Consensus Minimum MRI Protocol for the Child With Acute Recurrent or Chronic Pancreatitis.

Imaging plays an important role in the diagnosis and follow-up of children with acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP). Consensus is lacking for a minimum MRI protocol for the child with known or suspected ARP or CP. Lack of standardization contributes to variable diagnostic performance and hampers application of uniform interpretive criteria for clinical diagnosis and multicenter research studies. We convened a working group to achieve consensus for a minimum MRI protocol for children with suspected ARP or CP. The group included eight pediatric radiologists experienced in interpreting MRI for pediatric pancreatitis and one medical pancreatologist and functioned from November 2022 to March 2023. Existing clinical protocols were summarized across sites represented by group members, and commonly used sequences guided the group's discussion. The final consensus minimum MRI protocol includes five noncontrast sequences and two postcontrast sequences (which are required only in select clinical scenarios). The working group also provides recommended acquisition parameters, sequence-specific technical suggestions, and general recommendations for optimal imaging technique. We recommend that all sites imaging children with ARP and CP for clinical care, and particularly those engaged in cooperative group trials for pancreatitis, ensure that their local protocol includes these minimum sequences.

Congenital Infantile Fibrosarcoma Involving Pelvic Wall and Thigh Soft Tissues and Placenta, Presenting with Coagulopathy.

Infantile fibrosarcoma (IF) is a well characterized pediatric malignancy marked by gene rearrangements involving members of the NTRK family. In this report, we present a case of IF that presented in the inguinal region-proximal thigh and was initially thought to be a kaposiform hemangioendothelioma (KHE) because it presented with a bleeding diathesis thought to be Kasabach-Merritt phenomenon (KMP). Subsequently, the placental examination showed a neoplasm in the perivascular-subendothelial space of stem villi, initially thought to be myofibromatosis. Ultimately, a biopsy of the thigh mass showed IF with an NTRK3-ETV6 fusion. Subsequent FISH analysis of the placenta showed an ETV6 rearrangement confirming that it was also IF. Review of the laboratory studies suggests that disseminated intravascular coagulation may have been more likely than KMP, highlighting the difficulty in making this distinction in some cases. We believe this to be the first report of an IF presenting in a soft tissue site and the placenta, and discuss the possible mechanisms that could have allowed the IF in the leg to spread to the placenta.

Interobserver Agreement for CT and MRI Findings of Chronic Pancreatitis in Children: A Multicenter Ancillary Study Under the INSPPIRE Consortium.

BACKGROUND. Imaging findings represent key criteria for diagnosing chronic pancreatitis in children. Understanding radiologists' agreement for imaging findings is critical to standardizing and optimizing diagnostic criteria. OBJECTIVE. The purpose of this study is to evaluate the interobserver agreement among experienced pediatric radiologists for subjective, quantitative, and semiquantitative imaging findings of chronic pancreatitis in children. METHODS. In this retrospective study, CT or MRI examinations performed in children with chronic pancreatitis were submitted by six sites participating in the INSPPIRE (International Study Group of Pediatric Pancreatitis: In Search for a Cure) Consortium. One pediatric radiologist from each of the six sites reviewed examinations; three of the radiologists independently reviewed all CT examinations, and the other three radiologists independently reviewed all MRI examinations. Reviewers recorded 13 categoric imaging findings of chronic pancreatitis and measured pancreas thickness and pancreatic duct diameter. Agreement was assessed using kappa coefficients for the categoric variables and intraclass correlation coefficients (ICCs) for the continuous variables. RESULTS. A total of 76 CT and 80 MRI examinations performed in 110 children (65 girls and 45 boys; mean age, 11.3 ± 4.6 [SD] years) were reviewed. For CT, kappa coefficients for categoric findings ranged from -0.01 to 0.81, with relatively high kappa coefficients noted for parenchymal calcifications (κ = 0.81), main pancreatic duct dilatation (κ = 0.63), and atrophy (κ = 0.52). ICCs for parenchymal thickness measurements ranged from 0.57 in the pancreas head to 0.80 in the body and tail. The ICC for duct diameter was 0.85. For MRI, kappa coefficients for categoric findings ranged from -0.01 to 0.74, with relatively high kappa coefficients noted for main duct irregularity (κ = 0.74), side branch dilatation (κ = 0.70), number of dilated side branches (κ = 0.65), and main duct dilatation (κ = 0.64); kappa coefficient for atrophy was 0.52. ICCs for parenchymal thickness measurements ranged from 0.53 for the neck and body individually to 0.68 in the tail. ICC for duct diameter was 0.77. CONCLUSION. Interobserver agreement was fair to moderate for most CT and MRI findings of chronic pancreatitis in children. CLINICAL IMPACT. This study highlights challenges for the imaging diagnosis of pediatric chronic pancreatitis. Standardized and/or objective criteria are needed given the importance of imaging in diagnosis.

