Outcomes for Prenatally Diagnosed Right Congenital Diaphragmatic Hernia.

BACKGROUND: Right congenital diaphragmatic hernia (CDH) occurs less frequently than left CDH. Therefore, prognostic indicators for right CDH are not as well studied as for left CDH. METHODS: A retrospective review from a single, tertiary referral center (from 1994 until July 2013) of patients with unilateral right CDH was conducted. Prenatal characteristics were evaluated and correlated with survival to discharge and need for extracorporeal membranous oxygen (ECMO). RESULTS: In total, 34 patients were identified. There were 12 postnatal deaths and 2 fetal demises (6%), representing an overall mortality of 41%. Six patients required ECMO. Nine patients underwent fetal intervention and were analyzed separately. For patients not undergoing fetal intervention, the survival rate was 52% and a higher mean (±SD) lung-to-head ratio (LHR) was associated with survival (1.1 ± 0.4 vs. 0.8 ± 0.2, p = 0.03). There were no deaths or need for ECMO in any patient with an LHR ≥1.0. Of the 9 patients who underwent fetal intervention, survival was 78% and only 1 patient required ECMO. Fetal intervention was primarily tracheal occlusion (n = 8). CONCLUSIONS: An LHR <1.0 is associated with worse survival for right CDH and may also reflect the need for ECMO.

Amniotic band syndrome: a single-institutional experience.

INTRODUCTION: Amniotic band syndrome (ABS) is uncommon. We review our single-institutional experience to define its natural history and outcomes. MATERIALS AND METHODS: We conducted a retrospective analysis from a single, tertiary referral center of patients evaluated for and confirmed to have ABS from 1997 to 2012. RESULTS: Twenty-eight patients had confirmed ABS. The mean ± SD maternal age was 27.9 ± 5.9 years, and the mean gestational age at diagnosis was 20.7 ± 3.8 months. Oligohydramnios was reported in 4 patients. Eleven patients had membrane disruption, of whom 4 had undergone a prior percutaneous intervention. Extremities were the most common site affected (n = 20), followed by the umbilical cord (n = 7), abdomen (n = 5), limb-body wall complex (n = 5), head (n = 1), and chest (n = 1). Nine patients were felt to be candidates for fetal intervention; 5 underwent fetoscopic amniotic band lysis with 4 survivors. Overall survival, excluding 3 terminations, was 74%. There were 5 fetal demises and one neonatal death. Cord involvement was higher in nonsurvivors (67%) compared to survivors (19%, p = 0.05). DISCUSSION: ABS most commonly involves the extremities. Membrane disruption is not always present. Fetoscopic lysis is appropriate for select patients, and special consideration should be given for cord involvement, which is associated with a worse outcome.

Review of our experience with neuroblastoma and ganglioneuroblastoma in adults.

BACKGROUND: Neuroblastomas and ganglioneuroblastomas (NB/GNB) are malignant tumors that rarely occur in adults. Their disease progression and appropriate treatment are unclear. METHODS: All adults (age ≥18 years) were evaluated for histologically confirmed NB/GNB within our institution. Data were collected via chart review and direct patient contact. RESULTS: From 1980 to 2009, a total of 15 adult patients with NB/GNB were evaluated: six men (mean age 23 years, range 19-33 years) and nine women (mean 34 years, range 20-66 years). Their overall average age at diagnosis was 30 years. Tumor-related symptoms occurred in ten patients: Pain (abdominal 3, back 2, pelvic 1, groin 1) was more common than a mass (abdominal 2) or dysmenorrhea (1). Five patients had tumors found incidentally by computed tomography (4) or chest radiography (1). Primary tumor origins were in the pelvis (4), mediastinum (3), abdomen (2), adrenal gland (2), retroperitoneum (2), and mixed locations (2). Altogether, 12 patients underwent surgical resection (biopsy in 3; resections of R0 in 5, R1 in 3, R2 in 4). Ten underwent chemotherapy. Histology showed four GNBs and 11 NBs. Five patients with stage I disease survived a mean of 21 years (range 10-40 years). Two are alive today. Three stage III patients died at 2, 6, and 9 years after diagnosis (mean 5.7 years). Six of seven patients (one was lost to follow-up) with initial stage IV neuroblastoma died within 5 years (mean 2.7 years). NB and GNB patients had similar survivals. CONCLUSIONS: Adult-onset NB/GNB is rare. Symptoms appear to occur later when incurable stage IV disease is detected. Complete surgical resection can lead to long-term, disease-free survival in stage I patients.

