Brian J. Dillon

Intranodal Lymphangiography: Feasibility and Preliminary Experience in Children

PURPOSE To review an initial experience studying the lymphatic system using direct injection of ethiodized oil contrast medium into lymph nodes (ie, intranodal lymphangiography) in children with chylous disorders. MATERIALS AND METHODS Children with chylous disorders who underwent intranodal lymphangiography were included in this retrospective study. Under general anesthesia, ultrasonography was used to guide the placement of a small-bore (22-25-gauge) needle into an inguinal lymph node. Ethiodized oil contrast medium was very slowly injected into the node. Opacification of the lymphatic system was documented with fluoroscopic and digital substraction imaging and videofluoroscopic clips. RESULTS Five children (age range, 6 wk to 17 y) with chylous vaginorrhea (n = 1), postoperative chylothorax (n = 2), or spontaneous chylothorax (n = 2) underwent intranodal lymphangiography. The amount of ethiodized oil injected was 0.5-4.5 mL. Intranodal lymphangiography was successfully completed in four patients. One procedure was terminated because of patient motion and extravasation of contrast medium. Lymphangiographic findings included a spectrum of lymphatic channel disorders including incompetence, obstruction, collateralization, chylous reflux, and chylous leak. There were no complications. CONCLUSIONS The simplified technique of injecting contrast medium into a lymph node to opacify the lymphatic system in children can be an alternative to the more elaborate conventional lymphangiography.

Fibro-adipose vascular anomaly: clinical-radiologic-pathologic features of a newly delineated disorder of the extremity.

Background: The diagnosis and management of vascular anomalies of the extremities can be challenging as these disorders are uncommon and may clinically overlap. The aim of this paper is to describe the clinical, radiologic, and histopathologic features of fibro-adipose vascular anomaly (FAVA), a previously unrecognized disorder of the limb. Methods: The clinical, imaging, operative, and histopathologic data from patients with a unique intramuscular lesion of the extremities comprising dense fibrofatty tissue and slow-flow vascular malformations were retrospectively reviewed. Results: Sixteen patients diagnosed with FAVA of the extremity (3 male and 13 female individuals) met the clinical, radiologic, and histopathologic inclusion criteria. The age at presentation ranged from the time of birth to 28 years. The locations of the lesions were: calf (n=10), forearm/wrist (n=3), and thigh (n=3). Fourteen patients presented with severe pain. Seven of the patients with calf lesions had limited ankle dorsiflexion. On imaging, the complex intramuscular lesions replaced muscle fibers with fibrofatty overgrowth and phlebectasia (dilation of the veins). The extrafascial component comprised fatty overgrowth, phlebectasia, and an occasional lymphatic malformation. The histopathologic features comprised dense fibrous tissue, fat, and lymphoplasmacytic aggregates within atrophied skeletal muscle. Adipose tissue also infiltrated skeletal muscle at the periphery of the lesion. There were large, irregular, and sometimes excessively muscularized venous channels and smaller, clustered channels. Other findings include organizing thrombi, a lymphatic component, and dense fibrous tissue–encircled nerves. Conclusions: The constellation of clinical, radiologic, and histopathologic features constitutes a distinct entity comprising fibrofatty infiltration of muscle, unusual phlebectasia with pain, and contracture of the affected extremity. The clinical and radiologic findings permit the diagnosis of FAVA with major therapeutic implications. Level of Evidence: Level III.

Sclerotherapy of Abdominal Lymphatic Malformations with Doxycycline

PURPOSE To assess the safety and efficacy of percutaneous image-guided sclerotherapy with doxycycline as primary treatment of intraabdominal lymphatic malformations (LMs). MATERIALS AND METHODS Retrospective review was performed of all cases of abdominal, mesenteric, or retroperitoneal LMs referred to a single center that were subsequently treated with image-guided percutaneous sclerotherapy. RESULTS Ten patients were included, of whom six were male. The mean age was 13 years (range, 2-28 y). Preprocedural cross-sectional imaging demonstrated a macrocystic malformation in nine patients and a mixed macrocystic/microcystic malformation in one. The malformation was accessed under sonographic guidance, followed by injection of opacified sclerosant agent under fluoroscopic guidance. A drainage catheter was placed in eight cases, in which sclerotherapy was repeated through the catheter for another 1 day (n = 2) or 2 days (n = 6). Doxycycline was reconstituted at 10 mg/mL, with a mean per-session dose of 608 mg (range, 80-1,000 mg) and a mean total dose of 1,230 mg (range, 80-3,000 mg). Peritoneal spill was identified in one case, but the patient remained asymptomatic. No other complications were encountered. Follow-up imaging was available in eight patients: complete resolution was seen in seven, with partial resolution in one. There was no recurrence of clinical symptoms in the follow-up period. CONCLUSIONS Initial results indicate that percutaneous image-guided sclerotherapy of macrocystic intraabdominal LMs with doxycycline is a safe and effective procedure.

