Search

Introduction: Recently, placement of a ventriculoperitoneal shunt via a transumbilical approach has been reported., Patients and Methods: Herein, we report the repair of an umbilical hernia via the same incision and introduction of the distal end of a ventricultoperitoneal shunt into the peritoneal cavity in 3 patients. A case illustration is included., Results: Both hernia repair and placement of the distal end of the ventriculoperitoneal shunt were uncomplicated in our small case series., Conclusions: To our knowledge, simultaneous repair of an umbilical hernia followed by transumbilical shunt placement has not been reported. As umbilical hernias are so common in infants, this finding, based on our experience, should not exclude placement of peritoneal tubing in the same setting., (© 2017 S. Karger AG, Basel.)

Mirror movements usually seen in the Klippel-Feil syndrome are most commonly appreciated in the upper extremities. Lower extremity involvement is seen rarely and when observed, is found in conjunction with upper extremity mirror movements. We report what we believe to be the first case of mirror movements found exclusively in the lower extremities in a female patient presenting with tethered cord syndrome. Our hopes are that this report will help elucidate mechanisms involved with these anomalous movements, as currently there is no commonly accepted etiology.

A neonate male born cesarian due to a breech presentation was noted to have no spontaneous movements of the limbs after delivery. Radiographs were not demonstrative of pathology. However, MRI revealed a large intraspinal mass with significant distortion of the cervicothoracic spinal cord. At operation, a brown, fibro-gelatinous, moderately adherent mass was evident extradurally dorsal to the spinal cord. It was noted to extend anterolaterally to the left such that the cord was deviated anteriorly and to the right. There was no indication of the mass being under pressure but the cord was not pulsatile. There was sufficient mass to the anterolateral component of the cord that it appeared rotated to the right within the canal. The right cervical roots exited dorsally, with a markedly lengthened course through the spinal canal before exiting above their respective pedicles. Histology was that of blood clot. The patient clinically demonstrated no neurologic improvement post-operatively. Now, six months after surgery, the patient has still had no significant change in clinical function. To our review, this is the first reported case of a spontaneous spinal epidural hematoma mimicking a birth-related spinal injury., (Copyright 2004 S. Karger AG, Basel)

There are scant data in the literature regarding distances from the midline to the suboccipital segment of the vertebral artery, and to our knowledge, none addressing this measurement in the pediatric population. This study seeks to establish age-related distances (Y) from the laterally placed vertebral artery to the midline at the level of the posterior arch of the atlas in children. Measurements from the midline to the most medial portion of the vertebral artery at the level of the posterior arch of the atlas were made in 50 children who underwent CT imaging of the cervical spine. For all left sides, Y = 12-23 mm (mean 17 mm). For all right sides, Y = 10-25 mm (mean 17.5 mm). For girls, Y = 12-16 mm (mean 12.7 mm) for left sides and Y = 12-25 mm (mean 18.5 mm) for right sides. For boys, Y = 12-20 mm (mean 16 mm) for left sides and Y = 10-21 mm (mean 16.7 mm) for right sides. We have found that the most medial portion of the vertebral artery from the midpoint of the posterior arch of the atlas for all ages was approximately 17 and 17.5 mm for left and right sides, respectively. This distance was generally less for left sides, except in the 16-19 years age group. However, as a group, this distance did not vary more than 2.8 mm between left and right sides. These data should assist the neurosurgeon who operates in the suboccipital region in children., (Copyright 2003 S. Karger AG, Basel)

The authors report a patient with type II split cord malformation (SCM) and Klippel-Feil syndrome (KFS). Considering our review of the medical literature and present case report, we believe lumbar SCM and KFS in the same patient not to be coincidental findings but rather embryologically related entities. We propose that KFS in at least one of its phenotypes represents a forme fruste of occult spinal dysraphism., (Copyright 2003 S. Karger AG, Basel)