Multi-institutional experience with penetrating pancreatic injuries in children.

PURPOSE: Penetrating pancreatic injuries in children are uncommon and are not well described in the literature. We report a multi-institutional experience with penetrating pancreatic injuries in children. METHODS: A retrospective review of children sustaining penetrating pancreatic injuries was performed at eight pediatric trauma centers. RESULTS: Sixteen patients were identified. Eleven patients were male; (mean ± SE) age was 11.7 ± 1.2 years. The mechanism of injury was gun-shot wound in 14 patients and mean injury-severity score was 18 ± 3. All patients had associated injuries, most frequently small bowel injuries (n = 9). Patients had either grade I (n = 4), grade II (n = 7), or grade III (n = 4) injuries; there was a single grade V injury. All patients underwent exploratory celiotomy. Drainage of the injured pancreas was performed in 11 patients, and 2 patients underwent pancreatorrhaphy in addition to drainage; 3 underwent resection for grade III (n = 2) and grade V (n = 1) injuries. Thirteen patients required other intra-abdominal procedures. All patients required intensive care over a mean 11.0 ± 3.0 days. Mean duration of stay was 30.1 ± 5.6 days. Post-operative morbidity was 62.5% with no mortalities. CONCLUSIONS: Penetrating pancreatic injuries in children are uncommon and most often due to firearms. There is a high association with other injuries particularly hollow viscous perforation.

Accuracy of American College of Surgeons National Surgical Quality Improvement Program Pediatric for laparoscopic appendectomy at a single institution.

BACKGROUND: In October 2008, the American College of Surgeons revealed the National Surgical Quality Improvement Program (NSQIP) Pediatric in an effort to improve quality of surgical care in children. A 5% disagreement rate of data reported between institutions is accepted. The two goals of this study were to (1) determine if the random sampling performed with NSQIP data collection was representative of the population, and (2) verify that data captured in NSQIP was accurate. METHODS: For children undergoing laparoscopic appendectomy from April 2010-April 2011, demographic data, length of stay (LOS), and rates of surgical site infection (SSI) and postoperative abscess recorded in NSQIP (group 1) were compared with data from chart review (group 2). Secondarily, all NSQIP data were examined for accuracy by comparing relevant data points to existing databases. All disagreements were further examined with review of the medical chart. Unpaired t-test and χ(2) with Fisher's exact test were used in the statistical analysis. RESULTS: NSQIP Pediatric captured data from 126 children (group 1); group 2 had 525 children. There were no significant differences in age, body mass index, gender, race or LOS between the two groups. Rate of SSI was 1.6% in group 1 and 1.7% in group 2 (P = 0.92). Abscess rate was 1.6% in group 1 and 3.4% in group 2 (P = 0.28). There were six errors in the NSQIP database. One child was listed as having two SSI. One child with postoperative abscess was missed and another was not counted as they were not categorized correctly. Recorded LOS was incorrect for two children and the other had incorrect age. CONCLUSIONS: NSQIP Pediatric captured a representative sample of patients undergoing laparoscopic appendectomy. Errors were found in the reporting of outcomes for SSI and postoperative abscess in children undergoing laparoscopic appendectomy. Given the low incidence of these outcomes, there is little effect on percentages of complications reported.

Clinical outcomes following bowel resection versus reduction of intussusception.

BACKGROUND: Intussusception is most commonly managed with air-contrast reduction. However, when this fails, emergent operation with resection or manual reduction is indicated. It is not known if there are advantages to resection compared with manual reduction. METHODS: A retrospective review of all patients receiving operative care for intussusception from February 2000 to December 2011. Patients undergoing intestinal resection were compared with those treated with manual reduction alone. RESULTS: Of 111 patients, 49 underwent resection and 62 underwent manual reduction. Mean (±SD) time to oral intake favored manual reduction (2.1 ± 1.2 versus 2.6 ± 1.2 d, respectively, P=0.05). Manual reduction was associated with a greater need for repeat imaging (47% versus 18%, P=0.002) and the only recurrences were with manual reduction (8% versus 0%, P=0.1). Mean duration of stay was no different (P=0.36), nor was the need for reoperation (P=0.9). CONCLUSIONS: Patients undergoing manual reduction have an increased number of radiographic imaging procedures. The surgeon should have a low threshold for resection for intussusceptions requiring operative management.

