Although rare, CLOVES syndrome, a <i>PIK3CA</i>-related congenital overgrowth disorder, presents radiologists with opportunity for vital diagnostic and treatment planning. CLOVES syndrome is characterized by Congenital Lipomatous Overgrowth of the trunk, Vascular malformations, Epidermal naevi, and Skeletal and Spinal anomalies. Multimodal imaging findings can help distinguish CLOVES from other overgrowth syndromes such as Proteus syndrome and Klippel-Trenauny syndrome. We present multimodal imaging of four patients with CLOVES syndrome which demonstrate characteristic findings: - Thoracic lipomatous hyperplasia, a key sign of CLOVES syndrome in which predominantly thoracic lipomatous masses grow in the subcutaneous tissues and invade the pleura, mediastinum, and upper abdomen, often with superficial vascular malformations - Renal anomalies, including agenesis, hypoplasia, hydronephrosis, and cysts. - Vascular malformations, including venous, venolymphatic, and lymphatic malformations - Spinal cord defects, including spina bifida, medullary arteriovenous shunts, and congestive myopathy of the paravertebral venous plexus Management of CLOVES syndrome focuses on debulking of lipomatous masses, treatment of clinically significant vascular malformations, and medical management of renal and neurologic sequelae. To that end, the role of the radiologist is accurate diagnosis of the syndromic pattern, isolation of the extent of lipomatous masses for pre-operative planning, and identification of renal and spinal cord anomalies. The purpose of the poster is to: 1. Briefly review the types of congenital overgrowth disorders as demonstrated in various imaging modalities. 2. Focus on characteristic imaging findings of CLOVES syndrome. 3. Review the benefits and disadvantages of various imaging modalities. 4. Identify the most relevant radiologic findings for surgical, interventional and medical management. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Kinariwala Dhara, Daugherty Reza

Keywords: Congenital, Thoracic, Vascular Malformations

Comprehensive radiologic evaluation and accurate diagnosis of vascular malformations is vital to guide optimal treatment. Technical approaches and sclerotic agents used by interventional radiology vary by the type of malformation, anatomic location, and architecture of the malformation. Proper pre-procedural characterization of hard-to-treat lesions impacts procedural success and long-term outcomes. Most vascular malformations located superficially on the extremities and trunk, and can be evaluated with ultrasound and color Doppler. However, evaluation can be complicated by unusual locations and difficulty in characterization, requiring MRI and/or MRA. We present interesting pediatric vascular malformations challenging for both imaging diagnosis and treatment. The objectives of this poster include: 1. To review the classification and nomenclature of vascular malformations. 2. To present multimodal imaging findings of vascular malformations. 3. To discuss diagnostic imaging approaches of vascular malformations.modalities used. 4. To describe the treatment approaches for these malformations and outcomes including technical considerations and follow up. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Kinariwala Dhara, Daugherty Reza, Park Auh Whan

Keywords: Vascular Malformations

Pediatric esophageal varices almost uniformly occur as a consequence of portal hypertension in children with chronic liver disease or extrahepatic portal vein obstruction. Varices in these cases typically evolve in the distal esophagus at the intersection of the left gastric and esophageal veins. We report a rare case of “downhill” varices that developed in the proximal esophagus due to extensive chronic thrombosis of the SVC and brachiocephalic veins in a child with intestinal failure requiring lifelong central venous access for parenteral nutrition. We report a case of downhill varices associated with thrombosis of the SVC and brachiocephalic veins, secondary to a central line. A 14-year-old boy with an ACTG2 (actin gene) mutation-related history of parenteral nutrition dependent chronic intestinal pseudo-obstruction and central venous catheter-associated thrombi on prophylactic anticoagulation presented with new onset dysphagia. On endoscopy, Grade II esophageal varices without stigmata of recent bleeding were unexpectedly identified in the upper and middle esophagus. Imaging was then performed to assess for advanced liver disease or abnormal portal flow. CT of the abdomen and pelvis showed no splenomegaly, ascites, or radiographic signs of cirrhosis. There was, however, partial visualization of dilated para-esophageal vessels coursing from the chest. Subsequent chest CT demonstrated chronic occlusion of the right internal jugular and brachiocephalic veins, along with severe narrowing of the SVC. There were extensive venous collateral pathways through the azygos and para-esophageal venous system and engorged submucosal veins were present in the esophagus. Echocardiography confirmed a stable chronic thrombus in the SVC. Given the high risk of intervention, the decision was made to forgo immediate SVC balloon dilation and stenting and to continue active surveillance. This case highlights the important role of imaging in differentiating “downhill” esophageal varices from their “uphill” counterpart. This distinction is critical, as each entity necessitates a different treatment approach. Definitive management of downhill varices is aimed at alleviating SVC obstruction but carries significant risk and was deferred in this case. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Bharde Pravinkumar, Kinariwala Dhara, Venkatakrishna Shyam Sunder Billapura, Bale Christina, Acord Michael

Keywords: Venous Recanalization, PICC, Thrombosis