Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review

BackgroundHirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence.MethodsPatients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated.ResultsWe reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102).ConclusionFurther prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation.Level of evidenceLevel III.     

Decreased Incidence of Hirschsprung-Associated Enterocolitis During COVID-19 Across United States Children's Hospitals

BackgroundThe Coronavirus Disease 2019 pandemic provided a natural experiment to study the effect of social distancing on the risk of developing Hirschsprung's Associated Enterocolitis (HAEC).MethodsUsing the Pediatric Health Information System (PHIS), a retrospective cohort study of children (<18 years) with Hirschsprung's Disease (HSCR) across 47 United States children's hospitals was performed. The primary outcome was HAEC admissions per 10,000 patient-days. The exposure (COVID-19) was defined as April 2020–December 2021. The unexposed (historical control) period was April 2018–December 2019. Secondary outcomes included sepsis, bowel perforation, intensive care unit (ICU) admission, mortality, and length of stay.ResultsOverall, we included 5707 patients with HSCR during the study period. There were 984 and 834 HAEC admissions during the pre-pandemic and pandemic periods, respectively (2.6 vs. 1.9 HAEC admissions per 10,000 patient-days, incident rate ratio [95% confidence interval]: 0.74 [0.67, 0.81], p < 0.001). Compared to pre-pandemic, those with HAEC during the pandemic were younger (median [IQR]: 566 [162, 1430] days pandemic vs. 746 [259, 1609] days pre-pandemic, p < 0.001) and more likely to live in the lowest quartile of median household income zip codes (24% pandemic vs. 19% pre-pandemic, p = 0.02). There were no significant differences in rates of sepsis (6.1% pandemic vs. 6.1% pre-pandemic, p > 0.9), bowel perforation (1.3% pandemic vs. 1.2% pre-pandemic, p = 0.8), ICU admissions (9.6% pandemic vs. 12% pre-pandemic, p = 0.2), mortality (0.5% pandemic vs. 0.6% pre-pandemic, p = 0.8), or length of stay (median [interquartile range]: 4 [(Pastor et al., 2009; Gosain and Brinkman, 2015) 2,112,11 days pandemic vs. 5 [(Pastor et al., 2009; Tang et al., 2020) 2,102,10 days pre-pandemic, p = 0.4).ConclusionsThe COVID-19 pandemic was associated with significantly decreased incidence of HAEC admissions across US children's hospitals. Possible etiologies such as social distancing should be explored.Level of evidenceII.     

Nationwide outcomes of newborns with rectosigmoid versus long-segment Hirschsprung disease

PurposeHirschsprung Disease (HD) is a common congenital intestinal disorder. While aganglionosis most commonly affects the rectosigmoid colon (rectosigmoid HD), outcomes for patients in which aganglionosis extends to more proximal segments (long-segment HD) remain understudied. This study sought to compare postoperative outcomes among newborns with rectosigmoid and long-segment HD.MethodsThe Nationwide Readmission Database was queried from 2016 to 2018 for newborns with HD. Newborns were stratified into those with rectosigmoid or long-segment HD. Those who received no rectal biopsy or pull-through procedure during their newborn hospitalization were excluded. A propensity score-matched analysis (PSMA) of newborns with either type of HD was constructed utilizing 17 covariates including demographics, comorbidities, and congenital-perinatal conditions.ResultsThere were 1280 newborns identified with HD (82% rectosigmoid HD, 18% long-segment HD). Patients with rectosigmoid HD had higher rates of laparoscopic resections (35% vs. 12%) and less frequently received a concomitant ostomy (14% vs. 84%), both p < 0.001. Patients with long-segment HD were more likely to have a delayed diagnosis (12% vs. 5%) and require multiple bowel operations (19% vs. 4%), both p < 0.001. They experienced higher rates of complications, including small bowel obstructions (10% vs. 1%), infections (45% vs. 20%), and Hirschsprung-associated enterocolitis (11% vs. 5%), all p < 0.001. After PSMA, newborns with long-segment HD were found to have a longer length of stay and higher hospitalization costs.ConclusionNewborns with long-segment HD experience significant delays in diagnosis, surgery, and complications compared to those with rectosigmoid HD. This information should be utilized to improve healthcare delivery for this patient population.Type of StudyRetrospective comparative study.Level of EvidenceIII.     

