Raja Shaikh

Callosal holes: an unusual imaging appearance in systemic lupus erythematosus. A case report.

Systemic lupus erythematosus (SLE) has diverse imaging features. However, focal lesions in the corpus callosum are extremely rare in SLE with only few cases mentioned in the literature, with no mention of callosal holes in SLE. Callosal holes have been described as a characteristic finding in Susac syndrome and have been mentioned in Nipah virus encephalitis, Marchiafava Bignami disease and periventricular leukomalacia. We describe a case of SLE with callosal holes. The demonstration of callosal holes in this case brings the imaging appearance of SLE a step closer to that of Susac syndrome which is considered a clinically and radiologically close condition. It also adds to the list of imaging appearances of central nervous system SLE.

Catheter-Directed Thrombolysis in a Child with Bilateral Renal Artery Graft Thrombosis

A 5-year-old boy with midaortic syndrome who had undergone aortic bypass and bilateral renal artery grafts presented to the emergency department 1 year after surgery with symptoms of nausea, vomiting, and abdominal pain. Because of delay in diagnosis of bilateral renal artery thrombosis, his condition progressed to anuric renal failure. He underwent catheter-directed thrombolysis 7 days after presentation with administration of tissue plasminogen activator and heparin infusion over a 24-hour period. There was successful resolution of thrombus and complete recovery of renal function to baseline. The patient had normal renal function at 6-month follow-up.

Transjugular Retrograde Cannulation of the Portal Vein via Patent Ductus Venosus: Alternative Access for Endovascular Hepatic Interventions

The use of the patent ductus venosus via a transjugular approach to access the portal system for endovascular treatment of hepatic vascular anomalies in three infants is reported. Two patients had an arterioportal fistula, and one had a rapidly involuting congenital hemangioma. All patients underwent arteriography followed by embolization of the vascular anomalies without complications. This alternative route is technically simpler and likely safer than transarterial and transhepatic approaches.

Characterization of long-term outcomes for pediatric patients with epithelioid hemangioma

Epithelioid hemangioma (EH) is a rare benign vascular tumor that occurs in soft tissues and bone and presents between the third and sixth decades of life. Little is known about the clinical course and outcomes of pediatric EH. We report 11 patients diagnosed with EH at a median age of 14.4 years. One patient treated with interferon and one with sirolimus exhibited partial response for >2 years. Although a benign neoplasm, EH is difficult to manage without standard protocols and portends considerable morbidity. Our findings suggest medical management, particularly sirolimus, may benefit these patients; however, long‐term follow‐up is needed.

Pulmonary thromboembolic events in patients with congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities and Klippel-Trénaunay syndrome

OBJECTIVE Patients with Klippel-Trénaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities (CLOVES) syndrome have central phlebectasia and enlarged persistent embryonic veins that are often incompetent and prone to thromboembolism. The purpose of the study was to determine the presence of phlebectasia and the incidence of symptomatic pulmonary embolism (PE). METHODS A retrospective review was conducted of patients referred to the Vascular Anomalies Center at our institution during a 21-year period who were diagnosed with KTS and CLOVES syndrome. Of these, the patients who had PE were screened for thromboembolic risk factors in addition to phlebectasia and the presence of persistent embryonic veins. Treatment outcomes following subsequent endovascular and medical therapies were reported. RESULTS A total of 12 KTS patients of 96 (12.5%) and 10 CLOVES syndrome patients of 110 (9%) suffered PE. Fourteen patients (64%) developed PE after surgery or sclerotherapy. All of the patients had abnormally dilated central or persistent embryonic veins; 20 patients were treated with anticoagulation (1 died at the time of presentation, and no information was available for 1) after PE, and 14 (66%) patients underwent subsequent endovascular treatment. Five patients developed recurrent PE despite anticoagulation. Two of the patients died of PE. No patients treated with endovascular closure of dilated veins had subsequent evidence of PE. CONCLUSIONS Patients with KTS and CLOVES syndrome are at high risk for PE, particularly in the postoperative period.

Lymphaticovenous bypass of the thoracic duct for the treatment of chylous leak in central conducting lymphatic anomalies

BACKGROUND Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement. METHODS We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography. RESULTS Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well. CONCLUSIONS Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options. LEVEL OF EVIDENCE IV.

Percutaneous Image-Guided Cryoablation in Vascular Anomalies

Understanding and management of vascular anomalies has always been intriguing. These disorders exhibit an expected pattern of clinical presentation and progression, and characteristic imaging findings. Significant progress in understanding and treating patients with vascular anomalies has been made in the past quarter century. Newer multidisciplinary domains for treating these disorders with medical drugs and less invasive image-guided or surgical procedures are constantly evolving. Vascular anomalies can exhibit aggressive tumor-like behavior resulting in recurrence or persistent symptoms after treatment. Thermal ablation has been widely used in tumor treatment. This has generated interest on using thermal ablation for treating vascular anomalies. Percutaneous image-guided cryoablation is increasingly used for this purpose as compared with other ablation technologies. Availability of small caliber cryoprobes and the ability to monitor the freeze zone in real time have made this an attractive option to interventional radiologists. These experiences are relatively new and limited. It is helpful to understand the emerging role of this technology in the treatment of vascular anomalies.