Intercostal spinal nerve cryoablation for analgesia following pectus excavatum repair.

Pectus excavatum is a common chest wall deformity, most often treated during adolescence, that presents a significant postoperative pain control challenge for pediatric surgeons following surgical correction. The purpose of this article is to review the technique and outcomes of intercostal spinal nerve cryoablation for postoperative analgesia following surgical correction of pectus excavatum. Contemporary and historic literature were reviewed. Findings are summarized to provide a concise synopsis of the benefits of intercostal spinal nerve cryoablation relative to alternative analgesic modalities, as well as advocate for more widespread inclusion of this technique into multimodal pain regimens.

Applications for ultrasound in pediatric surgery.

Ultrasound (US) use within pediatric surgery is expanding rapidly. While US guidance for central line placement has been common practice for many years now, advances in the quality of images, portability of US machines, and a lack of radiation associated with imaging has led to broader application in many other aspects of surgery, ranging from diagnostics to performing operations under the direction of point-of-care ultrasound (POCUS). The relatively short learning curve for providers along with excellent image quality in children due to their small size provides an easy, effective imaging modality with diverse applications. Discussed here is a broad overview of the spectrum of US use within current pediatric surgical practices.

Ultrasound-Guided Epigastric Hernia Repair in the Pediatric Population: One Institution's Experience and Outcomes.

Introduction: Epigastric hernia repair is a common procedure performed on pediatric patients. These hernias have been demonstrated to be readily identified by ultrasound. This manuscript describes and assesses a novel technique in which ultrasound is used to identify and repair the hernias in a minimally invasive manner. Methods: A retrospective chart review evaluates all the ultrasound-guided epigastric hernia repairs done with prolene suture at one institution over 20 months. Operative details and postoperative complications were compared to patients who underwent traditional open epigastric hernia repair at the same institution during the same time frame. Results: Thirty-two pediatric patients underwent epigastric hernia repair between May 2021 and December 2022. Thirteen (41%) underwent the ultrasound-guided technique with prolene suture and using only a meniscus needle and no incision. Nineteen (59%) underwent standard open repair. There were no recurrences or postoperative complications in either group. The average operative time for the ultrasound hernia repair was 24.4 minutes, which was shorter than the average operative time of 33.6 minutes for the open repair (P = .08). In 3 ultrasound cases (23%), an additional epigastric hernia that had not been clinically apparent was identified and simultaneously repaired. Conclusions: Ultrasound-guided epigastria hernia repair is a feasible alternative to traditional open repair. It has a comparable safety and efficacy profile, has a short operative time, and has the advantages of being minimally invasive and having the ability to identify and repair concurrent epigastric hernias.

Ultrasound-Guided Enteropexy for Repair of Ostomy Prolapse.

Background: Enterostomies provide fecal diversion for numerous conditions, but anatomical complications-prolapse, stricture, and retraction-occur in up to 25%. Given up to 76% of these complications require surgical intervention, effective minimally invasive repair techniques for their management are needed. This article describes a new technique for prolapse repair utilizing image-guided surgery for incisionless repair of ostomy prolapse. Methods: To perform the procedure, the prolapsed bowel is reduced and evaluated for feasibility for ultrasound repair. Under direct ultrasound guidance sutures are used to pexy the bowel loop to the overlying fascia. Sutures are tied with knots and sutures buried below the skin to securely tack the bowel to the abdominal wall. Results: Four patients aged 2-10 years underwent ultrasound-guided enteropexy for repair of major prolapse of an end ileostomy (2 patients), loop colostomy, and end colostomy. All patients remained free of major prolapse for 3-10 months after the procedure, 2 of whom have progressed to ostomy takedown without complications. Conclusions: Ultrasound-guided enteropexy is a noninvasive effective way to manage ostomy prolapse.

Ultrasound-Guided Gastrostomy Tube Placement: An Evaluation of Postoperative Complications in the Pediatric Population.

