Appearance of intracranial cottonoids on intraoperative magnetic resonance imaging.

To our knowledge, this is a unique report of intraoperative magnetic resonance imaging (iMRI) of an intracranial cottonoid. The current literature describes images of cottonoids as a post-operative finding in the setting of an unintentionally retained foreign body; however, the iMRI images we present are important as the use of iMRI in the resection of complex tumors and epilepsy foci increases. This series of images was obtained during a craniotomy for tumor resection of a patient with dysembryoplastic neuroepithelial tumor. To determine both the degree of tumor resection and the amount of residual tumor, cottonoids were left in our patient's resection cavity and underwent iMRI. The ability to distinguish cottonoids in these images is important for intraoperative localization of resection margins and to prevent the retention of cottonoids.

In utero Diagnosis of Spinal Dermal Sinus.

INTRODUCTION: Congenital dermal sinus (CDS) is an open neural tube defect (NTD) that occurs in 1 in 2,500 births a year and often goes undetected until patients present with complications like infection and neurological deficits. Early diagnosis and repair of CDS may prevent formation of these complications. In utero diagnosis of these lesions may improve long-term outcomes by enabling referral to specialty services and planned postnatal repair; however, only 2 such cases have been reported in the literature. We present a third case of in utero diagnosis of CDS with a description and discussion of findings from surgical exploration and pathology. CASE PRESENTATION: Routine prenatal ultrasound scan detected a tethered cystic structure arising from the back of the fetus at 20 weeks of gestation. Dedicated fetal ultrasound confirmed the presence of a cystic lesion protruding through a lamina defect, while fetal magnetic resonance imaging showed an intact spinal cord and meninges, suggesting a diagnosis of CDS. Neurosurgery followed along closely and took the child for surgical exploration on day 2 of life. A fibrous stalk with an intradural component and associated cord tethering was excised. Histology showed fibrous tissue without an epithelial-lined lumen. CONCLUSION: CDS is a form of NTD that occurs from nondisjunction of the cutaneous ectoderm and neuroectoderm during formation of the neural tube. Slight differences in how this error occurs can explain variations seen in this spectrum of disease, including CDS without an epithelial-lined lumen as seen in this case. Newborns with CDS can go undiagnosed for years and present with long-term complications. Fetal imaging can assist in early recognition and surgical excision of CDS in newborns.

Subdural hematoma, retinal hemorrhage, and fracture triad as a clinical predictor for the diagnosis of child abuse.

OBJECTIVE: Nonaccidental trauma (NAT) is a major cause of traumatic death during infancy and early childhood. Several findings are known to raise the index of clinical suspicion: subdural hematoma (SDH), retinal hemorrhage (RH), fracture, and external trauma. Combinations of certain injury types, determined via statistical frequency associations, may assist clinical diagnostic tools when child abuse is suspected. The present study sought to assess the statistical validity of the clinical triad (SDH + RH + fracture) in the diagnosis of child abuse and by extension pediatric NAT. METHODS: A retrospective review of The University of Arizona Trauma Database was performed. All patients were evaluated for the presence or absence of the components of the clinical triad according to specific International Classification of Diseases (ICD)-10 codes. Injury type combinations included some variation of SDH, RH, all fractures, noncranial fracture, and cranial fracture. Each injury type was then correlated with the ICD-10 codes for child abuse or injury comment keywords. Statistical analysis via contingency tables was then conducted for test characteristics such as sensitivity, specificity, positive predictive value, and negative predictive value. RESULTS: There were 3149 patients younger than 18 years of age included in the quantitative analysis, all of whom had at least one component of the clinical triad. From these, 372 patients (11.8%) had a diagnosis of child abuse. When compared to a single diagnosis of either SDH, RH, all fractures, noncranial fracture, or cranial fracture, the clinical triad had a significantly greater correlation with the diagnosis of child abuse (100% of cases) (p < 0.0001). The dyad of SDH + RH also had a significantly greater correlation with a child abuse diagnosis compared to single diagnoses (88.9%) (p < 0.0001). The clinical triad of SDH + RH + fracture had a sensitivity of 88.8% (95% CI 87.6%-89.9%), specificity of 100% (95% CI 83.9%-100%), and positive predictive value of 100% (95% CI 99.9%-100%). The dyad of SDH + RH had a sensitivity of 89.1% (95% CI 87.9%-90.1%), specificity of 88.9% (95% CI 74.7%-95.6%), and positive predictive value of 99.9% (95% CI 99.6%-100%). All patients with the clinical triad were younger than 3 years of age. CONCLUSIONS: When SDH, RH, and fracture were present together, child abuse and by extension pediatric NAT were highly likely to have occurred.