Objective: We review our experience of endoscopic third ventriculocisternostomy (ETV) in children with hydrocephalus from central nervous system (CNS) infection or intraventricular hemorrhage to better elucidate success rates and predictors of success in these children., Methods: We performed a retrospective review of 12 children less than 21 years of age with ETV treated from 1999 to 2002 with a minimum follow-up of 12 months. Children selected for surgery had been diagnosed with hydrocephalus from perinatal germinal matrix hemorrhage or CNS infection and had neuroimaging consistent with obstruction of the aqueduct, fourth ventricular outlets or both at the time of ETV. Charts and imaging studies were reviewed to determine clinical outcomes and predictors of successful ETV. An ETV was deemed successful if the child did not require placement or replacement of a ventriculoperitoneal shunt., Results: Thirteen ETV were performed in 12 patients (7 boys and 5 girls; mean age 9.2 years, range 3.8-21 years), with an overall success rate of 60% for those with CNS infections and 71% for those with perinatal intraventricular hemorrhage at initial presentation. There were no significant complications from the procedure., Conclusions: ETV is a safe procedure, which in carefully selected children with etiologies of hydrocephalus thought to be 'communicating' in nature carries a satisfyingly high success rate. Further application and study of this modality in larger groups of patients with these causes of hydrocephalus is warranted., (Copyright 2003 S. Karger AG, Basel)

We report a pediatric patient who presented with hypogonadism and diabetes insipidus. Further evaluation revealed hypothyroidism. MRI of the brain revealed a suprasellar mass. Following frontal craniotomy for mass biopsy, histological analysis of the mass revealed benign lymphoid hyperplasia. The patient has had no progression of his mass or symptoms at most recent follow-up (10 months). We review this unusual case and other similar conditions. We believe this to be the first reported case of lymphoid hyperplasia without malignant cells of the suprasellar region., (Copyright 2003 S. Karger AG, Basel)

Introduction: Enlarged parietal foramina have historically been described as merely the normal parietal foramina (which usually transmit emissary veins connecting the occipital veins to the superior sagittal sinus) in which the diameter exceeds approximately 5.0 mm., Methods: We have observed well over 500 cadaveric wet and dry specimens for simultaneous bilateral enlarged and normal parietal foramina., Results: We have found two bony adult calvaria, which demonstrate both enlarged parietal 'foramina' and the normally appreciated parietal foramina., Conclusions: Although a rare occurrence, enlarged parietal foramina occur in conjunction with the normally sized and positioned parietal foramina. This dismisses earlier hypotheses that enlarged parietal foramina are merely parietal foramina that are increased in their diameter., (Copyright 2003 S. Karger AG, Basel)

We report a 2-week-old male with a large cystic mass in the left cervical and face region. No connection to the brain or spinal cord was discernable either at operation or on imaging. Histological study of this mass revealed central nervous tissue including neuroglia, ependymal cells, non-neuroblastic neurons, pigmented retinal epithelium, and choroid plexus. This case represents the rare ectopic 'brain'. We review this case as well as the literature concerning these unusual masses., (Copyright 2003 S. Karger AG, Basel)

We report two cases of children with occult spinal dysraphism who were also found to have many associated brain anomalies seen in the Chiari II malformation. No previous report has commented on the possible association between the Chiari II malformation and the 'closed' form of neural tube defect. One child had symptoms referable to pathology at the craniocervical junction. Neither child had cutaneous stigmata associated with occult spinal dysraphism over the caudal midline spine. These cases, although seemingly rare, lend support to the theories that the association between the Chiari II malformation and patients with myelomeningoceles is due to dysgenesis of the rostral and caudal neural tube. These cases are also important as refutation of earlier theories that link these two entities by proposing that the Chiari II malformation is due to overdrainage of cerebrospinal fluid at the site of myelomeningocele., (Copyright 2003 S. Karger AG, Basel)

The subgaleal shunt has been used for the temporary bypass of the normal cerebrospinal fluid (CSF) pathways. We retrospectively reviewed all subgaleal shunts placed at the Children's Hospital, Birmingham, Ala., USA, from 1997 to the present and examined all uses (e.g. indication, length of follow-up) of the subgaleal shunt outside its use for temporary CSF diversion in premature infants with intraventricular hemorrhage and subsequent hydrocephalus. The average length of survival of the primary subgaleal shunt in this population was 32.2 days. We have had good success with subgaleal shunts in children with malignant brain tumors, intraventricular abscesses, chronic truncal wounds, chronic subdural hygromas and meningitis. However, the greatest utility has been in those scenarios in which the peritoneal cavities were not currently, but with time would be, candidates for distal shunt implantation. Examples of these instances are patients with hydrocephalus and necrotizing enterocolitis or hydrocephalus and preoperative abdominal wall pathology such as omphalocele., (Copyright 2003 S. Karger AG, Basel)

