Bilateral Dacryoadenitis and Central Nervous System Involvement in a Child With Kimura Disease.

Kimura's disease (KD) is a systemic inflammatory condition characterized by lymphadenopathy and subcutaneous nodules in the head and neck region. The lesions have a distinctive histopathological pattern formed by follicular hyperplasia, eosinophilic infiltrates, fibrosis, and vessel proliferation. The disease may occur at all ages but predominates among young males with autoimmune dysfunctions. Visceral and orbital involvement is uncommon. We report a girl with KD who developed bilateral enlargement of the lacrimal glands and a lesion in the left lateral ventricle of the brain indistinguishable from a central nervous system neoplasia. A biopsy of both the lacrimal gland and the lateral ventricle was consistent with KD.

Psoas Abscess After Lumbar Facet Joint Injection: Case Report and Literature Review.

Low back pain (LBP) is a common condition. It is estimated that 84% of adults will present LBP symptoms at some point in their lives. Rarely, however, is LPB an indication of a serious medical condition, requiring further investigation. The treatment of non-specific LBP is based on non-pharmacological strategies, e.g., non-steroidal anti-inflammatory drugs (NSAID) or skeletal muscle relaxants. The use of epidural steroid injection (ESI) or facet joint injections relieves pain originating from degenerative spine disorders or a disk herniation, thereby providing rapid pain improvement, despite considerable long-term outcomes. Although rare, ESI complications can occur, and infections are infrequently described. This paper describes a rare case of an abscess in the psoas muscle, secondary to facet joint lumbar block. We report a male aged between 30 and 40 years old with LBP, who was submitted to a facet joint and ESI. The procedure evolved into a spinal infection of the psoas muscle. In addition, we present a brief literature review on psoas infections after spinal injection. Infection post facet joint lumbar block is very rare, with few publications in the literature. Early detection and the aggressive broad-spectrum antibiotic course must be initiated until adequate cultures are obtained and antibiotics prolong to at least four weeks.

Augmented reality and physical hybrid model simulation for preoperative planning of metopic craniosynostosis surgery.

OBJECTIVE: The main objective of neurosurgery is to establish safe and reliable surgical techniques. Medical technology has advanced during the 21st century, enabling the development of increasingly sophisticated tools for preoperative study that can be used by surgeons before performing surgery on an actual patient. Laser-printed models are a robust tool for improving surgical performance, planning an operative approach, and developing the skills and strategy to deal with uncommon and high-risk intraoperative difficulties. Practice with these models enhances the surgeon's understanding of 3D anatomy but has some limitations with regard to tactile perception. In this study, the authors aimed to develop a preoperative planning method that combines a hybrid model with augmented reality (AR) to enhance preparation for and planning of a specific surgical procedure, correction of metopic craniosynostosis, also known as trigonocephaly. METHODS: With the use of imaging data of an actual case patient who underwent surgical correction of metopic craniosynostosis, a physical hybrid model (for hands-on applications) and an AR app for a mobile device were created. The hybrid customized model was developed by using analysis of diagnostic CT imaging of a case patient with metopic craniosynostosis. Created from many different types of silicone, the physical model simulates anatomical conditions, allowing a multidisciplinary team to deal with different situations and to precisely determine the appropriate surgical approach. A real-time AR interface with the physical model was developed by using an AR app that enhances the anatomic aspects of the patient's skull. This method was used by 38 experienced surgeons (craniofacial plastic surgeons and neurosurgeons), who then responded to a questionnaire that evaluated the realism and utility of the hybrid AR simulation used in this method as a beneficial educational tool for teaching and preoperative planning in performing surgical metopic craniosynostosis correction. RESULTS: The authors developed a practice model for planning the surgical cranial remodeling used in the correction of metopic craniosynostosis. In the hybrid AR model, all aspects of the surgical procedure previously performed on the case patient were simulated: subcutaneous and subperiosteal dissection, skin incision, and skull remodeling with absorbable miniplates. The pre- and postoperative procedures were also carried out, which emphasizes the role of the AR app in the hybrid model. On the basis of the questionnaire, the hybrid AR tool was approved by the senior surgery team and considered adequate for educational purposes. Statistical analysis of the questionnaire responses also highlighted the potential for the use of the hybrid model in future applications. CONCLUSIONS: This new preoperative platform that combines physical and virtual models may represent an important method to improve multidisciplinary discussion in addition to being a powerful teaching tool. The hybrid model associated with the AR app provided an effective training environment, and it enhanced the teaching of surgical anatomy and operative strategies in a challenging neurosurgical procedure.