Successful management of an arteriovenous malformation with trametinib in a patient with capillary-malformation arteriovenous malformation syndrome and cardiac compromise.

Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is an autosomal dominant condition characterized by multifocal, noncontiguous pink patches on the skin that often have a surrounding pale halo. In some cases, an association with a fast flow, arteriovenous malformation (AVM) can be identified. Here, we describe a case report of a 16-year-old woman with CM-AVM syndrome and significant cardiac compromise successfully treated with trametinib, a mitogen-activated protein kinase (MEK) inhibitor.

Magnetic resonance imaging glossary of findings of pediatric pancreatitis and the revised Atlanta classification.

While still uncommon, the incidence of acute pancreatitis in children has been increasing over the last two decades. The Atlanta classification for acute pancreatitis, developed for adults, stratifies cases of acute pancreatitis based on imaging and clinical criteria. This classification scheme allows for standardized use of terminology to facilitate treatment and prognostication. Although US and CT should be used in critical or unstable patients, MRI is an ideal imaging modality in pediatric patients with acute pancreatitis because of its ability to characterize tissue without ionizing radiation. We review MRI examples specific to Atlanta classification terminology in pediatric patients. Chronic pancreatitis has also been increasingly diagnosed in children, and imaging plays a key role in the diagnosis and management of this insidious disease. MRI with magnetic resonance cholangiopancreatography is the optimal modality for assessing the pancreas in a child with known or suspected chronic pancreatitis because it provides tissue characterization and high-contrast imaging of the pancreatic duct without the use of invasive instrumentation or ionizing radiation. We also review and demonstrate accepted MRI findings of chronic pancreatitis.

Current State of Imaging of Pediatric Pancreatitis: AJR Expert Panel Narrative Review.

Pancreatitis is as common in children as it is in adults, though causes and accepted imaging strategies differ in children. In this narrative review we discuss the epidemiology of childhood pancreatitis and key imaging features for pediatric acute, acute recurrent, and chronic pancreatitis. We rely heavily on our collective experience in discussing advantages and disadvantages of different imaging modalities; practical tips for optimization of ultrasound, CT, and MRI with MRCP in children; and image interpretation pearls. Challenges and considerations unique to imaging pediatric pancreatitis are discussed, including timing of imaging, role of secretin-enhanced MRCP, utility of urgent MRI, severity prediction, autoimmune pancreatitis, and best methods for serial imaging. We suggest a methodical approach to pancreatic MRI interpretation in children and have included a sample structured report, and we provide consensus statements according to our experience imaging children with pancreatitis.

Radiologic Assessment of Skull Fracture Healing in Young Children.

BACKGROUND: Skull fractures are commonly seen after both accidental and nonaccidental head injuries in young children. A history of recent trauma may be lacking in either an accidental or nonaccidental head injury event. Furthermore, skull fractures do not offer an indication of the stage of healing on radiologic studies because they do not heal with callus formation as seen with long bone fractures. Thus, a better understanding on the timing of skull fracture resolution may provide guidance on the medical evaluation for accidental or nonaccidental head injury. OBJECTIVE: The aim of the study was to determine the time required for radiographic skull fracture resolution in children younger than 24 months. METHODS: This was a retrospective observational analysis of children younger than 24 months referred with skull fractures between January 2008 and December 2012. Analysis included children with accidental head injuries with a known time interval since injury and a negative skeletal survey who underwent serial radiographic studies. Complete healing of a skull fracture was defined as resolution of fracture lucency by radiograph. RESULTS: Of the 26 children who met inclusion criteria, 11 (42.3%) demonstrated resolution of skull fracture(s) on follow-up imaging. Fracture resolution on radiologic studies ranged from 2 to 18 weeks. Twelve fractures in 10 children demonstrated fracture resolution at 10 or more weeks after injury. CONCLUSIONS: Healing or resolution of a skull fracture can take months in children younger than 24 months. With the high variability in skull fracture presentation and large window to fracture resolution, unexplained or multiple skull fractures in children younger than 24 months may be the result of a single or multiple events of head trauma.

Multifocal infantile hemangiomas associated with concomitant giant hepatic rapidly involuting congenital hemangioma.

We present a 9-day-old girl with multifocal cutaneous and hepatic infantile hemangiomas as well as a hepatic rapidly involuting congenital hemangioma. These two distinct vascular tumors have rarely been reported to co-occur. We additionally review the sonographic features that distinguish a hepatic congenital hemangioma from the hepatic infantile hemangioma.

