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Discussed is the case of a 13-year-old male with no significant past medical history, who presented to the hospital for evaluation of multisystem inflammatory syndrome in children (MIS-C). He arrived with findings of conjunctivitis, strawberry tongue, tachycardia at rest, and hypoxemia with oxygen saturation of 85%. The patient was recently hospitalized 1 week prior for viral pneumonia and hypoxemia due to SARS-CoV-2. During his inpatient stay he received combination therapy of remdesivir and dexamethasone. Following, he weaned to room air and his pulse oximetry (SaO2) order was switched to intermittent with floor vitals prior to his discharge.

Bronchiectasis (BE) is defined as a permanent, irreversible dilation of the bronchial tree. In the pediatric population, this disease process is most commonly associated with patients with cystic fibrosis (CF). However, bronchiectasis unrelated to cystic fibrosis is increasingly noted as a cause of chronic respiratory related morbidity worldwide. Chronic inflammation and recurrent infection result in cellular cascades that lead to irreversible structural changes of the airways. When these architectural changes occur, they confer extensive risks to morbidity usually due to continued infections. In the adult population, bronchiectasis has been associated with chronic obstructive pulmonary disease, which is mainly caused by cigarette smoking. In this report, the authors reviewed various cases of bronchiectasis in the pediatric population at our institution. After a comprehensive case by case review, we compiled details of 3 cases of newly diagnosed bronchiectasis where the most likely inciting factor was the electronic cigarette use. Common features of the three cases included at least a year of e-cigarette use with conjunction of Tetrahydrocannabinol (THC) and radiologic findings of bronchiectasis, ground glass opacities, and nodule formation. This article is protected by copyright. All rights reserved.

Skeletal fractures, a common injury in physically abused children, often go undetected and untreated for significant lengths of time and are sometimes incidentally discovered radiographically. Our objective was to review current literature for scientific studies of pediatric fracture healing with associated timelines. We conducted a search of Embase, EBSCOhost, MEDLINE (PubMed), and Web of Science for literature published from the earliest available up to August 2018. We evaluated the included articles for quality, with consideration for use in clinical and forensic settings. Of a total of 313 full-text articles evaluated, 10 met study inclusion criteria. The patient age range among studies was 0-17 years, with children younger than 1 year included in the majority of studies. The fracture locations included in studies were primarily fractures of the upper limb and pectoral girdle, followed by fractures of the lower limb. The radiographic features of healing varied greatly among the studies. Timelines of common fracture healing variables differed significantly among studies. Scientific, radiographic studies of pediatric fracture healing are limited. Gaps in knowledge regarding fracture healing highlight the need for future research and validation studies. Fracture healing timelines derived from existing timelines should be used with caution.

Acute respiratory distress syndrome (ARDS) is a disabling and potentially lethal syndrome requiring prompt recognition and urgent interventions to prevent morbidity and mortality[1]. Although constipation is not generally recognized as a cause for ARDS or usually listed within the differential diagnosis, there have been case reports describing such an association[2,3]. We present the case of a patient with history of intermittent constipation presenting with progressive abdominal pain and an acute abdomen that required emergent surgical fecal decompaction. This was followed by hypoxemic respiratory distress leading to respiratory failure in the setting of severe constipation and aspirated feculent material. To our knowledge, this is the first published case report describing aspirated feculent material in a child with respiratory failure due to ARDS.

Imaging is extremely important throughout all phases of care provided to children with anorectal malformations (ARM). A preoperative determination of the patient specific malformation will help establish the operative plan. Moreover, the majority of ARM patients will have an associated anomaly that will require imaging workup for full understanding of those abnormalities prior to addressing the ARM. The complexity of ARM care will mandate continued imaging throughout the post-operative period even in those with straight forward malformations.

Background: Approximately 7% of adolescent girls have reproductive tract anomalies. Anomalies associated with complete outflow track obstruction present with primary amenorrhea and cyclic abdominal pain. Some outflow tract obstructions may be associated with renal, anorectal, vertebral, cardiac, tracheoesophageal, and limb anomalies. Outflow tract obstructions may occur at varying anatomic locations – vaginal, cervical, or uterine, each requiring different surgical management. Methods: We present the preoperative evaluation and surgical management of three different gynecologic outflow tract obstructions. Results: Physical exam, ultrasound, and MRI were obtained for each patient. The first obstruction was a transverse vaginal septum, which was treated with transperineal resection to allow menstrual egress. The second obstruction was identified as distal vaginal atresia and managed with operative pull-through of her native vagina. The third obstruction was found to be cervico-vaginal atresia and required hysterectomy and salpingectomy with preservation of the ovaries. Conclusions: Preoperative examination of the perineum and imaging with accurate interpretation is crucial to identify subtle but important distinctions in diagnosis, which can result in markedly different interventions ranging from transperineal resection of a transverse vaginal septum, vaginal pull-through procedure, to hysterectomy. The location of the gynecologic outflow obstruction dictates the proper surgical treatment of these patients. Keywords: Mullerian obstruction, Adolescent, Anorectal malformation

