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Optimizing Reconstruction in Craniosynostosis: Review of Nonsyndromic Patients Treated With a Novel Technique

Rudy, Hayeem L; Herman, Sean; Stern, Carrie S; Staffenberg, David A; Dowling, Kamilah; Goodrich, James T; Tepper, Oren M
PURPOSE/OBJECTIVE:Open cranial vault remodeling (CVR) with autologous split calvarial bone grafts redistributes and recontours an abnormal calvarium to create an expanded cranial vault in patients with craniosynostosis. We report a 12-year retrospective review of 162 nonsyndromic patients who underwent operative repair using our previously-described technique which portends excellent surgical outcomes and can be applied to patients of any age group and with any variety of suture fusion. METHODS:Data was gathered on patients who underwent CVR from 2005 to 2016. Surgical records for each patient were analyzed and included operative time, estimated blood loss, and intraoperative transfusion volumes. Intraoperative and postoperative complications, the need for revision surgery, postoperative length of stay, and follow-up records were also reviewed. Syndromic patients were excluded, as well as patients with incomplete data sets. Patients who underwent either anterior or posterior vault remodeling were compared. RESULTS:A total of 162 patients were included in this case series. Patients undergoing anterior CVR were significantly older than those undergoing posterior CVR (13.3 versus 11.0 months, P < 0.015) and also had significantly greater intraoperative red blood transfusion volumes (20.3 versus 15.3cc/kg, P < 0.0207) and longer operative time than posterior CVR patients (274.9 versus 216.7 minutes, P < 0.0001). No patients required reoperation for resorption or recurrence or persistent contour irregularities. There were no visual or neurological complications. Calvarial bone was successfully split in 100% of cases. CONCLUSIONS:This surgical approach to CVR results in good surgical outcomes with a low recurrence rate, while also maximizing operative efficiency, and minimizing total blood loss and transfusion volume. This technique can be applied to any affected suture in a patient with craniosynostosis and in patients of any age group.
PMID: 32569055
ISSN: 1536-3732
CID: 4492832

Five-year safety evaluation of maraviroc in HIV-1-infected treatment-experienced patients

Gulick, Roy M; Fatkenheuer, Gerd; Burnside, Robert; Hardy, W David; Nelson, Mark R; Goodrich, James; Mukwaya, Geoffrey; Portsmouth, Simon; Heera, Jayvant R
BACKGROUND: Maraviroc is unique among approved antiretroviral drugs in targeting the host-cell chemokine coreceptor type-5 receptor. With its novel mechanism of action, we sought to describe the 5-year safety profile of maraviroc. METHODS: Two large phase 3 studies of maraviroc enrolled HIV-infected treatment-experienced patients and followed them up for 5 or more years. Survival and selected clinical end points were identified and assessed. RESULTS: A total of 938 enrolled patients received maraviroc-containing regimens. Rates of death and selected clinical events (eg, hepatic failure, malignancy, and myocardial infarction) were low during follow-up. CONCLUSIONS: Maraviroc was generally safe in treatment-experienced participants for >5 years.
PMID: 24419064
ISSN: 1525-4135
CID: 892942

Successful treatment of post-shunt craniocerebral disproportion by coupling gradual external cranial vault distraction with continuous intracranial pressure monitoring

Sandler, Adam L; Daniels, Lawrence B 3rd; Staffenberg, David A; Kolatch, Eliezer; Goodrich, James T; Abbott, Rick
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.
PMID: 23540529
ISSN: 1933-0707
CID: 387002

Separation of craniopagus conjoined twins with a staged approach

Staffenberg, David A; Goodrich, James T
ABSTRACT: The separation of craniopagus conjoined twins is a very rare and complex challenge. As with many rare challenges, it presents initially as a deceptively simple problem requiring only the most basic clinical techniques. As in many reconstructive problems, this paradigm mandates that the neurosurgical team performs the separation with the plastic surgeons providing closure at the end of the separation. Historically, these approaches have included, as with the separation of many other types of conjoined twins, the use of tissue expansion before separation followed by separation surgery. In the best hands, at the most capable medical centers, the mortality reported in the literature for the past 50 years is greater than 50%. Craniofacial surgery frequently demands a coordinated effort between plastic surgery and neurosurgery and many other specializations; separating craniopagus twins takes this coordination to a stratospheric level. It is, however, this coordination that is of paramount importance. Success clearly requires an understanding of the complex interrelationship between the "separation" and the "reconstruction" and that decisions made for 1 aspect of the surgery will have a profound impact on another aspect of the surgery. The impact can be disastrous or, if planned well, can be advantageous.We were contacted to evaluate craniopagus conjoined male infant twins for separation. Radiographic studies suggested that the brains were separate, and their medical team suggested that they were "fit for separation." We reviewed the literature and reviewed our colleagues' experiences with similar cases around the world. It became clear that whether separation had been unsuccessful or successful, a variety of issues accompanied surgery as follows: (1) massive intraoperative hemorrhage, (2) cerebral edema, (3) venous infarcts, (4) swelling of flaps, and (5) dehiscence of repairs with cerebrospinal fluid (CSF) leak, meningitis, or brain exposure. Although the initial plan was to separate the twins in the same fashion as in previous cases (ie, single-stage separation surgery preceded by tissue expansion of the scalp), it was clear that this approach increases cerebral venous pressure during the separation component of surgery and therefore set up a cascade of events favoring failure rather than success. Wishing to favor success, we elected to design an open-ended multistaged separation to improve venous collateral circulation. We believe that this would improve venous drainage, prevent increased venous pressure, diminish cerebral edema, and favor the integrity of the dura and flap repair that would in turn lessen the risk of CSF leak. The stages would also allow the twins to recover from each stage before progressing to the next stage while continuing to receive nutritional support and physical therapy. Four major stages for 9 (1/2) months led to their successful separation. There has been no CSF leak or meningitis. To our knowledge, this technique has since been applied to 2 other sets of craniopagus with similar outcomes.A review of the pertinent literature, our rationale, and methodology are discussed in this article.
PMID: 23154370
ISSN: 1049-2275
CID: 182542