Results of staged repair of aortic disease in patients with Marfan syndrome

ObjectiveWe present our open surgical strategies for staged replacement of the thoracic and thoracoabdominal aorta in patients with Marfan syndrome.MethodsBetween October 1999 and December 2017, 82 patients with Marfan syndrome underwent 118 aortic repairs. We divided the aorta into 4 segments for categorization: (1) the aortic root, (2) aortic arch, (3) descending thoracic, and (4) abdominal aorta. Procedures were categorized according to the types of surgery. Staged repair was defined as a subsequent operation on a different segment of the aorta after initial repair (n = 111, 94.1%), and reoperation was defined as an operation on the same segment (n = 7, 5.9%).ResultsThe mean age at initial operation was 41.7 ± 14.9 years. Staged repairs included aortic root replacement (n = 42, 36%), total arch replacement (n = 11, 9.3%), combined aortic root and total arch replacement (n = 13, 11%), descending aorta replacement (n = 4, 3.4%), thoracoabdominal aortic repair (n = 36, 31%), and extensive arch-descending or thoracoabdominal repair (n = 5, 4.2%). Four patients received 3 staged repairs. Operative mortality was 0.8% (1/118). Stroke occurred in 1.7% (2/118), and spinal cord injury occurred in 1.7% (2/117). Overall survival was 95.8 ± 2.4% at 10-years. Twenty-four patients underwent replacement of the whole aorta after 2.5 ± 3.8 years following initial repair.ConclusionsOur strategies for staged replacement of the thoracic and thoracoabdominal aorta in patients with Marfan syndrome resulted in excellent early- and long-term outcomes.     

The Use of the Perioperative Nutrition Score in Postoperative Pediatric Inflammatory Bowel Disease Patients

Background and objectivesPreoperative malnutrition is associated with increased postoperative morbidity. The perioperative nutrition score (PONS) was developed to identify patients at risk of malnutrition. We sought to assess the correlation between preoperative PONS and postoperative outcomes in pediatric inflammatory bowel disease (IBD) patients.MethodsWe performed a retrospective cohort study of IBD patients, less than 21 years of age, who underwent elective bowel resection between June 2018 and November 2021. Patients were divided based upon whether they met PONS criteria. The primary outcome was postoperative surgical site infections.Results96 patients were included. Sixty-one patients (64%) met at least one PONS criteria, while 35 patients (36%) met none. PONS positive patients more frequently received preoperative TPN supplementation (p < .001). There was no difference in preoperative oral nutritional supplementation between groups. Patients that screened positive for PONS had a longer hospital stay (p = .002), more readmissions (p = .029), and more surgical site infections (p = .002).ConclusionsOur data highlight the prevalence of malnutrition in the pediatric IBD population. Patients who screened positive had worse postoperative outcomes. Further, very few of these patients received preoperative optimization with oral nutritional supplementation. There is a need for standardization of nutritional evaluation to improve preoperative nutritional status and postoperative outcomes.Level of EvidenceIII.Type of StudyRetrospective Cohort.     

Derivation and Validation of a Machine Learning Algorithm for Predicting Venous Thromboembolism in Injured Children

