The recent genomic and transcriptomic characterization of human craniopharyngiomas has provided important insights into the pathogenesis of these tumors and supports that these tumor types are distinct entities. Critically, the insights provided by these data offer the potential for the introduction of novel therapies and surgical treatment paradigms for these tumors, which are associated with high morbidity rates and morbid conditions. Mutations in the CTNNB1 gene are primary drivers of adamantinomatous craniopharyngioma (ACP) and lead to the accumulation of β-catenin protein in a subset of the nuclei within the neoplastic epithelium of these tumors. Dysregulation of epidermal growth factor receptor (EGFR) and of sonic hedgehog (SHH) signaling in ACP suggest that paracrine oncogenic mechanisms may underlie ACP growth and implicate these signaling pathways as potential targets for therapeutic intervention using directed therapies. Recent work shows that ACP cells have primary cilia, further supporting the potential importance of SHH signaling in the pathogenesis of these tumors. While further preclinical data are needed, directed therapies could defer, or replace, the need for radiation therapy and/or allow for less aggressive surgical interventions. Furthermore, the prospect for reliable control of cystic disease without the need for surgery now exists. Studies of papillary craniopharyngioma (PCP) are more clinically advanced than those for ACP. The vast majority of PCPs harbor the BRAF v600e mutation. There are now 2 reports of patients with PCP that had dramatic therapeutic responses to targeted agents. Ongoing clinical and research studies promise to not only advance our understanding of these challenging tumors but to offer new approaches for patient management.
Leslie C. Robinson, Sandro Santagata and Todd C. Hankinson
Matthew R. Fusco, Todd C. Hankinson and Curtis J. Rozzelle
Occipitoatlantoaxial rotatory fixation (OAARF) is a rare condition involving fixed rotational subluxation of the atlas in relation to both the occiput and axis. Atlantoaxial rotatory fixation (AARF) appears to precede OAARF in most cases, as untreated AARF may cause compensatory counter-rotation and occipitoaxial fixation at an apparently neutral head position. We report a case of OAARF in an 8-year-old girl with juvenile idiopathic arthritis. Cervical imaging demonstrated slight rightward rotation of the occiput at 7.63° in relation to C-2 and significant rightward rotation of C-1 at 65.90° in relation to the occiput and at 73.53° in relation to C-2. An attempt at closed reduction with halo traction was unsuccessful. Definitive treatment included open reduction, C-1 laminectomy, and occipitocervical internal fixation and fusion.
Monica Salazar Davern, Sumeet Garg and Todd C. Hankinson
This report describes the presentation and operative treatment of a 3-year-old boy who survived a motor vehicle accident that resulted in a C6–7 distraction injury, complete avulsion of the spinal cord, and gross spinal instability. Only 5%–10% of all spinal cord and vertebral column injuries occur in children. Survival after such an injury is exceptionally rare in very young patients and is associated with severe neurological deficits. The authors discuss the substantial ethical challenges involved in the care of a patient with this injury. To their knowledge, only two other cases of survival have been reported in pediatric patients following motor vehicle trauma resulting in complete injury to the lower cervical spinal cord.
Todd C. Hankinson, Elizabeth J. Fontana, Richard C. E. Anderson and Neil A. Feldstein
The traditional reasons for surgical intervention in children with single-suture craniosynostosis (SSC) are cosmetic improvement and the avoidance/treatment of intracranial hypertension, which has been thought to contribute to neurocognitive deficits. Despite considerable work on the topic, the exact prevalence of intracranial hypertension in the population of patients with SSC is unknown, although it appears to be present in only a minority. Additionally, recent neuropsychological and anatomical literature suggests that the subtle neurocognitive deficits identified in children with a history of SSC may not result from external compression. They may instead reflect an underlying developmental condition that includes disordered primary CNS development and early suture fusion. This implies that current surgical techniques are unlikely to prevent neurocognitive deficits in patients with SSC. As such, the most common indication for surgical treatment in SSC is cosmetic, and most patients benefit from considerable subjective cosmetic normalization following surgery. Pediatric craniofacial surgeons have not, however, agreed upon objective means to assess postoperative cranial morphological improvement. We should therefore endeavor to agree upon objective craniometric tools for the assessment of operative outcomes, allowing us to accurately compare the various surgical techniques that are currently available.
