(F)utility of preoperative pulmonary function testing in pectus excavatum to assess severity.

PURPOSE: The utility of pulmonary function testing (PFT) in pectus excavatum (PE) has been subject to debate. Although some evidence shows improvement from preoperative to postoperative values, the clinical significance is uncertain. A high failure-to-completion rate for operative PFT (48%) was identified in our large institutional cohort. With such a high non-completion rate, we questioned the overall utility of PFT in the preoperative assessment of PE and sought to evaluate if other measures of PE severity or cardiopulmonary function could explain this finding. METHODS: Demographics, clinical findings, and results from cardiac MRI, PFT (spirometry and plethysmography), and cardiopulmonary exercise tests (CPET) were reviewed in 270 patients with PE evaluated preoperatively between 2015 and 2018. Regression modeling was used to measure associations between PFT completion and cardiopulmonary function. RESULTS: There were no differences in demographics, symptoms, connective tissue disorders, or multiple indices of pectus severity and cardiac deformation in PFT completers versus non-completers. While regression analysis revealed higher RVEF, LVEF, and LVEF-Z scores, lower RV-ESV/BSA, LV-ESV/BSA, and LV-ESV/BSA-Z scores, and abnormal breathing reserve in PFT completers vs. non-completers, these findings were not consistent across continuous and binary analyses. CONCLUSIONS: We found that PFT completers were not significantly different from non-completers in most structural and functional measures of pectus deformity and cardiopulmonary function. Inability to complete PFT is not an indicator of pectus severity.

Outcomes From a Nurse Practitioner Led Dynamic Compression System Bracing Program for Pectus Carinatum.

BACKGROUND: Dynamic compression system (DCS) is often effective at treating pectus carinatum (PC). However, some patients will fail therapy. This study reports outcomes from a nurse-practitioner led bracing program, and evaluates what factors are predictive of successful therapy. METHODS: We performed a retrospective cohort study involving all patients treated with DCS bracing at our institution between February 2018 and February 2022. Patients with at least three visits were included. The primary outcome was achieving neutral chest. Factors considered potentially predictive included patient age, sex, initial pressure of correction (PIC), and the change in pressure of correction between the first two visits (deltaPC1). A Cox proportional hazards model was used for analysis, and Kaplan-Meier analyses estimated the median time to correction. RESULTS: 283 patients were evaluated. The median age was 14 (IQR 12-15), the majority were male (90.1 %) and white (92.6 %). The median PIC and deltaPC1was 4.13 PSI (IQR 3.17-5.3), and 1.34 PSI (IQR 0.54-2.25), respectively. 117 patients achieved correction. The median estimated time to correction was 7.5 months (95 % CI 5.9-10.1). In the final Cox model, greater deltaPC1 was associated with increased risk of correction (HR: 2.46; 95 % CI 2.03-2.98), and increased PIC was associated with decreased risk of correction up to one year of therapy (0-3 months HR 0.62, 95 % CI 0.50-0.78; 3-12 months HR 0.62; 95 % CI 0.45-0.85). CONCLUSIONS: DCS bracing administered by advanced care providers in collaboration with surgeons can effectively treat PC. The deltaPC1 and PIC are the factors most predictive of successful therapy. LEVEL OF EVIDENCE: Level III.

Pediatric Cervical Spine Injury Following Blunt Trauma in Children Younger Than 3 Years: The PEDSPINE II Study.

