Ultrasound Assessment of Ankle Syndesmotic Injuries in a Pediatric Population.

OBJECTIVE: To determine sensitivity and specificity for anterior-inferior tibiofibular ligament (AiTFL) integrity and tibiofibular clear-space (TFCS) cut-off points for dynamic evaluation using ultrasound (US) in a pediatric population. DESIGN: Prospective cohort study. SETTING: Tertiary care university-affiliated pediatric hospital patients between the ages of 12 and 18 sustaining acute ankle trauma with syndesmotic injury. INTERVENTIONS: Participants were assigned to the syndesmotic injury protocol that included a standardized MRI and US. MAIN OUTCOME MEASURES: Anterior-inferior tibiofibular ligament integrity for static assessment and TFCS measurements for dynamic assessment on US. For dynamic assessment, the distance between the distal tibia and fibula was first measured in neutral position and then in external rotation for each ankle. The US results on AiTFL integrity were compared with MRI, considered as our gold standard. Optimal cut-off points of TFCS values were determined with receiver operating characteristics curve analysis. RESULTS: Twenty-six participants were included. Mean age was 14.8 years (SD = 1.3 years). Sensitivity and specificity for AiTFL integrity were 79% and 100%, respectively (4 false negatives on partial tears). For dynamic assessment, the cut-off points for the differences in tibiofibular distance between the 2 ankles in 1) neutral position (TFCS N I-U ) and 2) external rotation (TFCS ER I-U ) were 0.2 mm (sensitivity = 83% and specificity = 80%) and 0.1 mm (sensitivity = 83% and specificity = 80%), respectively. CONCLUSIONS: Static US could be used in a triage context as a diagnostic tool for AiTFL integrity in a pediatric population as it shows good sensitivity and excellent specificity.

Rickets manifestations in a child with metaphyseal anadysplasia, report of a spontaneously resolving case.

INTRODUCTION: Rickets is not an unusual diagnosis for pediatricians even currently in developed countries. Children typically present with leg bowing, enlargement of wrists, rachitic rosary (swelling of costochondral junctions) and/or waddling gait. But not every child with growth delay and enlarged metaphyses is diagnosed with rickets. Metaphyseal anadysplasia (MAD) is a disorder of variable severity with metaphyseal flaring and irregularities, without vertebral abnormalities. MAD is characterized by an early onset and a regressive course in late childhood without treatment, despite persistent short stature. Autosomal dominant or recessive variants in the matrix metalloproteinase 13 gene (MMP13) are responsible for these transient metaphyseal changes. CASE PRESENTATION: We report a new pathogenic heterozygous variant in MMP13 (NM_002427.4: c.216G>C, p.Gln72His) in a toddler, initially thought to have rickets, and his father, with MAD phenotypes. Additionally, we review the seven reported MMP13 variants. CONCLUSION: One should keep a wide differential diagnosis in cases of suspected rickets, including skeletal dysplasias which might have a regressive course.

Management and outcomes of paediatric ileocolic intussusception at a paediatric tertiary care hospital: A retrospective cohort study.

BACKGROUND: Rapid reduction of ileocolic intussusception is important to minimize the compromise in blood flow to the affected bowel segment. This study aimed to quantify the potentially modifiable time between diagnosis and initiation of pneumatic reduction, identify factors associated with delays, and characterize the outcomes of pneumatic reduction in a recent cohort. METHODS: This retrospective observational study occurred at a tertiary care paediatric hospital with a consecutive sample of all children with ileocolic intussusception September 2015 through September 2018. The primary outcome was the time between ultrasound diagnosis of intussusception and the beginning of pneumatic reduction. Independent variables were age of the patient, time of day of arrival, transfer from another facility, and intravenous access prior to ultrasound. Outcomes of pneumatic reduction were expressed as proportions. RESULTS: There were 103 cases of ileocolic intussusception (among 257,282 visits) during the study period. The median time between diagnostic confirmation and initiation of reduction was 36 minutes. This was shorter for transferred patients and children with intravenous access prior to ultrasound. One perforation was identified at the beginning of reduction, without hemodynamic instability. Six children (5.8%) underwent either open (n=4) or laparoscopic surgery (n=2) for reduction failure. CONCLUSION: The median delay between diagnosis and initiation of reduction at this paediatric hospital was short, especially among patients transferred with a suspicion of intussusception and children with intravenous access prior to diagnosis. Complications from pneumatic reduction were infrequent.

