Medicine and Health Sciences Commons^™

Neonatal Renal Failure In The Setting Of Anorectal Malformation: A Case Report And Literature Review., Wendy Jo Svetanoff, Asma Ahmed, Richard J. Hendrickson, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

Anorectal malformations (ARMs) can occur in isolation or in association with other anomalies, most commonly those of the genitourinary systems. Morbidity and mortality are highest among patients who develop end-stage renal disease (ESRD) either from severe congenital anomalies (dysplastic kidneys) or from repeated infections in those who have vesicoureteral reflux or persistent recto-urinary fistulas. We describe our management strategy for a patient born with an ARM and bilateral dysplastic kidneys to highlight the nuances and complex decision-making considerations required in taking care of this complex patient population. Our patient is a male twin born at 32 weeks' gestational age who …

Efficacy Of Weight Reduction On Pediatric Nonalcoholic Fatty Liver Disease: Opportunities To Improve Treatment Outcomes Through Pharmacotherapy., Chance S. Friesen, Chelsea Hosey-Cojocari, Sherwin S. Chan, Iván L. Csanaky, Jonathan B. Wagner, Brooke Sweeney, Alec Friesen, Jason D. Fraser, Valentina Shakhnovich

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Obesity is the single greatest risk factor for nonalcoholic fatty liver disease (NAFLD). Without intervention, most pediatric patients with NAFLD continue to gain excessive weight, making early, effective weight loss intervention key for disease treatment and prevention of NAFLD progression. Unfortunately, outside of a closely monitored research setting, which is not representative of the real world, lifestyle modification success for weight loss in children is low. Bariatric surgery, though effective, is invasive and can worsen NAFLD postoperatively. Thus, there is an evolving and underutilized role for pharmacotherapy in children, both for weight reduction and NAFLD management. In this perspective article, …

Quality Outcomes For Pediatric Colorectal Surgery Treated During Short-Term International Medical Service Trips At A Dedicated Site In Honduras., Wilfried Krois, Rebecca M. Rentea, Pastora X. Hernandez, Juan Craniotis-Rios, Richard J. Wood, Marc A. Levitt, Carlos A. Reck-Burneo

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INTRODUCTION: Short-term international medical service trips (MSTs) provide specialized care in resource-constrained countries. There are limited data on immediate and long-term reported outcomes following specialty MST. We hypothesized that dedicated collaborative MST team and host institution produce outcomes and results comparable to those of high-income settings. Our primary aim was to analyze the long-term surgical and functional outcomes of our specialty-specific MSTs following five years of annual MST in Honduras.

METHODS: We performed a single-institution retrospective analysis of 56 children who underwent colorectal and pelvic reconstructive operations between 2014 and 2018. Demographics, diagnosis, comorbidities, type of repair, long-term complications, and …

Colostomy Takedown: Ischemic Complication Following Anorectal Malformation Surgery., Wendy Jo Svetanoff, Justin A. Sobrino Md, Grace S. Mitchell, Rebecca M. Rentea

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Introduction: Anorectal malformations (ARM) are complex disorders that often require staged reconstructions. We present a case and imaging findings of a child who developed issues following colostomy closure due to segmental colonic ischemia. Case Presentation. A 3-year-old female with Currarino syndrome presented with abdominal distention, blood-flecked stools, and prolonged cecostomy flush time. For her anorectal malformation, a colostomy was initially placed. A new colostomy was created at posterior sagittal anorectoplasty (PSARP) to allow the distal rectum to reach the anus without tension. Differentials for her presenting symptoms included a mislocation of the anus, stenosis at the anoplasty site, stricture …

Melanotic Neuroectodermal Tumor Of Infancy, Mohammad H. Almomani, Rebecca M. Rentea

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Melanotic neuroectodermal tumor of infancy (MNTI) is a rapidly growing benign tumor that arises from the neural crest. It typically occurs in infants in the head and neck region. It was described for the first time in 1918 by Krompecher, and since the origin was unknown, it was described as congenital melanocarcinoma. Until 1966, this tumor was characterized by variable names (e.g., retinal anlage tumor, pigmented congenital epulis, melanotic progonoma, pigmented teratoma, atypical melanoblastoma, etc.). However, Borello and Gorlin found that the tumor produces vanillylmandelic acid (VMA), which is produced by other types of tumors that arise from the neural …