Idiopathic acute eosinophilic pneumonia is a rare and potentially life-threatening condition that is defined by bilateral pulmonary infiltrates and fever in the presence of pulmonary eosinophilia. It often presents acutely in previously healthy individuals and can be difficult to distinguish from infectious pneumonia. Although the exact etiology of idiopathic acute eosinophilic pneumonia remains unknown, an acute hypersensitivity reaction to an inhaled antigen is suggested, which is further supported by recent public health risks of vaping (electronic cigarette) use and the development of lung disease. In this case, a patient with a year-long history of vaping in conjunction with tetrahydrocannabinol cartridge use who was diagnosed with idiopathic acute eosinophilic pneumonia with associated bilateral hilar lymphadenopathy is described.

Purpose Early diagnosis and treatment of slipped capital femoral epiphysis (SCFE) is important to prevent slip progression and avoid complications. We sought to determine if MRI findings in patients with unilateral SCFE could indicate ‘pre-slip’ or predict future SCFE in the contralateral hip. Methods A prospective study evaluated patients with unilateral SCFE over a two-year period. MRI of the asymptomatic hip was performed within the perioperative period. Patients were followed with radiographs until a contralateral slip occurred or until physeal closure. Demographics, clinical stability, severity, posterior slope angle (PSA), modified Oxford Bone Score (mOBS) and patency of the triradiate cartilage were recorded and statistical analysis performed. Results In all, 33 of 54 patients with unilateral SCFE were enrolled into the study. In all, 29 (87.8%) had complete follow-up. Five of the enrolled patients (15.2%) developed a sequential slip requiring in situ pinning. Six of 33 (18.2%) patients had positive MRI findings: four of which proceeded to sequential SCFE and two which did not. One sequential slip had a negative MRI. PSA predicted 1/11 sequential slips (sensitivity 9.09%, specificity 81.4%, positive predictive value (PPV) 11.1%, negative predictive value (NPV) 77.8%) and mOBS predicted 5/11 sequential slips (sensitivity 45.5%, specificity 93%, PPV 62.5%, NPV 87%). An open triradiate cartilage was present in 8/11 patients with sequential slips (sensitivity 72.7%, specificity 81.4%, PPV 50%, NPV 92.1%). Conclusion MRI findings consistent with ‘pre-slip’ were present in 66.7% of patients who developed a sequential SCFE. Further study on the utility/sensitivity of MRI in predicting sequential SCFE is warranted. Level of Evidence II, diagnostic

Background 3-dimensional prints (3DP) anecdotally facilitate surgeon understanding of anatomy and decision-making. However, the actual benefit to surgeons or patients has not been quantified. This study investigates how surgeon understanding of complex anatomy is altered by a 3DP compared to computed tomography (CT) scan or CT + digital reconstruction (CT + DR). Materials and methods Key anatomic features were segmented from a CT-abdomen/pelvis of pygopagus twins to build a DR and printed in color on a 3D printer. Pediatric surgery trainees and attendings (n = 21) were tested regarding anatomy identification and their understanding of point-to-point distances, scale, and shape. Results There was no difference between media regarding point-to-point distances. The 3DP led to an increased number of correct answers for questions of scale and shape compared to CT (P

Computed tomography (CT) is a widely used diagnostic tool in pediatric medicine. However, due to concerns regarding radiation exposure, it is essential to identify patient characteristics associated with higher radiation burden from CT imaging, in order to more effectively target efforts towards dose reduction. Our objective was to identify the effects of various demographic and clinical patient characteristics on radiation exposure from single abdomen/pelvis CT scans in children.CT scans performed at our institution between January 2013 and August 2015 in patients under 16 years of age were processed using a software tool that estimates patient-specific organ and effective doses and merges these estimates with data from the electronic health record and billing record. Quantile regression models at the 50th, 75th, and 90th percentiles were used to estimate the effects of patients' demographic and clinical characteristics on effective dose.2390 abdomen/pelvis CT scans (median effective dose 1.52mSv) were included. Of all characteristics examined, only older age, female gender, higher BMI, and whether the scan was a multiphase exam or an exam that required repeating for movement were significant predictors of higher effective dose at each quantile examined (all p0.05). The effects of obesity and multiphase or repeat scanning on effective dose were magnified in higher dose scans.Older age, female gender, obesity, and multiphase or repeat scanning are all associated with increased effective dose from abdomen/pelvis CT. Targeted efforts to reduce dose from abdominal CT in these groups should be undertaken.

