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.

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.

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.

Utility of Diffusion Tensor Imaging in Preterm Infants with Germinal Matrix Hemorrhage and Intraventricular Hemorrhage: A Systematic Review.

OBJECTIVE: Intraventricular hemorrhage (IVH) and germinal matrix hemorrhage (GMH) are the most common brain injuries in preterm infants. Neonates with these injuries are at greater risk of impaired neurodevelopmental outcome. Current guidelines recommend screening infants with cranial ultrasound (CUS); however, this is prone to missing subtle injury patterns, particularly within the posterior fossa. The present report sought to discuss the utility of diffusion tensor imaging (DTI) in preterm infants. METHODS: A systematic review of PubMed, Ovid MEDLINE, and Ovid EMBASE was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Included manuscripts were methodically scrutinized for quality, DTI use, and neurologic outcome. RESULTS: Twenty studies with 1574 infants who underwent DTI were included. There were 574 preterm infants with GMH-IVH on DTI. Twelve studies documented decreased fractional anisotropy, whereas 6 demonstrated structural segregation and asymmetrical white matter myelination in these infants. Seven studies documented concurrent CUS use with 2 studies comparing DTI findings with CUS findings. In both studies, DTI more accurately detected presence of GMH, especially within the cerebellum. Among GMH-IVH preterm infants, 58.5% demonstrated cognitive, intellectual, and language delays at follow-up (mean, 32.4 months). Additionally, lower fractional anisotropy values on initial DTI were associated with cognitive, language, and motor delays. CONCLUSIONS: Although DTI is more sensitive for picking up subtle injury patterns, CUS remains the standard of care when screening for injuries that would necessitate surgical intervention. DTI offers a refined understanding of the sequelae of GMH-IVH with microstructural changes found on DTI being associated with childhood motor and cognitive outcomes.

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.

A survey of the members of the American Society of Pediatric Neurosurgeons and factors associated with well-being.

OBJECTIVE: The goal of this study was to survey the members of the American Society of Pediatric Neurosurgeons (ASPN) to assess the prevalence and associated risks of burnout among pediatric neurosurgeons. The authors aimed to identify the factors that most significantly contributed to this risk to provide a baseline group of characteristics to improve physician well-being. METHODS: Institutional Review Board approval from the University of Arizona was obtained, and the 7-question and 9-question Mayo Physician Well-Being Index (WBI) was distributed to members of the ASPN (n = 275). This index screens for many different aspects of distress for physicians, including burnout risk, stress, depression, fatigue, suicidal ideation, and low career satisfaction. RESULTS: An analysis of 111 pediatric neurosurgeons (111/275 [40% response rate]) was completed. Respondent ages were distributed, with those aged 56-60 years representing the highest proportion (20%). University practice represented a majority (72%). One-third (32%) of respondents reported practicing greater than 25 years, and most physicians in the survey were married (76%). One-third of surgeons spend 61-70 hours working per week (33%), and a plurality are on call between 6 and 10 days per month (42%). Most surgeons reported treating fewer than 200 cases per year (37% reported 100-150 cases; 23%, 151-200). Most pediatric neurosurgeons (63%) stated their annual salary was sufficient. Analysis of each WBI question was performed to identify which factors specifically contributed to the risk of burnout. An overwhelming majority of respondents reported that they make significant efforts to do at least one thing each week that brings them joy (97%), and they either agree or strongly agree that they perform meaningful work (98% of all participants, 97% of females, and 98% of men, p = 0.010). Nearly half of all respondents (49%) reported feelings of burnout and a majority of them were female (67% of women and 42% of men, p = 0.021). Time, environment, case volumes, and quality-of-life concerns are all factors that significantly contribute to the overall risk of burnout and well-being. CONCLUSIONS: This survey study of the ASPN membership revealed a 49% rate of burnout with females at higher risk (67%). Factors associated with burnout were salary, more than 10 days on call per month, electronic medical record stressors, and work-life incongruity. The aforementioned notwithstanding, respondents believe pediatric neurosurgery is a meaningful career. This study provides evidence supporting a moral imperative toward recognition of burnout symptoms and a pivot point toward implementing change.