Background: Using ultrasound guidance has been demonstrated as a feasible alternative method for gastrostomy tube placement in the pediatric population. The aim of this study is to evaluate short- and long-term postoperative complications after ultrasound-guided gastrostomy tube placement (USGTP) and to compare them with complications after laparoscopic gastrostomy tube placement (LGTP). Methods: A retrospective chart review evaluated patients who underwent USGTP (n = 41) and LGTP (n = 120) at the same institution. Comparisons were made between the two groups in the context of demographics as well as 30-day and 6-month postoperative complications. A phone survey (n = 26) further identified USGTP complications potentially not captured in the electronic medical records. Results: There were no significant differences in age, gender, and indication for procedure between the two groups. Chart review revealed that USGTP and LGTP had statistically comparable rates of emergency department (ED) visits for postoperative complications. Among USGTP patients, 8% had a recorded ED visit within 30 days of the operation and 13% presented to the ED within 6 months, compared with 6% and 11%, respectively, in the LGTP group (P = .65, P = .69). The USGTP phone survey reported total complications over an average postoperative follow-up time of 34.6 months (range 8-87) and revealed a total ED visit rate of 35%, which is comparable with rates reported in the literature for minimally invasive feeding tube placement. Conclusion: USGTP is a safe and feasible alternative option for gastrostomy tube placement in the pediatric population and it has postoperative complication rates that are comparable with LGTP.

Evaluating the risk of peri-umbilical hernia after sutured or sutureless gastroschisis closure.

INTRODUCTION: We evaluate the incidence, outcomes, and management of peri­umbilical hernias after sutured or sutureless gastroschisis closure. METHODS: A retrospective, longitudinal follow-up of neonates with gastroschisis who underwent closure at 11 children's hospitals from 2013 to 2016 was performed. Patient encounters were reviewed through 2019 to identify the presence of a peri­umbilical hernia, time to spontaneous closure or repair, and associated complications. RESULTS: Of 397 patients, 375 had follow-up data. Sutured closure was performed in 305 (81.3%). A total of 310 (82.7%) infants had uncomplicated gastroschisis. Peri-umbilical hernia incidence after gastroschisis closure was 22.7% overall within a median follow-up of 2.5 years [IQR 1.3,3.9], and higher in those with uncomplicated gastroschisis who underwent primary vs. silo assisted closure (53.0% vs. 17.2%, p< 0.001). At follow-up, 50.0% of sutureless closures had a persistent hernia, while 16.4% of sutured closures had a postoperative hernia of the fascial defect (50.0% vs. 16.4%, p< 0.001). Spontaneous closure was observed in 38.8% of patients within a median of 17 months [9,26] and most frequently observed in those who underwent a sutureless primary closure (52.2%). Twenty-seven patients (31.8%) underwent operative repair within a median of 13 months [7,23.5]. Rate and interval of spontaneous closure or repair were similar between the sutured and sutureless closure groups, with no difference between those who underwent primary vs. silo assisted closure. CONCLUSION: Peri-umbilical hernias after sutured or sutureless gastroschisis closure may be safely observed similar to congenital umbilical hernias as spontaneous closure occurs, with minimal complications and no additional risk with either closure approach. LEVELS OF EVIDENCE: Level II.

Switching to centrifugal pumps may decrease hemolysis rates among pediatric ECMO patients.

Recent advances in ECLS technology have led to the adoption of centrifugal pumps for the majority of patients worldwide. Despite several advantages of centrifugal pumps, they remain controversial because a number of studies have shown increased rates of hemolysis. The aim of this study was to assess the impact of transitioning from roller to centrifugal pumps on hemolysis rates at our center. A retrospective analysis of all pediatric ECMO patients at a single center between 2005 and 2017 was undertaken. Hemolysis was defined as a plasma free hemoglobin >50 mg/dL. Multivariable logistic regression was performed correcting for several factors to determine risk factors for hemolysis and analyze outcomes among patients with hemolysis. Significant findings were those with p < 0.05. A total of 590 patients were identified during the study period. Multivariable logistic regression for risk factors for hemolysis showed roller pumps (OR 1.92, CI 1.11-3.33) and ECMO duration (OR 1.002 per hour, CI 1.00-1.01) to be significant factors. Rates of hemolysis significantly improved following conversion from roller to centrifugal pumps, with significantly lower rates of hemolysis in 2012, 2015, 2016, and 2017 when compared to the historical average with roller pumps from 2005 to 2009 (34.7%). Additionally, hemolysis was associated with an increased risk of death (OR 3.59, CI 2.05-6.29) when correcting for other factors. These data suggest decreasing rates of hemolysis with centrifugal pumps compared to roller pumps. Since hemolysis was also associated with increased risk of death, these data support the switch from roller to centrifugal pumps at ECMO centers.