Highly complex medical documents, including ultrasound reports, are greatly mismatched with patient literacy levels. While improving radiology reports for readability is a longstanding concern, few articles objectively measure the effectiveness of physician training for readability improvement. We hypothesized that writing styles may be evaluated using an objective two-dimensional measure and writing training could improve the writing styles of radiologists. To test it, a simplified “grade vs. length” readability metric is developed based on results from factor analysis of ten readability metrics applied to more than 500,000 radiology reports. To test the short-term effectiveness of a writing workshop, we measured the writing style improvement before and after the training. Statistically significant writing style improvement occurred as a result of the training. Although the degree of improvement varied for different measures, it is evident that targeted training could provide potential benefits to improve readability due to our statistically significant results. The simplified grade vs. length metric enables future clinical decision support systems to quantitatively guide physicians to improve writing styles through writing workshops.

Distraction injury of the spine with spinal cord transection and adjacent vascular injury is rarely described in the setting of child physical abuse. We report a 5-week-old infant boy who sustained these injuries after an abusive event. The clinical presentation, imaging findings, and recommended evaluation modalities are discussed. An overview of pediatric spinal column and vascular injuries secondary to physical abuse is given.

Introduction Atelectasis is a problem in sedated pediatric patients undergoing cross-sectional imaging, impairing the ability to accurately interpret chest computed tomography (CT) imaging for the presence of malignancy, often leading to additional maneuvers and/or repeat imaging with additional radiation exposure. Methods A quality improvement team established a best-practice protocol to improve the quality of thoracic CT imaging in young patients with suspected primary or metastatic pulmonary malignancy. The specific aim was to increase the percentage of chest CT scans obtained for the evaluation of pulmonary nodules with acceptable atelectasis scores (0-1) in patients aged 0-5 years with malignancy, from a baseline of 45% to a goal of 75%. Results A retrospective cohort consisted of 94 patients undergoing chest CT between February 2014 and January 2015 before protocol implementation. The prospective cohort included 195 patients imaged between February 2015 and April 2018. The baseline percentage of CT scans that were scored 0 or 1 on the atelectasis scale was 44.7%, which improved to 75% with protocol implementation. The mean atelectasis score improved from 1.79 (±0.14) to 0.7 (±0.09). Sedation incidence decreased substantially from 73.2% to 26.5% during the study period. Conclusions Using quality improvement methodology including standardization of care, the percentage of children with atelectasis scores of 0-1 undergoing cross-sectional thoracic imaging improved from 45% to 75%. Also, eliminating the need for sedation in these patients has further improved image quality, potentially allowing for optimal detection of smaller nodules, and minimizing morbidity.

A 4-yr-old female with congenital knee dislocations and joint laxity was noted to have a strong maternal family history comprising multiple individuals with knee problems and clubfeet. As the knee issues were the predominant clinical features, clinical testing included sequencing of LMX1B, TBX2, and TBX4, which identified no significant variants. Research genome sequencing was performed in the proband, parents, and maternal grandfather. A heterozygous in-frame deletion in FLNB c. 5468_5470delAGG, which predicts p.(Glu1823del), segregated with the disease. The variant is rare in the gnomAD database, removes a residue that is evolutionarily conserved, and is predicted to alter protein length. Larsen syndrome may present with pathology that primarily involves one joint and thus may be difficult to differentiate clinically from other skeletal dysplasias or arthrogryposis syndromes. The p.(Glu1823del) variant maps to a filamin repeat domain where other disease-causing variants are clustered, consistent with a probable gain-of-function mechanism. It has reportedly been observed in two individuals in the gnomAD database, suggesting that mild presentations of Larsen syndrome, like the individual reported here, may be underdiagnosed in the general population.

