Grace Z. Mak

Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula

BACKGROUND/PURPOSE Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care. METHODS A multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 childrens hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables. RESULTS Prenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%-100%); proximal pouch contrast study in 21% (0%-69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%-69%); perioperative antibiotics ≥24h in 69% (36%-97%); and transanastomotic tubes in 73% (21%-100%). CONCLUSION Contemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF. LEVEL OF EVIDENCE Type of study: Treatment study. Level IV.

Median arcuate ligament syndrome in the pediatric population

OBJECTIVES Median arcuate ligament syndrome (MALS) is a vascular compression syndrome with symptoms that overlap chronic functional abdominal pain (CFAP). We report our experience treating MALS in a pediatric cohort previously diagnosed with CFAP. PATIENTS AND METHODS We prospectively evaluated 46 pediatric (<21years of age) patients diagnosed with MALS at a tertiary care referral center from 2008 to 2012. All patients had previously been diagnosed with CFAP. Patients were evaluated for celiac artery compression by duplex ultrasound and diagnosis was confirmed by computed tomography. Quality of life (QOL) was determined by pre- and postsurgical administration of PedsQL™ questionnaire. The patients underwent laparoscopic release of the median arcuate ligament overlying the celiac artery which included surgical neurolysis. We examined the hemodynamic changes in parameters of the celiac artery and perioperative QOL outcomes to determine correlation. RESULTS All patients had studies suggestive of MALS on duplex and computed tomography; 91% (n=42) positive for MALS were females. All patients underwent a technically satisfactory laparoscopic surgical release resulting in a significant improvement in blood flow through the celiac artery. There were no deaths and a total of 9 complications, 8 requiring a secondary procedure; 33 patients were administered QOL surveys. 18 patients completed the survey with 15 (83%) patients reporting overall improvement in the QOL. Overall, 31/46 patients (67%) reported improvement of symptoms since the time of surgery. CONCLUSIONS MALS was found to be more common in pediatric females than males. Laparoscopic release of the celiac artery can be performed safely in the pediatric population. Surgical release of the artery and resultant neurolysis resulted in significant improvement in the blood flow, symptoms, and overall QOL in this cohort. The overall improvement in QOL outcome measures after surgery leads us to conclude that MALS might be earlier diagnosed and possibly treated in patients with CFAP. We recommend a multidisciplinary team approach to care for these complex patients.

Challenging surgical dogma in the management of proximal esophageal atresia with distal tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium

PURPOSE Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF. METHODS The Midwest Pediatric Surgery Consortium conducted a multicenter, retrospective study examining selected outcomes on infants diagnosed with proximal EA with distal TEF who underwent primary repair over a 5-year period (2009-2014), with a minimum 1-year follow up, across 11 centers. RESULTS 292 patients with proximal EA and distal TEF who underwent primary repair were reviewed. The overall mortality was 6% and was significantly associated with the presence of congenital heart disease (OR 4.82, p=0.005). Postoperative complications occurred in 181 (62%) infants, including: anastomotic stricture requiring intervention (n=127; 43%); anastomotic leak (n=54; 18%); recurrent fistula (n=15; 5%); vocal cord paralysis/paresis (n=14; 5%); and esophageal dehiscence (n=5; 2%). Placement of a transanastomotic tube was associated with an increase in esophageal stricture formation (OR 2.2, p=0.01). Acid suppression was not associated with altered rates of stricture, leak or pneumonia (all p>0.1). Placement of interposing prosthetic material between the esophageal and tracheal suture lines was associated with an increased leak rate (OR 4.7, p<0.001), but no difference in the incidence of recurrent fistula (p=0.3). Empiric postoperative antibiotics for >24h were used in 193 patients (66%) with no difference in rates of infection, shock or death when compared to antibiotic use ≤24h (all p>0.3). Hospital volume was not associated with postoperative complication rates (p>0.08). Routine postoperative esophagram obtained on day 5 resulted in no delayed/missed anastomotic leaks or a difference in anastomotic leak rate as compared to esophagrams obtained on day 7. CONCLUSION Morbidity after primary repair of proximal EA and distal TEF patients is substantial, and many common practices do not appear to reduce complications. Specifically, this large retrospective series does not support the use of prophylactic antibiotics beyond 24h and empiric acid suppression may not prevent complications. Use of a transanastomotic tube was associated with higher rates of stricture, and interposition of prosthetic material was associated with higher leak rates. Routine postoperative esophagram can be safely obtained on day 5 resulting in earlier initiation of oral feeds. STUDY TYPE Treatment study. LEVEL OF EVIDENCE III.

Pediatric Chronic Abdominal Pain and Median Arcuate Ligament Syndrome: A Review and Psychosocial Comparison.

Chronic abdominal pain (CAP) occurs in children and adolescents with a reported prevalence of 4% to 41% with significant direct and indirect costs to the child, family, and society. Median arcuate ligament syndrome (MALS) is a vascular compression syndrome of the celiac artery that may cause symptoms of epigastric pain and weight loss and is a frequently overlooked cause of CAP in the pediatric population. We have observed that the psychosocial presentation of patients with MALS is notable for various psychiatric comorbidities. In this article, we review MALS as well as our study results of the psychosocial profile of 30 MALS patients. Our data suggest that children and adolescents with MALS have similar psychosocial profiles to children with other gastrointestinal disorders resulting in CAP. The overlap of physical and psychosocial symptoms of patients who have MALS with other CAP disorders leads us to recommend that patients with CAP should be evaluated for MALS. [Pediatr Ann. 2016;45(7):e257-e264.].