Importance: There is variability in practice and imaging usage to diagnose cervical spine injury (CSI) following blunt trauma in pediatric patients. Objective: To develop a prediction model to guide imaging usage and to identify trends in imaging and to evaluate the PEDSPINE model. Design, Setting, and Participants: This cohort study included pediatric patients (<3 years years) following blunt trauma between January 2007 and July 2017. Of 22 centers in PEDSPINE, 15 centers, comprising level 1 and 2 stand-alone pediatric hospitals, level 1 and 2 pediatric hospitals within an adult hospital, and level 1 adult hospitals, were included. Patients who died prior to obtaining cervical spine imaging were excluded. Descriptive analysis was performed to describe the population, use of imaging, and injury patterns. PEDSPINE model validation was performed. A new algorithm was derived using clinical criteria and formulation of a multiclass classification problem. Analysis took place from January to October 2022. Exposure: Blunt trauma. Main Outcomes and Measures: Primary outcome was CSI. The primary and secondary objectives were predetermined. Results: The current study, PEDSPINE II, included 9389 patients, of which 128 (1.36%) had CSI, twice the rate in PEDSPINE (0.66%). The mean (SD) age was 1.3 (0.9) years; and 70 patients (54.7%) were male. Overall, 7113 children (80%) underwent cervical spine imaging, compared with 7882 (63%) in PEDSPINE. Several candidate models were fitted for the multiclass classification problem. After comparative analysis, the multinomial regression model was chosen with one-vs-rest area under the curve (AUC) of 0.903 (95% CI, 0.836-0.943) and was able to discriminate between bony and ligamentous injury. PEDSPINE and PEDSPINE II models' ability to identify CSI were compared. In predicting the presence of any injury, PEDSPINE II obtained a one-vs-rest AUC of 0.885 (95% CI, 0.804-0.934), outperforming the PEDSPINE score (AUC, 0.845; 95% CI, 0.769-0.915). Conclusion and Relevance: This study found wide clinical variability in the evaluation of pediatric trauma patients with increased use of cervical spine imaging. This has implications of increased cost, increased radiation exposure, and a potential for overdiagnosis. This prediction tool could help to decrease the use of imaging, aid in clinical decision-making, and decrease hospital resource use and cost.

Partnering with High-Risk Communities to Successfully Reduce Pediatric Injury Over Time.

PURPOSE: Injury is the leading cause of childhood morbidity and mortality. Injury prevention (IP) initiatives are often created in isolation from the communities most affected. We hypothesized that the use of a comprehensive approach to injury prevention through community partnerships will result in a measurable reduction in pediatric injuries. METHODS: The IP program at our free-standing level 1 pediatric trauma center developed partnerships within eight targeted high-risk communities. IP coordinators and community partners implemented programs driven by community-specific injury data and community input. Programs focused on home, bike, playground, pedestrian, and child passenger safety. Program components included in-home education with free safety equipment and installation; free bike helmet fittings and distribution; community playground builds; and car seat classes with education, free car seat distribution and installation. Using trauma registry data, we compared injuries rates in targeted communities with non-intervention communities county-wide over an eight-year period. RESULTS: Between 2012 and 2019, nearly 4000 families received home safety equipment and education through community partnerships. Approximately 2000 bike helmets, 900 car/booster seats, in addition to safety messages and education were provided across the intervention communities. Over this 8-year time period, the injury rates significantly decreased by 28.4%, across the eight targeted high-risk communities, compared to a 10.9% reduction in non-intervention communities across the county. CONCLUSIONS: Effective injury prevention can be achieved through partnerships, working in solidarity with community members to address actual areas of concern to them. Sharing data, seeking ongoing community input, continuously reviewing learnings, and implementing identified changes are crucial to the success of such partnerships. LEVEL OF EVIDENCE: Level III.

Retrospective study comparing outcomes of multimodal epidural and erector spinae catheter pain protocols after pectus surgery.