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.

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.

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.

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.

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.

Is postoperative CT scanning predictive of subdural electrode placement complications in pediatric epileptic patients?

AIMS: To understand the reliability of postoperative CT scans to predict the development of intracranial hemorrhagic complications associated with subdural electrode implants for monitoring intractable seizure, we reviewed the data of a consecutive series of children treated at our institution. METHODS: Forty children (mean age: 11.4 years) with subdural electrode implants were reviewed. The immediate postoperative CT scans were evaluated for the presence of hemorrhagic complications and/or brain swelling resulting in a midline shift. RESULTS: Twenty-six patients (65%) presented a postoperative midline shift (range = 2-10 mm; mean shift = 4.0 mm). Two children had a midline shift of >5 mm. Two patients with a shift of <5 mm at the first CT scan required a repeat craniotomy. These patients experienced worsening neurologic symptoms in a delayed fashion on postoperative days 1 and 4, respectively. This was correlated to an increase in midline shift of >5 mm. CONCLUSIONS: Subdural electrode implants in children are safe. The presence of a midline shift of <5 mm is common postoperatively. The presence and extension of the midline shift at the first CT scan does not seem to be predictive of the development of symptomatic complications with a mass effect. Complications happened in a delayed fashion.

Radiographic formation and progression of cervical syringomyelia in a child with untreated Chiari I malformation.

We present the case of a 17-month-old girl with an untreated Chiari I malformation who developed radiographic progression of syringomyelia over a 23-month period. The patient presented with initial symptoms of airway difficulties and gait ataxia, which did not progress clinically over this period of observation, despite recommendations for surgical decompression. At 17 months, there was a region of T(2) hyperintensity which progressed to become a syrinx within 3 months, enlarging over serial imaging studies. This is the first published report of radiographic progression from a 'presyrinx' state of T(2) hyperintensity to syringomyelia in a pediatric patient with untreated Chiari I malformation.

Cerebrospinal fluid shunting in children on renal dialysis. Report of two cases.

The concurrent need for both cerebrospinal fluid shunting for hydrocephalus and dialysis for end-stage renal disease is rare. No large case series are found in the literature. Because dialysis involves frequent access to either the peritoneal cavity or the venous system, concern over potential cerebrospinal shunt infection represents a point of concern. We present 2 cases in which patients underwent both dialysis and cerebrospinal fluid shunting. In one case, a child underwent ventriculoperitoneal shunting, while in the second case ventriculoatrial shunting was performed as the initial procedure. Management strategies and potential complications are discussed.

Meningiomas in children.

OBJECTIVE: Pediatric meningioma is a rare diagnosis. This retrospective review seeks to elucidate pertinent characteristics of pediatric patients presenting with meningioma. METHODS: The Seattle Children's Hospital and Regional Medical Center brain tumor database was surveyed from 1940 through 2004. Twenty patients were identified. RESULTS: The median age of the 20 patients was 13 years. Five patients had radiation-induced meningiomas. Five patients had neurofibromatosis type 2. Ten patients had spontaneously arising meningiomas, 2 of which were malignant. CONCLUSION: Patients with spontaneously arising meningiomas were younger than those with identified risk factors. There was no recurrence in patients with radiation-induced meningiomas. The only death occurred in a patient with a malignant meningioma.

Cervical spinal cord injury in abused children.

Five infants and toddlers who sustained cervical spinal cord injury as the result of child abuse are described. Three cases are previously unreported. Diagnosis was complicated by coexistent brain injuries and their treatments, subtle and/or evolving paralysis, and central cord syndrome, in which arm function is diminished but leg function is preserved. Definitive spinal imaging by magnetic resonance imaging (MRI), computed tomography, and plain radiographs was delayed because of life support efforts. When completed, the MRI was most sensitive to cord injury. Evidence of associated bony spinal injury was often absent or unapparent until healing occurred; 4 children had spinal cord injury without (or with minimal) radiological abnormality. The 3 children presenting to our hospital with cord injury represent 1% of the estimated cases of inflicted head injury seen during a 23-year period.