Objective: The posterior fossa (PF) has been found to be small in various forms of Chiari malformation. Explanations involving a connection between growth hormone deficiency (GHD) and Chiari I malformation (CIM) have been proposed. However, to date, no quantitative analysis of the PF of patients with CIM and GHD has been performed. Our study was performed to determine the geometry of the PF in children with GHD and CIM., Methods: Morphometric analysis of the PF was performed in 10 children with GHD and CIM (group 1), 20 children with GHD and no CIM (group 2) and 50 controls., Results: PF volumes for group 1 ranged from 128 to 259 +/- 33 ml, and for group 2, they ranged from 115 to 186.2 +/- 25.4 ml. Lengths of the foramen magnum for groups 1 and 2 had means of 36 and 38 mm, respectively. The mean basiocciput length and tentorial angle for groups 1 and 2 were 20 and 19 mm and 89 and 87.5 degrees, respectively., Conclusions: We have determined that children with GHD with or without CIM have no significant difference in their PF volume compared to controls. However, our data demonstrate significant underdevelopment of portions of the bony PF in both patients with GHD alone and in patients with GHD and CIM. Tentorial angles were elevated in noncontrol groups. We propose that this association is not due to an increased rate of 'midline' defects seen in GHD but rather a structurally distorted PF that is not capacious enough to house the entire developing rhombencephalon. These data will hopefully aid in the further understanding of the pathophysiology of CIM., (Copyright 2003 S. Karger AG, Basel)

The subgaleal shunt has been and is currently used for the temporary bypass of the normal cerebrospinal fluid (CSF) pathways. We retrospectively reviewed all subgaleal shunts placed at the Children's Hospital, Birmingham, Ala., USA, from 1993 to the present and examined the life expectancy of revisions of this temporary method of CSF diversion. The average length of survival of primary, secondary and tertiary subgaleal shunts in this population was 37.4, 32.4 and 19.6 days, respectively. This current review demonstrates that the majority of patients in whom a subgaleal shunt is utilized may continue to obtain therapeutic diversion of their CSF for greater than 2.5 months with intermittent subgaleal shunt revisions. In the majority of cases, this length of time is sufficient to manage and resolve issues that would make ventriculoperitoneal shunting undesirable., (Copyright 2003 S. Karger AG, Basel)

We report two children with hemihypertrophy and Chiari I malformation (CIM) and review the extant medical literature regarding CIM and overgrowth disorders. We propose that these two entities do not represent a spurious association but rather share a common dysembryology of mesoderm., (Copyright 2003 S. Karger AG, Basel)

The posterior atlanto-occipital membrane (PAO) contributes little to craniocervical stability and is generally underappreciated and incised with most suboccipital craniectomies. We report a case of a child who underwent posterior fossa decompression for Chiari I malformation with syringomyelia whose symptoms had not resolved months after surgery. A secondary exploratory operation revealed a healed constricting PAO which had been incised linearly at the initial operation. This patient's symptoms improved soon after the second surgery. We propose that attention be given to this membrane, and if it is incised with underlying dura mater as in a standard posterior fossa decompression, it should be removed or cauterized separately and laterally to inhibit its reformation., (Copyright 2003 S. Karger AG, Basel)

Objective: To determine whether intrauterine myelomeningocele repair performed at between 20 and 28 weeks gestation improves lower extremity function (LEF)., Methods: Thirty-seven consecutive patients who had undergone intrauterine repair of their myelomeningocele at Vanderbilt University Medical Center had their lower extremity function and radiographic level (first defective vertebral level) compared to these same parameters in 40 controls who had undergone traditional postgestational repair of their myelomeningocele at the Children's Hospital in Birmingham, Ala., USA., Results: Of all 77 patients (controls and study group), 13 had a LEF that matched their radiographic level, 27 had a LEF that was rostral to their radiographic level, and 37 had a LEF that was caudal to their radiographic level. Further stratification revealed that for the intrauterine repaired myelomeningoceles, 11% had no difference between LEF and radiographic level, 43% had a LEF that was rostral to their radiographic level, and 46% had a LEF that was caudal to their radiographic level. For those closed in a traditional manner, LEF matched their radiographic level, was rostral to their radiographic level, and was caudal to their radiographic level in 22.5%, 27.5%, and 50% respectively. However, the overall mean differences between institutions produced a p-value of 0.2026 (paired t-test)., Conclusions: Although the current timing of intrauterine myelomeningocele repair has been found to lessen the degree of herniation of the rhombencephalon and reduce the incidence of shunt-dependent hydrocephalus, it does not statistically improve LEF. Parents should be advised of these findings prior to surgical intervention so as to focus their expectations., (Copyright 2003 S. Karger AG, Basel)

