Accuracy of contrast-enhanced voiding urosonography using Optison™ for diagnosis of vesicoureteral reflux in children.

BACKGROUND: There is limited quality of evidence regarding the accuracy of contrast-enhanced voiding urosonography (ceVUS) for diagnosis of vesicoureteral reflux (VUR) compared to fluoroscopic voiding cystourethrography (VCUG), and minimal data on the use of the ultrasound contrast agent Optison™ for this purpose. OBJECTIVE: To compare the accuracy of ceVUS using Optison™ to VCUG, and to assess inter-rater agreement regarding presence and grading of VUR. STUDY DESIGN: In this retrospective investigation, all sequential ceVUS with Optison™ and VCUG studies performed in children between 2014 and 2017 were reviewed. Two raters independently graded all ceVUS studies using a 5-point scale. CeVUS sensitivity and specificity were estimated separately for each rater using the VCUG report as the ground truth for presence and degree of VUR. Logistic and ordinary linear regression models assessed rater-report agreement and inter-rater agreement for each kidney, Optison™ dose, and referral diagnosis. RESULTS: 97 children (51 females) with 101 paired studies were included. Sensitivity and specificity of ceVUS for VUR detection were identical for both raters: right kidney 75%/90.9%; left kidney 85.7%/78.9% (Figure). There was no statistically significant difference in disagreement between raters and the VCUG report for the right or left kidney. Inter-rater agreement on ceVUS grading was 90% and 88% for right and left kidneys, respectively. There was a significant negative association between fetal hydronephrosis vs urinary tract infection and disagreement between Rater 2 and the VCUG report for the left kidney. There were no other significant associations with respect to either kidney, Optison™ dose, or referral diagnosis. DISCUSSION: Our study showed that detection of VUR with ceVUS and Optison™ is comparable to fluoroscopic VCUG. Based on the VCUG reports, the incidence of VUR in our patient population was substantially lower than in the meta-analysis of Chua et al. and in the study of Kim et al. The explanation for the large discrepancy in VUR incidence may reflect differences in the patient populations, and in our reporting of VUR with respect to kidney number rather than to pelviureteral units. Study limitations include its retrospective nature and potential bias in terms of patient selection. Since VUR is an intermittent phenomenon, sequential rather than simultaneous performance of the ceVUS and fluoroscopic studies might have influenced VUR detection. CONCLUSION: A blinded comparison of ceVUS performed with Optison™ to fluoroscopic VCUG showed moderate-good sensitivity and specificity for diagnosis of VUR.

Refining sonographic criteria for paediatric appendicitis: combined effects of age-based appendiceal size and secondary findings.

OBJECTIVE: Appendiceal diameter is a primary sonographic determinant of paediatric appendicitis. We sought to determine if the diagnostic performance of outer appendiceal diameter differs based on age or with the addition of secondary sonographic findings. METHODS: We retrospectively reviewed patients aged less than 19 years who presented to the Boston Children's Hospital ED and had an ultrasound (US) for the evaluation of appendicitis between November 2015 and October 2018. Our primary outcome was the presence of appendicitis. We analysed the cases to evaluate the optimal outer appendiceal diameter as a predictor for appendicitis stratified by age (<6, 6 to <11, 11 to <19 years), and with the addition of one or more secondary sonographic findings. RESULTS: Overall, 945 patients met criteria for inclusion, of which 43.9% had appendicitis. Overall, appendiceal diameter as a continuous measure demonstrated excellent test performance across all age groups (area under the curve (AUC) >0.95) but was most predictive of appendicitis in the youngest age group (AUC=0.99 (0.98-1.00)). Although there was no significant difference in optimal diameter threshold between age groups, both 7- and 8-mm thresholds were more predictive than 6 mm across all groups (p<0.001). The addition of individual (particularly appendicolith or echogenic fat) or combinations of secondary sonographic findings increased the diagnostic value for appendicitis above diameter alone. CONCLUSIONS: Appendiceal diameter as a continuous measure was more predictive of appendicitis in the youngest group. Across all age groups, the optimal diameter threshold was 7 mm for the diagnosis of paediatric appendicitis. The addition of individual or combination secondary sonographic findings increases diagnostic performance.

Contrast-enhanced voiding urosonography part 2: urethral imaging.

