Mark J. Hogan

Quality Improvement Guidelines for Diagnostic Arteriography

PREAMBLE The membership of the Society of Interventional Radiology (SIR) Standards of Practice Committee represents experts in a broad spectrum of interventional procedures from the private and academic sectors of medicine. Generally, Standards of Practice Committee member dedicate the vast majority of their professional time to performing interventional procedures; as such, they represent a valid, broad expert constituency of the subject matter under consideration for standards production.

A Phase 1/2a Follistatin Gene Therapy Trial for Becker Muscular Dystrophy

Becker muscular dystrophy (BMD) is a variant of dystrophin deficiency resulting from DMD gene mutations. Phenotype is variable with loss of ambulation in late teenage or late mid-life years. There is currently no treatment for this condition. In this BMD proof-of-principle clinical trial, a potent myostatin antagonist, follistatin (FS), was used to inhibit the myostatin pathway. Extensive preclinical studies, using adeno-associated virus (AAV) to deliver follistatin, demonstrated an increase in strength. For this trial, we used the alternatively spliced FS344 to avoid potential binding to off target sites. AAV1.CMV.FS344 was delivered to six BMD patients by direct bilateral intramuscular quadriceps injections. Cohort 1 included three subjects receiving 3 × 10(11) vg/kg/leg. The distance walked on the 6MWT was the primary outcome measure. Patients 01 and 02 improved 58 meters (m) and 125 m, respectively. Patient 03 showed no change. In Cohort 2, Patients 05 and 06 received 6 × 10(11) vg/kg/leg with improved 6MWT by 108 m and 29 m, whereas, Patient 04 showed no improvement. No adverse effects were encountered. Histological changes corroborated benefit showing reduced endomysial fibrosis, reduced central nucleation, more normal fiber size distribution with muscle hypertrophy, especially at high dose. The results are encouraging for treatment of dystrophin-deficient muscle diseases.

Percutaneous treatment of lymphatic malformations.

OBJECTIVE: The aim of this study is to investigate a new treatment regimen for macrocystic and microcystic lymphatic malformations (LM) of the head and neck. STUDY DESIGN: The study represents a retrospective review of outcomes from new percutaneous treatments for lymphatic malformations. SUBJECTS AND METHODS: Thirty-one patients (age range, 2 days to 51 years of age) underwent percutaneous treatment for LM of the head and neck from 2001 to 2007. The LM involved the orbit, ear, parotid gland, face, and neck. Twenty-seven patients underwent primary treatment of LM; 4 were treated for recurrence after operative resection. Macrocysts ≥1 cm) were treated with dual-drug chemoablation (sequential intracystic sodium tetradecyl sulfate and ethanol); doxycycline was used for microcysts. Macrocysts and microcysts were treated after complete cyst aspiration with sonographic guidance. Fifty-four macrocysts and 125 microcysts were treated. The goal of treatment was complete cyst ablation documented by sonography or MR imaging. RESULTS: Mean number of treatments was 1.7 per patient; mean number of treatments for macrocysts was 1.1; mean treatments for microcysts was 1.7. Ablation efficacy was 179 of 179 (100%) cysts. Effective cyst ablation achieved effective clinical control with resolution of the external mass appearance. Treatments included massive head and neck mixed LM and cysts surrounding the facial nerve and brachial plexus. Infection occurred in 2 (6%) of 31 patients. No patient experienced postprocedural pain, skin necrosis, neuropathy, skin retraction, or myoglobinuria. CONCLUSION: Percutaneous sclerotherapy provides effective treatment for macrocystic and microcystic LM as primary treatment or for recurrence after surgical resection.

Diagnostic and interventional ultrasonography in neonatal and infant lumbar puncture.

Background. Lumbar puncture (LP) may be unsuccessful clinically, prompting image-guided LP by radiologists. Objective. To investigate the utility of ultrasound (US) in diagnosing the cause of failed LP and in guiding LP. Materials and methods. Neonates and infants referred for image-guided LP underwent spine US of the thecal sac. When indicated, image-guided LP was performed. Results. Forty-seven evaluations and interventions were performed in 32 patients. All patients were initially evaluated after failed blind LP attempts. Twenty-three of the initial US studies showed intrathecal and/or epidural echogenic hematoma, which obliterated the CSF space; 5 showed minimal fluid, and 4 had normal examinations. LP was deferred or cancelled in 14 cases based upon initial US findings. Image-guided LP was performed 32 times in 19 patients. US guidance was used in 26, fluoroscopy in 3, and fluoroscopy with US assistance in 3. Using US, LP was performed in 9 patients with no visible CSF: 2 samples were sufficient for culture only. Six patients had minimal CSF US: 4 provided usable CSF samples. Clear CSF space was seen in 11: all had successful LP. Conclusions. US can disclose the cause of failed LP, can help determine whether or not to intervene further, and can provide guidance for LP.

Pediatric thoracic outlet syndrome: a disorder with serious vascular complications

BACKGROUND Thoracic outlet syndrome (TOS), caused by compression of the neurovascular structures between the clavicle and scalene muscles, typically presents with neurologic symptoms in adults. We reviewed our experience with 25 adolescents and propose a diagnostic/treatment algorithm for pediatric TOS. METHODS From 1993 to 2005, 25 patients were treated with TOS. A retrospective chart review was performed with institutional review board approval. Demographics, clinical presentation, diagnostic studies, and treatment were evaluated. RESULTS Seven male (28%) and 18 female (72%) patients presented between the ages of 12 to 18 years. Thirteen (52%) had vascular TOS (11 venous, 2 arterial), 11 (44%) had neurologic TOS, and 1 had both. Vascular TOS included subclavian vein thrombosis (7), venous impingement (4), and arterial impingement (2). Three patients had hypercoagulable disorders, and 6 had effort thrombosis. Venography was diagnostic in 10 cases. Neurogenic TOS was diagnosed by clinical symptoms. Five patients with subclavian vein thrombosis underwent thrombolysis, with 3 maintaining long-term patency. Of 25 patients, 24 underwent transaxillary first rib resection. CONCLUSION Vascular complications are more common in adolescents with TOS than in adults. A diagnostic/treatment algorithm includes urgent venography and thrombolysis for venous TOS and a workup for hypercoagulability. Neurogenic TOS is diagnosed clinically, whereas other studies are rarely beneficial.