Objective: This study tests the hypothesis that the ability of Hering's nerve stimulation (HNS) to blunt seizure activity is dependent on the availability of dopamine in the amygdala., Methods: In 10 rats, Hering's nerve (HN) on the right side was isolated and placed on an electrode and penicillin was locally placed on each rat's left frontoparietal region to induce seizures. After the initiation of seizures, HN was stimulated. After the recurrence of seizure activity, the left basolateral amygdala was injected with 1.0 microl of normal saline, dopamine, haloperidol or 1% lidocaine in sequential tests. HN was stimulated after each injection and the latency and amplitude of the seizure activity were assessed., Results: Focal cortical penicillin induced seizures that resulted in tonic-clonic movement of the limbs and face that lasted 35-45 min. Tonic-clonic movements of the limbs and face of similar latency and amplitude were induced by repeated reapplication of penicillin in untreated rats. HNS decreased seizure activity, but infusion of haloperidol or lidocaine into the basolateral amygdala blocked this antiseizure effect of HNS. In contrast, infusion of saline or dopamine had no effect on the ability of HNS to blunt seizure activity. None of the amygdala injections altered the latency or amplitude of seizure activity., Conclusion: These results demonstrate that the ability of HNS to blunt seizure activity in the rat is dependent on an intact dopamine system in the basolateral amygdala. These data will hopefully be useful in furthering our understanding of the circuitry that allows peripheral nerve stimulation to alter seizure activity., (Copyright 2002 S. Karger AG, Basel)

Objective: We recently found that Hering's nerve stimulation (HNS) effectively blunts seizure activity. This study characterizes the cortical areas that are activated by HNS., Methods: Hering's nerve (HN) was stimulated in 3 dogs and 6 pigs, and then the brains of the animals were removed. The insular and mesial temporal cortices were removed and evaluated for increased neuronal activity by examining Fos-like activity., Results: In both the dogs and pigs, Fos activity was elevated in the anterior insular and mesial temporal cortices. Increased cortical activity was not noted in adjacent areas such as the frontal cortex., Conclusion: This study demonstrates that the mesial temporal and anterior insular cortices are selectively activated by stimulation of HN, thus suggesting cortical loci at which HNS might blunt seizure activity., (Copyright 2002 S. Karger AG, Basel)

Dolichoodontoid is defined as hypertrophy of the apical portion of the odontoid process of the axis. We report the first case of a dolichoodontoid process in a pediatric patient with other congenital anomalies. This rare entity has had three previous citations in the literature. Our patient presented with scoliosis in which subsequent MRI demonstrated a Chiari I malformation, a small thoracic syrinx and a dolichoodontoid process. The diagnosis of this malformation should be entertained in cases of craniocervical anomalies., (Copyright 2002 S. Karger AG, Basel)

There has been almost no evaluation or discussion of the symmetry of tonsillar ectopia in the medical literature. We measured the degree of left and right tonsillar herniation in 42 pediatric patients with a symptomatic Chiari I malformation and made clinical/radiological correlations. 18% of all patients with tonsillar asymmetry had clinical symptoms or physical findings referable to the inequality of their hindbrain herniation. In addition, 95% of patients with a coexisting syringomyelia had a right cerebellar tonsillar herniation greater than the left. We hypothesize that slight differences in posterior cranial fossa morphology may physically allow for greater unilateral herniation of the left or right cerebellar tonsil, which may be manifested in a patient's clinical presentation., (Copyright 2002 S. Karger AG, Basel)

