Diaphragmatic Defects in Infants: Acute Management and Repair.

Congenital diaphragmatic hernia (CDH) is a complex and highly variable disease process that should be treated at institutions with multidisciplinary teams designed for their care. Treatment in the neonatal period focuses on pulmonary hypoplasia, pulmonary hypertension, and cardiac dysfunction. Extracorporeal membrane oxygenation (ECMO) can be considered in patients refractory to medical management. Repair of CDH early during the ECMO course seems to improve mortality compared with other times for surgical intervention. The choice of surgical approach to CDH repair should consider the patient's physiologic status and the surgeon's familiarity with the operative approaches available, recognizing the pros/cons of each technique.

Difference in Presentation and Concomitant Intra-Abdominal Injury with Chance Fracture in Pediatric and Adult Populations.

BACKGROUND: Chance fracture (CFx) with concomitant intra-abdominal injury has variable occurrence rates ranging from 33 to 89%. No single study has compared the incidence of simultaneous abdominal injury between pediatric and adult populations. This study compares the rate of simultaneous intra-abdominal injury and chance fracture in these populations. METHODS: A retrospective review of pediatric and adult patients with chance fracture in comparable pediatric and adult trauma centers was performed. Patient demographics, mechanism of injury (MOI), and injury patterns were collected from 2002 to 2019 for pediatric patients and 2015 to 2018 for adults. Student t-test analyses were performed to determine statistical significance between the cohorts. RESULTS: The pediatric group had a similar incidence of abdominal solid organ injuries compared to adults (16 [20.5%] vs. 40 [19.7%], p<0.879), but the pediatric group had a greater number of total intra-abdominal (49 [62.8%] vs. 47 [23.1%], p < 0.001) and hollow organ injuries (40 [51.3%] vs. 17 [8.4%], p < 0.001). Motor vehicle collision was the most common mechanism of injury for both groups (72 pediatric [92.3%] vs. 85 adult [41.7%]) but adults suffered from more falls (3 pediatric vs. 81 adult, p < 0.001). Pediatric patients with CFx caused by MVCs had more intra-abdominal injuries (48 [66.7%] vs. 25[29.8%], p < 0.001) and hollow organ injuries compared to adults (39 [54.2%] vs. 8[9.5%], p < 0.001). CONCLUSION: In the setting of Chance fracture after trauma, pediatric patients are more likely to have a concomitant intra-abdominal organ injury (63% vs. 23%), especially hollow viscus injury (51.3% vs. 8.4%) compared with adults regardless of mechanism.

Pediatric Handlebar Injuries: More Than Meets the Abdomen.

OBJECTIVES: Injuries associated with bicycles can generally be categorized into 2 types: injuries from falling from/off bicycles and injuries from striking the bicycle. In the second mechanism category, most occur as a result of children striking their body against the bicycle handlebar. The purpose of this study was to evaluate the presentation, body location, injury severity, and need for intervention for pediatric handlebar injuries at a single level one pediatric trauma center and contrast these against other bicycle-related injuries in children. METHODS: This work is a retrospective review of the trauma registry over an 8-year period. Individual charts were then reviewed for patients' demographic factors, injury details, and other clinical/radiographic findings. Each patient was then categorized as either having a handlebar versus nonhandlebar injury. Additionally, each patient's injuries were classified according to affected body "zone(s)" and the need for intervention in relation to these injuries. During the course of chart review, several unique radiographic and history/physical findings were noted and are also reported. RESULTS: During the study period, 385 patients were identified that met study criteria. Bicycle handlebars were involved in 27.8% (107/385) of injuries and 72.2% (278/385) were nonhandlebar injuries. There were differences in injury severity score, Head Abbreviated Injury Scale, length of stay between patients with handlebar versus nonhandlebar injuries, respectively. There were also differences in incidence of injuries across most body zones between patients with handlebar versus nonhandlebar injuries. There was statistically significant difference in need for intervention for abdominal solid organ injuries among handlebar versus nonhandlebar injuries mechanisms (21.6% vs 0%; P = 0.026), respectively. Sixteen patients with a handlebar injury underwent abdominal computed tomography (CT), which found only pericolic/pelvic free fluid or were negative for any disease and had normal/mildly elevated liver function test results at the time of arrival with otherwise normal laboratory workup results. Two patients required laparotomy for bowel injury and presented with peritonitis less than 12 hours after injury. The remaining patients did not have peritonitis on examination and were discharged without operative intervention 12 to 24 hours after injury without further event. CONCLUSIONS: The bicycle handlebar is a unique mechanism of injury. The location, need for intervention, and the nature of the injury can vary significantly compared to other bicycle injuries. Handlebar injuries are more likely to cause abdominal and soft tissue injuries, whereas nonhandlebar injuries are more likely to cause extremity and skull/neck/central nervous system injuries. Because more than 20% of the reported handlebar injuries did not involve the abdomen or thoracoabdominal/extremity soft tissue as well as the variable presentation of handlebar injuries, it is imperative for the physician to consider this mechanism in all bicycle injuries. In addition, even within the same area of the body, handlebar injuries can be very different compared to nonhandlebar (i.e., orthopedic vs vascular injuries in the extremities). Physical examination and observation remain paramount when laboratory and radiographic workups are equivocal.