BackgroundVenous thromboembolism (VTE) causes significant morbidity in pediatric trauma patients. We applied machine learning algorithms to the Trauma Quality Improvement Program (TQIP) database to develop and validate a risk prediction model for VTE in injured children.MethodsPatients ≤18 years were identified from TQIP (2017–2019, n = 383,814). Those administered VTE prophylaxis ≤24 h and missing the outcome (VTE) were removed (n = 347,576). Feature selection identified 15 predictors: intubation, need for supplemental oxygen, spinal injury, pelvic fractures, multiple long bone fractures, major surgery (neurosurgery, thoracic, orthopedic, vascular), age, transfusion requirement, intracranial pressure monitor or external ventricular drain placement, and low Glasgow Coma Scale score. Data was split into training (n = 251,409) and testing (n = 118,175) subsets. Machine learning algorithms were trained, tested, and compared.ResultsLow-risk prediction: For the testing subset, all models outperformed the baseline rate of VTE (0.15%) with a predicted rate of 0.01–0.02% (p < 2.2e⁻¹⁶). 88.4–89.4% of patients were classified as low risk by the models.High-risk predictionAll models outperformed baseline with a predicted rate of VTE ranging from 1.13 to 1.32% (p < 2.2e⁻¹⁶). The performance of the 3 models was not significantly different.ConclusionWe developed a predictive model that differentiates injured children for development of VTE with high discrimination and can guide prophylaxis use.Level of EvidencePrognostic, Level II.Type of StudyRetrospective, Cross-sectional.     

The efficacy and safety of paclitaxel plus bevacizumab therapy in breast cancer patients with visceral crisis

BackgroundVisceral crisis in metastatic breast cancer (MBC) is defined as severe organ dysfunction requiring rapidly efficacious therapy. Although weekly paclitaxel plus bevacizumab (wPTX + BV) achieves a high response rate in human epidermal growth factor receptor 2 (HER2)-negative MBC, the efficacy and safety of wPTX + BV for visceral crisis is unclear.MethodsWe retrospectively investigated patients with MBC with visceral crisis who received wPTX + BV. Visceral crisis was defined as follows: liver dysfunction (aspartate or alanine aminotransferase >200 U/L or total bilirubin >1.5 mg/dl), respiratory dysfunction (carcinomatous lymphangiomatosis, SpO₂ <93% in ambient air or required thoracentesis), superior vena cava (SVC) syndrome, or bone marrow carcinomatosis. The primary outcome was the proportion of patients on-treatment with wPTX + BV after 12 weeks. We also investigated time to treatment failure (TTF), overall survival (OS), objective response rate (ORR), and adverse events.ResultsA total of 44 patients with respiratory dysfunction (n = 29), liver dysfunction (n = 10), bone marrow carcinomatosis (n = 7), and SVC syndrome (n = 2) were eligible for this investigation. The proportion of patients on-treatment with wPTX + BV after 12 weeks was 63% (30/44), and the other patients discontinued wPTX + BV because of adverse events (n = 5) and disease progression (n = 9). Median TTF and OS, and the ORR were 131 days and 323 days, and 41%, respectively. No treatment-related death occurred.Conclusion: wPTX + BV achieved favorable efficacy and safety for treating patients with visceral crisis and may therefore be considered an option for the treatment of this acutely severe clinical condition.     

Long-Term Esthetic Outcome of Different Treatment Modalities for Nontuberculous Mycobacterial Cervicofacial Lymphadenitis

BackgroundNontuberculous mycobacterial (NTM) cervicofacial lymphadenitis is a rare infection which almost exclusively occurs in children, most commonly children 0–5 years old. It can leave scars in highly visible areas. The present study aimed to evaluate the long-term esthetic outcome of different treatment modalities for NTM cervicofacial lymphadenitis.MethodsThis retrospective cohort study included 92 participants with a history of bacteriologically proven NTM cervicofacial lymphadenitis. All patients were diagnosed at least 10 years prior and were aged >12 years upon enrollment. Based on standardized photographs, the scars were assessed by subjects with the Patient Scar Assessment Scale, and by five independent observers with the revised and weighted Observer Scar Assessment Scale.ResultsThe mean age at initial presentation was 3,9 years and the mean follow-up time was 15.24 years. Initial treatments included surgical treatment (n = 53), antibiotic treatment (n = 29) and watchful waiting (n = 10). Subsequent surgery was performed in two patients, due to a recurrence after initial surgical treatment, and in 10 patients initially treated with antibiotic treatment or watchful waiting. Esthetic outcomes were statistically significantly better with initial surgery, compared to initial non-surgical treatment, based on patient scores of scar thickness, and based on observer scores of scar thickness, surface appearance, general appearance and the revised and weighted sum score of all assessment items.ConclusionsThe long-term esthetic outcome of surgical treatment was superior to non-surgical treatment. These findings could facilitate the process of shared decision making.Level of EvidenceLevel III     