Todd C. Hankinson, Eli Grunstein, Paul Gardner, Theodore J. Spinks and Richard C. E. Anderson
In rare cases, children with a Chiari malformation Type I (CM-I) suffer from concomitant, irreducible, ventral brainstem compression that may result in cranial neuropathies or brainstem dysfunction. In these circumstances, a 360° decompression supplemented by posterior stabilization and fusion is required. In this report, the authors present the first experience with using an endoscopic transnasal corridor to accomplish ventral decompression in children with CM-I that is complicated by ventral brainstem compression.
Two children presented with a combination of occipital headaches, swallowing dysfunction, myelopathy, and/or progressive scoliosis. Imaging studies demonstrated CM-I with severely retroflexed odontoid processes and ventral brainstem compression. Both patients underwent an endoscopic transnasal approach for ventral decompression, followed by posterior decompression, expansive duraplasty, and occipital-cervical fusion.
In both patients the endoscopic transnasal approach provided excellent ventral access to decompress the brainstem. When compared with the transoral approach, endoscopic transnasal access presents 4 potential advantages: 1) excellent prevertebral exposure in patients with small oral cavities; 2) a surgical corridor located above the hard palate to decompress rostral pathological entities more easily; 3) avoidance of the oral trauma and edema that follows oral retractor placement; and 4) avoidance of splitting the soft or hard palate in patients with oral-palatal dysfunction from ventral brainstem compression.
The endoscopic transnasal approach is atraumatic to the oral cavity, and offers a more superior region of exposure when compared with the standard transoral approach. Depending on their comfort level with endoscopic surgical techniques, pediatric neurosurgeons should consider this approach in children with pathological entities requiring ventral brainstem decompression.
Omar N. Syed, Todd C. Hankinson, William J. Mack, Neil A. Feldstein and Richard C. E. Anderson
Pediatric neurosurgeons frequently care for children with traumatic scalp and skull injury. Foreign objects are often observed on imaging and may influence the clinician's decision-making process. The authors report on 2 cases of poorly visualized hair beads that had become embedded into the skull during blunt trauma. In both cases, skull radiography and CT scanning demonstrated depressed, comminuted fractures with poorly demonstrated spherical radiolucencies in the overlying scalp. The nature of these objects was initially unclear, and they could have represented air that entered the scalp during trauma. In one case, scalp inspection demonstrated no evidence of the bead. In the other case, a second bead was observed at the site of scalp laceration. In both cases, the beads were surgically removed, the fractures were elevated, and the patients recovered uneventfully. Radiolucent fashion accessories, such as hair beads, may be difficult to appreciate on clinical examination and may masquerade as clinically insignificant air following cranial trauma. If they are not removed, these foreign bodies may pose the risk of an infection. Pediatric neurosurgeons should consider hair accessories in the differential diagnosis of foreign bodies that may produce skull fracture following blunt trauma.
R. Michael Scott
Todd C. Hankinson, J Mocco, Brent Kimball, Richard C. E. Anderson and Neil A. Feldstein
✓The authors describe the internal cranial expansion (ICE) procedure, a surgical technique that was used to treat two chronically shunt-treated children who presented with medically and surgically refractory intracranial hypertension despite the presence of functioning cerebrospinal fluid shunt systems. The ICE procedure was used as a means to increase intracranial volume without sacrificing calvarial rigidity. Intracranial volume was increased by 5% in one case and 10% in the other. Both patients have returned to their neurological and functional baselines, and they are free of symptoms related to intracranial hypertension.
Todd C. Hankinson, Elizabeth J. Campagna, Nicholas K. Foreman and Michael H. Handler
The current management paradigm for children with newly diagnosed diffuse intrinsic pontine glioma (DIPG) is to establish a diagnosis and begin therapy based on MR imaging findings correlated with an appropriate clinical presentation, and without a tissue diagnosis. This strategy assumes that pediatric neurosurgeons and neurooncologists uniformly interpret MR imaging findings in this population. This study sought to examine the consistency of North American pediatric neurosurgeons in assessing MR images in this patient population, and in their surgical plans based on the interpretation of those images.
