Tae Sung Park
David M. Katz, Jonathan D. Trobe, Karin M. Muraszko and Robert C. Dauser
✓ Four patients who developed increased intracranial pressure from ventricular shunt failure suffered a delay in diagnosis because magnetic resonance imaging of the brain did not show ventriculomegaly and because ophthalmic findings were initially overlooked or misinterpreted. None of the patients had the conventional manifestations of shunt failure: severe headache, nausea, vomiting, and depressed consciousness. Three patients suffered marked, permanent vision loss from chronic papilledema. These cases affirm that increased intracranial pressure may occur in shunt dependency without producing either conventional clinical symptoms or signs on imaging of the brain. Because ophthalmic manifestations may be the major clues to diagnosis, and because irreversible loss of vision is possible if these clues are overlooked, consideration should be given to periodic ophthalmological examination of shunt-dependent patients.
Celia C. D'Errico, Hamish M. Munro, Steven R. Buchman, Deborah Wagner and Karin M. Muraszko
Object. This prospective, randomized, placebo-controlled, double-blind trial was undertaken to assess the efficacy of aprotinin in reducing the need for blood transfusions in 39 children undergoing reconstructive craniofacial surgery.
Methods. Two demographically similar groups—a total of 39 patients with a mean age of 1.2 ± 1.2 years—were studied. The efficacy of aprotinin (240 mg/m2 administered intravenously over 20 minutes, followed by infusions of 56 mg/m2/hr) was compared with that of an equal infusion of 0.9% saline (placebo).
Patients in the aprotinin group received less blood per kilogram of body weight than patients in the placebo group (32 ± 25 ml/kg compared with 52 ± 34 ml/kg, respectively; p = 0.04). Those patients in whom aprotinin was administered experienced less change in their hematocrit levels during surgery (aprotinin −33 ± 13% compared with placebo −44 ± 9%, p = 0.01). Each patient underwent a transfusion as per study protocol, and there was no significant change in hematocrit levels from the beginning to the end of surgery. The surgical faculty judged blood loss in patients in the aprotinin group to be significantly less than usual (p = 0.03). The use of aprotinin was also associated with reduced blood transfusion requirements during the first 3 postoperative days (p = 0.03). There was no adverse event reported in either the aprotinin or placebo group.
Conclusions. Aprotinin decreased blood transfusion requirements in pediatric patients undergoing craniofacial reconstruction, thereby reducing the risks associated with exposure to banked blood components.
Ryszard M. Pluta, Alois Zauner, Jay K. Morgan, Karin M. Muraszko and Edward H. Oldfield
✓ Although proliferative arteriopathy has been postulated to play a role in the etiology of vasospasm after subarachnoid hemorrhage (SAH), histological and morphological studies examining cerebral vasospasm have produced conflicting results. To help settle this controversy, the authors used an in vivo label of cell division, bromodeoxycytidine, to assess cell proliferation in a primate model of SAH.
Fifteen cynomolgus monkeys received a clot of either whole blood (11 animals) or red blood cells (four animals) placed around the right middle cerebral artery (MCA). On the day of surgery continuous intravenous infusion of bromodeoxycytidine was begun and continued until the animal was sacrificed immediately after arteriography on Day 7, 12, or 27 following surgery. Sections from the right and left MCA's were stained with a monoclonal antibody against bromodeoxcytidine, and labeled cells were counted.
Arteriographic evidence of vasospasm occurred in nine monkeys on Day 7. On Day 12 and Day 27 no monkeys had persistent vasospasm. Placement of subarachnoid clot around the right MCA increased proliferative activity across all layers of the arterial wall. Most of the labeled cells were in the adventitia and the endothelium. Although there were more dividing cells in all layers of the right MCA than the left MCA (p < 0.01), the number of stained cells per section was limited (range 0.1 to 21.2, mean 8) and the occurrence of vasospasm was not associated with the number of dividing cells in the right MCA on Day 7, 12, 27, or for all days combined (p > 0.6).
Cerebral vasospasm after SAH was not associated with the extent of proliferation of cells in the vessel wall, nor could the intensity of the limited proliferative changes have been responsible for narrowing of the vessel diameter.
John M. Mesa, Frank Fang, Karin M. Muraszko and Steven R. Buchman
Successful surgical repair of unicoronal plagiocephaly remains a challenge for craniofacial surgeons. Many of the surgical techniques directed at correcting the stigmata associated with this craniofacial deformity (for example, ipsilateral supraorbital rim elevation [vertical dystopia], ipsilateral temporal constriction, C-shaped deformity of the face, and so on) are not long lasting and often result in deficient correction and the need for secondary revision surgery. The authors posit that the cause of this relapse was intrinsic deficiencies of the current surgical techniques. The aim of this study was to determine if correction of unilateral coronal plagiocephaly with a novel hypercorrection surgical technique could prevent the relapse of the characteristics associated with unicoronal plagiocephaly.
The authors performed a retrospective analysis of 40 consecutive patients who underwent surgical repair of unicoronal plagiocephaly at their institution between 1999 and 2009. In all cases, the senior author (S.R.B.) used a hypercorrection technique for surgical reconstruction. Hypercorrection consisted of significant overcorrection of the affected ipsilateral frontal and anterior temporal areas in the sagittal and coronal planes. Demographic, perioperative, and follow-up data were collected for comparison. The postsurgical appearance of the forehead was documented clinically and photographically and then evaluated and scored by 2 independent graders using the expanded Whitaker scoring system. A relapse was defined as a recurrence of preoperative features that required secondary surgical correction.
