Identification and successful treatment of congenital microfistulas with the aid of directional Doppler

Hitherto unidentifiable and therefore untreatable congenital microfistulas were detected with the aid of continuous-wave directional Doppler and a fine-beam pencil probe. The microfistulas formed part of the Klippel-Trenaunay (K-T) syndrome. The management of one case is reported in detail and in three others salient features are touched upon. Careful clinical and radiological examination failed to demonstrate any arteriovenous microfistulas. When Doppler ultrasound scanning was carried out, two discrete fistulas were discovered. Their extent and direction were mapped out accurately. Incisions were made directly over the markings displaying a pulsating capillary tuft of vessels. Further dissection exposed a feeding arteriole which was less than 1 mm. in diameter. Excision of the vascular malformations resulted in the cure of the patient. In another patient with the K-T syndrome in whom a cutaneous hemangioma involved the whole lower limb, in spite of a thorough and systematic search with a Doppler, no microfistulas could be demonstrated. It is suggested that all patients suffering from the K-T syndrome should be examined by Doppler ultrasound in the hope that microfistulas which elude radiodiagnostic techniques might be detected and treated surgically.