Case Series: Role of Pill Esophagram to Identify Pediatric Patients With Eosinophilic Esophagitis Amenable to Therapeutic Dilation.

Eosinophilic esophagitis (EoE) is a chronic disease of the esophagus that leads to esophageal remodeling. Dysphagia is a common symptom that likely results from the inflammatory process or remodeling. Identifying patients that may benefit from dilation can be challenging because of difficulties in detecting subtle narrowing in patients with EoE. Here we report the benefits of a pill esophagram in the detection of esophageal narrowing in EoE. We identified a series of children with EoE and symptoms of dysphagia who underwent barium esophagram with a barium-coated pill to assess symptoms. Three subjects had a normal fluoroscopic esophagram but had pill retention for greater than 5 minutes. Subsequent esophagoscopy and esophageal dilation revealed mucosal rent after dilation. Subtle esophageal narrowing may not be captured with barium esophagram alone in children with EoE and dysphagia. Use of the barium pill in symptomatic patients can assist in identifying patients who may benefit from esophageal dilation.

Implementation of a Timed Barium Esophagram Protocol for Assessment of Esophageal Function in Children.

OBJECTIVES: Timed barium esophagram (TBE) is a fluoroscopic study that is widely employed as an adjunctive tool for diagnosing esophageal emptying disorders in adults (eg, achalasia, esophagogastric junction outflow obstruction [EGJOO]) and for following response to treatment. We aimed to describe the characteristics and feasibility of a pediatric TBE protocol and provide a first report of the potential value of TBE for assessment of esophageal emptying in the pediatric population. METHODS: Retrospective chart review of pediatric patients at a tertiary pediatric hospital who underwent TBE from October 2017 to October 2019. Patient and test characteristics were summarized using descriptive statistics. Results from patients who had both TBE and high-resolution esophageal manometry (HRM) were used to generate ROC curves for TBE to identify esophageal emptying disorders. RESULTS: Twenty-two patients underwent 25 TBE. Fourteen of 23 (61%) received 150 mL barium volume per protocol. Nearly half (42%) of subjects could tolerate ingesting barium within 20 seconds. Nine individuals underwent HRM. The sensitivity of standard adult TBE criteria (1 cm barium column height at 5 minutes) to detect emptying disorder was 100%, specificity 40%. A modified diagnostic cutoff (1.6 cm height at 5 minutes) offered 100% sensitivity, 80% specificity. CONCLUSIONS: TBE is feasible and should be considered an adjunctive noninvasive screen for impaired esophageal emptying in children. There was heterogeneous adherence to protocol for timing and volume of barium; however, studies remained interpretable. This population may benefit from different diagnostic cutoffs than adults, and clinical judgment should be used until specific diagnostic cutoffs are determined in children.

Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two-site retrospective cohort study.

BACKGROUND: Castleman disease (CD) is an uncommon lymphoproliferative disorder that is rare in pediatric populations; the literature describing this population is sparse. We sought to describe pediatric CD, including unicentric CD (UCD) and human herpes virus-8 (HHV8)-negative multicentric CD (MCD), in a multi-institutional cohort. METHODS: We retrospectively reviewed 24 patients, aged 0 to 26 years at diagnosis, who were diagnosed with CD between January 1, 2005, and May 16, 2017, at two tertiary children's hospitals. Demographic and clinical data were collected. RESULTS: Most patients (75%, 18/24) presented with UCD. All patients with MCD were HHV8-negative. The most common histopathologic variant was hyaline vascular (75%, 18/24). Plasma cell variant occurred in 33% (2/6 [95% confidence intervals (CI), 4-78%]) of patients with HHV8-negative MCD and 17% (3/18 [95% CI, 4-41%]) of patients with UCD. Systemic symptoms were present in 4 of 6 of patients with HHV8-negative MCD and 8 of 18 of patients with UCD. Anemia and laboratory inflammation occurred in both UCD and MCD patients, with nonsignificantly higher rates of anemia and elevated C-reactive protein in MCD patients. All but two UCD patients underwent gross total resection as definitive therapy. Among HHV8-negative MCD patients, a combination of resection, chemotherapy, and immunotherapy was used. No UCD patients and three of six HHV8-negative MCD patients experienced disease progression/relapse prior to lasting remission. There were no deaths. CONCLUSION: Pediatric patients with CD most commonly have unicentric, hyaline vascular variant disease. Pediatric patients with both UCD and MCD commonly have systemic inflammation and, despite risk of progression/relapse in MCD patients, ultimately have excellent survival.

