hemorrhage is common, with varied clinical manifestations and causes. The most common causes include proliferative diabetic retinopathy, vitreous detachment with or without retinal breaks, and trauma. Less common causes include vascular occlusive disease, retinal arterial macroaneurysm, hemoglobinopathies, age-related macular degeneration, intraocular tumors, and others. The natural history depends on the underlying cause, and is generally more favorable in eyes without underlying disease. Treatment is directed at the underlying cause, such as laser photo-coagulation for proliferative diabetic retinopathy or for retinal breaks. Occasionally, hemorrhage does not resolve spontaneously and vitrectomy surgery is necessary and beneficial. New strategies for the treatment of vitreous hemorrhage, such as pharmacologic vitreous liquefaction, may be important in the future.
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