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Unraveling the risk factors, prognostic predictors, and evolving therapeutic approaches for phlegmasia cerulea dolens over 30 years

Ann Med. 2025 Dec;57(1):2577880. doi: 10.1080/07853890.2025.2577880. Epub 2025 Nov 2.

ABSTRACT

OBJECTIVE: Phlegmasia cerulea dolens (PCD) is the most severe form of deep vein thrombosis in the lower extremities. Clinical guidelines have not systematically outlined its risk factors, diagnostic methods, or treatment strategies. In this study, the literature published over the past 30 years is reviewed to summarize the clinical features of, diagnostic approaches to, prognostic factors of, and evolving management strategies for PCD.

METHODS: A retrospective analysis was conducted on 144 PCD patients from 1990 to 2024. Demographic data, clinical features, comorbidities, diagnostic methods, treatments, and prognostic outcomes were extracted. Descriptive statistical analysis was performed, and univariate and multivariate logistic regression were used to evaluate the impact of clinical factors on prognosis.

RESULTS: The majority of PCD cases involved the left lower limb, with 93.00% of patients having thrombosis extending into the iliocaval vein. Key risk factors included venous structural abnormalities, malignant tumors, and prior venous thromboembolism, with reproductive system cancers being the most common malignancy. Endovenous debulking techniques emerged as the preferred treatment method, reducing the risk of amputation and death by 70% compared with anticoagulation or thrombectomy. Patients with bilateral limb involvement, stage III PCD, or who underwent fasciotomy had significantly greater risks of amputation and death. The overall mortality rate was 18.75%, which was due primarily to metabolic acidosis and multiple organ failure.

CONCLUSION: Multimodal imaging should be recommended for the systematic evaluation of PCD to identify underlying malignancies and venous structural abnormalities. Endovenous debulking has demonstrated advantages in improving prognosis and should be recommended as a first-line treatment for PCD.

PMID:41176699 | DOI:10.1080/07853890.2025.2577880

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