✓ Sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. The typical clinical characteristics of this disease include painless cervical lymphadenopathy, fever, and weight loss. The condition can present with an extranodal mass in about 25% of patients, and isolated masses without lymph node involvement occur rarely. The authors describe a 5-year-old boy with cavernous sinus syndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopathological and magnetic resonance imaging characteristics are discussed.
Ellen G. Shaver, Susan L. Rebsamen, Anthony T. Yachnis and Leslie N. Sutton
Kimberly Hamilton, Susan Rebsamen, Shahriar Salamat and Raheel Ahmed
An extraosseous intradural presentation for a sacral chordoma in the pediatric age group has not been reported to date. This is a report on an 11-year-old boy who presented with an extraosseous, intradural sacral chordoma. He underwent gross-total resection and received adjuvant proton beam therapy. Neoplastic transformation of the notochord is reviewed to illustrate the developmental basis for the surgical anatomy and pathogenesis of the classic chordoma variant. Clinical and pathological features are reviewed to differentiate this chordoma presentation from classic osseous chordomas and ecchordosis physaliphora, a related benign developmental notochordal lesion. Finally, the role of developmental signaling in the pathogenesis of chordomas from postembryonic notochordal tissue is discussed.
Mark R. Kraemer, Joyce Koueik, Susan Rebsamen, David A. Hsu, M. Shahriar Salamat, Susan Luo, Sara Saleh, Taryn M. Bragg and Bermans J. Iskandar
Ventricular shunts have an unacceptably high failure rate, which approaches 50% of patients at 2 years. Most shunt failures are related to ventricular catheter obstruction. The literature suggests that obstructions are caused by in-growth of choroid plexus and/or reactive cellular aggregation. The authors report endoscopic evidence of overdrainage-related ventricular tissue protrusions (“ependymal bands”) that cause partial or complete obstruction of the ventricular catheter.
A retrospective review was completed on patients undergoing shunt revision surgery between 2008 and 2015, identifying all cases in which the senior author reported endoscopic evidence of ependymal tissue in-growth into ventricular catheters. Detailed clinical, radiological, and surgical findings are described.
Fifty patients underwent 83 endoscopic shunt revision procedures that revealed in-growth of ventricular wall tissue into the catheter tip orifices (ependymal bands), producing partial, complete, or intermittent shunt obstructions. Endoscopic ventricular explorations revealed ependymal bands at various stages of development, which appear to form secondarily to siphoning. Ependymal bands are associated with small ventricles when the shunt is functional, but may dilate at the time of obstruction.
Ventricular wall protrusions are a significant cause of proximal shunt obstruction, and they appear to be caused by siphoning of surrounding tissue into the ventricular catheter orifices.
Joyce Koueik, Carolina Sandoval-Garcia, John R. W. Kestle, Brandon G. Rocque, David M. Frim, Gerald A. Grant, Robert F. Keating, Carrie R. Muh, W. Jerry Oakes, Ian F. Pollack, Nathan R. Selden, R. Shane Tubbs, Gerald F. Tuite, Benjamin Warf, Victoria Rajamanickam, Aimee Teo Broman, Victor Haughton, Susan Rebsamen, Timothy M. George and Bermans J. Iskandar
Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study’s goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction.
Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression. Clinical data were entered into a database preoperatively and at 1–2 weeks, 3–6 months, and 1 year postoperatively. MR images were evaluated by 3 independent, blinded teams of neurosurgeons and neuroradiologists. The primary endpoint was improvement or resolution of the syrinx.
Eight clinical sites were chosen based on the results of a published questionnaire intended to remove geographic and surgeon bias. Data from 68 patients were analyzed after exclusions, and complete clinical and imaging records were obtained for 55 and 58 individuals, respectively. There was strong agreement among the 3 radiology teams, and there was no difference in patient demographics among sites, surgeons, or surgery types. Tonsillar reduction was not associated with > 50% syrinx improvement (RR = 1.22, p = 0.39) or any syrinx improvement (RR = 1.00, p = 0.99). There were no surgical complications.
This study demonstrated the feasibility of a prospective, multicenter surgical trial in CM-I/syringomyelia and provides pilot data indicating no discernible difference in 1-year outcomes between PFDD with and without tonsillar reduction, with power calculations for larger future studies. In addition, the study revealed important technical factors to consider when setting up future trials. The long-term sequelae of tonsillar reduction have not been addressed and would be an important consideration in future investigations.