Introduction of a novel magnetic resonance imaging-based scoring system for assessing disease activity in children with juvenile dermatomyositis.

Objectives: We aimed to develop and assess the reliability of a novel MRI-based scoring system for reporting the severity of MRI findings in children with suspected JDM. Methods: Nine consultant paediatric radiologists independently assessed and scored 40 axial and 30 coronal thigh MR images of children with suspected JDM on two occasions using the juvenile dermatomyositis magnetic resonance Imaging Score (JIS). JIS was calculated for both reads for each plane and each limb, with possible scores ranging from 0 (normal) to 100 (severe). Inter- and intraobserver agreement was calculated using the intraclass correlation coefficient (ICC) and two- and one-way random effects models, respectively. Bland-Altman plots of the difference in JIS against the average JIS were also produced for each rater. Results: Overall, the interobserver reliability and agreement was good-for axial images, JIS ranged from 46.8 to 61.0 [ICC = 0.88 (95% CI: 0.82, 0.92)] for the left limb and 47.9-61.4 [ICC = 0.87 (95% CI: 0.81, 0.92)] for the right limb. For coronal images, JIS ranged from 56.7 to 65.1 [ICC = 0.90 (95% CI: 0.85, 0.95)] for the left limb and 55.7 to 66.8 [ICC = 0.90 (95% CI: 0.84, 0.94)] for the right limb. The intraobserver reliability and agreement was good, with ICC ranging from 0.90 to 0.94. Conclusion: JIS is a semi-objective scoring system with potential to serve as a reliable biomarker of disease severity and response to therapeutic interventions in children with JDM.

Fluoroscopic balloon dilation of esophageal atresia anastomotic strictures in children and young adults: single-center study of 103 consecutive patients from 1999 to 2011.

PURPOSE: To determine whether fluoroscopic balloon dilation (FBD) is a safe and effective method of treating esophageal anastomotic stricture after surgical repair in an unselected patient population. MATERIALS AND METHODS: With ethics committee approval, records for 103 consecutive patients who underwent FBD with our interventional radiology service (1999-2011) were reviewed retrospectively. Patients underwent diagnostic contrast material-enhanced study prior to the first dilation. Dilations were performed by using general anesthesia. Outcomes were number and/or frequency of dilations, clinical effectiveness and response to dilations, esophageal perforation, requirement for surgery, and mortality. Data were expressed as mean ± standard deviation (with range). Comparisons were conducted by using the Fisher exact test and log-rank test. The significance level was set at P < .05. RESULTS: One hundred three patients (61 male patients, 59%) underwent 378 FBD sessions (median, two dilations per patient; range, 1-40 dilations). The median age at first FBD was 2.2 years (range, 0.1-19.5 years). The balloon catheter diameters ranged from 4 to 20 mm. FBD was successful in 93 patients (90%): 44 (47%) after single dilation and 49 (53%) after multiple dilations. There was no difference in the proportion of patients who required one dilation and were younger than 1 year versus those who were 1 year of age and older (P > .99; odds ratio, 1.07 [range, 0.43-2.66]). Ten patients (10%) required further procedures: Three underwent stent placement, three underwent esophageal stricture resection, and four underwent esophageal reconstruction. Four esophageal perforations (1%) developed after FBD. Antireflux surgery was performed in 18 patients (17%). There were no deaths. CONCLUSION: FBD for anastomotic strictures after esophageal atresia repair is feasible and acceptably safe and provides relief of symptoms in most patients (90%); however, about half require more than one dilation, and surgery is best predicted if more than 10 dilations are required.

Hypercapnia and acidosis during open and thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia: results of a pilot randomized controlled trial.

