Ann Marie Flannery

Anorectal malformations: Evaluation of associated spinal dysraphic syndromes*

The early recognition and treatment of correctable lesions of the terminal spinal cord in patients with anorectal malformations may preserve important neurologic function. Tethered cord and intraspinal masses are detectable with the use of high-resolution ultrasonography in the neonate. Fourteen infants and children with anorectal malformations and associated spinal dysraphism have been identified in our institution over the past 7 years. Six patients had cloacal exstrophy, and eight had imperforate anus (four high and four low lesions). The spinal lesions caused symptoms in only seven children; progressive neurologic deficit in five, and urinary incontinence or retention in two others. Five asymptomatic patients with cutaneous abnormalities on the back were studied and two were discovered during scoliosis evaluation. Imaging techniques included high-resolution ultrasonography, computed tomography with and without metrizamide myelography, and magnetic resonance imaging. Spinal sonography was highly accurate in the neonatal period. The application of ultrasonography can be of great advantage in early screening of patients with anorectal malformations and, in some cases, may eliminate the need for invasive imaging techniques.

Parameters of Care for Craniosynostosis

Background A multidisciplinary meeting was held from March 4 to 6, 2010, in Atlanta, Georgia, entitled “Craniosynostosis: Developing Parameters for Diagnosis, Treatment, and Management.” The goal of this meeting was to create parameters of care for individuals with craniosynostosis. Methods Fifty-two conference attendees represented a broad range of expertise, including anesthesiology, craniofacial surgery, dentistry, genetics, hand surgery, neurosurgery, nursing, ophthalmology, oral and maxillofacial surgery, orthodontics, otolaryngology, pediatrics, psychology, public health, radiology, and speech-language pathology. These attendees also represented 16 professional societies and peer-reviewed journals. The current state of knowledge related to each discipline was reviewed. Based on areas of expertise, four breakout groups were created to reach a consensus and draft specialty-specific parameters of care based on the literature or, in the absence of literature, broad clinical experience. In an iterative manner, the specialty-specific draft recommendations were presented to all conference attendees. Participants discussed the recommendations in multidisciplinary groups to facilitate exchange and consensus across disciplines. After the conference, a pediatric intensivist and social worker reviewed the recommendations. Results Consensus was reached among the 52 conference attendees and two post hoc reviewers. Longitudinal parameters of care were developed for the diagnosis, treatment, and management of craniosynostosis in each of the 18 specialty areas of care from prenatal evaluation to adulthood. Conclusions To our knowledge, this is the first multidisciplinary effort to develop parameters of care for craniosynostosis. These parameters were designed to help facilitate the development of educational programs for the patient, families, and health-care professionals; stimulate the creation of a national database and registry to promote research, especially in the area of outcome studies; improve credentialing of interdisciplinary craniofacial clinical teams; and improve the availability of health insurance coverage for all individuals with craniosynostosis.

Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 8: Management of cerebrospinal fluid shunt infection