Marvelous medicine: the untold story of the Wade-Dahl-Till valve [Historical Article]

Sandler, Adam L; Sturrock, Donald; Branfield, Jane; Abbott, Rick; Goodrich, James T; Biswas, Arundhati; Daniels, Lawrence B 3rd; Flamm, Eugene S
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.
PMID: 22546025
ISSN: 1933-0707
CID: 454932

Two-year safety and virologic efficacy of maraviroc in treatment-experienced patients with CCR5-tropic HIV-1 infection: 96-week combined analysis of MOTIVATE 1 and 2

Hardy, W David; Gulick, Roy M; Mayer, Howard; Fatkenheuer, Gerd; Nelson, Mark; Heera, Jayvant; Rajicic, Natasa; Goodrich, James
BACKGROUND: Maraviroc, the first approved CCR5 antagonist, demonstrated 48-week safety and virologic efficacy in CCR5-tropic HIV-infected, treatment-experienced patients; however, critical longer-term safety and durability of responses are unknown. METHODS: Two-year follow-up of 2 prospective, randomized, blinded studies of maraviroc once daily or twice daily, or placebo in treatment-experienced patients with R5-tropic HIV-1 receiving an optimized background regimen. Unblinding occurred after the week-48 visit of the last enrolled patient. Safety and virologic parameters were assessed through week 96. RESULTS: One thousand forty-nine patients were randomized and received study drugs. HIV-1 RNA was <50 copies per milliliter at week 96 in 39% and 41% of patients receiving maraviroc every day or twice a day, respectively. Among patients with HIV-1 RNA <50 copies per milliliter at week 48, 81% and 87% of patients receiving maraviroc every day or twice a day, respectively, maintained this response at week 96. At week 96, median CD4+ T-cell counts increased from baseline by 89 and 113 cells per cubic millimeter with maraviroc every day and twice a day, respectively. Exposure-adjusted rates of adverse events were similar with maraviroc or placebo. No new or unexpected events were observed after week 48. CONCLUSIONS: Maraviroc-containing antiretroviral regimens maintained durable responses in treatment-experienced patients with R5 HIV-1 through 96 weeks of treatment with a safety profile similar to placebo.
PMID: 20703158
ISSN: 1525-4135
CID: 892742

Anatomy, technology, art, and culture: toward a realistic perspective of the brain [Historical Article]

Cavalcanti, Daniel D; Feindel, William; Goodrich, James T; Dagi, T Forcht; Prestigiacomo, Charles J; Preul, Mark C
In the 15th century, brain illustration began to change from a schematic system that involved scant objective rendering of the brain, to accurate depictions based on anatomical dissections that demanded significant artistic talent. Notable examples of this innovation are the drawings of Leonardo da Vinci (1498-1504), Andreas Vesalius' association with the bottega of Titian to produce the drawings of Vesalius' De humani corporis fabrica (1543), and Christopher Wren's illustrations for Thomas Willis' Cerebri Anatome (1664). These works appeared during the Renaissance and Age of Enlightenment, when advances in brain imaging, or really brain rendering, reflected not only the abilities and dedications of the artists, but also the influences of important cultural and scientific factors. Anatomy and human dissection became popular social phenomena as well as scholarly pursuits, linked with the world of the fine arts. The working philosophy of these artists involved active participation in both anatomical study and illustration, and the belief that their discoveries of the natural world could best be communicated by rendering them in objective form (that is, with realistic perspective). From their studies emerged the beginning of contemporary brain imaging. In this article, the authors examine how the brain began to be imaged in realism within a cultural and scientific milieu that witnessed the emergence of anatomical dissection, the geometry of linear perspective, and the closer confluence of art and science.
PMID: 19722816
ISSN: 1092-0684
CID: 4006282

