Joseph H. Piatt

Clinical diagnosis of ventriculoperitoneal shunt failure among children with hydrocephalus.

Objective: To define the significance of various symptoms and signs in the diagnosis of ventriculoperitoneal shunt failure and infection. Methods: The observations that form the basis of this study were made in the course of 2 multicenter, prospective, randomized, controlled clinical trials of technical aspects of ventriculoperitoneal shunt surgery-the Shunt Design Trial and the Endoscopic Shunt Insertion Trial. At registration, basic demographic and baseline clinical data were recorded. At scheduled follow-up visits 3 months and 1, 2, and 3 years after surgery and at unscheduled visits, the presence or absence of various symptoms or signs was recorded. At each visit, the neurosurgeon-investigator made a determination about whether the shunt had reached an end point: mechanical obstruction, infection, overdrainage, or loculation of the ventricular system. Observations at the last follow-up visit for each patient constituted the data for the current study. Sensitivities, specificities, and likelihood ratios were calculated for each symptom and sign as tests for shunt failure from any cause and for failure by infection. Decision trees were constructed to analyze the relationships of various symptoms and signs in the diagnosis of shunt failure and infection. Results: Observations were available for analysis from 647 patient visits. A total of 248 shunts were judged to have failed (38%), and 55 were judged specifically to have failed by infection (8.5%). Bulging fontanel, fluid collection along the shunt, depressed level of consciousness, irritability, abdominal pain, nausea and vomiting, abnormal shunt pump test, accelerated head growth, and headache were strongly associated with shunt failure. Fever was strongly associated with shunt infection. Gross signs of wound infection were associated with shunt infection but were observed infrequently. Decision tree analysis confirmed the salience of bulging fontanel as a predictor of shunt failure. Fever and time since initial surgery were powerful predictors of shunt infection. Irritability emerged as an important observation in the identification of both shunt failure and shunt infection. Among children who underwent initial shunt insertion after 2 months of age, the absence of irritability, nausea/vomiting, and headache were powerful and generalizable predictors of the absence of shunt failure or infection. Conclusions: Analysis of symptoms and signs of ventriculoperitoneal shunt complications can inform clinical judgment in the assessment of children with hydrocephalus.

Intrathecal baclofen overdose and withdrawal

Abstract: Intrathecal baclofen (ITB) therapy is being used increasingly to treat medically intractable spasticity in children with cerebral palsy and spinal cord injuries. Baclofen overdose and withdrawal are potentially life-threatening complications of pump and spinal catheter system malfunction. We report a case of a 12-year-old boy, on long-term ITB therapy, who presents to our emergency department with an overdose of ITB, which is followed by withdrawal symptoms. The patient initially presented obtunded and in respiratory arrest. His symptoms of respiratory arrest, obtundation, fixed pupils, and hypotension mimicked other diagnoses, such as head trauma. The history obtained from the family about the pump reservoir being refilled just before the onset of symptoms led to the diagnosis. During hospitalization, as the patient recovered from the overdose, he began to experience symptoms of baclofen withdrawal, including hypertension, hyperthermia, and hallucinations. The pump was found to be disconnected and was revised. The patient was discharged home without permanent sequelae. With increased use of ITB, emergency medicine physicians must be aware of the mechanics of these pumps and the management of baclofen toxicity and withdrawal.

Spontaneous Involution of a Diencephalic Astrocytoma

We present the case of a child with a symptomatic diencephalic astrocytoma which involuted after needle biopsy without any adjuvant therapy. A tendency for certain childhood astrocytomas to regress after partial resection has been previously suggested. However, some authors have doubted whether or not such regression actually occurs. Our case radiographically documents the spontaneous involution of a low-grade astrocytoma.

Pediatric spinal injury in the US: epidemiology and disparities.