Time to Diagnosis in Cushing's Syndrome: A Meta-Analysis Based on 5367 Patients.

CONTEXT: Signs and symptoms of Cushing's syndrome (CS) overlap with common diseases, such as the metabolic syndrome, obesity, osteoporosis, and depression. Therefore, it can take years to finally diagnose CS, although early diagnosis is important for prevention of complications. OBJECTIVE: The aim of this study was to assess the time span between first symptoms and diagnosis of CS in different populations to identify factors associated with an early diagnosis. DATA SOURCES: A systematic literature search via PubMed was performed to identify studies reporting on time to diagnosis in CS. In addition, unpublished data from patients of our tertiary care center and 4 other centers were included. STUDY SELECTION: Clinical studies reporting on the time to diagnosis of CS were eligible. Corresponding authors were contacted to obtain additional information relevant to the research question. DATA EXTRACTION: Data were extracted from the text of the retrieved articles and from additional information provided by authors contacted successfully. From initially 3326 screened studies 44 were included. DATA SYNTHESIS: Mean time to diagnosis for patients with CS was 34 months (ectopic CS: 14 months; adrenal CS: 30 months; and pituitary CS: 38 months; P < .001). No difference was found for gender, age (<18 and ≥18 years), and year of diagnosis (before and after 2000). Patients with pituitary CS had a longer time to diagnosis in Germany than elsewhere. CONCLUSIONS: Time to diagnosis differs for subtypes of CS but not for gender and age. Time to diagnosis remains to be long and requires to be improved.

Behavioral and Biochemical Features of the Course and Surgical Treatment of Experimental Obstructive Hydrocephalus in Young Rats.

INTRODUCTION: Hydrocephalus is a multifactorial disease, affecting the dynamics of cerebrospinal fluid (CSF) and leading to severe neurological impairment in children; in spite of the recent advances in hydrocephalus research, it has many physiopathological aspects that still remain poorly understood, especially after treatment. OBJECTIVES: To analyze the clinical, radiological, histopathological, and biochemical aspects of kaolin-induced hydrocephalus in an experimental model, both in the acute phase and after shunt treatment, by means of behavioral tests, magnetic resonance imaging (MRI) scans, histopathological studies, and level of inflammatory interleukins in the CSF. METHODS: Seven-day-old Wistar rats were used and subdivided into three subgroups: treated hydrocephalic (n = 24), untreated hydrocephalic (n = 17), and controls (n = 5). The hydrocephalic groups underwent cisternal injection of 15% kaolin for induction of hydrocephalus at 7 days of age. The treated group was submitted to a ventricular-subcutaneous shunt (VSCS) 1 week after induction. All animals were euthanized at 21 days of age. They underwent motor function and memory testing as well as brain MRI scans. Histopathological analysis for glial fibrillary acidic protein and Ki-67 was done, and CSF was collected for measurement of IL-1ß, IL-6, and TNF-α. RESULTS: The average time to reach the water maze platform was highest in the untreated hydrocephalic group. The magnetization transfer rates were 37.21 and 33.76 before and after shunting, respectively. The mean astrocyte counts were 2.45, 1.36, and 90.5 for shunted, untreated, and control animals, respectively. The mean CSF IL-1ß concentrations were 62.3 and 249.6 pg/mL, the average IL-6 levels were 104.2 and 364.7 pg/mL, and the average TNF-α values were 4.9 and 170.5 pg/mL for the treated hydrocephalic group and the untreated group, respectively. CONCLUSIONS: Pups treated with a CSF shunt showed better performance on memory tests. VSCS did not revert demyelination caused by hydrocephalus. Likewise, reactive astrocytosis and cell proliferation over the germinal matrix were not reversed after shunting. Hydrocephalic animals had raised levels of inflammatory interleukins, which returned to normal after treatment.

Neurosurgical Management of Pott's Puffy Tumor in an Obese Adolescent with Asthma: Case Report with a Brief Review of the Literature.

