unpublished data from one trial), found that a favorable outcome (defined as a mRS 3) at one year was more likely with DHC compared with medical treatment alone (37 versus 15 percent, absolute difference, 22 percent, adjusted odds ratio [OR] 2.95, 99% CI 1.55-5.60) [49]. In addition, DHC reduced the risk of death at one year (29 versus 71 percent, absolute difference 42 percent, adjusted OR 0.16, 95% CI 0.10-0.24). The favorable outcome benefits of decompressive surgery were consistent across subgroups defined by age (<60 versus >60 years), sex, presence of https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 11/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate aphasia, baseline NIH Stroke Scale score, time to randomization (<24, 24 to 48 and >48 hours), and extent of infarction by vascular territory. However, among patients >60 years old, the proportion who achieved a favorable outcome varied markedly across studies, from 0 to 66 percent, and the authors concluded that the evidence of benefit in older patients remained uncertain. Similarly, too few patients were treated beyond 48 hours to draw reliable conclusions about late treatment. And parallel to the individual trial data, it was again seen that DHC led to increased numbers of patients with moderately severe or severe disability (mRS 4 or 5), although these disability outcomes were not individually analyzed. Thus, as demonstrated by meta-analyses of randomized controlled trials, the primary benefit of DHC across age groups is increased survival. Though DHC increases the number of survivors with moderate or severe disability as illustrated in the figure ( figure 2), it also increases the chances of more favorable outcomes, particularly in patients less than 60 years of age [49-51]. Although these studies assessed clinical efficacy using mRS as the neurological outcome assessment, they did not assess other metrics of outcome such as quality of life or cognitive outcomes. While DHC confers survival benefit in patients older than 60, it is less clear whether DHC improve clinical outcomes in these patients given that they generally do not achieve good neurological outcomes (ie, moderate or slight disability) regardless of treatment. The available data also suggest that DHC within 48 hours is more likely to be beneficial than later hemicraniectomy, as noted in earlier meta-analyses [48,52]. Less robust data suggest but do not establish that later surgery (ie, from 48 to 96 hours) may be beneficial [49,53]. Selection of patients General eligibility criteria for DHC are listed in the table ( table 3). Timing of surgery Some investigators have advocated early (within 24 hours of onset), prophylactic surgical decompression for large hemispheric infarction, before the appearance of signs of herniation. This approach has intuitive appeal since it is logical that early decompressive surgery (before the development of life-threatening herniation with secondary brain injury) should result in the best clinical outcomes [47]. There is also experimental evidence that early surgery is more likely to be beneficial [54,55]. Furthermore, there is clinical evidence that aggressive medical reversal of a single episode of transtentorial herniation in the absence of radiographic evidence of midbrain injury may permit good long-term outcome [56]. These findings suggest that herniation alone does not preclude benefit from surgical decompression and that medical management may be an appropriate initial therapeutic option to a bridge to DHC. https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 12/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate However, the data are limited and conflicting regarding the utility of selecting patients for surgical decompression based upon time since symptom onset to treatment. Timing of surgery has been analyzed for <24 hours, 24 to 48 hours, and 48 to 96 hours. In subgroup analyses of pooled data from three randomized controlled trials, there was no difference in outcome between patients assigned to early (<24 hours after stroke onset, n = 45) or later (24 to 48 hours, n = 38) decompressive surgery [52]. In the HAMLET trial, which evaluated DHC up to 96 hours after stroke onset, there was no benefit on any outcome measure for patients assigned to surgery after 48 hours (n = 25) [35]. However, small numbers of patients treated beyond 48 hours in the meta-analysis [52], HAMLET [35], and HeADDFIRST [57] preclude definitive conclusions about of the possible efficacy of surgical decompression after 48 hours. In a 2004 systematic review of 12 studies with over 100 patients who were treated with hemicraniectomy for malignant MCA territory infarction, the time to treatment, clinical signs of herniation, sex, and side of infarction did not predict outcome with decompressive surgery [40]. Other reports have not found time to treatment to be a predictive factor for outcome, although this may be confounded by variables such as age [58]. A retrospective study from the United States