Definition
- Severe hemispheric syndrome with characteristic symptoms and a predictable clinical course including hemiparesis, eye, and head deviation, a progressive decline in consciousness, pupillary dilatation, and increased intracranial pressure.
Numbers
- Occurs in up to 10% of stroke patients
- Mortality of up to 80%
- Due to severe postischemic cerebral edema → increased ICP → herniation
Clinical presentation
- Severe hemispheric stroke (hemiplegia, forced eye and head deviation) often with CT findings of major infarct within the first 12 hours.
- Most develop drowsiness shortly after admission.
- Day 1-2: Progressive deterioration
- Day 2-4: Transtentorial herniation
- Fatalities are often associated with:
- Severe drowsiness
- Dense hemiplegia
- Age > 45–50 yrs,
- Early parenchymal hypodensity involving >50% of the MCA distribution on CT scan,
- Midline shift > 8–10mm,
- Early sulci effacement
- Hyperdense artery sign in MCA.
Management options
Conventional measures to control ICP (with or without ICP monitor):
- Mortality is still high in this group, and elevated ICP is not a common cause of initial neurologic deterioration in large hemispheric stroke
- From Hamlet trial
- Recommendations for treatment in intensive care units. Treatment in the intensive care unit was continued at least until day 5 after stroke onset or until there was sufficient clinical improvement to permit transfer of the patient to a stroke unit. Recommendations for best medical treatment in a stroke unit were osmotherapy, elevation of the head, and maintenance of normothermia, normoglycaemia, and normovolaemia, as described below.
- Osmotherapy with mannitol or glycerol as soon as possible after randomisation and at a dose suffcient to reach a serum osmolality Of 315—20 mOsm.
- Intubation and mechanical ventilation if the patient's Glasgow coma score was ≤8, if there were signs Of respiratory insufficiency, or if the airway was compromised.
- Hyperventilation should be used only as a rescue measure in case of further
neurological deterioration or an uncontrolled increase in intracranial pressure, with jugular bulb oximetry to maintain venous oxygen saturation at higher than 50% or to a target pC02 of 28—32 mm Hg. - Invasive monitoring of intracranial pressure, preferably on the same side as the infarct.
- Sedation in the case of mechanical ventilation, further neurological deterioration, or an uncontrolled increase in intracranial pressure, preferably with propofol; the use of barbiturates was discouraged but, if necessary, muscle relaxants could be used.
- Treatment of blood pressure higher than 220/120 mm Hg with labetolol or
nitroprusside; hypotension or a reduction of cerebral perfusion pressure could be treated with catecholamines. - Elevation of the head to an angle of 30°.
- Maintenance of normothermia, normoglycaemia, and normovolaemia.
- From Destiny trial
- Conservative Treatment
- Osmotherapy: Indication—Any clinical or neuroradiologic signs of space-occupying brain edema. Mannitol (0.5 g/kg 4x /day, every 4 to 6 hours; maximum daily dose, 2.5 g/kg), glycerol (250 mL, 10% solution, 4x /day), or hydroxyethyl starch (6% hetastarch in 0.9% NaCl injection, 100–250 mL every 8 hours; maximum daily dose, 750 mL); target serum osmolality= 315 to 320 mOsm.
- Intubation and mechanical ventilation: Indication—Glasgow Coma Scale score <8, any signs of respiratory insufficiency (PO2<60 mm Hg, PCO2>48 mm Hg), or compromised airway. Ventilation mode left at discretion of the treating physician. Target parameters=PO2>75 mm Hg, PCO2 36–44 mm Hg. In case of raised intracranial pressure, target parameters=PO2>100 mm Hg, PCO2 35–40 mm Hg, tidal volume 8–10 mL/kg, 10–12 breaths per minute, minimum of 5 cm H2O of positive end-expiratory pressure.
- Hyperventilation: Ultimate ratio in case of further neurologic deterioration and/or uncontrolled increase in intracranial pressure. Target PCO2 28–32 mm Hg.
- Venous oxygenation (jugular bulb oximetry, saturation >50%).
- Intracranial pressure monitoring: Invasive measurement in the ipsilateral hemisphere.
- Sedation: Mode including use of muscle relaxants left at the discretion of the treating physician. Propofol recommended. Use of barbiturates discouraged.
- Blood pressure: Target parameters in formerly hypertensive patients=180/100–105 mm Hg, in formerly normotensive patients =160–180/90–100 mm Hg. Target parameters during the first 8 hours after decompressive surgery=140–160 mm Hg.
