Development and Validation of a Pediatric MRI-Based Perianal Crohn Disease (PEMPAC) Index-A Report from the ImageKids Study.

BACKGROUND: As part of the prospective multicenter ImageKids study, we aimed to develop and validate the pediatric MRI-based perianal Crohn disease (PEMPAC) index. METHODS: Children with Crohn disease with any clinical perianal findings underwent pelvic magnetic resonance imaging at 21 sites globally. The site radiologist and 2 central radiologists provided a radiologist global assessment (RGA) on a 100 mm visual analog scale and scored the items selected by a Delphi group of 35 international radiologists and a review of the literature. Two weighted multivariable statistical models were constructed against the RGA. RESULTS: Eighty children underwent 95 pelvic magnetic resonance imaging scans; 64 were used for derivation and 31 for validation. The following items were included: fistula number, location, length and T2 hyperintensity; abscesses; rectal wall involvement; and fistula branching. The last 2 items had negative beta scores and thus were excluded in a contending basic model. In the validation cohort, the full and the basic models had the same strong correlation with the RGA (r = 0.75; P < 0.01) and with the adult Van Assche index (VAI; r = 0.93 and 0.92; P < 0.001). The correlation of the VAI with the RGA was similar (r = 0.77; P < 0.01). The 2 models and the VAI had a similar ability to differentiate remission from active disease (area under the receiver operating characteristic curve, 0.91-0.94). The PEMPAC index had good responsiveness to change (area under the receiver operating characteristic curve, 0.89; 95% confidence interval, 0.69-1.00). CONCLUSIONS: Using a blended judgmental and mathematical approach, we developed and validated an index for quantifying the severity of perianal disease in children with CD. The adult VAI may also be used with confidence in children.

The burden of esophageal dilatations following repair of esophageal atresia.

AIM: To describe the burden of esophageal dilatations in patients following esophageal atresia (EA) repair. METHOD: A retrospective review was performed at The Royal Children's Hospital, Melbourne, of all neonates undergoing operative repair for EA over a 17-year period (1999-2015). Stricture was defined by radiological and/or intra-operative findings of narrowing at the esophageal anastomosis. Data recorded included EA type, perinatal details, operative approach, esophageal anastomosis outcome, dilatation requirement, and survival. Key endpoints were anastomotic leakage and tension, esophageal dilatation technique, dilatation frequency, fundoplication, and complications. RESULTS: During the study period, 287 newborn EA patients were admitted, of which 258 underwent operative repair and survived to primary discharge. Excluding 11 patients with isolated tracheoesophageal fistula, 247 patients were included in the final analysis. Intra-operative anastomotic tension was documented in 41/247 (16.6%), anastomotic leak occurred in 48/247 (19.4%), and fundoplication was performed in 37/247 (15.0%). Dilatations were performed in 149/247 (60.3%). Techniques included bougie-alone (92/149, 61.7%), combination of bougie and balloon (51/149, 34.2%), and balloon-alone (6/149, 4.0%). These patients underwent 1128 dilatations; median number of dilatations per patient was 4 (interquartile range 2-8). Long-gap EA and anastomotic tension were risk factors (p < 0.01) for multiple dilatations. Complications occurred in 13/1128 (1.2%) dilatation episodes: 11/13 esophageal perforation, 2/13 clinically significant aspiration. Perforations were rare events in both balloon (6/287, 2.1%) and bougie dilatations (4/841, 0.5%); one patient had a perforation from guidewire insertion. CONCLUSIONS: Esophageal dilatation occurred in a majority of EA patients. Long-gap EA was associated with an increased burden of esophageal dilatation. Perforations were rare events in balloon and bougie dilatations. TYPE OF STUDY: Original article - retrospective review. LEVEL OF EVIDENCE: II.

Skull base anatomy and surgical safety in isolated and CHARGE-associated bilateral choanal atresia.

