Hirschsprung-associated enterocolitis (HAEC) remains the greatest cause of morbidity and mortality in children with Hirschsprung disease. This chapter details the various approaches used to treat and prevent this disease process. This includes prevention of complications such as stricture formation, prophylaxis with rectal washouts and identification of high risk individuals. The chapter also details approaches to diagnose HAEC as well as to exclude other etiologies.

Prognostic study, level III.

Hirschsprung disease (HSCR) is a multigenic condition with variable presentation. Most commonly, it presents in the neonatal period as a functional intestinal obstruction secondary to failure of caudal migration of the enteric nervous system. Classically, this manifests as dilated proximal bowel and constricted distal bowel with absent ganglia and hypertrophic nerve trunks. When recognized early, medical and surgical therapies can be instituted to minimize associated morbidity and mortality. This article reviews current understanding of the etiology of HSCR, its multigenic associations, the historical evolution of HSCR diagnosis and treatment, and current HSCR therapies.

Purpose The effect of timing of onset of necrotizing enterocolitis (NEC) on outcomes has not been determined for the full-term infant. In this study we aimed to characterize the full-term NEC population and to evaluate onset of NEC. Methods We performed a two-center retrospective review of all full-term infants (≥ 37 weeks) with a diagnosis of NEC between 1990 and 2012. Patients were identified by ICD-9 and age. Early onset for NEC was ≤ 7 days and late onset after 7 days of life. Demographics, comorbidities, maternal factors, clinical factors, surgical intervention, complications, and mortality were evaluated. Wilcoxon’s test was performed on continuous variables and Fisher’s exact test on categorical data. A p-value b 0.05 was considered significant. Univariate outcomes with a p-value b 0.1 were selected for multivariable analysis. Results Thirty-nine patients (24 boys, 15 girls) with median EGA of 39 weeks were identified. Overall mortality was 18%. Univariate predictors of mortality included congenital heart disease and placement of an umbilical artery (UA) catheter. Multivariate analysis revealed late onset of NEC to be an independent predictor of mortality (OR 90.8, 95% CI 2.6–3121). Conclusion Full-term infants who develop NEC after 7 days of life, have congenital heart disease, and/or need UA catheterization have increased mortality.

Background Image‐guided percutaneous core needle biopsy (PCNB) is increasingly utilized to diagnose solid tumors. The objective of this study is to determine whether PCNB is adequate for modern biologic characterization of neuroblastoma. Procedure A multi‐institutional retrospective study was performed by the Pediatric Surgical Oncology Research Collaborative on children with neuroblastoma at 12 institutions over a 3‐year period. Data collected included demographics, clinical details, biopsy technique, complications, and adequacy of biopsies for cytogenetic markers utilized by the Children's Oncology Group for risk stratification. Results A total of 243 children were identified with a diagnosis of neuroblastoma: 79 (32.5%) tumor excision at diagnosis, 94 (38.7%) open incisional biopsy (IB), and 70 (28.8%) PCNB. Compared to IB, there was no significant difference in ability to accurately obtain a primary diagnosis by PCNB (95.7% vs 98.9%, P = .314) or determine MYCN copy number (92.4% vs 97.8%, P = .111). The yield for loss of heterozygosity and tumor ploidy was lower with PCNB versus IB (56.1% vs 90.9%, P < .05; and 58.0% vs. 88.5%, P < .05). Complications did not differ between groups (2.9 % vs 3.3%, P = 1.000), though the PCNB group had fewer blood transfusions and lower opioid usage. Efficacy of PCNB was improved for loss of heterozygosity when a pediatric pathologist evaluated the fresh specimen for adequacy. Conclusions PCNB is a less invasive alternative to open biopsy for primary diagnosis and MYCN oncogene status in patients with neuroblastoma. Our data suggest that PCNB could be optimized for complete genetic analysis by standardized protocols and real‐time pathology assessment of specimen quality.

Percutaneous liver biopsy is safe with a low rate of bleeding-related complications. Ensuring adequate sample length and careful patient selection may further increase the diagnostic yield.

S martphones are one of the fastest-growing sectors in the technology industry, and they continue to evolve to combine faster processors, better memory, and more efficient operating systems into a compact handheld device. Smartphones also offer a dynamic tool for use in personal and professional environments. 1 The role of smartphones in medicine continues to expand as additional uses and applications emerge. An estimated 80% of physicians, trainees, and medical students use smartphones, and this percentage is expected to increase.2-4 Smartphones provide a multifaceted platform for mobile health care, allowing users to access a vast amount of information and interact with resources conveniently and quickly.5 Applications range from patient monitoring to use as a tool for diagnosis, to communication and medical education.Recent advances in smartphone technology have led many educators to extend their teaching methods into the mobile learning environment, providing an ''anytime, anywhere'' approach to learning. Mobile learning has been shown to have efficacy within the traditional classroom environment, and brief communications via short message service (SMS) supplement interactive classroom sessions, resulting in enhanced interest in and attention to classroom activity.6 Given the nature of graduate medical education (GME), where trainees are expected to assimilate a vast amount of information that is constantly evolving, and often are away from traditional classroom settings, the benefits of mobile learning with its uninterrupted access to educational resources can be particularly advantageous.In this perspective, we characterize the current and potential uses of smartphone technology in GME and provide recommendations for future studies on incorporating smartphone technology as an educational platform. Smartphones and EducationThe smartphone, in addition to having voice and text communication capabilities, is equipped with Internet access, a high-quality camera, and a recording device. With each new generation, smartphones come with more memory, faster computing capabilities, bigger screens, and sharper resolution. These features allow for instant and reliable access not only to the Internet and its wealth of information, but also for constant social connectivity to personal and professional peer groups. Given these functionalities, the potential for smartphone technology to enhance traditional educational methods is tremendous.Smartphones provide several mechanisms through which educational interventions can be administered (T A B L E). Mobile learning makes use of web-based and mobile-based platforms already in common use among young adults and the public. In fact, up to 35% of Americans use a smartphone, 7 and development of educational material into text messages, applications (apps), or mobile website formats has the potential to target a broad audience. Incorporation of information on social networking sites into curricula also is feasible. The trend toward universal smartphone ownership and trainees' wil...

Laparoscopic duodenoduodenostomy for congenital duodenal obstruction is a technically challenging procedure with a steep learning curve. Despite a relatively high conversion rate, clinical outcomes remained similar to the traditional open repair in selected patients.