Monica E. Lopez

Clinical Characteristics and Risk Factors for Symptomatic Pediatric Gallbladder Disease

Objective: Our center previously reported its experience with pediatric gallbladder disease and cholecystectomies from 1980 to 1996. We aimed to determine the current clinical characteristics and risk factors for symptomatic pediatric gallbladder disease and cholecystectomies and compare these findings with our historical series. Study Design: Retrospective, cross-sectional study of children, 0 to 18 years of age, who underwent a cholecystectomy from January 2005 to October 2008. Results: We evaluated 404 patients: 73% girls; 39% Hispanic and 35% white. The mean age was 13.10 ± 0.91 years. The primary indications for surgery in patients 3 years or older were symptomatic cholelithiasis (53%), obstructive disease (28%), and biliary dyskinesia (16%). The median BMI percentile was 89%; 39% were classified as obese. Of the patients with nonhemolytic gallstone disease, 35% were obese and 18% were severely obese; BMI percentile was 99% or higher. Gallstone disease was associated with hemolytic disease in 23% (73/324) of patients and with obesity in 39% (126/324). Logistic regression demonstrated older age (P = .019) and Hispanic ethnicity (P < .0001) as independent risk factors for nonhemolytic gallstone disease. Compared with our historical series, children undergoing cholecystectomy are more likely to be Hispanic (P = .003) and severely obese (P < .0279). Conclusion: Obesity and Hispanic ethnicity are strongly correlated with symptomatic pediatric gallbladder disease. In comparison with our historical series, hemolytic disease is no longer the predominant risk factor for symptomatic gallstone disease in children.

Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee

OBJECTIVE Variable management practices complicate the identification of optimal strategies for infants with congenital diaphragmatic hernia (CDH). This review critically appraises the available evidence to provide recommendations. METHODS Six questions regarding CDH management were generated. English language articles published between 1980 and 2014 were compiled after searching Medline, Cochrane, Embase and Web of Science. Given the paucity of literature on the subject, all studies irrespective of their rank in the levels of evidence hierarchy were included. RESULTS Gentle ventilation with permissive hypercapnia provides the best outcomes. Initial high frequency ventilation may be considered but its overall efficacy is unproven. Routine inhaled nitric oxide (iNO) or other medical adjuncts for acute, severe pulmonary hypertension demonstrate no benefit. Evidence does not support routine administration of pre- or postnatal glucocorticoids. Mode of extracorporeal membrane oxygenation (ECMO) has little bearing on outcomes. While the overall timing of repair does not impact outcomes, early repair on ECMO has benefits. Open repair leads to significantly fewer recurrences. Polytetrafluoroethylene (PTFE) is the most durable patch repair material. CONCLUSIONS Limited high-level evidence prevents the development of robust management guidelines for CDH. Prospective, multi-institutional studies are needed to identify best practices and optimize outcomes.

Asymptomatic malrotation: Diagnosis and surgical management An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review

OBJECTIVE Patients with malrotation, or an intestinal rotation abnormality (IRA), can experience serious adverse events. Increasingly, asymptomatic patients are being diagnosed with malrotation incidentally. Patients with symptomatic malrotation require surgery in an urgent or semiurgent manner to address their symptoms. The treatment of asymptomatic or incidentally discovered malrotation remains controversial. METHODS Data were compiled from a broad search of Medline, Cochrane, Embase and Web of Science from January 1980 through January 2013 for five questions regarding asymptomatic malrotation. RESULTS There is minimal evidence to support screening asymptomatic patients. Consideration may be given to operate on asymptomatic patients who are younger in age, while observation may be appropriate in the older patient. If reliably diagnosed, atypical malrotation with a broad-based mesentery and malposition of the duodenum can be observed. Regarding diagnostic imaging, the standard of care for diagnosis remains the upper gastrointestinal contrast study (UGI), ultrasound may be useful for screening. A laparoscopic approach is safe for diagnosis and treatment of rotational abnormalities. Laparoscopy can aid in determining whether a patient has true malrotation with a narrow mesenteric stalk, has nonrotation and minimal risk for volvulus, or has atypical anatomy with malposition of the duodenum. It is reasonable to delay Ladd procedures until after palliation on patients with severe congenital heart disease. Observation can be considered with extensive education for family and caregivers and close clinical follow-up. CONCLUSIONS There is a lack of quality data to guide the management of patients with asymptomatic malrotation. Multicenter and prospective data should be collected to better assess the risk profile for this complex group of patients. A multidisciplinary approach involving surgery, cardiology, critical care and the patients caregivers can help guide a watchful waiting management plan in individual cases.