OBJECTIVE: We aimed to evaluate the effect of thoracoscopy in neonates on intraoperative arterial blood gases, compared with open surgery. BACKGROUND: Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TEF) can be repaired thoracoscopically, but this may cause hypercapnia and acidosis, which are potentially harmful. METHODS: This was a pilot randomized controlled trial. The target number of 20 neonates (weight > 1.6 kg) were randomized to either open (5 CDH, 5 EA/TEF) or thoracoscopic (5 CDH, 5 EA/TEF) repair. Arterial blood gases were measured every 30 minutes intraoperatively, and compared by multilevel modeling, presented as mean and difference (95% confidence interval) from these predictions. RESULTS: Overall, the intraoperative PaCO2 was 61 mm Hg in open and 83 mm Hg [difference 22 mm Hg (2 to 42); P = 0.036] in thoracoscopy and the pH was 7.24 in open and 7.13 [difference -0.11 (-0.20 to -0.01); P = 0.025] in thoracoscopy. The duration of hypercapnia and acidosis was longer in thoracoscopy compared with that in open. For patients with CDH, thoracoscopy was associated with a significant increase in intraoperative hypercapnia [open 68 mm Hg; thoracoscopy 96 mm Hg; difference 28 mm Hg (8 to 48); P = 0.008] and severe acidosis [open 7.21; thoracoscopy 7.08; difference -0.13 (-0.24 to -0.02); P = 0.018]. No significant difference in PaCO2, pH, or PaO2 was observed in patients undergoing thoracoscopic repair of EA/TEF. CONCLUSIONS: This pilot randomized controlled trial shows that thoracoscopic repair of CDH is associated with prolonged and severe intraoperative hypercapnia and acidosis, compared with open surgery. These findings do not support the use of thoracoscopy with CO2 insufflation and conventional ventilation for the repair of CDH, calling into question the safety of this practice. The effect of thoracoscopy on blood gases during repair of EA/TEF in neonates requires further evaluation. (ClinicalTrials.gov Identifier: NCT01467245).

Balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia: a systematic review.

Surgical repair of oesophageal atresia may result in anastomotic strictures. These strictures are often treated by balloon dilatation (BD) and currently balloon dilatation (fluoroscopic or endoscopic) is the preferred primary treatment method. Here we review the current evidence of the outcomes of balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia. We searched the standard databases (January, 1960-May, 2012) to identify all studies that reported outcomes of balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia in children. Data, reported as median (range), were analysed and compared. Outcomes were success of BD, number of BD sessions, number of oesophageal perforations, need for other surgical interventions and mortality. Five studies were found to be relevant (n = 139; 81 [58%] male children). The total number of dilatation sessions was 401 (2.9 dilatations per child patient). General anaesthesia was used in two (40%) studies; sedation in a further two (40%) studies and one (20%) study used a combination of both. The size of balloon catheter ranged from 4 mm to 22 mm. Seven perforations were reported (1.8% per dilatation session), of which only one (14%) required surgery. No deaths were recorded. Balloon dilatation for anastomotic strictures post-EA repair is safe, and associated with a low perforation and mortality rates. Most perforations are amenable to conservative management.

Internet-based measurement of visual assessment skill of trainee radiologists: developing a sensitive tool.

OBJECTIVE: Expert radiologists exhibit high levels of visual diagnostic accuracy from review of radiological images, doing so after accumulating years of training and experience. To train new radiologists, learning interventions must focus on the development of these skills. By developing a web-based measure of image assessment, a key part of visual diagnosis, we aimed to capture differences in the performance of expert, trainee and non-radiologists. METHODS: 12 consultant paediatric radiologists, 12 radiology registrars, and 39 medical students were recruited to the study. All participants completed a two-part, online task requiring them to visually assess 30 images (25 containing an abnormality) drawn from a library of 150 paediatric skeletal radiographs assessed prior to the study. Participants first identified whether an image contained an abnormality, and then clicked within the image to mark its location. Performance measures of identification accuracy, localisation precision, and task time were collected. RESULTS: Despite the difficulties of web-based testing, large differences in performance, both in terms of the accuracy of abnormality identification and in the precision of abnormality localisation were found between groups, with consultant radiologists the most accurate both at identifying images containing abnormalities (p < 0.001) and at localising abnormalities on the images (p < 0.001). CONCLUSIONS: Our data demonstrate that an online measurement of radiological skill is sufficiently sensitive to detect group level changes in performance consistent with the development of expertise. ADVANCES IN KNOWLEDGE: The developed tool will allow future studies assessing the impact of different training strategies on cognitive performance and diagnostic accuracy.

Clinical negligence claims in pediatric surgery in England: pattern and trends.

