Cytochemical Characterization of Cerebrospinal Fluid Macrophage Inclusions in Pediatric Patients Receiving Intrathecal Nusinersen (SPINRAZA®) for Spinal Muscular Atrophy.

Introduction: Spinal muscular atrophy (SMA) is a debilitating neuromuscular disorder caused by biallelic deletion of the SMN1 gene. Nusinersen, an antisense oligonucleotide delivered intrathecally, binds to the pre-mRNA of SMN1's pseudogene, SMN2, to prevent exon skipping and produce functional SMN protein to compensate for the deficiency caused by SMN1 deletion.

Case Presentation: We reviewed 15 cerebrospinal fluid (CSF) cytology specimens from 8 patients receiving nusinersen for SMA.

A comprehensive institutional overview of intrathecal nusinersen injections for spinal muscular atrophy.

Background: Spinal muscular atrophy (SMA) is an autosomal-recessive neuromuscular disorder resulting in progressive muscle weakness. In December 2016, the U.S.

Safety and effectiveness of percutaneous cholecystostomy in critically ill children who are immune compromised.

Background: Acalculous cholecystitis is known to develop in critically ill patients without cystic duct obstruction. In the past, treatment for acalculous cholecystitis has been cholecystectomy; however, many children who are critically ill are Percutaneous cholecystostomy is likely the procedure of choice in this subgroup of patients.

Objective: To assess the safety and effectiveness of percutaneous cholecystostomy in critically ill and immune-compromised children with acalculous cholecystitis.

Benefit of fluoroscopically guided intraarticular, long-acting corticosteroid injection for subtalar arthritis in juvenile idiopathic arthritis.

Background: Children with arthritis may endure a lifetime of disfigurement, dysfunction, and pain if acute inflammation progresses to chronic changes in the joint cartilage and underlying bone. Intraarticular steroids have become an integral component of treatment, but at times are difficult to deliver to joints, such as the subtalar joint, that have complex anatomies.

Objective: We describe our technique and outcomes using fluoroscopically guided intraarticular subtalar steroid injection in patients with active symptoms of juvenile idiopathic arthritis (JIA).

CT-guided percutaneous steroid injection for management of inflammatory arthropathy of the temporomandibular joint in children.

Objective: The purposes of this study were to retrospectively review an injection technique, to develop a grading system for evaluation of imaging findings, and to report preliminary outcome related to percutaneous CT-guided steroid injection into the temporomandibular joints of children with inflammatory arthropathy.

Conclusion: CT-guided steroid injection into the temporomandibular joint of children with inflammatory arthropathy results in clinical and imaging improvement in a substantial proportion of children treated.

Benefit of intraarticular corticosteroid injection under fluoroscopic guidance for subtalar arthritis in juvenile idiopathic arthritis.

Objective: To determine the demographics of subtalar arthritis, the response to intraarticular corticosteroid injection, and the injection complication rate in a clinic sample of children with juvenile idiopathic arthritis (JIA).

Methods: A chart review was performed of all patients at a tertiary medical center who underwent subtalar corticosteroid injection during the past 5 years. Injection of 1 ml of triamcinolone hexacetonide or acetonide into the midsubtalar joint was performed using a lateral oblique approach under fluoroscopic guidance.

Interventional radiology: a modular approach.

The authors offer a modular approach to the development of new procedures in the field of pediatric interventional radiology as a conceptual model and a springboard for further discussion.

Utility of corticosteroid injection for temporomandibular arthritis in children with juvenile idiopathic arthritis.

Objective: To assess the effects of computed tomography (CT)-guided injection of corticosteroid into the temporomandibular joint (TMJ) in children with juvenile idiopathic arthritis (JIA) and clinical and magnetic resonance imaging (MRI) evidence of TMJ inflammation.

Methods: Twenty-three children ages 4-16 years with JIA and MRI evidence of TMJ inflammation received CT-guided TMJ injections of corticosteroid (triamcinolone acetonide [n = 16] or triamcinolone hexacetonide [n = 7]). Jaw pain or dysfunction and maximal incisal opening (MIO) distance were assessed before and after injection.

Inadvertent extra-epidural catheter placement in an infant.

We report the inadvertent passage of an epidural catheter threaded from the caudal space out of a lumbar intervertebral foramen and into the lower thoracic paravertebral space in an infant. We identified the errant catheter by radiography and removed it without sequelae.

Transgluteal approach for draining pelvic fluid collections in pediatric patients.

Purpose: To evaluate a transgluteal approach for draining pelvic fluid collections in pediatric patients.

Materials And Methods: Institutional review board approval and informed consent for interventional procedures were obtained. From September 1995 to December 2002, 140 abdominopelvic fluid collections were drained at two institutions.

Pseudoaneurysm in children: diagnosis and interventional management.

Pseudoaneurysms (PAn) are uncommon in adults and even less common in children. They are most often encountered after iatrogenic arterial injury. Presentation may be substantially delayed after the iatrogenic event, and diagnosis can be difficult, especially when the PAn occurs in an unexpected location.

Oral and intravenous caffeine for treatment of children with post-sedation paradoxical hyperactivity.

Background: Paradoxical hyperactivity (PH) is a known complication of sedation in children, especially with barbiturates such as pentobarbital. The accompanying inconsolable irritability and agitation, similar to behaviors reported in children with attention deficit hyperactivity disorder (ADHD), is uncomfortable for the child and anxiety-provoking for parents and health-care workers. Our objective was to describe our experience with oral (PO) and intravenous (IV) caffeine as a treatment for sedation-induced PH.

Closed reduction with CT-guided screw fixation for unstable sacroiliac joint fracture-dislocation.

Background: Unstable posterior pelvic ring fractures and dislocations are uncommon but potentially life-threatening injuries in children. Early definitive management reduces risk of immediate complications as well as chronic pain and gait dysfunction. Conventional operative therapy carries substantial risk of extensive blood loss and iatrogenic neurological and vascular injury.

CT-guided percutaneous lung biopsy in children.

Purpose: To describe techniques and evaluate outcomes of computed tomography (CT)-guided percutaneous lung biopsy in children.

Materials And Methods: Between April 1992 and June 2003, 64 patients (32 male, 32 female) with a mean age of 10.8 years (0.

A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy.

Background/purpose: Congenital hyperinsulinism (HI) causes severe hypoglycemia in neonates and infants. Recessive mutations of the beta-cell K(ATP) channel genes cause diffuse HI, whereas loss of heterozygosity together with inheritance of a paternal mutation cause focal adenomatous HI. Although these 2 forms of HI are clinically identical, focal HI can be cured surgically.

Transmural migration of gastrostomy tube retention discs.

Background: Accidental dislodgment is one of the most frequent causes of avoidable cost and consternation related to gastrostomy tubes. The Sacks-Vine gastrostomy tube, inserted in an antegrade fashion by a percutaneous technique, is extremely stable due to the construction of its disc retention device. However, transmural migration of the retention disc is a known severe delayed complication associated with this tube.

Imaging and intervention in the gastrointestinal tract in children.

Vascular and interventional techniques have become an integral component of modern pediatric healthcare. Minimally invasive procedures of the gastrointestinal tract now comprise a large part of any active pediatric interventional practice. Magnetic resonance cholangiopancreatography offers a reliable, non-invasive means to evaluate patients with possible pancreatic or biliary pathology.