The mean age of the patients at the time of the operation was 13 months (range 8–28 months). The mean follow-up duration was 57 months (range 3 months to 9.8 years). The postsurgical hypercorrection appearance persisted on average 6–8 months but gradually dissipated and normalized. No patients exhibited a relapse of unicoronal plagiocephalic characteristics that required surgical correction. In all cases the aesthetic results were excellent. Only 3 patients required reoperation for the management of persistent calvarial bone defects (2 cases) and removal of a symptomatic granuloma (1 case).
Our study demonstrates that patients who undergo unicoronal plagiocephaly repair with a hypercorrection surgical technique avoid long-term relapse. Our results suggest that the surgical technique used in the correction of unilateral coronal synostosis is strongly associated with the prevention of postsurgical relapse and that the use of this novel method decreases the need for surgical revision.
Jennifer Strahle, Béla J. Selzer, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher
The authors investigated the effect of a tablet computer on performance-level settings of a programmable shunt valve.
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.
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.
Based on these results, exposure to tablet devices may alter programmable shunt valve settings.
D. Andrew Wilkinson, Kyle Johnson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher
The goal of this analysis was to define temporal and geographic trends in the surgical treatment of Chiari malformation Type I (CM-I) in a large, privately insured health care network.
The authors examined de-identified insurance claims data from a large, privately insured health care network of over 58 million beneficiaries throughout the United States for the period between 2001 and 2014 for all patients undergoing surgical treatment of CM-I. Using a combination of International Classification of Diseases (ICD) diagnosis codes and Current Procedural Terminology (CPT) codes, the authors identified CM-I and associated diagnoses and procedures over a 14-year period, highlighting temporal and geographic trends in the performance of CM-I decompression (CMD) surgery as well as commonly associated procedures.
There were 2434 surgical procedures performed for CMD among the beneficiaries during the 14-year interval; 34% were performed in patients younger than 20 years of age. The rate of CMD increased 51% from the first half to the second half of the study period among younger patients (p < 0.001) and increased 28% among adult patients between 20 and 65 years of age (p < 0.001). A large sex difference was noted among adult patients; 78% of adult patients undergoing CMD were female compared with only 53% of the children. Pediatric patients undergoing CMD were more likely to be white with a higher household net worth. Regional variability was identified among rates of CMD as well. The average annual rate of surgery ranged from 0.8 surgeries per 100,000 insured person-years in the Pacific census division to 2.0 surgeries per 100,000 insured person-years in the East South Central census division.
Analysis of a large nationwide health care network showed recently increasing rates of CMD in children and adults over the past 14 years.
Wajd N. Al-Holou, Karin M. Muraszko, Hugh J. Garton, Steven R. Buchman and Cormac O. Maher
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.
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.
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.
Patients presenting with secondary or multiple repeat TCS may benefit from surgical untethering.
Wajd N. Al-Holou, Hugh J. L. Garton, Karin M. Muraszko, Mohannad Ibrahim and Cormac O. Maher
Pineal cysts are a frequent incidental finding on intracranial imaging. In adults, the prevalence of pineal cysts is estimated to be 1.1–4.3%. However, the prevalence is not well established in younger patients.
The authors retrospectively reviewed a consecutive series of 14,516 patients 25 years of age and younger, who underwent brain MR imaging at a single institution over an 11-year period. In patients identified with pineal cysts, the authors analyzed the images according to cyst size, signal characteristics, enhancement pattern, and evidence of local mass effect. Patient characteristics including demographics and other intracranial diagnoses were collected in the pineal cyst population and compared with a randomly selected age- and sex-matched control patient population. The data were evaluated using univariate and multivariate logistic regression, linear regression, and ANOVA.
The authors identified 288 pineal region cysts (2.0%). The prevalence of pineal cysts was higher in female (2.4%) than in male patients (1.5%; p < 0.001). Pineal cysts were identified in patients of all ages, with an increased prevalence found in older patients (p < 0.001). Pineal cyst size was similar for all age and sex groups.
Pineal cysts are common in the pediatric population, with an increased prevalence in girls and in older patients.
Wajd N. Al-Holou, Cormac O. Maher, Karin M. Muraszko and Hugh J. L. Garton
The authors reviewed their experience with pediatric pineal cysts to define the natural history and clinical relevance of this common intracranial finding.
The authors identified all patients with pineal cysts who had been clinically evaluated at their institution over an 11.5-year interval and were < 25 years of age at the time of diagnosis. All inclusion criteria were met in 106 patients, and included repeated MR imaging as well as repeated clinical evaluation over at least a 6-month interval.
The mean age at diagnosis was 11.7 ± 7.2 years. Forty-two patients were male and 64 were female. On follow-up MR imaging evaluation at a mean interval of 3.0 years from the initial study, 98 pineal cysts had no increase in size and no change in imaging appearance. Six pineal cysts increased in size and 2 others had a change in imaging characteristics without associated growth. Younger age was associated with cyst change or growth on follow-up imaging (p = 0.02). The mean age of patients with cysts that changed or grew was 5.5 years, and the mean age of patients with stable pineal cysts was 12.2 years. Initial cyst size and appearance on MR imaging were not significant predictors of growth or change in imaging appearance at follow-up. Similarly, the patient's sex was not a significant predictor of growth or change in imaging characteristics.
Follow-up imaging and neurosurgical evaluation may be considered optional in older children with pineal cysts.