Enlarging traumatic superficial temporal artery pseudoaneurysm from a lacrosse ball injury: illustrative case.

BACKGROUND: The development of a mobile, growing, pulsatile mass after blunt head trauma to the forehead area, resulting in a superficial temporal artery pseudoaneurysm, is a very rare outcome. Most pseudoaneurysms are diagnosed with ultrasound, computed tomography (CT), and/or magnetic resonance imaging and treated via resection or, occasionally, embolization. OBSERVATIONS: The authors describe a case of a young male lacrosse player who presented with a bulging, partially pulsatile mass in the right forehead region 2 months after trauma from a high-velocity ball striking his head while helmeted. The authors reviewed 12 patients in the literature and describe each patient's epidemiological features, nature of the trauma, and onset of the lesion after the trauma, as well as the diagnostic methods and treatments for each case. LESSONS: Overall, CT and ultrasound appear to be the easiest and most used methods of diagnosis, and resection under general anesthesia is the most common treatment method.

A Rare Neonatal Intramedullary Immature Teratoma Undiagnosed in utero: A Case Report.

BACKGROUND AND IMPORTANCE: Immature teratoma is a known pediatric tumor. However, spinal variants are rare and can present both a diagnostic and therapeutic challenge, particularly regarding aggression as it pertains to extent of resection, likelihood of recurrence and concordant prognosis, and the need and efficacy of adjuvant therapies. CLINICAL PRESENTATION: The patient is a 27-day-old female who presented with 10 days of poor feeding, irritability, and progressive hypotonia. Although upon immediate presentation emergency providers' differential diagnoses included meningitis, inborn error of metabolism, and genetic neurodegenerative disease, a subsequent magnetic resonance (MR) imaging of the total spine revealed a large intradural intramedullary mass extending from the medulla to the thoracic cord at T12. The patient underwent multilevel cervical and thoracic laminectomies/laminoplasty for maximal safe resection. Histopathology revealed mostly mature tissue elements originating from all 3 germ layers, interspersed with foci of immature neuroepithelium, consistent with grade 1 immature teratoma. Following surgical intervention, the patient regained strength and spontaneous movement and underwent physical therapy. Follow-up MR imaging of the total spine was obtained every 3 months, and at 9 months, recurrence was demonstrated, which was successfully treated with chemotherapy. Further surveillance MR imaging of the total spine has demonstrated cystic myelomalacia changes without definite tumor recurrence, at 5-year follow-up. Clinically, the patient has developed scoliosis without weakness, pain, or urinary symptoms. CONCLUSION: This case demonstrates an exceptionally rare and unusual variant neoplasm in a neonate and highlights the difficulty of diagnosis and the important role of MR imaging. It also illustrates the importance of gross total resection, the risk of recurrence, and the need for close radiographic follow-up of these lesions. It also provides a useful example of the efficacy of adjuvant chemotherapy in treating recurrence.

5-Aminolevulinic acid-assisted resection of pediatric dysembryoplastic neuroepithelial tumor: illustrative case.

BACKGROUND: 5-Aminolevulinic acid (5-ALA) is approved as an adjunct for the resection of high-grade gliomas and is associated with improved outcomes. Dysembryoplastic neuroepithelial tumors (DNETs) are benign glioneural tumors occurring primarily in pediatric patients and often manifesting with seizure disorder. The goal of the surgical intervention is to obtain gross-total resection, which is associated, in the majority of cases, with seizure freedom. OBSERVATIONS: The authors present the first case report of a pediatric patient who underwent gross-total resection of a 5-ALA-positive DNET with no evidence of recurrent seizures (Engel class I). LESSONS: Fluorescence-guided surgery using 5-ALA facilitated gross-total resection of the mass.

Neurosurgical management of a rare congenital supratentorial neurenteric cyst with associated nasal dermal sinus: case report.