Irrigation versus suction alone during laparoscopic appendectomy for perforated appendicitis: a prospective randomized trial.

BACKGROUND: The efficacy of irrigating the peritoneal cavity during appendectomy for perforated appendicitis has been debated extensively. To date, prospective comparative data are lacking. Therefore, we conducted a prospective, randomized trial comparing peritoneal irrigation to suction alone during laparoscopic appendectomy in children. METHODS: Children younger than 18 years with perforated appendicitis were randomized to peritoneal irrigation with a minimum of 500 mL normal saline, or suction only during laparoscopic appendectomy. Perforation was defined as a hole in the appendix or fecalith in the abdomen. The primary outcome variable was postoperative abscess. Using a power of 0.8 and alpha of 0.05, a sample size of 220 patients was calculated. A battery-powered laparoscopic suction/irrigator was used in all cases. Pre- and postoperative management was controlled. Data were analyzed on an intention-to-treat basis. RESULTS: A total of 220 patients were enrolled between December 2008 and July 2011. There were no differences in patient characteristics at presentation. There was no difference in abscess rate, which was 19.1% with suction only and 18.3% with irrigation (P = 1.0). Duration of hospitalization was 5.5 ± 3.0 with suction only and 5.4 ± 2.7 days with group (P = 0.93). Mean hospital charges was $48.1K in both groups (P = 0.97). Mean operative time was 38.7 ± 14.9 minutes with suction only and 42.8 ± 16.7 minutes with irrigation (P = 0.056). Irrigation was felt to be necessary in one case (0.9%) randomized to suction only. In the patients who developed an abscess, there was no difference in duration of hospitalization, days of intravenous antibiotics, duration of home health care, or abscess-related charges. CONCLUSIONS: There is no advantage to irrigation of the peritoneal cavity over suction alone during laparoscopic appendectomy for perforated appendicitis. The study was registered with clinicaltrials.gov at the inception of enrollment (NCT00981136).

Interval appendectomy after perforated appendicitis: what are the operative risks and luminal patency rates?

BACKGROUND: The need for interval appendectomy after nonoperative management of a perforated appendicitis is being questioned owing to recent studies that estimated recurrence rates as low as 5% because of obliteration of the appendiceal lumen. We review our experience with interval appendectomy in this subset of patients to determine the postoperative outcomes and luminal patency rates. METHODS: A retrospective review was conducted of all children treated nonoperatively for a perforated appendicitis followed by elective interval appendectomy during the past 10 years. The data collected included initial hospitalization, convalescence period, perioperative course, and luminal patency rates. RESULTS: A total of 128 patients were identified, of whom 55% were male. Their mean ± SD age was 9.1 ± 4.2 years. The mean interval from the initial presentation to appendectomy was 65.9 ± 20.3 d. All but 2 of the patients underwent laparoscopic appendectomy with 3 conversions to open surgery. The mean operative time was 43.6 ± 19.2 min. The complication rate was 9%, including 1 postoperative abscess, 1 reoperation for bleeding, and 1 readmission for Clostridium difficile infection. Six patients had a superficial wound infection, and 2 patients underwent outpatient procedures for suture granuloma. No risk factors for complications were identified. Of the specimens, 16% had obliterated lumens. CONCLUSIONS: Major postoperative morbidity for interval appendectomy after a perforated appendicitis is low and should not be a deterrent in offering interval appendectomy to this subset of patients.

Pediatric dermatofibrosarcoma protuberans: multi-institutional outcomes.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) in children is uncommon. METHODS: Retrospective review of patients ≤ 18 y with histopathologic diagnosis of DFSP from 1980-2010 treated at one of two tertiary referral centers. RESULTS: Fifteen patients were treated for pathologically-confirmed DFSP (mean ± SE age 11.9 ± 1.3 y). There was a female preponderance: 12 females, 3 males. Follow-up was available in all patients over a median 5.8 y (range 0.2-20.1 y). Patients presented with subcutaneous nodule (n = 4), cutaneous plaque (n = 3), or red papule (n = 3); the description of the lesion was not available for five patients. Abdominal wall, lower extremity, and scalp were the most frequent sites involved (n = 4 each). Median time from onset of the lesion to pathologic diagnosis was 182 d (range 5-1, 114 d). All 15 patients underwent initial excisional biopsy, and the diagnosis of DFSP was made in 14. Thirteen patients then underwent re-excision (within a mean ± SE 20.4 ± 4.1 d) with negative margins. None of these patients had a recurrence. There were two recurrences: one with positive margins on excisional biopsy who did not have immediate re-excision; the other with a misdiagnosis on excisional biopsy. Both underwent re-excision with negative margins and have not had any further recurrence. No patients received adjuvant therapy. There were no disease-related deaths. CONCLUSIONS: DFSP is a rare pediatric malignancy. Its presentation is variable, delaying diagnosis, which is made with excisional biopsy. Excision with clear margins is critical for cure.