analyzed 1301 patients from the Nationwide Inpatient Sample (2002-2011) who had decompressive craniectomy for stroke [59]. Of these, 726 had surgery within 48 hours. When time was evaluated as a continuous variable, later surgery was associated with increased risk of discharge to institutional care (OR 1.17, 95% CI 1.05-1.31) and poor outcome (OR 1.12, 95% CI 1.02-1.23). Procedure The surgical technique of DHC and durotomy varies in the literature. In general, a large question mark-shaped incision is made in the scalp, starting from the midline, that includes the frontal, parietal, and temporal lobes. A large incision is essential to the success of the procedure to avoid both incomplete release of pressure and further injury to the brain where it is forced against the edges of the craniotomy. A diameter of 12 cm or larger is recommended [3]. The protocol for one pilot trial defined required margins of the skull defect as follows [57]: Anterior: from the floor of the anterior cranial fossa at the mid-pupillary line Posterior: to 4 cm posterior to the external auditory canal Superior: to 1 cm lateral to the superior sagittal sinus Inferior: to the floor of the middle cranial fossa https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 13/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate Bone is removed and can be stored in a frozen tissue bank or sewn into the peritoneal cavity of the patient if this is to be reused for later cranioplasty (replacement of skull). The dura is opened with a cruciate incision to allow the brain to swell outwards. In some reports, infarcted tissue is resected ("strokectomy") [60,61]. Other surgeons advocate against tissue resection, as the removal of islands of normal functioning tissue not apparent at the time of surgery may worsen outcome. However, removal of large areas of infarcted tissue may be required in certain cases to ensure adequate decompression and reduce the risk of herniation. Complications Possible complications of craniectomy include hydrocephalus, external brain tamponade, infections, seizures, and paradoxical herniation [62]. The "sinking skin flap" syndrome (SSFS), also termed "syndrome of the trephined," is a delayed complication of craniectomy that can occur when atmospheric pressure exceeds intracranial pressure [62]. The major clinical features are the sunken appearance of the skin over the skull defect and severe orthostatic headache. Associated symptoms may include focal neurologic deficits, seizures, and altered mental status. Unchecked, SSFS may progress to paradoxical brain herniation, coma, and death. In the DECIMAL trial, with follow-up data available from 27 patients, SSFS developed at three to five months after hemicraniectomy in three patients (11 percent) [63]. Radiologic SSFS developed in another four patients (15 percent) and was generally asymptomatic except for partial seizures affecting one patient. Management of SSFS and paradoxical herniation, which is a neurocritical care emergency, requires measures that increase intracranial pressure, such as Trendelenburg position, intravenous hydration, clamping of cerebrospinal fluid drainage, and discontinuation of hyperosmolar measures [62]. In the absence of paradoxical herniation, SSFS may respond to intravenous fluid administration and supine position with head turned down to the side of the craniectomy [63]. Cranioplasty is the definitive treatment for this syndrome. Independent of SSFS, cranioplasty is a necessary second-phase procedure for stroke survivors with hemicraniectomies. While the timing of cranioplasties varies, there is evidence that earlier cranioplasty (potentially within same hospitalization) is associated with enhanced neurologic recovery [64], potentially attributable to restoration of cerebral hemo- and hydrodynamics. SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Stroke in adults".) https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 14/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate SUMMARY AND RECOMMENDATIONS Malignant hemispheric infarction This type of stroke is characterized by the development of space-occupying cerebral edema that is severe enough to produce brain tissue shifts and herniation. Clinical features typically include forced gaze deviation, visual field deficit, hemiplegia, and aphasia or neglect, depending on the hemisphere involved. The most common etiology is cardioembolic or thrombotic occlusion of the internal carotid artery or the proximal segment (stem or M1 segment) of the middle cerebral artery (MCA). The mortality is as high as 78 percent due to herniation of the temporal lobe on to the brainstem. (See 'Description' above and 'Presentation and progression' above.) Predictors of malignant edema Markers that may predict the development of malignant edema after a large hemispheric infarction include the early onset of decreased consciousness, younger age, higher National Institutes of Health Stroke Scale (NIHSS) scores on admission, and evidence of early infarction involving >50 percent of the MCA on initial head computed tomography (CT) or magnetic resonance imaging (MRI). (See 'Clinical predictors of progression' above and 'Radiologic predictors of progression' above.) Medical treatment options These include management of increased intracranial pressure and cerebral edema, and supportive medical care or withdrawal of medical care for those who do not desire aggressive therapy. Standard medical therapy for large hemispheric infarctions involves close monitoring for neurologic complications, such as depressed mental status and herniation; management of intracerebral pressure, with measures such as elevation of the head of the bed, osmotic therapy, and brief periods of hyperventilation as needed; and prevention of secondary complications such as aspiration and deep venous thrombosis. (See 'Medical management' above and 'Palliative care' above.) Outcomes with hemicraniectomy Decompressive hemicraniectomy (DHC) for malignant hemispheric infarction substantially reduces mortality. However, most surviving patients are left with major disability. Thus, the dilemma for patients, families, and medical decision- makers is that surgery may leave patients alive with severe disability, while medical management alone most often results in death. Among patients older than 60 years of age with malignant hemispheric infarction, DHC can increase survival, yet it is less clear whether DHC will confer clinical benefit in these patients, and most who survive are left with severe to very severe disability. (See 'Surgical decompression or medical care only?' above and 'Efficacy' above.) https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 15/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate Possible complications of DHC include hydrocephalus, infections, seizures, intracranial hemorrhage and fluid collections, sinking skin flap syndrome, and paradoxical herniation. (See 'Complications' above.) Our approach For patients age 60 years or younger with infarction involving >50 percent of the MCA territory associated with a decreased level of consciousness, who are thus at high risk of developing malignant edema, we suggest decompressive hemicraniectomy (DHC) if surgery can be initiated within 48 hours of stroke onset (Grade 2B). Hemicraniectomy should occur only after a thorough discussion with the patient (if cognizant), family, or appropriate medical decision-makers (eg, health care proxy); they should understand that hemicraniectomy increases the odds of survival and may increase the chance of surviving with only moderate disability, but most patients who survive are left with severe to very severe disability. The alternative treatment of aggressive medical therapy for malignant edema may be considered if surgery is deferred. (See 'Surgical decompression or medical care only?' above and 'Decompressive hemicraniectomy' above and 'Medical management' above.) 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Topic 1096 Version 27.0 https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 21/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate GRAPHICS National Institutes of Health Stroke Scale (NIHSS) Administer stroke scale items in the order listed. Record performance in each category after each subscale exam. Do not go back and change scores. Follow directions provided for each exam technique. Scores should reflect what the patient does, not what the clinician thinks the patient can do. The clinician should record answers while administering the exam and work quickly. Except where indicated, the patient should not be coached (ie, repeated requests to patient to make a special effort). Instructions Scale definition Score 1a. Level of consciousness: The investigator must choose a response if a full 0 = Alert; keenly responsive. 1 = Not alert; but arousable by minor evaluation is prevented by such obstacles as stimulation to obey, answer, or respond. an endotracheal tube, language barrier, orotracheal trauma/bandages. A 3 is scored only if the patient makes no movement (other than reflexive posturing) in response to noxious stimulation. 2 = Not alert; requires repeated stimulation to attend, or is obtunded and requires strong or painful stimulation to make movements (not stereotyped). _____ 3 = Responds only with reflex motor or autonomic effects or totally unresponsive, flaccid, and areflexic. 1b. Level of consciousness questions: The patient is asked the month and his/her age. The answer must be correct - there is no 0 = Answers both questions correctly. 1 = Answers one question correctly. 