FP786 : Traumatic Hyphema in Sickle Cell Prone Western Odisha Patients :Its Prognosis and Management

Dr. Nilamadhab Pradhan, S16689, Dr. Sumita Mohapatra, Dr.Harshavardhan V K, Dr. Sujana Inampudi

FP786,PRESENTING AUTHOR:   NILAMADHAB PRADHAN  (P18210)

CO-AUTHORS:PROF(DR) SUMITA MOHAPATRA,HARSHAVARDHAN V K,SUJANA INAMPUDI

INTRODUCTION:

Sickle cell disease is highly prevalent (2-12%) in western odisha districts. According to a statement released by state health department of odisha, out of 5.35 lakhs people suffered by sickle cell hemoglobinopathy(SCH),only 5lakhs belong to the 13 western odisha districts.

Medical and surgical management of traumatic hyphema in patients with SCH differs in some respects from the management of patients with normal hemoglobin.SCH patients have a higher incidence of elevated IOPs,optic nerve ischemia,macular ischemia and optic atrophy.

The normal anterior chamber environment has a lower oxygen content than that in circulating blood or in viscera and a higher concentration of ascorbic acid.Both the conditions induce and maintain sickling of red blood cells.

So the increased susceptibility to  elevated IOP may be due to stagnation of blood in small vessels,excessive deoxygenation of erythrocytes,erythrostasis,sickling,increased blood viscosity and further reduction in blood flow.This cascade of events could result in arteriole occlusion by sickled erythrocytes.Also, to the extent that increased IOP decreases anterior chamber perfusion,it may contribute to increased anterior chamber sickling,which would also serve to further increase in IOP.

According to Goldberg,aqueous humor is the most noxious fluid in the body as far as sickling is concerned . Repeated or excessive dosages of hyperosmotics and diuretics should be avoided,as they may cause hemoconcentration and increased blood viscosity in the ocular microvasculature.Systemic carbonic anhydrase inhibitors not only promote hemoconcentration but also induce systemic acidosis which is known to promote erythrocyte sickling.Besides lowering the aqueous humor PH,acetazolamide increases the concentration of ascorbic acid in aqueous humor and ascorbate may exacerbate the sickling process itself possibly by acting as a reducing agent.Methazolamide creates less systemic acidosis than acetazolamide.So if a systemic carbonic anhydrase inhibitor is needed, then methazolamide should be preferred in patients of SCH.Regarding topical carbonic anhydrase inhibitors,no clinical trials reported acid-base and electrolyte imbalancen of anterior chamber.Timolol should be used as primary agent and topical brimonidine is added if additional agent is required.If a further agent is required, dorzolamide is preferred  before using methazolamide.

In study of Crouch and Frenkel,two patients who developed optic atrophy in setting of traumatic hyphema,both of whom had sickle cell trait, had IOP varying between 35 and 39 mm HG (for 2 and 4 days respectively).So early surgical intervention may be undertaken to get rid of sickled environment of AC responsible for raised  IOP and SCH specific medical therapy may be continued for follow up.

AIM OF THE STUDY:

To study the disease course,prognosis and management of trumatic hyphema in sickle cell hemoglobinopathy patients.

MATERIALS AND METHODS:

It is prospective study conducted at Regional Institute of Ophthalmology from  APR 15 to MAR 16 aged 14 to 32 years(mean 25±4.77 yrs),M:F::20:2 comprising study group(SG) and 12 to 36 years(mean 24.96±4.88yrs),M:F::28:2 comprising control group(CG). SG includes 18 sickle cell trait patients detected during routine screening as the patients attending RIO  are from sickle cell prevalent areas   and 4 sickle cell disease patients who are already diagnosed at the time of reporting.CG includes 30 age matched  non SCD patients.After taking careful history regarding time,type of injury,sickle cell status,associated blood dyscrasias,consumption of anticoagulants and antithrombotics – initial and final visual acuity, serial IOP measurements  ,grades of hyphema,corneal blood staining,secondary hemorrhage noted,response to medical therapy observed and accordingly surgical intervention in form of AC paracentesis done.

Inclusion criteria:

1-reporting within 48 hours of injury

2-closed globe injury

3-patients giving consent and ready to adhere to the follow up schedule.

Exclusion criteria:

1-reporting after 48 hours of injury

2-open globe injury

3-other pathologic eye diseases and systemic disorders like blood dyscrasias,haematological

disorders

4-receiving other antithrombotics and anticoagulants within 2 weeks of injury

5-Microhyphemas

6-prior intraocular surgery

All patients advised routine haematological tests,tests to identify blood dyscrasias(PT,APTT),serum electrolytes,fasting blood sugar.For suspected open globe injury CT scan of orbit done and B scan to rule out other intraocular injuries.