The predictive value of Haller index in patients undergoing pectus bar repair for pectus excavatum.

BACKGROUND: The Haller index (HI) remains the standard metric to quantify the severity of pectus excavatum deformity. However, little data exist to determine how well this parameter correlates with the difficulty or early outcomes of repair. METHODS: The study population was comprised of all patients who underwent pectus bar repair for pectus excavatum on whom adequate preoperative images on computed tomography allowed for Haller index calculation, from December 1999 to February 2010. Patients with two bars placed for repair were excluded. All images were reviewed blinded to outcome and Haller index was calculated. Pearson's correlation was used to evaluate the relationship between age, length of operation, postoperative complications, and length of hospitalization. The correlations were performed on the entire population and then individual age groups analyzed: 5-11 y, 12-16 y, and over 17 y. Two-tailed P values were determined from the correlation coefficient and significance was defined as P ≤ 0.05. RESULTS: HI was available for 262 patients. There were 66 patients aged 5-11 y, 165 patients aged 12-16 years, and 30 patients over 17 y. The population was 80% male. In the entire population, there was a small correlation between postoperative pneumothorax and HI (R = 0.131, P = 0.05). There was no correlation between age, operative time, postoperative bar infection, or length of stay. No significant correlations existed in any of the individual age groups. CONCLUSION: The Haller index holds no correlation with age, operative time, postoperative bar infection, or length of stay.

Absence of evidence of translocation of GLUT2 to the apical membrane of enterocytes in everted intestinal sleeves.

INTRODUCTION: Traditional models of intestinal glucose absorption confine GLUT2 to the basolateral membrane. Evidence suggests that GLUT2 is translocated to the apical membrane when the enterocyte is exposed to high luminal glucose concentrations. HYPOTHESIS: GLUT2 translocates to the apical membrane by a PKC signaling mechanism dependent on activity of SGLT1 and the cellular cytostructure. METHODS: Transporter-mediated glucose uptake was studied in rat jejunum using everted sleeves under seven conditions: Control, SGLT1 inhibition (phlorizin), GLUT2 inhibition (phloretin), both SGLT1 and GLUT2 inhibition, PKC inhibition (calphostin C or chelerythrine), and disruption of cellular cytostructure (nocodazole). Each condition was tested in iso-osmotic solutions of 1, 20, or 50 mM glucose for 1 or 5 min incubations (n = 6 rats each). RESULTS: Control rats exhibited a saturable pattern of uptake at both durations of incubation. Phlorizin (P ≤ 0.006 each) inhibited markedly and phloretin (P ≤ 0.01 each) inhibited partially glucose uptake in all concentrations and time. Phloretin and phlorizin together completely inhibited uptake (P = 0.004 each). Calphostin C, chelerythrine, and nocodazole had little effect on glucose uptake at either 1 or 5 min. Inhibition of SGLT1 led to near complete cessation of transporter-mediated glucose uptake, while GLUT2 inhibition led to partial inhibition, suggesting some constitutive expression of GLUT2 in the apical membrane. Disruption of PKC signaling or cytoskeletal integrity partially inhibited transporter-mediated glucose uptake only in 1 mM glucose, suggesting a non-specific effect. CONCLUSIONS: Under these conditions, it does not appear that GLUT2 is translocated to the apical membrane on the cellular cytostructure in response to PKC signaling.

Treatment of appendicitis in neutropenic children.