Sonography-guided percutaneous liver biopsies in children.

OBJECTIVE The purpose of this study was to evaluate the safety and efficacy of sonography-guided percutaneous core needle liver biopsy in infants and children. MATERIALS AND METHODS We conducted a retrospective analysis of all patients who underwent sonography-guided percutaneous core needle liver biopsies over a 7.5-year period by pediatric interventionalists at a single tertiary center. RESULTS A total of 597 procedures were performed in 470 patients (270 male and 200 female), with a mean age of 10.5 years (age range, 1 month-21 years). The main indications for biopsies were abnormal liver enzymes (n=129, 21.6%), grading and staging of chronic hepatitis B or C (n=105, 17.6%), evaluation of transplanted liver (n=111, 18.6%), iron overload (n=73, 12.2%), miscellaneous other diffuse parenchymal abnormalities (n=124, 20.7%), and focal hepatic lesions (n=55, 9.2%). The procedures were performed either under sedation (n=311, 52.1%) or general anesthesia (n=286, 47.9%). Diagnostic yield was obtained in 596 biopsies (99.8%) from an average of 2.4 cores in patients with diffuse disease (n=541, 90.6%) and 6.5 cores in patients with focal disease (n=55, 9.2%). Ten patients (1.7%) experienced a major complication, including pneumothorax (n=1, 0.2%), abdominal wall pseudoaneurysm (n=1, 0.2%), and symptomatic bleeding (n=8, 1.3%). Five of these children required transfusion, two were only admitted for observation, and one required surgical evacuation. There were no procedure-related deaths. Minor complications (n=49, 8.2%) included a symptomatic subcapsular hematoma (n=35) and stable small hemoperitoneum (n=9). CONCLUSION Sonography-guided percutaneous core liver biopsy is a safe and effective procedure in children that has a high diagnostic yield and very low complication rate.

Delayed Formation and Rupture of a Pseudoaneurysm After Transjugular Liver Biopsy in a Pediatric Bone Marrow Transplant Patient: Imaging and Endovascular Treatment

Dear Editor: A 7-year-old girl with relapsed pre–B cell acute lymphoblastic leukemia was referred for liver biopsy for abnormal liver function (high alanine aminotransferase, aspartate aminotransferase, and bilirubin values) 2 months after a second bone marrow transplantation (BMT). In the weeks preceding the liver biopsy, she had developed feeding intolerance, vomiting, diarrhea, and pneumatosis intestinalis. Endoscopic biopsies confirmed graft versus host disease (GVHD) in the stomach, duodenum, colon, and rectum. The differential diagnosis for the liver dysfunction included GVHD and total parenteral nutrition cholestasis. On the evening before she underwent liver biopsy, her laboratory values were as follows: hemoglobin 12.3 g/dL, hematocrit 35.7%, white blood cell count 7,810/lL, platelets 41,000/lL, prothrombin time 11.9 s, international normalized ratio of prothrombin time 1.13, partial thromboplastin time 27.6 s, and fibrinogen 296 mg/dL. Given her moderate thrombocytopenia, she received platelet transfusions, and the decision was made to perform the biopsy via a transjugular approach. On the morning of the procedure, her platelet count was 101,000/lL. The right internal jugular vein was accessed by ultrasound guidance and micropuncture technique, and an 8.5French vascular sheath was placed. Through this sheath, the 7-French LABS 200 sheath (Cook Bloomington, IN) was introduced into the right hepatic vein, and five passes were made with the 19-gauge cutting cannula under fluoroscopic and sonographic guidance. Multiple passes were necessary because several of the cores were fragmented. Postbiopsy venography and ultrasound were normal (Fig. 1). An ultrasound performed for abdominal pain 5 h after the biopsy demonstrated trace perihepatic fluid and unchanged free fluid in the pelvis. The hematocrit level remained stable. On postoperative day 5, a computed tomographic (CT) scan of the abdomen and pelvis with contrast performed for chest pain demonstrated a normal liver with free fluid in the pelvis (Fig. 2A). On postoperative day 8, an abdominal ultrasound performed for right upper quadrant pain showed a small amount of free fluid in the pelvis. On postoperative day 13, her hematocrit dropped 8 points in a 24-h period to 19.6%, and she developed abdominal distention. Urgent enhanced CT and ultrasound scans demonstrated a large liver hematoma around an actively filling pseudoaneurysm. Increased echogenic fluid in the pelvis was also noted, indicating rupture of the pseudoaneurysm into the peritoneal cavity (Fig. 2B, C). The patient was taken emergently to the angiography suite. Under general anesthesia, selective angiography of the hepatic artery with a 0.038-inch 4-Fr vertebral catheter demonstrated rapid filling of a large pseudoaneurysm fed by the right hepatic artery branch to segment VI (Fig. 3A, B). Access to the feeding vessel was obtained with a coaxial Prowler 14 microcatheter (Cordis Miami, FL). The tip of the microcatheter was gently advanced into the ‘‘neck’’ of the pseudoaneurysm, and embolization was carried out with 67% n-butyl cyanoacrylate glue opacified with Ethiodol. Care was taken to embolize the feeding vessel proximal and distal to the neck as well as the neck B. J. Dillon A. I. Alomari (&) Department of Radiology, Division of Vascular and Interventional Radiology, The Children’s Hospital Boston, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA e-mail: ahmad.alomari@childrens.harvard.edu