A multifactorial severity score for left congenital diaphragmatic hernia in a high-risk population using fetal magnetic resonance imaging.

BACKGROUND: Adverse outcomes for infants born with left congenital diaphragmatic hernia (CDH) have been correlated with fetal imaging findings. OBJECTIVE: We sought to corroborate these correlations in a high-risk cohort and describe a predictive mortality algorithm combining multiple imaging biomarkers for use in prenatal counseling. MATERIALS AND METHODS: We reviewed fetal MRI examinations at our institution from 2004 to 2016 demonstrating left-side CDH. MRI findings, hospital course and outcomes were recorded and analyzed using bivariate and multivariable analysis. We generated a receiver operating curve (ROC) to determine a cut-off relation for mortality. Finally, we created a predictive mortality calculator. RESULTS: Of 41 fetuses included in this high-risk cohort, 41% survived. Per bivariate analysis, observed-to-expected total fetal lung volume (P=0.007), intrathoracic position of the stomach (P=0.049), and extracorporeal membrane oxygenation (ECMO) requirement (P<0.001) were significantly associated with infant mortality. Youden J statistic optimized the ROC for mortality at 24% observed-to-expected total fetal lung volume (sensitivity 64%, specificity 82%, area under the curve 0.72). On multivariable analysis, observed-to-expected total fetal lung volume ± 24% was predictive of mortality (adjusted odds ratio, 95% confidence interval: 0.09 [0.02, 0.55]; P=0.008). We derived a novel mortality prediction calculator from this analysis. CONCLUSION: In this high-risk cohort, decreased observed-to-expected total fetal lung volume and stomach herniation were significantly associated with mortality. The novel predictive mortality calculator utilizes information from fetal MR imaging and provides prognostic information for health care providers. Creation of similar predictive tools by other institutions, using their distinct populations, might prove useful in family counseling, especially where there are discordant imaging findings.

Biologic Mesh Underlay in Thoracoscopic Primary Repair of Congenital Diaphragmatic Hernia Confers Reduced Recurrence in Neonates: A Preliminary Report.

Purpose: The purpose of this study was to determine if utilization of biologic mesh underlay during thoracoscopic congenital diaphragmatic hernia (CDH) primary repair (PR) results in reduced 5-year hernia recurrence rates. Methods: A retrospective review was completed for all primarily repaired CDH utilizing a thoracoscopic approach from January 1, 2003 to June 31, 2013. Patients were included in the study cohort if they had a thoracoscopic PR of their CDH without any mesh reinforcement or with biologic mesh underlay. Charts were then reviewed for no less than 5 years postoperatively for reports of clinical and/or radiographic hernia recurrence. The cumulative annual hernia recurrence as well as other demographic factors were analyzed. Results: Within the study period, 46 patients were identified that met study criteria. Three patients were lost to follow-up. Fifteen of the remaining patients (15/43: 34.9%) had a biologic underlay. Within the cohort, seven recurrences were noted within 5 years of the index procedure (7/43; 16.7%). Four recurrences occurred within the first postoperative year, and all occurred by the third postoperative year. One recurrence was in a patient with a biologic underlay at 4 months after repair. This was a clinically/radiographically silent 4 mm defect and noted at laparoscopy for another indication (1/15: 6.6%). The remainder occurred in primarily repaired patients without mesh reinforcement (6/28: 21.4%). Conclusions: Thoracoscopic PR of CDH can be successfully performed in select patients. The use of a biologic mesh underlay in this subset of patients appears to confer reduced hernia recurrence.