Cecostomy in children with defecation disorders

Administration of antegrade enemas through a cecostomy is a therapeutic option for children with severe defecation disorders. The purpose of this study is to report our 4-year experience with the cecostomy procedure in 31 children with functional constipation (n = 9), Hirschsprungs disease (n = 2), imperforate anus (n = 5), spinal abnormalities (n = 8), and imperforate anus in combination with tethered spinal cord (n = 7). Data regarding complications, antegrade enemas used, symptoms, and quality of life were retrospectively obtained. Placement of cecostomy tubes was successful in 30 of 31 patients. Soiling episodes decreased significantly in children with functional constipation (P = 0.01), imperforate anus (P < 0.01), and spinal abnormalities (P = 0.04). Quality of life improved in patients with functional constipation and imperforate anus. No difference in complications was found between percutaneous and surgical placement. Use of antegrade enemas via cecostomy improved symptoms and quality of life in children with a variety of defecation disorders.

Ten-Year Experience Using Antegrade Enemas in Children

OBJECTIVE To describe a single-center, 10-year experience with the use of antegrade enemas. STUDY DESIGN Retrospective analysis of 99 patients treated with antegrade enemas at Nationwide Childrens Hospital. RESULTS Study subjects (median age 8 years) were followed for a mean time of 46 months (range 2-125 months) after cecostomy placement. Seventy-one patients had the cecostomy placed percutaneously and 28 by surgery. Thirty-five patients had functional constipation and 64 patients an organic disease (spinal abnormalities, cerebral palsy, imperforate anus, Hirschsprungs disease). While using antegrade enemas, 71% became symptom-free, in 20 subjects symptoms improved, in 2 subjects symptoms did not change, and in 7 subjects symptoms worsened. Poor outcome was associated with surgical placement of the cecostomy (P < .001), younger age (P = .02), shorter duration of symptoms (P = .01), history of Hirschsprungs disease (P = .05), cerebral palsy (P = .03), previous abdominal surgery (P = .001), and abnormal colonic manometry (P = .004). In 88%, successful irrigation solution included use of a stimulant laxative, and subjects who used a stimulant did significantly better (P < .001) than subjects who started without a stimulant. In 13 patients, the cecostomy was removed 49.7 months after placement without recurrence of symptoms. Major complications occurred in 12 patients and minor complications in 47. CONCLUSIONS Antegrade enemas represent a successful and relatively safe therapeutic option in children with severe defecatory disorders. Prognostic factors are identified.

Joint Quality Improvement Guidelines for Pediatric Arterial Access and Arteriography: From the Societies of Interventional Radiology and Pediatric Radiology

Manraj K. S. Heran & Francis Marshalleck & Michael Temple & Clement J. Grassi & Bairbre Connolly & Richard B. Towbin & Kevin M. Baskin & Josee Dubois & Mark J. Hogan & Sanjoy Kundu & Donald L. Miller & Derek J. Roebuck & Steven C. Rose & David Sacks & Manrita Sidhu & Michael J. Wallace & Darryl A. Zuckerman & John F. Cardella & Society of Interventional Radiology Standards of Practice Committee and Society of Pediatric Radiology Interventional Radiology Committee

Interventional radiology treatment of empyema and lung abscesses

Pneumonias in children can be complicated by pleural effusions, empyema and abscesses. The incidence of these complications is increasing, correlated to an increased virulence of the pneumococcal bacterium. These complications may prolong morbidity and lead to decreased pulmonary function. Traditionally, patients were treated medically with antibiotics, and refractory complications were treated surgically with large bore chest tube placement and thoracotomy. Improvements included instilling fibrinolytics into the chest tubes and video-assisted thoracoscopic surgery, which expedited recovery and improved outcomes. Image guided techniques from interventional radiology have been developed as an alternative to treat these patients with minimal invasiveness. These therapies have achieved high success and low complication rates, and are the preferred first-line procedures when available.

Neonatal total parenteral nutrition ascites from liver erosion by umbilical vein catheters

Background. A preterm infant was found to have total parenteral nutrition (TPN) ascites after infusion through a low umbilical vein catheter (UVC). Objective. To evaluate the clinical and imaging findings of neonates with TPN ascites after infusion through UVCs. Materials and methods. Eight patients with TPN ascites were identified over three years. Charts were abstracted for clinical data. Plain-film, ultrasound (US), and contrast studies through the UVCs were examined to determine UVC placement, presence of liver injury, and confirmation of intraperitoneal extravasation from the UVC. Results. All eight patients with TPN ascites presented with hypotension and abdominal distension. All had UVCs overlying the liver on plain film. Catheters were in place a mean of 8.9 days prior to TPN extravasation. US in four patients showed hepatic parenchymal damage around the UVC tip. Contrast studies in six patients showed intraperitoneal spill. Conclusion. While low UVC placement may sometimes be clinically unavoidable, TPN administered through abnormally positioned UVCs is not without risk.