The authors describe the case of a patient with the classic clinical presentation and radiographic features of a nasal dermal sinus with an associated intracranial cyst; however, histopathology revealed that the intracranial cyst was neurenteric instead of the typical epidermoid or dermoid cyst. Preoperative assessment included CT and MRI, which revealed a direct communication between the patient's nasal polypoid lesion and the anterior skull base via the foramen cecum. At the hands of a multidisciplinary plastic surgery and neurosurgery team, the patient underwent concurrent gross-total resection of the nasal polypoid lesion, the intracranial intradural cystic lesion, and their interconnecting tract.

Cerebrospinal Fluid Total Tau is Increased in Normal Pressure Hydrocephalus Patients who Undergo Successful Lumbar Drain Trials.

BACKGROUND: Idiopathic normal pressure hydrocephalus (INPH) is a neurologic disease that affects <1% of those aged >65 years, but is difficult to distinguish from other diseases that present in this age group, such as Alzheimer's disease. Large volume lumbar puncture and an external lumbar drain trial (ELD) are used to make a clinical diagnosis of INPH, but the accuracy of ELD is suspected. OBJECTIVE: To investigate proteomic cerebrospinal fluid (CSF) biomarker patterns in patients with INPH undergoing ELD to develop a quantitative diagnostic. METHODS: Twenty patients with suspected INPH underwent an ELD trial and the CSF biomarkers AB1-42, total tau, and tau phosphorylated at amino acid 181 (p-tau) were quantified with immunoassays in specimens taken prior to ELD placement, after the ELD trial, and from ventricular samples collected at the time of permanent ventriculoperitoneal shunt placement. RESULTS: CSF total tau was elevated, on average, in pre- and post-ELD samples from patients who failed to improve clinically during the ELD trial, but the findings were marginally significant after correction for multiple comparisons. AB1-42 and p-tau concentrations were not significantly different in patients who either did or did not clinically improve after the ELD. CONCLUSIONS: CSF total tau is a potential novel biomarker for suspected INPH patients who will clinically improve, or have clinically improved, after an ELD trial. The small sample size of this study, which was due to the relative rarity of this condition, indicates that larger studies are needed to confirm the utility of this approach.

Transanal presentation of a distal ventriculoperitoneal shunt catheter: Management of bowel perforation without laparotomy.

BACKGROUND: Bowel perforation is a serious but rare complication after a ventriculoperitoneal shunt (VPS) procedure. Prior studies have reported spontaneous bowel perforation after VPS placement in adults of up to 0.07%. Transanal catheter protrusion is a potential presentation of VPS bowel perforation and places a patient at risk for both peritonitis and ventriculitis/meningitis via retrograde migration of bacteria. This delayed complication can be fatal if unrecognized, with a 15% risk of mortality secondary to ventriculitis, peritonitis, or sepsis. CASE DESCRIPTION: We describe a unique case of a patient with distal VPS catheter protrusion from the anus whose bowel perforation did not cause clinical sequelae of infection. We were able to manage the patient without laparotomy. CONCLUSIONS: A subset of patients can be managed without laparotomy and only with externalization of the ventricular shunt with antibiotics until the cerebrospinal fluid cultures finalize without growth.

Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) as a Cognitive Evaluation Tool for Patients with Normal Pressure Hydrocephalus.

External lumbar drainage (ELD) is recognized as a screening method for ventriculo-peritoneal shunting (VPS) candidacy for possible normal pressure hydrocephalus (NPH). This study focused on the ELD predictability of the cognitive outcome after VPS for NPH. In addition, Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) was examined in ELD cognition screening. ELD results were considered positive with any improvement in gait and/or cognition. Among 36 patients examined for possible NPH, 26 underwent VPS because of positive ELD. Cognitive outcome after VPS was assessed at 6-month follow-up. The RBANS scores, examined pre- and post-ELD, were evaluated statistically to identify consistency with the neuropsychologist judgment and the predictability of cognitive outcome after VPS. Among 26 shunted patients, gait was improved in 24. Cognitive improvement was rated in 19, and there were 9 false negative and 5 false positive in ELD cognition screening. The neuropsychologist judgment in ELD cognition screening is most consistent with the RBANS score in delayed memory. The patients rated as improved in cognition after VPS had significantly lower RBANS scores pre-ELD in immediate memory and delayed memory. If both scores at pre-ELD were ≤ 80 (13 patients), all were rated as improved in cognition after VPS. ELD screening was highly predictive of clinical gait improvement but not of cognitive improvement after VPS for possible NPH. Particularly among patients with a positive ELD gait response, pre-ELD low RBANS scores in memory predicted cognitive improvement after VPS. RBANS seems effective in evaluating cognition for NPH.

