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Matthew E. Fewel and Hugh J. L. Garton

✓ Migration of distal ventriculoperitoneal shunt tubing is known to occur in a wide of variety of locations. The authors report an unusual complication involving a previously confirmed intraperitoneal shunt catheter that migrated into the heart and pulmonary vasculature. Radiographic evidence suggested that this occurred secondary to cannulation of a segment of the external jugular vein with a shunt trochar during tunneling of the distal catheter. This is the sixth reported case of a peritoneal shunt tube migrating proximally into the heart.

The authors review the literature regarding migration of distal tubing into the heart and pulmonary artery. Based on imaging studies obtained in the present case, the authors posit that the mechanism for this unusual type of shunt migration is inadvertent penetration of either the internal or external jugular vein during the initial tunneling procedure. Negative intrathoracic pressure and slow venous flow then draws the catheter out of the peritoneum and into the vasculature. The distal catheter then migrates into the right side of the heart and pulmonary artery. Diagnosis and management of this type of complication is discussed.

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Jacob R. Joseph, Brandon W. Smith and Hugh J. L. Garton

Blunt prenatal trauma is known to have consequences to the developing brain, and can result in subdural hematoma (SDH) or epidural hematoma (EDH). The authors present a case of blunt prenatal trauma resulting in a fetal SDH, intraparenchymal hematoma, and intraventricular hemorrhage, and perform a systematic review of the literature. This systematic review was conducted according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Relevant studies (up to April 2016) that reported on cases of fetal SDH or EDH after blunt prenatal trauma were identified from the PubMed database. The primary outcome was fetal mortality, and the secondary outcome was neurological outcome. Fourteen studies were included in the analysis, comprising a total of 14 patients including the present case. The average gestational age at discovery of hemorrhage was 30.1 weeks. Nine mothers were in a motor vehicle collision and 3 were assaulted; the mechanism of injury for 2 mothers was not defined. Twelve patients had SDH, 1 had EDH, and 1 had conflicting reports. Three patients had intrauterine fetal demise, and 3 died in the neonatal period after birth. Three patients had persistent neurological deficit, and 5 were neurologically intact. Fetal SDH or EDH after blunt trauma to the mother trauma is rare and is associated with mortality. However, a significant number of patients can have good neurological outcomes.

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Jennifer Strahle, Andrew J. Odden, Cormac O. Maher and Hugh J. L. Garton

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Hugh J. L. Garton, Paul Park and Stephen M. Papadopoulos

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Zhe Guan, Todd Hollon, J. Nicole Bentley and Hugh J. L. Garton

Epidermoid cysts (ECs) are uncommon pediatric tumors that often occur in the cerebellopontine angle. Although cyst rupture is a recognized complication, the radiographic evolution of an EC following rupture and the resultant parenchymal brainstem edema have not been reported. The authors present the case of a 13-year-old female with a newly diagnosed cerebellopontine angle EC who presented with worsening headaches, photophobia, and emesis. Magnetic resonance imaging demonstrated significant pericystic brainstem edema and mass effect with effacement of the fourth ventricle. Refractory symptoms prompted repeat imaging, revealing cyst enlargement and dense rim enhancement. Resection of the EC resolved both her symptoms and the brainstem edema. This case documents the radiographic evolution of EC rupture and subsequent clinical course.

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Brandon W. Smith, Jennifer Strahle, Erick Kazarian, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

OBJECT

It is unclear if there is a relationship between Chiari malformation Type I (CM-I) and body mass index (BMI). The aim of this study was to identify the relationship between BMI and cerebellar tonsil position in a random sample of people.

METHODS

Cerebellar tonsil position in 2400 subjects from a cohort of patients undergoing MRI was measured. Three hundred patients were randomly selected from each of 8 age groups (from 0 to 80 years). A subject was then excluded if he or she had a posterior fossa mass or previous posterior fossa decompression or if height and weight information within 1 year of MRI was not recorded in the electronic medical record.

RESULTS

There were 1310 subjects (54.6%) with BMI records from within 1 year of the measured scan. Of these subjects, 534 (40.8%) were male and 776 (59.2%) were female. The average BMI of the group was 26.4 kg/m2, and the average tonsil position was 0.87 mm above the level of the foramen magnum. There were 46 subjects (3.5%) with a tonsil position ≥ 5 mm below the level of the foramen magnum. In the group as a whole, there was no correlation (R2 = 0.004) between BMI and cerebellar tonsil position.

CONCLUSIONS

In this examination of 1310 subjects undergoing MRI for any reason, there was no relationship between BMI and the level of the cerebellar tonsils or the diagnosis of CM-I on imaging.