Background We report a female patient with endocrine abnormalities, hypogonadotropic hypogonadism and amazia (breasts aplasia/hypoplasia but normal nipples and areolas) in a rare syndrome: Van Maldergem syndrome (VMS). Case presentation Our patient was first evaluated at age 4 for intellectual disability, craniofacial features, and auditory malformations. At age 15, she presented with no breast development and other findings consistent with hypogonadotropic hypogonadism. At age 37, she underwent whole exome sequencing (WES) to identify pathogenic variants. WES revealed compound heterozygous variants in DCHS1 (rs145099391:G > A, p.P197L & rs753548138:G > A, p.T2334 M) [RefSeq NM_003737.3], diagnostic of Van Maldergem syndrome (VMS-1). VMS is a rare autosomal disorder reported in only 13 patients, characterized by intellectual disability, typical craniofacial features, auditory malformations, hearing loss, skeletal and limb malformations, brain abnormalities with periventricular neuronal heterotopia and other variable anomalies. Our patient had similar phenotypic abnormalities. She also had hypogonadotropic hypogonadism and amazia. Based on the clinical findings reported, two previously published patients with VMS may also have been affected by hypogonadotropic hypogonadism, but endocrine abnormalities were not evaluated or mentioned. Conclusion This case highlights an individual with VMS, characterized by compound heterozygous variants in DCHS1. Our observations may provide additional information on the phenotypic spectrum of VMS, including hypogonadotropic hypogonadism and amazia. However, the molecular genetic basis for endocrine anomalies observed in some VMS patients, including ours, remains unexplained. Electronic supplementary material The online version of this article (10.1186/s13633-017-0052-z) contains supplementary material, which is available to authorized users.

Preoperative assessment of tissue anatomy and accurate surgical planning is crucial in conjoined twin separation surgery. We developed a new method that combines three-dimensional (3D) printing, assembling, and casting to produce anatomic models of high fidelity for surgical planning. The related anatomic features of the conjoined twins were captured by computed tomography (CT), classified as five organ groups, and reconstructed as five computer models. Among these organ groups, the skeleton was produced by fused deposition modeling (FDM) using acrylonitrile-butadiene-styrene. For the other four organ groups, shell molds were prepared by FDM and cast with silica gel to simulate soft tissues, with contrast enhancement pigments added to simulate different CT and visual contrasts. The produced models were assembled, positioned firmly within a 3D printed shell mold simulating the skin boundary, and cast with transparent silica gel. The produced phantom was subject to further CT scan in comparison with that of the patient data for fidelity evaluation. Further data analysis showed that the produced model reassembled the geometric features of the original CT data with an overall mean deviation of less than 2 mm, indicating the clinical potential to use this method for surgical planning in conjoined twin separation surgery.

Radiation burden from the use of computed tomography (CT) imaging may not be evenly distributed. We tested whether overweight and obese children receive higher radiation burden from CT imaging.An automated software tool (DoseWizard) assessed patient-level radiation dose from CT and merged results with clinical data from the electronic health record. CT scans of the head, chest, and abdomen/pelvis (A/P) performed at our institution from January 2013 to August 2015 on patients aged16 y were processed using this software. Patients were categorized as overweight/obese if they had a body mass index (BMI) greater than the 85th percentile for age. Radiation exposures were compared between groups. Higher dose CT scans were identified as having a radiation dose in the top decile. Multivariate analysis identified factors associated with high-dose CT scans.About 7212 CT scans were included. Overweight/obese patients received similar radiation dose for head CT as compared with normal weight patients (1.51 versus 1.49 mSv, P = 0.04) but higher radiation dose for chest (1.14 versus 0.81 mSv, P 0.001) and A/P (1.97 versus 1.43 mSv, P 0.001). In multivariable regression models, being overweight/obese increased the odds of a higher dose chest CT (odds ratio 2.24, P 0.001) and A/P CT (odds ratio 7.24, P 0.001).Overweight and obese children receive higher radiation burden from CT imaging and are one group that deserves consideration of dose monitoring. This software tool can be used to measure changes in radiation exposure and run clinical decision support in future studies targeting high radiation exposure to children.