INTRODUCTION: Bilateral choanal atresia (BCA) is associated with a high incidence of congenital abnormalities that include skull base anomalies and defects. Surgical repair of BCA is necessary in the early neonatal period and any altered anatomy of the adjacent skull base will heighten the risk of intracranial injury. This risk may be further increased in patients with CHARGE syndrome. OBJECTIVES: To measure surgically relevant nasal and skull base dimensions in neonates with BCA in order to determine whether any difference exists between isolated and CHARGE syndrome associated subgroups, thereby optimizing the safety of surgical repair. METHODS: A retrospective review of medical charts and computed tomography was undertaken at a tertiary pediatric hospital of all neonates diagnosed with BCA between 2004 and 2016. Isolated and CHARGE syndrome subgroups of BCA were identified from clinical records and CT data was analyzed and compared between the two. The skull base parameters measured were choanal width, choanal height, mid-nasal skull base height and skull base slope. RESULTS: Of the 13 patients included, 3 had CHARGE syndrome and 10 had isolated BCA. Whilst the difference in mid-nasal height approached significance for the two groups, numbers were too small for a statistical difference to be identified. The mean value for choanal width in the isolated BCA group was significantly less the largest series of normative data available in the literature for comparison (p < 0.001). No skull base anomalies were noted in either group. CONCLUSION: While this is a small study with limited numbers, it is the first that has attempted to identify and measure the posterior nasal and skull base anatomy most pertinent to avoiding skull base injury in the surgical management of BCA.

Splenic cyst and its management in a 21-month-old boy: a rare complication of invasive meningococcal disease.

Splenic complications of invasive meningococcal disease (IMD) are well recognised, though cyst formation is rare, particularly in paediatric populations. The best approach to their management is not yet established. This case outlines the management of a splenic cyst in a 21-month-old boy following severe IMD. The case took place in the context of an acute emergence of serogroup W prompting significant media attention and subsequent change in vaccination practice at a jurisdictional level in Australia. The patient was critically unwell early in the illness, then later a collection in the left upper quadrant was detected, shown on ultrasound to be a 11.6×7.7 cm splenic cyst. In this case, the cyst was managed by ultrasound-guided drainage tube insertion. The residual collection was small and stable on subsequent imaging.

Magnetic resonance imaging findings in children with tinnitus.

OBJECTIVE: Tinnitus in adults is generally investigated by contrast-enhanced magnetic resonance imaging (MRI) to rule out the diagnosis of acoustic neuroma. Acoustic neuroma is rare in children and, therefore, the role of MRI in children with tinnitus is unclear. This study was undertaken to determine the value of MRI in the investigation of tinnitus in children. METHODS: Retrospective study of children younger than 18 years who underwent MRI for the investigation of tinnitus over a 10-year period. RESULTS: Sixty-five patients were identified, but there were only 34 who had also undergone audiologic assessment. Among the 25 patients with normal audiology, MRI abnormalities were present in 9, but these were all thought to be nonspecific. Nine patients had abnormal audiograms and the MRI was abnormal in 4 of these cases, which included 3 children who were found to have multiple sclerosis. CONCLUSION: Magnetic resonance imaging would appear to be mandatory in the investigation of tinnitus in children who are found to have sensorineural hearing loss, particularly to rule out the diagnosis of multiple sclerosis. Although our study does not support the routine use of MRI in children with normal audiology, the numbers in our series are too small for a conclusive recommendation.

Paediatric MRCP: 10 year experience with 195 patients.

PURPOSE: Magnetic resonance cholangiopancreatography (MRCP) has now largely substituted endoscopic retrograde cholangiopancreatography (ERCP) in evaluating the biliary tree in adults giving its high sensitivity and specificity. Whilst smaller series published to date suggests this may be extrapolated to the paediatric population, its routine use in children is still debatable. The aim of our study is therefore to review the utility of MRCP in a large series of paediatric patients. METHODS AND MATERIALS: All MRCPs performed in our institution were examined for diagnostic quality, spectrum of diagnoses and correlation with direct cholangiography (DC) were available. Correlation with histopathology results and final clinical diagnosis was made in the subset of patients in whom a MRCP had been performed to evaluate the presence of primary sclerosing cholangitis (PSC). RESULTS: There were 245 MRCP examinations performed on 195 patients and 219 were diagnostic. There was 100% MRCP and DC concordance in the 16 cases where both had been performed. MRCP yielded a sensitivity of 89% in the subset of patients with PSC. CONCLUSION: MRCP was a valuable diagnostic tool in our paediatric population. Image quality is sufficiently diagnostic and shows good correlation with clinical diagnosis in conditions encountered in our population, including primary sclerosing cholangitis, post liver transplant biliary strictures, post surgical complications, dilated common bile ducts, choledochal cysts, cholelithiasis and choledocholithaisis.

Fate, complications and MRI implications of retention anchor suture placed during gastrostomy in children.

Retrograde radiologic gastrostomy is one of several techniques used for placing a gastrostomy and is a common technique used in children. The use of a retention anchor suture (RAS) is an important component of this procedure. This pictorial essay explores the normal course and passage of the RAS, as well as abnormal migration, various complications and the implications of the RAS with regard to MRI safety.