Posterior Urethral Valve, George Bingham, Rebecca M. Rentea

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Posterior urethral valves are one of the most common causes of urinary tract obstruction in the pediatric population. They are obstructing membranous folds in the lumen of the posterior part of the urethra and are exclusive to male patients. Posterior urethral valves were first described by Morgagni in 1717 and then by Langenbeck in 1802, who reported valve-like folds in dissected cadavers. Posterior urethral valves can lead to a spectrum of pathology both inside and outside the urinary system. This includes acute retention, chronic kidney disease, and in severe cases, pulmonary hypoplasia secondary to low amniotic fluid levels.

Posterior urethral …

Thymectomy, Bryan Bennett, Rebecca M. Rentea

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Excerpt

Thymectomy is the resection of the thymus gland. This anterior mediastinal organ can enlarge as in myasthenia gravis and thymoma and harbor malignant cells such as in thymic carcinoma or neuroendocrine tumors. The first thymectomies were performed incidentally in conjunction with thyroidectomies for Grave disease by Garre and Sauerbruch, but it was not until Blalock and colleagues that multiple series were performed with adequate results.

This also included patients with myasthenia gravis but without thymomas. The gold standard approach for thymectomy is a median sternotomy or transsternal approach, but this has evolved to less invasive techniques such as upper …

Temporary Abdominal Closure Techniques, Laura R. Brown, Rebecca M. Rentea

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Critically ill patients with underlying intraabdominal hypertension due to sepsis or injury are often managed with a damage control laparotomy (DCL), which involves the minimum intervention necessary to save the patient’s life. In order to save time, the abdomen may be left open to facilitate a second look to confirm the control of bleeding or sepsis before fascial closure. In the interim, some patients will develop bowel and abdominal wall edema with a high-volume resuscitation, especially if the crystalloid fluid is used primarily.

The many causes and management of intraabdominal hypertension as well as the indications for a damage control …

Tisagenlecleucel Infusion In Patients With Relapsed/Refractory All And Concurrent Serious Infection., Erin Hall, Dwight E. Yin, Rakesh K. Goyal, Atif Ahmed, Grace S. Mitchell, Shawn D. St Peter, Terrie Flatt, Ibrahim A. Ahmed, Weijie Li, Richard J. Hendrickson, Keith August, Douglas Myers

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BACKGROUND: Tisagenlecleucel, an anti-CD19 chimeric antigen receptor T (CAR-T) cell therapy, has demonstrated durable efficacy and a manageable safety profile in pediatric and young adult patients with relapsed/refractory B-cell acute lymphoblastic leukemia (B-ALL) in the ELIANA pivotal trial and real-world experience. Experience from investigator-led studies prior to ELIANA suggests that infections and inflammatory conditions may exacerbate the severity of cytokine release syndrome (CRS) associated with CAR-T cell therapy, leading to extreme caution and strong restrictions for on-study and commercial infusion of tisagenlecleucel in patients with active infection. CRS intervention with interleukin (IL)-6 blockade and/or steroid therapy was introduced late in …

Simulation Training And Skill Assessment In Obstetrics And Gynecology, Thomas G. Weiss, Rebecca M. Rentea

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Simulation is a valuable teaching tool that increases skill and knowledge acquisition in obstetric and gynecologic clinical scenarios. There are increasing options for simulation from animal and human cadaver, low-fidelity (inanimate simulator), and virtual reality simulation. The role of obstetric and gynecologic simulation also plays a key role in both outcomes-based as well as competency-based medical education.

Simulators were initially created for flight training for pilots in the 1920s, and the ability to reproduce clinical situations has been utilized in obstetric and gynecologic education. Simulations used in medical training were used in the 1960s with standardized patients and mannequins. Simulation …