2 = Answers neither question correctly. partial credit for being close. Aphasic and stuporous patients who do not comprehend the questions will score 2. Patients unable to speak because of endotracheal intubation, orotracheal trauma, severe dysarthria from _____ any cause, language barrier, or any other problem not secondary to aphasia are given a 1. It is important that only the initial answer be graded and that the examiner not "help" the patient with verbal or non-verbal cues. 1c. Level of consciousness commands: The 0 = Performs both tasks correctly. _____ patient is asked to open and close the eyes 1 = Performs one task correctly. and then to grip and release the non-paretic hand. Substitute another one step 2 = Performs neither task correctly. command if the hands cannot be used. Credit is given if an unequivocal attempt is made but not completed due to weakness. If the patient does not respond to command, https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 22/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate the task should be demonstrated to him or her (pantomime), and the result scored (ie, follows none, one or two commands). Patients with trauma, amputation, or other physical impediments should be given suitable one-step commands. Only the first attempt is scored. 2. Best gaze: Only horizontal eye 0 = Normal. movements will be tested. Voluntary or reflexive (oculocephalic) eye movements will 1 = Partial gaze palsy; gaze is abnormal in one or both eyes, but forced deviation or total gaze paresis is not present. be scored, but caloric testing is not done. If the patient has a conjugate deviation of the 2 = Forced deviation, or total gaze paresis not overcome by the oculocephalic eyes that can be overcome by voluntary or reflexive activity, the score will be 1. If a maneuver. patient has an isolated peripheral nerve paresis (cranial nerves III, IV or VI), score a 1. Gaze is testable in all aphasic patients. Patients with ocular trauma, bandages, pre- _____ existing blindness, or other disorder of visual acuity or fields should be tested with reflexive movements, and a choice made by the investigator. Establishing eye contact and then moving about the patient from side to side will occasionally clarify the presence of a partial gaze palsy. 3. Visual: Visual fields (upper and lower quadrants) are tested by confrontation, 0 = No visual loss. 1 = Partial hemianopia. using finger counting or visual threat, as appropriate. Patients may be encouraged, but if they look at the side of the moving fingers appropriately, this can be scored as normal. If there is unilateral blindness or 2 = Complete hemianopia. 3 = Bilateral hemianopia (blind including cortical blindness). enucleation, visual fields in the remaining eye are scored. Score 1 only if a clear-cut _____ asymmetry, including quadrantanopia, is found. If patient is blind from any cause, score 3. Double simultaneous stimulation is performed at this point. If there is extinction, patient receives a 1, and the results are used to respond to item 11. 4. Facial palsy: Ask - or use pantomime to encourage - the patient to show teeth or 0 = Normal symmetrical movements. _____ 1 = Minor paralysis (flattened nasolabial raise eyebrows and close eyes. Score fold, asymmetry on smiling). symmetry of grimace in response to noxious stimuli in the poorly responsive or non- https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 23/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate comprehending patient. If facial 2 = Partial paralysis (total or near-total trauma/bandages, orotracheal tube, tape or paralysis of lower face). other physical barriers obscure the face, these should be removed to the extent 3 = Complete paralysis of one or both sides (absence of facial movement in the upper and lower face). possible. 5. Motor arm: The limb is placed in the 0 = No drift; limb holds 90 (or 45) degrees appropriate position: extend the arms for full 10 seconds. (palms down) 90 degrees (if sitting) or 45 degrees (if supine). Drift is scored if the arm 1 = Drift; limb holds 90 (or 45) degrees, but drifts down before full 10 seconds; does not falls before 10 seconds. The aphasic patient is encouraged using urgency in the voice hit bed or other support. 2 = Some effort against gravity; limb cannot get to or maintain (if cued) 90 (or 45) and pantomime, but not noxious stimulation. Each limb is tested in turn, degrees, drifts down to bed, but has some effort against gravity. beginning with the non-paretic arm. Only in _____ the case of amputation or joint fusion at the shoulder, the examiner should record the 3 = No effort against gravity; limb falls. score as untestable (UN), and clearly write the explanation for this choice. 4 = No movement. UN = Amputation or joint fusion, explain:________________ 5a. Left arm 5b. Right arm 6. Motor leg: The limb is placed in the appropriate position: hold the leg at 30 degrees (always tested supine). Drift is scored if the leg falls before 5 seconds. The aphasic patient is encouraged using urgency in the voice and pantomime, but not 0 = No drift; leg holds 30-degree position for full 5 seconds. 1 = Drift; leg falls by the end of the 5-second period but does not hit bed. 2 = Some effort against gravity; leg falls to bed by 5 seconds, but has some effort against gravity. noxious stimulation. Each limb is tested in turn, beginning with the non-paretic leg. Only in the case of amputation or joint _____ 3 = No effort against gravity; leg falls to fusion at the hip, the examiner should record the score as untestable (UN), and bed immediately. 