Consensus in Guidelines for Evaluation of DSD by the Texas Children's Hospital Multidisciplinary Gender Medicine Team

The Gender Medicine Team (GMT), comprised of members with expertise in endocrinology, ethics, genetics, gynecology, pediatric surgery, psychology, and urology, at Texas Childrens Hospital and Baylor College of Medicine formed a task force to formulate a consensus statement on practice guidelines for managing disorders of sexual differentiation (DSD) and for making sex assignments. The GMT task force reviewed published evidence and incorporated findings from clinical experience. Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) was used to assess the quality of evidence presented in the literature for establishing evidence-based guidelines. The task force presents a consensus statement regarding specific diagnostic and therapeutic issues in the management of individuals who present with DSD. The consensus statement includes recommendations for (1) laboratory workup, (2) acute management, (3) sex assignment in an ethical framework that includes education and involvement of the parents, and (4) surgical management.

Serial Fecal Microbiota Transplantation Alters Mucosal Gene Expression in Pediatric Ulcerative Colitis

Serial Fecal Microbiota Transplantation Alters Mucosal Gene Expression in Pediatric Ulcerative Colitis

Development and validation of an ultrasound scoring system for children with suspected acute appendicitis

BackgroundTo facilitate consistent, reliable communication among providers, we developed a scoring system (Appy-Score) for reporting limited right lower quadrant ultrasound (US) exams performed for suspected pediatric appendicitis.ObjectiveThe purpose of this study was to evaluate implementation of this scoring system and its ability to risk-stratify children with suspected appendicitis.Materials and methodsIn this HIPAA compliant, Institutional Review Board-approved study, the Appy-Score was applied retrospectively to all limited abdominal US exams ordered for suspected pediatric appendicitis through our emergency department during a 5-month pre-implementation period (Jan 1, 2013, to May 31, 2013), and Appy-Score use was tracked prospectively post-implementation (July 1, 2013, to Sept. 30,2013). Appy-Score strata were: 1 = normal completely visualized appendix; 2 = normal partially visualized appendix; 3 = non-visualized appendix, 4 = equivocal, 5a = non-perforated appendicitis and 5b = perforated appendicitis. Appy-Score use, frequency of appendicitis by Appy-Score stratum, and diagnostic performance measures of US exams were computed using operative and clinical finding as reference standards. Secondary outcome measures included rates of CT imaging following US exams and negative appendectomy rates.ResultsWe identified 1,235 patients in the pre-implementation and 686 patients in the post-implementation groups. Appy-Score use increased from 24% (37/155) in July to 89% (226/254) in September (P < 0.001). Appendicitis frequency by Appy-Score stratum post-implementation was: 1 = 0.5%, 2 = 0%, 3 = 9.5%, 4 = 44%, 5a = 92.3%, and 5b = 100%. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were 96.3% (287/298), 93.9% (880/937), 83.4% (287/344), and 98.8% (880/891) pre-implementation and 93.0% (200/215), 92.6% (436/471), 85.1% (200/235), and 96.7% (436/451) post-implementation – only NPV was statistically different (P = 0.012). CT imaging after US decreased by 31% between pre- and post-implementation, 8.6% (106/1235) vs. 6.0% (41/686); P = 0.048). Negative appendectomy rates did not change (4.4% vs. 4.1%, P = 0.8).ConclusionA scoring system and structured template for reporting US exam results for suspected pediatric appendicitis was successfully adopted by a pediatric radiology department at a large tertiary children’s hospital and stratifies risk for children based on their likelihood of appendicitis.

Correlating surgical and pathological diagnoses in pediatric appendicitis

BACKGROUND The stratification of appendicitis into simple and complex variants has far-reaching implications. While the operative diagnosis made by the surgeon dictates clinical management, the pathologic diagnosis often differs and is frequently used for coding and reimbursement. The purpose of this study was to examine discrepancies between the operative and pathologic diagnoses with subsequent correlation to clinical outcomes. METHODS Patients with acute appendicitis from July 2011 to July 2012 were identified. Diagnoses included simple (normal, acute, and suppurative) and complex (gangrenous and perforated). We evaluated the inter-rater reliability between pathologic and operative diagnoses in the five appendicitis categories. Clinical outcomes of deep and superficial surgical site infections were evaluated according to the pathologic and surgical diagnosis. RESULTS During the study period, we identified 1166 patients with acute appendicitis. The surgeon and pathologist agreed on the specific diagnosis (acute, suppurative, gangrenous, perforated, normal) in 48% of patients (kappa 0.289, 95% CI 0.259-0.324, p=0.001). Agreement on disease severity (simple vs. complex) improved to 82%. The operative diagnosis more accurately predicted infectious complications than the pathologic diagnoses. CONCLUSION Significant discordance exists between surgical and pathologic diagnoses. While the relevance of this discordance to clinical outcomes is still not clear, a potential for incorrect hospital coding and subsequent reimbursement exists. Future quality improvement projects should focus on standardizing the surgical and pathologic diagnoses.

