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William E. Whitehead, Andrew Jea, Shobhan Vachhrajani, Abhaya V. Kulkarni, and James M. Drake

✓ The authors present a technique in which real-time ultrasound monitoring is used to aid the insertion of cerebrospinal fluid (CSF) shunt ventricular catheters in children without patent fontanelles. Experience with the technique is reviewed.

Intraoperative ultrasound is used to identify the compartments of the lateral ventricles and the choroid plexus prior to catheter insertion. Distance and trajectory to the best location for the hole-bearing segment of the catheter are determined and the catheter is inserted while real-time ultrasound monitoring is performed. Ten pediatric patients without open fontanelles underwent CSF shunt placement with the aid of transcranial ultrasound guidance between July and December 2006. After enlarging an occipital or frontal bur hole to a diameter of 2 cm to accommodate a small-footprint ultrasound probe, a ventricular catheter was carefully advanced into the frontal or occipital horn of the lateral ventricle while continuous ultrasound monitoring was performed. All catheters were inserted with a single pass through the brain. The final position of the ventricular catheter was visualized using intraoperative ultrasound. Postoperative computed tomography scans revealed all ventricular catheters placed accurately into the intended compartment of the ventricular system (for example, frontal horn or trigone). No procedure-related complications were noted.

Real-time transcranial ultrasound monitoring through an enlarged bur hole is a feasible, safe, and effective technique for the placement of ventricular catheters in pediatric patients without a patent fontanelle.

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Andrew Jea, Michael D. Taylor, Peter B. Dirks, Abhaya V. Kulkarni, James T. Rutka, and James M. Drake

✓The authors describe the novel use of C-1 lateral mass screws in four children 8 years of age or younger, in whom occipitocervical or atlantoaxial fusion was performed for trauma or os odontoideum. The authors retrospectively reviewed the demographics and procedural data of four children, ranging in age from 2 to 8 years, who required and underwent surgical fixation. Although C1–2 screw/rod constructs involving individual C-1 lateral mass screws and C-2 pars interarticularis or pedicle screws have been widely applied in adults, only C1–2 transarticular screw fixation has been reported in children less than 8 years of age. This report demonstrates the successful results of rigid occipitocervical and atlantoaxial fusion in four children in whom C-1 lateral mass screws were placed as part of a screw/rod construct. There was one instance of a vertebral artery injury, and the lessons learned from this complication are discussed.

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Douglas L. Brockmeyer

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Andrew Jea, Merdas Al-Otibi, Arnaud Bonnard, and James M. Drake

Object

The use of laparoscopy-assisted placement of the distal catheter of a ventriculoperitoneal (VP) shunt has been well described in the literature, and several advantages of laparoscopy over laparotomy have been documented.

Methods

The authors retrospectively reviewed the charts of 11 consecutive children with hydrocephalus of various origins who underwent surgery for initial VP shunt placement or for revision of an existing VP shunt system between July 1, 2006, and December 31, 2006. The peritoneal catheter in all of these cases was implanted using a laparoscopic procedure and with the aid of a pediatric general surgeon. Laparoscopy-assisted placement of the distal catheter was successful in all patients. There were no periprocedural complications. There was one complication, an incisional hernia, noted in the short-term follow-up period.

Conclusions

The authors illustrate that laparoscopy-assisted implantation of a peritoneal catheter is a safe, effective, minimally invasive, and technically easy approach for VP shunt placement or revision in children. It allows accurate placement of the distal catheter in the peritoneal cavity, enables retrieval of fractured catheter segments, and allows confirmation of the patency of the shunt system.

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Demitre Serletis, Patricia Parkin, Eric Bouffet, Manohar Shroff, James M. Drake, and James T. Rutka

Object

The authors review their experience with massive plexiform neurofibromas (PNs) in patients with pediatric neurofibromatosis Type 1 (NF1) to better characterize the natural history and management of these complex lesions.

Methods

The authors performed a retrospective review of data obtained in seven patients with NF1 in whom massive PNs were diagnosed at The Hospital for Sick Children in Toronto, Ontario, Canada. These patients attended routine follow-up examinations conducted by a number of specialists, and serial neuroimaging studies were obtained to monitor disease progression.

The most common presenting feature of PN was that of a painful, expanding lesion. Furthermore, two patients harbored multiple, distinct PNs affecting different body sites. With respect to management, two patients were simply observed, undergoing serial neuroimaging studies; two patients underwent biopsy sampling of their plexiform lesions; two patients underwent attempted medical treatment (farnesyl transferase inhibitor, R11577, and cyclophosphamide chemotherapy); and three patients required surgical debulking of their PNs because the massive growth of these tumors caused functional compromise. Ultimately, one patient died of respiratory complications due to progressive growth of the massive PN lesion.

Conclusions

In this review of their experience, the authors found certain features that underscore the presentation and natural history of PNs. The management of these complex lesions, however, remains unclear. Slow-growing PNs may be observed conservatively, but the authors' experience suggests that resection should be considered in selected cases involving significant deterioration or functional compromise. Nevertheless, patients with massive PNs will benefit from close surveillance by a team of specialists to monitor for ongoing disease progression.

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Peter Kan, Marion L. Walker, James M. Drake, and John R. W. Kestle

Object

Slit ventricle syndrome (SVS) is a delayed complication of shunt insertion and occurs only in children with slitlike ventricles after shunt placement. Although SVS appears to be related to early shunt placement, its predisposing factors are largely unknown.