Angiographic demonstration of an aberrant abdominal umbilical artery in a patient with penoscrotal transposition and caudal regression

Penoscrotal transposition (PST) is a rare congenital anomaly characterized by caudal location of the penis with respect to the scrotum. PST may be seen in isolation or associated with caudal regression syndrome. We present a case of an infant born with penoscrotal transposition, bladder agenesis, a solitary dysplastic kidney, and caudal regression. The patient developed anuria and was referred for angiography for preoperative planning for renal transplantation and genital reconstruction. Angiography demonstrated an aberrant abdominal umbilical artery, an anomaly classically associated with sirenomelia but also has been described in caudal regression.

A periureteric venous ring diagnosed by MRI: an unusual cause of flank pain.

We describe a pediatric patient with upper tract collecting system dilatation caused by a periureteric venous ring. The diagnosis was made after MR urography. This rare inferior vena caval anomaly has not been documented in the pediatric literature, nor have the MRI appearances been described. MRI is ideally suited for the assessment of suspected retrocaval ureters in order to identify this variant so that an appropriate surgical approach can be planned.

Paget-Schroetter Syndrome in 21 Children: Outcomes after Multidisciplinary Care

OBJECTIVE To review the presentation, management, and outcomes of Paget-Schroetter syndrome (PSS) in children and propose a multidisciplinary treatment algorithm involving pediatric and vascular surgery, interventional radiology, and hematology. STUDY DESIGN Patients with PSS presenting between 2003 and 2013 were reviewed. Demographics, symptoms, therapies, and functional outcomes were noted. Data from early patients informed the development of a multidisciplinary treatment algorithm applied to later patients. RESULTS Of 21 patients, mean ± SD age was 16 ± 1.6 years and 11 (52%) were male. Of patients with complete presentation data, common symptoms were edema (84%), discoloration (58%), and pain (58%). Thrombophilia workup revealed one heterozygote for factor V Leiden, 2 patients with factor VIII elevation and 1 patient with mildly low antithrombin. The most recent 8 patients were treated according to an algorithm developed by a multidisciplinary working group through experience with the first 13 cases. All patients underwent a venogram, endovascular intervention (including 15 receiving catheter-directed thrombolysis), and operative ipsilateral thoracic outlet decompression (first rib resection, anterior scalenectomy, and venolysis). Postoperative complications included hemothorax (2), pneumothorax (1), and recurrent thrombosis (2). Follow up duration was 12 ± 9.5 months. Symptoms recurred transiently in 1 patient. CONCLUSION Pediatric patients with PSS can be treated successfully using a multidisciplinary treatment algorithm including anticoagulation, catheter-directed thrombolysis, and operative decompression of the thoracic outlet. Early outcomes are promising.

Acute Limb Ischemia in an 8-Year-Old Patient: A Case Report

We report the case of an 8-year-old patient with a history of nephrotic syndrome, who presented to the emergency department with right foot pain. The patients mother described intermittent pain that woke her son from sleep and was accompanied by the foot turning purple and becoming cold to touch. Physical examination revealed capillary refill of over 10 seconds in the right and less than 2 seconds in the left foot. Ankle-brachial indices (ABIs) were 0.0 on the right and 0.96 on the left. The patient was admitted and started on therapeutic intravenous heparin. After consultation with his parents, right lower extremity angiography and thrombolysis was performed over 2 days. He subsequently underwent fasciotomy and amputation of the tip of all 5 toes. Eighteen months later, there is no leg length discrepancy, he is walking with foot inserts and has normal ABIs bilaterally.