Klippel-Trenaunay syndrome is a rare disorder consisting of the triad of vascular and/or lymphatic malformations, capillary malformations, and soft tissue or bony hypertrophy. Symptom control is the mainstay of treatment for these patients, with many of the symptoms never fully being relieved. In this case report, we present the case of a 46-year-old man with chronic lower extremity ulcerations unresponsive to wound care therapy. Owing to the chronic nature of his wounds and associated pain, reconstruction of his iliac vein was performed using polytetrafluoroethylene graft and an arteriovenous fistula.
Alkaptonuria is a rare autosomal-recessive metabolic disorder of tyrosine degradation which results in elevated levels of circulating homogentisic acid. Ochronosis occurs when homogentisic acid polymerizes and deposits in connective tissue. Ochronotic lesions in the carotid arteries have not been described. In this report, we describe a 65-year-old man with alkaptonuria, with hypertension and hyperlipidemia, who underwent an uneventful carotid endarterectomy for an asymptomatic high-grade internal carotid artery stenosis. Histology revealed homogentisic acid deposits as black-brownish areas in the intima. He was noted to have an impressive heavily brown-black pigmented discoloration of the carotid plaque. Cardiovascular involvement is a rare consequence of alkaptonuria and is manifested by pigment deposition at the areas influenced by shear stress and turbulence.
6% vs 13.1%; P < .0001), chronic obstructive pulmonary disease (24.3% vs 17.5%; P ¼ .001), and wound infection (15% vs 23.5%; P < .0001). After propensity matching, the cohort comprised 452 patients in the NAA group and 904 patients in the GA group with no difference in baseline characteristics. NAA was associated with reduced rate of ventilator requirement for >48 hours (2.4% vs 0.2%; P ¼ .0014), bleeding requiring transfusion (17.5% vs 8%; P < .0001), and overall morbidity (29.3% vs 19%; P < .0001) as well as with shorter length of hospital stay (6.8 6 9.3 days vs 5.3 6 6.1 days; P ¼ .0026) and total operating time (237.8 6 109 minutes vs 202.4 6 113 minutes; P < .0001) compared with GA (Table). Conclusions: NAA is an infrequently used anesthesia technique in patients with significant comorbidities undergoing hybrid LER. NAA is associated with decreased perioperative morbidities and hospital length of stay compared with GA and should be preferentially used in this population of patients.
occlusion. Mean delay of surgery from last symptom was 3.7 6 5.4 days (median, 2 days; range 0-60 days) and urgent surgery (<48 hours) was performed in 65 cases (38.4%). Mean duration of surgery was 96 6 28 minutes; first and second clamping time were 3.7 6 1.1 minutes and 2.4 6 0.7 minutes, respectively. Standard CEA was performed in 107 patients (63.3%) while 62 (36.6%) received eversion CEA. There were no perioperative deaths; perioperative RNCR was 1.8% (major stroke: n ¼ 2, 1.1%; minor stroke: n ¼ 1, 0.6%). The presence of ischemic cerebral lesion at the preoperative computed tomography scan (OR, 2.55; P ¼ .45), contralateral carotid stenosis or occlusion (OR, 0.52; P ¼ .99), clamping time (OR, 0.27; P ¼ .10), urgent setting (OR, 0.17; P ¼ .08), as well as operator experience (OR, 1.2; P ¼ .66) and time period (OR, 0.89; P ¼ .72) were not significantly associated with an increased RNCR risk.Conclusions: Routine shunting with delayed insertion after plaque removal seems to be a safe and effective technique, that contributed to maintain a consistently low RNCR in neurologically symptomatic patients, independent from major clinical factors, operator, and time period. Standardization of the surgical technique may be mandatory to maintain results over time.
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