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Alexandra D. Beier, Shobhan Vachhrajani, Simon H. Bayerl, Claudia Y. Diaz Aguilar, Maria Lamberti-Pasculli and James M. Drake

Object

Diagnosis and management of atlantoaxial rotatory subluxation (AARS) is challenging because of its variability in clinical presentation. Although several treatment modalities have been employed, there remains no consensus on the most appropriate therapy. The authors explore this issue in their 9-year series on AARS.

Methods

Records of patients diagnosed radiologically and clinically with AARS between May 2001 and March 2010 were retrospectively reviewed. Of 40 patients identified, 24 were male and were on average 8.5 years of age (range 15 months–16 years). Causes of AARS included trauma, congenital abnormalities, juvenile rheumatoid arthritis, infection, postsurgical event, and cryptogenic disease. Four patients had dual etiologies. Symptom duration varied: 29 patients had symptoms for less than 4 weeks, 5 patients had symptoms between 1 and 3 months, and 6 patients had symptoms for 3 months or more.

Results

Treatment with a cervical collar was sufficient in 21 patients. In 1 patient collar management failed and halter traction was used to reduce the subluxation. Seven patients underwent initial halter traction, but in 4 the subluxation progressed and the patients required halo traction. A halo vest was placed in 2 patients on presentation because the rotatory subluxation was severe; both patients required subsequent operative fusion. One patient required decompression and fusion due to severe canal compromise and myelopathy. All patients requiring fusion presented with subacute symptoms.

Conclusions

Management of AARS varies due to the spectrum of clinical presentations. Patients presenting acutely without neurological deficits can likely undergo collar therapy; those in whom the subluxation cannot be reduced or who present with a neurological deficit may require traction and/or surgical fixation. Patients presenting subacutely may be more prone to requiring operative intervention.

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Sanjay Gupta, Shobhan Vachhrajani, Abhaya V. Kulkarni, Michael D. Taylor, Peter Dirks, James M. Drake and James T. Rutka

Object

Extraaxial infections of the CNS, including subdural empyema and epidural abscess, are rare but potentially life-threatening conditions. Symptoms are usually progressive, and early diagnosis is therefore important. Early intervention with appropriate treatment offers the best opportunity for eradicating the infection and promoting maximal neurological recovery.

Methods

The medical records of children with extraaxial CNS infection over the last 24 years at the Hospital for Sick Children were analyzed. Only those patients with radiological and/or operative confirmation of the diagnosis of subdural empyema or epidural abscess were included in the study. Demographic and clinical data were collected to determine the outcomes after such infections and factors that predict for such outcomes.

Results

The authors identified 70 children who fulfilled the inclusion criteria. Sinusitis was the most common etiology and was seen in 38 patients. All of these patients were older than 7 years of age at diagnosis. Subdural empyemas were diagnosed in 13 patients following bacterial meningitis, and they were found primarily in infants within the 1st year of life. Other etiological factors included otogenic infection (4 cases), postneurosurgical infection (7 cases), and hematogenous spread of infection (7 cases including 6 cases of spinal epidural abscess). Streptococcus anginosus and Staphylococcus aureus were the most common pathogens identified. Sixty-four patients (91.4%) underwent at least 1 neurosurgical procedure. Seizures and cerebral edema from cortical vein thrombosis were the most common complications.

Conclusions

Due to variable etiology, identification of the responsible microorganism through neurosurgical drainage followed by long-term intravenous antibiotics remains the mainstay in treating extraaxial CNS infections. Optimal outcome is achieved with early diagnosis and therapy.

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Editorial

Achondroplasia

Arnold H. Menezes

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James A. J. King, Shobhan Vachhrajani, James M. Drake and James T. Rutka

Object

Achondroplasia is the most common form of human short-limbed dwarfism. The pediatric neurosurgeon is frequently required to treat children with achondroplasia who have hydrocephalus, cervicomedullary compression (CMD), and spinal canal stenosis. Accordingly, the authors have reviewed the experience of neurosurgery in children with achondroplasia at The Hospital for Sick Children.

Methods

The medical records and neurosurgery database at The Hospital for Sick Children were searched to identify all children with achondroplasia who underwent at least 1 neurosurgical procedure between 1956 and the present.

Results

Twenty-nine children with achondroplasia underwent 85 surgical procedures: 52 for CSF diversion in 12 patients, 20 for CMD in 18 patients, 8 for spinal disorders in 4 patients, and 5 for miscellaneous purposes in 4 patients. The CSF shunts were placed almost exclusively before 1990 and were associated with a significant number of complications. Patients undergoing CMD did very well, with only 1 patient failing to improve clinically.

Conclusions

This review provides a historical perspective on the evolution of treatment of pediatric patients with achondroplasia. The use of CSF diversion procedures, formerly fraught with complications, is now rare following the realization of the natural history of CSF space enlargement in these patients. Cervicomedullary compression is more commonly recognized due to better imaging. Central apnea is now better detected by routine sleep studies. Spine surgery, although rare, requires evaluation of both spinal stenosis and instability. These patients are best evaluated by a multidisciplinary team.

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Ibrahim Ahmed, Kurtis I. Auguste, Shobhan Vachhrajani, Peter B. Dirks, James M. Drake and James T. Rutka

Object

Epidermoid tumors are benign lesions representing 1% of all intracranial tumors. There have been few pediatric series of intracranial epidermoid tumors reported previously. The authors present their experience in the management of these lesions.

Methods

The neurosurgical database at the Hospital for Sick Children was searched for children with surgically managed intracranial epidermoid tumors. The patients' charts were reviewed for demographic data, details of clinical presentation, surgical therapy, and follow-up. Ethics board approval was obtained for this study.

Results

Seven children, all girls, were identified who met the inclusion criteria between 1980 and 2007. The average age at surgery was 11.2 years (range 8–15 years), and the mean maximal tumor diameter was 2.1 cm. Headache was the most common presenting symptom, and 1 tumor was found incidentally. Most patients had normal neurological examinations, but meningism was found in 2 cases. There were 3 cerebellopontine angle lesions, 1 pontomedullary lesion, and 3 supratentorial tumors. Hydrocephalus developed in 1 patient after aseptic meningitis, and she underwent shunt placement. There were no operative deaths. Complete resection could be performed in 2 patients. One patient experienced a small recurrence that did not require a repeated operation, while 1 subtotally resected lesion recurred and the patient underwent a second operation.

Conclusions

Intracranial epidermoid tumors are rare in the pediatric population. Total resection is desirable to minimize the risk of postoperative aseptic meningitis, hydrocephalus, and tumor recurrence. Aggressive neurosurgical resection may be associated with cranial nerve or ischemic deficits, however. In these cases, neurosurgical judgment at the time of surgery is warranted to ensure maximum resection while minimizing postoperative neurological deficits.

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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.