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Tae Sung Park and James M. Johnston

✓ Selective dorsal rhizotomy is a well-established surgical procedure for improving lower-extremity spasticity in children with cerebral palsy. The standard technique requires an L1–S1 laminectomy or laminoplasty for visualization of all dorsal nerve roots exiting at their respective foramina. The authors describe a rhizotomy procedure that requires a single-level laminectomy at the level of the conus, with the advantages of decreased operating time and postoperative pain as well as a minimal risk of progressive lumbar instability. This procedure is both effective and well tolerated in appropriately selected children and adults, and has had one cerebrospinal fluid lead requiring operative repair in more than 1500 patients treated at the authors' institution since 1991.

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Brandon A. Sherrod, James M. Johnston and Brandon G. Rocque

OBJECTIVE

Hospital readmission rate is increasingly used as a quality outcome measure after surgery. The purpose of this study was to establish, using a national database, the baseline readmission rates and risk factors for patient readmission after pediatric neurosurgical procedures.

METHODS

The American College of Surgeons National Surgical Quality Improvement Program–Pediatric database was queried for pediatric patients treated by a neurosurgeon between 2012 and 2013. Procedures were categorized by current procedural terminology (CPT) code. Patient demographics, comorbidities, preoperative laboratory values, operative variables, and postoperative complications were analyzed via univariate and multivariate techniques to find associations with unplanned readmissions within 30 days of the primary procedure.

RESULTS

A total of 9799 cases met the inclusion criteria, 1098 (11.2%) of which had an unplanned readmission within 30 days. Readmission occurred 14.0 ± 7.7 days postoperatively (mean ± standard deviation). The 4 procedures with the highest unplanned readmission rates were CSF shunt revision (17.3%; CPT codes 62225 and 62230), repair of myelomeningocele > 5 cm in diameter (15.4%), CSF shunt creation (14.1%), and craniectomy for infratentorial tumor excision (13.9%). The lowest unplanned readmission rates were for spine (6.5%), craniotomy for craniosynostosis (2.1%), and skin lesion (1.0%) procedures. On multivariate regression analysis, the odds of readmission were greatest in patients experiencing postoperative surgical site infection (SSI; deep, organ/space, superficial SSI, and wound disruption: OR > 12 and p < 0.001 for each). Postoperative pneumonia (OR 4.294, p < 0.001), urinary tract infection (OR 4.262, p < 0.001), and sepsis (OR 2.616, p = 0.006) also independently increased the readmission risk. Independent patient risk factors for unplanned readmission included Native American race (OR 2.363, p = 0.019), steroid use > 10 days (OR 1.411, p = 0.010), oxygen supplementation (OR 1.645, p = 0.010), nutritional support (OR 1.403, p = 0.009), seizure disorder (OR 1.250, p = 0.021), and longer operative time (per hour increase, OR 1.059, p = 0.029).

CONCLUSIONS

This study may aid in identifying patients at risk for unplanned readmission following pediatric neurosurgery, potentially helping to focus efforts at lowering readmission rates, minimizing patient risk, and lowering costs for health care systems.

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James M. Johnston and Tae Sung Park

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Albert N. Martins, Archimedes Ramirez, James Johnston and P. Robert Schwetschenau

✓ Sixty-six patients with symptomatic herniated lumbar discs refractory to the usual conservative management were allocated at random into one of two treatment groups according to a double-blind protocol: 31 received chymopapain intradiscally (chemonucleolysis) and 35 received a placebo intradiscally. Symptoms remained significantly improved 1 year or more after injection for 55% of those treated with chymopapain and for 46% of those treated with placebo. The difference is not statistically significant. However, to discard chemonucleolysis on the basis of this one small clinical trial may be premature. Since continuing controversy has re-established a climate in which another double-blind study of chemonucleolysis is ethically feasible and scientifically desirable, we favor additional clinical trials under a tightly controlled protocol to help resolve the issue.

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Jonathan Pindrik, Brandon G. Rocque, Anastasia A. Arynchyna, James M. Johnston and Curtis J. Rozzelle

OBJECTIVE

Endoscopic third ventriculostomy (ETV) with choroid plexus (CP) cauterization (CPC) represents a viable treatment option for congenital hydrocephalus in infants younger than 2 years. Imaging studies complement clinical data in the evaluation of treatment success or failure. The objectives of this study were to investigate novel radiographic markers—cerebrospinal fluid (CSF) turbulence and CP visualization—and their ability to reflect or predict clinical outcomes following ETV/CPC.