CRITERIA FOR INPATIENT TREATMENT:

1-presence of other ocular injuries

2-suspected child abuse

3-sickle cell hemoglobinopathy

4- large hyphemas filling more than 50% of anterior chamber

5-incapable of self care and may not be compliant with the treatment regimen

RESULTS:

Hyphema grading

Microhyphema RBCs suspended in AC without formation of layered clot
Grade 1 Layered blood filling less than 1/3 rd of AC
Grade 2 Layered blood filling 1/3rd to ½ of AC
Grade 3 Layered blood filling ½ to less than total volume of AC
Grade 4 Blood volume filling the entire AC

TABLE 1

           HYPHEMA       Study group(SG)      Control group(CG)
Grade 1                8                     12
Grade 2                8                     10
Grade 3                4                       5
Grade 4                2                       3

TABLE 2

Types of injury               SG                       CG
Assault               12                        18
RTA                 4                         5
Domestic violence                 2                         2
Others                 4                         5

TABLE 3(Serial IOP measurements in different grades of hyphema)

Hyphema grade Mean IOP day 1  Mean IOP day 2 Mean IOP day6
    SG     CG       SG     CG       SG     CG
Grade 1      32     22.41      29.12      18.66      20.75     15.08
Grade 2      37     25.8      34.12       20.1      20.37      15.9
Grade 3     40.25     33      40.75       28.6      21.25      18
Grade 4      49.5     38.33      47       37      22      25

TABLE 4( Corneal blood staining)

    SG   3(13.63%)
    CG   5(16.66%)

 

TABLE 5(Secondary hemorrhage)

SG 2(9.1%)
CG 3(10%)

TABLE 6(Surgical intervention in form of AC paracentesis)

SG 20(90.9%)
CG 3   (10%)

 

According to Goldberg,anterior chamber is the most noxious fluid in the body as far as sickling is concerned.AC tap erythrocytes were fixed with glutaraldehyde-formaline solution,centrifuged,smeared, stained and counted.For comparison, at the same time  venous blood drawn,processed and counted.

Sickled erythrocytes Sickle cell disease(4) Sickle cell trait(18)
AC tap 58.75% 10.16%
Venous blood 22.25% 1.11%

TABLE 7(Visual acuity )

          Visual acuity                      DAY 1                        DAY 6
      SG       CG        SG        CG
             6/6-6/18 6(27.27%) 10(33.33%) 16(72.72%) 21(70%)
             6/18-6/60 10(45.45%) 12(40%) 3(13.63%) 4(13.33%)
             6/60-PL+ 6(27.27%) 8(26.66%) 3(13.63%) 5(16.66%)

Discussion:

→Small grades(1 and 2) of hyphems are more common than grade 3 and grade 4 hyphemas in both groups.

→Assault is the common cause for traumatic hyphemas in both groups.

→Mean IOP on day 1 in SCH patients is significantly higher than that of non SCH  patients(p<.05).The IOP is severely elevated despite small amount of intracameral blood in SCH patients.

→After institution of medical therapy , the mean IOP is reduced near to normal range in small grade 1 and grade 2 hyphemas in non SCH patients except the grade 3 and grade 4 hyphemas.

Whereas even after proper SCH specific medical therapy, the mean IOP is more than 25 mm Hg in SCH patients in all grades of hyphemas on day two.

→According to Crouch and Frenkel, optic atrophy developed in 2 SCH traumatic hyphemas whose IOP varying between 35 and 39 mm Hg for 2 and 4 days respectively.Goldberg and Deutsch suggested surgical intervention to be undertaken if mean IOP is greater than 24 mm Hg over the first 24 hours to avoid optic nerve ischemia and macular ischemia in SCH patients.

→After day  two,surgical intervention in form of AC  paracentesis was carried out and SCH specific medical therapy continued and IOP was brought down to normal range in all grades of hyphemas in SCH patients.

Whereas in non SCH patients,AC paracentesis is required mainly in patients who developed corneal blood staining in grade 3 and grade 4 hyphemas

→AC paracentesis is required in 90.9% cases in SCH patients whereas only 10% cases required AC paracentesisin non SCH patients.

→Goldberg suggested raised IOP is due to sickled erythrocytes in AC.

AC tap showed 58.75% sickled erythrocytes in comparison to 22.25% in venous blood in sickle cell disease patients and 10.16% sickled erythrocytes in AC tap in comparison to only 1.11%in venous blood in sickle cell trait patients  showing statistical significance with p <.05 .

→Visual outcome was good  after proper medical and timely surgical intervention in both groups except the higher grades of hyphemas with corneal blood staining.Corneal blood staining was seen in 13.63% cases in SG and 16.66% in CG  without  statistical significance. These cases were seen in grade 3 and grade 4 hyphemas in both groups.

CONCLUSION:

Sickled erythrocytes are  mostly responsible for raised IOP even in small grades of hyphemas in SCH patients and cause of failure of medical therapy.So early AC paracentesis is to be carried out to get rid of the sickled environment in AC and specific medical therapy to be continued to prevent complications like secondary glaucoma,optic nerve ischemia and macular ischemia.

REFERENCE:

  • Goldberg MF.sickled erythrocytes,hyphema and secondary glaucoma,ophthalmic surgery.1979;10:17-31
  • Goldberg MF.the diagnosis and treatment of secondary glaucoma after hyphema in sickle cell patients.Am J Ophthal 1979;87:43-49
  • Deutsch TA, Weinreb RN, Goldberg MF, indications for surgical management of hyphema in sickle cell patients.Arch ophthal 1984;102:566-569
  • Crouch and Frenkel.amino caproic acid in treatment of traumaic hyphema.Am J Ophthal1976;81:355-360
  • Kennedy RH et al.traumatic hyphema in defined population;Am J Ophthal;1988:106:123-130
  • Greenberg MS et al; a simple and inexpensive screening test for sickle hemoglobin ;1972;286:1143-1144
  • Shluey Y et al.traumatic hyphema outcomes of outpatient management;1998:105:851-855
  • William Walton et al. management of traumatic hyphema;survey of ophthalmology;jul-aug 2002 vol 4.
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