Reinnervating the penis in spina bifida patients in the United States: ilioinguinal-to-dorsal-penile neurorrhaphy in two cases.

INTRODUCTION: Penile sensation is absent in some patients with myelomeningocele owing to the dysfunction of the pudendal nerve. Here, we describe the introduction of penile sensation via ilioinguinal-to-dorsal-penile neurorrhaphy in two patients with penile anesthesia due to neural tube defects. AIM: To establish penile sensation via ilioinguinal-to-dorsal-penile-nerve neurorrhaphy. METHODS: A 20-year-old and a 35-year-old male with L5/S1 myelomeningocele were both highly functioning and ambulatory, with intact ilioinguinal nerve distribution sensation but anesthesia of the penis and glans. They were sexually active and able to ejaculate antegrade. Both had high International Index of Erectile Function scores for confidence to achieve erection sufficient for intercourse. An incision was made from anterior superior iliac crest to the glans penis to expose the inguinal canal and ilioinguinal nerve. The ilioinguinal and dorsal penile nerve were transected and anastomosed. The anastomotic site was then wrapped in a hemostatic agent and a drain was left in place. For penile rehabilitation, both patients were instructed to stimulate the penis while looking at the genitalia to encourage redistribution of perceived sensation. MAIN OUTCOME MEASURES: Presence of erogenous penile sensation was tested by neurologic examination and patient feedback, and patients completed sexual health questionnaires. RESULTS: Both patients reported paresthesias of the groin with penile stimulation 1 month after surgery. Both patients are now 24 months postoperative and have erogenous sensation on the ipsilateral glans and shaft during intercourse. Neither patient has difficulty achieving or maintaining erections. CONCLUSIONS: We present two patients with dorsal penile reinnervation via the ilioinguinal nerve. Although nerve reinnervation has been used in urological procedures, this is the first description of an attempt to resupply penile sensation via the dorsal penile nerve in the United States with a minimum of 18 months follow-up. Early follow-up suggests successful neuronal remapping and regained sensation of the penis.

Complex pediatric cervical spine surgery using smaller nonspinal screws and plates and intraoperative computed tomography.

OBJECT: The treatment of craniocervical instability in children is often challenging due to their small spine bones, complex anatomy, and unique syndromes. The authors discuss their surgical experience with 33 cases in the treatment of 31 children (≤ 17 years of age) with craniocervical spine instability using smaller nontraditional titanium screws and plates, as well as intraoperative CT. METHODS: All craniocervical fusion procedures were performed using intraoperative fluoroscopic imaging and electrophysiological monitoring. Nontraditional spine hardware included smaller screw sizes (2.4 and 2.7 mm) from the orthopedic hand/foot set and mandibular plates. Twenty-three of the 33 surgical procedures were performed with intraoperative CT, which was used to confirm adequate position of the spine hardware and alignment of the spine. RESULTS: The mean patient age was 9.5 years (range 2-17 years). Eleven children underwent a posterior C1-2 transarticular screw fusion, 17 had an occipitocervical fusion, and 3 had a posterior subaxial cervical fusion. The follow-up duration ranged from 9 to 72 months (mean 53 months). All children demonstrated successful fusion at their 3-month follow-up visit, except 1 patient whose unilateral C1-2 transarticular screw fusion required a repeat surgery before proper fusion was achieved. Of the 47 C1-2 transarticular screws that were placed, 13 were 2.4 mm, 15 were 2.7 mm, 7 were 3.5 mm, and 12 were 4.0 mm. Eighteen of the 47 C1-2 transarticular screws were suboptimally placed. Eleven of these misplaced screws were removed and redirected within the same operation because these surgeries benefitted from the use of intraoperative CT; 6 of the 7 remaining suboptimally placed screws were left in place because a second surgery for screw replacement was not warranted. The other suboptimally placed C1-2 screw was replaced during a repeat operation due to failure of fusion. Use of intraoperative CT was invaluable because it enabled the authors to reposition suboptimal C1-2 transarticular screws without necessitating a second operation. CONCLUSIONS: Successful craniocervical fusion procedures were achieved using smaller nontraditional titanium screws and plates. Intraoperative CT was a helpful adjunct for confirming and readjusting the trajectory of the screws prior to leaving the operating room, which decreases overall treatment costs and reduces complications.

