Imaging
One should consider CT of head in patients with signs or symptoms of central neurologic dysfunction to exclude intracranial hemorrhage.
DIFFERENTIAL DIAGNOSIS
- Bleeding and clotting disorders:
- Platelet disorders (qualitative and quantitative)
- Hereditary factor deficiencies
- Acquired disorders (vitamin K deficiency, liver disease)
- Disseminated intravascular coagulation
TREATMENT
PRE HOSPITAL
IV fluid resuscitation with hemorrhage
INITIAL STABILIZATION/THERAPY
- Rehydrate with 0.9% NS IV fluid.
- Bleeding or end-organ ischemia may not be controlled by any treatment except plasmapheresis.
- In patients with anemia and a leukemic picture, avoid blood transfusion until plasmapheresis is performed to avoid exacerbation of HVS.
ED TREATMENT/PROCEDURES
- Hydration, supportive care, and early hematologist consultation are initial ED management.
- Phlebotomy or emergent plasma exchange: This temporizing measure can be performed in a patient with HVS and severe neurologic findings like coma or seizures:
- Easily performed in the ED and is useful in acute severe cases if plasmapheresis not readily available
- Simply draw off (100–200 mL) of whole blood and replace volume with isotonic saline.
- Should be performed in consultation with hematologist when possible.
- Treatment of choice in patients with polycythemia vera.
- Plasmapheresis/leukapheresis:
- In stable patients: 40 mL/kg of body weight
- In critical patients: 60 mL/kg of body weight
- Side effects include hypocalcemia with use of a citrate-containing anticoagulant and dysrhythmia (rare).
- Many patients require more than 1 plasmapheresis.
- Definitive treatment for HVS. Should be performed in consultation with plasmapheresis/hematology team.
- Leukapheresis is reserved as the initial treatment in patients with hyperleukocytosis (usually WBC >100,000)
- ED physician can help in urgent situations by establishing or facilitating the establishment of large-bore central dialysis catheter, caution should be taken to avoid bleeding complications of this procedure
FOLLOW-UP
DISPOSITION
Admission Criteria
- Patients with hyperviscosity and significant symptoms or any evidence of end-organ ischemia or hemorrhage should be admitted for treatment of the underlying hematologic disorder.
- ICU admission for the following:
- Hemorrhage
- Altered mental status
- Acute MI
Discharge Criteria
Discharge after definitive treatment of the underlying disorder.
Issues for Referral
All patients with HVS should be referred to hematologist.
PEARLS AND PITFALLS
- Avoid diuretics in patients with HVS because they can increase blood viscosity.
- The classic triad of symptoms of HVS includes visual disturbances, bleeding, and neurologic manifestations.
ADDITIONAL READING
- Adams BD, Baker R, Lopez JA, et al. Myeloproliferative disorders and the hyperviscosity syndrome.
Emerg Med Clin North Am
. 2009;27:459–476.
- Blum W, Porcu P. Therapeutic apheresis in hyperleukocytosis and hyperviscosity syndrome.
Semin Thromb Hemost.
2007;33(4):350--354.
- Kwaan HC. Role of plasma proteins in whole blood viscosity: A brief clinical review.
Clin Hemorheol Microcirc.
2010;44(3):167–176.
- Somer T, Meiselman HJ. Disorders of blood viscosity.
Ann Med
. 1993;25(1):31–39.
See Also (Topic, Algorithm, Electronic Media Element)
Disseminated Intravascular Coagulation
CODES
ICD9
- 273.3 Macroglobulinemia
- 289.0 Polycythemia, secondary
ICD10
- C88.0 Waldenstrom macroglobulinemia
- D75.1 Secondary polycythemia
HYPHEMA
Jamil D. Bayram
•
Sami H. Uwaydat
BASICS
DESCRIPTION
- Blood in anterior chamber (AC) of the eye (between iris and cornea).
- Hyphema: Grossly visible layering of blood.
- Microhyphema: Suspended RBCs visible by slit-lamp only.
- Genetics:
- Genetic predisposition is related to hereditary blood dyscrasias (see below).
ETIOLOGY
- Blunt trauma: Most common (70–80%).
- Anteroposterior compression of the globe with simultaneous equatorial globe expansion causing rupture of iris stromal/ciliary body vessels
- Penetrating trauma: Direct injury to stromal vessels or sudden ocular decompression.
- Spontaneous: Less common, lower incidence of complications:
- Tumors:
- Melanoma
- Retinoblastoma
- Xanthogranuloma
- Metastatic tumors
- Blood dyscrasias:
- Hemophilia
- Leukemia
- Thrombocytopenia
- Von Willebrand disease
- Blood thinners: Aspirin, Coumadin, heparin
- Neovascularization of iris: In proliferative diabetic retinopathy, retinal vein occlusion, carotid stenosis.
- Postsurgical: Cataract extraction, trabeculectomy, pars plana vitrectomy.
ALERT
In children with no history of trauma, suspect child abuse.
DIAGNOSIS
SIGNS AND SYMPTOMS
- Photophobia
- Blurring of vision
- Decreased visual acuity
- Ocular pain
- Nausea/vomiting
History
- Previous visual acuity
- Prior eye surgery
- Prior glaucoma treatment.
- Past medical history (blood disorders including sickle cell disease).
- Mechanism of trauma.
- Exact time of injury and of visual loss.
- History of excessive tearing after injury.
ALERT
History of excessive tearing may indicate open globe injury.
Physical-Exam
- General physical exam with emphasis on associated bodily injuries.
- Periorbital ecchymosis
- Eyelid lacerations
- Enophthalmos (depression of the globe within the orbit)
- Limited ocular movement with diplopia (may indicate orbital floor fracture)
- Proptosis (may indicate retro-orbital hemorrhage)
- Ocular exam:
- Visual acuity
- Rule out open globe (positive Seidel sign, corneal laceration, diffuse subconjunctival hemorrhage, decreased ocular motility, prolapse of intraocular structures)
- Pupillary reaction to light (check for afferent pupillary defect prior to using dilating drops)
- Tonometry for intraocular pressure (IOP) measurement
ALERT
Exclude open globe injury before measuring IOP
- Slit-lamp exam; look for layer of blood in AC:
- 4 grades of hyphema depending on percentage of AC occlusion by blood:
- Grade I: <1/3
- Grade II: 1/3–1/2
- Grade III: >1/2
- Grade IV: Total (called 8-ball hyphema; blood is dark and filling 100% of AC)
- High-grade hyphemas are:
- More likely to rebleed (25% of grade I compared with 67% of grade III)
- More likely to develop glaucoma and corneal staining
- Less likely to recover visual acuity
- Dilated fundus exam (avoid pressure on globe)