Objective: To determine a solution capable of discerning adipose versus nervous tissue, to aid in surgical separation of the adipose tissue which appears to be visually indistinguishable from nervous tissue in lipomyelomeningoceles (LMMs)., Methods: The following solutes (in normal saline) were investigated, both at 25 and 37 degrees C: beta-carotene, vitamin D, vitamin E, lecithin, hydrogen peroxide, lipase, protease, hyaluronidase, partially purified collagenase, purified collagenase, trypsin, trypsin plus purified collagenase and non-solute-containing saline (control). Each solution was applied to a pediatric lipoma to determine gross effects over a period of approximately 30 min. If a solution appeared to affect the adipose tissue grossly, studies of functional in vivo sensory evoked and spontaneous potentials using that particular solution were conducted upon sheep spinal cord, nerve roots, dura and peripheral nerve. Additionally, histological studies were conducted to determine the effect of that solution upon adipose tissue, spinal cord, myelin, dura and nerve roots., Results: Of all solutions investigated, partially purified collagenase type 1 (T1C; Lot M0M4322, Code CLS-1, Worthington Biochemical Corporation, Lakewood, N.J., USA) at 37 degrees C was the most successful in grossly altering the consistency and appearance of adipose tissue. This change was more apparent over 20-30 min following application of the solution to the adipose tissue. Solutions not containing T1C did not show appreciable results; purified collagenase plus trypsin did not appear comparable or superior to T1C. No significant histological or functional change was noted when comparing the spinal cord, nerve rootlets, myelin, dura or peripheral nerve from the T1C-treated group versus normal (untreated) control groups., Conclusion: T1C appears to be a potentially effective solution for application during LMM surgery in the acute setting, and such use of an adjunct solution may significantly aid in the safe surgical resection of LMMs. Pending further research, this technique may be applied for other indications which require discernment or alteration of adipose versus nervous tissue., (Copyright 2002 S. Karger AG, Basel)

Objective: To determine the anatomical and surgical feasibility of placement of a stimulator around the nerve of Hering (HN; a branch of cranial nerve nine). We previously determined the success of HN stimulation in controlling epileptiform activity in a canine model study. The present study assessed the ability to extrapolate the ease of surgical dissection and surrounding anatomy to humans., Methods: Fifteen human cadavers (8 male, 7 female) were studied. The HN on each side was specifically evaluated in each specimen with respect to its presence, size, surgically accessible length below the mandibular angle, relationship to the internal carotid artery (ICA) and carotid bifurcation and relation to the common facial vein (CFV), thyroid cartilage (superior edge) and superior cervical ganglion (SCG)., Results: All 15 cadavers exhibited specimens of HN bilaterally, with the nerve diameter ranging from 0.5 to 1.5 mm (mean 1.0 mm); in relation to the ICA, the HN coursed anteromedially in 60%, anteriorly in 33% and anterolaterally in 7% of cases, and appeared to distinctly emerge at the level of the carotid bifurcation in all specimens. The surgically accessible length below the mandibular angle ranged from 6 to 30 mm (mean 15 mm). Relative to the carotid bifurcation, the superior edge of the thyroid cartilage was -35 to 33 mm (mean -5.4 mm) above; the CFV, which was clearly identifiable in only two thirds of the specimens, was -10 to 15 mm (mean 3.0 mm) above; and the inferiormost portion of the SCG was 6-30 mm (mean 16.6 mm) above., Conclusion: This study indicates that the HN was clearly present and surgically accessible in all human specimens. Based on the present study and our previous canine study, HN stimulation could be an important therapy for medically intractable epilepsy., (Copyright 2002 S. Karger AG, Basel)

The horizontal sacrum has never been described as an indicator of a tethered spinal cord following myelomeningocele closure. We retrospectively analyzed 30 children with a myelomeningocele for this progressive change and its correlation with symptoms. At least two lateral radiographs over time were examined and the lumbosacral angle (LSA) was measured. We found that the majority of these children had an LSA that was greater than would be expected in patients with late and decreased ambulatory abilities. Also, we observed that the LSA was often inappropriately increased at the time many of these patients presented with symptoms indicative of a tethered spinal cord. We hypothesize that the LSA in this group of children is altered by a tethered spinal cord and that the horizontal nature of the sacrum may predate the clinically appreciable symptoms of a tethered spinal cord., (Copyright 2002 S. Karger AG, Basel)