INTRODUCTION: There are no optimal postoperative analgesia regimens for Nuss procedures. We compared the effectiveness of thoracic epidurals (EPI) and novel ambulatory erector spinae plane (ESP) catheters as part of multimodal pain protocols after Nuss surgery. METHODS: Data on demographics, comorbidities, perioperative details, length of stay (LOS), in hospital and post discharge pain/opioid use, side effects, and emergency department (ED) visits were collected retrospectively in children who underwent Nuss repair with EPI (N = 114) and ESP protocols (N = 97). Association of the group with length of stay (LOS), in hospital opioid use (intravenous morphine equivalents (MEq)/kg over postoperative day (POD) 0-2), and oral opioid use beyond POD7 was analyzed using inverse probability of treatment weighting (IPTW) with propensity scores, followed by multivariable regression. RESULTS: Groups had similar demographics. Compared to EPI, ESP had longer block time and higher rate of ketamine and dexmedetomidine use. LOS for ESP was 2 days IQR (2, 2) compared to 3 days IQR (3, 4) for EPI (p < 0.01). Compared to EPI, ESP group had higher opioid use (in MEq/kg) intraoperatively (0.32 (IQR 0.27, 0.36) vs. 0.28 (0.24, 0.32); p < 0.01) but lower opioid use on POD 0 (0.09 (IQR 0.04, 0.17) vs. 0.11 (0.08, 0.17); p = 0.03) and POD2 (0.00 (IQR 0.00, 0.00) vs. 0.04 (0.00, 0.06) ; p < 0.01). ESP group also had lower total in hospital opioid use (0.57 (IQR 0.42, 0.73) vs.0.82 (0.71, 0.91); p < 0.01), and shorter duration of post discharge opioid use (6 days (IQR 5,8) vs. 9 days (IQR 7,12) (p < 0.01). After IPTW adjustment, ESP continued to be associated with shorter LOS (difference -1.20, 95% CI: -1.38, -1.01, p < 0.01) and decreased odds for opioid use beyond POD7 (OR 0.11, 95% CI: 0.05, 0.24); p < 0.01). However, total in hospital opioid use in MEq/kg (POD0-2) was now similar between groups (difference -0.02 (95% CI: -0.09, -0.04); p = 0.50). The EPI group had higher incidence of emesis (29% v 4%, p < 0.01), while ESP had higher catheter malfunction rates (23% v 0%; p < 0.01) but both groups had comparable ED visits/readmissions. DISCUSSION/CONCLUSION: Compared to EPI, multimodal ambulatory ESP protocol decreased LOS and postoperative opioid use, with comparable ED visits/readmissions. Disadvantages included higher postoperative pain scores, longer block times and higher catheter leakage/malfunction. LEVELS OF EVIDENCE: Level III.

Does age matter? Cardiopulmonary profiles of adolescents versus adults with pectus excavatum presenting for surgical evaluation.

PURPOSE: We sought to analyze differences in presentation and cardiopulmonary function between those referred for surgical consultation as adolescents (11-17 years) versus adults (18 + years). METHODS: Presenting symptoms, past medical history, and results from cardiac MRI (CMR), pulmonary function testing (PFT), and cardiopulmonary exercise testing (CPET) were reviewed in 329 patients evaluated preoperatively between 2015 and 2018. Adjusted regression modeling was used to measure associations between pectus indices and clinical endpoints of cardiopulmonary function. RESULTS: Our sample included 276 adolescents and 53 adults. Adults presented more frequently with chest pain (57% vs. 38%, p = 0.01), shortness of breath (76% vs. 59%, p = 0.02), palpitations (21% vs. 11%, p = 0.04), and exercise intolerance (76% vs. 59%, p = 0.02). Their Haller indices (5.2 [4.2, 7.0] vs. 4.7 [4.0, 5.7], p = 0.05) and cardiac asymmetry (1.8 [0.5] vs. 1.6 [0.5], p = 0.02) were also higher. In continuous outcome analysis, adolescents had higher FEV1/FVC on PFT and higher work on CPET (p < 0.01). CONCLUSIONS: Adults with pectus excavatum were more symptomatic than adolescents with deeper, more asymmetric deformities, decreased FEV1/FVC and exercise capacity. These findings may support earlier versus later repair to prevent age-related decline. Further studies are warranted.

The Severity of Pectus Excavatum Defect Is Associated With Impaired Cardiopulmonary Function.