METHODS

Hydrocephalic patients younger than 2 years who were initially treated by ETV/CPC at the senior authors' institution between March 2013 and February 2014 were retrospectively reviewed. Clinical data, as well as the visualization of CSF turbulence and CP on pre- and postoperative fast-sequence MRI, were recorded. Radiographic images were reviewed by a blinded observer based on specific criteria for the visualization of CSF turbulence and CP. Data were collected and analyzed using descriptive statistics, including Fisher's exact test for comparisons. The research team obtained appropriate institutional review board approval for this study, without the need for informed consent.

RESULTS

Among the 32 patients (53% male and 47% female) studied, 18 of 32 (56%) responded favorably to initial or repeat ETV/CPC, with 13 of 32 (41%) patients requiring 1 surgery. Of the 19 (59%) patients whose initial ETV/CPC failed, 8 of 19 (42%) patients underwent repeat ETV/CPC, with 5 of 8 (63%) patients responding favorably. Radiographic CSF turbulence appeared more frequently following ETV/CPC failure than after ETV/CPC success (55% vs 18%, respectively; p = 0.02). The sensitivity and specificity of CSF turbulence as a radiographic marker for ETV/CPC failure were 80% and 58%, respectively. The radiographic depiction of CP disappearance following ETV/CPC from pre- to postoperative imaging occurred in 20 of 30 patients (67%). Among the patients who responded unsuccessfully to ETV/CPC and ultimately required secondary shunt insertion, 71% (10 of 14 patients) demonstrated CP persistence on postoperative imaging. In contrast, 6% (1 of 18) of patients who were treated successfully by ETV/CPC demonstrated the presence of CP on follow-up imaging. This difference reached statistical significance (p = 0.0001). The visualization of CP persistence despite ETV/CPC reflected treatment failure with 91% sensitivity and 81% specificity. The sensitivity of either or both radiographic markers to suggest ETV/CPC failure was 77%, while their specificity (both markers absent, thereby indicating ETV/CPC success) was 81%.

CONCLUSIONS

Radiographic markers correlate with clinical outcomes following the treatment of infantile hydrocephalus with ETV/CPC. Specifically, CSF turbulence may indicate ongoing pathological CSF flow dynamics, while CP absence following ETV/CPC may predict shunt independence. Future studies that incorporate prospective review and formal intra- and interobserver reliability estimates may help corroborate the utility of these radiographic markers.

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James M. Johnston, David D. Limbrick Jr., Wilson Z. Ray, Stephanie Brown, Joshua Shimony and Tae Sung Park

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.

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James M. Johnston Jr., Francesco T. Mangano, Jeffrey G. Ojemann, Tae Sung Park, Edwin Trevathan and Matthew D. Smyth

Object

The purpose of this study was to better define the incidence of complications associated with placement of subdural electrodes for localization of seizure foci and functional mapping in children.

Methods

The authors retrospectively reviewed the records of 112 consecutive patients (53 boys, 59 girls; mean age 10.9 years, range 10 months–21.7 years) with medically intractable epilepsy who underwent invasive monitoring at the Pediatric Epilepsy Center at St. Louis Children’s Hospital between January 1994 and July 2005. There were 122 implantation procedures (85 grids and strips, 32 strips only, five grids only, four with additional depth electrodes), with a mean monitoring period of 7.1 days (range 2–21 days). Operative complications included the need for repeated surgery for additional electrode placement (5.7%); wound infection (2.4%); cerebrospinal fluid leak (1.6%); and subdural hematoma, symptomatic pneumocephalus, bone flap osteomyelitis, and strip electrode fracture requiring operative retrieval (one patient [0.8%] each). There were four cases of transient neurological deficit (3.3%) and no permanent deficit or death associated with invasive monitoring.

Conclusions

Placement of subdural grid and strip electrodes for invasive video electroencephalographic monitoring is generally well tolerated in the pediatric population. The authors found that aggressive initial electrode coverage was not associated with higher rates of blood transfusion or perioperative complications, and reduced the frequency of repeated operations for placement of supplemental electrodes.