AIMS OF THE STUDY: We hypothesized that there has been an increase in the number of successful litigation claims in pediatric surgery in England. Our aim was to report the incidence, causes, and costs of clinical negligence claims against the National Health Service (NHS) in relation to pediatric surgery. MATERIALS AND METHODS: We queried the NHS Litigation Authority (NHSLA) on litigation claims among children undergoing pediatric surgery in England (2004-2012). We decided a priori to only examine closed cases (decision and payment made). Data included year of claim, year of payment of claim, payment per claim, paid-to-closed ratio, and severity of outcome of clinical incident. RESULTS: Out of 112 clinical negligence claims in pediatric surgery, 93 (83%) were finalized-73 (65%) were settled and damages paid to the claimant and 20 (18%) were closed with no payment, and 19 (17%) remain open. The median payment was £13,537 (600-500,000) and median total cost borne by NHSLA was £31,445 (600-730,202). Claims were lodged at a median interval of 2 (0-13) years from time of occurrence with 55 (75%) cases being settled within the 3 years of being received. The commonest reasons for claims were postoperative complications (n=20, 28%), delayed treatment (n=16, 22%), and/or diagnosis (n=14, 19%). Out of 73, 17 (23%) closed claims resulted in case fatality. Conclusion: Two-thirds of all claims in pediatric surgery resulted in payment to claimant, and the commonest reasons for claims were postoperative complications, delayed treatment, and/or diagnosis. Nearly a quarter of successful claims were in cases where negligence resulted in case fatality. Pediatric surgeons should be aware of common diagnostic and treatment shortfalls as high-risk areas of increased susceptibility to clinical negligence claims.

Supraventricular tachycardia precipitated by a peripherally inserted central catheter in an infant with gastroschisis.

Gastroschisis is a common congenital abdominal wall defect requiring surgical correction in early neonatal life. The time to full enteral feeds among infants with gastroschisis is variable. Gastroschisis remains a common surgical cause of intestinal failure in neonates. Peripherally inserted central catheters (PICC) are commonly used to provide long-term venous access for parenteral nutrition during the time enteral feeds are being slowly established. The PICC is placed under fluoroscopic guidance with the distal end of the catheter usually placed at the junction of the superior vena cava with the right atrium. In rare circumstances, the catheter tip may advance into intracardiac chambers, precipitating supraventricular re-entrant tachycardia. We report a case of an infant who was antenatally diagnosed with uncomplicated gastroschisis in whom a supraventricular tachycardia was precipitated by the PICC in the right atrium.

Histologic and immunohistochemical features associated with outcome in neonatal necrotizing enterocolitis.

INTRODUCTION: The aim of the study was to compare histopathologic and immunohistochemical markers between survivors and nonsurvivors of surgical necrotizing enterocolitis (NEC). METHODS: With appropriate ethical approval, archived resection specimens were identified for patients with NEC (Bell Stages II and III) for whom outcome data (survival yes/no) were available. For each specimen, a severely affected part of the bowel and the least affected area, usually the margin, were analyzed. Histologic findings were scored as no necrosis/mucosal necrosis/full-thickness necrosis and immunohistochemistry staining for inflammatory markers vascular cell adhesion protein (VCAM), CD68, CD20, intercellular adhesion molecule (ICAM), human leukocyte antigen (HLA-DR), CD3, Cleaved Caspase-3 (CC3), forkhead box P3 (FOXP3), CD62p, and C4d were performed and scored on a semiquantitative scale (0; no staining to 10, strong extensive staining). All samples were identified by only their study number throughout and the samples were analyzed completely blinded to all clinical information. Data were compared using chi-square test for trend (histologic data) or Mann-Whitney U test. RESULTS: A total of 123 slides from 60 patients (birth weight 1.3 ± 0.1 kg, gestational age at birth 29.3 ± 0.6 weeks) were examined. Seventy-four specimens (60%) were from survivors and 49 specimens (40%) were from those who subsequently died. There was no relationship between histologic severity of necrosis (none/mucosal/full thickness) and mortality (p = 0.58). VCAM (adhesion molecule; p = 0.005) and CC3 (a marker of apoptosis, p = 0.008) expression was significantly elevated in nonsurvivors, whereas there were no differences in CD68, CD20, ICAM, HLA-DR, CD3, FOXP3, CD62p, or C4d expression. CONCLUSIONS: There is a poor relationship between histologic severity of bowel necrosis and patient survival in infants undergoing bowel resection for NEC. There is statistically increased expression of VCAM reflecting severity of systemic inflammatory response and evidence of increased apoptosis in the form of CC3 expression in those who subsequently die, but no histologic features can predict outcome.

