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Background: Pneumatosis intestinalis (PI) is characterized by the presence of intramural gas in the gastrointestinal (GI) tract. The overall aim of this study was to review risk factors and outcome of pediatric oncology patients at our institution who developed PI. Procedure: Patients diagnosed with PI between 2007 and 2018 were identified from ICD-10 coding of radiology reports at Memorial Sloan Kettering Kids, a tertiary pediatric oncology center. Outcomes of interest were (a) resolution and time to resolution of PI, (b) surgical intervention within 2 weeks of diagnosis of PI, or (c) death secondary to PI. To capture the resolution of PI, we defined the "time to recovery (TTR)" as the time elapsed between date of PI diagnosis and the date of recovery.Results: Forty-two patients were identified. Within 30 days of diagnosis of PI, three patients had surgical intervention for PI (7%) and two patients died (5%) due to non-PI causes. Median TTR of PI was 4.5 days (95% CI: 3-7 days). In univariable and multivariable analyses, only steroid use in the prior 30 days was significantly associated with a faster TTR of PI (HR = 2.27 [95% CI: 1.17-4.41], p = .02).Conclusions: This is the largest case series of patients with PI in the pediatric oncology population, which reveals significantly lower surgical and mortality rates than other published PI series. For the majority of patients, conservative medical management is indicated. A prospective study is warranted to define diagnosis and management guidelines for PI in the pediatric oncology population in a cooperative group setting.

Background Ehlers–Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications. Case presentation Here, we present a case of a spontaneous sigmoid perforation in a 14-year-old boy. He was initially treated with laparotomy, oversew of the sigmoid perforation and a diverting ileostomy. He developed a complete wound dehiscence and enteroatmospheric fistulae. These were managed with a combination of negative pressure wound therapy and Eakin (TG Eakin™) pouch changes. We discuss the clinical features and presentation of EDS type IV, the surgical implications of managing patients with the condition, and the challenges in management of enteroatmospheric fistulae in children. Conclusions Ehlers–Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children.

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