Case 343: Kaposiform Lymphangiomatosis.

Area of Science:

• Internal Medicine
• Pediatrics
• Radiology

Background:

• A 17-year-old male presented with a 2-year history of left-sided breast enlargement (gynecomastia).
• The patient also reported multiple long bone fractures from minor trauma over 8 years and intermittent hemoptysis.

Purpose of the Study:

• To investigate the underlying cause of gynecomastia, recurrent fractures, and thrombocytopenia in a young male patient.
• To characterize the imaging findings associated with the patient's complex clinical presentation.

Main Methods:

• Clinical history and physical examination.
• Laboratory investigations including platelet count and hormonal profile.
• Diagnostic thoracentesis for hemorrhagic fluid.
• Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI).
• Echocardiography to assess cardiac structures.

Main Results:

• Laboratory results revealed thrombocytopenia (platelet count 100,000/µL) with a normal hormonal profile.
• Thoracentesis yielded minimal hemorrhagic fluid.
• Imaging studies demonstrated diffuse pericardial thickening.

Conclusions:

• The combination of gynecomastia, fractures, thrombocytopenia, and pericardial thickening suggests a rare systemic disorder requiring further investigation.
• Multimodality imaging played a crucial role in evaluating the extent of the disease.