Case History

1
Case History

• HPI on 2/26, 19 yo AAF with sickle cell disease,
  acute headache, left arm weakness, dysarthria,
  trouble walking.
• PH SS by electrophoresis no h/o ACS, stroke.
  Infrequent crises, rare transfusions. In 5/92,
  pneumonia. In 12/06, fetal death at 25 weeks,
  hypertension.
• PE BP 207/150, afebrile, P 100, R 18
• Lab wbc 20.7, hgb 9.0, plt 491 PT 14.5, INR
  1.15, PTT 28.7.

2
Case History

• Differential diagnosis
• Ischemic stroke
• Hemorrhagic stroke
• Endocarditis (embolism)
• Antiphospholipid antibody syndrome
• Carotid/vertebral artery dissection
• Meningitis
• Cerebral venous thrombosis

3
Stroke Presentation

• Ischemic stroke
• Hemiparesis, aphasia, monoparesis, seizure
• Hemorrhagic stroke
• Severe headache

4
Case History
CT Large R parietal hemorrhage, edema, midline
shift 8 mm inferiorly, subarachnoid blood
tracking along the falx. Angio proximally
occluded A1 segment of R ACA. R ACA territory
supplied from anterior communicating artery and
regrograde pial collaterals from R MCA branches.
Possible R ACA infarction with hemorrhagic
transformation.
5
Initial Noncontrast CT
6
Angiography
Left Internal Carotid
Right Internal Carotid
A1 seg
A1 seg
7
Stroke in SSD
Stroke-free survival by genotype
Ohene-Frempong et al, Blood 1998 91 288-94.
8
Stroke in SSD - SS Genotype
Likelihood of stroke 11 by age 20, 15 by age
30 n 2,500
Ohene-Frempong et al, Blood 1998 91 288-94.
9
Stroke in SSD - SS Genotype
75 are ischemic at ages lt20 75 are
hemorrhagic at ages 20-29
Ohene-Frempong et al, Blood 1998 91 288-94.
10
Stroke in SSD
(BP 207/150)
(WBC 20,700) (Hgb 9.0)
But no mean or reference values are described
Ohene-Frempong et al, Blood 1998 91 288-94.
11
Stroke in SSD
Pathophysiology Distal internal carotid,
proximal MCA and ACA are stenotic or occluded in
SS patients with stroke. Proliferative
vasculopathy, with reduced vasodilitation in
response to hypoxia. Most common sites of
ischemic stroke are parenchymal areas supplied by
ACA and MCA, and the border zones between their
distal circulations. Mortality 24-40
hemorrhagic, 0 ischemic
Ohene-Frempong et al, Blood 1998 91 288-94.
12
Stroke in SSD
Risk factors TCD 200 cm/s - 10 per year
ischemic stroke risk. Prophylaxis chronic
transfusion reduces ischemic stroke risk 92.
Insufficient data for hemorrhagic
stroke. Treatment Exchange transfusion? Data for
ischemic stroke, in children, from Washington
University. No information about effect on
neurological outcome.
13
Initial Treatment of Stroke in SSD
Children (mean age 6.3, range 1.4-14 years),
surviving gt 5 years after (ischemic) stroke
Hulbert et al, J Pediatr 2006 149 710-2
14
Stroke in SSD, lt24 Hours
Simple transfusion increases the risk of second
stroke. All received chronic transfusion for 5
yrs.
Exchange n38
Simple n14
RR 5 (CI 1.3-18.6)
Hulbert et al, J Pediatr 2006 149 710-2
15
Exchange Transfusion Target
Oxygen transport versus hemoglobin
Aim for Hgb S lt 30, Hgb 10 g/dl
16
Hemorrhagic Stroke (General)

• Associated with hypertension gt aneurysms gt
  Moyamoya
• Hypertension seen in 50 of patients with ICH
• Lowering of BP proposed to convert ischemic
  tissue to infarct (though evidence is
  inconclusive). Therefore
• Control BP gingerly, SBPlt160, DBP lt110
• Fix coagulopathy (if present)
• Prevent seizures

17
Case History
2/27 Exchange transfusion extubated
amlodipine, clonidine, hydralazine, labetalol
dilantin, Keppra. Hgb S 20, Hgb 10.5, Plt 141,
Cr 1.2 BP 166/108 3/4 Transfer to floor, intact
UE strength, speaking. BP 162/100 to 189/123,
Tmax 38.7 CXR - clear 3/5 Respiratory distress.
Hgb S 27, Hgb 7.9, Plt 114, Cr 1.9, HIT neg.
PaO2 99. Not intubated. To MICU. CXR - diffuse
bilateral infiltrates R gt L Lung Scan - Low
probability for PE CT New L frontal bleed, mass
effect, edema
18
Head CT
Mar 5
19
Acute Lung Injury?
Feb 26
Mar 5
20
Organ Dysfunction in Stroke

• Non-neurologic organ dysfunction is common after
  brain injury
• Acute Lung Injury
• 100 with traumatic head injury and coma, 20 ALI
  (Bratton Davis, Neurosurgery 1997 40 707-12)
• 620 with aneurysmal SAH, 27 ALI (Kahn et al,
  Crit Care Med 2006 34 196-202)

21
Mechanisms of Acute Lung Injury

• Neuogenic pulmonary edema
• Bilateral pulmonary edema, normal LV function,
  normal volume status
• Usually day 0-3, but as late as day 14
• Mortality 10
• Catecholamine-induced pulmonary and systemic
  vasoconstriction, increased vascular permeability?