CT head in children.

The advances in computerized technology (CT) technique over the last few decades have greatly modified imaging protocols in children. The range of pathologies that can now be demonstrated has broadened with the advent of newer techniques such as CT perfusion and the ability to perform complex reconstructions. Increasing speed of scanning and reduction in scan time have influenced the need for sedation and general anaesthetic as well as impacting on motion artefact. Additionally, concerns about radiation safety and avoidance of unnecessary radiation have further impacted on the inclusion of CT in the imaging armamentarium. Justification and image optimisation are essential. It is important to familiarize oneself with the appearances of normal variants or age related developmental changes. CT does however remain an appropriate investigation in a number of conditions.

Imaging assessment of congenital and acquired abnormalities of the portal venous system.

Abnormalities of the portal venous system are a heterogeneous group of conditions that can cause substantial morbidity and mortality and may lead to complications during surgery or percutaneous interventions involving the portal venous system. High-resolution computed tomography, ultrasonography, and magnetic resonance imaging permit a comprehensive, noninvasive evaluation of the portal venous system, enabling the detection of both structural and functional abnormalities. However, an understanding of the embryologic development of the normal portal venous anatomy and anatomic variants is essential to accurately interpret the imaging findings. Knowledge of the characteristic appearances of abnormalities of the portal venous system allows a more confident diagnosis, permitting timely treatment and more informed guidance of surgical procedures and percutaneous interventions, which may lead to an improved outcome.

Polyflex stenting of tracheomalacia after surgery for congenital tracheal stenosis.

Polyflex self-expanding stents (Rüsch, Germany) were used in three young children who had presented with life-threatening long-segment tracheal stenosis with bronchial stenosis in two cases. Two children had slide tracheoplasties and subsequently aortic homografts and another tracheal resection and autotracheoplasty. However, in all cases persistent lower tracheal malacia necessitated stenting. Complications of granuloma, stent migration or dislodgement occurred in all cases. A fatal tracheo-aortic fistula occurred in one child. Granuloma in one was treated successfully with steroids. One child survives.

Ultrasound diagnosis of an internal carotid artery pseudoaneurysm in a young child.

Pseudoaneurysm of the carotid artery is a rare complication of deep neck space infection. It carries a high mortality rate even when treated appropriately. We report a case of a pseudoaneurysm of the internal carotid artery in a 2-year-old child who had a neck infection without an associated abscess. The diagnosis was not obvious on MRI but was confirmed by ultrasonography. This was successfully managed by angiographic embolization, followed by antibiotic and anticoagulant treatment. The combination of MRI and ultrasonography, without the use of invasive or radiation techniques, was sufficient for the diagnosis of pseudoaneurysm of the internal carotid artery.

Are localized intestinal perforations distinct from necrotizing enterocolitis?

BACKGROUND/PURPOSE: Localized intestinal perforation (LP) is thought to be a distinct entity when compared with perforation associated with necrotizing enterocolitis (NEC). Studies have indicated that LP is more amenable to percutaneous drainage and associated with a better outcome. We sought to determine whether LP and NEC could be distinguished based on clinical parameters alone. METHODS: A retrospective review of 40 neonates with gastrointestinal perforations between January 1990 and May 1998 was performed. All had undergone laparotomy and had histologic specimens available for evaluation. RESULTS: Twenty-one neonates had necrotizing enterocolitis (NEC), and 19 had localized perforation (LP) based on histologic criteria. More neonates with LP were exposed to prenatal indomethacin (37% v 5%; P <.05), received intravenous dexamethasone (42% v 10%; P <.05), had umbilical artery catheters (63% v 14%; P <.05), and had a higher white blood cell (WBC) count (27.1 +/- 23.1 v 14.3 +/- 11.5; P <.05). More neonates with NEC had pneumatosis intestinalis (47% v 11%; P <.05). No significant differences existed in enteral feeding (16% LP v 38% NEC) or overall mortality rate (37% LP v 38% NEC). No statistical differences in the timing of perforation or clinical presentation were found. CONCLUSIONS: NEC and LP are difficult to distinguish based on clinical parameters alone. The authors did find associations between LP and prenatal indomethacin, intravenous dexamethasone, umbilical artery catheters, and a higher WBC count. Mortality rate and clinical outcome were nearly identical in both groups. Pneumatosis intestinalis, thought to be pathognomonic for NEC, was seen on abdominal radiograph in 2 babies with histologically proven LP.