Final Pr. ID: Poster #: EDU-096
Lymphatic malformations (LMs) are low-flow vascular malformations which are composed of dilated lymphatic channels, forming septated cyst-like structures (2). LMs are the second most common type of vascular malformation, second to venous malformations (1). The most common locations are in the neck, followed by the axillary region. On MRI, LMs are multiloculated, T2 hyperintense lesions, which may have fluid-fluid levels, and are without flow voids (1, 2). They can involve multiple tissue planes and do not regard anatomical and fascial boundaries (2). Cystic lymphatic malformations are further divided into microcystic, macrocystic, or mixed, based on the size of their cystic components. Macrocystic LMs are composed of larger cysts, while microcystic LMs are composed of smaller cysts and may appear solid on imaging.
We present ten cases of microcystic, macrocystic, and mixed lymphatic malformations in a variety of anatomical locations. In addition to demonstrating the imaging findings, we present a review of the literature in regards to each anatomical region. Lymphatic malformations in this presentation include right orbit (n=1), mediastinum (n=2), pulmonary bronchovascular bundles/pleural space (n=1), retroperitoneum (n=1), mesentery (n=2), perirectal and scrotal (n=1), lower extremity (n=2). 6 of these children also had additional sites of T2 hyperintense disease involving the bones or spleen, suggestive of additional lymphangiomas.
This presentation summarizes ten cases of lymphatic malformations in a variety of less common anatomical locations and a review of the pertinent literature.
1. Flors L, Leiva-Salinas C, Maged IM et al. (2011) MR Imaging of Soft-Tissue Vascular Malformations: Diagnosis, Classification, and Therapy Follow-up. Radiographics 31:1321-1340.
2. White CL, Olivieri B, Restrepo R et al. (2016) Low-flow vascular malformation pitfalls: from clinical examination to practical imaging evaluation- part 1, lymphatic malformation mimickers. AJR 206: 940 – 951. Read More
Keywords: Lymphatic malformation, Vascular Malformation
Final Pr. ID: Poster #: EDU-051
Generalized Lymphatic Anomaly (GLA) is a rare multisystem congenital disorder originating from the abnormal development of the lymphatic system which occur under the spectrum of Complex Lymphatic Anomaly (CLA). In addition to GLA, other CLAs include Kaposiform Lymphatic Anomaly (KLA) and Gorham-Stout Disease (GSD). Lymphatic malformations (LM) associated with GLA are usually apparent at birth or by two years of age. GLA can affect almost any organ of the body but is most commonly associated with lymphatic abnormalities in the skin, abdominal/thoracic viscera and bone.
Multisite soft tissue LM can occur in all CLAs, with macrocystic lymphatic malformations being most common in GLA. These lesions can be found in the mediastinum, retroperitoneum, and subcutaneous tissue. Abdominal viscera involved include the spleen and liver. The frequency of focal splenic lesions is higher with GLA and KLA in comparison to GSD. On MRI, the lesions exhibit marked T2 hyperintensity with no discernable enhancement. In patients with larger splenic lesions, areas of T1 hyperintensity have been documented. Liver lesions in GLA have a similar appearance to the previously described splenic lesions. Nakamura et al. found that more than 30 focal splenic lesions and/or focal splenic lesions with maximum diameters greater than >10 mm were observed only in patients with GLA. On contrast enhanced CT, the lesions are generally well-circumscribed and hypodense. Within the thorax, mediastinal LMs can be seen in GLA but are more common in KLA and GSD. Chylous effusions can occur in all of the CLAs, although it has been reported that effusions in GLA were more likely to be associated with mediastinal involvement. Osseus involvement is common in patients with GLA. GLA has a predilection for the appendicular skeleton, in which the ribs are most affected although cranial, vertebral and lower extremity lesions have been reported. The lesions are usually non-contiguous with medullary destruction and sparing of the cortex. This is in contrast to GSD where cortical destruction, progressive osteolysis, contiguous lesions, and soft tissue infiltration are more common.
1) Illustrate the most common imaging characteristics by each organ system affected by GLA and how to differentiate GLA from other CLAs.
2) Raise awareness of the optimal imaging evaluation in patients with GLA.
3) Outline an approach to multidisciplinary management of patients with GLA through a vascular anomalies center. Read More
Final Pr. ID: Poster #: CR-017
Cystic teratoma and lymphatic malformation (LM) are two generally benign differential considerations for congenital masses which demonstrate overlapping clinical and imaging findings. It is important to distinguish between these etiologies as cystic teratomas may require surgical excision whereas lymphatic malformations may be treated with minimally invasive sclerotherapy. We present two contrasting cases: a cervical neck teratoma mimicking a LM and an abdominal LM resembling a cystic teratoma.
Case 1: A 9-month-old female presented with a right craniofacial neck mass discovered on prenatal screening. Postnatal ultrasound demonstrated a complex multiloculated cystic lesion with peripheral vascularity. MRI confirmed a predominantly cystic, mixed soft tissue and cystic right anterolateral neck mass, most consistent with mixed type lymphatic malformation. Despite two rounds of percutaneous sclerotherapy with doxycycline, the lesion continued to enlarge on repeat MRI, crossing the midline and causing mass effect on the trachea with associated airway compromise. Respiratory distress was exacerbated by COVID-19 infection requiring intubation and eventual tracheostomy. She was started on sirolimus and underwent a third sclerotherapy treatment with doxycycline and STS without improvement. Surgical debulking was performed with pathology returning as mature multicystic teratoma composed of endodermal, neuroglial and mesodermal elements with minimal fat.
Case 2: A 13-year-old male with no significant past medical history presented with three weeks of right-sided abdominal pain and constipation. CT showed a right abdominal multiseptated cystic lesion. MRI confirmed a 27 cm multicystic abdominal mass with small soft tissue component and calcification with mass effect on the small bowel and right ureter causing partial small bowel obstruction and hydronephrosis, most consistent with a large cystic teratoma. However, following surgical excision pathology demonstrated a multicystic mass containing vascular structures with lymphocytic aggregates and nodules consistent with LM.
Expanding on these cases, this exhibit will also review the clinical presentations and management of cystic teratoma and LM with an emphasis on imaging workup and radiology-pathology correlation. It is important for radiologists and their interdisciplinary colleagues to be cognizant of the overlapping features of these conditions to avoid errors in diagnosis and management as their treatments differ. Read More
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
Final Pr. ID: Poster #: EDU-095
Soft tissue vascular malformations encompass a wide variety of lesions throughout the body. Appropriate treatment for these lesions is dependant on accurate classification and diagnosis. Unfortunately, inappropriate nomenclature and description by the radiologist can lead to confusion and possibly mismanagement. Read More
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:
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.
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
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.
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
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.
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