- Positioning: Plane head positioning, elevation of 15–30° recommended in case of severely increased intracranial pressure, depending on CPP, or in patients at high risk of infection.
- Body core temperature: Target=normothermia. Treatment started at >37.5°C. Use of antipyretics, external or intravasal cooling left at the discretion of the treating physician.
- Blood glucose level: Target parameters=80–110 mg/dL. Treatment started at >140 mg/dL with insulin. Hypoglycemia treated with 10% or 20% glucose.
- Fluid management: Target=normovolemia; avoid hyponatremia.
- Prophylaxis of deep venous thrombosis: Weight-adjusted low-molecular-weight heparin.
- No seizure prophylaxis.
Hemicraniectomy (decompressive craniectomy):
Indications: No firm indications.
- NICE guidelines 2022
- Nice new guidelines don’t look at age.
- Performed within 48 hours of symptom onset
- Clinical features in keeping with MCA infarct, 15 on the NIHSS > 15
- Item 1a of the NIHSS > 1
- CT evidence of infarct of >50% of MCA territory:
- With or without additional infarction in the territory of the anterior or posterior cerebral artery on the same side or
- With infarct volume greater than 145 cm3, as shown on diffusion-weighted MRI scan.
- Age < 60 years
- Lin et al 2021
- None of the patients over the age of 60 in Zhao et al or in DESTINY II achieved a mRS score of 0 to 2 at 6- or 12-month follow-up.
- Chances of obtaining a functional outcome after surgical decompression in patients over age 60 to 65 years in which the patient would be able to ambulate independently are slim and that most likely the patient would require significant assistance with many routine daily activities
- Imaging
- CT: 50% of MCA area infarct
- DWI volume: >145cm3
- DO NOT CARE ABOVE MLShift
- Neurology
- NIHSS > 15
- Associated drop in NIHSS 1a>0
- NOT drop in GCS
- Surgery within 48hrs (referred to Nsx within 24hrs after symptom onset) = 72 hrs from onset
- After 48 hrs no effect on outcome
- Prestroke mRS <=1
- Soft indication
- More strongly considered in nondominant hemisphere (usually right)
- Lin et al 2021
- No enough data to suggest dominant hemisphere outcome is worse
- Aphasia might not be worse than motor deficit
- Aphasia is poorly studied as measuring tools do not test for aphasia specifically while there are a lot of testing tool for motor deficits
Outcome
- (retrospective carter et al 1997)
- In non dominat hemisphgere stroeks
- Can reduce mortality to as low as 32% (37% in all comers) with surprising reduction of hemiplegia
- In dominant hemisphere strokes,
- With only mild-moderate aphasia (better results occur with early surgery, especially if surgery is performed before any changes associated with herniation occur).
- Meta-analysis of 3 randomized controlled trials found that hemicraniectomy within 48 hours after stroke onset resulted in decreased mortality and increased the number of patients with a favourable functional outcome.
- Lin et al 2021
- Study Characteristics and Findings of 8 Randomized Controlled Trials of Decompressive Hemicraniectomy vs Medical Management only
- ARR indicates absolute risk reduction; CT, computed tomography; DWI, diffusion-weighted imaging; GCS, Glasgow Coma Scale; MRI, magnetic resonance imaging; mRS, modified Rankin Scale; and NIHSS, National Institutes of Health Stroke Scale.
- The additional survivors gained in the surgical groups achieved different mRS outcomes across the board, but the majority of survivors had an mRS score of 4 or 5 at 12 months.
- What is considered favourable outcome
- It is difficult to generalize the definition of a favourable outcome and thus exists the variability in outcome measures between the studies;
- However, a multicentred international survey of over 1800 physicians found that while a mRS score of 3 was considered acceptable by 79% of the respondents, only 38% considered a mRS score of 4 to be acceptable.
- Mortality
- Absolute death reduction: 41.4%
- NNT to prevent 1 death 2.4
- Functional improvement at 1 year
- mRS score 0 to 3 (NNT=4, absolute risk reduction 23%)
- mRS score 0 to 4 (NNT=2, absolute risk reduction 51%)
- HeADDFIRST
- Had the lowest mortality rate in the conservative medical treatment group at 6 months (40.0%, with DESTINY having the second lowest mortality rate at 53.3%, with a range of mortality rates 40.0%–77.8% among the 6 trials that studied mortality rates at 6 months),
- Mortality rate in the surgical arm was similar to that in the other studies.
