Fig. 13.1
A case of malignant middle cerebral infarction. (a) Development of malignant middle cerebral infarction in a 40-year-old adult male. Presented with acute left hemiparesis. Left. Initial presentation. Early global cerebral swelling with preservation of grey/white interface and minimal sulcal effacement. Right. Eight hours. Following failure of endovascular therapy, there is now a large right middle cerebral artery infarct with effacement of cerebral sulci, effacement of the lateral ventricle and midline shift. (b) Temporal evolution of the “malignant” middle cerebral artery infarct. Left. Thirty-eight hours. Progressive midline shift with involvement of the basal ganglia. Right. Forty-eight hours. Clinical deterioration and prior to surgical decompression. Further progression with involvement of middle cerebral artery territory and basal ganglia
The procedure was first described in 1894 by Annandale and thereafter by Kocher in 1901. Its use gained popularity in the early 1970s predominantly in the setting of severe traumatic brain injury (TBI). However, the combination of poor clinical outcomes and experimental studies suggesting that decompression may actually worsen cerebral oedema led to the procedure being almost abandoned. Throughout the 1980s there was a resurgence in interest, and an increasing number of retrospective studies described the use of the procedure not only following severe TBI and stroke but also in the context of subarachnoid haemorrhage, severe intracranial infection and dural sinus thrombosis [1].
Overall there appeared to be a considerable amount of evidence that decompressive surgery could reduce mortality; however evidence that surgical intervention improved clinical outcome was less forthcoming. Certainly in the context of stroke, surgical intervention will not reverse the ischaemic deficit that precipitated the cerebral swelling, and the concern amongst clinicians was that any reduction in mortality came at the expense of an unacceptable increase in the number of survivors with severe disability and dependency. In order to address this issue, a number of prospective randomised controlled trials were conducted.
13.1 Current Evidence for Clinical Efficacy of Decompressive Hemicraniectomy in Stroke
13.1.1 The European Stroke Trials: Patients Under 60 Years of Age
In the early 2000s, three trials were independently conducted in Europe which compared decompressive hemicraniectomy with standard medical therapy within 48 h of presentation for patients under 60 years of age who developed clinical deterioration following middle cerebral artery infarction. The DECIMAL and DESTINY trials were interrupted early because of slow recruitment and a significant difference in mortality between the treatment groups favouring surgery [2, 3]. The HAMLET trial was stopped because it was thought that it would be highly unlikely that a statistically significant difference would be seen for the primary neurological outcome measure which was defined as good (modified Rankin Score [mRS] 0–3) or poor (mRS 4–6) [4].
What each trial independently demonstrated was that survival was improved in those patients who were randomised to receive decompressive surgery. However each individual trial was insufficiently powered to determine whether there was an improvement in favourable outcome. Thereafter, a pooled analysis of the 93 patients involved in all three trials was performed, and this confirmed not only the significant reduction in mortality, but it was also concluded that there was an increase in the number of patients with a favourable outcome (Table 13.1).
mRS | Hemicraniectomy | Conservative |
---|---|---|
Pooled analysis of the European stroke trials | ||
n = 51 patients (%) | n = 42 patients (%) | |
6 | 11 pts (22%) | 30 pts (71%) |
5 | 2 pts (4%) | 2 pts (5%) |
4 | 16 pts (31%) | 1 pt (2%) |
3 | 15 pts (29%) | 8 pts (19%) |
2 | 7 pts (14%) | 1 pt (2%) |
DESTINY II trial | ||
n = 49 patients (%) | n = 63 patients (%) | |
6 | 16 pts (33%) | 44 pts (70%) |
5 | 14 pts (28%) | 8 pts (13%) |
4 | 16 pts (32%) | 9 pts (15%) |
3 | 3 pts (7%) | 2 pts (3%) |
2 | 0 | 0 |
On initial examination, this would appear to provide compelling evidence for the efficacy of surgical decompression for patients less than 60 years of age (as per the trial enrolment criteria); however, there were some issues that required clarification. In the first instance, in order to obtain this positive result, favourable outcome had to be redefined. Previously, in the stroke literature the mRS had been used to assess outcome, and this was usually dichotomised into favourable (mRS 0–3) or unfavourable (mRS 4–6). The implication being that the aim of surgery is to achieve an outcome which is felt to be acceptable to the person on whom the procedure is being performed. Notwithstanding some limitations of the mRS, the fundamental issue that denotes a favourable outcome is that a person has a degree of independence.
In the pooled analysis, favourable was reclassified such that it included patients with a mRS of 4. This would therefore include patients who cannot walk unaided and cannot look after their bodily needs which is an outcome that has been regarded as unfavourable for many years. Indeed, closer examination of the data confirms that the increase in survival came almost directly at the expense of an increase in the number of patients with a mRS of 4 [6]. Amongst the survivors who were randomised to receive standard medical care, 75% (9 of 12) had a mRS of 3 or less, an outcome previously defined as favourable. A similar favourable outcome was only achieved in the 55% of the patients treated surgically (22 of 40). It would seem that the most likely outcome following medical therapy is either death or a favourable outcome (mRS 0–3), whereas surgery considerably increases the risk of survival with a mRS of 4 [6].
