Lack of Mucosal Cholinergic Innervation Is Associated With Increased Risk of Enterocolitis in Hirschsprung's Disease.

BACKGROUND & AIMS: Hirschsprung's disease (HSCR) is a congenital intestinal motility disorder defined by the absence of enteric neuronal cells (ganglia) in the distal gut. The development of HSCR-associated enterocolitis remains a life-threatening complication. Absence of enteric ganglia implicates innervation of acetylcholine-secreting (cholinergic) nerve fibers. Cholinergic signals have been reported to control excessive inflammation, but the impact on HSCR-associated enterocolitis is unknown. METHODS: We enrolled 44 HSCR patients in a prospective multicenter study and grouped them according to their degree of colonic mucosal acetylcholinesterase-positive innervation into low-fiber and high-fiber patient groups. The fiber phenotype was correlated with the tissue cytokine profile as well as immune cell frequencies using Luminex analysis and fluorescence-activated cell sorting analysis of colonic tissue and immune cells. Using confocal immunofluorescence microscopy, macrophages were identified in close proximity to nerve fibers and characterized by RNA-seq analysis. Microbial dysbiosis was analyzed in colonic tissue using 16S-rDNA gene sequencing. Finally, the fiber phenotype was correlated with postoperative enterocolitis manifestation. RESULTS: The presence of mucosal nerve fiber innervation correlated with reduced T-helper 17 cytokines and cell frequencies. In high-fiber tissue, macrophages co-localized with nerve fibers and expressed significantly less interleukin 23 than macrophages from low-fiber tissue. HSCR patients lacking mucosal nerve fibers showed microbial dysbiosis and had a higher incidence of postoperative enterocolitis. CONCLUSIONS: The mucosal fiber phenotype might serve as a prognostic marker for enterocolitis development in HSCR patients and may offer an approach to personalized patient care and new therapeutic options.

In utero Plastic Surgery in Zurich: Successful Use of Distally Pedicled Random Pattern Transposition Flaps for Definitive Skin Closure during Open Fetal Spina Bifida Repair.

BACKGROUND: One of the intraoperative challenges of fetal spina bifida repair is skin closure when there is an extended skin defect. Thus, we examined whether distally pedicled random pattern transposition flaps (TFs) are a valid option to overcome this problem. SUBJECTS AND METHODS: All patients undergoing in utero repair of spina bifida with application of a TF for back skin closure were analyzed focusing on intraoperative flap characteristics and postoperative flap performance. RESULTS: In 30 (70%) of the 43 fetuses a primary skin closure was achieved, in 5 (12%) a skin substitute was used, and in 8 (18%) a TF was applied. Flap raising and insertion was uneventful and perfusion was sufficient in all 8 fetuses (100%). In 3 fetuses (37%) the donor sites were closed primarily, and in 5 (63%) a skin substitute was used for coverage. At birth, 7 flaps were viable and provided robust skin coverage over the center of the former lesion. Complications included a small skin defect with CSF leakage in 1 patient (13%). CONCLUSION: During open fetal spina bifida repair, TFs can be safely and efficaciously used to obtain solid and durable skin coverage over lesions too large to allow conventional primary skin closure.

Predictors of pediatric blunt cerebrovascular injury.

BACKGROUND/PURPOSE: Blunt cerebrovascular injury (BCVI) is clinically challenging because these injuries are hard to detect and can have serious neurological consequences, and optimal screening criteria have not been established for children. This study aims to determine risk factors for BCVI in pediatric patients and to evaluate screening practices in a single institutional series. METHODS: A retrospective review of all pediatric blunt trauma patients evaluated over a 10-year period was performed. Demographic, clinical, and radiographic data were reviewed, including the presence of adult risk factors for BCVI. Logistic regression analyses were performed with statistical significance established at p<0.05. RESULTS: Of the 11,596 patients evaluated during the study period, 1018 (8.8%) had at least one adult risk factor for BCVI, but only 62 (6.1% of those with risk factors) underwent angiographic evaluation. Overall, 11 BCVIs were observed, resulting in an incidence of 0.095%. All 11 patients with BCVI had at least one risk factor. Multivariate logistic regression analysis identified cervical spine fracture (OR 36.88 [8.36, 169.95]), GCS score ≤ 8 (OR 16.42 [2.16, 102.33]), male gender (OR 10.52 [1.33, 363.30]), Le Fort II or III facial fracture (OR 63.71 [2.16, 1124.68]), and ISS (unit OR 1.10 [1.04, 1.17]) as independent risk factors for BCVI. CONCLUSION: Adult screening criteria for BCVI appear appropriate for pediatric patients, but most at-risk children are not being screened. LEVEL OF EVIDENCE: Level III (retrospective case-control study).

