Purpose: The overall incidence of congenital vascular malformations in the general population is 1.5%. Approximately two thirds of them are malformations of venous predominance. Abnormalities of the deep venous trunks have been observed in association with large superficial compensatory varices in these type of malformations. Knowledge of the integrity of the deep venous system is important in their management because excision of the enlarged superficial veins may be deleterious if there is aplasia or hypoplasia of the deep venous trunks. The objective was to investigate the prevalence and nature of deep venous anomalies that occur in patients with congenital vascular malformations of venous predominance both in our series and in the series from the medical literature.
Methods: From the last 35 years of medical literature, we reviewed seven series of congenital vascular malformations that provided pertinent information on the subject of our study. We also reviewed our own series of 392 patients with congenital vascular malformations studied at Children's Hospital of Mexico City (1963-1983; n = 223 children) and at Walter Reed Army and National Naval Medical Centers (1984-1998; n = 169 children). Of 392 patients, 257 (65.5%) had malformations of venous predominance; these were the subject of our analysis. Prevalence of the following deep venous anomalies was recorded: phlebectasia, aplasia or hypoplasia of venous trunks, aneurysms, and avalvulia. Diagnosis was made by one or more of the following methods: Doppler scanning, duplex scanning, plethysmography, computerized tomography, magnetic resonance imaging, and angiography.
Results: At least one anomaly of the deep venous system was present in 47% of the congenital vascular malformations of venous predominance reviewed. Phlebectasia was recorded in 36% of the cases, and aplasia or hypoplasia of deep venous trunks was observed in 8% of the cases. Venous aneurysms also were present in 8% of the cases; avalvulia was recorded in 7% of the cases.
Conclusion: Anomalies of the deep venous system occur in almost one half of congenital vascular malformations of venous predominance. The most common is the relatively innocuous phlebectasias that occur in over one third of cases. Aplasia/hypoplasia, venous aneurysms, and avalvulia were less frequent, each less than 10%; but failure to detect the latter three anomalies may lead to serious therapeutic errors.