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Albert N. Martins, James S. Johnston, James M. Henry, Thomas J. Stoffel and Giovanni Di Chiro

✓ The cases of six patients are presented to delineate the clinical profile of delayed radiation necrosis of the brain. In five the diagnosis was verified histologically. Symptoms most often begin 9 months to 2 years after radiotherapy. Progressive visual impairment and dementia are common following perisellar irradiation, while hemispheric signs predominate following irradiation of the cerebrum. Cerebrospinal fluid protein may be elevated. Focal delta slowing is usually present on electroencephalography. The necrotic brain may appear on radionuclide brain scan as an area of abnormal uptake and also act as an avascular space-occupying lesion. With computerized tomography, radiation necrosis appears as an intracerebral area with diminished absorption coefficient that is often enhanced with intravenous contrast medium. The syndrome may be sufficiently characteristic to eliminate the need for surgical exploration and biopsy in some cases. Cumulative experience suggests that the risk-to-benefit ratio of radiotherapy becomes increasingly unfavorable for most patients with benign intracranial neoplasms when the standard brain tumor dose of 5000 to 7000 rads is fractionated at greater than 200 rads per day.

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Giant intracranial teratoma with epignathus in a neonate

Case report and review of the literature

James M. Johnston, Nilesh A. Vyas, Alex A. Kane, David W. Molter and Matthew D. Smyth

✓Epignathus, a congenital oropharyngeal teratoma, is a rare clinical entity with variable clinical outcomes described in the literature. Even fewer cases of epignathus with intracranial extension have been reported, all with poor outcomes. In this manuscript, the authors present a case of epignathus with intracranial extension, emphasizing clinical presentation, imaging findings, a staged surgical approach, multidisciplinary management, and outcome.

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Daxa M. Patel, R. Shane Tubbs, Gigi Pate, James M. Johnston Jr. and Jeffrey P. Blount

Object

Surveillance imaging of the cerebral ventricles can be valuable in following up children with shunt-treated hydrocephalus. There also, however, has been recent increased awareness and concern over the potential risk associated with imaging-related radiation exposure in children. Magnetic resonance imaging represents an imaging alternative that does not use ionizing radiation; however, its practical utility has been limited due to the near-uniform requirement for sedation or general anesthesia in children. Magnetic resonance imaging without sedation is often futile because of the movement artifact produced by the nonsedated pediatric patient. Some studies have demonstrated the feasibility of using fast-sequence MRI (fsMRI), but the reported experiences are limited. The authors have incorporated fsMRI into their routine shunt surveillance imaging paradigms and report here a 5-year experience with this modality.

Methods

The authors initially started using fsMRI for routine surveillance in a single clinic in 2008 and have gradually increased their institutional utilization of this modality as experience has accumulated and protocols have been refined. Imaging sequences obtained for each child include an axial T2-weighted half-Fourier acquisition single-shot turbo spin-echo (HASTE), coronal T2-weighted HASTE, and sagittal T2-weighted HASTE images. The authors conducted a retrospective chart and imaging review. They rated each fsMR image according to 5 visibility parameters: 1) ventricle size, 2) ventricle configuration, 3) presence or absence of transependymal flow, 4) presence or absence of motion artifact, and 5) visualization of the ventricular catheter. Each parameter was graded as 1 (present) or 0 (absent). Thus, the maximum value assigned to each scan could be 5 and the minimum value assigned to each scan could be 0. Interrater reliability between pairs of observers was calculated using the Kendall's tau-b and intraclass coefficients.

Results

Two hundred patients underwent fsMRI. No child required sedation. The average duration of examinations was approximately 3.37 minutes, and mean age of the patients was 5.7 years. Clinically useful images were attained in all cases. Overall quality of the fsMRI studies based on the 5 different visibility parameters showed that 169 images (84.5%) included 4 or 5 parameters (score ≥ 4) and had statistically significant excellent quality. The Kendall's tau-b for the overall fsMRI ratings was 0.82 (p = 0.002) and the intraclass coefficient was 0.87 (p < 0.0001).

Conclusions

In the present cohort of 200 patients, fsMRI studies were shown to have an excellent overall quality and a statistically significant high degree of interrater reliability. Consequently, the authors propose that fsMRI is a sufficiently effective modality that eliminates the need for sedation and the use of ionizing radiation and that it should supplant CT for routine surveillance imaging in hydrocephalic patients.