A PRESing case of visual changes and confusion.

Visual disturbances are an uncommon pediatric chief complaint. Usually, after a complete ocular exam including visual acuity, most causes are benign and not life-threatening. Children with abnormal visual complaints who have underlying medical conditions, such as SLE or other autoimmune conditions, a recipient of a transplant, renal disease, and even eclampsia require closer scrutiny. We report a 10-year-old female with a history of systemic lupus erythematosus complicated by hypertension and cardiomyopathy secondary to lupus who presented to the emergency department with a history of vision loss and headache. Head computer tomography demonstrated findings of posterior reversible encephalopathy syndrome (PRES). PRES is a clinical disease associated with cranial radiological findings of heterogenous etiologies that is often reversible. Prompt recognition and treatment are important in preventing permanent damage, long term morbidity and even death.

Fibrous hamartoma of infancy associated with bony remodeling and muscle atrophy.

Fibrous hamartoma of infancy (FHI) is a benign, poorly demarcated, unencapsulated hamartoma contiguous with surrounding fat. This case highlights pronounced bony remodeling and muscle atrophy associated with FHI in a 6-week-old girl. Emphasis is placed on pathologic diagnosis and mechanisms, treatment considerations, and the multidisciplinary approach utilized in the management of the disorder.

Liver Doppler Findings in Pediatric Patients After Total Pancreatectomy and Islet Autotransplantation.

OBJECTIVES: This study was intended to establish normal values for velocities in the hepatic artery and portal veins in pediatric patients after total pancreatectomy and islet autotransplantation (TPIAT). METHODS: A retrospective review was performed of liver Doppler studies in pediatric patients after TPIAT over 6 years at an academic children's hospital. Doppler velocities in the liver vasculature and the hepatic artery resistive index were recorded. RESULTS: Sixty-five pediatric patients were evaluated. There were no cases of portal vein thrombosis or other hepatic vascular complications. The mean hepatic artery peak systolic velocity was 157.6 cm/s (SE, 60.9 cm/s), with a mean resistive index of 0.57 (SE, 0.09). The mean main portal vein velocity was 31.9 cm/s (SE, 12.9 cm/s). CONCLUSIONS: Portal vein thrombosis is rare in pediatric patients after TPIAT. A wide range of hepatic artery and portal vein velocities are encountered in children immediately after TPIAT without hepatic vascular complications and can be considered normal.

Total Pancreatectomy With Islet Autotransplantation Resolves Pain in Young Children With Severe Chronic Pancreatitis.

OBJECTIVES: Fear of diabetes and major surgery may prohibit referral of young children severely affected by pancreatitis for total pancreatectomy with islet autotransplant (TPIAT). We evaluated outcomes in our youngest TPIAT recipients, 3 to 8 years of age at surgery. METHODS: Medical records were reviewed for 17 children (9 girls) ages 8 years or younger undergoing TPIAT from 2000 to 2014. Most (14/17) had genetic risk factors for pancreatitis. Since 2006, TPIAT recipients were followed prospectively with health questionnaires including assessments of pain and narcotic use, and scheduled hemoglobin A1c (HbA1c) and mixed-meal tolerance tests (6 mL/kg Boost HP) before surgery, and at regular intervals after. Patients are 1 to 11 years post-TPIAT (median 2.2 years). Data are reported as median (25th, 75th percentile). RESULTS: All had relief of pain, with all 17 patients off narcotics at most recent follow-up. Hospitalization rates decreased from 5.0 hospitalization episodes per person-year of follow-up before TPIAT, to 0.35 episodes per person-year of follow-up after TPIAT. Fourteen (82%) discontinued insulin, higher than the observed insulin independence rate of 41% in 399 patients older than 8 years of age undergoing TPIAT over the same interval (P = 0.004). Median post-TPIAT HbA1c was 5.9% (5.6%, 6.3%), and within patient post-TPIAT mean HbA1c was ≤6.5% for all but 2 patients. CONCLUSIONS: Young children with severe refractory chronic pancreatitis may be good candidates for TPIAT, with high rates of pain relief and insulin independence, and excellent glycemic control in the majority.

Two case reports of retained steel insulin pump infusion set needles.

Insulin pumps are common in the management of type 1 diabetes (T1D). We report two cases of metal insulin infusion set needles which broke off the tubing and remained embedded in the soft tissue of two boys with T1D (five needles in one case, and one needle in the other). The patient with five retained needles was asymptomatic and had a normal physical examination, and the missing needles were only detected using pelvic X-ray; the second patient had only mild discomfort. While these are the first such cases reported in the medical literature, there may be other cases which have gone unnoticed, suggesting the potential need to explore the safety of this product further.