Venoarterial Extracorporeal Life Support for Neonatal Respiratory Failure: Indications and Impact on Mortality.

Venoarterial (VA) extracorporeal life support (ECLS) for neonatal respiratory failure is associated with increased mortality compared with venovenous (VV) ECLS. It is unclear whether this is a causal relationship or reflects differences in baseline disease severity between infants managed with these two strategies. Our objective was to identify clinical variables associated with the preferential selection of VA over VV ECLS, as these may confound the association between VA ECLS and increased mortality. We identified documented indications for preferential VA selection through chart review. We then assessed how the presence of common indications impacted mortality. Thirty-nine cases met eligibility. Severity of hypotension/degree of inotropic support and ventricular dysfunction on echocardiogram before cannulation were the most common specific indications for preferential VA ECLS. Mortality was 12.5% when neither high inotropic support nor ventricular dysfunction was present. Mortality rose to 20% with high inotropic support and 25% with ventricular dysfunction present alone and to 50% when both were present. We conclude that severe hypotension and ventricular dysfunction before ECLS cannulation are common indications for VA ECLS that likely influence survival. Research assessing the impact of ECLS cannulation mode on survival should adjust for baseline differences between groups for these important variables.

Failed Intussusception Reduction in Children: Correlation Between Radiologic, Surgical, and Pathologic Findings.

OBJECTIVE: The objective of this study was to identify causes of irreducible intussusception after contrast enema and to correlate imaging findings with surgical and histopathologic findings. MATERIALS AND METHODS: Between 2005 and 2013, a total of 543 children underwent reduction of intussusception with the use of an enema technique (hereafter referred to as "enema reduction"). The medical records of 72 children (56 boys [mean age, 24.8 months; range, 3.8 months to 10.9 years] and 16 girls [mean age, 14.2 months; range, 1.5 months to 6.9 years) who underwent unsuccessful reduction and were treated surgically were retrospectively analyzed. The data collected included information on the cause of intussusception, the risk factors noted on ultrasound, operative management, outcome, and the length of the hospital stay. The imaging findings for these patients were compared with findings for statistically similar age-matched control subjects. RESULTS: Ultrasound detected 56 of 57 cases of intussusception, but it failed to detect the lead point in three cases and failed to detect ischemic necrosis in seven cases. Positive predictors of failed enema reduction were the presence of a distal mass and observation of the dissecting sign. Of the 72 patients who underwent surgical treatment of intussusception, 26 (36.1%) underwent laparoscopy, 38 (52.8%) underwent laparotomy, and eight (11.1%) underwent conversion from laparoscopy to laparotomy. Surgical reduction was performed in 61.1% of cases, small bowel resection in 19.4%, ileocecectomy in 12.5%, and self-reduction in 69%. Pathologic lead points (noted in 25% of cases) included lymphoid hyperplasia (n = 7), Meckel diverticulum (n = 3), Burkitt lymphoma (n = 3), enteric duplication cyst (n = 2), juvenile polyp (n = 2), and adenovirus appendicitis (n = 1). The length of the hospital stay was significantly longer after laparotomy. CONCLUSION: The distalmost location of the intussusception mass and presence of the dissecting sign on images obtained during contrast enema have a higher positive predictive value for failed reduction. Screening ultrasound decreases the number of unnecessary contrast enemas performed; however, classic pathologic lead points, such as Burkitt lymphoma and Meckel diverticulum, may be difficult to diagnose with the use of ultrasound. Laparotomy and laparoscopy are equally safe and efficacious in reducing intussusception, with the length of the hospital stay after laparoscopy significantly shorter than that noted after laparotomy. Most failed enema reductions are idiopathic, and pathologic lead points are noted in 25% of cases.

Immediate operative management of the fetus with airway anomalies resulting from congenital malformations.

Prenatal diagnosis has transformed the outcome of fetuses with airway obstruction. The thorough evaluation of prenatal imaging allows for categorizing fetuses with airway compromise into those who will require a special mode of delivery and those who can be delivered without any special resources. The ex-utero intrapartum treatment (EXIT) approach allows accessing the airway while the fetus is under placental support, converting a potentially catastrophic situation into a controlled one. An expert multidisciplinary team is the key to success.

Distal Humeral Epiphyseal Separation in a Newborn.