Successful Treatment of Fibro-Adipose Vascular Anomaly with Sirolimus

BackgroundThe purpose of this study was to present our initial experience in using sirolimus therapy to treat fibro-adipose vascular anomaly (FAVA).MethodsWe retrospectively reviewed the medical records of eight patients with FAVA who were treated with sirolimus at our hospital between July 2017 and October 2020.ResultsSix girls (75%) and two boys (25%) were included in the cohort; the average age was 8 years (range, 1–13 years). Vascular tumors developed mainly on the extremities, including the forearm (n = 2; 25.0%), calf (n = 4; 50.0%), and thigh (n = 2; 25.0%). The predominant symptoms included swelling of the lesion (n = 8; 100%), pain (n = 7; 87.5%), contracture (n = 3; 37.5%), and phlebectasia (n = 3; 37.5%). Magnetic resonance imaging was the primary method used for FAVA diagnosis, and all patients underwent enhanced MRI. All lesions were heterogeneous with hyperintense T1 signals. The fat-suppressed T2-weighted images also revealed heterogeneous hyperintense masses, thus indicating fibrofatty infiltration. All eight patients received a sirolimus treatment regimen after FAVA diagnosis. One patient underwent tumor resection but experienced recurrence, whereas the other six patients underwent biopsy. Histological examination revealed that the lesions consisted of fibrofatty tissue with abnormal venous channels and anomalous lymphatic vascular components. Sirolimus softened the masses and caused tumor shrinkage within 5.25 ± 2.6 weeks (range, 2–10 weeks) after treatment initiation. The tumors also involuted rapidly and became stable within 7.75 ± 2.25 months after treatment initiation (range, 6–12 months). All seven patients experiencing pain reported relief within 3.8 ± 1.8 weeks (range, 2–7 weeks) after initiation of sirolimus therapy. Sirolimus alleviated but did not fully resolve the contracture in three patients. Remarkably, five patients exhibited a complete response, and three patients exhibited a partial response. At the time of the last follow-up, three patients had begun to gradually taper off sirolimus after 24 months of treatment and maintained a low blood sirolimus concentration. No serious adverse effects were observed during treatment.ConclusionFAVA is a complex vascular malformation that appears to respond well to sirolimus treatment. Thus, sirolimus may be an effective and safe treatment for FAVA.Level of evidenceLEVEL IV.     

Management of Primary Spontaneous Pneumothorax in Children: A Single Institution Protocol Analysis

BackgroundThe Midwest Pediatric Surgery Consortium (MWPSC) suggested a simple aspiration of primary spontaneous pneumothorax (PSP) protocol, failing which, Video-Assisted Thoracoscopic Surgery (VATS) should be considered. We describe our outcomes using this suggested protocol.MethodsA single institution retrospective analysis was conducted on patients between 12 and 18 years who were diagnosed with PSP from 2016 to 2021. Initial management involved aspiration alone with a ≤12 F percutaneous thoracostomy tube followed by clamping of the tube and chest radiograph at 6 h. Success was defined as ≤2 cm distance between chest wall and lung at the apex and no air leak when the clamp was released. VATS followed if aspiration failed.ResultsFifty-nine patients were included. Median age was 16.8 years (IQR 15.9, 17.3). Aspiration was successful in 33% (20), while 66% (39) required VATS. The median LOS with successful aspiration was 20.4 h (IQR 16.8, 34.8), while median LOS after VATS was 3.1 days (IQR 2.6, 4). In comparison, in the MWPSC study, the mean LOS for those managed with a chest tube after failed aspiration was 6.0 days (±5.5). Recurrence after successful aspiration was 45% (n = 9), while recurrence after VATS was 25% (n = 10). Median time to recurrence after successful aspiration was sooner than that of the VATS group [16.6 days (IQR 5.4, 19.2) vs. 389.5 days (IQR 94.1, 907.0) p = 0.01].ConclusionSimple aspiration is safe and effective initial management for children with PSP, although most will require VATS. However, early VATS reduces length of stay and morbidity.Level of evidenceIV. Retrospective study.     