Background Ultrasound is preferred over computed tomography (CT) for diagnosing appendicitis in children to avoid undue radiation exposure. We previously reported our experience in instituting a standardized appendicitis ultrasound template, which decreased CT rates by 67.3%. In this analysis, we demonstrate the ongoing cost savings associated with using this template. Methods Retrospective chart review for the time period preceding template implementation (June 2012-September 2012) was combined with prospective review through December 2015 for all patients in the emergency department receiving diagnostic imaging for appendicitis. The type of imaging was recorded, and imaging rates and ultrasound test statistics were calculated. Estimated annual imaging costs based on pretemplate ultrasound and CT utilization rates were compared with post-template annual costs to calculate annual and cumulative savings. Results In the pretemplate period, ultrasound and CT rates were 80.2% and 44.3%, respectively, resulting in a combined annual cost of $300,527.70. Similar calculations were performed for each succeeding year, accounting for changes in patient volume. Using pretemplate rates, our projected 2015 imaging cost was $371,402.86; however, our ultrasound rate had increased to 98.3%, whereas the CT rate declined to 9.6%, yielding an annual estimated cost of $224,853.00 and a savings of $146,549.86. Since implementation, annual savings have steadily increased for a cumulative cost savings of $336,683.83. Conclusions Standardizing ultrasound reports for appendicitis not only reduces the use of CT scans and the associated radiation exposure but also decreases annual imaging costs despite increased numbers of imaging studies. Continued cost reduction may be possible by using diagnostic algorithms.

Background Decreased skeletal muscle mass, or sarcopenia, has been shown to be associated with worse postoperative recovery and a higher risk of complications in adult surgical patients. We hypothesized that pediatric patients with complicated appendicitis may experience sarcopenic changes over the course of their treatment. Methods The medical records and computed tomography scans of 36 pediatric complex appendicitis patients who had both preoperative and postoperative computerized tomography scans at our hospital were reviewed. Changes in psoas muscle area were examined using linear mixed models with random patient-level intercept and time effects. Results The median change in body mass index among all patients from admission to discharge was −0.8 kg/m2 (interquartile range: −1.3 to −0.2). The mean percentage change in psoas muscle area per day over the course of appendicitis-related treatment was −0.81% (95% confidence interval: −1.12 to −0.50) (P 0.10 for all interactions). Conclusions Our data suggest that pediatric patients with complex appendicitis experience sarcopenic changes during their hospital admission. Given previous reports that sarcopenia is a significant predictor of worse surgical outcomes, more investigation is warranted to assess whether these changes are associated with postsurgical complications and to evaluate potential interventions that may prevent these changes.

BACKGROUND: Neurotoxicity is a significant complication of calcineurin inhibitor use, and posterior reversible encephalopathy syndrome has been reported. Limited data exist on the use of alternative immunosuppression regimens in the management of posterior reversible encephalopathy syndrome in transplant recipients. METHODS: We present the immunosuppression management strategy of a girl who underwent bilateral lung transplantation for cystic fibrosis 6 months earlier, then suddenly developed a grand mal seizure due to posterior reversible encephalopathy syndrome diagnosed by magnetic resonance imaging of the brain. In an effort to reduce her tacrolimus dose, an alternative immunosuppressant regimen combining tacrolimus and sirolimus was used. RESULTS: After the modification of her immunosuppressant regimen, there was rapid clinical improvement with no further seizures. Her brain findings had resolved on magnetic resonance imaging 2 months later. Over the next 6 months, allograft function remained stable and surveillance transbronchial biopsies found no allograft rejection on the combined sirolimus and tacrolimus therapy. CONCLUSIONS: Tacrolimus-associated neurotoxicity resolved in a lung transplant recipient with a combined tacrolimus and sirolimus regimen. This combined therapy appears to be an effective alternative for lung transplant recipients that allow them to receive the benefits of both drugs but at lower doses, which reduces the risk for adverse effects.

Background and Objectives MRI is commonly used in the pediatric population and often requires sedation or general anesthesia to complete. This study used data from a pediatric accountable care organization (ACO) to investigate trends in MRI utilization and in the requirement for anesthesia to complete MRI examinations. Methods The Partners for Kids (PFK) ACO claims database was queried for MRI examination encounters involving patients 0 to 18 years old from 2009 to 2014, with utilization expressed as encounters per 10,000 PFK members-months. Data were limited to 2011 to 2014 to ensure consistent billing of anesthesia services. Encounters were classified according to the presence of procedure codes for anesthesia or sedation. Results MRI utilization was approximately constant over the study period at 11 to 12 encounters per 10,000 member-months. The need for anesthesia increased from 21% to 28% of encounters over 2011 to 2014. The latter increase was shared across 1- to 6-year-old, 7- to 12-year-old, and 12- to 18-year-old subgroups. In multivariable regression analysis of monthly utilization, increasing need for anesthesia could not be attributed to secular trends in patient demographics or types of examinations ordered. Paid cost data were available for outpatient MRIs, and MRIs with sedation accounted for an increasing share of these costs (from 22% in 2011 to 33% in 2014). Conclusion There was an increasing need for anesthesia services to complete MRI examinations in this pediatric population, resulting in increasing cost of MRI examinations and presenting a challenge to ACO cost containment.