Pott's puffy tumor is a rare and severe complication of frontal sinusitis, characterized by the progressive swelling of the frontal soft tissues secondary to a subperiosteal abscess. Radiological imaging with ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) are important diagnostic tools in establishing diagnosis and treatment planning. Early surgery along with intravenous antibiotics are required in order to achieve a good recovery. The authors report a case of Pott's puffy tumor in an obese 14-year-old male, with a previous history of asthma and a chronic use of steroids, treated with neurosurgical debridement followed by a combined course of intravenous (IV) and oral antibiotics, who had a favorable outcome upon long-term follow-up. In addition, a brief review of the current medical literature was performed for a discussion on the diagnostic and therapeutic features of this pathology.

Intracranial teratoma in children: the role of chromosome 21 trisomy.

Teratomas are very rare intracranial tumors and cytogenetic information on this group remains rare. We report a case of a mature teratoma with abnormal +21 trisomy in tumor karyotype ocurring in a non-Down syndrome(DS) infant. Additionally, the evidence for the contribution of chromosome 21 trisomy in this neoplasia are briefly reviewed. The 6-month-old male baby presented with a posterior fossa tumor. Histological evaluation of tumor specimen showed a mature teratoma composed of fully differentiated ectodermal, mesodermal and endodermal components. Although somatic karyotyping of the index case was normal, composite tumor karyotype depicted 47,XY,+21[6]/46,XY[6]. Besides previous reports of children with DS and intracranial teratomas, this is the first report to describe the occurrence of an isolated chromosome 21 trisomy within the tumor of a non-DS child. The participation of chromosome 21 in this rare pediatric tumor, either somatic or restricted to tumor specimen,may deserve special interest and further investigation.

Epidermoid cyst mimicking an intrinsic brainstem tumor.

OBJECTIVE: To describe an atypical clinical and radiological presentation of a brainstem epidermoid cyst in a child and to provide a review of the medical literature on brainstem epidermoid cysts in children. MATERIAL AND METHOD: Review of medical records and operative notes of an unusual case of a patient with a brainstem epidermoid cyst. MEDLINE literature search using the terms brainstem, epidermoid cyst and children. RESULTS: Gross total resection of the cyst was achieved. The patient had an uneventful recovery. CONCLUSION: Epidermoid cysts are rare tumors of the brain and children. The management of these tumors can be quite challenging. A good clinical and neuroradiological evaluation pre-operatively is fundamental for a successful surgical treatment. Surgical resection should be as radical as possible without putting the patient's neurological status into risk.

Hydrocephalus in posterior fossa tumors in children. Are there factors that determine a need for permanent cerebrospinal fluid diversion?

OBJECTIVE: We searched for factors that could predispose towards persistent hydrocephalus in children with posterior fossa (PF) tumors in order to determine the need for permanent cerebrospinal fluid (CSF) diversion. METHODS: The clinical records of 64 children who underwent surgery for PF tumors in the Pediatric Neurosurgery division of the Hospital of Clinics, Ribeirao Preto, Brazil, from 1990 to 2006, were retrospectively reviewed. The patients' ages ranged from 3 months to 18 years. The factors evaluated included age at surgery, severity of hydrocephalus (ventricular index), tumor location, size of the tumor, extent of tumor resection, and histology. RESULTS: Ventricular index, measured from the initial neuroradiological image, age at surgery, and location of the tumor were significantly associated with definitive postoperative CSF diversion (shunt or endoscopic third ventriculostomy), which was necessary for 34% of the patients. CONCLUSIONS: Young children with severe preoperative hydrocephalus and a midline tumor should be considered at risk when preoperative treatment decisions are made.

Aplasia cutis congenita of the scalp: is there a better treatment strategy?

BACKGROUND: Aplasia cutis congenita (ACC) is a rare disease of unknown etiology, involving any site of the body. The scalp is the most frequent location, followed by the forearms, knees, both sides of the trunk, and neck, in decreasing order of frequency. Superficial lesions may heal spontaneously and seldom result in morbidity or mortality. However, in patients with large scalp and skull defects, there are risks of infection and bleeding. Conservative treatment has been described and advocated, but some authors have highlighted the disadvantages of this treatment modality. On the other hand, several authors claim that aggressive surgical treatment has an important role for large defects. MATERIALS AND METHODS: In this paper, we report three newborns with ACC of the scalp, two large defects and a medium one, respectively, treated conservatively and surgically. CONCLUSION: The management of ACC of the scalp is still controversial. Our series suggests that conservative treatment should be performed for initial management in newborns.

Fetal exposure to sodium valproate associated with Baller-Gerold syndrome: case report and review of the literature.

