Case report: Restrictive cardiomyopathy presenting with complete thromboembolism occlusion of the terminal part of the abdominal aorta in a preadolescent Saudi girl.

Restrictive cardiomyopathy (RCM) is a rare disease in children, accounting for <5% of all pediatric cardiomyopathies. It may be idiopathic or may be a secondary to a systemic disease. The disease is characterized by normal systolic function with impaired ventricular filling caused by stiff ventricular walls. Children with RCM often present with symptoms of exercise intolerance, shortness of breath, weakness, and chest discomfort. Thromboembolism events are an unusual presentation of RCM. We are reporting a preadolescent female from the eastern province of Saudi Arabia who presented with sudden right lower limb swelling, paresthesia, and pain caused by a complete occlusion of the terminal part of the abdominal aorta and both common iliac arteries. Echocardiography revealed dilated atria, normal ventricle dimensions and two floating thrombi in the left atrium. The patient was successfully managed with an anticoagulant, surgical thrombectomy and cardiac transplantation.

The ugly face of deep vein thrombosis: Phlegmasia Cerulea Dolens-Case report.

INTRODUCTION: Phlegmasia Cerulea Dolens (PCD), a rare & life-threatening condition caused by a massive deep venous thrombosis that is associated with arterial occlusion caused by the subsequent compartment syndrome. CASE PRESENTATION: A 56-year-old male was diagnosed as a case of extensive left femoral DVT & pulmonary embolism. Two days after being managed by systemic thrombolytics & heparin, his condition worsened as he developed cyanosis of the affected limb, compartment syndrome & foot drop so he was referred to our facility for further management. CT venogram showed a thrombosis of the left popliteal vein extending into the left common iliac vein confirming the diagnosis of PCD & May-Turner syndrome. We adopted a limb preserving approach using a pharmacomechanical catheter directed thrombolysis (PCDT). The patient recovered fully with a complete resolution of his foot drop. DISCUSSION: Several treatment options have been suggested to improve the outcomes of PCD, but due to the rarity of this condition a gold standard treatment is still controversial. But regardless of the chosen approach, there is an urgent need to decrease the thrombus burden to prevent further adverse sequelae like amputation or even death which can be achieved by using PCDT as it was demonstrated in our case. CONCLUSION: Our case shows that a rare entity of DVT as PCD could be a result of improper management of acute proximal DVT in the background of anatomical variabilities & that despite the late presentation of such a rare condition there still a role for a limb preserving approach with endovascular techniques.