Objective It is recognised that 5% – 10 % of children with macrocephaly and autism spectrum disorder (ASD) and/or intellectual disability (ID) have a heterozygous pathogenic mutation in the PTEN tumour suppressor gene that is associated with PTEN hamartoma tumour syndrome. However, the clinical features and course in children with a pathogenic PTEN mutation are unclear and have not been well documented. Study objectives We undertook a retrospective chart review of children ( PTEN mutations to ascertain clinical findings, clinical course and possible outcomes. Results Clinical and molecular data were collected and analysed for 47 patients with PTEN mutation from 38 eligible families. Macrocephaly (average head circumference of + 5.7 SD) with developmental delay, ID and/or ASD were the most common presenting signs/symptoms (66 %). Clinical features included dermatological findings (66 %), gastrointestinal (GI) symptoms (34 %), ASD diagnosis (50 %), abnormal brain imaging (53 % of those examined) and abnormal thyroid imaging (26 %). Conclusions This is the largest survey of clinical features in children with PTEN pathogenic mutations to date. It confirms earlier reports of increased rates of neurodevelopmental disorders. Dermatological, GI and thyroid abnormalities are age dependent and may not be present at the time of diagnosis, requiring regular monitoring and medical surveillance. Early paediatric diagnosis is important for institution of medical and developmental surveillance as well as for testing other at- risk family members.

Background Tracheobronchial anomalies are rare congenital malformations that are typically managed conservatively. Several reports have documented their increased incidence in patients with congenital heart disease. However, none of these reports have detailed the incidence found among patients with hypoplastic left heart syndrome (HLHS). Airway obstruction, whether by extrinsic compression or an undiagnosed tracheobronchial anomaly, in the perioperative period may have significant morbidity in this tenuous population. Methods From June 2003 to August 2011, 164 consecutive patients with HLHS underwent a palliative surgical procedure for their cardiac disease. Sixty-three of these patients received either multidetector computed tomography (CT) of the chest or cardiac magnetic resonance imaging (MRI). A total of 124 studies (106 CT, 18 MRI) were performed during this time span. The studies were reviewed independently by a pediatric cardiologist and a pediatric radiologist. Length of intubation, intensive care unit (ICU) stay, and hospital stay were also reviewed for all patients. Results Three of the 63 patients had a congenital abnormality of the tracheobronchial tree (4.8%), which is higher than the incidence that has been reported in the general population. Two of the patients had bilateral left-sided bronchus with an absence of the right upper lobe bronchus. The third patient was found to have a very rudimentary right upper lobe bronchus with absence of the right upper lobe of the lung. The mean intubation time was not significantly different between the groups (P = .615). There was no significant difference of either the total ICU or hospital stay between the two groups. Conclusion Our study demonstrates a higher incidence of tracheobronchial anomalies among patients with HLHS, a severe form of cyanotic congenital heart disease. Patients with a tracheobronchial abnormality did not show a difference in morbidity during the postoperative time period.

A 7 year old male with a history of congenital neutropenia and growth hormone deficiency presented with abdominal pain, fevers, and diarrhea. Imaging and endoscopy revealed significant inflammation of the ascending colon with stenosis at the level of the hepatic flexure. A right hemicolectomy was performed, and pathologic findings were consistent with diffuse intestinal ganglioneuromatosis. Due to recurrent mass effect at the intestinal anastomotic site detected radiologically, a second intestinal resection was performed 7 months later. Genetic testing was negative for mutations in the RET protooncogene, NF1 and PTEN tumor suppressor genes. We report a case of diffuse intestinal ganglioneuromatosis in a child with congenital neutropenia.

External radiation to the head and neck can lead to an increased incidence of thyroid nodules. We investigated whether patients requiring repeated head and neck imaging for the management of shunted hydrocephalus had a higher incidence of ultrasound-detected thyroid nodules compared to reports of comparable age.Patients treated at our institution for shunted hydrocephalus from 1990 to 2003 were contacted. Enroled patients underwent a thyroid ultrasound. Demographic data and radiation exposure history were obtained retrospectively.Thyroid nodules were identified sonographically in 15/112 patients (13.6 %). Patients with thyroid nodules were older (mean 24.3 ± 7.6 years) than those without (mean 18.4 ± 8.0 years) (p = 0.005). Those with a detectable thyroid nodule had a longer follow up time compared to those who did not (mean 21.9 ± 5.5 vs. 15.1 ± 7 years, respectively) (p = 0.018).Patients with shunted hydrocephalus are exposed to substantial head and neck radiation from diagnostic imaging and have a higher incidence of thyroid nodules detected by ultrasonography. These patients should be provided ongoing surveillance for detection of thyroid nodules and the possibility of malignancy.

