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Simon G. Heuts, Samuel S. Bruce, Brad E. Zacharia, Zachary L. Hickman, Christopher P. Kellner, Eric S. Sussman, Michael M. McDowell, Rachel A. Bruce and E. Sander Connolly Jr.

Object

Large intracerebral hemorrhage (ICH), compounded by perihematomal edema, can produce severe elevations of intracranial pressure (ICP). Decompressive hemicraniectomy (DHC) with or without clot evacuation has been considered a part of the armamentarium of treatment options for these patients. The authors sought to assess the preliminary utility of DHC without evacuation for ICH in patients with supratentorial, dominant-sided lesions.

Methods

From September 2009 to May 2012, patients with ICH who were admitted to the neurological ICU at Columbia University Medical Center were prospectively enrolled in that institution's ICH Outcomes Project (ICHOP). Five patients with spontaneous supratentorial dominant-sided ICH underwent DHC without clot evacuation for recalcitrant elevated ICP. Data pertaining to the patients' characteristics and outcomes of treatment were prospectively collected.

Results

The patients' median age was 43 years (range 30–55 years) and the ICH etiology was hypertension in 4 of 5 patients, and systemic lupus erythematosus vasculitis in 1 patient. On admission, the median Glasgow Coma Scale (GCS) score was 7 (range 5–9). The median ICH volume was 53 cm3 (range 28–79 cm3), and the median midline shift was 7.6 mm (range 3.0–11.3 mm). One day after surgery, the median decrease in midline shift was 2.7 mm (range 1.5–4.6 mm), and the median change in GCS score was +1 (range −3 to +5). At discharge, all patients were still alive, and the median GCS score was 10 (range 9–11), the median modified Rankin Scale (mRS) score was 5 (range 5–5), and the median NIHSS (National Institutes of Health Stroke Scale) score was 22 (range 17–27). Six months after hemorrhage, 1 patient had died, 2 were functionally dependent (mRS Score 4–5), and 2 were functionally independent (mRS Score 0–3). Outcomes for the patients treated with DHC were good compared with 1) outcomes for all patients with spontaneous supratentorial ICH admitted during the same period (n = 144) and 2) outcomes for matched patients (dominant ICH, GCS Score 5–9, ICH volume 28–79 cm3, age < 60 years) whose cases were managed nonoperatively (n = 5).

Conclusions

Decompressive hemicraniectomy without clot evacuation appears feasible in patients with large ICH and deserves further investigation, preferably in a randomized controlled setting.

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Eric S. Sussman, Christopher P. Kellner, Michael M. McDowell, Samuel S. Bruce, Simon G. Heuts, Zong Zhuang, Rachel A. Bruce, Jan Claassen and E. Sander Connolly Jr.

Intracerebral hemorrhage (ICH) is the most deadly and least treatable subtype of stroke, and at the present time there are no evidence-based therapeutic interventions for patients with this disease. Secondary injury mechanisms are known to cause substantial rates of morbidity and mortality following ICH, and the inflammatory cascade is a major contributor to this post-ICH secondary injury. The alpha-7 nicotinic acetylcholine receptor (α7-nAChR) agonists have a well-established antiinflammatory effect and have been shown to attenuate perihematomal edema volume and to improve functional outcome in experimental ICH. The authors evaluate the current evidence for the use of an α7-nAChR agonist as a novel therapeutic agent in patients with ICH.

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Michael M. McDowell, Simon J. Hanft, Sophie A. Greenberg, Rahmatullah Rahmati, Vincent Carrao, Sidney Eisig and Richard C. E. Anderson

The authors report on the surgical management of an extensive lesion of the upper cervical spine that required an uncommon transmandibular approach to facilitate exposure, resection, and stabilization in a pediatric patient. A 6-year-old boy with a large aneurysmal bone cyst of the C-2 vertebra presented with progressive weakness and right-sided neck pain. The lesion extended laterally into the soft tissue of the neck, inferiorly to C-4, and posteriorly around the spinal cord. A transmandibular osteotomy was performed to provide adequate exposure for complete resection of the mass and anterior C1–3 instrumentation and fusion. Subsequently, the patient underwent occiput to C-4 posterior instrumentation and fusion. The patient tolerated the operation well and had regained all function at 3 and 11 months' follow-up. No neurological complications or problems of speech, swallowing, or respiration occurred. Even in pediatric patients, the transmandibular approach for the treatment of upper cervical spine lesions is an effective method of maximizing exposure for complex lesions requiring resection and stabilization.