Preoperative tendon retraction,not smoking,is a risk factor for failure with continuity after rotator cuff repair

BackgroundSmoking is a poor prognostic factor for healing after rotator cuff repair and is associated with inferior results. We hypothesized that smokers would have higher recurrent tear rates and more postoperative myotendinous junction (MTJ) retraction in healed repairs than nonsmokers three months postoperatively.MethodsRotator cuff repairs (RCRs) were retrospectively reviewed over a 2-year period. Patients underwent magnetic resonance imaging (MRI) within 6 months prior to surgery and again at 3 months postoperatively. Seventy-nine patients were included and stratified by smokers versus nonsmokers. Baseline patient demographics, tear characteristics, and surgical factors were collected. Preoperative and postoperative MRIs were assessed to quantify the MTJ position and to establish the recurrent tear rate.ResultsFor the total cohort (nonsmokers, n = 56; smokers, n = 23), significant differences in age, race, and traumatic onset of injury existed between groups. There were no significant differences in recurrent tear between smokers (26%) and nonsmokers (27%), but nonsmokers were more satisfied. For patients with healed RCRs (nonsmokers, n = 41; smokers, n = 17), there were significant differences in race. On univariate analysis, nonsmokers had a significantly more lateral MTJ postoperatively (P = 0.05). On multivariable regression analysis, medialized postoperative MTJ position in healed cuffs was driven only by greater preoperative rotator cuff retraction preoperatively. There were no significant differences in MTJ position based on smoking status for patients with healed RCRs.ConclusionSmoking does not appear to be an independent risk factor for postoperative MTJ retraction in healed RCRs, also known as failure in continuity. Preoperative tear size and retraction play the biggest roles in predicting postoperative MTJ position, regardless of smoking status. There are no significant differences in patient-reported outcomes for patients with healed RCRs, but nonsmokers had more satisfaction following RCR in the total cohort.Level of EvidenceLevel III; Retrospective cohort study; Diagnostic study     

Distribution and Surgical Management of Visceral Ewing Sarcoma Among Children and Adolescents

IntroductionEwing sarcoma (EWS) is a highly malignant tumor of bone and soft tissue that occasionally arises from viscera. Visceral EWS (V-EWS) is challenging to manage given its varied organ distribution and often late-stage presentation. We aimed to characterize our institutional experience with V-EWS, focusing on its surgical management, and to compare V-EWS outcomes against those with osseous (O-EWS) and soft tissue EWS (ST-EWS).MethodsRetrospective review of all EWS patients ≤21 years presenting to a single institution between 2000 and 2022. Patient- and disease-specific characteristics were compared. Overall and relapse-free survival were estimated using Kaplan Meier methods and log-rank test.Results156 EWS patients were identified: 117 O-EWS, 20 ST-EWS, and 19 V-EWS. V-EWS arose in the kidney (n = 5), lung (n = 5), intestine (n = 2), esophagus (n = 1), liver (n = 1), pancreas (n = 1), adrenal gland (n = 1), vagina (n = 1), brain (n = 1), and spinal cord (n = 1). No significant demographic differences were detected between EWS groups. V-EWS was more frequently metastatic at presentation (63.2%; p = 0.005), yet no significant overall or relapse-free survival differences emerged between EWS groups, with similar follow-up intervals. While V-EWS required multiple unique operative strategies to gain primary control, no significant difference in treatment strategies appeared between groups. Surgery-only primary control was associated with improved overall and relapse-free survival in all groups.ConclusionsV-EWS presents unique management challenges in children and adolescents given its variable sites of origin. This large cohort is the first to describe the surgical management and outcomes of V-EWS, demonstrating more frequent metastatic presentation, while achieving similar survival across groups.Level of evidenceLevel 2 – Cohort Study.     