Two siblings with unbalanced t(10;21) translocations and non-neuropathic neuropathic bladder.

We describe an unusual case of two siblings, aged 5 years and 5 months, with an unbalanced t(10;21) translocation and concomitant non-neuropathic neuropathic bladder abnormalities, whose father was a known balanced carrier of the translocation, t(10;21). Postnatally both siblings had normal spine and neurological examination. However, ultrasound scans and micturition cystourethrograms revealed grossly distended non-neuropathic neuropathic bladder. In both siblings, the bladder abnormalities responded to clean intermittent catheterisation. On follow-up, dimercaptosuccinic acid scans for both siblings showed equal split function with no scarring.

The outcome of critically ill neonates undergoing laparotomy for necrotising enterocolitis in the neonatal intensive care unit: a 10-year review.

PURPOSE: To evaluate outcomes in critically ill neonates with necrotising enterocolitis (NEC) undergoing a laparotomy in the neonatal intensive care unit (NICU). METHODS: This is a retrospective review of neonates diagnosed with NEC who underwent a laparotomy on NICU between 2001 and 2011. Demographic, diagnostic, operative and outcome data were analysed. Nonparametric comparison was used. Data are reported as median (range). RESULTS: 221 infants with NEC were referred for surgical evaluation; 182 (82%) underwent surgery; 15 (8%) required a laparotomy on NICU. Five had NEC totalis, 4 multifocal disease and 6 focal disease. Five had an open and close laparotomy, 8 stoma with/without bowel resection and 2 bowel resection and primary anastomosis. Ten (67%) died at a median of 6.5-hours (2-72) postoperatively; 2 died at 72 and 264-days. The 30-day mortality rate was higher (p=0.01) among infants undergoing a laparotomy on NICU (10/15; 67%) than in theatre (54/167; 32%). There was no significant difference in mean Paediatric Index of Mortality 2 Scores between survivors and nonsurvivors (p=0.55). Three (20%) infants remain alive with no or minimal disability at 1.4 (0.5-7.5) years. CONCLUSION: Laparotomy for NEC on NICU is a treatment option for neonates who are too unstable to transfer to theatre. However, with 67% dying within 6.5-hours and a further 13% after months in hospital, we must consider whether surgery is always in their best interests. Development of a prediction model to help distinguish those at highest risk of long-term morbidity and mortality could help with decision making in this difficult situation.

What role does laparoscopy play in the diagnosis and immediate treatment of infants with necrotizing enterocolitis?

PURPOSE: Laparotomy is the preferred option for the surgical treatment of necrotizing enterocolitis (NEC); however, laparoscopy may offer benefit, particularly when it is uncertain if the operation is necessary. Our aim was to review the current evidence for the use of diagnostic and therapeutic laparoscopy in NEC. MATERIALS AND METHODS: A systematic review of published literature (1990-July 2012) found in MEDLINE, EMBASE, the Cochrane Library Controlled Trials Register, and the Cumulative Index to Nursing and Allied Health reporting the use of laparoscopy for management of acute NEC was undertaken. RESULTS: Seven studies (six case series and one case report) reporting the use of laparoscopy in 44 infants (weighing 0.5-2.9 kg) with NEC were identified and included for review. There were no randomized controlled trials. Laparoscopic techniques differed among the studies, with standard laparoscopy (gas) used in 28 (64%) cases, fluorescein-aided assessment in 8 cases (18%), and gasless laparoscopy in 8 cases. Eight (18%) infants did not require further surgery following laparoscopy: 4 (9%) had no evidence of NEC, 2 (5%) had no evidence of perforation and/or intestinal gangrene, and 2 (5%) had NEC totalis precluding further surgery. In the remaining 36 (86%) infants in whom further procedures were required following laparoscopy, 9 had placement of a peritoneal drain, 20 had a stoma, and 7 had intestinal anastomosis. Perforation was detected in 25 (57%) infants; however, among the 19 infants who had no detectable perforation at initial laparoscopy, 1 infant (5%) subsequently required laparotomy for a missed perforation. Six (14%) infants died: 2 had care withdrawn due to pan-intestinal NEC, 2 died of ongoing NEC, and 2 of recurrent NEC following recovery from the acute episode. CONCLUSIONS: Laparoscopy is a useful tool in the armamentarium of surgical management of NEC, with one-fifth of patients not requiring further surgery. Laparoscopy can be useful in selected babies with suspected NEC to avoid unnecessary laparotomy and minimize the trauma of surgery.