- It was the most stringent in enforcing a
- Standardised medical protocol,
- Had the strictest neuroimaging criteria (required evidence of midline shift) for enrolment,
- Was one of only 2 trials with the longest enrolment time (enrolled up to 96 hours) after stroke onset.
- All 3 of these factors may have reduced the mortality benefit of decompressive hemicraniectomy by optimising medical therapy, enrolling a cohort with more significant oedema and thus worse surgical morbidity and mortality, and performing surgery after significant secondary brain injury had already incurred given the longer time to decompression.
- This trial raises the question of whether there should be a new focus on optimising medical treatment in LHI that may ultimately negate the current perceived mortality benefits of surgical decompression; more research is required to make firm conclusions about this.
Trials | Time criteria (h) | Age criteria (y) | NIHSS (or GCS) criteria | Neuroimaging criteria | Prestroke mRS criteria Primary | Primary outcome | Findings |
DECIMAL (2007, France) 45; n=38 | <24 | 18-55 | >15; NIHSS 1a≥1 | Brain CT ischemia >50% MCA territory, brain MRI DWI infarct volume >145cm^3 | 0-1 | mRS score 0–3 at 6 mo | No significant difference in favourable functional outcome (mRS score 0–3: 25% of surgical group vs 5.6% of medical group; p=0.18) at 6 mo; significant reduction in mortality (25% vs 78%; p<0.0001) in surgical group at 6 mo |
DESTINY (2007, Germany)46; n=32 | 12-36 | 18-60 | >18 for right-sided lesion; >20 for left−sided lesion; NIHSS 1a≥1 | Brain CT ischemia ≥2/3 MCA territory including part of basal ganglia | 0-1 | mRS at 6 mo (0–3 vs 4–6) | No significant difference in favourable functional outcome (mRS score 0–3) at 6 mo (47% of surgical group vs 27% of medical group; OR 2.44 [95% CI, 0.55–10.83]); significant reduction in mortality (survival rate 88% vs 47%; OR 6.37 [95% CI, 1.35–29.17]) in surgical group at 30 d |
HAMLET (2009, Netherlands) 47; n=64 | <96 | 18-60 | >15 for right-sided lesion; >20 for left-sided lesion | Brain CT ischemia ≥2/3 MCA territory | 0-1 | mRS at 12 mo (0–3 vs 4–6) | No significant difference in unfavourable functional outcome (mRS score 4–6: 75% vs 75%, ARR 0% [95% CI, −21 to 21]) at 12 mo; significant reduction in mortality (22% vs 59%, ARR 38% [95% CI, 15–60]) in surgical group at 12 mo |
Slezins et al48 (2012, Latvia); n=24 | <48 | >18 | >15 | Brain CT or MRI ischemia ≥50% MCA territory; or >145cm^3 infarct volume | 0-1 | mRS at 12 mo (0–4 vs 5–6) | Reduction in mortality in surgical group (survival rate 46% vs 8%; P =0.06 Fisher exact test) |
Zhao et al49 (2012, China); n=47 | <48 | 18-80 | (GCS eye and motor score ≤9) | Brain CT ischemia ≥2/3 MCA territory and space-occupying edema | 0-1 | mRS at 6 mo (0–4 vs 5–6) | Significant reduction in unfavourable outcomes (mRS score 5–6: 33.3% vs 82.6%; ARR 49.3% [95% CI, 24.9–73.7]) and in mortality (12.5% vs 60.9%; ARR 48.4% [95% CI, 24.4–72.3]) in surgical group at 6 mo |
HeADDFIRST (2014, North America)8; n=24 | <96 | 18-75 | >17; NIHSS 1a <2 | Brain CT ischemia ≥50% MCA territory within 5 h or complete MCA territory within 48 h; and anterior septum pellucidum shift from midline of ≥7.5 mm OR pineal gland shift from midline ≥ 4 mm (with deterioration in NIHSS 1a ≥2) | 0-2 | Death at 21 d | No significant reduction in mortality at 21 d (21% of surgical group vs 40% of medical group; ARR 19% [90% CI, −13–50]) |