Appropriate surgical planning is important for improved clinical outcome and minimal complications in many surgical operations, such as a conjoined twin separation surgery. We combine 3D printing with casting and assembling to produce a solid phantom of high fidelity to help surgeons for better preparation of the conjoined twin separation surgery. 3D computer models of individual organs were reconstructed based on CT scanned data of the conjoined twins. The models were sliced, processed, and converted to an appropriate format for Fused Deposition Modeling (FDM). The skeletons of the phantom were printed directly by FDM using Acrylonitrile-Butadiene-Styrene (ABS) material, while internal soft organs were fabricated by casting silicon materials of different compositions in FDM printed molds. The skeleton and the internal organs were then assembled with appropriate fixtures to maintain their relative positional accuracies. The assembly was placed in a FMD printed shell mold of the patient body for further casting. For clear differentiation of different internal organs, CT contrast agents of different compositions were added in the silicon cast materials. The produced phantom was scanned by CT again and compared with that of the original computer models of the conjoined twins in order to verify the structural and positional fidelity. Our preliminary experiments showed that combining 3D printing with casting is an effective way to produce solid phantoms of high fidelity for the improved surgical planning in many clinical applications.

BACKGROUND Recent data suggest magnetic resonance imaging (MRI) is the best method to analyze the status of the cartilage and subchondral bone in patients with juvenile osteochondritis dissecans (JOCD). METHODS MRI analysis of 122 knees and 132 JOCD lesions in 109 patients who underwent arthroscopic treatment for osteochondritis dissecans lesions of the knee between March 2003 and January 2011. RESULTS Agreement between MRI and arthroscopic grading was 62.1%. MRI sensitivity was 92% and specificity was 55%. Positive predictive value of MRI was 33% and negative predictive value of MRI was 97%. In a multivariable logistic regression model, the odds of a unstable lesion on the lateral femoral condyle nonweight-bearing location were 15.7 times greater than the odds of an unstable lesion on the medial femoral condyle weight-bearing area (95% confidence interval: 2.6-95.7, P=0.003.) The odds of the lateral femoral condyle weight-bearing lesion having an unstable grade were also greater than for a medial femoral condyle weight-bearing lesion, but the results were not statistically significant (odds ratio, 1.70, P=0.349). CONCLUSIONS A high T2 signal retrograde to the lesion may commonly appear with an early, stable arthroscopic grade lesion. MRI continues to be reliably sensitive to JOCD lesions and a good predictor of low-grade, stable lesions. However, MRI predictability of high-grade, unstable JOCD lesions is less reliable. Lesions in atypical locations, such as the nonweight-bearing surface of the lateral femoral condyle, more commonly present as higher, arthroscopic grade lesions. LEVEL OF EVIDENCE Level IV, retrospective case series.

Concern about an increased lifetime risk of cancer in children who have undergone a single computed tomography (CT) scan prompted us to review utilization of this diagnostic test in our appendicitis population.From 1998 to 2001, the records of 720 children admitted to our hospital with a diagnosis of appendicitis were reviewed for adjunct diagnostic modalities, including ultrasonography (USG) and CT scanning. Negative appendectomy rates were determined by the final pathologic report. Statistical comparisons were made using the chi(2) test, and significance was assigned at P.05.The use of ultrasound scan for diagnosing appendicitis decreased from 20.0% in 1998 to 7.0% in 2001 (P.01). Conversely, the use of CT scans increased from 17.6% in 1998 to 51.3% in 2001 (P.001). During this time period the difference in the negative appendectomy rate was not statistically significant (P0.20). Of the negative appendectomies, 11 of these patients had a USG interpreted as positive for appendicitis (22.0%), and 9 had a CT scan interpreted as positive (18.0%).Liberal use of CT scans in diagnosing appendicitis in children has not resulted in a decreased negative appendectomy rate. Potentially harmful radiation exposure should prompt pediatric surgeons to reevaluate the role of CT scanning in the management of children with suspected appendicitis.

Background Lateral thoracic expansion is a procedure that has been described to enlarge the thoracic cage in patients with Jeune's asphyxiating thoracic dystrophy. The procedure involves separating ribs from their periosteum and plating them together in an expanded fashion with titanium struts. We have speculated that the ribs heal in this situation, despite the absence of surrounding periosteum, and that new rib formation occurs in the liberated periosteum. Methods Radiographic studies of patients who have undergone lateral thoracic expansion were reviewed for evidence of rib healing or periosteal new bone formation. Results This study presents radiologic evidence that rib healing actually occurs, as does periosteal ossification. Conclusions Lateral thoracic expansion creates additional chest wall that is formed of autologous tissue, fully healed, and not ultimately dependent on titanium struts.