Distal humeral epiphyseal separations are rare and treatment strategies are not well defined. The case of a full-term male newborn with a distal humeral epiphyseal separation as the result of a birth trauma was reviewed. A literature review of this topic was undertaken to better understand its occurrence, diagnosis, and treatment options. The patient sustained a distal humeral epiphyseal separation during a vaginal delivery. Deformity and decreased movement in the elbow were observed. Radiographs and subsequent ultrasound were used to make the diagnosis of distal humeral epiphyseal separation. Given the displaced and acute nature of the fracture, a closed reduction and percutaneous pinning was performed. Intraoperatively, this was greatly facilitated by an elbow arthrogram. Immobilization consisted of a posterior plaster splint and swathe. Postoperative follow-up with clinical and radiographic examination showed abundant bony healing and early restoration of function. Ultrasound is useful to confirm the diagnosis of a distal humeral epiphyseal separation for elbow injuries in very young patients. However, once the diagnosis is confirmed, an intraoperative elbow arthrogram helps highlight the fracture fragments and ensures proper reduction and fixation of the fracture. [Orthopedics. 2016; 39(4):e764-e767.].

Vessel sealing comparison: old school is still hip.

BACKGROUND: Ligation with either absorbable or non-absorbable sutures has been the traditional state of the art, but a proliferation of technology now offers a host of methods to close and divide vessels. Only limited data are available that objectively compare different vessel sealing methods. The objective of this study was to compare a broad variety of methods of surgical vessel closure in a reproducible, independent, standardized test-to-failure ex vivo pressure challenge. METHODS: Ten of the most common surgical sealing devices were represented in this study, including both mechanical and energy devices. Unfixed porcine carotid arteries were selected for testing. They were connected to a pump, and automated controlled infusion was initiated. Upon identification of a leak at the source of sealing, the maximum pressure in mmHg was logged. RESULTS: There were a total of 184 trials conducted using the 10 vessel sealing methods. The average burst pressure across all trials was 1100 mmHg with a range of 51.3-5171 mmHg. Suture-based methods displayed the highest average pressure until failure. Stapling methods showed the lowest burst pressures. All methods showed mean burst pressures above the "physiologically relevant" level of 250 mmHg. CONCLUSIONS: This study presents an independent, reproducible, ex vivo comparison of multiple methods of surgical arterial closure. In these laboratory conditions, tests to failure demonstrated widely varying sealing strength, highly dependent on method. All hemostatic modalities tested are capable of securing vessels safely and well above physiologic blood pressures, while suture-based methods were significantly stronger than other mechanical methods or modern energy devices.

Compartment Pressures in Children With Normal and Fractured Forearms: A Preliminary Report.

INTRODUCTION: Acute compartment syndrome (ACS) can lead to irreversible damage if fasciotomy is not performed in a timely manner. Needle manometry is a tool to confirm suspected ACS. The threshold for compartment pressures that can be tolerated has been debated. The aim of this study is to assess the normal compartment pressures in noninjured forearms of children. Further, we sought to quantify the maximum tolerable compartment pressures in fractured forearms of children, thus establishing a baseline and providing guidance in evidence-based decision making to evaluate children with suspected ACS. METHODS: This prospective study included children up to the age of 16 years with forearm fractures that needed reduction with or without osteosynthesis. Between June 2009 and March 2013, 41 children were included. Mean age was 9.25 years (range, 4 to 15.4 y). We used needle manometry to measure the pressures in the superficial and deep volar as well as in the dorsal compartments (DCs) on both the forearms. The mean pressures between compartments in healthy versus injured arms were analyzed using a 1-sided, paired t test. RESULTS: On the injured side, the mean compartment pressure was 19.12 mm Hg (range, 3 to 49 mm Hg) in the deep volar compartment, 15.56 mm Hg (range, 5 to 37 mmHg) in the DC, and 14.8 mm Hg (range, 2 to 35 mm Hg) in the superficial volar compartment. On the noninjured side, the mean compartment pressure was 12.9 mm Hg (range, 6 to 31 mm Hg) in the DC, 10.22 mm Hg (range, 3 to 22 mm Hg) in the deep volar compartment, and 9.66 mm Hg (range, 3 to 21 mm Hg) in the superficial volar compartment. We measured an absolute compartment pressure of >30 mm Hg in 15 patients on the fractured side. Three of them had an absolute compartment pressure of >45 mm Hg. Only 1 had ACS. This patient underwent fasciotomy and was excluded for further analysis. On follow-up (mean, 24.84 mo), no patient was found to have any sequelae of ACS. DISCUSSION: This is the first study to report normal compartment pressure measurements in noninjured forearms and in fractured forearms without clinical suspicion of ACS in children.The mean compartment pressure measured in the deep volar compartment (DVC) in healthy children was 10.22 mm Hg (range, 3 to 22 mm Hg) and therefore slightly higher than in adults. Some children with fractures tolerated absolute compartment pressures >30 mm Hg without clinical signs of ACS. Fasciotomy in children under close observation could eventually be delayed despite surpassing the accepted pressure limits for adults. LEVEL OF EVIDENCE: Level I-prognostic.

Laparoscopy in Pediatric Abdominal Trauma: A 13-Year Experience.