BACKGROUND: Appendicitis in the neutropenic patient places the clinician in a precarious position; balancing the timing and risks of surgery and the risk of an uncontrolled infectious source in the abdomen. METHODS: Multi-center retrospective review from 2000 to 2010 of appendicitis occurring in patients with neutropenia secondary to chemotherapy. Patient demographics and surgical outcomes were tracked. RESULTS: There were 11 patients, mean age of 11 y (3-17 y); six were male. Mean weight was 46.9 kg (18.1-72.6 kg). Mean body mass index was 20.9 kg/m(2) (16.8-27.3 kg/m(2)). There were five acute lymphocytic leukemias, four acute myeloblastic leukemias, one T-Cell lymphoma, and one Ewing's sarcoma. Mean presenting white blood cell count was 1900 (0.2-4.4). Average absolute neutrophil count was 900 (0.00-2.6). Computed tomography scan was used in all patients. Appendectomy was performed within 24 h of presentation in all patients, three were perforated. Mean time to first feeding was 1 d (range, 0-5 d), goal feeds at 3 d (range, 1-6 d ). Mean length of stay from appendicitis was 4 d. CONCLUSION: Early appendectomy for appendicitis in neutropenic patients appears to be tolerated well with a low risk of surgical complications.

Diagnosis and management of pediatric endocrine neoplasms.

PURPOSE OF REVIEW: To guide the clinician in the diagnostic evaluation of endocrine neoplasms, to distinguish between benign and malignant and determine when surgical referral is indicated. RECENT FINDINGS: Thyroid nodules are uncommon but malignant in as many as 27% of patients. Fine needle aspiration should be considered in adolescents, in which accuracy is as high as 90%; surgical resection should be undertaken in all preadolescents (<13 years) with a thyroid nodule. Prognosis for most primary thyroid malignancies is favorable. Primary hyperparathyroidism is rare and due to an adenoma in up to 70% of patients. Surgical resection carries a cure rate of 95% with the use of intraoperative parathyroid hormone assays. Adrenal neoplasms cover a wide spectrum of disorder. They are functional in 95% of patients and require a thorough diagnostic evaluation prior to surgical resection. Malignant lesions of the adrenal gland carry a poor prognosis when complete surgical resection cannot be achieved. Carcinoids are rare neuroendocrine neoplasms, primarily of the appendix, associated with carcinoid syndrome in 10% of patients. The indolent course warrants aggressive surgical control. SUMMARY: Endocrine neoplasms are unusual in the pediatric population. Their presence should raise concern about a multiple endocrine neoplasia syndrome and appropriate diagnostic and endocrine work-up. Most neoplasms will require surgical resection.

Post-ERCP pancreatitis in pediatric patients.

OBJECTIVES: Pancreatitis is a known complication of endoscopic retrograde cholangiopancreatography (ERCP). Our aim was to assess the prevalence and severity of ERCP-associated pancreatitis using established criteria. MATERIALS AND METHODS: Retrospective review of patients younger than 18 years undergoing ERCP complicated by post-ERCP pancreatitis defined by the 1991 consensus statement. Patients with chronic pancreatitis were studied separately using modified criteria. Risk factors for post-ERCP pancreatitis were analyzed. RESULTS: Three hundred forty-three ERCPs were performed in 224 patients. Two hundred seventy-six ERCPs were performed in patients without chronic pancreatitis, 7 of which were complicated by post-ERCP pancreatitis (prevalence 2.5%). Patients undergoing diagnostic-only ERCP were less likely to develop post-ERCP pancreatitis (P<0.01). Sixty-seven procedures were performed on patients with chronic pancreatitis; 10 developed postprocedure pain requiring or prolonging hospitalization (prevalence 14.9%). Pancreatic duct stenting was a risk factor for post-ERCP pain in this subset of patients (P=0.02). CONCLUSIONS: The prevalence of post-ERCP pancreatitis is low-2.5% excluding patients with chronic pancreatitis and 4.96% overall. Therapeutic procedures and the presence of chronic pancreatitis are risk factors for post-ERCP pancreatitis.

Perspectives on pediatric bariatric surgery: identifying barriers to referral.