Does Use of a Feeding Protocol Change Outcomes in Gastroschisis? A Report from the Midwest Pediatric Surgery Consortium.

INTRODUCTION: Gastroschisis feeding practices vary. Standardized neonatal feeding protocols have been demonstrated to improve nutritional outcomes. We report outcomes of infants with gastroschisis that were fed with and without a protocol. MATERIALS AND METHODS: A retrospective study of neonates with uncomplicated gastroschisis at 11 children's hospitals from 2013 to 2016 was performed.Outcomes of infants fed via institutional-specific protocols were compared with those fed without a protocol. Subgroup analyses of protocol use with immediate versus delayed closure and with sutured versus sutureless closure were conducted. RESULTS: Among 315 neonates, protocol-based feeding was utilized in 204 (65%) while no feeding protocol was used in 111 (35%). There were less surgical site infections (SSI) in those fed with a protocol (7 vs. 16%, p = 0.019). There were no differences in TPN duration, time to initial oral intake, time to goal feeds, ventilator use, peripherally inserted central catheter line deep venous thromboses, or length of stay. Of those fed via protocol, less SSIs occurred in those who underwent sutured closure (9 vs. 19%, p = 0.026). Further analyses based on closure timing or closure method did not demonstrate any significant differences. CONCLUSION: Across this multi-institutional cohort of infants with uncomplicated gastroschisis, there were more SSIs in those fed without an institutional-based feeding protocol but no differences in other outcomes.

Outcomes in gastroschisis: expectations in the postnatal period for simple vs complex gastroschisis.

OBJECTIVE: To provide generalizable estimates for expected outcomes of simple gastroschisis (SG) and complex gastroschisis (CG) patients from a large multi-institutional cohort for use during counseling. STUDY DESIGN: A retrospective study of 394 neonates with gastroschisis at 11 children's hospitals from January 2013 to March 2017 was performed. Analysis by Fisher's exact tests and Wilcoxon rank sum tests were performed. Outcomes of complex and simple gastroschisis are reported. RESULT: There were 315 (80%) SG and 79 (20%) CG. CG had increased time from birth to closure (6 vs 4.4 days), closure to goal feeds (69 vs 23 days), ventilator use (90% vs 73%), SSIs (31% vs 11%), NEC (14% vs 6%), PN use (71 vs 24 days), LOS (104.5 vs 33 days), and mortality (11% vs 0%). CONCLUSION: This study provides generalizable estimates for expected outcomes of patients with both SG and CG that can be utilized during counseling. CG has significantly worse in-hospital outcomes.

Ultrasound-guided pediatric inguinal hernia repair.

PURPOSE: Inguinal hernias are amongst the most common surgical conditions in children. Typically, these repairs are performed through an open or laparoscopic approach, using a high ligation of the hernia sac. The use of ultrasound has been described in identifying and evaluating hernia contents in children. Our goal was to determine if ultrasound guidance could be used to perform a high ligation of the hernia sac in pediatric patients. METHODS: Following IRB approval, a retrospective review of all female patients at a single center undergoing ultrasound guided inguinal hernia repair between 2017 and 2018 was performed. Pre-operative characteristics, intra-operative outcomes, and post-operative outcomes were all evaluated. Laparoscopy was used to evaluate the repair and evaluate for a contralateral hernia. Male patients did not undergo ultrasound inguinal hernia repair to avoid damage to the vas deferens and vessels. RESULTS: A total of 10 patients with 13 hernias total were found during the study period. A total of one patient was converted to a laparoscopic repair. No patients were found to have an inappropriate repair or a missed contralateral hernia, and there were no vascular injuries or injuries to surrounding structures. No patients had a hernia recurrence during the study period. CONCLUSION: This study demonstrates the safety and feasibility of ultrasound guided inguinal hernia repairs in female pediatric patients. Further study is needed to compare these repairs to existing techniques, evaluate for recurrences over time, and evaluate if these repairs can be performed without general anesthetic in some patients.