Langerhans Cell Histiocytosis With Vertebral Involvement Diagnosed and Treated Over the Last 15 Years in a Single Canadian Pediatric Academic Institution.

We report 11 children with vertebral lesion of Langerhans cell histiocytosis (LCH) diagnosed and treated between 2000 and 2015. Vertebral lesions were usually present at LCH diagnosis. No child developed neurologic symptoms. Among 29 vertebral lesions, only 2 were unstable. Chemotherapy was used in all children but 3. A LCH recurrence was observed in 6 patients, involving vertebrae in 4 cases. All children were disease-free at their last follow-up. Sequelae were more often radiologic than clinical. Since potential recurrences and incomplete bone regeneration exist, discussion about optimal treatment and long-term follow-up of vertebral lesions are essential.

Management and recurrence of spontaneous pneumothorax in children.

OBJECTIVE: No guideline clearly prescribes an approach to management of spontaneous pneumothorax in children. The objectives of this study were to evaluate practice variation in the management of spontaneous pneumothorax in children and its probability of recurrence. METHODS: This study was a retrospective chart review followed by a phone follow-up that included all children who had visited a tertiary care paediatric hospital for a first episode of spontaneous pneumothorax between 2008 and 2017. The primary outcomes were the management of pneumothorax (observation, oxygen, needle aspiration, intercostal chest tube, surgery) and the probability of recurrence. All charts were evaluated by a rater using a standardized report form and 10% of the charts were evaluated in duplicate. All children/families were contacted by phone to assess recurrence. The primary analyses were the proportions of each treatment modalities and recurrence, respectively. RESULTS: During the study period, 76 children were deemed eligible for the study. Among them, 59 had a primary spontaneous pneumothorax while 17 were secondary. The most common first therapeutic approaches were chest tube insertion (31), oxygen alone (27), and observation (14). A total of 54 patients were available for follow-up among whom a recurrence was observed in 28 (37% of the total cohort or 52% of available children). CONCLUSION: Chest tube insertion was the first line of treatment in about 40% of children with a first spontaneous pneumothorax. In this population, the recurrence probability is established between 37 and 52% and the majority occurs in the following months.

Traumatic sternal segment dislocation in a 3-year-old girl: Sonographic findings.

Sternal fractures are uncommon in the pediatric population, and sternal segment dislocations are even rarer with only a few cases reported in the literature. Most cases are secondary to direct trauma to the chest, but nontraumatic dislocations have been reported. The diagnosis can be difficult to establish with standard radiographs, while CT is not desirable in the pediatric population due to the associated irradiation. Ultrasound (US) can be used as the first-line modality to evaluate the sternum. We report the US findings associated with a case of traumatic sternal segment dislocation in a 3-year-old girl. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:45-49, 2017.

Ultrasound evaluation of foot deformities in infants.

Foot deformity in infants is the most common congenital musculoskeletal condition. A precise diagnosis can sometimes be impossible to establish clinically. Radiologic imaging plays a major role in the evaluation of musculoskeletal abnormalities. However conventional imaging techniques, such as plain radiographs of the foot, are of very little help in this age group because of the lack of ossification of the tarsal bones. US presents a significant advantage because it permits the visualization of cartilaginous structures. This leads to the detailed assessment of foot deformities in infants. Furthermore, US can also be used as a dynamic imaging modality. Different scanning views are beneficial to evaluate the complete anatomy of the foot; depending on the suspected clinical diagnosis, some planes are more informative to display the pathological features of a specific deformity. We describe the US findings of five of the most common foot deformities referred to our pediatric orthopedic clinic (clubfoot, simple metatarsus adductus, skewfoot, and oblique and vertical talus). For each deformity we propose a specific imaging protocol based on US to provide an accurate diagnosis. US is a complementary tool to the clinical examination for determining the diagnosis and the severity of the deformity and also for monitoring the efficacy of treatment. Radiologists investigating foot deformities in infants should consider using US for the detailed assessment of the foot in this age group.

Vascular Anomalies Associated with Esophageal Atresia and Tracheoesophageal Fistula.