Ultrasound (US) has been increasingly used as an important imaging tool to assess the urethra in children. The earliest reports of pediatric urethral sonography involved imaging the urethra in a non-voiding state, during physiological voiding of urine, and after instillation of saline. The introduction of US contrast agents has continued to improve visualization of urethral anatomy. Contrast-enhanced US of the urethra can be performed during the voiding phase of a standard contrast-enhanced voiding urosonography (ceVUS) exam or with retrograde instillation of a contrast agent, depending on the exam indication. Both techniques are well tolerated by children and provide accurate information about urethral pathology and periurethral soft tissues. This article reviews the technical aspects and imaging findings of urethral pathologies in children using contrast-enhanced US, both by the voiding and retrograde instillation techniques.

Contrast-enhanced ultrasound of the spleen, pancreas and gallbladder in children.

Gray-scale and color/power Doppler ultrasound (US) are the first-line imaging modalities to evaluate the spleen, gallbladder and pancreas in children. The increasing use of contrast-enhanced ultrasound (CEUS) as a reliable and safe method to evaluate liver lesions in the pediatric population promises potential for imaging other internal organs. Although CEUS applications of the spleen, gallbladder and pancreas have been well described in adults, they have not been fully explored in children. In this manuscript, we present an overview of the applications of CEUS for normal variants and diseases affecting the spleen, gallbladder and pancreas. We highlight a variety of cases as examples of how CEUS can serve in the diagnosis and follow-up for such diseases in children. Our discussion includes specific examination techniques; presentation of the main imaging findings in various benign and malignant lesions of the spleen, gallbladder and pancreas in children; and acknowledgment of the limitations of CEUS for these organs.

Contrast-enhanced ultrasound of pediatric lungs.

In addition to radiography, ultrasound (US) has long proved to be a valuable imaging modality to evaluate the pediatric lung and pleural cavity. Its many inherent advantages, including real-time performance, high spatial resolution, lack of ionizing radiation and lack of need for sedation make it preferable over other imaging modalities such as CT. Since the introduction of ultrasound contrast agents (UCAs), contrast-enhanced ultrasound (CEUS) has become a valuable complementary US technique, with many well-established uses in adults and evolving uses in children. Lung CEUS applications are still not licensed and are performed off-label, although the added value of CEUS in certain clinical scenarios is increasingly reported. The limited evidence of CEUS in the evaluation of pediatric lungs focuses primarily on community-acquired pneumonia and its complications. In this clinical setting, CEUS is used to confidently and accurately diagnose necrotizing pneumonia and to delineate pleural effusions and empyema. In addition to intravenous use, UCAs can be administered directly into the pleural cavity through chest catheters to improve visualization of loculations within a complex pleural effusion, which might necessitate fibrinolytic therapy. The purpose of this paper is to present the current experience on pediatric lung CEUS and to suggest potential additional uses that can be derived from adult studies.

Does age affect the test performance of secondary sonographic findings for pediatric appendicitis?

BACKGROUND: Secondary sonographic findings of appendicitis can aid image analysis and support diagnosis with and without visualization of an appendix. OBJECTIVE: We sought to determine if age affected the test performance of secondary findings for pediatric appendicitis. MATERIALS AND METHODS: We performed a medical record review of emergency department patients younger than 19 years of age who had a sonogram for suspected appendicitis. Our primary patient outcome was appendicitis, as determined by pathology or by image-confirmed perforation/abscess. Our primary analysis was test performance of secondary sonographic findings as recorded by sonographers on the final diagnosis of appendicitis stratified by age (<6 years, 6 to <11 years, 11 to <19 years). RESULTS: A total of 1,219 patients with suspected appendicitis were evaluated by ultrasound, and 1,147 patients met the criteria for analysis. Of the 1,147 patients, 431 (37.6%) had a final diagnosis of appendicitis. Across all age groups, echogenic fat was the most accurate secondary finding (92.5% [95% confidence interval (CI): 91.0, 94.0]) and free fluid was the least accurate secondary finding (54.7% [95% CI: 51.8, 57.5]). There was no significant difference in the age-stratified test performance of secondary sonographic findings except that (1) appendicolith was a more accurate predictor in patients <6 years old than in the middle group (P<0.001) or the oldest group (P<0.001), and (2) free fluid was a more accurate predictor in the middle group than in the oldest group (P=0.02). CONCLUSION: There are no significant differences in the age-stratified test performance of secondary sonographic findings in the prediction of pediatric appendicitis except that appendicolith is more predictive in younger patients.

Ultrasound Assessment of Quadriceps Femoris Muscle Thickness in Critically Ill Children.