13.1.2 The DESTINY II Trial: Patients Over 60 Years of Age
The DESTINY II trial investigated the use of decompressive hemicraniectomy in patients over 60 years of age. In a similar design to the previous European trials, patients were randomised within 48 h of presentation to either surgical decompression or standard medical therapy. The results of this study confirmed that surgical intervention significantly reduced mortality; however the conclusion that “hemicraniectomy increased survival without severe disability” requires clarification (Table 13.1).
Most of the survivors in the hemicraniectomy group were adjudged to be either a mRS of either 4 or 5 which would seem to confirm the results of the previous studies that an increase in survival comes at the expense of survival with dependency. However, the finding that 63% of the survivors in the hemicraniectomy group gave retrospective consent to treatment would appear to support ongoing use of the procedure in this age group because it is difficult to argue that it is not on a patient’s best interest to have surgery if they are able to state that they are satisfied with their outcome and they would do the same again.
Yet closer examination of results shows that amongst the 27 survivors in the hemicraniectomy group, only 11 could adequately answer this question. The remaining 16 patients had to have a surrogate response from their next of kin because they themselves could not adequately answer the question because they had either severe aphasia or neuropsychological deficits. Given that only 7% (or three patients) achieved a mRS of 3, the remaining 24 patients had either mRS of 4 or 5. Assuming those patients with a mRS of 3 responded positively to the question regarding retrospective consent, amongst the remaining 24 patients, 16 patients could not walk without assistance, could not take care of their basic bodily needs and did have sufficient neurocognitive function such that they could answer a relatively simple question. Given what many informed commentators of sound mind would regard as an unacceptable outcome, these findings would encourage a reconsideration of the aforementioned conclusion [7].
Notwithstanding these observations, there is no doubt that the results of the trials provide good quality level 1 evidence on which to base clinical decision-making especially when considering patient selection and timing of surgery.
13.2 Patient Selection
When considering patient selection, age has always been an important factor because, generally, older patients have reduced brain plasticity, increased comorbidities and are less able to cope with the stress of surgical intervention and subsequent rehabilitation. However, in the light of the evidence currently available, the time may have come for a more nuanced approach to patient selection that seeks to explore an individual’s healthcare preferences once they have lost competency.
The fundamental issue rests the acceptability or otherwise of survival with severe disability, and broadly speaking there will be three categories of healthcare preferences that require consideration. Firstly, there are those patients whose preferences are unknown; secondly, there are those patients who have expressed an opinion that survival is paramount even with severe disability; and finally, there are those patients who expressed a view that survival with disability would be unacceptable.
13.2.1 Patients with No Previously Documented or Expressed Healthcare Preferences
Realistically, in the emotionally charged atmosphere of an acute stroke, it would be very difficult to withhold therapy in a person under 60 years of age if there was at least some chance of survival with an acceptable level of disability, and the possibility of unacceptable dependency was acknowledged and accepted by those involved in making the decision. Treatment based on such reasoning can be justified even if the eventual outcome seems unacceptable to the injured party because risks and uncertainties are inevitable in all fields of medicine. It could also be argued given the alternative would be not to survive at all, a young person may quite reasonably be given the chance to “risk” survival with a mRS of 4 in the hope that they will either improve to achieve a mRS of 3 or learn to accept a level of disability that they might previously have deemed unacceptable.
For patients over 60 years of age, it must be acknowledged that there is only a very small chance that that person may achieve independence and there is a high chance that they may be left with very significant neurocognitive and neuropsychological deficits. Ideally, the acceptability or otherwise of this outcome for that particular individual must be fully explored prior to surgical intervention.
13.2.2 Survival Is Paramount
There is little doubt that certain individuals may feel that life is sacrosanct and worth preserving under any circumstances, and this may be based on certain religious, cultural or personal values. These individuals may also be willing to “run the risk” of survival with severe disability, in the hope that they may achieve a good functional outcome. They might also want the opportunity to adapt and learn to live with a level of disability that they and many others, perhaps, might previously have thought to be unacceptable. Whilst these views may fall outside what is deemed acceptable to the majority, where possible, these views should be acknowledged and acted upon. For this group of patient’s surgical intervention, following the development of “malignant” cerebral infarction is entirely reasonable.
13.2.3 Survival with Disability Is Unacceptable
The final group of patients will be those patients who have previously expressed a view (either previously voiced or documented) that they would not want to survive with severe disability. In these circumstances the surgeon cannot reasonably assume that they would be able to obtain consent for the operation and, if they did proceed, would have to justify acting on their own judgement against a properly considered assessment of the wishes of the patient.