BACKGROUND: Repair of pectus excavatum has cosmetic benefits, but the physiologic impact remains controversial. The aim of this study was to characterize the relationship between the degree of pectus excavatum and cardiopulmonary dysfunction seen on cardiac magnetic resonance (CMR) imaging, cardiopulmonary exercise testing (CPET), and pulmonary function testing (PFT). METHODS: A single-center analysis of CMR, CPET, and PFT was conducted. Regression models evaluated relationships between pectus indices and the clinical end points of cardiopulmonary function. RESULTS: Data from 345 CMRs, 261 CPETs, and 281 PFTs were analyzed. Patients were a mean age of 15.2 ± 4 years, and 81% were aged <18 years. The right ventricular ejection fraction (RVEF) was <0.50 in 16% of patients, left ventricular ejection fraction (LVEF) was <0.55 in 22%, RVEF Z-score was < -2 in 32%, and the LVEF Z-score was < -2 in 18%. CPET revealed 33% of patients had reduced aerobic fitness. PFT results were abnormal in 23.1% of patients. Adjusted analyses revealed the Haller index had significant (P < .05) inverse associations with RVEF and LVEF. CONCLUSIONS: The severity of pectus excavatum is associated with ventricular systolic dysfunction. Pectus excavatum impacts right and left ventricular systolic function and can also impact exercise tolerance. The Haller index and correction index may be the most useful predictors of impairment.

Ventilatory limitations are not associated with dyspnea on exertion or reduced aerobic fitness in pectus excavatum.

Exercise intolerance and chest pain are common symptoms in patients with pectus excavatum. To assess if the anatomic extent of pectus deformities determined by the correction index (CI) is associated with a pulmonary impairment at rest and during exercise we performed a retrospective review on pectus patients in our center who completed a symptom questionnaire, cardiopulmonary exercise test (CPET), pulmonary function tests (PFT), and chest magnetic resonance imaging. Of 259 patients studied, dyspnea on exertion and chest pain was reported in 64% and 41%, respectively. Peak oxygen uptake (VO2 ) was reduced in 30% and classified as mild in two-thirds. A pulmonary limitation during exercise was identified in less than 3%. Ventilatory limitations on PFT was found in 26% and classified as mild in 85%. Obstruction was the most common abnormal pattern (11%). There were no differences between patients with normal or abnormal PFT patterns for the CI, VO2, or percentage reporting dyspnea or chest pain. Scatter plots demonstrated significant but weak inverse relationships between the CI and lung volumes at rest and during exercise. Multivariable linear regression modeling evaluating predictors of VO2 demonstrated positive associations with the forced expiratory volume at one second and a negative association with the CI. We conclude that resting PFT patterns have poor correlation with the anatomic extent of the pectus defect, symptomatology or aerobic fitness. Pulmonary limitations on CPET are uncommon and lung volumes during exercise are only minimally associated with the CI.

Pediatric Myocardial T1 and T2 Value Associations with Age and Heart Rate at 1.5 T.

The objective of the study was to determine normal global left ventricular reference values for T1 and T2 in children. This is a retrospective study that included healthy subjects, age 5-19 years, who underwent CMR for the indication of pectus excavatum from 2018 to 2019. Linear regression models were used to determine associations of native T1 and T2 values to heart rate, age, and other CMR parameters. 102 patients with a mean age of 14.0 ± 2.4 years were included (range 5.4-18.8). 87 (85%) were males and 15 (15%) were females. The mean global T1 was 1018 ± 25 ms and the mean T2 was 53 ± 3 ms. T1 was negatively correlated with age (r = - 0.39, p < 0.001) and positively correlated with heart rate (r = 0.32, p < 0.001) by univariate analysis. Multivariable analysis showed that age and heart rate were independently associated with T1. T2 demonstrated a weak negative correlation with age (r = - 0.20, p = 0.047) and no correlation with heart rate. There was no difference in T1 (p = 0.23) or T2 (p = 0.52) between genders. This study reports normal pediatric T1 and T2 values at a 1.5 Tesla scanner. T1 was dependent on age and heart rate, while T2 was less dependent on age with no correlation with heart rate.