OBJECTIVES: Evaluate the reliability of ultrasound to measure quadriceps femoris muscle thickness in critically ill children and to describe serial changes in quadriceps femoris muscle thickness in relation to fluid balance and nutritional intake. DESIGN: Prospective observational study. SETTING: Tertiary care children's hospital. PATIENTS: Inpatients age 3 months to 18 years recently admitted to the ICU who were sedated and mechanically ventilated at the time of the first ultrasound scan. METHODS: Prospective observational study to examine the reliability of averaged ultrasound measurements of quadriceps femoris muscle thickness. Change in average quadriceps femoris muscle thickness over time was correlated with fluid balance and nutritional intake. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Averaged quadriceps femoris muscle thickness demonstrated good to excellent reliability when comparing pediatric critical care providers to pediatric radiologists and when comparing between different pediatric critical care providers. We found no significant association between fluid balance over 1 or 3 days and change in quadriceps femoris muscle thickness over the same time frame. However, there was a significant association between percent of goal calories (p < 0.001) or percent of goal protein (p < 0.001) over 6 days and change in quadriceps femoris muscle thickness over the same time frame. CONCLUSIONS: Averaged ultrasound measurements of quadriceps femoris muscle thickness demonstrate good to excellent reliability, are not confounded by fluid balance, and are useful for tracking changes in muscle thickness that are associated with nutritional intake. Ultrasound-based assessment of quadriceps femoris is a clinically useful tool for evaluating muscle mass and may be a proxy for nutritional status.

Contrast-enhanced ultrasound for musculoskeletal indications in children.

The increasing use of contrast-enhanced ultrasound (CEUS) has opened exciting new frontiers for musculoskeletal applications in adults and children. The most common musculoskeletal-related CEUS applications in adults are for detecting inflammatory joint diseases, imaging skeletal muscles and tendon perfusion, imaging postoperative viability of osseous and osseocutaneous tissue flaps, and evaluating the malignant potential of soft-tissue masses. Pediatric musculoskeletal-related CEUS has been applied for imaging juvenile idiopathic arthritis and Legg-Calvé-Perthes disease and for evaluating femoral head perfusion following surgical hip reduction in children with developmental hip dysplasia. CEUS can improve visualization of the capillary network in superficial and deep tissues and also in states of slow- or low-volume blood flow. In addition, measurements of blood flow imaging parameters performed by quantitative CEUS are valuable when monitoring the outcome of treatment interventions. In this review article we present current experience regarding a wide range of CEUS applications in musculoskeletal conditions in adults and children, with emphasis on the latter, and discuss imaging techniques and CEUS findings in musculoskeletal applications.

Contrast-enhanced ultrasound of the pediatric brain.

Brain contrast-enhanced ultrasound (CEUS) is an emerging application that can complement gray-scale US and yield additional insights into cerebral flow dynamics. CEUS uses intravenous injection of ultrasound contrast agents (UCAs) to highlight tissue perfusion and thus more clearly delineate cerebral pathologies including stroke, hypoxic-ischemic injury and focal lesions such as tumors and vascular malformations. It can be applied not only in infants with open fontanelles but also in older children and adults via a transtemporal window or surgically created acoustic window. Advancements in CEUS technology and post-processing methods for quantitative analysis of UCA kinetics further elucidate cerebral microcirculation. In this review article we discuss the CEUS examination protocol for brain imaging in children, current clinical applications and future directions for research and clinical uses of brain CEUS.

Leveraging the Combined Predictive Value of Ultrasound and Laboratory Data to Reduce Radiation Exposure and Resource Utilization in Children with Suspected Appendicitis.

BACKGROUND: Previous investigation has shown that the combined predictive value of white blood cell count and ultrasound (US) findings to be superior to either alone in children with suspected appendicitis. The purpose of this study was to evaluate the impact of a diagnostic clinical pathway (DCP) leveraging the combined predictive value of these tests on computed tomography (CT) utilization and resource utilization. METHODS: Retrospective cohort study comparing 8 mo of data before DCP implementation to 18 mo of data following implementation. The pathway incorporated decision-support for disposition (operative intervention, observation, or further cross-sectional imaging) based on the combined predictive value of laboratory and US data (stratifying patients into low, moderate, and high-risk groups). Study measures included CT and magnetic resonance imaging utilization, imaging-related cost, time to appendectomy, and negative appendectomy rate. RESULTS: Ninety-seven patients in the preintervention period were compared with 319 patients in the postintervention period. Following DCP implementation, CT utilization decreased by 86% (21% versus 3%, P < 0.001). Mean time to appendectomy decreased from 8.5 to 7.2 h (P < 0.001), and the negative appendectomy rate remained unchanged (5% versus 4%, P = 0.54). Magnetic resonance imaging utilization increased following pathway implementation (1% versus 7%, P = 0.02); however, median imaging-related cost was significantly lower in the postimplementation period ($283/case to $270/case, P = 0.002) CONCLUSIONS: In children with suspected appendicitis, implementation of a DCP leveraging the combined predictive value of white blood cell and US data was associated with a reduction in CT utilization, time to appendectomy, and imaging-related cost.