Introduction: The removal of the choroid plexus from the lateral ventricles was attempted by Dandy in the early 20th century but later discarded as complications arose and other methods of cerebrospinal fluid (CSF) diversion were introduced. We compare our experience with a variation of this operation to CSF diversion in patients with hydranencephaly or near hydranencephaly., Methods: The hospital and office charts of patients with a diagnosis of hydranencephaly were reviewed from the two institutions spanning the career of the senior author. Thirteen patients were identified, of whom 9 underwent CSF diversionary procedures (group A) and 4 underwent choroid plexectomy (group B)., Results: The mean number of reoperations (2 in group A, 0 in group B), neurosurgical readmissions (1.5 in group A, 0 in group B) and days of hospitalization related to neurosurgical readmissions (43.5 in group A, 0 in group B) were all less in patients who underwent choroid plexectomy. The total incidence of complications related to surgery was also lower in this group (7 in group A, 0 in group B)., Conclusion: In our experience, choroid plexectomy in patients with hydranencephaly reduces the incidence of reoperation and readmission, the number of days of hospitalization related to the surgical procedure and the total number of complications in comparison to patients undergoing CSF diversion. Further neurosurgical intervention is minimized as is the financial burden from multiple emergency department visits and radiological procedures for shunt evaluation. Choroid plexectomy is a viable alternative to CSF diversion in patients with hydranencephaly and a rapidly enlarging head. It avoids the chronic issues and complications surrounding CSF diversion in this difficult group of patients., (Copyright 2002 S. Karger AG, Basel)

Complications of posterior cranial fossa decompression for Chiari malformations are uncommon. We present three patients who developed infratentorial supracerebellar hygromas causing acute hydrocephalus after posterior cranial fossa decompression., (Copyright 2002 S. Karger AG, Basel)

Historically, there have been no accounts of an association between simple metopic ridging and the Chiari I malformation. We prospectively evaluated 50 children with simple metopic ridges and found that 30% of these patients had a Chiari I malformation. During the period in which this screening for simple metopic ridges was performed, only 9% of the total patient population (324) was diagnosed as having a Chiari I malformation. We hypothesize that small decreases in anterior fossa volume in children with a simple metopic ridge and no other clinical findings of trigonocephaly may increase their chance of having a Chiari I malformation that becomes symptomatic. In addition, simple metopic ridging should perhaps be added to the list of bony anomalies associated with the Chiari I malformation, such as atlantal assimilation and retroverted odontoid processes. Future studies aimed at calculating anterior fossa volumes in children with a Chiari I malformation and imaging all children with simple metopic ridges are now necessary to ascertain if there is an increased risk of these patients becoming symptomatic with a hindbrain hernia or having a hindbrain hernia, respectively., (Copyright 2001 S. Karger AG, Basel)

The purpose of this study is to determine whether there is a relationship between the conus level and patients with a Chiari I malformation. This retrospective MRI study evaluated conus levels and degrees of tonsillar ectopia in 26 children ages 5-16 years with a Chiari I malformation. Our results show that there seems to be no relationship between the conus level and the amount of tonsillar ectopia that a child with a Chiari I malformation might have. In fact, there seemed to be an inverse relationship between these two entities. Interestingly, we have also found that of all coni located at the L2-L3 disc space or below, these patients had a syrinx associated with their Chiari I malformation and that all malformations without a syrinx had a 'normally' positioned conus medullaris., (Copyright 2001 S. Karger AG, Basel.)

Neural tube defects (NTD) are one of the most common birth defects and are caused by both environmental and genetic factors. The approach to identifying the genes predisposing to NTD, through linkage analysis and candidate gene analysis, is reviewed along with characteristics of a large, nationally ascertained cohort of families. Results from specific assessments of p53, PAX3 and MTHFR failed to suggest that these genes play a major role in NTD development in these families. Advances in genetic laboratory and statistical techniques have made this a prime opportunity for investigation into the causes of complex disorders, such as NTD. However, traditional approaches may prove to be challenging due to the difficulty of ascertaining samplable multiplex families., (Copyright 2000 S. Karger AG, Basel)