Trends in pediatric-adjusted shock index predict morbidity in children with moderate blunt injuries.

PURPOSE: Trending the pediatric-adjusted shock index (SIPA) after admission has been described for children suffering severe blunt injuries (i.e., injury severity score (ISS) ≥ 15). We propose that following SIPA in children with moderate blunt injuries, as defined by ISS 10-14, has similar utility. METHODS: The trauma registry at a single institution was queried over a 7 year period. Patients were included if they were between 4 and 16 years old at the time of admission, sustained a blunt injury with an ISS 10-14, and were admitted less than 12 h after their injury (n = 501). Each patient's SIPA was then calculated at 0, 12, 24, 36, and 48 h (h) after admission and then categorized as elevated or normal at each time frame based on previously reported values. Trends in outcome variables as a function of time from admission for patients with an abnormal SIPA to normalize as well as patients with a normal admission SIPA to abnormal were analyzed. RESULTS: In patients with a normal SIPA at arrival, elevation within the first 24 h of admission correlated with increased length of stay (LOS). Increased transfusion requirement, incidence of infectious complications, and need for in-patient rehabilitation were also seen in analyzed sub-groups. An elevated SIPA at arrival with increased length of time to normalize SIPA correlated with increased length of stay LOS in the entire cohort and in those without head injury, but not in patients with a head injury. No deaths occurred within the study cohort. CONCLUSIONS: Patients with an ISS 10-14 and a normal SIPA at time of arrival who then have an elevated SIPA in the first 24 h of admission are at increased risk for morbidity including longer LOS and infectious complications. Similarly, time to normalize an elevated admission SIPA appears to directly correlate with LOS in patients without head injuries. No correlations with markers for morbidity could be identified in patients with a head injury and an elevated SIPA at arrival. This may be due to small sample size, as there were no relations to severity of head injury as measured by head abbreviated injury scale (head AIS) and the outcome variables reported. This is an area of ongoing analysis. This study extends the previously reported utility of following SIPA after admission into milder blunt injuries.

Multicenter pre-operative assessment of pediatric ovarian malignancy.

PURPOSE: The purpose of this study was to develop a pre-operative risk assessment tool for childhood and adolescent ovarian malignancy, in order to guide operative management of pediatric ovarian masses. METHODS: We conducted a retrospective analysis of patients <18 years old who underwent ovarian surgery at two quaternary care pediatric centers over 4 years (1/1/13-12/31/16). Probability of malignancy was estimated based on imaging characteristics (simple cyst, heterogeneous, or solid), maximal diameter, and tumor markers (α-fetoprotein, ß-human chorionic gonadotropin). RESULTS: Among 188 children with ovarian masses, 11% had malignancies. For simple cysts, there were no malignancies (0/24, 95% CI = 0-17%). Among solid lesions, 44% (15/34, 95% CI = 28-62%) were malignant. Among marker-elevated heterogeneous masses, 40% (2/5, 95% CI = 12-77%) were malignant. Conversely, small (≤10 cm) and large (>10 cm) marker-negative heterogeneous lesions had malignancy proportions of 0% (0/39, 95% CI = 0-11%) and 5% (2/40, 95% CI = 1-18%), respectively. CONCLUSIONS: Given the malignancy estimates identified from these multi-institutional data, we recommend an attempt at ovarian-sparing resection for simple cysts or tumor marker-negative heterogeneous lesions ≤10 cm. Oophorectomy is recommended for solid masses or heterogeneous lesions with elevated markers. Finally, large (>10 cm) heterogeneous masses with non-elevated markers warrant a careful discussion of ovarian-sparing techniques. Complete surgical staging is mandatory regardless of operative procedure. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level I.

Review of Early Postoperative Metrics for Children Undergoing Resection of Congenital Pulmonary Airway Malformations and Report of Pleuropulmonary Blastoma at a Single Institution.

