Medicine and Health Sciences Commons^™

State Of The Art Bowel Management For Pediatric Colorectal Problems: Spinal Anomalies., Elizaveta Bokova, Ninad Prasade, John Rosen, Irene Isabel P. Lim, Marc A. Levitt, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Patients with spinal abnormalities often struggle with fecal and/or urinary incontinence (up to 87 and 92%, respectively) and require a collaborative approach to bowel management in conjunction.

METHODS: To define existing approaches and propose state-of-the-art bowel management, a literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published July 2013 and July 2023.

RESULTS: Patients with spinal anomalies have impaired innervation of the rectum and anal canal, decreasing the success rate from laxatives and rectal enemas. Thus, transanal irrigations and antegrade flushes are widely utilized in this group of patients. Based …

State Of The Art Bowel Management For Pediatric Colorectal Problems: Hirschsprung Disease., Elizaveta Bokova, Ninad Prasade, Sanjana Janumpally, John Rosen, Irene Isabel P. Lim, Marc A. Levitt, Rebecca M. Rentea

Manuscripts, Articles, Book Chapters and Other Papers

After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with …

Prevalence, Management And Efficacy Of Treatment In Portal Vein Obstruction After Paediatric Liver Transplantation: Protocol Of The Retrospective International Multicentre Portal Registry., Bader A. Alfares, Hubert P J Van Der Doef, Barbara E. Wildhaber, Thomas Casswall, Greg Nowak, Martin Delle, Denise Aldrian, Valeria Berchtold, Georg F. Vogel, Piotr Kaliciński, Malgorzata Markiewicz-Kijewska, Adam Kolesnik, Jesús Q. Bernabeu, María Mercadal Hally, Mauricio Larrarte K, Paolo Marra, Michela Bravi, Domenico Pinelli, Mureo Kasahara, Seisuke Sakamoto, Hajime Uchida, Vidyadhar Mali, Marion Aw, Stéphanie Franchi-Abella, Emmanuel Gonzales, Florent Guérin, Guillermo Cervio, Julia Minetto, Sergio Sierre, Martin De Santibañes, Victoria Ardiles, Jimmy Walker Uno, Helen Evans, David Duncan, John Mccall, Steffen Hartleif, Ekkehard Sturm, Jai Patel, Marumbo Mtegha, Raj Prasad, Cristina T. Ferreira, Luiza S. Nader, Marco Farina, Catalina Jaramillo, Manuel I. Rodriguez-Davalos, Peter Feola, Amit A. Shah, Phoebe M. Wood, Michael R. Acord, Ryan T. Fischer, Bhargava Mullapudi, Richard J. Hendrickson, Rajeev Khanna, Viniyendra Pamecha, Amar Mukund, Khalid Sharif, Girish Gupte, Simon Mcguirk, Gilda Porta, Marco Spada, Tommaso Alterio, Giuseppe Maggiore, Winita Hardikar, Marisa Beretta, Rudi Dierckx, Ruben H J De Kleine, Reinoud P H Bokkers

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INTRODUCTION: Portal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO.

METHODS AND ANALYSIS: The Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (agedyears) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are …

State Of The Art Bowel Management For Pediatric Colorectal Problems: Functional Constipation., Elizaveta Bokova, Wendy Jo Svetanoff, John Rosen, Marc A. Levitt, Rebecca M. Rentea

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BACKGROUND: Functional constipation (FC) affects up to 32% of the pediatric population, and some of these patients are referred to pediatric surgery units to manage their constipation and/or fecal incontinence. The aim of the current paper is to report the recent updates on the evaluation and management of children with FC as a part of a manuscript series on bowel management in patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and FC.

METHODS: A literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published within the last 5-10 years.

RESULTS: The first …

State Of The Art Bowel Management For Pediatric Colorectal Problems: Anorectal Malformations., Elizaveta Bokova, Wendy Jo Svetanoff, Joseph Lopez, Marc A. Levitt, Rebecca M. Rentea

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Up to 79% of patients with anorectal malformations (ARMs) experience constipation and/or soiling after a primary posterior sagittal anoplasty (PSARP) and are referred to a bowel management program. We aim to report the recent updates in evaluating and managing these patients as part of the manuscript series on the current bowel management protocols for patients with colorectal diseases (ARMs, Hirschsprung disease, functional constipation, and spinal anomalies). The unique anatomic features of ARM patients, such as maldeveloped sphincter complex, impaired anal sensation, and associated spine and sacrum anomalies, indicate their bowel management plan. The evaluation includes an examination under anesthesia and …

Pediatric Bowel Management Options And Organizational Aspects., Elizaveta Bokova, Wendy Jo Svetanoff, Marc Aaron Levitt, Rebecca M. Rentea

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A bowel management program (BMP) to treat fecal incontinence and severe constipation is utilized for patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and functional constipation, decreasing the rate of emergency department visits, and hospital admissions. This review is part of a manuscript series and focuses on updates in the use of antegrade flushes for bowel management, as well as organizational aspects, collaborative approach, telemedicine, the importance of family education, and one-year outcomes of the bowel management program. Implementation of a multidisciplinary program involving physicians, nurses, advanced practice providers, coordinators, psychologists, and social workers leads to rapid center growth and …

Association Of Satisfaction With Treatment Decision And Failure Of Nonoperative Management Of Appendicitis In Children., Rebecca M. Rentea, Shawn D. St Peter

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No abstract provided.