OBJECTIVE: To report the incidence of congenital vascular anomalies in a cohort of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) while describing the clinical presentation, diagnosis, and consequences, and to evaluate the diagnostic value of esophagram in diagnosing an aberrant right subclavian artery (ARSA). METHODS: All patients born with EA/TEF between 2005 and 2013 were studied. Preoperative echocardiography reports, surgical descriptions of primary esophageal repair, and esophagrams were reviewed retrospectively. RESULTS: Of the 76 children born with EA/TEF included in this study, 14 (18%) had a vascular malformation. The incidence of a right aortic arch (RAA) was 6% (5 of 76), and that of an aberrant right subclavian artery (ARSA) was 12% (9 of 76). RAA was diagnosed in the neonatal period by echocardiography (4 of 5) or surgery (1 of 5), and ARSA was diagnosed by echocardiography (7 of 9) or later on the esophagram (2 of 9). Respiratory and/or digestive symptoms occurred in 9 of the 14 patients with vascular malformation. Both long-gap EA and severe cardiac malformations necessitating surgery were significantly associated with vascular anomalies (P<.05). The sensitivity of the esophagram for diagnosing ARSA was 66%, the specificity was 98%, the negative predictive value was 95%, and the positive predictive value was 85%. CONCLUSION: ARSA and RAA have an incidence of 12% and 6% respectively, in patients with EA/TEF. A computed tomography angioscan is recommended to rule out such malformations when stenting of the esophagus is indicated, before esophageal replacement surgery, and when prolonged (>2 weeks) use of a nasogastric tube is considered.

Physical impairments, activity limitations, and participation restrictions of childhood acute lymphoblastic leukemia survivors with and without hip osteonecrosis: a PETALE cohort study.

PURPOSE: Long-term musculoskeletal complications represent a growing burden for survivors of childhood acute lymphoblastic leukemia (cALL). This study aimed to describe physical impairments, activity limitations, and participation restrictions in a high-risk subgroup of cALL survivors of the PETALE cohort. METHODS: This cross-sectional study, using observational data from the PETALE cohort, included a subgroup of survivors who presented high-risk criteria for late effects. Outcomes measures consisted of hip magnetic resonance imaging, maximal isometric muscle strength (MIMS) or torque (MIMT), range of motion (ROM), Near Tandem Balance (NTB), 6-Minute Walk Test (6MWT), Five Time Sit-to-Stand Test (FTSST), and health-related quality of life. Descriptive statistics and regression analyses were performed. RESULTS: Survivors (n = 97, 24.2 ± 6.7 years old) showed limited grip strength, FTSST, and NTB performance compared to reference values (p < 0.001). Thirteen participants (14.6%, 18 hips) had hip osteonecrosis (ON) (53.8% male). Higher severity hip ON was found in female survivors (66.7% vs. 22.2%). Survivors with hip ON had reduced hip external rotation ROM compared to those without (p < 0.05). Relationships were found between MIMS and ROM outcomes (r = 0.32, p < 0.01) and with 6MWT (r = 0.39-0.41, p < 0.001). Our multiple linear regression model explained 27.6% of the variance of the 6MWT. CONCLUSIONS: Survivors in our subgroup had clinically significant physical impairments and activity limitations, and those with hip ON showed worst hip impairment outcomes. IMPLICATIONS FOR CANCER SURVIVORS: These findings emphasize the importance of long-term follow-up including physical therapy assessment to help early identification and management of physical impairments and activity limitations in survivors of cALL.

Ultrasound measurements in clubfoot treated with the Ponseti method and risk factors for recurrence: A retrospective study.

Background: Recurrence remains the main challenge in the treatment of clubfoot. The primary goal of this study is to determine if ultrasound measurements are associated with recurrence after successful management with the Ponseti method. Furthermore, other factors are identified which can be associated with recurrence of the deformity. Methods: Seventy-six infants (114 idiopathic clubfeet), all treated with the Ponseti technique were reviewed. All patients had an ultrasound evaluation by the same radiologist at the beginning of the treatment. Recurrence, defined as the need to return to Ponseti casting, was recorded at a mean follow-up of 5 years. Measurements of association with recurrence were obtained for the following ultrasound measures: the medial talonavicular displacement (MTa-N), the medial malleolus to navicular distance (MM-N), the talocalcaneal angle (Ta-C), and the distal tibial physis to proximal calcaneal apophysis distance (Ti-C). Subsequently, a multivariate logistic regression analysis modeling recurrence examined patients' characteristics, compliance, Achilles tenotomy, and ultrasound measurements. Results: Recurrence rate was 22% noted in 17 patients. On univariate analysis, relapse was associated with increased MTa-N (p = 0.038), decreased MM-N (p = 0.008), and decreased Ti-C (p = 0.023). On multivariate analysis, we identified the Ti-C as the only ultrasound measurement significantly associated with recurrence (p = 0.026). Other significant predictors for relapse in this study were noncompliance with orthosis (OR = 139.0 (95% CI: 8.7-2224.0), p < 10-3), and omitting percutaneous Achilles tenotomy in clubfoot treatment (OR = 23.9 (95% CI: 1.2-493.6), p = 0.041). Conclusion: The Ti-C sonographic measurement at the start of treatment can be a useful adjunct to help identify high-risk patients for recurrence of deformity. Non-compliance with bracing and omitting percutaneous Achilles tenotomy are also predictive factors. Level of evidence: Prognostic study, Level III.