Methods

Baseline data in 737 children who had received shunts were obtained from the databases of two previous clinical trials. Ventricular size before shunt placement and at the last routine follow up was measured using the fronto-occipital horn ratio (FOHR). Ventricles with an FOHR less than or equal to 0.2 at follow up were classified as slitlike ventricles. A univariate analysis was performed on data obtained in children with more than 1 year of clinical and radiographic follow up to identify risk factors for developing slitlike ventricles. These results were entered into a multivariate analysis to identify independent predictors of slitlike ventricles.

Two hundred forty-four children had more than 1 year of clinical and radiographic follow-up data. The 23 patients (9.4%) who developed slitlike ventricles had shunts inserted at a younger age (42 compared with 134 days, p = 0.09) and were more likely to have developed hydrocephalus secondary to infection (37.5%), head injury (25.0%), or aqueductal stenosis (22.2%). Slitlike ventricles were seen in 10.8% of patients with differential-pressure valves, 10.5% of patients with Delta valves, and 3.6% of patients with Orbis-Sigma valves (p = 0.007). Regression analysis supported the role of the valve type in developing slitlike ventricles.

Conclusions

Age at shunt insertion and valve type appear to be the modifiable risk factors for developing slitlike ventricles. If the authors of subsequent studies can further validate these conclusions, slow-draining valves and delayed shunt insertion might be used to decrease the incidence of slitlike ventricles and SVS.

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John R. W. Kestle, Hugh J. L. Garton, William E. Whitehead, James M. Drake, Abhaya V. Kulkarni, D. Douglas Cochrane, Cheryl Muszynski, and Marion L. Walker

Object

Approximately 10% of cerebrospinal fluid (CSF) shunt operations are associated with infection and require removal or externalization of the shunt, in-hospital treatment with antibiotic agents, and insertion of a new shunt. In a previous survey, the authors identified substantial variation in the duration of antibiotic therapy as well as the duration of hospital stay. The present multicenter pilot study was undertaken to evaluate current strategies in the treatment of shunt infection.

Methods

Patients were enrolled in the study if they had a successful treatment of a CSF shunt infection proved by culture of a CSF specimen. Details of their care and the incidence of culture-proved reinfection were recorded.

Seventy patients from 10 centers were followed up for 1 year after their CSF shunt infection. The initial management of the infection was shunt externalization in 17 patients, shunt removal and external ventricular drain insertion in 50, and antibiotic treatment alone in three. Reinfection occurred in 18 patients (26%). Twelve of the 18 reinfections were caused by the same organism and six were due to new organisms. The treatment time varied from 4 to 47 days, with a mean of 17.4 days for those who later experienced a reinfection compared with 16.2 days for those who did not. The most common organism (Staphylococcus epidermidis, 34 patients) was associated with a reinfection rate of 29% and a mean treatment time of 12.8 days for those who suffered reinfection and 12.5 days for those who did not.

Conclusions

Reinfection after treatment of a CSF shunt infection is alarmingly common. According to the data available, the incidence of reinfection does not appear to be related to the duration of antibiotic therapy.

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John R. W. Kestle, D. Douglas Cochrane, and James M. Drake

Object

The potential for increased complications related to the arrival of new residents in July each year has not previously been demonstrated in the neurosurgical literature. The authors investigated this phenomenon in children undergoing cerebrospinal fluid shunt surgery.

Methods

Data were obtained from a multicenter hydrocephalus clinical trials database and from hospital admission records in English-speaking Canada. Data pertaining to patients treated in July and August were compared with those pertaining to patients treated during the remainder of the year. The incidence of shunt failure, shunt infection, neurological deficits, wound infection, technical errors, and death were compared using a chi-square test for categorical outcomes, means for continuous outcomes, and survival analysis for time-dependent outcomes.

In the hydrocephalus clinical trials database, 138 of 737 patients were treated in July and August. The median duration of shunt lifespan (hereafter referred to as “shunt survival”) was 1.7 years for patients treated during the summer months and 2.4 years for those treated throughout the rest of the year (p = 0.10); for shunt infection the figures were 13.8 and 8.8% (p = 0.08) of the total number of cases, and for wound dehiscence they were 2.9 and 0.7% (p = 0.05), respectively. When all shunt procedures were included, an examination of shunt survival and infection incidence rates recorded in the Canadian Hospital Discharge Database seemed to imply a significant advantage to having surgery between September and June (log-rank statistic = 7.10, p = 0.008).

Conclusions

The data suggest a “July effect” on some outcomes related to shunt surgery, but the effect was small. Nonetheless, the potential morbidity of shunt failure, infection, and the cost of treatment indicate that continued vigilance and appropriate supervision of new staff by attending surgeons is warranted.

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R. Michael Scott

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James M. Drake

Object

The author describes the paucity of information known about occult tethered cord syndrome and summarizes the argument for using a nonsurgical approach in these cases.

Methods

A review of what we do and do not know about this syndrome is provided. Surgical procedures to divide the terminal filum in patients with symptoms of tethered spinal cord without the imaging correlates are said to result in clinical improvement, yet there is little physiological evidence to support the surgical release of the filum in the absence of other anatomical lesions. Validated diagnostic and outcome measures are also lacking, which makes the interpretation of reported results exceedingly difficult. Finally, reports used to support surgical intervention are limited by small size, the absence of control groups, and observer bias.

Conclusions

Without conclusive clinical evidence, the arguments supporting surgery for occult tethered cord syndrome must be viewed cautiously.