Background: It has been postulated that intrauterine myelomeningocele repair might improve neurologic outcome in patients with myelomeningocele. A total of 59 such procedures have been performed at Vanderbilt University. Preliminary results suggested that the degree of hindbrain herniation is reduced by intrauterine repair. In an attempt to further quantify the possible benefits of this surgery, a subset of these patients was brought back to Vanderbilt for study., Methods: A group of 26 patients who had undergone intrauterine myelomeningocele repair underwent an extensive evaluation which included manual muscle testing, MR imaging and precise determination of the anatomic level of their lesions as well as multiple other tests. The results of this analysis were compared to those in 2 groups of historical controls., Results: In this group of patients intrauterine myelomeningocele repair substantially reduced the incidence of moderate to severe hindbrain herniation (4 vs. 50%). The incidence of shunt-dependent hydrocephalus was more modestly reduced (58 vs. 92%). The average level of leg function closely matched the average anatomic level of the lesion in both the fetal surgery and control groups., Conclusion: The most dramatic effect of intrauterine repair appears to be on hindbrain herniation. A less dramatic, but significant, reduction in shunt-dependent hydrocephalus is also seen. Prospective patients should be cautioned not to expect improvement in leg function as the result of this surgery. The potential benefits of surgery must be carefully weighed against the potential risks of prematurity.

The standard approach for sectioning of the filum terminale for a tethered spinal cord can be achieved via a limited S(1) exposure. This is performed with the commonly believed idea that the filum fuses with the dura at S(2). We dissected 27 cadavers to exclusively look at the level at which the filum pierces/fuses with the dura and also the level at which the dural sac ends. Most of the fila fused at S(2) with a range from L(5) to S(3). The majority of dural sacs ended at S(2) with a range from S(1) to S(3). However, 15% of the fila (4 of 27) fused above the S(1) level. In addition, 11% of the fila (3 of 27) fused off the midline. We hope that this anatomical information may be useful for neurosurgeons when standard approaches fail to identify the filum at its usual level and location.

Using a combined search of the Children's Hospital (Birmingham, Ala., USA) medical records and the Jefferson County Health Department death records, we reviewed all shunt-related deaths that occurred between January 1990 and July 1996. Of these, we excluded patients who died of nonhydrocephalus-related reasons, such as bronchopulmonary dysplasia, as well as patients who had other serious neurological illnesses such as brain tumor and hydranencephaly. Twenty-eight patients died of shunt-related causes in the 6.5-year period. A survival analysis showed that 96% survived 32 months after first shunting. Of 28 patients, 23 were beyond help prior to medical evaluation. However, at least 10 of these patients had symptoms suggestive of shunt failure at least 24 h and as long as 2 weeks prior to their demise. We conclude that hydrocephalic children still die of shunt failure despite the modern technology of the 1990s. Some of these causes may be avoidable through early detection of symptoms. Guidelines to patients, families, and primary caregivers should be emphasized.

A case of a mature posterior cervical teratoma resembling two fingers and a case of a lumbosacral limb are presented. These are at least the seventh and eighth reported paraspinous or occipital teratomas with recognizable extremity development. Both anomalies had intradural extension and required exploration to decrease the likelihood of future spinal cord tethering. Preoperative evaluation, surgical technique, postoperative follow-up, and review of the literature are presented.

Objective: The management results of pediatric brain tumors should have improved with progress in neurosurgery, intra- and perioperative care, and adjunctive therapy. We assessed the outcomes of 88 consecutive children under 2 years of age with brain tumors managed in the modern era, primarily by two neurosurgeons, and compared the results within the study group over time and to historical controls., Methods: Medical records were reviewed for diagnosis, location, surgery, surgical morbidity and mortality, adjunctive therapy, and long-term results. Outcomes were available on all 88 patients., Results: Primitive neuroectodermal tumors, astrocytomas, ependymoma, and choroid plexus papillomas in descending order of frequency accounted for two thirds of tumors. Supratentorial location predominated (60%), although in the 1- to 2-year group, there was a slight majority of infratentorial tumors. Surgical mortality and immediate morbidity were 9 and 26%, respectively, with substantial improvements in the last half of the series (2 and 16% with long-term morbidity of 11%). 53 of 88 (60%) of our patients are alive, and all children treated since January 1, 1994, except for 1 operative death, remain alive., Conclusion: Children less than 2 years of age remain a multidisciplinary challenge. Improved neuroimaging, surgical and pediatric intensive care management, and neuro-oncological care seems to have improved outcome both with respect to tumor control and neurological function. Aggressive surgery is possible with a good outcome generally expected. Hopefully this will set the stage for either surgical cure in children with benign tumors or combined surgical and adjunctive cure in patients with malignant tumors.

Congenital anomalies of the vertebral artery, including aneurysms and fenestrations, or partial duplication of the extracranial vertebral artery are rarely seen. Presented here is an unusual case of bilateral congenital anomalies of the extracranial vertebral arteries. The right vertebral artery demonstrated an aneurysm and the left a partial duplication. The aneurysm was felt to be the source of the embolic phenomenon to the posterior circulation. This was treated with detachable balloon catheter placement.