Maraviroc for previously treated patients with R5 HIV-1 infection

Gulick, Roy M; Lalezari, Jacob; Goodrich, James; Clumeck, Nathan; DeJesus, Edwin; Horban, Andrzej; Nadler, Jeffrey; Clotet, Bonaventura; Karlsson, Anders; Wohlfeiler, Michael; Montana, John B; McHale, Mary; Sullivan, John; Ridgway, Caroline; Felstead, Steve; Dunne, Michael W; van der Ryst, Elna; Mayer, Howard
BACKGROUND: CC chemokine receptor 5 antagonists are a new class of antiretroviral agents. METHODS: We conducted two double-blind, placebo-controlled, phase 3 studies--Maraviroc versus Optimized Therapy in Viremic Antiretroviral Treatment-Experienced Patients (MOTIVATE) 1 and MOTIVATE 2--with patients who had R5 human immunodeficiency virus type 1 (HIV-1) only. They had been treated with or had resistance to three antiretroviral-drug classes and had HIV-1 RNA levels of more than 5000 copies per milliliter. The patients were randomly assigned to one of three antiretroviral regimens consisting of maraviroc once daily, maraviroc twice daily, or placebo, each of which included optimized background therapy (OBT) based on treatment history and drug-resistance testing. Safety and efficacy were assessed after 48 weeks. RESULTS: A total of 1049 patients received the randomly assigned study drug; the mean baseline HIV-1 RNA level was 72,400 copies per milliliter, and the median CD4 cell count was 169 per cubic millimeter. At 48 weeks, in both studies, the mean change in HIV-1 RNA from baseline was greater with maraviroc than with placebo: -1.66 and -1.82 log(10) copies per milliliter with the once-daily and twice-daily regimens, respectively, versus -0.80 with placebo in MOTIVATE 1, and -1.72 and -1.87 log(10) copies per milliliter, respectively, versus -0.76 with placebo in MOTIVATE 2. More patients receiving maraviroc once or twice daily had HIV-1 RNA levels of less than 50 copies per milliliter (42% and 47%, respectively, vs. 16% in the placebo group in MOTIVATE 1; 45% in both maraviroc groups vs. 18% in MOTIVATE 2; P<0.001 for both comparisons in each study). The change from baseline in CD4 counts was also greater with maraviroc once or twice daily than with placebo (increases of 113 and 122 per cubic millimeter, respectively, vs. 54 in MOTIVATE 1; increases of 122 and 128 per cubic millimeter, respectively, vs. 69 in MOTIVATE 2; P<0.001 for both comparisons in each study). Frequencies of adverse events were similar among the groups. CONCLUSIONS: Maraviroc, as compared with placebo, resulted in significantly greater suppression of HIV-1 and greater increases in CD4 cell counts at 48 weeks in previously treated patients with R5 HIV-1 who were receiving OBT. ( numbers, NCT00098306 and NCT00098722.)
PMID: 18832244
ISSN: 0028-4793
CID: 892592

Sacral appendage in a child with an FGFR2 mutation: a report and review [Case Report]

Shanske, Alan L; Staffenberg, David; Goodrich, James T
PMID: 18629881
ISSN: 1552-4833
CID: 3319712

The craniopagus malformation: classification and implications for surgical separation

Stone, James L; Goodrich, James T
Craniopagus twins (CPT) are an uncommon, highly fascinating accident of nature. The clinical pathology of this complex entity is reviewed and placed in perspective. A logical classification aids understanding of the anomaly, and is essential to gauge outcome from separation attempts. 'Partial forms' lack significant shared dural venous sinuses (SDVS) and 'Total forms' with SDVS also exhibit more severe compressional brain distortion. Our classification consists of Partial Angular (PA), Partial Vertical (PV), Total Angular (TA) and Total Vertical (TV, formerly O'Connell Types I-III). Total vertical has a continuous cranium, and inter-twin axial facial rotation <40 degrees (Type I), 140-180 degrees (Type II) or intermediate (Type III). The term 'Angular' denotes an inter-twin longitudinal angle below 140 degrees , regardless of axial rotation. Our review categorized 64 well-delineated CPT, including 41 operative separation attempts in small children since initial success in 1952. Just over one-half were TV, almost one-third TA, and partial forms accounted for the remaining one-sixth. About 30% of CPT had shared or fused brain tissue, and a similar percentage of TA twins shared a posterior fossa. Partial forms had significantly higher birth weights, were separated at an earlier age (6 versus 11 months) and had lower mortality and better outcome compared with Total forms. A multi-staged surgical separation for Total CPT had a significantly better mortality than single-staged separation. Discussion emphasizes embryological, anatomical and clinical aspects of the malformation, with emphasis upon obstacles to a successful outcome.
PMID: 16597654
ISSN: 1460-2156
CID: 2264672