4 = No movement. clearly write the explanation for this choice. UN = Amputation or joint fusion, explain:________________ 6a. Left leg 6b. Right leg 7. Limb ataxia: This item is aimed at finding evidence of a unilateral cerebellar lesion. 0 = Absent. _____ 1 = Present in one limb. Test with eyes open. In case of visual defect, 2 = Present in two limbs. ensure testing is done in intact visual field. The finger-nose-finger and heel-shin tests https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 24/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate are performed on both sides, and ataxia is UN = Amputation or joint fusion, scored only if present out of proportion to explain:________________ weakness. Ataxia is absent in the patient who cannot understand or is paralyzed. Only in the case of amputation or joint fusion, the examiner should record the score as untestable (UN), and clearly write the explanation for this choice. In case of blindness, test by having the patient touch nose from extended arm position. 8. Sensory: Sensation or grimace to pinprick when tested, or withdrawal from noxious 0 = Normal; no sensory loss. 1 = Mild-to-moderate sensory loss; patient feels pinprick is less sharp or is dull on the stimulus in the obtunded or aphasic patient. Only sensory loss attributed to stroke is affected side; or there is a loss of superficial scored as abnormal and the examiner pain with pinprick, but patient is aware of being touched. should test as many body areas (arms [not hands], legs, trunk, face) as needed to accurately check for hemisensory loss. A 2 = Severe to total sensory loss; patient is not aware of being touched in the face, arm, and leg. score of 2, "severe or total sensory loss," should only be given when a severe or total loss of sensation can be clearly demonstrated. Stuporous and aphasic patients will, therefore, probably score 1 or 0. The patient with brainstem stroke who has bilateral loss of sensation is scored 2. If _____ the patient does not respond and is quadriplegic, score 2. Patients in a coma (item 1a=3) are automatically given a 2 on this item. 9. Best language: A great deal of information about comprehension will be obtained during the preceding sections of 0 = No aphasia; normal. _____ 1 = Mild-to-moderate aphasia; some obvious loss of fluency or facility of comprehension, without significant the examination. For this scale item, the patient is asked to describe what is limitation on ideas expressed or form of happening in the attached picture, to name the items on the attached naming sheet and expression. Reduction of speech and/or comprehension, however, makes to read from the attached list of sentences. conversation about provided materials difficult or impossible. For example, in Comprehension is judged from responses here, as well as to all of the commands in conversation about provided materials, examiner can identify picture or naming the preceding general neurological exam. If visual loss interferes with the tests, ask the card content from patient's response. patient to identify objects placed in the hand, repeat, and produce speech. The 2 = Severe aphasia; all communication is through fragmentary expression; great need for inference, questioning, and guessing by intubated patient should be asked to write. The patient in a coma (item 1a=3) will automatically score 3 on this item. The the listener. Range of information that can https://www.uptodate.com/contents/malignant-cerebral-hemispheric-infarction-with-swelling-and-risk-of-herniation/print 25/39 7/6/23, 12:02 PM Malignant cerebral hemispheric infarction with swelling and risk of herniation - UpToDate examiner must choose a score for the be exchanged is limited; listener carries patient with stupor or limited cooperation, burden of communication. Examiner cannot but a score of 3 should be used only if the patient is mute and follows no one-step identify materials provided from patient response. commands. 3 = Mute, global aphasia; no usable speech or auditory comprehension. 10. Dysarthria: If patient is thought to be 0 = Normal. normal, an adequate sample of speech must be obtained by asking patient to read or 1 = Mild-to-moderate dysarthria; patient slurs at least some words and, at worst, can repeat words from the attached list. If the patient has severe aphasia, the clarity of be understood with some difficulty. 2 = Severe dysarthria; patient's speech is so slurred as to be unintelligible in the absence articulation of spontaneous speech can be rated. Only if the patient is intubated or has _____ of or out of proportion to any dysphasia, or is mute/anarthric. other physical barriers to producing speech, the examiner should record the score as untestable (UN), and clearly write an UN = Intubated or other physical barrier, explain:________________ explanation for this choice. Do not tell the patient why he or she is being tested.