Nonprogrammable and programmable cerebrospinal fluid shunt valves: a 5-year study.

OBJECT: Programmable valves (PVs) for shunting CSF have increasingly replaced nonprogrammable valves (NPVs). There have been only a few longer-term studies (≥ 5 years) conducted that have compared the effectiveness of NPVs with that of PVs for children with hydrocephalus, and only 1 study has reported NPVs as being favorable over PVs. The objective of this retrospective study was to compare the long-term survival of these 2 types of shunt valves. METHODS: The authors collected data for all patients who underwent CSF shunt insertion or revision between January 1, 2000, and December 31, 2008. Patients underwent follow-up for a minimum of 2 years postoperatively. Statistical analyses were done using chi-square, Kaplan-Meier survival curve, and multivariate analyses. RESULTS: A total of 616 valves were implanted, of which 313 were PVs and 303 were NPVs. Of these, 253 were original shunt implantations and 363 were revisions. The proportion of 5-year survival for NPVs (45.8%) was significantly higher than that for PVs (19.8%) (p = 0.0005, log-rank). The NPVs that survived longer than 6 months also survived through the 5th year better than the PVs (p = 0.0001). CONCLUSIONS: The authors' data suggest that NPVs survive longer than PVs in children, but there is a need for prospective, case-control studies to confirm these data.

Symptomatic spinal cord compression from an intradural arachnoid cyst with associated syrinx in a child: case report.

Symptomatic intradural extramedullary arachnoid cysts in children are rare, and of the previously reported pediatric cases in the current literature, none to our knowledge were associated with a spinal cord syrinx. We describe an 8-year-old child who presented with paraparesis and regression of bowel and bladder control. An intradural extramedullary arachnoid cyst was identified on preoperative magnetic resonance imaging, with an associated spinal cord syrinx. We describe the preoperative imaging, surgical management, and clinical course of this patient, who had improvement in his paraparesis. This paper reviews relevant pediatric literature and the etiology of arachnoid cysts and associated spinal cord syrinx formation.

Endoscopic repair of a rare basioccipital meningocele associated with recurrent meningitis.

Anatomical variants of the basiocciput are uncommon and usually clinically benign. While the majority remain undetected, these anomalies rarely manifest as CSF rhinorrhea or recurrent meningitis associated with meningocele. Compromise of the leptomeninges provides an avenue of ingress for pathological organisms and can lead to recurrent meningitis, necessitating operative repair of the defect to prevent infection. A review of the literature reveals only 3 cases in which a congenital basioccipital defect has been associated with a meningocele requiring surgical repair. The authors present a case of recurrent meningitis in an infant with a congenital basioccipital meningocele treated with a minimally invasive endoscopic technique. At the 2-year follow-up the repair remained successful, with no evidence of recurrence of the meningocele or CSF infection. The literature regarding the etiology and treatment of these lesions was reviewed, with an emphasis on the safety and efficacy of the endoscopic approach. Note that recurrent meningitis in the setting of a skull base defect may indicate the presence of other congenital anomalies that will necessitate multidisciplinary care for a patient's long-term well-being.

Human motor evoked potential responses following spinal cord transection: an in vivo study.

Motor evoked potential (MEP) monitoring has been used increasingly in conjunction with somatosensory evoked potential monitoring to monitor neurological changes during complex spinal operations. No published report has demonstrated the effects of segmental spinal cord transection on MEP monitoring. The authors describe the case of an 11-year-old girl with lumbar myelomeningocele and worsening thoracolumbar scoliosis who underwent a T11-L5 fusion and spinal transection to prevent tethering. Intraoperative MEP and somatosensory evoked potential monitoring were performed, and the spinal cord was transected in 4 quadrants. The MEPs were lost unilaterally as each anterior quadrant was sectioned. This is the first reported case that demonstrates the link between spinal cord transection and MEP signaling characteristics. Furthermore, it demonstrates the relatively minor input of the ipsilateral ventral corticospinal tract in MEP physiology at the thoracolumbar junction. Finally, this study further supports the use of MEPs as a specific intraoperative neuromonitoring tool.