OBJECTnIn the US, race and economic status have pervasive associations with mechanisms of injury, severity of injury, management, and outcomes of trauma. The goal of the current study was to examine these relationships on a large scale in the setting of pediatric spinal injury.nnnMETHODSnAdmissions for spinal fracture without or with spinal cord injury (SCI), spinal dislocation, and SCI without radiographic abnormality were identified in the Kids Inpatient Database (KID) and the National Trauma Data Bank (NTDB) registry for 2009. Patients ranged in age from birth up to 21 years. Data from the KID were used to estimate nationwide annual incidences. Data from the NTDB were used to describe patterns of injury in relation to age, race, and payor, with corroboration from the KID. Multiple logistic regression was used to model rates of mortality and spinal fusion.nnnRESULTSnIn 2009, the estimated incidence of hospital admission for spinal injury in the US was 170 per 1 million in the population under 21 years of age. The incidence of SCI was 24 per 1 million. Incidences varied regionally. Adolescents predominated. Patterns of injury varied by age, race, and payor. Black patients were more severely injured than patients of other races as measured by Injury Severity Scale scores. Among black patients with spinal injury in the NTDB, 23.9% suffered firearm injuries; only 1% of white patients suffered firearm injuries. The overall mortality rate in the NTDB was 3.9%. In a multivariate analysis that included a large panel of clinical and nonclinical factors, black race retained significance as a predictor of mortality (p = 0.006; adjusted OR 1.571 [1.141-2.163]). Rates of spinal fusion were associated with race and payor in the NTDB data and with payor in the KID: patients with better insurance underwent spinal fusion at higher rates.nnnCONCLUSIONSnThe epidemiology of pediatric spinal injury in the US cannot be understood apart from considerations of race and economic status.

Jefferson fractures of the immature spine. Report of 3 cases.

Jefferson fractures of the immature spine have received little attention in the study of pediatric spinal trauma. Fractures through synchondroses are a possibility in the immature spine, in addition to fractures through osseous portions of the vertebral ring, and they create opportunities for misinterpretation of diagnostic imaging. The authors describe 3 examples of Jefferson fractures in young children. All 3 cases featured fractures through an anterior synchondrosis in association with persistence of the posterior synchondrosis or a fracture of the posterior arch. The possibility of a Jefferson fracture should be considered for any child presenting with neck pain, cervical muscle spasm, or torticollis following a head injury, despite a seemingly normal cervical spine study. Jefferson fractures in young children are probably much more common than previously recognized.

Macrocephaly in infancy: benign enlargement of the subarachnoid spaces and subdural collections

OBJECTIVE Benign enlargement of the subarachnoid spaces (BESS) is a common finding on imaging studies indicated by macrocephaly in infancy. This finding has been associated with the presence of subdural fluid collections that are sometimes construed as suggestive of abusive head injury. The prevalence of BESS among infants with macrocephaly and the prevalence of subdural collections among infants with BESS are both poorly defined. The goal of this study was to determine the relative frequencies of BESS, hydrocephalus, and subdural collections in a large consecutive series of imaging studies performed for macrocephaly and to determine the prevalence of subdural fluid collections among patients with BESS. METHODS A text search of radiology requisitions identified studies performed for macrocephaly in patients ≤ 2 years of age. Studies of patients with hydrocephalus or acute trauma were excluded. Studies that demonstrated hydrocephalus or chronic subdural hematoma not previously recognized but responsible for macrocephaly were noted but not investigated further. The remaining studies were reviewed for the presence of incidental subdural collections and for measurement of the depth of the subarachnoid space. A 3-point scale was used to grade BESS: Grade 0, < 5 mm; Grade 1, 5-9 mm; and Grade 2, ≥ 10 mm. RESULTS After exclusions, there were 538 studies, including 7 cases of hydrocephalus (1.3%) and 1 large, bilateral chronic subdural hematoma (0.2%). There were incidental subdural collections in 21 cases (3.9%). Two hundred sixty-five studies (49.2%) exhibited Grade 1 BESS, and 46 studies (8.6%) exhibited Grade 2 BESS. The prevalence of incidental subdural collections among studies with BESS was 18 of 311 (5.8%). The presence of BESS was associated with a greater prevalence of subdural collections, and higher grades of BESS were associated with increasing prevalence of subdural collections. After controlling for imaging modality, the odds ratio of the association of BESS with subdural collections was 3.68 (95% CI 1.12-12.1, p = 0.0115). There was no association of race, sex, or insurance status with subdural collections. Patients with BESS had larger head circumference Z-scores, but there was no association of head circumference or age with subdural collections. Interrater reliability in the diagnosis and grading of BESS was only fair. CONCLUSIONS The current study confirms the association of BESS with incidental subdural collections and suggests that greater depth of the subarachnoid space is associated with increased prevalence of such collections. These observations support the theory that infants with BESS have a predisposition to subdural collections on an anatomical basis. Incidental subdural collections in the setting of BESS are not necessarily indicative of abusive head injury.