Predictors of Vertebral Deformity in Long-Term Survivors of Childhood Acute Lymphoblastic Leukemia: The PETALE Study.

BACKGROUND: The prevalence of vertebral deformities in long-term survivors of childhood acute lymphoblastic leukemia (ALL) is unknown. Our objectives were to identify the prevalence of vertebral deformities and their risk factors among long-term childhood ALL survivors. METHODS/RESULTS: We recruited 245 (49% male) long-term childhood ALL survivors from the Preventing Late Adverse Effects of Leukemia Cohort (French-Canadian ALL survivors treated between the years 1987 and 2010 with the Dana Farber Cancer Institute clinical trials protocols, who did not experience disease relapse and/or receive hematopoietic stem cell transplant). Median age at recruitment was 21.7 years (range, 8.5-41) and median time since diagnosis was 15.1 years (range, 5.4-28.2). All participants underwent spine radiograph and dual-energy X-ray absorptiometry scans. The prevalence of vertebral deformity was 23% with 88% classified as grade 1 according to the Genant method. The majority of vertebral deformities were clinically silent. Regression analysis confirmed male sex (risk ratio [RR] = 1.94; 95% confidence interval [CI], 1.16-3.24; P = 0.011), higher glucocorticoid cumulative dose (RR = 1.05; 95% CI, 1.00-1.10; P = 0.032), and back pain (RR = 2.44; 95% CI, 1.56-3.84; P < 0.001) as predictors of prevalent vertebral deformity. Sex differences in vertebral deformity predictors emerged. CONCLUSIONS: We report a significant prevalence of vertebral deformities in this young cohort. Male sex, cumulative glucocorticoid dose, and back pain were identified as predictors of prevalent vertebral deformity. Back pain emerging as a strong predictor of vertebral deformity underscores the importance of ongoing bone health surveillance in survivors with persistent vertebral deformities treated with these earlier protocols.

Enzyme replacement therapy in pediatric patients with Gaucher disease: what should we use as maintenance dosage?

INTRODUCTION: No consensus exists on the minimal dose of enzyme replacement therapy (ERT) effective to maintain therapeutic goals in pediatric Gaucher patients. OBJECTIVE: Evaluate the efficacy of low dosage ERT to maintain treatment goals. RESULTS: Six patients had a maintenance dose of 30-35U/kg/month. All patients, with the exception of one L444P/L444P homozygote, maintained therapeutic goals. DISCUSSION: A low maintenance dose may be adequate in most pediatric patients. L444P homozygotes may require a higher maintenance dosage.

Segmentation of the spinous process and its acoustic shadow in vertebral ultrasound images.

Spinal ultrasound imaging is emerging as a low-cost, radiation-free alternative to conventional X-ray imaging for the clinical follow-up of patients with scoliosis. Currently, deformity measurement relies almost entirely on manual identification of key vertebral landmarks. However, the interpretation of vertebral ultrasound images is challenging, primarily because acoustic waves are entirely reflected by bone. To alleviate this problem, we propose an algorithm to segment these images into three regions: the spinous process, its acoustic shadow and other tissues. This method consists, first, in the extraction of several image features and the selection of the most relevant ones for the discrimination of the three regions. Then, using this set of features and linear discriminant analysis, each pixel of the image is classified as belonging to one of the three regions. Finally, the image is segmented by regularizing the pixel-wise classification results to account for some geometrical properties of vertebrae. The feature set was first validated by analyzing the classification results across a learning database. The database contained 107 vertebral ultrasound images acquired with convex and linear probes. Classification rates of 84%, 92% and 91% were achieved for the spinous process, the acoustic shadow and other tissues, respectively. Dice similarity coefficients of 0.72 and 0.88 were obtained respectively for the spinous process and acoustic shadow, confirming that the proposed method accurately segments the spinous process and its acoustic shadow in vertebral ultrasound images. Furthermore, the centroid of the automatically segmented spinous process was located at an average distance of 0.38 mm from that of the manually labeled spinous process, which is on the order of image resolution. This suggests that the proposed method is a promising tool for the measurement of the Spinous Process Angle and, more generally, for assisting ultrasound-based assessment of scoliosis progression.

