The second most frequent central nervous system involvement pattern in Langerhans cell histiocytosis (LCH) is a rare condition documented in a number of reports called “neurodegenerative LCH” (ND-LCH). Magnetic resonance images confirming the presence of the disease usually demonstrate striking symmetric bilateral hyperintensities predominantly in the cerebellum, basal ganglia, pons, and/or cerebral white matter. The authors here describe for the first time in the literature a patient with ND-LCH and concomitant hydrocephalus initially treated using endoscopic third ventriculostomy (ETV). This 9-year-old boy, who had undergone chemotherapy for skin and lung LCH without central nervous system involvement at the age of 10 months, presented with acute ataxia, headaches, and paraparesis and a 1-year history of gradually increasing clumsiness. Magnetic resonance images showed obstructive hydrocephalus at the level of the aqueduct of Sylvius and signs of ND-LCH. After registering high intracranial pressure (ICP) spikes with an intraparenchymal pressure monitor, an ETV was performed. A second ETV was required months later because of ostomy occlusion, and finally a ventriculoperitoneal shunt was placed because of ostomy reocclusion. Endoscopic third ventriculostomy was initially considered the treatment of choice to divert cerebrospinal fluid without leaving a ventriculoperitoneal shunt and to obtain biopsy specimens from the periinfundibular recess area. The third ventriculostomy occluded twice, and an endoscopic aqueduct fenestration was unsuccessful. The authors hypothesized that an inflammatory process related to late ND disease was responsible for the occlusions. Biopsy specimens from the infundibular recess and fornix column did not show histopathogical abnormalities. Increased ICP symptoms resolved with cerebrospinal fluid diversion. This case is the first instance of ND-LCH with hydrocephalus reported in the literature to date. Shunt placement rather than ETV seems to be the favorable choice in relieving elevated ICP.
Amir Kershenovich, Angela V. Price, Korgun Koral, Stan Goldman and Dale M. Swift
Atif Haque, Angela V. Price, Frederick H. Sklar, Dale M. Swift, Bradley E. Weprin and David J. Sacco
Rigid fixation of the upper cervical spine has become an established method of durable stabilization for a variety of craniocervical pathological entities in children. In children, specifically, the use of C1–2 transarticular screws has been proposed in recent literature to be the gold standard configuration for pathology involving these levels. The authors reviewed the use of rigid fixation techniques alternative to C1–2 transarticular screws in children. Factors evaluated included ease of placement, complications, and postoperative stability.
Seventeen patients, ranging in age from 3 to 17 years (mean 9.6 years), underwent screw fixation involving the atlas or axis for a multitude of pathologies, including os odontoideum, Down syndrome, congenital instability, iatrogenic instability, or posttraumatic instability. All patients had preoperative instability of the occipitocervical or atlantoaxial spine demonstrated on dynamic lateral cervical spine radiographs. All patients also underwent preoperative CT scanning and MR imaging to evaluate the anatomical feasibility of the selected hardware placement. Thirteen patients underwent C1–2 fusion, and 4 underwent occipitocervical fusion, all incorporating C-1 lateral mass screws, C-2 pars screws, and/or C-2 laminar screws within their constructs. Patients who underwent occipitocervical fusion had no instrumentation placed at C-1. One patient's construct included sublaminar wiring at C-2. All patients received autograft onlay either from from rib (in 15 patients), split-thickness skull (1 patient), or local bone harvested within the operative field (1 patient). Nine patients' constructs were supplemented with recombinant human bone morphogenetic protein at the discretion of the attending physician. Eight patients had surgical sacrifice of 1 or both C-2 nerve roots to better facilitate visualization of the C-1 lateral mass. One patient was placed in halo-vest orthosis postoperatively, while the rest were maintained in rigid collars.
All 17 patients underwent immediate postoperative CT scanning to evaluate hardware placement. Follow-up was achieved in 16 cases, ranging from 2 to 39 months (mean 14 months), and repeated dynamic lateral cervical spine radiography was performed in these patients at the end of their follow-up period. Some, but not all patients, also underwent delayed postoperative CT scans, which were done at the discretion of the treating attending physician. No neurovascular injuries were encountered, no hardware revisions were required, and no infections were seen. No postoperative pain was seen in patients who underwent C-2 nerve root sacrifice. Stability was achieved in all patients postoperatively. In all patients who underwent delayed postoperative CT scanning, the presence of bridging bone was shown spanning the fused levels.
Screw fixation of the atlas using lateral mass screws, in conjunction with C-2 root sacrifice in selected cases, and of the axis using pars or laminar screws is a safe method for achieving rigid fixation of the upper cervical spine in the pediatric population.