Standardization of clinical care pathway leads to sustained decreased length of stay following Nuss pectus repair: A multidisciplinary quality improvement initiative.

BACKGROUND AND PURPOSE: Postoperatively, standardized clinical care pathways (SCCPs) help patients reach necessary milestones for discharge. The objective of this study was to achieve 90% compliance with a pectus specific SCCP within 9 months of implementation. We hypothesized that adherence to a pectus SCCP following the Nuss procedure would decrease postsurgical length of stay (LOS). METHODS: A multidisciplinary team implemented the pectus SCCP, including goals for mobility, lung recruitment, pain control, intake, and output. The full protocol included 42 components, tracked using chart reviews and a patient-directed checklist. The primary process measure was compliance with the pectus SCCP. The primary outcome measure was LOS; secondary outcomes were patient charges, patient satisfaction, and hospital readmission. RESULTS: Total study patients were n = 509: 159 patients pre-intervention, 350 patients post-intervention (80 implementation group; 270 sustain group). SCCP compliance data were collected on 164 patients post-intervention - 80 implementation, 84 sustain. LOS, ED visits, and hospital readmissions were recorded for all 509 patients. Mean LOS decreased from 4.5 days to 3.4 days, with >90% adherence to the pectus SCCP postintervention. There were no readmissions owing to pain despite earlier termination of epidural analgesia. Total patient charges decreased by 30% and patient satisfaction was high. CONCLUSION: Using quality improvement methodology with strict adherence to a pectus SCCP, we had significant reduction in LOS and patient charge without compromising effective postoperative pain management or patient satisfaction. TYPE OF STUDY: Clinical research; quality improvement. LEVEL OF EVIDENCE: V.

The chest wall gender divide: females have better cardiopulmonary function and exercise tolerance despite worse deformity in pectus excavatum.

PURPOSE: Pectus excavatum (PE) is a chest wall deformity of variable severity and symptomatology. Existing female-specific literature highlights breast asymmetry and cosmetic reconstruction. We sought to evaluate gender differences in cardiopulmonary function. METHODS: Cardiac MRIs, pulmonary function tests (PFTs), and cardiopulmonary exercise tests (CPETs) were reviewed in 345 patients undergoing preoperative evaluation for PE. Regression modeling was used to evaluate associations between gender and clinical endpoints of cardiopulmonary function. RESULTS: Mean age was 15.2 years, 19% were female, 98% were white. Pectus indices included median Haller Index (HI) of 4.8, mean depression index (DI) of 0.63, correction index (CI) of 33.6%, and Cardiac Compression Index (CCI) of 2.79. Cardiac assessment revealed decreased right and left ventricular ejection fraction (RVEF, LVEF) in 16% and 22% of patients, respectively. PFTs and CPETs were abnormal in ~ 30% of patients. While females had deeper PE deformities-represented by higher pectus indices-they had superior function with higher RVEF, LVEF Z-scores, FEV1, VO2 max, O2 pulse, work, and breathing reserve (p < 0.05). CONCLUSION: Despite worse PE deformity and symptomatology, females had a better cardiopulmonary function and exercise tolerance than males. Further research is needed to assess the precise mechanisms of this phenomenon and postoperative outcomes in this population.

Small bowel-small bowel intussusception with high grade obstruction due to intramural submucosal ileal hamartoma in a 5-year-old child: A case report.