Pathologic concordance of resected metastatic nonseminomatous germ cell tumors in the chest

ObjectiveMen with metastatic nonseminomatous germ cell tumors (NSGCTs) often present with residual chest tumors after chemotherapy. We examined the pathologic concordance of intrathoracic disease and outcomes based on the worst pathology of disease resected at first thoracic surgery.MethodsA retrospective analysis was performed of consecutive patients undergoing thoracic resection for metastatic NSGCT in our institution between 2005 and 2018.ResultsEighty-nine patients (all men) were included. The median age was 29 years (interquartile range [IQR], 23-35 years). Primary sites were testis (n = 84; 94.4%) and retroperitoneum (n = 5; 5.6%). Eighty-seven patients received chemotherapy before undergoing surgery. Nineteen patients (21.3%; group 1) had malignancy resected at first surgery (OR1), and the other 70 patients had benign disease at OR1 (78.7%; group 2). Concordant pathology between lungs was 85.2% in group 1 and 91% in group 2, and between lung and mediastinum was 50% in group 1 and 72.7% in group 2. Despite no teratoma at OR1, 3 patients (15.8%) in group 2 had resection of teratoma (n = 2) or malignancy (n = 1) at future surgery. After a mean follow-up of 65.5 months (IQR, 23.1-89.2 months) for group 1 and 47.7 months (IQR, 13.0-75.1 months) for group 2, overall survival was significantly worse for group 1 (68.4% vs 92.9%; P = .03).ConclusionsThe wide range of pathology resected in patients with intrathoracic NSGCT metastases requires careful decision making regarding treatment. Pathologic concordance between lungs is better than that between lung and mediastinum in patients with intrathoracic NSGCT metastases. Aggressive surgical management should be considered for all residual disease due to the low concordance between sites and the potential for excellent long-term survival even in patients with chemotherapy-refractory disease.     

Mortality after tricuspid valve procedures: A 27-year,single-center experience

ObjectiveTo assess mortality after tricuspid valve (TV) surgery in a large single-center patient cohort.MethodsData from 392 TV procedures performed between 1989 and 2015 in 388 adult patients were retrospectively reviewed. The patients were divided into groups according to the type of concomitant procedure, ie, coronary artery bypass grafting (CABG) (TV + CABG group; n = 87), other valve surgery (TV + valve group; n = 240), or an isolated TV procedure with or without another minor procedure (isolated TV group; n = 65), and the era of the operation, ie, 1989-2005 (n = 173) or 2006-2015 (n = 219). Control groups of patients who underwent other valve procedures and/or CABG during the same time periods were used for comparison.ResultsDuring the most recent era, the annual number of TV procedures increased 2.4-fold, mainly for TV + valve procedures (2.8-fold). Within the TV + valve group, a larger proportion of patients had mild-to-moderate tricuspid regurgitation (grade ≤2) compared with the first-time period (P = .001). The TV + CABG group had significantly greater mortality than both the other groups during both time periods, whereas isolated TV procedure had the lowest mortality rates with the exception of the TV + valve group during the most recent era (P = .41). Survival for patients undergoing TV + valve procedures has improved significantly during the last decade (P = .001) and was comparable with that for other valve operations during this period.ConclusionsIn the last decade, TV repair has been performed more frequently and at lower grades of tricuspid regurgitation compared with previously, and mortality after TV procedures has decreased.     

Long-Term Male Sexual Function and Fecal Incontinence Outcomes for Adult Patients with Hirschsprung Disease or Anorectal Malformation

BackgroundThis study sought to characterize sexual function and fecal incontinence related quality of life (QOL) outcomes for adult males with anorectal malformation (ARM) or Hirschsprung's Disease (HD).MethodsWe conducted a cross-sectional survey study of male patients ≥18 years with ARM or HD. Patients were identified from our institutional database, contacted and consented by telephone, and sent a REDCap survey via email. The International Index of Erectile Function (IIEF-5) and Male Sexual Health Questionnaire (MSHQ) evaluated erectile dysfunction (ED) and ejaculatory dysfunction (EjD), respectively. The Cleveland Clinic Incontinence Score (CCIS) and the Fecal Incontinence Quality of Life Scale (FIQLS) assessed fecal incontinence-related outcomes. A linear regression analysis of IIEF-5 scores compared to CCIS scores was used to evaluate for an association between ED and incontinence.ResultsOf 63 patients contacted, 48 completed the survey. The median age for respondents was 22.5 years (IQR 20–25). There were 19 patients with HD and 29 patients with ARM. On the IIEF-5 survey, 35.3% report some level of ED. On the MSHQ-EjD survey, the median score was 14 out of 15 (IQR 10.75–15), indicating few EjD concerns. The median CCIS was 5 (IQR 2.25–7.75) and the median FIQL scores ranged from 2.7 to 3.5 depending on the domain assessed, demonstrating some QOL challenges secondary to fecal incontinence. On linear regression analysis, IIEF-5 and CCIS scores were weakly associated (B = −0.55, p = 0.045).ConclusionsMale adult patients with ARM or HD may have ongoing concerns with sexual function and fecal incontinence.Level of EvidenceLevel 4.Type of StudyCross-Sectional Survey Study.     