Tracheobronchial anomalies are rare congenital malformations that are typically managed conservatively. Several reports have documented their increased incidence in patients with congenital heart disease. However, none of these reports have detailed the incidence found among patients with hypoplastic left heart syndrome (HLHS). Airway obstruction, whether by extrinsic compression or an undiagnosed tracheobronchial anomaly, in the perioperative period may have significant morbidity in this tenuous population.From June 2003 to August 2011, 164 consecutive patients with HLHS underwent a palliative surgical procedure for their cardiac disease. Sixty-three of these patients received either multidetector computed tomography (CT) of the chest or cardiac magnetic resonance imaging (MRI). A total of 124 studies (106 CT, 18 MRI) were performed during this time span. The studies were reviewed independently by a pediatric cardiologist and a pediatric radiologist. Length of intubation, intensive care unit (ICU) stay, and hospital stay were also reviewed for all patients.Three of the 63 patients had a congenital abnormality of the tracheobronchial tree (4.8%), which is higher than the incidence that has been reported in the general population. Two of the patients had bilateral left-sided bronchus with an absence of the right upper lobe bronchus. The third patient was found to have a very rudimentary right upper lobe bronchus with absence of the right upper lobe of the lung. The mean intubation time was not significantly different between the groups (P = .615). There was no significant difference of either the total ICU or hospital stay between the two groups.Our study demonstrates a higher incidence of tracheobronchial anomalies among patients with HLHS, a severe form of cyanotic congenital heart disease. Patients with a tracheobronchial abnormality did not show a difference in morbidity during the postoperative time period.

Purpose We conducted this study to determine if preoperative magnetic resonance imaging (MRI) cross-sectional area measurements would correlate with intraoperative graft size in hamstring anterior cruciate ligament (ACL) reconstructions. Methods We retrospectively reviewed ACL reconstructions performed by a single surgeon using a quadruple-looped hamstring allograft. Preoperative MRI axial images were used to determine the combined cross-sectional area of the semitendinosis and gracilis tendons. These cross-sectional areas were correlated to the intraoperative graft size. Results We found a strong correlation between the MRI cross-sectional areas and graft size. If the combined cross-sectional areas were ≥18mm 2 , there was an 88% probability of obtaining a graft of sufficient size at the time of surgery. Conclusions We conclude that our technique is a reliable option to assist the surgeon with preoperative determination of graft size. This is valuable to the orthopaedist to more accurately discuss graft options with the patient and improve preoperative preparation with respect to graft choice. Level of Evidence: Level II, development of diagnostic criteria on the basis of consecutive patients with universally applied gold standard.

Background . In 1995, we reported the use of lateral thoracic expansion in a patient with symptomatic Jeune's asphyxiating thoracic dystrophy. We have subsequently used lateral thoracic expansion 16 times on 10 patients during 7 years. This article reports our outcomes and provides surgical details. Methods . Charts of all patients undergoing lateral thoracic expansion were reviewed. Eight of the 10 patients had symptomatic Jeune's syndrome. The other 2 had similar thoracic deformities limiting thoracic capacity. In half of the patients the procedures were performed bilaterally. Results . All patients older than 1 year of age were symptomatically benefited by lateral thoracic expansion. Functional and anatomic measurements documented thoracic enlargement in several patients who had comparable preoperative and postoperative studies. However, 2 infants with significant underlying airway disease did not improve and went on to succumb to that aspect of their disease despite enlargement of the thorax. Fracture of the titanium ministruts has been a recurrent problem, and we now use larger struts. Conclusions . Lateral thoracic expansion is a safe and effective procedure in selected patients with Jeune's syndrome older than 1 year of age as judged by short-term and midterm follow-up. More experience and longer follow-up are required to discern the place of the lateral thoracic expansion in the overall management of these patients.

We present the case of a 15-year-old male with sporadic Burkitt's lymphoma/leukemia. The patient presented with right lower quadrant abdominal pain and masses in the terminal ilium and pelvis, and was subsequently demonstrated to have involvement of the bone marrow. We discuss differential diagnoses and approach to diagnose and stage this disease. A review of the clinical, radiologic, and pathologic features of Burkitt's lymphoma/leukemia are also presented.