Polyorchidism: a torted right-sided supernumerary testis.

Polyorchidism is a rare congenital anomaly defined by the presence of more than two histologically proven testes. The commonest variant is triorchidism, the supernumerary testis being commonly reported on the left side. Most cases of polyorchidism are found incidentally in association with undescended testis, hydrocele, hernia or torsion. We report a right-sided triorchidism in a 15-year-old boy found at time of groin exploration for an irreducible right inguinal hernia.

Outcomes of diverting jejunostomy for severe necrotizing enterocolitis.

BACKGROUND/PURPOSE: A diverting jejunostomy without bowel resection is an option for surgical management of extensive necrotizing enterocolitis (NEC). We aimed to determine outcomes of infants who underwent this operation. METHODS: We collected clinical and outcome data on infants undergoing a diverting jejunostomy with no bowel resection as a primary procedure for extensive NEC. Data are median (range). RESULTS: Seventeen neonates underwent a diverting jejunostomy. Eleven (65%) had multifocal disease, whereas 6 (35%) had pan-intestinal involvement. Perforation was seen in 7 (41%), all with multifocal disease. The stoma was placed 12 cm (8-45) from the duodenojejunal flexure. Six infants (35%) died, 4 of these within a day of operation, owing to persisting instability. Intestinal continuity was achieved in all survivors after 52 (17-83) days, and only 1 infant (9%) had a colonic stricture. Seven infants recovered without the need for further intestinal resection distal to the jejunostomy. In those that survived, parenteral nutrition was needed for 2.2 months (1.3-18.0). A single patient had short bowel syndrome. CONCLUSIONS: A diverting jejunostomy is a useful surgical procedure that allows high survival and enteral autonomy in the treatment of extensive NEC. In most patients, the affected intestine recovers without further intestinal resection.

Decreased cerebral oxygen saturation during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia in infants.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TOF) can be repaired thoracoscopically, but this may cause hypercapnia, acidosis, and reduced cerebral oxygenation. We evaluated the effect of thoracoscopy in infants on cerebral oxygen saturation (cSO(2)), arterial blood gases, and carbon dioxide (CO(2)) absorption. METHODS: Eight infants underwent thoracoscopy (6 CDH and 2 EA/TOF). Serial arterial blood gases were taken. Regional cSO(2) was measured using near-infrared spectroscopy. Absorption of insufflated CO(2) was calculated from exhaled (13)CO(2)/(12)CO(2) ratio measured by mass spectrometry. RESULTS: CO(2) absorption increased during thoracoscopy with a maximum 29% ± 6% of exhaled CO(2) originating from the pneumothorax. Paco(2) increased from 9.4 ± 1.3 kPa at the start to 12.4 ± 1.0 intraoperatively and then decreased to 7.6 ± 1.2 kPa at end of operation. Arterial pH decreased from 7.19 ± 0.04 at the start to 7.05 ± 0.04 intraoperatively and then recovered to 7.28 ± 0.06 at end of operation. Cerebral hemoglobin oxygen saturation decreased from 87% ± 4% at the start to 75% ± 5% at end of operation. This had not recovered by 12 (74% ± 4%) or 24 hours (73% ± 3%) postoperatively. CONCLUSIONS: This preliminary study suggests that thoracoscopic repair of CDH and EA/TOF may be associated with acidosis and decreased cSO(2). The effects of these phenomena on future brain development are unknown.