Kaposiform lymphangiomatosis has overlapping imaging features with central conducting lymphatic anomaly and generalized lymphatic anomaly. Presence of mediastinal or retroperitoneal enhancing and infiltrative soft-tissue disease along the lymphatic distribution, hemorrhagic effusions and moderate thrombocytopenia (50-100,000/μl) should favor diagnosis of kaposiform lymphangiomatosis.

Image-guided percutaneous cryoablation is a safe and effective option for treatment of symptomatic FAVA lesions.

No abstract

No abstract

To evaluate the utility of inversion scout (TI-scout) obtained during cardiac magnetic resonance imaging (CMR) in diagnosing myocardial amyloid infiltration. A retrospective analysis of CMR exams in 39 patients (24 males, age range 29–77 years) was performed. Imaging was performed on a 1.5T system, and included steady state cine, post contrast TI-scout and delayed enhancement images. Evaluations included studies in 13 patients with myocardial amyloidosis and 26 patients without myocardial amyloidosis. To characterize abnormal nulling, the time to myocardial nulling on the TI scout was compared to the null times of the blood pool and spleen for each scan. The sensitivity and specificity of different tissue nulling abnormalities for myocardial amyloidosis were computed. The null times of tissues in 18/26 (69 %) patients in the non-amyloid group followed a consistent order with the blood pool null time preceding the myocardial nulling which was equal to that of splenic nulling (Type 1 pattern). This order differed in all 13 patients with myocardial amyloidosis described as three non-mutually exclusive nulling categories: 10 patients had myocardial null time preceding or coincident with blood pool (Type 2 pattern); in 11 patients myocardial null time was non-coincident with splenic nulling (Type 3 pattern); and in 8 patients myocardial null time was non-coincident with both blood pool AND splenic nulling (Type 4 pattern). While no patient exhibited Type 4 nulling pattern in the non-amyloid group, 1/26 patient had a Type 2 and 7/26 patients had a Type 3 nulling pattern. A sensitivity of 100 % was obtained when either Type 2 OR Type 3 nulling was present while a specificity of 100 % was obtained when both Type 2 AND Type 3 nulling were present together (Type 4 pattern). Our study demonstrates that the pattern of nulling on the TI scout sequence CMR has potential diagnostic utility for the presence of myocardial amyloidosis. The temporal pattern of myocardial, blood pool and splenic nulling needs to be carefully evaluated on the TI scout sequence and could prove useful in other infiltrative cardiomyopathies.

No abstract

No abstract

Background: Fibroadipose vascular anomaly (FAVA) is a recently-defined vascular malformation often involving the extremities and presenting in childhood. Patients may present to orthopaedic surgeons with pain, swelling, joint contractures, and leg length discrepancy. There is no established therapy or treatment paradigm. We report on outcomes following surgical excision for patients with this condition. Methods: Between 2007 and 2016, all 35 patients that underwent excision of lower-extremity FAVA were retrospectively reviewed using a combination of medical records, radiologic findings, and telemedicine reviews. Results: Mean age at initial presentation was 12.3±6.8 years. Mean follow-up from time of definitive diagnosis at our institution was 66 months (range: 12 to 161 mo). Mean follow-up after surgery was 35 months (range: 6 to 138 mo). Females were affected more than males (71% vs. 29%). The most common location of FAVA was in the calf (49%), followed by the thigh (40%). The most commonly involved muscle was gastrocnemius (29%), followed by the quadriceps (26%). At latest follow-up after surgery, there was an improvement in the proportion of patients with pain at rest (63% vs. 29%), pain with activity (100% vs. 60%), as well as analgesia use (94% vs. 37%). Fourteen patients (40%) had symptomatic residual disease or recurrence of FAVA requiring further treatment. Six patients (17%) required further surgery and 6 (17%) required further interventional radiologic procedures. Three patients (9%) required eventual amputation for intractable pain and loss of function. Lesions with direct nerve involvement were associated with persistent neuropathic symptoms at latest follow-up (P=0.002) as well as symptomatic residual disease and/or recurrence requiring further treatment (P=0.01). Seventeen patients (49%) had 19 preoperative joint contractures. Eighteen of the 19 contractures (95%) had sustained improvement at latest follow-up. Conclusions: In carefully selected patients, surgical excision of FAVA results in improvement of symptoms. However, symptomatic residual disease and/or recurrence are not uncommon. Direct nerve involvement is associated with a worse outcome. Level of Evidence: Level IV—case series.