DESTINY II (2014, Germany)50 n=112 | <48 | >60 | >14 for right-sided lesion; >19 for left−sided lesion; NIHSS 1a ≥1 | ≥2/3 MCA territory including basal ganglia | 0-1 | mRS score 0–4 at 6 mo | Significant improvement in favourable outcome (mRS score 0-4 : 38% vs 18%; OR, 2.91 [95% CI, 1.06-7.49]) and in mortality (33% vs 70%) in surgical group at 6 mo |
HeMMI (2015, Philippines)51; n=24 | <72 | 18-65 | (GCS 6–14 for right-sided lesion; 5–9 for left-sided lesion; or, GCS 15 on arrival with subsequent deterioration with NIHSS 1a ≥1) | Brain CT ischemia >50% MCA territory | 0-2 | mRS at 6 mo (0–3 vs 4–6) | No significant difference in favourable functional outcome (mRS score 0–3: 23% of surgical group vs 38% of medical group; ARR 13% [95% CI, −23 to 50]) or mortality (39% of surgical group vs 55% of medical group; ARR −16% [95% CI, −56 to 23]) at 6 mo |
Destiny trial:
- Decompressive surgery
- Large (reversed) question mark–shaped skin incision based at the ear
- Removal of a bone flap (diameter >12 cm, including the frontal, parietal, temporal, and parts of the occipital squama)
- Removal of additional temporal bone so that the floor of the middle cerebral fossa can be explored
- Opening of the dura and insertion of an augmented dural patch consisting of either homologous periost and/or temporal fascia
- No resection of infarcted brain tissue
- Fixation of the dura at the margin of the craniotomy
- Reapproximation and securing of the temporal muscle and skin flap
- Insertion of a sensor for registration of intracranial pressure
- Cranioplasty in surviving patients after 6–8 weeks, with the stored bone flap or artificial bone flap
- CPP indicates cerebral perfusion pressure.
Other options that have not improved outcome:
- Agents to lyse clot
- Hyperventilation
- Mannitol
- Barbiturate coma
Predicting who will need decompression
Summary of 4 Risk Scores for Predicting the Development of Malignant Cerebral Edema After a Hemispheric Infarct
MBE score¹⁴ | Kasner Index¹⁵ | EDEMA score¹⁶ | DASH score¹⁷ |
NIHSS | History of hypertension | Basal cistern effacement | DWI ASPECTS |
≤8=0 | No=0 | No=0 | >3=0 |
9-17=1 | Yes=1 | Yes=3 | ≤3=1 |
≥18=2 | ㅤ | ㅤ | ㅤ |
ASPECTS | Congestive heart failure | Glucose ≥150 mg/dL | ACA territory involvement |
>8=0 | No=0 | No=0 | No=0 |
≤7=1 | Yes=1 | Yes=2 | Yes=1 |
Collateral score | White blood cell count | Previous stroke | M1 susceptibility vessel sign |
≥2=0 | ≤10000/µL=0 | Yes=0 | No=0 |
<2=2 | >10000/µL=1 | No=1 | Yes=1 |
Revascularization failure | CT involvement >50% MCA territory | tPA or thrombectomy | Hyperglycemia (glucose ≥145 mg/dL) |
Success=0 | No=0 | Yes=0 | No=0 |
Failure=1 | Yes=1 | Yes=1 | Yes=1 |
ㅤ | CT involvement additional territories | Midline shift | ㅤ |
ㅤ | No=0 | 0 mm=0 | ㅤ |
ㅤ | Yes=1 | 0–3 mm=1 | ㅤ |
ㅤ | ㅤ | 3–6 mm=2 | ㅤ |
ㅤ | ㅤ | 6–9 mm=4 | ㅤ |
ㅤ | ㅤ | >9 mm=7 | ㅤ |
Max: 6 points | Max: 5 points | Max: 14 points | Max: 4 points |
0: 0.0% rate of MBE | 0: 31% rate of fatal brain edema | >7: PPV 93% for potentially fatal malignant edema | 0: 9.1% rate of malignant MCA infarction |
6: 100.0% rate of MBE | 5: 100% rate of fatal brain edema | ㅤ | 4: 100.0% rate of malignant MCA infarction |
≥5: PPV 91.7% | ㅤ | ㅤ | ㅤ |
C statistic=0.88 | C statistic=0.70 | C statistic=0.76 | C statistic=0.88 |
ACA indicates anterior cerebral artery; ASPECTS, Alberta Stroke Program Early CT Score; DASH, DWI ASPECTS; ACA territory involvement, M1 susceptibility vessel sign, hyperglycemia; DWI, diffusion-weighted imaging; EDEMA, enhanced detection of edema in malignant anterior circulation stroke; MBE, malignant brain edema; MCA, middle cerebral artery; mRS, modified Rankin Scale; NIHSS, National Institutes of Health Stroke Scale; PPV, positive predictive value; and tPA, tissue-type plasminogen activator.