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Editorial

Tethered cord release

Joseph H. Piatt Jr.

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Jennifer Strahle, Béla J. Selzer, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

Object

The authors investigated the effect of a tablet computer on performance-level settings of a programmable shunt valve.

Methods

Magnetic field strength near the tablet computer with and without a cover was recorded at distances between 0 and 100 mm. Programmable valves were exposed to the tablet device at distances of less than 1 cm, 1–2.5 cm, 2.5–5 cm, 5–10 cm, and greater than 10 cm. For each distance tested, the valves were exposed 100 times to the tablet with the cover, resulting in 500 total valve exposures. The tablet alone, without the cover, was also tested at distances of less than 1 cm for 30 valve exposures. Changes in valve performance-level settings were recorded.

Results

The maximum recorded magnetic flux density of a tablet with a cover was 17.0 mT, and the maximum recorded magnetic flux density of the tablet alone was 7.6 mT. In 100 exposures at distances between 0 and 1 cm, 58% of valves had different settings following exposure. At distances greater than 1 cm but less than 2.5 cm, 5% of valves in 100 exposures had setting changes. Only a single setting change was noted in 100 exposures at distances greater than 2.5 cm but less than 5 cm. No setting changes were noted at distances greater than 5 cm, including 100 exposures between 5 and 10 cm, and 100 exposures of more than 10 cm. For the 30 valve exposures to the tablet without a cover, 20 valve performance-level changes (67%) were noted.

Conclusions

Based on these results, exposure to tablet devices may alter programmable shunt valve settings.

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Wajd N. Al-Holou, Karin M. Muraszko, Hugh J. Garton, Steven R. Buchman and Cormac O. Maher

Object

After primary repair of a myelomeningocele or a lipomyelomeningocele, patients can present with symptoms of secondary tethered cord syndrome (TCS). After surgical untethering, a small percentage of these patients can present with multiple repeat TCS. In patients presenting with secondary or multiple repeat TCS, the role as well the expected outcomes of surgical untethering are not well defined.

Methods

Eighty-four patients who underwent spinal cord untethering after at least 1 primary repair were retrospectively evaluated using scaled and subjective outcome measures at short-term and long-term follow-up visits. Outcomes were analyzed for predictive measures using multivariate logistic regression.

Results

Surgical untethering was performed in 66 patients with myelomeningoceles and 18 patients with lipomyelomeningoceles. Fourteen patients underwent multiple repeat spinal cord untethering. Patients were followed up for an average of 6.2 years. Most patients had stability of function postoperatively. Motor function and weakness improved in 7 and 16% of patients at 6 months, respectively, and 6 and 19% of patients at long-term follow-up evaluation, respectively. Of the patients who presented with back pain, 75% had improvement in symptoms at 6 months postoperatively. Younger age at untethering was significantly associated with worse long-term neurological outcomes. The number of previous untethering procedures, original diagnosis, sex, anatomical level, and degree of untethering had no effect on surgical outcomes.

Conclusions

Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.

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Paul Steinbok, Hugh J. L. Garton and Nalin Gupta

Object

Tethered cord syndrome (TCS) is associated with a number of congenital anomalies involving early development of the spinal cord. These include myelomeningocele, spinal cord lipoma, low-lying conus medullaris, and a fibrofatty terminal filum. Occult TCS occurs in patients when clinical features indicate a TCS but the typical anatomical abnormalities are lacking. It is controversial whether surgical release of the terminal filum leads to clinical improvement in a patient who does not have a previously identified anatomical abnormality. To assess the clinical standard used by practicing pediatric neurosurgeons, a practice survey was conducted at the 2004 Annual Meeting of the Joint Section for Pediatric Neurological Surgery of the American Association of Neurological Surgeons/Congress of Neurological Surgeons.

Methods

The survey examined clinical decision making for a same-case scenario with differing appearance on imaging studies. There was a clear consensus regarding diagnosis and treatment in the patient with symptoms, a low-lying conus medullaris, and a fatty terminal filum. The vast majority of respondents (85%) favored surgical untethering for this patient. A majority of respondents (67%) also favored treatment for the patient having symptoms and a fatty terminal filum. There was, however, significant disagreement regarding the diagnosis and treatment of disease in one patient with symptoms and an inconclusive magnetic resonance imaging study. Some respondents clearly favored surgery, whereas others believed that this patient did not meet the diagnostic criteria for TCS.

Conclusions

The results of this survey support the development of a randomized clinical trial to address the benefit of surgery for occult TCS.