Faculty Bibliography

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.

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.

OBJECT/OBJECTIVE: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 degrees 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. METHODS: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. RESULTS: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. CONCLUSIONS: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.

OBJECT: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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

OBJECT/OBJECTIVE:Cervical spine clearance after trauma in children 0-3 years of age is deceptively difficult. Young children may not be able to communicate effectively, and severe injuries may require intubation and sedation. Currently, no published guidelines are available to aid in decision-making in these complex situations. The purpose of this study was to determine whether a safe and effective protocol-driven system could be developed for clearance of the cervical spine in noncommunicative children between 0 and 3 years of age. METHODS:Children 0-3 years of age, including intubated patients, who were admitted after trauma activation at Primary Children's Medical Center in Salt Lake City or the Children's Hospital of New York from 2002 to 2006 were managed according to a cervical spine clearance protocol. Data were collected in a prospective fashion. Radiographic and clinical methods of clearing the cervical spine, as well as the type and management of injuries, were recorded. RESULTS:A total of 2828 pediatric trauma activations required cervical spine clearance during the study period. Of these, 575 (20%) were children <or= 3 years of age who were admitted to the hospital. To facilitate clearing the cervical spine in these children, plain radiographs (100%), CT studies (14%), and MR images (10%) were obtained. Nineteen ligamentous injuries (3.3%) and 9 fractures/dislocations (1.5%) were detected, with 4 patients requiring operative stabilization (0.7%). No late injuries have been detected. CONCLUSIONS:The protocol used has been effective in detecting cervical spine injuries in noncommunicative children after trauma. The combination of clinical information and radiographic studies is essential for safely clearing the cervical spine in these complex situations. Clearance of the cervical spine without CT or MR imaging studies is possible in the majority of cases, even in very young patients.

OBJECT/OBJECTIVE: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. METHODS: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. RESULTS:Six patients (16%) required shunt placement, all with an AD > or = 20 mm (mean 23.8 mm) at the time of imaging. Eight patients had presented with an AD > or = 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. CONCLUSIONS:When assessed using MR imaging, an AD > or = 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.

Germ cell tumors are a heterogeneous group of neoplasms derived from residual primordial tissue. These tumors are commonly found in the brain, testes, or ovaries, where they are termed germinomas, seminomas, or dysgerminomas, respectively. Like several other tumor types, germ cell tumors often harbor an immune cell infiltrate that can include substantial numbers of B cells. Yet little is known about whether the humoral immune response affects germ cell tumor biology. To gain a deeper understanding of the role B cells play in this tumor family, we characterized the immune cell infiltrate of all three germ cell tumor subtypes and defined the molecular characteristics of the B cell Ag receptor expressed by tumor-associated B cells. Immunohistochemistry revealed a prominent B cell infiltrate in the microenvironment of all tumors examined and clear evidence of extranodal lymphoid follicles with germinal center-like architecture in a subset of specimens. Molecular characterization of the Ig variable region from 320 sequences expressed by germ cell tumor-infiltrating B cells revealed clear evidence of Ag experience, in that the cardinal features of an Ag-driven B cell response were present: significant somatic mutation, isotype switching, and codon insertion/deletion. This characterization also revealed the presence of both B cell clonal expansion and variation, suggesting that local B cell maturation most likely occurs within the tumor microenvironment. In contrast, sequences from control tissues and peripheral blood displayed none of these characteristics. Collectively, these data strongly suggest that an adaptive and specific humoral immune response is occurring within the tumor microenvironment.