✓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, J Mocco, Brent Kimball, Richard C. E. Anderson and Neil A. Feldstein
Todd C. Hankinson, Leif-Erik Bohman, Geoffrey Heyer, Maureen Licursi, Saadi Ghatan, Neil A. Feldstein and Richard C. E. Anderson
Children with sickle cell anemia (SCA) and moyamoya syndrome carry a significant risk of ischemic stroke. Given the success of encephaloduroarteriosynangiosis (EDAS) or pial synangiosis in the treatment of moyamoya disease, the purpose of this study was to examine whether it reliably and durably protected children with SCA and moyamoya syndrome against cerebrovascular complications.
The authors retrospectively reviewed a series of 12 patients with SCA who developed clinical and/or radiological evidence of moyamoya syndrome and underwent EDAS.
Eleven patients (92%) presented following a cerebrovascular accident (CVA), transient ischemic attack (TIA), or seizure. Magnetic resonance (MR) imaging or angiography suggested moyamoya vascular changes, and cerebral angiography confirmed the diagnosis in all 12 patients. At the time of surgery, the median age was 12.3 years (range 6.8–19.4 years). Ten (83%) of 12 patients had a history of CVA, and 4 of these patients were compliant with a transfusion protocol at the time of their CVA. Bilateral (7 patients) or unilateral (5 patients) EDAS was performed without complications. The mean follow-up period was 46.8 months (range 8.1–106 months). During the follow-up period, 2 patients (16.7%) suffered cerebrovascular events. One patient, who was stroke-free preoperatively, suffered a CVA 3 weeks after the procedure. The other patient suffered a single left lower-extremity TIA 18 months following right-sided EDAS. She returned to her neurological baseline condition and remains stable 53 months postoperatively. Seven patients underwent follow-up angiography or MR angiography, and evidence of revascularization was noted in all cases. At this time, no patient has developed progressive disease requiring a contralateral procedure after unilateral EDAS.
The EDAS procedure is a safe and effective treatment option in patients with SCA who develop moyamoya syndrome.
Todd C. Hankinson, Alfred T. Ogden, Peter Canoll, James H. Garvin, Michael Kazim, Jeffrey N. Bruce, Neil A. Feldstein and Richard C. E. Anderson
✓ Soft-tissue glomus tumors (or glomangiomas) are unrelated to neuroendocrine paragangliomas (glomus tympanicum, jugulare, and vagale). The authors present the first reported case of an orbital soft-tissue glomus tumor in a child. An 8-year-old girl developed rapidly progressive right-eye blindness, proptosis, and a sixth cranial nerve palsy. Magnetic resonance imaging demonstrated a homogeneously enhancing lesion extending from the right orbit through the superior orbital fissure to the cavernous sinus and middle cranial fossa. A biopsy specimen demonstrated the lesion to be a soft-tissue glomus tumor. Following angiography and embolization, a gross-total resection of the tumor was achieved. The patient was treated with adjuvant proton-beam radiotherapy. At 24 months follow-up her proptosis and sixth cranial nerve palsy had resolved and there was no evidence of tumor recurrence.
R. Michael Scott
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.
Todd C. Hankinson, Monique Vanaman, Peter Kan, Sherelle Laifer-Narin, Robert DeLaPaz, Neil Feldstein and Richard C. E. Anderson
Pediatric neurosurgeons are increasingly called on to provide prognostic data regarding the antenatal diagnosis of ventriculomegaly. This study was designed to determine if there is a correlation between prenatal MR imaging results and the need for ventricular shunt placement during the neonatal period.
The authors retrospectively reviewed the prenatal MR imaging data of 38 consecutive patients who had been referred for neurosurgical consultation following the diagnosis of ventriculomegaly. The outcome measure was placement of a ventricular shunt. Assessed parameters included prenatal atrial diameter (AD), gestational age at MR imaging, time between imaging studies, presence of concomitant CNS anomalies, laterality of ventriculomegaly, fetal sex, and temporal evolution of ventriculomegaly. Logistic regression analysis was completed with the calculation of appropriate ORs and 95% CIs.
Six patients (16%) required shunt placement, all with an AD ≥ 20 mm (mean 23.8 mm) at the time of imaging. Eight patients had presented with an AD ≥ 20 mm. Atrial diameter was the only presenting feature that correlated with shunt placement (OR 1.58, 95% CI 1.10–2.25, p = 0.01). Logistic regression analysis revealed no statistical correlation between the need for ventricular shunting and gestational age at MR imaging, time between imaging studies, fetal sex, presence of additional CNS anomalies, and laterality of the ventriculomegaly.
When assessed using MR imaging, an AD ≥ 20 mm at any gestational age is highly associated with the need for postnatal shunting. Patients with concomitant CNS anomalies did not require shunts at a greater rate than those with isolated ventriculomegaly. Further studies are required to assess the long-term outcome of this patient population.
Todd C. Hankinson, Anthony M. Avellino, David Harter, Andrew Jea, Sean Lew, David Pincus, Mark R. Proctor, Luis Rodriguez, David Sacco, Theodore Spinks, Douglas L. Brockmeyer and Richard C. E. Anderson
The object of this study was to assess a multiinstitutional experience with pediatric occipitocervical constructs to determine whether a difference exists between the fusion and complication rates of constructs with or without direct C-1 instrumentation.
Seventy-seven cases of occiput-C2 instrumentation and fusion, performed at 9 children's hospitals, were retrospectively analyzed. Entry criteria included atlantooccipital instability with or without atlantoaxial instability. Any case involving subaxial instability was excluded. Constructs were divided into 3 groups based on the characteristics of the anchoring spinal instrumentation: Group 1, C-2 instrumentation; Group 2, C-1 and C-2 instrumentation without transarticular screw (TAS) placement; and Group 3, any TAS placement. Groups were compared based on rates of fusion and perioperative complications.
Group 1 consisted of 16 patients (20.8%) and had a 100% rate of radiographically demonstrated fusion. Group 2 included 22 patients (28.6%), and a 100% fusion rate was achieved, although 2 cases were lost to follow-up before documented fusion. Group 3 included 39 patients (50.6%) and demonstrated a 100% radiographic fusion rate. Complication rates were 12.5, 13.7, and 5.1%, respectively. There were 3 vertebral artery injuries, 1 (4.5%) in Group 2 and 2 (5.1%) in Group 3.
High fusion rates and low complication rates were achieved with each configuration examined. There was no difference in fusion rates between the group without (Group 1) and those with (Groups 2 and 3) C-1 instrumentation. These findings indicated that in the pediatric population, excellent occipitocervical fusion rates can be accomplished without directly instrumenting C-1.
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.
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.
Report of 4 cases
R. Shane Tubbs, Nadine Bradley, Daniel Harmon, Todd C. Hankinson, David R. Kelly and John C. Wellons III
Lymphatic malformations that involve the nervous system are uncommon. The authors review their experience with involvement of the brachial plexus and its branches by cystic hygromas.
A retrospective review of the authors' experience with pathology of the pediatric brachial plexus revealed 4 cases involving patients with compression of this structure and its branches due to cystic hygroma. Although such cases are apparently rare, the neurosurgeon should consider malformations of the lymphatic system in the differential diagnosis of masses involving the brachial plexus and its branches.