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Amit Ayer, Alexander Campbell, Geoffrey Appelboom, Brian Y. Hwang, Michael McDowell, Matthew Piazza, Neil A. Feldstein and Richard C. E. Anderson

In this report, the evidence, mechanisms, and rationale for the practice of artificial cranial deformation (ACD) in ancient Peru and during Akhenaten's reign in the 18th dynasty in Egypt (1375–1358 BCE) are reviewed. The authors argue that insufficient attention has been given to the sociopolitical implications of the practice in both regions. While evidence from ancient Peru is widespread and complex, there are comparatively fewer examples of deformed crania from the period of Akhenaten's rule. Nevertheless, Akhenaten's own deformity, the skull of the so-called “Younger Lady” mummy, and Tutankhamen's skull all evince some degree of plagiocephaly, suggesting the need for further research using evidence from depictions of the royal family in reliefs and busts. Following the anthropological review, a neurosurgical focus is directed to instances of plagiocephaly in modern medicine, with special attention to the conditions' etiology, consequences, and treatment. Novel clinical studies on varying modes of treatment will also be studied, together forming a comprehensive review of ACD, both in the past and present.

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Benjamin C. Kennedy, Taylor B. Nelp, Kathleen M. Kelly, Michelle Q. Phan, Samuel S. Bruce, Michael M. McDowell, Neil A. Feldstein and Richard C. E. Anderson

OBJECT

Chiari malformation Type I (CM-I) is associated with a syrinx in 25%–85% of patients. Although posterior fossa decompression (PFD) without dural opening is an accepted treatment option for children with symptomatic CM-I, many surgeons prefer to open the dura if a syrinx exists. The purpose of this study was to investigate the frequency and timing of syrinx resolution in children undergoing PFD without dural opening for CM-I.

METHODS

A retrospective review of 68 consecutive pediatric patients with CM-I and syringomyelia who underwent PFD without dural opening was conducted. Patient demographics, presenting symptoms and signs, radiographic findings, and intraoperative ultrasound and neuromonitoring findings were studied as well as the patients’ clinical and radiographic follow-up.

RESULTS

During the mean radiographic follow-up period of 32 months, 70% of the syringes improved. Syrinx improvement occurred at a mean of 31 months postoperatively. All patients experienced symptom improvement within the 1st year, despite only 26% of patients showing radiographic improvement during that period. Patients presenting with sensory symptoms or motor weakness had a higher likelihood of having radiographic syrinx improvement postoperatively.

CONCLUSIONS

In children with CM-I and a syrinx undergoing PFD without dural opening, syrinx resolution occurs in approximately 70% of patients. Radiographic improvement of the syrinx is delayed, but this does not correlate temporally with symptom improvement. Sensory symptoms or motor weakness on presentation are associated with syrinx resolution after surgery.

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Eric Reseland, Nitin Agarwal, Michael M. McDowell, Jeremy G. Stone, Daniel A. Tonetti, Issam A. Awad, Charles J. Hodge, Karen S. Koenig, Allan H. Friedman and Robert M. Friedlander

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Benjamin C. Kennedy, Randy S. D’Amico, Brett E. Youngerman, Michael M. McDowell, Kristopher G. Hooten, Daniel Couture, Andrew Jea, Jeffrey Leonard, Sean M. Lew, David W. Pincus, Luis Rodriguez, Gerald F. Tuite, Michael L. Diluna, Douglas L. Brockmeyer, Richard C. E. Anderson and Pediatric Craniocervical Society

OBJECT

The long-term consequences of atlantoaxial (AA) and occipitocervical (OC) fusion and instrumentation in young children are unknown. Anecdotal reports have raised concerns regarding altered growth and alignment of the cervical spine after surgical intervention. The purpose of this study was to determine the long-term effects of these surgeries on the growth and alignment of the maturing spine.

METHODS

A multiinstitutional retrospective chart review was conducted for patients less than or equal to 6 years of age who underwent OC or AA fusion with rigid instrumentation at 9 participating centers. All patients had at least 3 years of clinical and radiographic follow-up data and radiographically confirmed fusion. Preoperative, immediate postoperative, and most recent follow-up radiographs and/or CT scans were evaluated to assess changes in spinal growth and alignment.

RESULTS

Forty children (9 who underwent AA fusion and 31 who underwent OC fusion) were included in the study (mean follow-up duration 56 months). The mean vertical growth over the fused levels in the AA fusion patients represented 30% of the growth of the cervical spine (range 10%–50%). Three different vertical growth patterns of the fusion construct developed among the 31 OC fusion patients during the follow-up period: 1) 16 patients had substantial growth (13%–46% of the total growth of the cervical spine); 2) 9 patients had no meaningful growth; and 3) 6 patients, most of whom presented with a distracted atlantooccipital dislocation, had a decrease in the height of the fused levels (range 7–23 mm). Regarding spinal alignment, 85% (34/40) of the patients had good alignment at follow-up, with straight or mildly lordotic cervical curvatures. In 1 AA fusion patient (11%) and 5 OC fusion patients (16%), we observed new hyperlordosis (range 43°–62°). There were no cases of new kyphosis or swan-neck deformity, evidence of subaxial instability, or unintended subaxial fusion. No preoperative predictors of these growth patterns or alignment were evident.

CONCLUSIONS

These results demonstrate that most young children undergoing AA and OC fusion with rigid internal fixation continue to have good cervical alignment and continued growth within the fused levels during a prolonged follow-up period. However, some variability in vertical growth and alignment exists, highlighting the need to continue close long-term follow-up.