Association of Socioeconomic Status with Empowerment,Depression, and Anxiety in Pregnancies Complicated by Fetal Surgical Anomalies

BackgroundWomen with pregnancies complicated by fetal surgical anomalies experience significant psychosocial burden. There remains a need to determine the impact that socioeconomic status has on maternal empowerment, anxiety, and depression.MethodsA survey study was conducted from 5/2021–5/2022 to quantify empowerment, anxiety, and depression in women with fetal surgical anomalies. Surveys administered included Pregnancy Related Empowerment Scale (PRES), Patient Empowerment Scale (PES), State Trait Anxiety Inventory (STAI), and Depression Anxiety and Stress Scale (DASS). Two-sample t-test was used to compare survey scores across socioeconomic groups.ResultsSeventy-four patients were recruited. Mothers more commonly preferred English as primary language (n = 61, 82%) and were non-Hispanic (n = 43, 58%). Lower empowerment scores were observed in Hispanic mothers (PRES, p = 0.03; PES, p = 0.04) and mothers who preferred Spanish (PRES, p = 0.04; PES, p = 0.06) as primary language. Both non-Hispanic (p = 0.88) and English speaking (p = 0.75) women had higher STAI scores, but neither was significantly different. DASS was not significantly higher for Hispanic (p = 0.79) or Spanish speaking mothers (p = 0.47).ConclusionHispanic and Spanish speaking women with pregnancies complicated by fetal surgical anomalies have significantly decreased empowerment scores. These findings suggest a need for development of culturally competent, targeted interventions to improve maternal empowerment in this high-risk population.Level of EvidenceLevel II.Type of StudyCross-Sectional Survey Study.     

Surgical outcomes of acute type A aortic dissection in patients undergoing cardiopulmonary resuscitation

ObjectivesThe surgical indications for acute type A aortic dissection (AAAD) in patients in cardiopulmonary arrest remain controversial. Outcomes of AAAD for patients who underwent cardiopulmonary resuscitation (CPR) were evaluated.MethodsBetween 2004 and 2018, of the 519 patients who underwent AAAD repair, 34 (6.6%) required CPR before or on starting AAAD repair. The patients were divided into 2 groups, survivors (n = 13) and nonsurvivors (n = 21), to compare the early operative outcomes, including mortality and neurological events.ResultsThe major cause of cardiovascular collapse requiring CPR was aortic rupture/cardiac tamponade (n = 21 [61.8%]), followed by coronary malperfusion (n = 12 [35.3%]) and acute aortic valve regurgitation (n = 3 [8.8%]). There were 3 (23.1%) patients in the survivors group and 11 (52.4%) in the nonsurvivors group who required ongoing CPR at the beginning of AAAD repair (P = .039). Of these patients, 1 survivor and 6 nonsurvivors could not achieve return of spontaneous circulation after pericardiotomy (P = .045). Although the duration from onset or arrival to the operating room was similar (P = .35 and P = .49, respectively), overall duration of CPR was shorter in survivors (10 minutes [range, 7.5-16 minutes] vs 16.5 minutes [range, 15-20 minutes]; P = .044). All survivors without any neurological deficits showed return of spontaneous circulation after pericardiotomy. Multivariate regression modeling showed that CPR duration >15 minutes was a significant risk factor for in-hospital mortality (P = .0040).ConclusionsCPR duration beyond 15 minutes may be a contraindication for AAAD repair. Moreover, we should reconsider surgery for patients who cannot achieve return of spontaneous circulation after pericardiotomy.     