PURPOSE: The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB). MATERIALS AND METHODS: An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed. RESULTS: A total of 51 patients with a pathologic diagnosis of CPAM (n = 45; 88.2%) or PPB (n = 6; 11.8%) underwent surgical resection. Among patients treated for PPB, one death occurred approximately 13 months after diagnosis. Although four patients with PPB (four out of six; 66.7%) had radiographic indicators highly suggestive of malignancy prior to surgery, two had a preoperative diagnosis of CPAM (two out of six; 33.3%). Twenty-four patients (24 out of 45; 53.3%) with CPAM underwent resection after developing symptoms and 21 (21 out of 45; 46.7%) were symptomatic at the time of surgery. Mann-Whitney's tests revealed a statistically significant difference in postoperative length of stay (median: 6 vs. 3 days; p < 0.001) and days with thoracostomy tube in place (median 3 vs. 2 days; p = 0.003) for symptomatic versus asymptomatic patients, respectively. CONCLUSION: CPAM patients appear to recover faster from surgery, if performed before the onset of symptoms. There may be a benefit to waiting until at least 3 months of age to complete resection in the asymptomatic patient. A low threshold for resection should be maintained in patients where delineating CPAM from PPB is difficult.

Radiographic and Clinical Factors in Pediatric Patients With Surgical Small-bowel Intussusception.

BACKGROUND: When evaluating a pediatric patient with abdominal pain, identification of a small bowel-to-small bowel intussusception (SBI) on radiologic imaging can create a diagnostic dilemma. The clinical significance and need for surgical exploration of SBI is highly variable, as most of them are considered clinically insignificant. We hypothesize that combination of clinical and radiologic factors in an exclusively SBI population will yield factors that guide the clinician in making operative decisions. METHODS: A comprehensive database from a pediatric tertiary hospital was reviewed from January 1, 2011, to December 31, 2016, for any radiographic study mentioning intussusception. Results were reviewed for patients having only SBI (i.e., not ileocolic intussusception), and this comprised the study cohort. The electronic medical records for these patients were reviewed for clinical presentation variables, need for operative intervention, and identification of the intussusception during surgery. Patients with SBI due to enteral feeding tubes were excluded from the study. RESULTS: Within the study period, 139 patients were identified with an SBI on radiologic imaging. Univariate analysis yielded numerous clinical and radiologic factors highly predictive of the need for surgical intervention. However, upon multivariate analysis, only a history of prior abdominal surgery (odds ratio [OR]: 7.2; CI: 1.1-46.3), the presence of focal abdominal pain (OR: 22.1; CI: 4.2-116.3), and the intussusception length (cm; OR: 10.6; CI: 10.3-10.8) were correlated with the need for surgical intervention. CONCLUSIONS: SBI is a disease process with a highly variable clinical significance. The presence of focal abdominal pain, a history of prior abdominal surgery, and the intussusception length are the greatest predictors of the need for operative intervention. LEVEL OF EVIDENCE: Level II.

Clinical factors and high-resolution manometry predicting response to surgery for achalasia in children.

BACKGROUND: Esophageal achalasia is an uncommon condition in children. Although many interventions exist for the management of this disorder, esophageal (Heller) myotomy offers one of the most durable treatments. Our institution sought to review patients undergoing Heller myotomy concentrating on preoperative clinical factors that might predict postoperative outcomes. MATERIALS AND METHODS: All patients from January 1, 2007, to December 31, 2016, who underwent surgical treatment for achalasia at our tertiary pediatric hospital were identified and included in the study cohort. Electronic medical records for these patients were reviewed for clinical presentation variables, nonsurgical preoperative treatment, surgical approach, clinical response to surgery, need for postoperative treatment for ongoing symptoms, and high-resolution manometry (HRM) data. RESULTS: Twenty-six patients were included in the study, and all underwent myotomy with partial fundoplication (median age: 14.4 y [interquartile range 11.6-15.5]). At a median follow-up of 9.75 mo (interquartile range 3.5-21 mo), 16 (61.5%) patients reported good resolution of their dysphagia symptoms with surgery alone. Two patients (7.7%) had perforation of the gastrointestinal tract requiring surgical intervention. Eight patients (30.8%) required additional treatment for achalasia, with 5 (19.2%) of these undergoing additional surgery or endoscopic treatment. Patients who had preoperative dilation did not have good resolution of their dysphagia (n = 2; P = 0.037). Two of four patients undergoing postoperative dilation had preoperative dilation. None of these patients underwent preoperative manometry. There was a statistically significant difference in the ages of patients who required postoperative intervention and those who did not (14.1 versus 15.2 y old, respectively; P = 0.043). In patients who reported improvement of gastroesophageal reflux disease/reflux type symptoms after Heller myotomy, lower esophageal residual pressure (29.1 versus 18.7 mmHg; P = 0.018) on preoperative HRM was significantly higher than in those who did not report improvement after surgery. Higher upper esophageal mean pressure (66.6 versus 47.8 mmHg; P = 0.05) also predicted good gastroesophageal reflux disease/reflux symptom response in a similar manner. CONCLUSIONS: Current analysis suggests that preoperative dilation should be used cautiously and older patients may have a better response to surgery without need for postoperative treatment. In addition, preoperative HRM can aid in counseling patients in the risk of ongoing symptoms after surgery and may aid in determining if a fundoplication should be completed at the index procedure. Further research is needed to delineate these factors. LEVEL OF EVIDENCE: Level III.