The surgical management of Rapunzel syndrome: a case series and literature review.

BACKGROUND/PURPOSE The surgical removal of a trichobezoar is the rare end complication of the psychiatric disorders trichotillomania and trichophagia. The more severe form of the disease is termed Rapunzel syndrome, where the bezoar extends from the gastric body beyond the pylorus into the duodenum. Traditional therapy has included endoscopy, often with subsequent laparotomy, and associated psychiatric intervention. We present the largest and most recent series of patients with trichobezoars managed in a single institution. METHODS A retrospective review of all cases of trichobezoar at our institution from 2003 to 2011 was performed. Demographic data, presenting complaints, imaging, surgical treatment, and subsequent management were collected. RESULTS All 7 patients were female, ages 5 to 23 years (mean, 11.5 years). Although multiple imaging modalities were necessary for preoperative diagnosis, most patients were accurately diagnosed without endoscopic evaluation (85%). All patients required an exploratory laparotomy for definitive treatment. At laparotomy, 5 patients were found to have postpyloric extension of the trichobezoar (71%). One of 7 patients had a wound infection postoperatively. There were no other surgical complications or recurrences requiring further exploration. CONCLUSIONS Our series of trichobezoar patients appear to have a high rate of Rapunzel syndrome, and perhaps postpyloric extension should be considered the rule rather than the exception. Our series demonstrates that diagnosis can be established with a thorough history combined with radiography, and treatment should be a combination of laparoscopy and/or laparotomy with psychiatric consultation.

Risk factors for surgery in pediatric intussusception in the era of pneumatic reduction

INTRODUCTION Surgical treatment is still necessary for intussusception management in a subgroup of patients, despite advances in enema reduction techniques. Early identification of these patients should improve outcomes. METHODS The medical records of patients treated for intussusception at our institution from 2006 to 2011 were reviewed. Univariate and multivariate analyses, including stepwise logistic regression, were performed. RESULTS Overall, 379 patients were treated for intussusception, and 101 (26%) patients required operative management, with 34 undergoing intestinal resection. The post-operative complication rate was 8%. On multivariate analysis, failure of initial reduction (OR 9.9,p=0.001 95% CI, 4.6-21.2), a lead point (OR 18.5,p=0.001 95% CI, 6.6-51.8) or free/interloop fluid (OR 3.3,p=0.001 95% CI, 1.6-6.7) or bowel wall thickening on ultrasound (OR 3.3,p=0.001 95% CI, 1.1-10.1), age <1 year at reduction (OR 2.7,p=0.004, 95% CI, 1.4-5.9), and abdominal symptoms>2 days (OR 2.9,p=0.003, 95% CI, 1.4-5.9) were significantly associated with a requirement for surgery. Similarly, a lead point (OR 14.5, p=0.005 95% CI, 2.3-90.9) or free/interloop fluid on ultrasound (OR 19.8, p=0.001 95% CI, 3.4-117) and fever (OR 7.2, p=0.023 95% CI, 1.1-46) were significantly associated with the need for intestinal resection. CONCLUSION Abdominal symptoms>2 days, age<1 year, multiple ultrasound findings, and failure of initial enema reduction are significant predictors of operative treatment for intussusception. Patients with these findings should be considered for early surgical consultation or transfer to a hospital with pediatric surgical capabilities.

Surgical wound misclassification: A multicenter evaluation

BACKGROUND Surgical wound classification (SWC) is used by hospitals, quality collaboratives, and Centers for Medicare and Medicaid to stratify patients for their risk for surgical site infection. Although these data can be used to compare centers, the validity and reliability of SWC as currently practiced has not been well studied. Our objective was to assess the reliability of SWC in a multicenter fashion. We hypothesized that the concordance rates between SWC in the electronic medical record and SWC determined from the operative note review is low and varies by institution and operation. STUDY DESIGN Surgical wound classification concordance was assessed at 11 participating institutions between SWC from the electronic medical record and SWC from operative note review for 8 common pediatric surgical operations. Cases with concurrent procedures were excluded. A maximum of 25 consecutive cases were selected per operation from each institution. A designated surgeon reviewed the included operative notes from his/her own institution to determine SWC based on a predetermined algorithm. RESULTS In all, 2,034 cases were reviewed. Overall SWC concordance was 56%, ranging from 47% to 66% across institutions. Inguinal hernia repair had the highest overall median concordance (92%) and appendectomy had the lowest (12%). Electronic medical records and reviewer SWC differed by up to 3 classes for certain cases. CONCLUSIONS Surgical site infection risk stratification by SWC, as currently practiced, is an unreliable methodology to compare patients and institutions. Surgical wound classification should not be used for quality benchmarking. If SWC continues to be used, individual institutions should evaluate their process of assigning SWC to ensure its accuracy and reliability.