Epidural metastases from endodermal sinus tumor arising from benign sacral teratoma. Case report and review of the literature.

The recurrence of benign sacral teratomas is a small but significant possibility. Recurrence as an endodermal sinus tumor (EST) with epidural metastases, however, has not been previously reported. The authors describe a case of a mature sacrococcygeal teratoma in a 4-day-old female patient that recurred after 22 months as an EST with epidural metastases. The child presented with abdominal pain, urinary retention, and difficulty walking. On imaging, a large pelvic mass and an epidural spinal mass were revealed. The patient's alpha-fetal protein (AFP) level was 68,000 ng/ml. Her neurological examination was significant for 3/5 plantar and dorsiflexion strength bilaterally. The patient underwent L-3, L-4, and L-5 bilateral laminectomies followed by subtotal resection of the recurrent pelvic tumor. Pathological testing of samples of both the recurrent pelvic and the extradural spinal tumors led to a diagnosis of EST. The patient underwent four cycles of chemotherapy with normalization of her AFP level to 13 ng/ml. In the weeks that followed, her AFP level steadily rose again to 167 ng/ml. Follow-up imaging revealed no tumor recurrence. The patient underwent a second course of chemotherapy followed by two tandem courses of high-dose chemotherapy with autologous stem-cell rescue. Since completing this therapy the patient has been clinically stable with an AFP level of 1.3 ng/ml for 14 months. At the 1-year follow-up examination, her plantar and dorsiflexion strength had markedly improved to 4+/5 bilaterally. To the authors' knowledge, this is the first case of a mature sacrococcygeal tumor that recurred as an EST and caused spinal canal compromise.

Apnea caused by mesial temporal lobe mass lesions in infants: report of 3 cases.

Apnea as an isolated manifestation of seizures is well described in neonates but is only occasionally observed in infants. This article reports 3 infants presenting with apneic events as the main manifestation of seizure, documented by video electroencephalogram, all with mesial temporal lobe lesions. The 3 infants, after normal pregnancy and delivery at term, showed the first apnea at the age of 4 to 10 months. Although the interictal electroencephalogram result was often normal, ictal video electroencephalogram in all infants showed focal rhythmic discharges with or without generalization. Magnetic resonance imaging showed an inferior mesial temporal lobe mass lesion in all infants. All patients underwent lesion removal, and they are seizure free with normal psychomotor development.

Implications of a neurosurgical intervention in a patient with a surgically repaired hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) accounts for 4-9% of congenital heart disease in children. The mortality rate among children with HLHS undergoing cardiac repair is well documented, but comparable data for noncardiac surgical procedures are not well known. Medically intractable epilepsy is one of those problems, which arises as a complication of cardiovascular surgery in HLHS and necessitates neurosurgical intervention. There is no published knowledge about neurosurgical procedures performed in children with HLHS in the English literature. Thus, we present a 10-year-old boy who developed medically intractable epilepsy after cardiac surgery for HLHS. The aim of this study is to outline the pre-, intra- and postoperative precautions needed for neurosurgical intervention in HLHS patients to decrease morbidity and mortality.

Spontaneous subdural fluid collections following transection of a fatty filum terminale: case report and review of the literature.

We present the case of a 6-year-old girl who developed bilateral subdural fluid collections following transection of her fatty filum terminale. The patient presented to our emergency department 3 weeks subsequent to surgery, reporting symptoms of headache, nausea, and vomiting. The presence of bilateral subdural fluid collections was confirmed by head computerized tomography. Subdural fluid collections and hematomas have been associated with intracranial hypotension and excessive cerebrospinal fluid leakage; however, there are relatively few cases of subdural fluid collections/hematomas following spine surgery reported in the literature. To our knowledge, this is a unique description of development and resolution of subdural fluid collections following surgical transection of a fatty filum terminale.