Trends in pediatric adjusted shock index predict morbidity and mortality in children with severe blunt injuries.

PURPOSE: The utility of measuring the pediatric adjusted shock index (SIPA) at admission for predicting severity of blunt injury in pediatric patients has been previously reported. However, the utility of following SIPA after admission is not well described. METHODS: The trauma registry from a level-one pediatric trauma center was queried from January 1, 2010 to December 31, 2015. Patients were included if they were between 4 and 16years old at the time of admission, sustained a blunt injury with an Injury Severity Score≥15, and were admitted less than 12h after their injury (n=286). Each patient's SIPA was then calculated at 0, 12, 24, 36, and 48h after admission and then categorized as elevated or normal at each time frame based upon previously reported values. Trends in outcome variables as a function of time from admission for patients with an abnormal SIPA to normalize as well as patients with a normal admission SIPA to abnormal were analyzed. RESULTS: In patients with a normal SIPA at arrival, 18.4% of patients who developed an elevated SIPA at 12h after admission died, whereas 2.4% of patients who maintained a normal SIPA throughout the first 48h of admission died (p<0.01). Among patients with an elevated SIPA at arrival, increased length of time to normalize SIPA correlated with increased length of stay (LOS) and intensive care unit (ICU) LOS. Similarly, elevation of SIPA after arrival in patients with a normal initial SIPA correlated to increased LOS and ICU LOS. CONCLUSIONS: Patients with a normal SIPA at time of arrival who then have an elevated SIPA in the first 24h of admission are at increased risk for morbidity and mortality compared to those whose SIPA remains normal throughout the first 48h of admission. Similarly, time to normalize an elevated admission SIPA appears to directly correlate with LOS, ICU LOS, and other markers of morbidity across a mixed blunt trauma population. Whether trending SIPA early in the hospital course serves only as a marker for injury severity or if it has utility as a resuscitation metric has not yet been determined. TYPE OF STUDY: Prognostic. LEVEL OF EVIDENCE: Level II.

Sclerotherapy for the management of rectal prolapse in children.

PURPOSE: Rectal prolapse is a commonly occurring and usually self-limited process in children. Surgical management is indicated for failures of conservative management. However, the optimal approach is unknown. The purpose of this study is to determine the efficacy of sclerotherapy for the management of rectal prolapse. METHODS: This was a retrospective review of children <18years with rectal prolapse who underwent sclerotherapy, predominantly with peanut oil (91%), between 1998 and 2015. Patients with imperforate anus or cloaca abnormalities, Hirschprung disease, or prior pull-through procedures were excluded. RESULTS: Fifty-seven patients were included with a median age of 4.9years (interquartile range (IQR) 3.2-9.2) and median follow-up of 52months (IQR 8-91). Twenty patients (n=20/57; 35%) recurred at a median of 1.6months (IQR 0.8-3.6). Only 3 patients experienced recurrence after 4months. Nine of the patients who recurred (n=9/20; 45%) were re-treated with sclerotherapy. This was successful in 5 patients (n=5/9; 56%). Two patients (n=2/20; 10%) experienced a mucosal recurrence which resolved with conservative management. Forty-four patients were thus cured with sclerotherapy alone (n=44/57; 77%). No patients undergoing sclerotherapy had an adverse event. Thirteen patients (n=13/20; 65%) underwent rectopexy after failing at least one treatment of sclerotherapy. Three of these patients (n=3/13; 23%) recurred following rectopexy and required an additional operation. CONCLUSIONS: Injection sclerotherapy for children with rectal prolapse resulted in a durable cure of prolapse in most children. Patients who recur following sclerotherapy tend to recur within 4months. Another attempt at sclerotherapy following recurrence is reasonable and was successful half of the time. Sclerotherapy should be the preferred initial treatment for rectal prolapse in children and for the initial treatment of recurrence. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Treatment Study.