Contrast enhanced colostography: New applications in preoperative evaluation of anorectal malformations.

INTRODUCTION: Understanding details of anatomic relationships between the colon and surrounding structures is a critical piece of preoperative planning prior to surgical repair of anorectal malformations (ARMs). Traditional imaging techniques involve ionizing radiation, distention of the rectum with supraphysiologic intraluminal pressures, and sometimes require sedation. Recent developments in the field of contrast agents have allowed the emergence of an ultrasound-based technique that can avoid these requirements while continuing to provide high resolution structural information in three dimensions. METHODS: Fourteen children (13 male, 1 female, age 1-11 months) with ARMs underwent contrast enhanced colostography (ceCS) in addition to traditional preoperative imaging techniques to delineate anatomic relationships of pelvic structures. RESULTS: ceCS and traditional imaging yielded concordant anatomic information, including structural relationships and fistulous connections, in 10/14 patients (71%). ceCS detected fistulous connection in 2/13 patients (15%) that were not seen by traditional imaging. Ultrasonography failed to detect the fistulous connection in one patient. CONCLUSIONS: ceCS is a safe, effective and flexible method for defining important structural information in ARM patients. When compared with traditional methods, it provided equivalent or superior results 93% of the time and bears consideration as a standard tool in preoperative planning for this population. TYPE OF STUDY: Retrospective Comparative Study. LEVEL OF EVIDENCE: Level III.

Fetal Echoplanar Imaging: Promises and Challenges.

Fetal magnetic resonance imaging (MRI) has been gaining increasing interest in both clinical radiology and research. Echoplanar imaging (EPI) offers a unique potential, as it can be used to acquire images very fast. It can be used to freeze motion, or to get multiple images with various contrast mechanisms that allow studying the microstructure and function of the fetal brain and body organs. In this article, we discuss the current clinical and research applications of fetal EPI. This includes T2*-weighted imaging to better identify blood products and vessels, using diffusion-weighted MRI to investigate connections of the developing brain and using functional MRI (fMRI) to identify the functional networks of the developing brain. EPI can also be used as an alternative structural sequence when banding or standing wave artifacts adversely affect the mainstream sequences used routinely in structural fetal MRI. We also discuss the challenges with EPI acquisitions, and potential solutions. As EPI acquisitions are inherently sensitive to susceptibility artifacts, geometric distortions limit the use of high-resolution EPI acquisitions. Also, interslice motion and transmit and receive field inhomogeneities may create significant artifacts in fetal EPI. We conclude by discussing promising research directions to overcome these challenges to improve the use of EPI in clinical and research applications.

A new variant of type III jejunoileal atresia.

Jejunoileal atresia (JIA) is a congenital defect that can result in significant loss of bowel length. The traditional classification of JIA was first proposed by Grosfeld and includes 4 subtypes. Among these, type IIIB, or apple-peel atresia, is characterized by a proximal atretic jejunum and a distal segment of spiraled bowel that terminates at the cecum. Owing to this anatomy, patients with type IIIB JIA are at increased risk for short bowel syndrome and intestinal failure. In this report, we described the case of a neonate with a prenatal diagnosis of JIA. At exploration, she was initially found to have a type IIIB atresia. However, instead of terminating at the cecum, the distal spiraled segment was followed by 75 cm of normal small bowel and mesentery. Surgical correction proceeded with minimal resection and primary anastomosis. She recovered well from this procedure, tolerated full enteral nutrition by mouth, and displayed good weight gain at outpatient follow-up. Owing to the unique anatomy of the gastrointestinal tract in this case report, we propose the addition of a new class of JIA, type IIIC, to better reflect its prognostication and surgical management.

Prenatal imaging throughout gestation in Beckwith-Wiedemann syndrome.

