Publications by authors named "Erin N Kiehna"

Introduction: To evaluate the outcomes and demographics of encephalocele patients who were born and received treatment in our neonatal ICU and conduct a PRISMA literature review.

Methods: An Institutional Review Board (IRB)-approved retrospective cohort study was undertaken to investigate the results of treating encephalocele patients at Jackson Memorial Hospital (JMH) from 1998 to 2022. The study focused on assessing outcomes and the impact of maternal socioeconomic factors, such as religion, age, and education, along with the timing of diagnosis, in connection with a systematic review.

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Choroid plexus carcinoma (CPC) is a rare brain tumor that occurs most commonly in very young children and has a dismal prognosis despite intensive therapy. Improved outcomes for patients with CPC depend on a deeper understanding of the mechanisms underlying the disease. Here we developed transgenic models of CPCs by activating the oncogene and deleting the tumor suppressor gene in murine neural stem cells or progenitors.

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OBJECTIVE Rathke cleft cysts (RCCs) are sellar lesions that are commonly encountered in adults but infrequently diagnosed in the pediatric population. As a result, the optimal management of pediatric RCCs remains a subject of controversy. Only 2 prior surgical series have been published on pediatric RCCs and no study has compared the presentation and outcomes of surgically versus conservatively managed cases.

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Article Synopsis
  • The study investigates how different parts of a single tumor can exhibit varying genetic and transcriptional profiles, complicating the search for effective biomarkers and therapies.
  • It found that while medulloblastomas showed consistent transcriptomic profiles across biopsies, somatic mutations in local tumors were often inconsistent, impacting targeted treatment approaches.
  • The research suggests that for successful clinical trials of targeted therapies, it's crucial to confirm that identified mutations are present throughout the entire tumor, not just in isolated samples.
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Pediatric high-grade glioma is a rare tumor characterized by high mortality. The authors report the first case of a high-grade glioma associated with a GOPC(FIG)-ROS1 fusion in a pediatric patient. The patient underwent gross-total resection at the age of 4 years, followed by adjuvant high-dose chemotherapy and autologous hematopoietic stem cell rescue.

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OBJECTIVE When complete resection of craniopharyngioma is not achievable or the sequelae are prohibitive, limited surgery and radiation therapy have demonstrated excellent local disease control while minimizing treatment-related sequelae. When residual tissue exists, there is a propensity for further cyst development and expansion during and after radiation therapy. This can result in obstructive hydrocephalus, visual changes, and/or clinical decline.

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OBJECT Hemispherectomy for unilateral, medically refractory epilepsy is associated with excellent long-term seizure control. However, for patients with recurrent seizures following disconnection, workup and investigation can be challenging, and surgical options may be limited. Few studies have examined the role of repeat hemispherotomy in these patients.

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OBJECT Recently published data have suggested an increase in adverse outcomes in pediatric patients after insertion or revision of a ventricular CSF diversion shunt after a same-day weekend procedure. The authors undertook an evaluation of the impact of weekend admission and time to shunting on surgery-related quality outcomes in pediatric patients who underwent ventricular shunt insertion or revision. METHODS Pediatric patients with hydrocephalus who underwent ventriculoperitoneal, ventriculoatrial, or ventriculopleural shunt placement were selected from the 2000-2010 Nationwide Inpatient Sample and Kids' Inpatient Database.

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Background: Infantile Hemangiomas (IH) are the most common benign tumor of infancy, occurring in over 10% of newborns. While most IHs involute and never require intervention, some scalp IHs may cause severe cosmetic deformity and threaten tissue integrity that requires surgical excision.

Case Description: We present our experience with two infants who presented with large scalp IH.

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Object: The intrathoracic pressure regulator (ITPR) is a novel noninvasive device designed to increase circulation and blood pressure. By applying negative pressure during the expiratory phase of ventilation it decreases intrathoracic pressure and enhances venous return, which increases cardiac output. It is possible that the ITPR may both decrease intracranial pressure (ICP) and increase cerebral perfusion pressure (CPP) in brain-injured patients by decreasing cerebral venous blood volume and increasing cardiac output.

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Purpose: To assess visual outcome prospectively after conformal radiation therapy (CRT) in children with optic pathway glioma.

Methods And Materials: We used CRT to treat optic pathway glioma in 20 children (median age 9.3 years) between July 1997 and January 2002.

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Object: The Consolidated Standards for Reporting of Trials (CONSORT) criteria were published in 1996 to standardize the reporting and improve the quality of clinical trials. Despite having been endorsed by major medical journals and shown to improve the quality of reported trials, neurosurgical journals have yet to formally adopt these reporting criteria. The purpose of this study is to evaluate the quality and reporting of randomized controlled trials (RCTs) in neurosurgery and the factors that may affect the quality of reported trials.

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Central nervous system hemorrhages are an uncommon but severe complication of hemophilia, occurring in only 2-8% of children with hemophilia. Less than 10% of these CNS hemorrhages are intraspinal. The authors report on their care of an infant with hemophilia A who presented with irritability, meningismus, and decreased spontaneous movement.

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Object: The treatment of craniopharyngioma is highly controversial. Continued advances in limited surgery and radiation therapy have maintained excellent local disease control while minimizing treatment-related sequelae. Further analyses of outcomes are necessary to characterize the long-term effects of radiation therapy.

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Context: Pseudotumor cerebri has only been described after successful surgery for Cushing's disease (CD) in case reports. We sought to establish the incidence and timing of its occurrence, identify predisposing factors, characterize the clinical presentations and their severity, and examine the effects of treatment in patients who underwent surgery for CD.

Setting: This study was conducted at two tertiary care centers: The University of Virginia and the National Institutes of Health.

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Purpose: To prospectively assess the impact of conformal radiation therapy (CRT) and demographic and clinical variables on four measures of attention in pediatric and young adult patients with localized primary brain tumors.

Patients And Methods: We prospectively evaluated 120 patients with primary brain tumors, ages 2 to 24.4 years (median, 9.

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Object: A Phase II trial of conformal radiation therapy (CRT) for craniopharyngioma was conducted to determine whether the irradiated volume could be safely reduced to decrease effects on cognitive function.

Methods: Between July 1997 and January 2003, 28 pediatric patients (median age 7.3 +/- 4.

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Purpose: Model the effects of radiation dosimetry on IQ among pediatric patients with central nervous system (CNS) tumors.

Methods And Materials: Pediatric patients with CNS embryonal tumors (n = 39) were prospectively evaluated with serial cognitive testing, before and after treatment with postoperative, risk-adapted craniospinal irradiation (CSI) and conformal primary-site irradiation, followed by chemotherapy. Differential dose-volume data for 5 brain volumes (total brain, supratentorial brain, infratentorial brain, and left and right temporal lobes) were correlated with IQ after surgery and at follow-up by use of linear regression.

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Purpose: To assess the effects of radiation dose-volume distribution on the trajectory of IQ development after conformal radiation therapy (CRT) in pediatric patients with ependymoma.

Methods And Materials: The study included 88 patients (median age, 2.8 years +/- 4.

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Purpose: To assess sustained attention, impulsivity, and reaction time during radiotherapy (RT) for pediatric patients with localized primary brain tumors.

Methods And Materials: Thirty-nine patients (median age 12.3 years, range 5.

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