OBJECT The objective of this systematic review was to answer the following question: What is the optimal treatment strategy for CSF shunt infection in pediatric patients with hydrocephalus? METHODS The US National Library of Medicine and the Cochrane Database of Systematic Reviews were queried using MeSH headings and key words relevant to the objective of this systematic review. Abstracts were reviewed, after which studies meeting the inclusion criteria were selected and graded according to their quality of evidence (Classes I-III). Evidentiary tables were constructed that summarized pertinent study results, and based on the quality of the literature, recommendations were made (Levels I-III). RESULTS A review and critical appraisal of 27 studies that met the inclusion criteria allowed for a recommendation for supplementation of antibiotic treatment using partial (externalization) or complete shunt hardware removal, with a moderate degree of clinical certainty. However, a recommendation regarding whether complete shunt removal is favored over partial shunt removal (that is, externalization) could not be made owing to severe methodological deficiencies in the existing literature. There is insufficient evidence to recommend the use of intrathecal antibiotic therapy as an adjunct to systemic antibiotic therapy in the management of routine CSF shunt infections. This also holds true for other clinical scenarios such as when an infected CSF shunt cannot be completely removed, when a shunt must be removed and immediately replaced in the face of ongoing CSF infection, or when the setting is ventricular shunt infection caused by specific organisms (for example, gram-negative bacteria). CONCLUSIONS Supplementation of antibiotic treatment with partial (externalization) or complete shunt hardware removal are options in the management of CSF shunt infection. There is insufficient evidence to recommend either shunt externalization or complete shunt removal as the preferred surgical strategy for the management of CSF shunt infection. Therefore, clinical judgment is required. In addition, there is insufficient evidence to recommend the combination of intrathecal and systemic antibiotics for patients with CSF shunt infection when the infected shunt hardware cannot be fully removed, when the shunt must be removed and immediately replaced, or when the CSF shunt infection is caused by specific organisms. The potential neurotoxicity of intrathecal antibiotic therapy may limit its routine use. RECOMMENDATION Supplementation of antibiotic treatment with partial (externalization) or with complete shunt hardware removal is an option in the management of CSF shunt infection. STRENGTH OF RECOMMENDATION Level II, moderate degree of clinical certainty. RECOMMENDATION There is insufficient evidence to recommend either shunt externalization or complete shunt removal as a preferred surgical strategy for the management of CSF shunt infection. Therefore, clinical judgment is required. STRENGTH OF RECOMMENDATION Level III, unclear degree of clinical certainty. RECOMMENDATION There is insufficient evidence to recommend the combination of intrathecal and systemic antibiotics for patients with CSF shunt infection in whom the infected shunt hardware cannot be fully removed or must be removed and immediately replaced, or when the CSF shunt infection is caused by specific organisms. The potential neurotoxicity of intrathecal antibiotic therapy may limit its routine use. STRENGTH OF RECOMMENDATION Level III, unclear degree of clinical certainty.

Lymphangiolipoma of the thoracic spine in a pediatric patient with Proteus syndrome

Proteus syndrome is a rare hamartomatous disorder involving macrodactyly, hemihypertrophy, and subcutaneous lymphangiomas; fewer than 25 cases have been reported worldwide. We report a case of a thoracic epidural lymphangiolipoma in a 5-year-old boy with Proteus syndrome. Computerized axial tomography (CT) of the thoracic spine revealed a left posterior mediastinal mass that extended into the spinal canal through adjacent neural foramina. No sign of spinal cord compression was observed despite the extensive volume of tumor within the spinal canal. Surgical debulking utilizing a T3-10 laminectomy resulted in gross total resection of the tumor. Microscopic examination of the surgical specimen revealed a lymphangiolipoma. No previous report of spinal cord involvement has been reported in this syndrome. A detailed discussion of the phenotypic features and probable mode of genetic transmission is included.

The clinical and radiological evaluation of absence of the corpus callosum

A retrospective and prospective analysis of children with a diagnosis of complete absence of the corpus callosum (ACC) at the Childrens Memorial Hospital in Chicago over a 5-year period was performed. The diagnosis was based on the computed tomography (CT) and/or magnetic resonance (MR) images. From this material, 105 children with a diagnosis of ACC were analyzed on the basis of clinical symptomatology and radiological studies (CT, MR and ultrasound). Eighty-three percent of our children were symptomatic. The most common symptoms and signs were macrocephaly with hydrocephalus and seizures. MR was the best radiological imaging modality for evaluating children with ACC and associated brain anomalies. The most common associated brain anomalies with ACC in decreasing frequency in our children were: interhemispheric cyst with hydrocephalus, Dandy-Walker malformation, migrational disorders, absence of the inferior vermis, cephaloceles and lipoma of the interhemispheric fissure.

Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 1: Introduction and methodology

This clinical systematic review of and evidence-based guidelines for the treatment of pediatric hydrocephalus were developed by a physician volunteer task force. They are provided as an educational tool based on an assessment of current scientific and clinical information as well as accepted approaches to treatment. They are not intended to be a fixed protocol, because some patients may require more or less treatment. In Part 1, the authors introduce the reader to the complex topic of hydrocephalus and the lack of consensus on its appropriate treatment. The authors describe the development of the Pediatric Hydrocephalus Systematic Review and Evidence-Based Guidelines Task Force charged with reviewing the literature and recommending treatments for hydrocephalus, and they set out the basic methodology used throughout the specific topics covered in later chapters.