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

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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 …

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 …

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

Manuscripts, Articles, Book Chapters and Other Papers

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 …

Patent Urachus, Kayla B. Briggs, Rebecca M. Rentea

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atent urachus refers to one condition in a rare spectrum of disorders referred to as urachal anomalies. These conditions result from the failure of the involution of normal embryologic tissues that serve to empty the fetal bladder. The location and amount of persistent tissue dictate the presenting symptoms. Some of these urachal anomalies are obvious at birth, while others are more subtle and not diagnosed until adulthood or only incidentally discovered after imaging is obtained for other reasons. Historically, surgical resection of urachal anomalies was routinely undertaken, given the potential for malignancy in retained ectopic tissue. Early surgical resection has …

Oophorectomy, Ashli A. Lawson, Rebecca M. Rentea

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Oophorectomy is the surgical removal of the ovary and can be unilateral or bilateral. Hysterectomy is the most common major gynecologic surgery, but salpingo-oophorectomy remains a frequent concomitant surgery. Surgery for ovarian pathology alone is still often encountered in females of all ages. While surgical removal of one ovary does not grossly affect the hormonal milieu of a patient, bilateral oophorectomy will render a patient infertile and menopausal with ensuing physiologic changes and risks. Forward-thinking discussions should be held with the patient before undertaking an oophorectomy.

Retroperitoneal Hematoma, Christopher Mondie, Rebecca M. Rentea

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Retroperitoneal hematoma is defined as bleeding into the retroperitoneal space. This clinical entity is often occult and under-recognized by clinicians and is a cause of significant morbidity and mortality. Often patients do not manifest clinically apparent signs and symptoms until a substantial amount of blood loss has occurred. It is not uncommon for patients to present in frank hemorrhagic shock due to an underlying retroperitoneal hematoma. The retroperitoneal space lies directly posterior to the peritoneal cavity. An organizational schema dividing the retroperitoneal space into three different “zones” is widely prevalent in the surgical literature. The central-medial zone (Zone I) falls …

A Simple Technique For The Management Of Refractory Gastrostomy Site Complications A Technical Innovation In Gastrostomy Tube Site Revision, Rebecca M. Rentea, Wendy Jo Svetanoff, Charlene Dekonenko, Courtney Pisano, Charles L. Snyder

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Introduction

Longstanding gastrostomy sites are prone to leakage and excoriation secondary to protrusion of gastric mucosa into the tract, dilation of the site over time, and development of refractory granulation tissue. Surgical revision with creation of a new gastric and skin exit site can be difficult. We describe a technique to re-site a gastrostomy tube (GT) utilizing the existing GT site.

Materials and methods

For one patient with gastrostomy site refractory complications, we re-sited the GT using the existing site. A clamp was placed through the old GT site and was palpated through the abdominal wall. An incision over the …

Pentalogy Of Cantrell, Muhammad Khawar Sana, Rebecca M. Rentea

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Pentalogy of Cantrell (POC) is a collection of five congenital midline birth anomalies that present a distinctive challenge for clinicians and surgeons. Those five defects are of the heart, pericardium, diaphragm, sternum, and abdominal wall. This condition has been divided into two categories, complete or partial. Complete, as the name indicates, refers to the presence of all five defects, while others may present with only partial defects. It is also referred to as thoracoabdominal ectopia cordis, a condition where the heart is covered by an omphalocele-like membrane. Ectopia cordis (EC) is often found in fetuses with POC. Infants usually have …

Transanal-Only Swenson-Like Pull-Through For Late Diagnosed Hirschsprung Disease., Rebecca M. Rentea, Devin R. Halleran, Hira Ahmad, Elias Maloof, Richard J. Wood, Marc A. Levitt

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Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A transanal-only pull-through is a well-described approach but in the newborn period risks a situation whereby the transition zone is higher than the sigmoid. We present our experience with the unique patient population of older HD patients in whom the transition zone was reliably reachable via a single-stage transanal approach, performed in prone position. Patients between 2 and 6 years of age with a rectal or sigmoid …

The Early "Unnatural" History Following Surgical Repair Of Ventricular Septal Defects., Sathish M. Chikkabyrappa, Justin T. Tretter, Arpan R. Doshi, Sujatha Buddhe, Puneet Bhatla, Achi Ludomirsky

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Introduction: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed.

Methods: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed.