PURPOSE: Prenatal occurrence and timing of appearance of associated features in Beckwith-Wiedemann syndrome (BWS) are unknown. We reviewed our BWS patients with serial fetal imaging and correlated these with postnatal findings. METHODS: All BWS patients with fetal ultrasound (US) or magnetic resonance imaging (MRI) from 2000 to 2016 were reviewed to determine the presence of polyhydramnios, placentamegaly, macrosomia, macroglossia, retrognathia, omphalocele, visceromegaly, and hemihypertrophy. These observations were correlated with postnatal findings. Data were analyzed by Mann-Whitney U test. RESULTS: Nine BWS patients underwent 42 fetal imaging studies with median of five (range of two to six) studies per patient between 13 and 35 weeks gestation. All prenatal findings were confirmed postnatally with complete concordance. All patients with omphalocele were detected early in gestation but other postnatal findings less predictably so. All omphaloceles were small, and were found significantly earlier in gestation than macrosomia (P = 0.004) and macroglossia (P = 0.012). Visceromegaly and retrognathia were less frequent, with no significant differences in median gestational age from omphalocele when prenatally identified. CONCLUSIONS: In BWS, omphalocele is the most common prenatal finding and routinely observed in early gestation with 100% accuracy. Associated findings of macrosomia, macroglossia, visceromegaly, and retrognathia, when present, are detected later in gestation. Imaging in later gestation may reveal additional abnormalities that support a BWS diagnosis.

Interobserver and Intra-Observer Reliability of the Urinary Tract Dilation Classification System in Neonates: A Multicenter Study.

PURPOSE: The Urinary Tract Dilation classification system was designed to be more objective and reproducible than currently available grading systems. We evaluated the reliability and consistency of the system in newborns. MATERIALS AND METHODS: Of 1,046 infants 0 to 90 days old undergoing ultrasound for hydronephrosis 243 were randomly selected for study inclusion. Seven readers (4 radiologists and 3 urologists) at 4 institutions classified complete, de-identified ultrasound studies on a Web based platform. Interobserver and intra-observer agreement was evaluated using the Fleiss kappa statistic. RESULTS: Interobserver agreement for Urinary Tract Dilation risk score was moderate among the 7 readers (kappa = 0.421, 95% CI 0.404-0.438). Interobserver agreement using the Society for Fetal Urology scale was worse than with the Urinary Tract Dilation classification (kappa = 0.344, 95% CI 0.330-0.359). All 7 readers assigned the same Urinary Tract Dilation score in 19.3% of cases (47 of 243). In 38.7% of cases (94 of 243) at least 3 readers assigned a Urinary Tract Dilation score different from that assigned by the other readers. In 7% of cases (17 of 243) at least 3 readers assigned a score of P0/P1, while at least 3 readers scored the same cases as P2/P3. At least 3 different Urinary Tract Dilation risk scores were assigned to the same patient in 30.45% of patients (74 of 243). Among individual Urinary Tract Dilation elements calyceal dilatation and bladder status had the highest disagreement. Five readers regraded 80 cases and agreed with their previous Urinary Tract Dilation risk score in 63.8% to 75.0% of cases (kappa 0.458 to 0.729). CONCLUSIONS: Interobserver agreement using the Urinary Tract Dilation grading system is fair to moderate, with variable agreement on individual elements of the system. Agreement was higher for the Urinary Tract Dilation system compared to the Society for Fetal Urology scale.

The Distended Fetal Hypopharynx: A Sensitive and Novel Sign for the Prenatal Diagnosis of Esophageal Atresia.

BACKGROUND/PURPOSE: Although advances have been made in the prenatal diagnosis of esophageal atresia (EA), most neonates are not identified until after birth. The distended hypopharynx (DHP) has been suggested as a novel prenatal sign for EA. We assess its diagnostic accuracy and predictive value on ultrasound (US) and magnetic resonance imaging (MRI), both alone and in combination with the esophageal pouch (EP) and secondary signs of EA (polyhydramnios and a small or absent fetal stomach). METHODS: We retrospectively reviewed fetal US and MRI reports and medical records of 88 pregnant women evaluated for possible EA from 2000 to 2016. Seventy-five had postnatal follow-up that confirmed or disproved the diagnosis of EA and were included in our analysis. RESULTS: Seventy-five women had 107 study visits (range 1-4). DHP and/or EP were seen on US and/or MRI in 36% of patients, and 78% of those patients had EA. DHP was 24% more sensitive for EA than EP, while EP was 30% more specific. After 28weeks of gestation, DHP had a predictive accuracy for EA of 0.929 (P=0.001). CONCLUSIONS: DHP is a sensitive additional prenatal sign of EA. More accurate diagnosis of EA allows for improved counseling regarding delivery, postnatal evaluation, and surgical correction. TYPE OF STUDY: Diagnostic. LEVEL OF EVIDENCE: Level II.