Intrasacral meningocele: case report and review of the literature

We have presented a case of a 28-year-old woman with an intrasacral meningocele. Diagnosis of this case was aided by the use of MRI. The literature regarding this problem has been reviewed.

Pediatric neurosurgery work force : A pilot study

The physician work force of tomorrow is influenced by the number of physicians and specialists trained today. Little information exists about the need for pediatric neurosurgeons in the next 10 years or about the activity of pediatric neurosurgeons currently in practice. The objective of this study was to obtain preliminary data about pediatric neurosurgeons and current work loads. A questionnaire was distributed to physicians attending the Pediatric Section Meeting of the American Association of Neurological Surgeons, 1997, 93 questionnaires were returned. A majority of the respondents were between 35 and 55 years of age. The largest group geographically was from the northeast. Fifty-five respondents described their practice as academic and of that group, 48% reported that their practice was either exclusively or at least 95% pediatric neurosurgery. Self-described work load varied with geographic location. In the northeast, only 26% of practitioners felt they were not busy enough, whereas in the southwest, 60% reported not being busy enough. Academic practitioners were more likely than other respondents to describe their practice as too busy or appropriately busy. Thirty-six respondents indicated plans to either expand their practice or retire within the next 10 years. This pilot study demonstrates the potential for future growth in pediatric neurosurgery as well as providing information about the current self-reported work load and its distribution over practice types and geographic locations. This study provides guidance for development of future studies to improve the sensitivity and usefulness of acquired data.

Cautions in the Conservative Management of Epidural Hematomas

A second important observation about the authors’ work is that these patients were managed in a center that could provide immediate operative intervention in the face of deterioration. In other words, this paper should not be taken as an endorsement for the nonoperative management of such patients in institutions where they would need to be transferred to get operative intervention. Patient safety, above all, must be maintained. The group of patients described in this report were by and large self-selected as they are described as not having being transferred to the institution until up to 72 h after the injury. As a group they would tend to do better, as those with a more rapidly progressing epidural hematoma would have certainly evidenced signs and symptoms warranting intervention prior to this time. Finally, the decreased use of imaging studies through the course of the series is another important feature. Once the authors had established that they understood the natural history of this disorder, it became far less necessary to document the persistence of the epidural hematoma with a CT scan and prolonged hospitalization, and far easier to release these patients to allow them to be re-imaged in 2–3 months to document the resolution of the hematoma. In conclusion, this paper makes excellent points regarding the nonoperative management of epidural hematomas in the pediatric population. We emphasize, however, that this practice is safe only under specific highly constrained situations, as described above. Dr. Mohammad A. Jamous and colleagues [1] present a small series of epidural hematomas managed conservatively. The authors reviewed 6 cases admitted to their medical center between 2003 and 2007. They discussed their management plan for the conservative, i.e. nonoperative, management of some epidural hematomas. They report good outcome in all 6 cases, as well as a decreased dependence on imaging studies as their experience with this type of management matured. The authors offer the opinion that pediatric epidural hematomas can be managed nonoperatively, but do emphasize the use of this technique only in specialized pediatric neurosurgical centers. The management technique described in this article is a sound one that has been presented before in other forums and has acceptance in the pediatric neurosurgery community. Several key points, however, have to be kept in mind when utilizing these techniques, including the location of the hemorrhage, the management in a center where neurosurgery services can be provided, the timing of the presentation, and the need for close observation. A review of the series reveals that the location of injuries were primarily the parietal or parietal-occipital region. This location adds to the safety of nonoperative management by affording a relatively large space for hematoma expansion. This position contrasts with epidurals located in the temporal fossa or posterior fossa, where the smaller constrained space may lead to seriously increased intracranial pressure. In addition, the epidurals were by and large venous epidural hematomas which are under significantly less pressure than hematomas coming from arterial origins, such as the classic middle meningeal artery injury. Published online: June 3, 2009

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.