Sutureless vs sutured abdominal wall closure for gastroschisis: Operative characteristics and early outcomes from the Midwest Pediatric Surgery Consortium.

PURPOSE: To report outcomes of sutured and sutureless closure for gastroschisis across a large multi-institutional cohort. METHODS: A retrospective study of infants with uncomplicated gastroschisis at 11 children's from 2014 to 2016 was performed. Outcomes of sutured and sutureless abdominal wall closure were compared. RESULTS: Among 315 neonates with uncomplicated gastroschisis, sutured closure was performed in 248 (79%); 212 undergoing sutured closure after silo and 36 undergoing primary sutured closure. Sutureless closure was performed in 67 (21%); 37 primary sutureless closure, 30 sutureless closure after silo placement. There was no significant difference in gestational age, gender, birth weight, total days on TPN, and time from closure to initial oral intake or goal feeds. Sutureless closure patients had less general anesthetics, ventilator use/time, time from birth to final closure, antibiotic use after closure, and surgical site/deep space infections. Subgroup analysis demonstrated primary sutureless closure had less ventilator use and anesthetics than primary sutured closure. Sutureless closure after silo led to less ventilator use/time, anesthetics, and antibiotics compared to those with sutured closure after silo. CONCLUSION: Sutureless abdominal wall closure of neonates with gastroschisis was associated with less general anesthetics, antibiotic use, surgical site/deep space infections, and decreased ventilator time. These findings support further prospective study by our group. LEVEL OF EVIDENCE: Level III.

Development of a multi-institutional registry for children with operative congenital lung malformations.

INTRODUCTION: The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. METHODS: After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. RESULTS: Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1-12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6-11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). CONCLUSION: This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients. LEVEL OF EVIDENCE: Level II.

Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium.

PURPOSE: The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium. METHODS: After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015). RESULTS: Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p < 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p < 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1-12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0-13.0). CONCLUSIONS: Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion. LEVEL OF EVIDENCE: Level III.

Short-term respiratory outcomes of neonates with symptomatic congenital lung malformations.

INTRODUCTION: The purpose of this study was to evaluate short-term respiratory outcomes in neonates with symptomatic congenital lung malformations (CLM). METHODS: Consecutive newborns who underwent surgical resection of a CLM were retrospectively reviewed. Demographic, prenatal, and outcomes data were analyzed as appropriate (p < 0.05). RESULTS: Twenty-one neonates were managed at a median gestational age of 36.2 weeks [interquartile range (IQR), 33.8-39.0]. Endotracheal intubation was required in 14 (66.7%) for a median of 7.5 days [interquartile range (IQR), 3.0-25.8]. Three (14.3%) children underwent ex utero intrapartum treatment-to-resection, and another 14 (66.7%) had neonatal lung resections performed at a median age of 2.0 days (IQR, 0.08-19.5 days). Excluding one patient who received comfort care at birth, all neonates survived to hospital discharge with a median length of hospitalization of 36.5 days (IQR, 23.8-56.5). More than one-quarter were discharged on supplemental oxygen by nasal cannula. Based on a median follow up of 35.5 months (IQR, 19.0-80.8), CLM-related morbidity was still evident in 55.0%. CONCLUSION: Our study suggests a high incidence of complications and chronic respiratory morbidity after neonatal lung resection for symptomatic CLMs. These data highlight the need to provide realistic expectations in perinatal counseling discussions with families and the importance of coordinating appropriate multidisciplinary follow up for these children. LEVEL OF EVIDENCE: Level IV.

Diagnostic accuracy of imaging studies in congenital lung malformations.

