Purpose: Six cases of adventitial cystic disease were studied, and the
existing theories of the aetiology of adventitial cystic disease were
reviewed to present evidence in support of a variation of the develop
mental hypothesis that might explain the sites of occurrence of this r
are condition. Methods: Cases of adventitial cystic disease were colle
cted by interrogation of the records of a group of vascular surgeons i
n the Johannesburg area. After reviewing the relevant literature, the
sites of occurrence of 323 cases of adventitial cystic disease were do
cumented, and the theories of the formation of adventitial cystic dise
ase were reviewed. The embryological origin of those vessels in which
adventitial cystic disease occurs was investigated. Clinical cases wer
e collected in private practice vascular referral centers. The clinica
l features, treatment, and subsequent course of six cases of adventiti
al cystic disease (four related to the popliteal artery, one in the fe
moral artery, and one in the radial artery) are included. Results: All
cases of adventitial cystic disease reported have occurred in the non
axial arteries, which form at a later stage than the axial vessels dur
ing limb differentiation and development. It is therefore postulated t
hat during limb bud development cell rests derived from condensations
of mesenchymal tissue destined to form the knee, hip, wrist, or ankle
joints are incorporated into the nearby and adjacent nonaxial vessels
during development of these vessels in the 15-22-week stage. These new
ly forming nonaxial vessels develop from vascular plexuses during the
same stage of development, and in close proximity to the adjacent cond
ensing joint structures. It is further postulated that these cell rest
s are then responsible for the formation of adventitial cystic disease
later in Life, when the mucoid material secreted results in a mass le
sion within the arterial or venous wall. Conclusion: There is evidence
supporting the hypothesis that adventitial cystic disease is a develo
pmental condition occurring in the nonaxial blood vessels.