BACKGROUND: Pediatric obesity is a growing problem affecting the health of our youth. We sought to identify the barriers to pediatric bariatric referral at a tertiary referral center. METHODS: We performed a survey of pediatricians and family practitioners at a single institution to assess their perspectives on pediatric obesity. RESULTS: A total of 61 physicians completed the survey (response rate 46%). All believed pediatric obesity is a major problem, and 82.0% noted an increase in the incidence during a mean period of 15 years (range 3-25). Of the 61 physicians, 88.5% used nonoperative weight loss techniques, with only 1.8% reporting satisfactory results. However, 42.6% had referred a patient (adult or pediatric) for a bariatric procedure, of whom 84.6% were satisfied with the operative outcomes. Despite the high satisfaction with bariatric procedures, 88.5% would be unlikely or would never refer a child for a bariatric procedure, and 44.3% would be somewhat or very likely to refer an adolescent. CONCLUSION: Physicians caring for children recognize the growing problem of childhood and adolescent obesity. Despite the poor outcomes with nonoperative methods and the high satisfaction with the outcomes of bariatric procedures, physicians are still reluctant to refer children and adolescents for surgical weight loss procedures.

Pancreaticobiliary and duodenal perforations after periampullary endoscopic procedures: diagnosis and management.

OBJECTIVE: To review our experience with management of pancreaticobiliary and duodenal (PB/D) perforations after periampullary endoscopic interventions. Although pancreaticobiliary and duodenal perforations after periampullary endoscopic procedures are rare, their management has not been well described. PATIENTS: Individuals who experienced pancreaticobiliary and duodenal perforations. MAIN OUTCOME MEASURES: Comorbidities, interventions performed, mechanism/site of perforation, management, and hospital morbidity/mortality. RESULTS: Seventy-five perforations (0.6%) occurred in 12,427 procedures; 20 perforations (27%) occurred during biliary stricture dilatation, 18 (24%) during diagnostic endoscopic retrograde cholangiopancreatography, and 15 (20%) during management of choledocholithiasis. Perforations were caused by guidewire insertion in 24 patients (32%), sphincterotomy in 11 (15%), passage of the endoscope in 8 (11%), or stent migration in 7 (9%) and were identified during the index procedure in 45 patients (60%). Delayed presentations included pain in 33 patients (44%), leukocytosis in 26 (35%), and/or fever in 13 (17%) and were diagnosed using computed tomography in 19 patients (25%) and abdominal radiography in 10 (13%); 9 cases (12%) were diagnosed more than 24 hours after the procedure. Indications for operative treatment were gaping duodenal perforations and perforations in patients with surgically altered anatomy. Indications for nonoperative management included contained bile duct perforations and focal duodenal perforations. Management was nonoperative in 53 patients (71%) and operative in 22 (29%). Patients with duodenal perforations, higher American Society of Anesthesia status (P<.01 each), and older age (mean +/- SEM, 65 +/- 4 vs 55 +/- 2 years; P = .02) were more likely to require operative management. Hospital stay (mean +/- SEM, 16 +/- 4 vs 4 +/- 1 days; P<.05) and mortality (13% vs 4%; P<.05) were greater in operative patients (P<.05 each). CONCLUSIONS: Most (70%) pancreaticobiliary and duodenal perforations secondary to periampullary endoscopic interventions can be managed nonoperatively. Most biliary perforations can be managed nonoperatively; a requirement for operative treatment increases the mortality rate.

Long-term effects of extrinsic denervation on VIP and substance P innervation in circular muscle of rat jejunum.

Intestinal denervation contributes to enteric motor dysfunction after small bowel transplantation (SBT). Our aim was to determine long-term effects of extrinsic denervation on function of nonadrenergic, noncholinergic innervation with substance P and vasoactive intestinal polypeptide (VIP). Contractile activity of jejunal circular muscle strips from six age-matched, naive control rats (NC) and eight rats 1 year after syngeneic SBT was studied in tissue chambers. Spontaneous contractile activity did not differ between groups. Exogenous VIP inhibited contractile activity dose-dependently to a comparable degree in both groups. The VIP antagonist ([D-p-Cl-Phe(6),Leu(17)]-VIP) and the nitric oxide synthase inhibitor L-NG-nitro-arginine did not affect VIP-induced inhibition but increased contractile activity during electrical field stimulation (EFS) in both groups. Exogenous substance P increased contractile activity dose-dependently, greater in NC than SBT. The substance P antagonist ([D-Pro(2),D-Trp(7,9)]-substance P) inhibited effects of exogenous substance P and decreased the excitatory EFS response. Immunohistofluorescence showed tyrosine hydroxylase staining after SBT indicating sympathetic reinnervation. In jejunal circular muscle after chronic denervation, response to exogenous substance P, but not VIP, is decreased, whereas endogenous release of both neurotransmitters is preserved. Alterations in balance of excitatory and inhibitory pathways occur despite extrinsic reinnervation and might contribute to enteric motor dysfunction after SBT.