Incidental Findings on Brain and Spine Imaging in Children

In recent years, the utilization of diagnostic imaging of the brain and spine in children has increased dramatically, leading to a corresponding increase in the detection of incidental findings of the central nervous system. Patients with unexpected findings on imaging are often referred for subspecialty evaluation. Even with rational use of diagnostic imaging and subspecialty consultation, the diagnostic process will always generate unexpected findings that must be explained and managed. Familiarity with the most common findings that are discovered incidentally on diagnostic imaging of the brain and spine will assist the pediatrician in providing counseling to families and in making recommendations in conjunction with a neurosurgeon, when needed, regarding additional treatments and prognosis.

Constitutional LZTR1 Mutation Presenting with a Unilateral Vestibular Schwannoma in a Teenager

Schwannomatosis is a rare neurofibromatosis clinically diagnosed by age‐dependent criteria, with bilateral vestibular schwannoma and/or a constitutional NF2 mutation representing exclusion criteria. Following SMARCB1 germline mutations, constitutional mutations in LZTR1 were discovered. We report on the molecular investigation in a patient presenting at 14 years with a unilateral vestibular schwannoma, ultimately causing blindness and unilateral hearing loss, in the absence of other schwannomas or a positive family history.

Donald H. Reigel

Accessible online at: http://BioMedNet.com/karger Pediatric Neurosurgery was founded by Bruce Hendrick and Don Reigel in 1985. Some questioned the need for a journal dedicated solely to pediatric neurosurgery. Since then, Pediatric Neurosurgery has become the official journal of the American Society of Pediatric Neurosurgeons (ASPN) and the publication voice of American pediatric neurosurgery. Throughout the 14 years of publication, Pediatric Neurosurgery has had one Managing Editor, Donald Reigel. Don saw the need for a high-quality pediatric neurosurgical journal, and with minimal support brought it into existence. Nearly single-handedly, he sustained the journal through some difficult times. Don Reigel did the same thing for the ASPN 20 years ago. He saw the need, convinced practicing pediatric neurosurgeons that the time had come, called 17 pediatric neurosurgeons together in Pittsburgh, and established the ASPN. Don Reigel later served as the President of ASPN. He also served as Chairman of the Pediatric Section of the American Association of Neurological Surgeons and as Chairman of the American Board of Pediatric Neurological Surgery. I would argue that Don Reigel has had a greater influence on the development of organized pediatric neurosurgery in North America than any other person. Through all of this, Don never took his eyes off the prize, a better life for the child. Don Reigel retired from the active practice of pediatric neurosurgery and as Managing Editor of Pediatric Neurosurgery in June of 1998, to dedicate his full attention to the Woodlands Project. Many of you are familiar with this project. Don and Gay Reigel have and will continue to assist young people with chronic disease reach their maximum potential. They have taught us much about children with central nervous system disease and how to care for them. I attended one of Don’s retirement dinners and was amazed by the outpouring of love and respect shown for this great man by his colleagues, community leaders, and families of the children. Donald, we wish you Godspeed. Thank you from the Editorial Staff of Pediatric Neurosurgery.

Fracture of the anterior arch of atlas after minor trauma of the immature spine postlaminectomy.

Abstract Torticollis is a common complaint in the pediatric emergency department. Here, we report what we believe to be the first example in a young child of a fracture of the anterior arch of the atlas associated with an acquired, postsurgical defect of the posterior arch. A brief review of pediatric cervical spine injuries and fractures is presented. Atlas laminectomy may predispose patients to isolated atlas fractures even with minor trauma. Those fractures, however, are stable and treated by hard cervical orthosis.