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Adam L. Sandler, Arundhati Biswas, and James Tait Goodrich

In 1915, faced with 2 patients with large skull defects, W. Wayne Babcock, an obstetrician-gynecologist-turned-general surgeon, operating in a modest North Philadelphia hospital, did something extraordinary: he went to the hospital kitchen to look for a cranial graft. Based heavily on archival and other primary sources, the authors tell the remarkable tale of the “soup bone” cranioplasties of the Samaritan Hospital and place these operations within the context of the early modern American hospital.

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Adam L. Sandler, Lawrence B. Daniels III, David A. Staffenberg, Eliezer Kolatch, James T. Goodrich, and Rick Abbott

A subset of hydrocephalic patients in whom shunts are placed at an early age will develop craniocerebral disproportion (CCD), an iatrogenic mismatch between the fixed intracranial volume and the growing brain. The lack of a reliable, reproducible method to diagnose this condition, however, has hampered attempts to treat it appropriately. For those practitioners who acknowledge the need to create more intracranial space in these patients, the lack of agreed-upon therapeutic end points for cranial vault expansion has limited the use of such techniques and has sometimes led to problems of underexpansion. Here, the authors present a definition of CCD based primarily on the temporal correlation of plateau waves on intracranial pressure (ICP) monitoring and headache exacerbation. The authors describe a technique of exploiting continued ICP monitoring during progressive cranial expansion in which the goal of distraction is the cessation of plateau waves. Previously encountered problems of underexpansion may be mitigated through the simultaneous use of ICP monitors and gradual cranial expansion over time.

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Grzegorz Miekisiak, Kristen Yoo, Adam L. Sandler, Tobias B. Kulik, Jiang-Fan Chen, and H. Richard Winn

Object

The authors tested the hypothesis that adenosine, acting through the A2A receptor, is not involved in hypercarbic hyperemia by assessing the effects of increased PaCO2 on cerebral blood flow (CBF) in vivo in wild-type and A2A receptor knockout mice. In addition, they evaluated the effect of abluminal pH changes in vitro on the diameter of isolated perfused penetrating arterioles harvested from wild-type and A2A receptor knockout mice.

Methods

The authors evaluated in a blinded fashion the CBF response during transient (60-second) hypercapnic (7% CO2) hypercarbia in anesthetized, ventilated C57Bl/6 wild-type and adenosine A2A receptor knockout mice. They also evaluated the hypercarbic response in the absence and presence of the nonselective and selective adenosine antagonists.

Results

Cerebral blood flow was measured using laser Doppler flowmetry. There were no differences between the CBF responses to hypercarbia in the wild-type and the knockout mice. Moreover, the hypercarbic hyperemia response was not affected by the adenosine receptor antagonists. The authors also tested the response to alteration in abluminal pH in isolated perfused, pressurized, penetrating arterioles (average diameter 63.3 ± 3.6 μm) harvested from wild-type (6 mice) and knockout (5 mice) animals. Arteriolar dilation in response to a decrease in abluminal pH, simulating the change in vivo during hypercarbia, was similar in wild-type (15.9 ± 2.6%) and A2A receptor knockout (17.7 ± 1.3%) mice. With abluminal application of CGS 21680 (10−6 M), an A2A receptor agonist, wild-type arterioles dilated in an expected manner (9.8 ± 0.7%), whereas A2A receptor knockout vessels had minimal response.

Conclusions

The results of the in vivo and in vitro studies in wild-type and A2A receptor knockout mice support the authors' hypothesis that hypercarbic vasodilation does not involve an adenosine A2A receptor–related mechanism.

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Kristy Tan, Avital Meiri, Wenzhu B. Mowrey, Rick Abbott, James T. Goodrich, Adam L. Sandler, Asif K. Suri, Michael L. Lipton, and Mark E. Wagshul

OBJECTIVE

The object of this study was to use diffusion tensor imaging (DTI) and tract-based spatial statistics (TBSS) to characterize the long-term effects of hydrocephalus and shunting on white matter integrity and to investigate the relationship of ventricular size and alterations in white matter integrity with headache and quality-of-life outcome measures.

METHODS

Patients with shunt-treated hydrocephalus and age- and sex-matched healthy controls were recruited into the study and underwent anatomical and DTI imaging on a 3-T MRI scanner. All patients were clinically stable, had undergone CSF shunt placement before 2 years of age, and had a documented history of complaints of headaches. Outcome was scored based on the Headache Disability Inventory and the Hydrocephalus Outcome Questionnaire. Fractional anisotropy (FA) and other DTI-based measures (axial, radial, and mean diffusivity; AD, RD, and MD, respectively) were extracted in the corpus callosum and internal capsule with manual region-of-interest delineation and in other regions with TBSS. Paired t-tests, corrected with a 5% false discovery rate, were used to identify regions with significant differences between patients and controls. Within the patient group, linear regression models were used to investigate the relationship between FA or ventricular volume and outcome, as well as the effect of shunt-related covariates.

RESULTS

Twenty-one hydrocephalus patients and 21 matched controls completed the study, and their data were used in the final analysis. The authors found significantly lower FA for patients than for controls in 20 of the 48 regions, mostly posterior white matter structures, in periventricular as well as more distal tracts. Of these 20 regions, 17 demonstrated increased RD, while only 5 showed increased MD and 3 showed decreased AD. No areas of increased FA were observed. Higher FA in specific periventricular white matter tracts, tending toward FA in controls, was associated with increased ventricular size, as well as improved clinical outcome.

CONCLUSIONS

The study shows that TBSS-based DTI is a sensitive technique for elucidating changes in white matter structures due to hydrocephalus and chronic CSF shunting and provides preliminary evidence that DTI may be a valuable tool for tailoring shunt procedures to monitor ventricular size following shunting and achieve optimal outcome, as well as for guiding the development of alternate therapies for hydrocephalus.

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Adam L. Sandler, Donald Sturrock, Jane Branfield, Rick Abbott, James T. Goodrich, Arundhati Biswas, Lawrence B. Daniels III, and Eugene S. Flamm

On December 5, 1960, 4-month-old Theo Dahl, the only son of best-selling author Roald Dahl (1916–1990), had his skull shattered in a horrific traffic accident. What began as a personal tragedy for the Dahl family would soon evolve into a dogged crusade by Dahl to expand upon preexisting valve technology with the goal of developing a shunt that would not become obstructed. Based upon exclusive access to private archives of the Dahl estate, as well as interviews with those involved, this article tells the intricate tale of one famous father's drive to significantly alter the natural history of pediatric hydrocephalus. Dahl's collaboration with British toymaker Stanley Wade and pioneering pediatric neurosurgeons Joseph Ransohoff, Kenneth Shulman, and Kenneth Till to create the Wade-Dahl-Till (WDT) valve is examined in detail. The ensuing rift between the American and British contingents, the valve's multiple design revisions, and the goal of creating an affordable shunt for children in developing countries are among the issues addressed. The development of the WDT valve marked a significant turning point in the surgical management of pediatric hydrocephalus in general and in shunt valve technology in particular.

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Adam L. Sandler, Oren M. Tepper, James T. Goodrich, Rani Nasser, Arundhati Biswas, and Rick Abbott

While autologous split calvaria remains the preferred material for use in pediatric cranioplasty, it may be difficult to split the bone neatly into two distinct pieces, especially in infants and young children. In this paper, the authors present a technique in which numerous split pieces of bone can be readily joined together and conformed to the shape of the specific defect using a customized template and 3D trellis-like basket.