CASE REPORT: We report three patients with a history of maternal valproate use during pregnancy who presented with a combination of metopic suture synostosis and upper limb malformations, which could be diagnosed as Baller-Gerold syndrome (BGS). The patients underwent surgical treatment for the craniofacial deformity, during which standard frontocranial reconstruction was performed. REVIEW OF THE LITERATURE: Only 32 patients have been reported in the world literature and these cases support the emerging view that BGS is not a distinct syndrome, but should instead be considered to be an heterogeneous phenotype with variable etiology. CONCLUSIONS: Our case series suggests for the first time that fetal sodium valproate exposure may also cause this phenotype.

Simultaneous repair of myelomeningocele and shunt insertion.

METHODS: Simultaneous shunt placement and neural tube repair are described and compared with a concomitant series of patients with delayed shunting. Twenty-eight patients with a myelomeningocele underwent closure of neural tube defects at our Institution from 1998 to 2001. Eleven patients (Group 1) had concomitant surgery performed after birth. Group 2 (7 out of 28) included patients without hydrocephalus at birth, in Group 3 (4 out of 28) the children did not develop hydrocephalus, and Group 4 (6 out of 28) patients were born outside our hospital and referred for surgical care after birth. DISCUSSION: Simultaneous insertion of shunt and correction of a myelomeningocele do not pose an additional risk to the child and do have some advantages, facilitating healing of the back without CSF leakage and protecting the brain from the effects of progressive ventricular dilatation. Patients with a myelomeningocele born outside the hospital are prone to infectious complications.

Tratamento de hidrocefalia com derivação ventrículo-peritoneal: análise de 150 casos consecutivos no Hospital das Clínicas de Ribeirão Preto

INTRODUCTION/OBJECTIVES: This study has analyzed 150 consecutive cases of treatment of hydrocephalus by ventriculoperitoneal shunt in the Faculty of Medicine of Ribeirão Preto's Hospital between May 1997 and July 2000, in order to stablish the main characteristics of the patients and proceedings , in particular the etiologies, diagnosis, complications, final outcome and associated factors. METHODS: Medica records as a source for assesment of the selected variables. RESULTS: The congenital and the acquired etiologies had the same incidence, with the complexes malformations and the complications of prematurity and meningitis with distinction in each group respectively. The main complications were malfunction of the valve (33%) and infection (15%). Including the operations caused by complications, there were 2,5 surgical proceedings to each patient, on average. In the final examination, 40% of the patients had some level of neurological deficit. The main etiologies related to this deficits were prematurity , meningitis and the complexes malformations. DISCUSSION: This report has contributed to characterize the evolution and treatment of hydrocephalus in our environment, producing basis to make comparisons with the literature and with other services. The rate of neurological deficit agrees with the literature. The rate of infection is higher, maybe because it is a Universitary Hospital. A longer period of observation would be necessary to compare the incidences of mechanical complications.

INTRODUÇÃO: O presente trabalho analisou 150 casos consecutivos de tratamento da hidrocefalia com DVP no Hospital das Clínicas de Ribeirão Preto entre março de 1997 e julho de 2000. OBJETIVO: Traçar as principais características dos pacientes e dos procedimentos, com ênfase nas etiologias, diagnóstico, complicações, seqüelas e fatores associados. MÉTODOS: Prontuários médicos como fonte para quantificar as variáveis selecionadas. RESULTADOS: As etiologias congênitas e adquiridas tiveram a mesma incidência, destacando-se a mielomeningocele no primeiro grupo e a prematuridade e a meningite no segundo. As principais complicações foram o mal funcionamento da válvula (33%) e a infecção (15%). Incluídas as cirurgias devido às complicações, houve 2,5 procedimentos por paciente em média. No último retorno, 40% dos pacientes apresentavam algum grau de retardo do desenvolvimento neuro-psico-motor (RDNPM). As principais etiologias relacionadas a RDNPM foram prematuridade, meningite e malformações complexas. Discussão: O trabalho serviu como ferramenta para ajudar a caracterizar a história natural da hidrocefalia e de seu tratamento em nosso meio, fornecendo base para uma melhor compreensão da mesma e para comparação com a literatura e com outros serviços. A taxa de RDNPM está condizente com a literatura. A taxa de infecção está mais elevada, podendo haver relação com o fato de ser este um hospital-escola. Maior tempo de seguimento seria necessário para comparação da incidência de complicações mecânicas.