CLOVES Syndrome Diagnosis and Treatment in an Adult Patient.

CLOVES syndrome is a rare, nonheritable sporadic overgrowth disorder. In the world 130-200 cases have been reported. This is the first case of CLOVES described in Portugal, which had been not been diagnosed for the last 36 years. With this paper, the authors look to highlight the clinical features of this syndrome so that it does not go unrecognized in daily practice. The authors also underline the efficacy and safety of sirolimus, and that this treatment should not be denied, even in adult patients.

Lower Extremity Fibro-Adipose Vascular Anomaly-Case Report.

Fibro-adipose vascular anomaly (FAVA) is a painful complex vascular malformation, characterized by muscle fibrofatty infiltration, usually in lower limb, associated with contracture of the ipsilateral extremity. This article describes the first case of FAVA reported in Portugal successfully treated with surgery. A 9-year-old female was admitted complaining of a painful mass in the right leg. The MRI scan showed the presence of a 5 × 4 × 4 cm mass in the right leg consistent with FAVA located in the peroneus longus muscle. The patient underwent resection of the involved muscle. She had 6 months of follow-up without any relevant clinical event. The authors also highlight the difference between FAVA and venous malformation. Early surgery is a treatment option that can prevent long-term consequences, as contracture and movement limitation.

Neointimal Hyperplasia.

Neointimal hyperplasia is a physiologic healing response to injury to the blood vessel wall, involving all the three arterial layers and it occurs in the presence of internal (endovascular) or external (surgical) injury. It is a highly complex process involving several tissues (perivascular, vessel wall, and blood) and numerous cell lineages with multiple molecular signaling networks. So, there is a number of possible targets for inhibition of this process. There are known risk factors for Intimal Hyperplasia, such as diabetes, female gender, presence of systemic inflammation, type of arteries treated, types of surgical and endovascular materials, presence of turbulent flow and genetic status. The present paper discusses the pathophysiology of neointimal hyperplasia and the strategies to prevention and treatment of it.

Occlusion of inferior vena cava: a singular presentation of abdominal aortic aneurysm.

Even though the majority of abdominal aortic aneurysm s(AAAs) are asymptomatic, they can occasionally manifest as a result of adjacent structures involvement. Although the most frequent venous complication of AAA is rupture into the inferior vena cava (IVC), venous compression can infrequently occur. The authors report a particularly rare case of compression and thrombosis of the IVC by AAA. Patient was treated by preoperative placement of an IVC filter to impede pulmonary embolism and subsequently by open surgical repair. Besides discussing the circumstances associated with IVC compression by AAA, the authors also discuss the treatment strategy adopted and the possible alternatives.