Results: The majority of …

Successful Brace Treatment Of Pectus Carinatum In Osteogenesis Imperfecta Using The Dynamic Compression System., Beth A. Orrick, Amy L. Pierce, Charles L. Snyder, Uri S. Alon

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Osteogenesis imperfecta (OI) is a genetic disorder of collagen resulting in a "fragile" skeleton with increased fracture risk and other complications, dependent on the specific variant. Pectus deformities of the chest wall, while not common, can be associated with OI. The use of a pectus carinatum brace in a patient with OI poses unknown risks for fractures and adverse treatment outcomes. We successfully applied external compression bracing using the dynamic compression system to one such patient. This case illustrates the ability to treat an OI patient with pectus carinatum using a nonsurgical brace, without complications, resulting in an excellent cosmetic …

Percutaneous Transhepatic Cholecysto-Cholangiography (Ptcc): An Alternative To Intraoperative Cholangiography In High Risk Infants Suspect For Biliary Atresia, Joseph Sujka, Katrina L. Weaver, Ashwini S. Poola, Douglas C. Rivard, Richard J. Hendrickson

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Infants with cholestasis and conjugated hyperbilirubinemia persisting beyond two weeks of age must be evaluated promptly to exclude biliary atresia (BA). BA progresses to cirrhosis and liver failure if portoenterostomy (PE) is not performed in a timely fashion. The gold standard to diagnose BA is intraoperative cholangiography (IOC) via laparotomy. Neonates that are critically ill or have significant co-morbidities may be high-risk surgical candidates, and thus minimizing operative time or avoiding an operation may be beneficial. An additional modality for excluding BA is percutaneous transhepatic cholecysto-cholangiography (PTCC). We present three infants undergoing PTCC by interventional radiology (IR) revealing patency of …

Benchmarking Rectal Cancer Care: Institutional Compliance With A Longitudinal Checklist., William C. Chapman, Pamela Choi, Alexander T. Hawkins, Steven R. Hunt, Matthew L. Silviera, Paul E. Wise, Matthew G. Mutch, Sean C. Glasgow

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BACKGROUND: In 2012, the American Society of Colon and Rectal Surgeons published the Rectal Cancer Surgery Checklist, a consensus document listing 25 essential elements of care for all patients undergoing radical surgery for rectal cancer. The authors herein examine checklist adherence in a mature, multisurgeon specialty academic practice.

MATERIALS AND METHODS: A retrospective medical record review of patients undergoing elective radical resection for rectal adenocarcinoma over a 23-mo period was conducted. Checklists were completed post hoc for each patient, and these results were tabulated to determine levels of compliance. Subgroup analyses by compliance and experience levels of the treating surgeon …

Effect Of Hemiepiphysiodesis On The Growth Plate: The Histopathological Changes And Mechanism Exploration Of Recurrence In Mini Pig Model., Jing Ding, Jin He, Zhi-Qiang Zhang, Zhen-Kai Wu, Fang-Chun Jin

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Purpose: Hemiepiphysiodesis has been widely used to correct angular deformity of long bone in immature patients. However, there is a limited knowledge about the biomechanical effect of this technique on the histopathological changes of the growth plate and the mechanism of recurrence of malformation after implant removal. We aimed to evaluate the biomechanical effect of hemiepiphysiodesis on the histopathological changes of the growth plate and the mechanism of recurrence of malformation after implant removal in Bama miniature pigs, and to explore the role of asymmetric stress during this procedure.

Methods: Eight 3-month-old male Bama miniature pigs sustained surgeries on the …

In Vivo Evaluation Of Stem Cell Aggregates On Osteochondral Regeneration., Banupriya Sridharan, Amy D. Laflin, Michael A. Holtz, Donna M. Pacicca, Nicholas K. Wischmeier, Michael S. Detamore

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To date, many osteochondral regenerative approaches have utilized varied combinations of biocompatible materials and cells to engineer cartilage. Even in cell-based approaches, to date, no study has utilized stem cell aggregates alone for regenerating articular cartilage. Thus, the purpose of this study was to evaluate the performance of a novel stem cell-based aggregate approach in a fibrin carrier to regenerate osteochondral defects in the Sprague-Dawley rat trochlear groove model. Two different densities of rat bone marrow mesenchymal stem cell (rBMSC) aggregates were fabricated by the hanging drop technique. At 8 weeks, the cell aggregates supported the defects and served as …

Re-Tubularization Of Highly-Ischemic Anti-Mesenteric Border (Rehab): A Novel Bowel Preservation Technique In Complex Gastroschisis., Richard J. Hendrickson, Ashwini S. Poola, Katherine W. Gonzalez, Joel Lim, Tolulope A. Oyetunji

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Complex gastroschisis with bowel necrosis poses an operative challenge. Surgeons must weigh the decision between resection versus preservation of ischemic bowel. As one of the leading causes of short bowel syndrome, aggressive resection in complicated gastroschisis subjects children to prolonged dependence on parenteral nutrition and its attendant complications. Herein, we describe a novel technique aimed towards bowel preservation in complex gastroschisis patients with severe bowel ischemia with the ultimate goal for enteral autonomy.