The authors created an online survey and invited all members of the American Society of Pediatric Neurosurgeons and the American Association of Neurological Surgeons/Congress of Neurological Surgeons Joint Section for Pediatric Neurosurgery to participate. The survey consisted of 58 questions, and 48 pertained to representative MR images from 16 children who presented to The Children's Hospital of Colorado with diffuse pontine tumors. Based on the imaging presented and a standard clinical scenario, the respondent was asked if she or he believed a lesion to be “typical” or “atypical,” whether she or he would biopsy the lesion, and what surgical approach would be chosen. The remaining 10 questions pertained to respondent demographics and his or her practice regarding tissue preservation and interest in participating in a multicenter trial that included tumor biopsy in selected cases. Rates at which each lesion was considered to be typical or atypical and rates of recommended biopsy were calculated.
Surveys were received by 269 individuals. Eighty-six responses were received (32.0%). No tumor was judged to be either typical or atypical by all respondents. Individual surgeons varied widely regarding how many of the tumors were judged as typical or warranted a biopsy. The percentage of respondents who disagreed with the majority opinion regarding whether a tumor qualified as typical ranged from 2.3% to 48.8%, with a median of 28.6%. More than 75% agreement regarding whether a tumor was typical or atypical was found in 7 (43.8%) of 16 cases. The κ statistic regarding typicality was 0.297 ± 0.0004 (mean ± SEM), implying only fair agreement. For every tumor, at least 1 respondent who believed the lesion to be typical in appearance would nevertheless biopsy the lesion. Of those respondents who considered a lesion to be typical, a median of 5.1% (range 1.2%–66.7%) would choose to biopsy this lesion. Of those respondents who considered a lesion atypical, a median of 18.3% (range 3.7%–100%) would choose not to biopsy the lesion. Of 85 responses to the question, “Would you be willing to biopsy a typical diffuse pontine glioma as a part of a multicenter trial?,” 59 (69.4%) of 85 respondents answered that they would.
Although making a diagnosis based on radiographic evidence alone represents a well-established management paradigm for children with suspected DIPG, this study demonstrates considerable inconsistency on the part of pediatric neurosurgeons in the application of this strategy to individual patients. As such, the practice of diagnosing DIPG based on imaging characteristics and clinical history alone does not reach the appropriate threshold to be considered a standard of care.
Seerat Poonia, Sarah Graber, C. Corbett Wilkinson, Brent R. O'neill, Michael H. Handler and Todd C. Hankinson
Postoperative management following the release of simple spinal cord–tethering lesions is highly variable. As a quality improvement initiative, the authors aimed to determine whether an institutional protocol of discharging patients on postoperative day (POD) 1 was associated with a higher rate of postoperative CSF leaks than the prior protocol of discharge on POD 2.
This was a single-center retrospective review of all children who underwent release of a spinal cord–tethering lesion that was not associated with a substantial fascial or dural defect (i.e., simple spinal cord detethering) during 2 epochs: prior to and following the institution of a protocol for discharge on POD 1. Outcomes included the need for and timing of nonroutine care of the surgical site, including return to the operating room, wound suturing, and nonsurgical evaluation and management.
Of 169 patients identified, none presented with CSF-related complications prior to discharge. In the preintervention group (n = 113), the postoperative CSF leak rate was 4.4% (5/113). The mean length of stay was 2.3 days. In the postintervention group, the postoperative CSF leak rate was 1.9% (1/53) in the patients with postdischarge follow-up. The mean length of stay in that group was 1.3 days.
At a single academic children's hospital, a protocol of discharging patients on POD 1 following uncomplicated release of a simple spinal cord–tethering lesion was not associated with an increased rate of postoperative CSF leaks, relative to the previous protocol. The rates identified are consistent with the existing literature. The authors' practice has changed to discharge on POD 1 in most cases.