BACKGROUND: Although fetal ultrasound, fetal MRI and postnatal CT are now widely used in the evaluation of congenital lung malformations (CLM), their diagnostic accuracy remains undefined. OBJECTIVE: To correlate prenatal and postnatal imaging studies with pathological data after CLM resection. DESIGN: Retrospective, descriptive case series study. SETTING: A North American tertiary care centre. PATIENTS: One hundred and three consecutive lung resections for a suspected CLM between 1 January 2005 and 31 December 2015. MAIN OUTCOME MEASURES: Diagnostic accuracy of imaging diagnosis compared with pathological evaluation. RESULTS: Pathological diagnoses included congenital pulmonary airway malformation ((CPAM) n=45, 44%), bronchopulmonary sequestration (BPS; n=25, 24%), CPAM/BPS hybrid lesions (n=22, 21%) and pleuropulmonary blastoma (n=2, 2%). Fetal ultrasound detected 85 (82.5%) lesions and correctly diagnosed whether or not a lesion was a CPAM in 75% of cases (sensitivity 93%, specificity 32%). Fetal MRI had a similar concordance rate (73%) but was superior in correctly determining whether a systemic feeding vessel was present in 80% of cases (sensitivity 71%, specificity 88%) compared with an ultrasound accuracy rate of 72% (sensitivity 49%, specificity 93%). By comparison, postnatal CT correctly diagnosed whether a CPAM was present in 84% of cases (sensitivity 86%, specificity 77%) and whether a systemic feeding vessel was present in 90% of cases (sensitivity 92%, specificity 88%). CONCLUSIONS: Fetal ultrasound remains an important tool in the detection and evaluation of congenital lung malformations. However, it does not correctly predict histology in approximately 25% of prenatally detected CLMs and remains limited by relatively poor sensitivity for systemic feeding vessels pathognomic for a bronchopulmonary sequestration. These data suggest the importance of obtaining additional cross-sectional imaging, preferably a postnatal CT scan, in all patients to help counsel families and to guide in the optimal management of these lesions.

Comparing outcomes with thoracic epidural and intercostal nerve cryoablation after Nuss procedure.

BACKGROUND: This study aimed to evaluate postoperative outcomes after minimally invasive repair of pectus excavatum (Nuss procedure) using video-assisted intercostal nerve cryoablation (INC) compared to thoracic epidural (TE). MATERIALS AND METHODS: We performed a single center retrospective review of pediatric patients who underwent Nuss procedure with INC (n = 19) or TE (n = 13) from April 2015 to August 2017. Preoperative, intraoperative, and postoperative characteristics were collected. The primary outcome was length of stay (LOS) and secondary outcomes were intravenous and oral opioid use, pain scores, and complications. Opioids were converted to oral morphine milligram equivalents per kilogram (oral morphine equivalent [OME]/kg). Mann-Whitney U test was used for continuous and chi-squared analysis for categorical variables. RESULTS: There were no significant differences in patient characteristics, except Haller Index (INC: median [interquartile range] 4.3 [3.6-4.9]; TE: 3.2 [2.8-4.0]; P = 0.03). LOS was shorter with INC (INC: 3 [3-4] days; TE: 6 [5-7] days; P < 0.001). Opioid use was higher intraoperatively (INC: 1.08 [0.87-1.37] OME/kg; TE: 0.46 [0.37-0.67] OME/kg; P = 0.002) and unchanged postoperatively (INC: 1.78 [1.26-3.77] OME/kg; TE: 1.82 [1.05-3.37] OME/kg; P = 0.80), and prescription doses were lower at discharge in INC (INC: 30 [30-40] doses; TE: 42 [40-60] doses; P = 0.005). There was no significant difference in postoperative complications (INC: 42.1%; TE: 53.9%; P = 0.51). CONCLUSIONS: INC during Nuss procedure reduced LOS, shifting postoperative opioid use earlier during admission. This may reflect the need for improved early pain control until INC takes effect. Prospective evaluation after INC is needed to characterize long-term pain medication requirements.

Conversion from laparoscopic to open appendectomy: decreased risk at dedicated children's hospitals.

