Vascular Anomalies

[size=6]A 6-month-old baby is being treated with sirolimus for a lymphatic malformation of the head and neck. Sirolimus levels are being maintained between 4-8 ng/mL. The child requires placement of a gastrostomy tube that is planned for the following week. In preparation for surgery, sirolimus should be a stopped today and resumed 14 days after surgery b stopped today and resumed 5 days after surgery c administered without interruption of dosing d stopped today and resumed 6 months after surgery[/size]

A newborn infant presents with this right knee lesion at birth. What is the best next step in his management? [size=20px]a Observation b Propranolol c Sirolimus d Radiation therapy e Wide excision[/size]

[size=20px]A 14-month-old girl has a 7 cm left axillary, macrocystic lymphatic malformation. The most appropriate initial management is: ​ A Observation B Percutaneous sclerotherapy C Excision D Intravenous Sirolimus​[/size]

A 2-week old asymptomatic baby girl presents with the posterior left neck mass shown and associated ultrasound. There is no anterior extension. The mass was present at birth and has not changed. A fetal US @ 20 weeks gestation was normal. How will you proceed?

An otherwise healthy 12 year old boy presents to your clinic for evaluation of a “bump” on his left anterior shoulder/axilla. His parents report that he was born with a small bruise that they assumed was secondary to birth trauma. The bruise never went away and the area has grown with him over time. On exam, the area is soft. If you hold pressure on the area if becomes smaller. A photo of the area is shown here: [size=20px]What is the most likely diagnosis? A Lymphatic malformation B Venous malformation C Congential hemangioma D Arteriovenous malformation​[/size]

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[size=20px]An asymptomatic 2 month old boy presents with a moderate sized left cervical mass. There was poor prenatal care and no prenatal diagnosis. MRI of the neck shows a mixed-cystic lymphatic malformation which was judged not amenable to sclerotherapy. Recent studies have shown benefit with adjuvant administration of which of the following? A Prednisone B Rapamycin C Tacrolimus D Methotrexate E Cyclophosphamide​[/size]

[size=20px]A ten week old former term infant presents with increasing abdominal girth and increased work of breathing. She has multiple cutaneous hemangiomas on her scalp and trunk. An echocardiogram is normal. On abdominal ultrasound she is found to have numerous lesions in the liver consistent with diffuse hemangioma. What is the best next course of action for this baby? A Biopsy hepatic lesions B Start propranolol C Start interferon D Observation E Biopsy cutaneous lesions[/size]

[size=20px]A 3 month old infant with a growing ulcerated scalp infantile hemangioma (IH) is started on propranolol therapy at a dose of 1 mg/kg/d. After one week the dose is increased to 2 mg/kg/d and within 24h the infant experiences an episode of severe wheezing, without associated infectious symptoms, that requires pediatric ICU admission and nebulized bronchodilator therapy. The next appropriate step in the care of this child is: A Bronchoscopy with bronchoalveolar lavage B Discontinuation of propranolol C Allergy testing D Oral amoxicillin for 1 week E Budesonide (Pulmicort) inhaler therapy[/size]

[size=20px]A 1-month old baby is being seen in the clinic for follow up of a liver mass. The mass was seen on ultrasound prenatally. Postnatally, the baby remained hemodynamically normal. Alpha fetoprotein was normal for age and is appropriately decreasing with time. Ultrasound at birth showed a 4cm hepatic hemangioma. The ultrasound today was interpreted as unchanged in size. This baby would be at risk for A Heart failure B Thrombocytopenia C Hypothyroidism D Development of metachronous lesions E Involution of the lesion[/size]

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