Are racial differences in hospital mortality after coronary artery bypass graft surgery real? A risk-adjusted meta-analysis

BackgroundDespite several reports, there are still conflicting data on the influence of ethnicity on mortality rates associated with coronary artery bypass grafting (CABG). We aimed to get further insights into the effect of race on mortality following CABG by performing a risk adjusted meta-analysis.MethodsRelevant studies were searched on PubMed, Embase, BioMed Central, and the Cochrane Central register. Pairwise meta-analysis was used to estimate the relative risk of hospital death of black, Hispanic, and Asian patients using white patients as reference. Risk adjusted meta-analytic estimates were obtained using generic inverse variance methods with random effect model.ResultsA total of 28 studies were selected for analysis. A total of 21 studies reported on hospital mortality in black (n = 222,892) versus white (n = 3,884,043) patients, 7 studies reported on Hispanic (n = 91,256) versus white (n = 1,458,524) and 9 studies reported on Asian (n = 27,820) versus white (n = 1,081,642). When compared with white patients, adjusted risk of hospital death was significantly greater for black patients (adjusted odds ratio [OR], 1.25; 95% confidence interval [CI], 1.13-1.39; P < .001), and not statistically different for Asian (OR, 1.33; 95% CI, 0.99-1.77; P = .05) and Hispanic patients (adjusted OR, 1.08; 95% CI, 0.94-1.23; P = .26). Meta-regression showed a significant trend toward lower mortality rates in most recent series in both black (P = .02) and white (P = .0007) and Asian (P = .01) but not for Hispanic (P = .41). However, as mortality rates were lower across the different races, the relative disadvantage between the study groups persisted, which may explain the lack of interaction between study period and race effect on mortality for black (adjusted P = .09), Asian (adjusted P = .63), and Hispanic (adjusted P = .97) patients.ConclusionsThe present meta-analysis showed that despite progress is being made in lowering in-hospital mortality rates among the major racial/ethnic groups, ethnical disparities in hospital mortality after CABG remain.     

Chest Wall Reconstruction in Pediatric Patients with Chest Wall Tumors: A Systematic Review

BackgroundResection of pediatric chest wall tumors can result in large defects requiring reconstruction for function and cosmesis. Multiple reconstructive methods have been described. We performed a systematic review of the literature to describe commonly used approaches and outcomes.MethodsA systematic literature search was performed for English-language publications describing chest wall tumor resection and reconstruction using implantable materials in patients ≤21 years, excluding soft tissue resection only, sternal resection, and reconstruction by primary repair or muscle flaps alone. Data were collected on diagnoses, reconstructive method, and outcomes. Rigid chest wall reconstruction was compared to mesh reconstruction.ResultsThere were 55 articles with 188 patients included. The median age was 12 years. Most tumors were malignant (n = 172, 91.5%), most commonly Ewing's sarcoma (n = 65, 34.6%), followed by unspecified sarcomas (n = 34, 18.1%), Askin's tumor (n = 16, 8.5%; a subset of Ewing's sarcoma) and osteosarcoma (n = 16, 8.5%). A median of 3 ribs were resected (range 1–12). Non-rigid meshes were most common (n = 138, 73.4%), followed by rigid prostheses (n = 50, 26.6%). There were 19 post-operative complications (16.8%) and 22.2% of patients developed scoliosis. There were no significant differences in complications (20.5% rigid vs. 10.6% non-rigid, p = 0.18) or scoliosis (22.7% vs. 14.0%, p = 0.23) by reconstruction method, but complications after rigid reconstruction were more likely to require surgery (90.0% vs. 53.9%, p = 0.09). The median follow-up duration was 24 months.ConclusionsIn this review of the literature, there were no significant differences in overall post-operative complications or scoliosis development by reconstruction method, yet complications after rigid reconstruction were more likely to require surgical intervention.Level of evidenceLevel IV.