Appropriate Needle Length for Emergent Pediatric Needle Thoracostomy Utilizing Computed Tomography.

Objective: Needle thoracostomy is a life-saving procedure. Advanced Trauma Life Support guidelines recommend insertion of a 5 cm, 14-gauge needle for pneumothorax decompression. High-risk complications can arise if utilizing an inappropriate needle size. No study exist evaluating appropriate needle length in pediatric patients. Utilizing computed tomography (CT), we determined the needle length required to access the pleural cavity in children matched to Broselow™ Pediatric Emergency Tape color. Methods: Three investigators reviewed chest CTs of children <13 years of age obtained between 2010 and 2015. Patient exclusions included those with a chest wall mass, muscle disease, pectus deformity, anasarca, prior open thoracotomy, inadequate imaging, or missing height documentation. We established 4 groups based upon Broselow™ color as determined by recorded height. Investigators, trained by a pediatric board-certified radiologist, obtained standardized CT measurements of chest wall thickness at 4 points: right/left second intercostal space at the midclavicular line (ICS-MCL) and right/left fourth intercostal space in the anterior axillary line (ICS-AAL). Our outcome was the median chest wall thickness and 95% confidence intervals for each Broselow grouping and anatomic site. Results: A total of 273 chest CTs were reviewed, of which 23 were excluded, for a resultant study population of 250 scans and 498 total measurements. Median patient age was 4 years, 52.8% were male. Children measuring Broselow Gray/Pink (<68 cm), had a median chest wall thickness at the 2nd ICS-MCL of 1.57 cm (95% CI 1.42 cm, 1.72 cm), 4th ICS-AAL 1.67 cm (95% CI 1.48 cm, 1.86 cm). Broselow Red/Purple (68.1-90 cm): 2nd ICS-MCL of 1.96 cm (95% CI 1.84 cm, 2.08 cm), 4th ICS-AAL 1.73 cm (95% CI 1.62 cm, 1.84 cm). Broselow Yellow/White (90.1-115cm): 2nd ICS-MCL of 2.12 cm (95% CI 2.03 cm, 1.22 cm), 4th ICS-AAL 1.91 cm (95% CI 1.8 cm, 2.01 cm). Broselow Blue/Orange/Green (>115.1 cm): 2nd ICS-MCL of 2.45 cm (95% CI 2.3 cm, 2.6 cm), 4th ICS-AAL 2.19cm (95% CI 2.02 cm, 2.36 cm). Conclusion: Median chest wall thickness varies little by height or location in children <13 years of age. The standard 5-cm needle is twice the chest wall thickness of most children. Commercially available 14 g or 16 g standard-length 3.8 cm (1½ inch) needles are of adequate length to access the pleural cavity, regardless of height as measured by Broselow LBT.

Radiological features of healing in newborn clavicular fractures.

OBJECTIVES: Given the frequency of abusive fractures among infants, and the lack of research and or evidence for the phases of fracture healing seen in this age group, this study aims to describe a timetable of radiological features of fracture healing among infants in the first months of life. METHODS: We completed a retrospective cross-sectional time-series study of birth-related clavicle fractures from 2006-2013. A total of 108 digital images were available for review from 61 infants. The presence or absence of four features of healing including periosteal reaction, callus formation, bridging callus and remodelling were scored by three radiologists. RESULTS: The level of agreement between the radiologists was good to high (0.60-0.90). Features of healing were first seen at 7 days (periosteal reaction), 11 days (callus), 20 days (bridging) and 35 days (remodelling), respectively. The peak periods that each feature was present are as follows: periosteal reaction 11-42 days, callus 12-61 days, bridging 22-63 days and remodelling 49-59 days. CONCLUSIONS: Birth-associated clavicle fractures in infants follow a logical progression of healing changes. Understanding the expected progression and timing of fracture healing may be helpful as it pertains to the timing of injury in cases of abuse in infants. KEY POINTS: • Large study describing the time frames of fracture healing in young infants. • Features of fracture healing develop in a logical progression. • Evidence provided for determining fractures are consistent with a proposed time frame. • It is of critical importance to have sound evidence for the dating of fractures.

18F-FDG PET/CT evaluation of children and young adults with suspected spinal fusion hardware infection.

