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.

Penetrating Trauma To The Axillary Artery.

Axillary artery injuries due to penetrating trauma are relatively uncommon. Management of these injuries is challenging due to the complex local anatomy, rigid chest walls, and associated injuries. Open exposure with direct open vascular repair has been the mainstay of operative management. We report a clinical case of a 51-year-old man victim of penetrating trauma to the axillary artery caused by a chain-saw and repaired by open surgery with a great saphenous vein interposition graft.

Endovascular Treatment Of A Symptomatic Thoracic Aorta Thrombi.

Aortic mural thrombus is a rare condition with 0.45% incidence in the general population, being the thoracic aorta the most affected portion. In the absence of an atherosclerotic wall lesion, other specific conditions should be studied and excluded. The authors describe two clinical cases of a 64 years old male and a 48 years old female that despite a non- -atherosclerotic diseased aorta, had a thoracic mural thrombus which presented clinically with mesenteric and lower limb microembolization, respectively. Once presented with peripheral embolization, the aim should be to exclude the embolic source and prevent end organ malfunction. TEVAR has been developed as a therapeutic solution to exclude the embolic source, with a high rate of technical success and few comorbidities associated. Long term anti-coagulation is debatable but may prevent further embolization events.

Hemodialysis Acess - A Creative Attitude is Necessary.

Creating and maintaining a functional vascular access (VA) is a critical factor in the survival of a dialysis patient. It implies a creative attitude either to maintain its functionality or to build a new one wherever possible, being it autologous or synthetic. We describe the VA history of a 59 years-old male patient, with extreme obesity, which started in 2012 with failed attempts of VA construction in both forearms until a functional brachiocephalic arteriovenous fistula (AVF) in the right upper limb was achieved. However, it required ligation due to severe venous hypertension secondary to central venous disease related to previous CVC use. As he had no good superficial conduit in the left arm we decided to harvest the arterialized right cephalic vein and implant it in the left arm, creating an autologous arteriovenous shunt between the brachial artery and axillary vein (AV). Despite initial patency, it failed irreversibly approximately one year after creation. As no more superficial veins were available in the upper limbs, a prosthetic access was the next step. We decided for a hybrid graft (HG) between the left brachial artery and the AV because of the patient's biotype and scarred axilla that impeded a safe re-intervention on the AV. This graft was used between 2015 and 2017 with multiple interventions to maintain patency. In 2017 a significant diffuse prosthesis deterioration and reduced AVF flow were noticed with no possible segmental reconstruction. We were then forced to proceed with subtotal graft substitution preserving the outflow stented segment of the HG, using an early cannulation graft to prevent CVC use. After this successful reconstruction, the patient started hemodialysis on the following day with no intercurrences registered.