Tarek Y. El Ahmadieh, Cody B. Wolfe, Joyce Koueik, Bradley E. Weprin, Bermans J. Iskandar and Angela V. Price
Neuroendoscopy has demonstrated safety and efficacy in the treatment of a host of pediatric neurosurgical pathologies. With the increase in its applicability, several associated complications have been described in the literature. A common practice in pediatric neurosurgery is the use of Gelfoam sponge pledget in the burr hole, followed by bone fragments and dust (obtained from the created burr hole), to cover the dural defect. This technique is used to enhance burr hole sealing and potentially prevent CSF leakage from the surgical site. Reports on intracranial bone dust migration associated with this technique are scarce. The authors report 2 cases of intracranial migration of bone fragments after an endoscopic third ventriculostomy and an endoscopic colloid cyst resection. The bone fragment migration was thought to be caused by negative pressure from a lumbar puncture in one case and external trauma to the head in the other. As endoscopy becomes more widely used, it is important to be aware of this potential complication that may in some cases require an intervention. A review of the cases reported in the literature is provided and a technique is suggested to help prevent this complication.
Anthony M. Kaufmann and Angela V. Price
Peter Jannetta was a neurosurgery resident when he proposed the neurovascular compression theory. He built upon the astute observations of Dandy, Gardner, and others who, in the era before the operating microscope, had successfully ventured into the posterior fossa. In 1965, Jannetta performed cranial nerve microdissections for dental students and identified the trigeminal portio intermedia. He proposed that preservation of these sensory fibers may avoid complete facial numbness, and together with Robert Rand developed a subtemporal transtentorial approach for selective rhizotomy for trigeminal neuralgia (TN). Such rash surgery, using an operating microscope, was then forbidden at their University of California, Los Angeles center, so they collaborated with John Alksne to perform the first surgery at Harbor General Hospital. Upon visualizing the trigeminal nerve root, Jannetta was surprised to see a pulsating superior cerebellar artery compressing the nerve and said “That’s the cause of the tic.” He also hypothesized that alleviating the observed vascular cross-compression may be curative.
A few months later, while assessing a patient with hemifacial spasm, Jannetta had the epiphany that this was the same disease process as TN, but instead affecting the facial nerve. The patient consented to what would become Jannetta’s first microvascular decompression procedure. The senior faculty members who had forbidden such surgery were away, so the supervising neurosurgeon, Paul Crandall, granted the approval to perform the surgery and assisted. Via a retromastoid approach with the patient in the sitting position and using the operating microscope, Jannetta identified and alleviated the culprit neurovascular compression, with a cure resulting.
Jannetta presented his neurovascular compression theory and operative findings to the neurosurgical patriarchy of the time. Elders of the field were generally not inclined to accept the bold speculations of an untested neurosurgeon, and were often determined to discredit the new “cure” of the old diseases. Over decades of refining his surgical technique, documenting the outcomes, and enduring the skepticism he often faced, Jannetta’s theory and his microvascular decompression procedure withstood critical analysis and have become recognized as one the great discoveries and advances in neurosurgery and medicine.
M. Burhan Janjua, Sumanth Reddy, William C. Welch, Amer F. Samdani, Ali K. Ozturk, Steven W. Hwang, Angela V. Price, Bradley E. Weprin and Dale M. Swift
The risk of readmission after brain tumor resection among pediatric patients has not been defined. The authors’ objective was to evaluate the readmission rates and predictors of readmission after pediatric brain tumor resection.
Nationwide Readmissions Database (NRD) data sets from 2010 to 2014 were searched for unplanned readmissions within 30 days of the discharge date after pediatric brain tumor resection. Patient demographic variables included sex, age, expected payment source (Medicaid or private insurance), and median annual household income. Readmission events for chemotherapy, radiation therapy, or further tumor resection were not included.
Of 282 patients (12.7%) readmitted within 30 days of the index event, the median time to readmission was 10 days (IQR 5–19 days). The most common reason for readmission was hydrocephalus, which accounted for 19% of readmission events. Other CNS-related complications (24%), surgical site infections or septicemia (14%), seizures (7%), and hematological disorders (7%) accounted for other major readmission events. The median charge for readmission events was $35,431, and the median length of readmission stay was 4 days. In multivariate regression, factors associated with a significant increase in readmission risk included Medicaid as the primary payor, discharge from the index event with home health services, and fluid and electrolyte disorders during the index event.
More than 10% of pediatric brain tumor patients have unplanned readmission events within 30 days of discharge after tumor resection. Medicaid patients and those with preoperative or early postoperative fluid and electrolyte disturbances may benefit from early or frequent outpatient visits after tumor resection.