INTRODUCTION: Intussusception is a common cause of emergency in children. We report a unusual case of intestinal obstruction due to small bowel-small bowel intussusception with an intestinal hamartoma as the lead point. PRESENTATION OF THE CASE: A 5 year old boy presented to the ED with worsening abdominal pain, emesis and bloody diarrhea. An abdominal radiograph showed evidence of small bowel obstruction and US confirmed a small bowel-small bowel intussusception with a likely cystic mass as the lead point. Given these findings, surgery was performed and revealed an intestinal hamartoma as a lead point. DISCUSSION: Intussusception in children is mostly idiopathic and usually amenable to reduction by air enema. The presence of a lead point is unusual and usually requires surgical intervention. CONCLUSION: We report an unusual case of intussusception with small bowel obstruction due to intestinal hamartoma, a rare pathologic finding as a lead point for intussusception. Ultrasound was useful for diagnosing the intussusception and confirming the presence of a lead point.

Occult RV systolic dysfunction detected by CMR derived RV circumferential strain in patients with pectus excavatum.

AIMS: To investigate the right ventricular (RV) strain in pectus excavatum (PE) patients using cardiac magnetic resonance tissue tracking (CMR TT). MATERIALS AND METHODS: Fifty consecutive pectus excavatum patients, 10 to 32 years of age (mean age 15 ± 4 years), underwent routine cardiac magnetic resonance imaging (CMR) including standard measures of chest geometry and cardiac size and function. The control group consisted of 20 healthy patients with a mean age of 17 ± 5 years. RV longitudinal and circumferential strain magnitude was assessed by a dedicated RV tissue tracking software. RESULTS: Fifty patients with images of sufficient quality were included in the analysis. The mean right and left ventricular ejection fractions were 55 ± 5% and 59 ± 4%. The RV global longitudinal strain was -21.88 ± 4.63%. The RV circumferential strain at base, mid-cavity and apex were -13.66 ± 3.09%, -11.31 ± 2.79%, -20.73 ± 3.45%, respectively. There was no statistically significant decrease in right ventricular or left ventricular ejection fraction between patients and controls (p > 0.05 for each). There was no significant difference in RV global longitudinal strain between two groups (-21.88 ± 4.63 versus -21.99 ± 3.58; p = 0.93). However, there was significant decrease in mid-cavity circumferential strain magnitude in pectus patients compared with controls (-11.31 ± 2.79 versus -16.19 ± 2.86; p < 0.001). PE patients had a significantly higher basal circumferential strain (-13.66 ± 3.09% versus -9.76 ± 1.79; p < 0.001) as well as apical circumferential strain (-20.73 ± 3.45% versus -12.07 ± 3.38) than control group. CONCLUSION: Mid-cavity circumferential strain but not longitudinal strain is reduced in pectus excavatum patients. Basal circumferential strain as well as apical circumferential strain were increased as compensatory mechanism for reduced mid-cavity circumferential strain. Further studies are needed to establish clinical significance of this finding.

Surgical site infection related to use of elastomeric pumps in pectus excavatum repair. Lessons learned from root cause analysis.

BACKGROUND: Pectus excavatum repair (PEX) is among the most painful thoracic procedures performed. Continuous peripheral nerve blockade (CPNB) is known to be efficacious in optimizing pain control while limiting narcotic use in adult thoracic procedures. It was introduced in May 2015 as a bridge to oral pain control in children undergoing PEX. Consequently, the surgical site infection (SSI) rate increased from 2.7% to 27.7%. METHODS: SSI surveillance followed national guidelines. The abrupt increase prompted root cause analysis and cessation of CPNB use. A dynamic systems model of SSI in PEX was developed. Statistical analysis compared SSI outcomes with and without CPNB. RESULTS: From May 2015 to June 2015, 21 PEX were performed; 11 with CPNB. 6 SSIs were observed. Use of CPNB significantly (p=0.008) increased SSI incidence. Haller index, number of bars, usage of Fiberwire®, methicillin resistant S. aureus colonization and length of stay did not differ. Root cause analysis revealed the proximity of CPNB catheters to the wound, the use of CPNB with implanted hardware and a delayed utilization of CPNB catheters to be of concern. CONCLUSION: Introduction of CPNB coincided with a significant increase in SSI. Further study is needed to assess the safety of CPNB in pediatric PEX. LEVEL OF EVIDENCE: Level III treatment study.

Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia.

PURPOSE: The purpose of this study was to define the hematologic response to total splenectomy (TS) or partial splenectomy (PS) in children with hereditary spherocytosis (HS) or sickle cell disease (SCD). METHODS: The Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium registry collected hematologic outcomes of children with CHA undergoing TS or PS to 1 year after surgery. Using random effects mixed modeling, we evaluated the association of operative type with change in hemoglobin, reticulocyte counts, and bilirubin. We also compared laparoscopic to open splenectomy. RESULTS: The analysis included 130 children, with 62.3% (n=81) undergoing TS. For children with HS, all hematologic measures improved after TS, including a 4.1g/dl increase in hemoglobin. Hematologic parameters also improved after PS, although the response was less robust (hemoglobin increase 2.4 g/dl, p<0.001). For children with SCD, there was no change in hemoglobin. Laparoscopy was not associated with differences in hematologic outcomes compared to open. TS and laparoscopy were associated with shorter length of stay. CONCLUSION: Children with HS have an excellent hematologic response after TS or PS, although the hematologic response is more robust following TS. Children with SCD have smaller changes in their hematologic parameters. These data offer guidance to families and clinicians considering TS or PS.

Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.

Fetal MRI of hereditary multiple intestinal atresia with postnatal correlation.

Hereditary multiple intestinal atresia (HMIA) is an extremely uncommon cause of congenital bowel obstruction. The morbidity and mortality of this disease differ significantly from those of isolated intestinal atresias and non-hereditary forms of multiple intestinal atresia. Most notably, despite successful operative repairs of the atresias found in this disease, HMIA maintains a 100% lethality rate from continued post-operative intestinal failure and an associated severe immunodeficiency. We present a case of HMIA evaluated with fetal MRI and subsequently diagnosed by a combination of corroborative postnatal imaging with surgical exploration and pathological examination.

Role of computed tomography and clinical findings in pediatric blunt intestinal injury: a multicenter study.

PURPOSE: Although computed tomographic (CT) scans are accurate in diagnosing solid-organ injuries, their ability to diagnose a blunt intestinal injury (BII) is limited, occasionally requiring repeated imaging. The purpose of this study was to evaluate the role of clinical findings as well as original and repeated CT imaging in the ultimate decision to operate for BII. METHODS: An 18-institution record review of children (≤ 15 years) diagnosed with a BII confirmed during surgery between 2002 and 2007 was conducted by the American Pediatric Surgery Association Trauma Committee. The incidence of imaging, repeated imaging, and final reported indications for operative exploration were evaluated. RESULTS: Among 331 patients identified with a BII, 292 (88%) underwent at least 1 abdominal CT scan. Sixty-two (19%) underwent at least 1 repeated scan before operation. Forty-seven percent of children who underwent a CT scan were taken to the operating room based primarily on clinical indications (fever, abdominal pain, shock or elevated white blood cell count), whereas 31% were operated on based on both a clinical and CT indication and 22% were operated on based on a CT indication alone (P < 0.001). Although free air was the most common radiographic indication for surgery, 13% of patients with a repeated scan had free air diagnosed on their first CT. Most children undergoing a repeated CT (84%) had findings on the original scan suggesting a BII. Among the 10 patients whose first CT scan result was normal, only 1 went to the operating room based only on radiographic findings. Children who had their first CT scan at a referring hospital were more likely to have a repeated study compared with those imaged at a trauma center (33% vs 13%, P < 0.0001). CONCLUSIONS: Although abdominal CT imaging may contribute to diagnosing intestinal injury after blunt trauma, most children undergo operation based on clinical findings. Repeated imaging should be limited to select patients with diagnostic uncertainty to avoid unneeded delay and radiation exposure.