Pediatric contrast-enhanced ultrasound in the United States: a survey by the Contrast-Enhanced Ultrasound Task Force of the Society for Pediatric Radiology.

BACKGROUND: The United States Food and Drug Administration (FDA) recently approved an ultrasound (US) contrast agent for intravenous and intravesical administration in children. OBJECTIVE: Survey the usage, interest in and barriers for contrast-enhanced US among pediatric radiologists. MATERIALS AND METHODS: The Contrast-Enhanced Ultrasound Task Force of the Society for Pediatric Radiology (SPR) surveyed the membership of the SPR in January 2017 regarding their current use and opinions about contrast-enhanced US in pediatrics. RESULTS: The majority (51.1%, 166) of the 325 respondents (26.7% of 1,218) practice in either a university- or academic affiliated group. The most widely used US contrast agent was Lumason® 52.3% (23/44). While lack of expertise and training were reported barriers, all respondents who are not currently using US contrast agents are considering future use. CONCLUSION: Interest in pediatric contrast US is very high. Education and training are needed to support members who plan to adopt contrast US into practice.

Early Outcomes of Vein Grafting for Reconstruction of Brachial Arterial Injuries in Children.

PURPOSE: We postulated that, with microsurgical technique, vascular reconstruction with interpositional vein grafts in the pediatric population is safe and results in sustained vascular patency and excellent midterm outcomes. METHODS: Twenty children with brachial artery injuries were treated with interpositional vein grafting at a tertiary pediatric hospital from 1995 to 2013. Medical records were evaluated for demographic, clinical, and radiographic data. Ten patients were available for longer-term follow-up. Pain at rest, pain with exercise, and temperature intolerance were assessed with visual analog scale (VAS) (range, 0-5). Functional outcomes were assessed using the Pediatric Outcomes Data Collection Instrument (PODCI) and Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaires. Graft patency and flow were characterized via duplex sonography. Peak flow velocity and resistive index (RI) were compared with the contralateral extremity. Median patient age at the time of injury was 7.75 years (range, 4.6-11.5 years) and median follow-up was 1.75 years (range, 0.3-6.3 years). RESULTS: All patients had perfused hands with palpable radial pulses at follow-up. There were no clinically meaningful differences in motion, sensibility, or strength between affected and unaffected limbs. Mean VAS scores for pain at rest, pain with exercise, and temperature intolerance were 0.4, 0.4, and 0.6, respectively. Mean global PODCI and DASH scores were 98.0 and 5.1, respectively. Nine of 10 patients had patent arteries with normal flow patterns. In the patient with graft occlusion, there was collateralization around the elbow with normal reconstitution of the distal vessels. Mean peak flow velocity proximal and distal to the graft were 77.7 cm/s and 66.5 cm/s, respectively. Mean RI of the graft were 0.84 and 0.77, respectively. CONCLUSIONS: Brachial artery reconstruction using interpositional vein graft and microsurgical technique is safe and effective and results in excellent functional outcomes in children. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Radiographic measurement of intestinal length among children with short bowel syndrome: Retrospective determination remains problematic.

PURPOSE: Small bowel length is the most reliable predictor of enteral independence in pediatric short bowel syndrome. Retrospectively measured bowel lengths on upper GI with small bowel follow-through (UGI/SBFT) were compared to operative measurements. METHODS: A pediatric radiologist and surgical trainees blinded to operative measurements retrospectively analyzed UGI/SBFT studies using the digital radiography curved measurement tool. Children with SBS and severe intestinal failure (parenteral nutrition >90days) at a multidisciplinary intestinal failure program 2002-2015 were included. Data were expressed as median (Q1, Q3). RESULTS: Thirty-six children aged 0.8 (0.4, 3.7) years were analyzed. Fifty-six percent had intestinal malrotation, and 58% had prior serial transverse enteroplasty. Studies were conducted within 10 (7, 20) days of surgery. Intraoperative bowel length was 90cm (45, 142), while UGI/SBFT measurement by radiologist was 45cm (28, 63), with a mean difference of 47cm (SD 58cm, p<0.001) and a mean percent error of 50%. Radiographic assessment underestimated intestinal length in 83% of patients. CONCLUSION: Bowel length measured retrospectively from upper GI with small bowel follow-through studies usually underestimated intraoperative bowel length. The limits of agreement were too wide for this technique to be clinically useful. Operative measurement remains necessary to assess intestinal length and rehabilitation potential. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level III.