Diagnostic imaging of spinal deformities: reducing patients radiation dose with a new slot-scanning X-ray imager.

STUDY DESIGN: Clinical trial comparing image quality and entrance dose between Biospace EOS system, a new slot-scanning radiographic device, and a Fuji FCR 7501S computed radiography (CR) system for 50 patients followed for spinal deformities. OBJECTIVE: Based on their physical properties, slot-scanners show the potential to produce image quality comparable to CR systems using less radiation. This article validates this assertion by comparing a new slot-scanner to a CR system through a wide-ranging evaluation of dose and image quality for scoliosis examinations. SUMMARY OF BACKGROUND DATA: For each patient included in this study, lateral and posteroanterior images were acquired with both systems. For each system, entrance dose was measured for different anatomic locations. METHODS: Dose and image quality being directly related, comparable images were obtained using the same radiograph tube voltage on both systems while tube currents were selected to match signal-to-noise ratios on a phantom. Different techniques were defined with respect to patient's thickness about the iliac crests. Given dose amplitudes expected for scoliosis examinations, optically stimulated luminescence dosimeters were chosen as optimal sensors. Two radiologists and 2 orthopedists evaluated the images in a randomized order using a questionnaire targeting anatomic landmarks. Visibility of the structures was rated on a 4 level scale. Image quality assessment was analyzed using a Wilcoxon signed-rank tests. RESULTS: Average skin dose was reduced from 6 to 9 times in the thoracoabdominal region when using the slot-scanner instead of CR. Moreover, image quality was significantly better with EOS for all structures in the frontal view (P < 0.006) and lateral view (P < 0.04), except for lumbar spinous processes, better seen on the CR (P < 0.003). CONCLUSION: We established that the EOS system offers overall enhanced image quality while reducing drastically the entrance dose for the patient.

Wireless capsule endoscopy for obscure small-bowel disorders: final results of the first pediatric controlled trial.

BACKGROUND AND AIMS: Obscure small-bowel disorders are jejunal and ileal lesions undiagnosed by traditional imaging techniques (endoscopic, radiologic). We evaluated the diagnostic usefulness and safety of capsule endoscopy for obscure small-bowel disorders in children and adolescents. METHODS: Comparative, prospective, self-controlled trials in patients (age, 10-18 y) suspected to have either small-bowel Crohn's disease, polyps, or obscure gastrointestinal (GI) bleeding. Capsule results were compared with the diagnostic imaging studies normally used in this age group. RESULTS: Among 20 patients suspected of Crohn's disease, multiple lesions consistent with this diagnosis were observed by capsule endoscopy in 50%. Small-bowel Crohn's disease was ruled out in 8 patients. Eosinophilic enteropathy was found in 2 others. For polyp detection (n = 6), capsule endoscopy yielded 100% concordance with the control studies when analyzed per patient. However, capsule endoscopy revealed a greater number (50%) of polyps. Among patients with obscure bleeding (n = 4), the capsule examination confirmed a diagnosis of vascular malformations in 3. Capsule endoscopy more accurately identified the precise source of bleeding compared with angiography. All 30 capsule studies were well tolerated, although 1 capsule was retained owing to an inflammatory stenosis. The capsule eventually was expelled after corticosteroid therapy. CONCLUSIONS: Capsule endoscopy correctly diagnosed or excluded a bleeding source, small-bowel polyps, or Crohn's disease of the small bowel in 29 of 30 patients. Capsule endoscopy permits an accurate, noninvasive approach for diagnosing obscure small bowel lesions in children over the age of 10.

Fetal hydronephrosis: is there hope for consensus?

This review article aims at summarizing the data regarding fetal and neonatal hydronephrosis, at correlating controversial data with the differences in the practice of obstetrical sonography from one country to another, and finally, at presenting our own criteria for fetal renal collecting system dilatation along with our own guidelines of postnatal investigation.