Ovarian Torsion in an Adolescent with Beckwith-Wiedemann Syndrome and Unilateral Tubo-ovarian Hyperplasia.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is the most common pediatric overgrowth syndrome. BWS has a broad phenotypic presentation along with an increased propensity to develop various embryonal tumors. There are very few reported cases of gonadal hyperplasia in BWS patients in the existing literature. CASE: We describe a 13-year-old girl with BWS who presented with an episode of abdominal pain and was found to have torsion and necrosis of a markedly hyperplastic right ovary and fallopian tube. We present a brief literature review on ovarian hyperplasia in BWS patients for which we used an online search of the databases PubMed, Embase, Ovid Medline, and Cochrane. RESULTS AND CONCLUSION: Through an extensive literature search, we only found 3 previous reports of ovarian hyperplasia in BWS patients, all in postmortem specimens. Our case highlights a potentially important aspect of visceral organ hyperplasia in patients with BWS that could remain indolent until adolescence and might present as an abrupt-onset abdominopelvic catastrophe.

Imaging and clinical findings in segmental arterial mediolysis (SAM).

Segmental arterial mediolysis (SAM) is an uncommon, non-atherosclerotic, non-inflammatory arteriopathy that tends to affect the medium-sized splanchnic branches of the aorta along with renal, carotid, cerebral, and coronary arteries. The clinical presentation ranges from asymptomatic to severe, life-threatening intra-abdominal hemorrhage and shock. SAM overlaps clinically and radiologically with other inflammatory vasculitides. This article describes the pathologic-radiologic correlation, imaging findings, and the management of the disease. Radiologists should be familiar with this disease entity as imaging plays a crucial role in the diagnosis.

Beta Blockade as Treatment for Intracranial Infantile Hemangioma: Case Report and Literature Review.

BACKGROUND: Intracranial infantile hemangiomas are extremely rare, with only 36 patients reported in literature. Treatment for intracranial infantile hemangiomas has been mostly limited to surgery, steroids, and interferon therapy. Propranolol, which is often used to treat cutaneous infantile hemangiomas, is not currently standard treatment for intracranial infantile hemangiomas. PATIENT DESCRIPTION: We present a one-month old boy with an intracranial infantile hemangioma treated with propranolol. RESULTS: This boy was being treated with oral propranolol for a supraclavicular infantile hemangioma. Subsequent brain magnetic resonance imaging (MRI) scan showed evidence of an associated intracranial infantile hemangioma in the right cerebellopontine angle. Repeat brain MRI scan after two months of propranolol treatment demonstrated a significant reduction in the size of the intracranial infantile hemangioma. CONCLUSIONS: This is the first report of successful therapy of an intracranial infantile hemangioma with propranolol.

Radiologic case study: diabetic myonecrosis of the posterior compartments of both thighs.

Enhance your diagnostic skills with this "test yourself" monthly column, which features a radiograph and challenges you to make a diagnosis.