Berthiaume Zygun, Crit Care Clin 2007 22
753-66
22
Mechanisms of Acute Lung Injury

• Pneumonia
• Incidence 20 in SAH
• S. aureus, S. pneumoniae, H. influenzae
• Day 3-4 peak
• Nosocomial (intubated, ventilator-associated)
• Aspiration (decreased airway reflexes)

Berthiaume Zygun, Crit Care Clin 2007 22
753-66
23
Case History
3/6 Headache, talking, sl decreased L grip
strength. Tx 1 U PRBC. Afebrile. Lasix cefipime,
vancomycin. Hgb 6.8, wbc 27.9, plt 168 ANA neg,
ANCA neg LE dopplers - DVT L common femoral
vein CXR - improved R lung densities TEE -
impaired relaxation, normal EF, no
vegetations CT, MRI - unchanged 3/7 Temporary
IVC filter placed. Return to Neurology floor 3/8
Afebrile, BP 158/97 to 204/126
24
Prognosis by Organ Involvement

• 242 patients with aneurysmal SAH
• Overall mortality 23
• 133 with isolated mild-moderate CNS dysfunction -
  9 mortality
• 46 with isolated CNS failure (stupor,
  hemiparesis, coma, or moribund) - 17 mortality
• 43 with CNS failure and 1 other organ system
  failure - 65 mortality

Gruber et al, Crit Care Med 1999 27 505-14
25
Prognosis by Organ Involvement

• 61 with aneurysmal SAH and 1 extracerebral organ
  system failure (OSF)
• 47 with PaO2/FiO2 lt150 - 62 mortality
• 26 with (HR x CVP/MAP) gt20 - 80 mortality
• 15 with Plt lt50,000 - 80 mortality

Gruber et al, Crit Care Med 1999 27 505-14
26
Prognosis by Organ Involvement

• 61 with aneurysmal SAH and 1 extracerebral organ
  system failure (OSF)
• Overall mortality 68
• 31 with one extracerebral OSF - 31 mortality
• 12 with two extracerebral OSF - 91 mortality
• 10 with 3 extracerebral OSF - 100 mortality

Gruber et al, Crit Care Med 1999 27 505-14
27
Case History
3/9 Transient respiratory distress, hypoxia.
Completely flaccid. Transfer to MICU. Afebrile,
BP 160/107. Hgb 9.1, wbc 29.8, plt 418, Cr 1.8.
CXR - R lung collapse, pleural effusions 3/10
Tmax 38.9 3/11 Tmax 39.1, Cr 3.0 CXR - some
reexpansion of R lung 3/12 Cr 2.9 CXR - R lung
completely reexpanded CT - decreased L frontal,
R parietal hematomas, decreasing vasogenic edema
and mass effect
28
Effusions, Mucus Plug?
Mar 9
Mar 12
3 pm
8 pm
29
Case History
3/14 Transfer to Medicine floor. Hgb S 31, Hgb
8.5, wbc 19.8, plt 247, Cr 2.8. 3/17 Transfuse
1 U PRBC, Cr 3.4 U/S - no hydronephrosis 3/19
BP 190/134. Hgb S 34. Cr 3.7 CT - Possible new
small L frontoparietal hemorrhage with
surrounding edema 3/20 Transfuse 2 U PRBC, Cr
3.8 3/22 Unable to speak. HD catheter placed,
dialyzed. BP 195/142. Cr 4.6
30
Head CT
Mar 19
31
Renal Dysfunction in SAH

• 242 with aneurysmal SAH
• 33 (14) required hemodialysis, not strongly
  correlated with survival or outcome

Gruber et al, Crit Care Med 1999 27 505-14
32
Case History
3/23 Decreasing mental status. Tmax 38.9, BP
168/124 CT - Unchanged from 3/19 3/24
Transient hypotension, afebrile, P 83, BP 89/47.
3/26 Stopped breathing. CPR, suctioned,
intubated, not shocked. Transfer to CCU.
Afebrile, BP 77/45 post code. Hgb 9.8, wbc 12.1,
plt 234. PaO2 223, FiO2 50. 3/27 Pupils fixed,
dilated. Cr 4.8 CT - new hemorrhage medial L
frontoparietal, extending into ventricular
system. Hydrocephalus and herniation of
cerebellar tonsils. 3/29 extubated, expired
33
Head CT
Mar 26
34
Summary

• 19 yo female with SS disease, expired after
  repeated intracerebral hemorrhages
• Risk factors for hemorrhagic stroke include
  hypertension, SSD in the third decade of life
• Morbidity/mortality from hemorrhagic stroke is
  high
• Pathogenesis of hemorrhagic stroke in SSD is not
  clear
• Value of acute exchange transfusion for ischemic
  stroke is not established, but seems reasonable
• Value of exchange transfusion in hemorrhagic
  stroke is not established