Perinatal spinal cord injury is a relatively uncommon, but a frequently misdiagnosed disorder. Often, the injury is not suspected and an erroneous diagnosis is made. We present five cases of perinatal spinal cord injury. In four, the referring physicians (including pediatric neurologists) misdiagnosed the condition. In view of these diagnostic difficulties, we review the clinical, radiographic and pathologic aspects of these injuries. Serious spinal injuries occasionally occur in the perinatal period. With improving medical care, many infants with less severe injuries are surviving the neonatal period. Therefore, the prompt recognition of neonatal spinal cord damage is essential and allows for optimal treatment of the injured child.

A series of 16 patients with chronic or subacute subdural hematomas treated with continuous external drainage of the subdural space is reviewed. Of these only 44% went on to require subdural-peritoneal shunt placement. There were no complications in treatment and no clinical or laboratory evidence of infection in any case. The outcome, measured by neurological examination, was not different between the shunted and nonshunted groups. In conclusion, continuous external drainage of the subdural space in chronic and subacute hematomas of infancy frequently is an effective, definitive treatment. This approach should be considered as the initial procedure prior to subdural-peritoneal shunting.

We have reviewed the presentation, diagnosis, management, and outcome of 43 consecutive patients with the Chiari I malformation who were evaluated over a 12-year period and were managed by a single surgeon. The delay from the time of presentation to that of diagnosis was significantly less in the pediatric group when compared to the adult group. Also, the delay in diagnosis before 1985 was significantly longer than after 1985, the year magnetic resonance imaging became widely available in clinical practice. We were able to show the patients who did not have hydrosyringomyelia were unlikely to develop scoliosis. Surgery resulted in overall improvement in both symptoms and signs, but the improvement in symptoms was more marked than the improvement in signs. There was positive correlation between improvement in hydrosyringomyelia and the improvement in signs and symptoms. Surgery was associated with no significant permanent injury as a result of the procedure in any of the patients. The effect of Chiari surgery on scoliosis was inconclusive.

A significant cohort of children with central nervous system tumors will fail initial therapeutic intervention and demonstrate recurrent disease. Treatment of children with these recurrent tumors requires a comprehensive multidisciplinary approach, with consideration of both histologic classification and anatomic location of the tumor. The choice of the specific therapeutic modality selected should reflect institutional experience and seek to advance the treatment of brain tumors in children. Children at Duke Medical Center with biologically indolent tumors and clinical or significant radiographic evidence of recurrence (low-grade/benign tumors with greater than 2-year recurrence-free interval) are usually treated by repeat surgical intervention. Children whose initial therapy did not include radiotherapy are considered for irradiation, while those previously irradiated are observed or occasionally considered for chemotherapy. Children with biologically aggressive tumors (high-grade lesions or early recurrence) are offered neurosurgical intervention if there is considerable mass effect, and subsequently enrolled on phase II chemotherapy protocols, reflecting an institutional commitment to exploring the role of this modality in the treatment of pediatric brain tumors. Children with recurrent brain tumors may derive benefit from this aggressive comprehensive approach, achieving a prolonged and quality survival.

Three patients with parietal cephaloceles underwent evaluation and treatment at Duke University Medical Center between 1984 and 1987. All presented within the first 2 years of life with painful swelling near the vertex of the head. All patients had skull films and computed tomography, and two underwent magnetic resonance imaging (MRI). All 3 children had associated hindbrain deformities; two with Dandy-Walker malformation, the third with a Chiari II malformation. Each child eventually developed hydrocephalus. MRI is the procedure of choice to evaluate these patients, providing direct sagittal imaging of the posterior fossa and craniocervical junction, and displaying communication of the cephalocele with intracranial structures as well as associated venous vascular anomalies.

Twelve patients with shunt infection occurring more than 6 months following shunt implantation or revision were identified and their charts reviewed. These cases were accumulated over a 9-year period, and delays from shunt surgery to detection were as long as 11 years. For 5 patients, no antecedent infection or surgery could be identified as a presumptive cause of the shunt infection. Propionibacterium species and Staphylococcus epidermidis species were the most common organisms identified. The risk of late onset of infection at this institution is less than 1% per year.