Magnetic Mini-Mover Procedure for pectus excavatum IV: FDA sponsored multicenter trial.

PURPOSE: The Magnetic Mini-Mover Procedure (3MP) is a minimally invasive treatment for prepubertal patients with pectus excavatum. This multicenter trial sought to supplement safety and efficacy data from an earlier pilot trial. METHODS: Fifteen patients with pectus excavatum had a titanium-enclosed magnet implanted on the sternum. Externally, patients wore a custom-fitted magnetic brace. Patients were monitored closely for safety. Efficacy was determined by the Haller Index (HI) and satisfaction surveys. After 2 years, the implant was removed. RESULTS: Mean patient age was 12 years (range 8-14), and mean pretreatment HI was 4.7 (range 3.6-7.4). The device was successfully implanted in all patients. Mean treatment duration was 25 months (range 18-33). Posttreatment chest imaging in 13 patients indicated that HI decreased in 5, remained stable in 2, and increased in 6. Seven out of 15 patients had breakage of the implant's titanium cables because of fatigue fracture. Eight out of 13 patients were satisfied with their chest after treatment. CONCLUSION: The 3MP is a safe, minimally invasive, outpatient treatment for prepubertal patients with pectus excavatum. However, the magnetic implant design led to frequent device breakage, confounding analysis. The HI indicated mixed efficacy, although surveys indicated most patients perceived a benefit. STUDY TYPE/LEVEL OF EVIDENCE: Case series, treatment study. Level IV.

Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease.

OBJECTIVE: To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. BACKGROUND: H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications. METHODS: A multicenter retrospective review of all H-type TEF patients treated at 14 tertiary children's hospital from 2002-2012 was performed. Data were systematically collected concerning associated anomalies, operative techniques, hospital course, and short and long-term outcomes. Descriptive analyses were performed. RESULTS: We identified 102 patients (median 9.5 per center, range 1-16) with H-type TEF. The overall survival was 97%. Most patients were repaired via the cervical approach (96%). The in-hospital complication rate, excluding vocal cord issues, was 16%; this included an 8% post-operative leak rate. Twenty-two percent failed initial extubation after repair. A total of 22% of the entire group had vocal cord abnormalities (paralysis or paresis) on laryngoscopy that were likely because of recurrent laryngeal nerve injury. Nine percent required a tracheostomy. Only 3% had a recurrent fistula, all of which were treated with reoperation. CONCLUSIONS: There is a high rate of recurrent laryngeal nerve injury after H-type TEF repair. This underscores the need for meticulous surgical technique at the initial repair and suggests that early vocal cord evaluation should be performed for any post-operative respiratory difficulty. Routine evaluation of vocal cord function after H-type TEF repair should be considered. THE LEVEL OF EVIDENCE RATING: Level IV.

Inflammatory myofibroblastic tumors in children.

BACKGROUND: Inflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children. METHODS: Retrospective review from 1993 to 2014 of patients ≤18years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers. RESULTS: Thirty-two patients were diagnosed with IMFT. Mean (±SD) age was 9.3±5.7years at diagnosis. Tumor location was variable: abdomen/pelvis (28%), head/neck region (22%), intrathoracic (22%), genitourinary (9%), bowel (6%) liver (6%), and musculoskeletal (6%). Median follow-up was 2.6±4.6years, with 3 recurrences and 2 deaths, which occurred only after recurrence. Positive microscopic margin after resection was associated with recurrence, compared to those that had a negative margin (40% vs. 0%, p=0.04). Recurrence was associated with increased mortality (67% vs 0%, p=0.01). Time from first symptoms to resection was shorter in those with recurrence (25.8±22 vs. 179±275days, p=0.01) and in nonsurvivors (44.0±8.0 vs. 194.3±53.4days, p=0.02). Adjuvant chemotherapy, not including steroid monotherapy, either given before or after resection, was administered more often to nonsurvivors (100% vs 4%, p=0.009), and use of corticosteroids was also higher in the nonsurvivors (100% vs. 15%, p=0.04). CONCLUSIONS: IMFT is a rare pediatric neoplasm with variable locations. Complete excision is critical for cure. Proposed guidelines for diagnosis, treatment and surveillance of theses tumors in children are reported.