BACKGROUND: Evaluation of the child with spinal fusion hardware and concern for infection is challenging because of hardware artifact with standard imaging (CT and MRI) and difficult physical examination. Studies using (18)F-FDG PET/CT combine the benefit of functional imaging with anatomical localization. OBJECTIVE: To discuss a case series of children and young adults with spinal fusion hardware and clinical concern for hardware infection. These people underwent FDG PET/CT imaging to determine the site of infection. MATERIALS AND METHODS: We performed a retrospective review of whole-body FDG PET/CT scans at a tertiary children's hospital from December 2009 to January 2012 in children and young adults with spinal hardware and suspected hardware infection. The PET/CT scan findings were correlated with pertinent clinical information including laboratory values of inflammatory markers, postoperative notes and pathology results to evaluate the diagnostic accuracy of FDG PET/CT. An exempt status for this retrospective review was approved by the Institution Review Board. RESULTS: Twenty-five FDG PET/CT scans were performed in 20 patients. Spinal fusion hardware infection was confirmed surgically and pathologically in six patients. The most common FDG PET/CT finding in patients with hardware infection was increased FDG uptake in the soft tissue and bone immediately adjacent to the posterior spinal fusion rods at multiple contiguous vertebral levels. Noninfectious hardware complications were diagnosed in ten patients and proved surgically in four. Alternative sources of infection were diagnosed by FDG PET/CT in seven patients (five with pneumonia, one with pyonephrosis and one with superficial wound infections). CONCLUSION: FDG PET/CT is helpful in evaluation of children and young adults with concern for spinal hardware infection. Noninfectious hardware complications and alternative sources of infection, including pneumonia and pyonephrosis, can be diagnosed. FDG PET/CT should be the first-line cross-sectional imaging study in patients with suspected spinal hardware infection. Because pneumonia was diagnosed as often as spinal hardware infection, initial chest radiography should also be performed.

Diffuse cavitary lung lesions.

An 11-year-old girl presented with a 2-month history of progressively worsening cough, daily fevers, and weight loss. A chest radiograph revealed multiple cystic cavitary lung lesions. An extensive infectious work-up was negative. Chest CT verified multiple cavitary lung lesions bilaterally, and [F-18]2-fluoro-2-deoxy-D-glucose ((18)F-FDG) positron emission tomography with CT (PET/CT) showed increased uptake in the lung lesions as well as regional lymph nodes. Subsequent biopsy of an involved lymph node confirmed classical Hodgkin lymphoma, nodular sclerosis type. This case represents an unusual presentation for a child with Hodgkin lymphoma and demonstrates a role for (18)F-FDG PET/CT in evaluating a child with cavitary lung lesions.

Model to estimate abdominal wall thickness in children undergoing placement or replacement of gastrostomy devices.

OBJECTIVES: Abdominal wall thickness (AWT) is a key measurement when placing or replacing low profile gastrostomy devices. This measurement varies, depending on nutritional status and body habitus. We developed a mathematical model to estimate AWT using a compendium of body measurements. METHODS: Ultrasonography was used to measure AWT at the initial gastrostomy site in subjects aged 22 days to 24 years old. Other body measurements (height, weight, waist circumference and distance from xiphisternum to pubis) were also obtained. Multiple linear regression was used to develop two separate models using age of 2 years to separate the groups. For analysis, AWT is log transformed. RESULTS: Data from 97 subjects were used for analysis. The final model for those ≤24 months old is the following: ln(Estimated AWT) = -1.255 + 0.082*(1 if age 3-24 months, 0 if <3 months) + 0.022*(waist circumference in cm). The final model for those >24 months old is the following: ln(Estimated AWT) = -1.335 + 0.271*(1 if age >84 months, 0 if 24-84 months) + 0.082*(BMI) CONCLUSION: This model to estimate AWT is useful for determining the length of a gastrostomy device at initial placement and with subsequent changes. More data are needed to refine and further validate the model. LEVEL OF EVIDENCE: Level IV, study of prognostic test.

Simultaneous occurrence of right adrenocortical tumor and left adrenal neuroblastoma in an infant with Beckwith-Wiedemann syndrome.

Children with Beckwith-Wiedemann syndrome (BWS) have increased risk for development of embryonal tumors. We present the case of an infant with BWS who has hypomethylation of LIT1 gene in the 11p15.5 chromosomal region and at 6 months of age presented with simultaneous occurrence of neuroblastoma arising from the left adrenal gland and a right adrenocortical tumor. She underwent surgical resection of both tumors and remains tumor free 18 months after surgery.