Introduction Abdominal injuries are common, costly, deadly, and a source of considerable uncertainty in pediatric trauma. In some circumstances, laparoscopy offers a diagnostic and therapeutic alternative with less morbidity than standard exploration, and more certainty than most imaging. In this study, we review our experience with laparoscopic exploration for trauma. We hypothesized that laparoscopy could be employed effectively in stable blunt or penetrating trauma patients in whom diagnostic uncertainty was unresolved by imaging. Patients and Methods A retrospective review of the trauma database identified all children admitted to our Level I pediatric trauma center between January 1, 2000, and December 31, 2012, requiring surgical abdominal exploration. The utilization of laparoscopy and laparotomy was charted over the 13-year period. Negative laparoscopies and laparotomies and nontherapeutic laparoscopies and laparotomies were examined to investigate clinical scenarios in which laparotomy might have been avoided. Statistical analyses were performed using descriptive statistics, simple linear regression analysis, and Mann-Whitney U test (p < 0.05). Results Over the 13-year study period, there were 16,321 trauma admissions. Of these, 119 patients (0.7%) required surgical abdominal exploration: 81 patients underwent laparotomy and 38 patients underwent laparoscopy. In 13 patients (34.2%), laparoscopic exploration ruled out injuries. In nine patients (23.7%), laparoscopy identified an injury for which no surgical intervention was necessary. In nine patients (23.7%), an injury was repaired laparoscopically. In seven cases (18.4%), the identified injury required conversion to laparotomy. There were no missed injuries. In the laparotomy group, a less invasive approach could have yielded the same information in 8.7% of patients. Laparoscopy was more likely to be used after a qualitative change in institutional minimally invasive surgical capability. Conclusion Laparoscopy reliably resolves diagnostic uncertainty in selected cases of pediatric abdominal blunt and penetrating trauma. In a hemodynamically stable patient with a concerning exam and inconclusive imaging, laparoscopy provides sensitive diagnostic capability and opportunity for definitive repair with diminished surgical morbidity.

Utility of neurocognitive testing of mild traumatic brain injury in children treated and released from the emergency department.

PRIMARY OBJECTIVE: To assess feasibility and utility of neurocognitive testing of children evaluated and discharged from the ED with mild traumatic brain injury (MTBI). METHODS: Paediatric blunt trauma patients (aged 11-18 years) evaluated in the ED for MTBI and control patients with isolated lower extremity injury were prospectively enrolled. All patients were administered a validated neurocognitive test (ImPACT(©)). Wilcoxon sign rank tests were used to compare reported symptoms and neurocognitive performance between subjects and controls, as well as to matched normative data. RESULTS: Thirty-nine subjects and 46 controls were enrolled. The MTBI patients had a mean age of 13.9 years (53.8% male). An abnormal symptom score was reported in 89.7% of MTBI subjects (mean score = 29.4, normal ≤ 8), differing significantly (p < 0.05) from controls, in whom 39.1% demonstrated an abnormal score (mean score = 8.7). In all neurocognitive test domains, visual motor speed and reaction time, MTBI patients demonstrated lower scores than normative data (p < 0.05). CONCLUSIONS: Patients with MTBI were more likely than control subjects to have scores on any or all neurocognitive domains below the 25th percentile and 10th percentile. In the ED setting, acute neurocognitive testing of MTBI in children is feasible. This highlights the importance of structured follow-up for this treated and released population.

Systemic hypertension in giant omphalocele: An underappreciated association.

PURPOSE: To evaluate the incidence, severity and duration of systemic hypertension in infants born with giant omphalocele (GO). METHODS: A retrospective review of patients born from 2003 through 2013 with a GO or intestinal atresia (control population) and managed at a single institution was performed. The hospital course was reviewed including all blood pressures, method of omphalocele repair, requirement for antihypertensive medications and renal function. RESULTS: Forty-five GO and 20 control patients met criteria for the study. Thirty-three GO patients underwent Schuster repair and 12 GO patients underwent delayed repair after epithelialization. Overall, 78% of GO patients had episodes of hypertension (82% Schuster and 67% delayed repair) compared to 15% of control patients (P<0.001). The majority of episodes were transient and occurred in the postoperative period (97%). Hypertension was persistent in 4 GO patients. These patients required antihypertensive medication at discharge, which was discontinued as an outpatient. No patient demonstrated significant evidence of renal abnormalities as indicated by renal ultrasound, urinalysis and/or serum creatinine level at the time of hypertension. CONCLUSION: Episodes of systemic hypertension are frequent in patients with GO. Episodes are often post-operative, transient and can be present in patients undergoing either a delayed or Schuster repair. A small subset of patients will have persistent hypertension requiring antihypertensive medication that can be weaned off in an outpatient setting.