Stecher Priscilla,  Ordonez Alvaro,  Noor Abass

Final Pr. ID: Poster #: CR-023

Klippel-Trenaunay Syndrome (KTS) is a rare cutaneous vascular malformation syndrome involving capillary, venous, and lymphatic malformations often involving a unilateral lower limb. Patients with KTS can also have disease involvement of the gastrointestinal (GI) tract, predisoposing patients to GI bleeding. Here, we present a case of a 15-year-old male with known congenital Klippel-Trenaunay Syndrome involving the left lower extremity presenting with acutely worsening rectal bleeding. The patient presented with two weeks of rectal bleeding, fatigue, and weakness. On initial evaluation, the patient was found to have acute drop in hemoglobin concerning for active GI bleeding. Initial imaging included a tagged red blood cell scan, which demonstrated diffuse increased radiotracer activity along the left hemiabdomen, consistent with active bleed. However, the etiology/source of bleeding at this time could not be determined due to the extensive abdominal involvement. Further evaluation included CT angiography of the abdomen and pelvis, which revealed a large, extensive venous malformation extending throughout the wall of the descending and rectosigmoid colon, with venous malformation extending throughout the left lateral pelvic musculature. Arterial phase imaging demonstrated abnormal enhancement of the mucosa of the descending and rectosigmoid colon, and venous phase imaging demonstrated contrast blushing throughout the descending colon concerning for active bleeding, without clear identifiable source. Due to decline in clinical status and refractory anemia requiring massive transfusion, the patient was evaluated surgically and subsequently underwent emergent colectomy and left lower quadrant ostomy placement. Intraoperative findings demonstrated a massively dilated sigmoid colon with complex tangle of cavernous venous malformations involving approximately two feet of bowel. In setting of a technically challenging operation, the patient’s post-operative course has been complicated by rectal stump dehiscence, multiple pelvic abscesses, and guarded prognosis. In summary, KTS is a rare syndrome involving extensive capillary, lymphatic, and venous malformations, with gastrointestinal involvement predisposing patients to potential life-threating GI bleeding. Here we review a case of a 15-year-old male with KTS presenting with refractory GI bleeding in an extensive colonic vascular malformation necessitating emergent colectomy. Read More

Authors:  Stecher Priscilla , Ordonez Alvaro , Noor Abass

Keywords:  Klippel-Trenaunay Syndrome, Venous Malformation, Rectal Bleeding

Patel Nimai,  Swana Hubert,  Johnson Craig

Final Pr. ID: Poster #: SCI-023

To assess the incidence, scope, clinical findings and imaging characteristics of GU pathology due to underlying KTS in pediatric patients with the goal of improved diagnosis and outcomes for children with this potentially fatal disorder. Read More

Authors:  Patel Nimai , Swana Hubert , Johnson Craig

Keywords:  Klippel-Trenaunay syndrome, lymphatic malformation, Venous malformation

Guillen Gutierrez Cinthia,  Rodriguez Garza Claudia,  De Luna Vega Raul,  Hernández Grimaldo Edgar,  Elizondo Riojas Guillermo

Final Pr. ID: Poster #: EDU-010 (S)

Vascular abnormalities are commonly observed in pediatric patients, with an estimated prevalence of at least 4.5%.
The current classification scheme for vascular anomalies was developed in 2014 by ISSVA (International Society for the Study of Vascular Abnormalities) and is based on the work of Mulliken and Glowacki of 1982.
This classification emphasizes the fundamental difference between vascular tumors and vascular malformations.
The diagnosis of vascular abnormalities is based on clinical history and physical examination.
US: Screening, proper characterization
TC: It allows assessing the extent of the lesion and the relationship with adjacent structures
MRI: It's the ideal imaging study and reflects its histological composition.

The most commons anomalies are:
Hemangiomas:
The most frequent vascular tumors are lobed and highly vascular lesions.
They have significant enhancement to the administration of contrast medium, but only moderate T2 hyperintensity that reflects their highly cellular nature.

Venous Malformation:
Malformed venous channels with slow blood flow with thrombosis and flebolite formation
Important, often heterogeneous and peripheral enhancement with central progression.
Hyperintense T2 signal reflects low cellularity

Lymphatic malformations:
Dilated lymphatic channels and cysts. They can be macro or microcystic
Macrocystic variants appear as cysts with a very bright T2 signal and minimal enhancement (if any), bleeding is a common complication with liquid-liquid level formation.

Arteriovenous Malformations:
The main finding is empty flow due to high flow, which infiltrate the tissues without evidence of a free mass.
There may be a mild enhancement and T2 hyperintensity that reflects tissue edema

Treatment:
In most cases, conservative treatment is recommended, but when a patient suffers from clinical complications sclerotherapy of the nidus becomes mandatory.
A multidisciplinary approach is needed.

Sclerosing agents
Alcohol 98%: Strong endothelial damage, high response rate, less expensive, easy to obtain. Painful during the procedure, high complication rate, penetrative effect on the deep vascular layer
Ethanolamine oleate: Excellent thrombogenic effect, chemical damage to the vascular wall, less toxic effect than absolute ethanol. It can induce acute renal failure due to hemolytic effect, less endothelial damage than absolute ethanol.
Polidocanol: Overhydration of endothelial cells, almost painless procedure. May induce reversible cardiac arrest Read More

Authors:  Guillen Gutierrez Cinthia , Rodriguez Garza Claudia , De Luna Vega Raul , Hernández Grimaldo Edgar , Elizondo Riojas Guillermo

Keywords:  Hemangioma, Lymphatic Malformation, Venous Malformation

Hughes Nicole,  Phelps Andrew,  Meyer Anna,  Courtier Jesse,  Mackenzie John,  Zapala Matthew

Final Pr. ID: Poster #: EDU-042

Accurate diagnosis of venous malformations can be challenging, and confidently diagnosing them is important for accurate treatment. A thorough understanding of the etiology, imaging appearance, and important mimickers of venous malformations is critical for the radiologist to provide an accurate assessment for the clinician. Read More

Authors:  Hughes Nicole , Phelps Andrew , Meyer Anna , Courtier Jesse , Mackenzie John , Zapala Matthew

Keywords:  MRI, venous malformation, Whole body MRI, Blood pool MRI contrast agent