PURPOSE: The advent of laparoscopy has revolutionized surgical practice within the last 30 years. Conversion to open surgery, however, remains necessary at times, even for the most experienced laparoscopic surgeon. METHODS: The kids' inpatient database was analyzed for 2006, 2009, and 2012 for patients who underwent laparoscopic appendectomy and conversion to open (CPT 470.1 and V64.41, respectively). Variables included in multivariable analysis were determined based on those variables found to have significance on univariate analysis. RESULTS: A total of 104,865 patients, ages 0-17 years, underwent laparoscopic appendectomy during the three study periods. Of these, 2370 (2.2%) laparoscopic surgeries were converted to open appendectomy. Multivariable logistic regression showed significantly higher rates of conversion amongst patients with peritonitis (OR 6.7, p < 0.001) or abscess (OR 14.3, p < 0.001), obesity (OR 2.02, p < 0.001), age > 13 years (OR 1.53 for ages 13-15, OR 1.77 for ages 16-17, p < 0.001 for both), or cared for at rural hospitals (OR 1.55, p = 0.002). Rates of conversion decreased over time for children at adult hospitals and at urban hospitals, regardless of teaching status (p < 0.001 for both). CONCLUSION: Risk factors for conversion from laparoscopic to open appendectomy included abscess, peritonitis, increased age, obesity, male gender, socioeconomic status and treatment at a non-pediatric-specific hospital, and the overall rate is decreasing over time.

Comparison of early versus delayed strategies for repair of congenital diaphragmatic hernia on extracorporeal membrane oxygenation.

PURPOSE: For the last seven years, our institution has repaired infants with CDH that require ECMO early after cannulation. Prior to that, we attempted to decannulate before repair, but repaired on ECMO if we were unable to wean after two weeks. This study compares those strategies. METHODS: From 2002 to 2016, 65 infants with CDH required ECMO. 67.7% were repaired on ECMO, and 27.7% were repaired after decannulation. Data were compared between patients repaired ≤5days after cannulation ("early protocol", n=30) and >5days after cannulation or after de-cannulation ("late protocol", n=35). We used Cox regression to assess differences in outcomes between groups. RESULTS: Survival for the early and late protocol groups was 43.3% and 68.8%, respectively (p=0.0485). For patients that were successfully decannulated before repair, survival was 94.4%. Moreover, the early repair protocol was associated with prolongation of ECMO (16.8±7.4 vs. 12.6±6.8days, p=0.0216). After multivariate regression, the early repair protocol was an independent predictor of both mortality (HR=3.48, 95% CI=1.28-9.45, p=0.015) and days on ECMO (IRR=1.39, 95% CI=1.07-1.79, p=0.012). All bleeding occurred in patients repaired on ECMO (29.5%, 13/44). CONCLUSIONS: Our data suggest that protocolized CDH repair early after ECMO cannulation may be associated with increased mortality and prolongation of ECMO. However, early repair is not necessarily harmful for those patients who would otherwise be unable to wean from ECMO before repair. Further work is needed to better move towards individualized patient care. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.

Steroid use for refractory hypotension in congenital diaphragmatic hernia.

PURPOSE: Guidelines for diagnosis and treatment of adrenal insufficiency (AI) in newborns with congenital diaphragmatic hernia (CDH) are poorly defined. METHODS: From 2002 to 2016, 155 infants were treated for CDH at our institution. Patients with shock refractory to vasopressors (clinically diagnosed AI) were treated with hydrocortisone (HC). When available, random cortisol levels <10 µg/dL were considered low. Outcomes were compared between groups. RESULTS: Hydrocortisone was used to treat AI in 34% (53/155) of patients. That subset of patients was demonstrably sicker, and mortality was expectedly higher for those treated with HC (37.7 vs. 17.6%, p = 0.0098). Of the subset of patients with random cortisol levels measured before initiation of HC, 67.7% (21/31) had low cortisol levels. No significant differences were seen in survival between the high and low groups, but mortality trended higher in patients with high cortisol levels that received HC. After multivariate analysis, duration of HC stress dose administration was associated with increased risk of mortality (OR 1.11, 95% CI 1.02-1.2, p = 0.021), and total duration of HC treatment was associated with increased risk of sepsis (OR 1.04, 95% CI 1.005-1.075, p = 0.026). CONCLUSION: AI is prevalent amongst patients with CDH, but prolonged treatment with HC may increase risk of mortality and sepsis.