Paradigm Shifts in the Treatment of Appendicitis

Acute appendicitis is the most common cause of emergent surgery in children. Historically, surgical dogma dictated emergent appendectomy due to concern for impending perforation. Recently, however, there has been a paradigm shift in both the understanding of its pathophysiology as well as its treatment to more nonoperative management. No longer is it considered a spectrum from uncomplicated appendicitis inevitably progressing to complicated appendicitis over time. Rather, uncomplicated and complicated appendicitis are now considered two distinct pathophysiologic entities. This change requires not only educating the patients and their families but also the general practitioners who will be managing treatment expectations and caring for patients long term. In this article, we review the pathophysiology of appendicitis, including the differentiation between uncomplicated and complicated appendicitis, as well as the new treatment paradigms. [Pediatr Ann. 2016;45(7):e235-e240.].

In Patients with Celiac Artery Compression Syndrome, Does Surgery Improve Quality of Life?

Symptomatic celiac artery compression is a controversial diagnosis that should be considered in patients with chronic abdominal pain of unknown etiology despite an extensive medical evaluation. Once suspected, patients should undergo screening mesenteric duplex. Diagnosis is confirmed with the findings of elevated celiac artery velocities which normalize with respiration followed by CT angiogram showing the typical “J-hook” conformation of the celiac artery. Patients should then undergo evaluation by a multi-disciplinary team to appropriately select patients for surgical treatment. Surgical options include release of the median arcuate ligament, with or without neurolysis of the celiac nerve plexus, and with or without concomitant revascularization procedures. Approaches can be open, laparoscopic, robotic, or retroperitoneal. Surgical treatment has an overall success rate with 70–80 % patients reporting improved abdominal pain and quality of life. Post-operatively, patients can have persistent or recurrent abdominal pain and should undergo re-evaluation for possible need for revascularization procedure for stenosis of the celiac artery or celiac plexus block if the celiac artery is normalized. Additionally, some of these patients will have persistent pain consistent with a functional gastrointestinal disorder that will then require medical management.

Development of a multi-institutional clinical research consortium for pediatric surgery

BACKGROUND Multicenter clinical research studies in pediatric surgery have been largely limited to relatively small case-series and retrospective reviews because of the rarity of many of the diseases we treat and difficulty coordinating and executing multi-institutional studies. Creation of a collaborative research network can provide the needed patient population and infrastructure to perform high quality multi-institutional studies. METHODS In 2013, eleven academic pediatric surgery centers within the United States formed a research consortium to develop and conduct multicenter clinical research projects to advance the practice of pediatric surgery. RESULTS We present our process for creating, developing, and maintaining this consortium including initial regional geographic limitation, charter development with by-laws and procedures for adopting studies, and research infrastructure including a central website for study monitoring and central reliance institutional review board process. CONCLUSION Our model could be reproduced or adapted by other institutions to develop or strengthen other research collaboratives. LEVEL OF EVIDENCE Type of study: retrospective, IV.

Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium

PURPOSE Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. METHODS A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 childrens hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. RESULTS In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54-2.65) vs. 2.57 kg (2.00-3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32-37) vs. 37 weeks (35-39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29). CONCLUSION RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach. LEVEL OF EVIDENCE Level III.

Evaluation of a Water-Soluble Contrast Protocol for Non-operative Management of Pediatric Adhesive Small Bowel Obstruction

BACKGROUND/PURPOSE We examined outcomes before and after implementing an enteral water-soluble contrast protocol for management of pediatric adhesive small bowel obstruction (ASBO). METHODS Medical records were reviewed retrospectively for all children admitted with ASBO between November 2010 and June 2017. Those admitted between November 2010 and October 2013 received nasogastric decompression with decision for surgery determined by surgeon judgment (preprotocol). Patients admitted after October 2013 (postprotocol) received water-soluble contrast early after admission, were monitored with serial examinations and radiographs, and underwent surgery if contrast was not visualized in the cecum by 24 h. Group outcomes were compared. RESULTS Twenty-six patients experienced 29 admissions preprotocol, and 11 patients experienced 12 admissions postprotocol. Thirteen (45%) patients admitted preprotocol underwent surgery, versus 2 (17%) postprotocol patients (p = 0.04). Contrast study diagnostic sensitivity as a predictor for ASBO resolution was 100%, with 90% specificity. Median overall hospital LOS trended shorter in the postprotocol group, though was not statistically significant (6.2 days (preprotocol) vs 3.6 days (postprotocol) p = 0.12). Pre- vs. postprotocol net operating cost per admission yielded a savings of

Screening practices and associated anomalies in infants with anorectal malformations: Results from the Midwest Pediatric Surgery Consortium

8885.42. CONCLUSIONS Administration of water-soluble contrast after hospitalization for pediatric ASBO may play a dual diagnostic and therapeutic role in management with decreases in surgical intervention, LOS, and cost. TYPE OF STUDY Retrospective comparative study. LEVEL OF EVIDENCE Level III.