Bilateral Femoral

Congenital Aplasia of the Deep Arteries

J. Schmidt, MD, B. Paetz, MD, J.R. Allenberg, MD, Heidelberg, West Germany

A patient with bilateral congenital aplasia of the deep femoral arteries is described. The condition was accompanied by aneurysms of the popliteal arteries. Besides the academic interest of such an anatomical rarity, the abnormality is of clinical importance insofar as acute ischemia of the extremity may occur in the presence of superficial femoral artery occlusion. (Ann Vasc Surg 1990;4:498-501).

KEY WORDS: Aplasia, deep femoral artery; congenital vascular disease; arteries, femoral.

Variations in the large arteries of the thigh are rare. We describe a patient with bilateral congenital aplasia of the deep femoral arteries and concomi- tant aneurysms of the popliteal arteries. Besides the academic interest in such an anatomical rarity, a vari- ation of this kind can change the operative strategy in the presence of an arterial occlusive disease.

CASEREPORT A 31-year-old man, 187 cm tall and weighing 83 kg, was

admitted complaining of the sudden onset of a sharp, burning pain in his right calf while carrying heavy stones two days prior to admission. On the day of admission the patient described continuous rest pain and numbness in the right leg.

On examination there were no peripheral pulses on the right leg distal to the common femoral artery, while normal pulses were noted on the contralateral side. Electrocardiogram showed normal sinus rhythm, and in addition his coagulation profile was completely normal.

Clinical diagnosis of an acute thrombotic occlusion of

From the Department of Vascular Surgery, University of Heidelberg, West Germany. Reprint requests: Jan Schmidt, MD, clo Andrew L . Warshaw, MD, Professor of Surgery, Harvard Medical School, Massachusetts General Hospital, Wang Ambu- latory Care Center, Suite 336, Boston, Massachusetts 02114.

the right superficial femoral artery was made and an arteriogram was performed (Fig. l ) , which showed: (1) Occlusion of the right superficial femoral and popliteal arteries; (2) an aneurysm of the left popliteal artery; (3) bilateral aplasia of the deep femoral arteries; and (4) the blood supply to the upper thigh feeding through a large branch of the internal iliac and superficial femoral artery.

Ultrasound examination of the right popliteal fossa revealed an occluded aneurysm; not even rudimentary parts of the deep femoral arteries could be demonstrated in the groin (Figs. 2a,b).

Subsequently, we performed a femoropopliteal vein bypass in the right leg. Pedal pulses were palpable post- operatively. Ten days later we reconstructed the opposite side to prevent thrombotic occlusion. The histology of the bypassed popliteal aneurysms showed focal, proliferating atherosclerosis without signs of inflammation.

Following the second procedure, the patient had an uneventful postoperative course.

DISCUSSION

Ruge [l] and Bluntschli [2] described anomalies of the arteries of the upper thigh. The deep femoral artery, which is characterized by the branching off of the perforator arteries [3], may vary significantly in its morphologic appearance. These authors each published a case of a duplicated deep femoral artery (4.5 and 9.7 cm versus 4.4 and 9.0 cm distal to the inguinal ligament). Bluntschli [2] postulated that

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Fig. 1. Arteriography of 31-year-old patient with bilat- eral, congenital aplasia of deep femoral arteries and concomitant aneurysms of popliteal arteries. No per- forator artery can be identified. On right side aneu- rysm underwent thrombotic occlusion. Upper thigh musculature is supplied by large branch of internal Iliac artery and 17 and 20 cm distal to inguinal liga- ment originating branch of superficial femoral artery, respectively.

this rare variation was caused by a separation of an originally single stem in early ontogenesis.

During ontogenesis (Week 5 through Week 8) a fifth lumbar artery, the so-called sciatic artery in the

Fig. 2. Inguinal region of same patient as in Fig.1 in (a) sonographic longitudinal and (b) cross section. One can recognize head of femur (FK) with more superficially-lying common femoral vein (V) and ar- tery (A). No rudimentary parts of deep femoral artery can be identified.

posterior upper thigh, forms the first primitive ves- sel supplying the lower extremity (Fig. 3a). The femoral artery, which develops later from the ex- ternal iliac artery, forms an arterial network (rete femorale) and, reaches the lower thigh as the saphe- nous artery. Between the femoral. and the sciatic artery several anastomoses are formed. One of these anastomoses continues to grow towards the distal part of the sciatic artery, which becomes the popliteal artery (Fig. 3b). In the last phase the sciatic artery undergoes regression and the inferior gluteal artery, along with the concomitant arterial branch of the sciatic nerve, 'remain as remnants [4]. The proximal part of the rete femorale differentiates into the deep femoral artery, which in this way

500 BILATERAL CONGENITAL APLASIA ANNALS OF VASCULAR SURGERY

Development of the lower limb arteries

5Jh week - 7.th week - 8.th week

L inguinal

Rete temorale

Ramus communlcans

u- Popiiteal artery

mus concomitans the sciatic nerve

Superficial femoral

ng. 3.1 ng. 3.2 no. 3.3

Fig. 3. Ontogenetic development of human upper thigh arteries modified after Greebe [5]. (a) lschiadic artery (axial artery) at dorsal aspect of upper thigh gains contact to rete femorale by ramus communicans superior distally and by (b) femoral artery branching off from external iliac artery proximally. (c) One anastomoses of this capillary network differentiates into superficial femoral artery, while proximal part of rete femorale forms deep femoral artery.

develops independently from the two other large upper thigh arteries (Fig. 3c) [ 5 ] .

Unilateral aplasia of the external iliac artery and the superficial femoral artery [6], hypoplasia [7,8] and bilateral absence of the pelvic arteries [9-111, hypoplasia of the femoral arteries [12], and persis- tence of the sciatic artery [ 13,141 have already been described. Direct continuation of the external iliac artery into the deep femoral artery in the absence of a superficial femoral artery [15] and duplication of the common femoral artery just above the femoral bifurcation [15,16] have also been described.

Apart from multiple variations of the small upper thigh arteries, such as the medial and lateral circum- flex femoral arteries, abnormalities of the deep femoral artery are relatively rare [1,2,13,17,18]. Usually the deep femoral artery leaves the common femoral artery about 30-40 mm distal to the inguinal ligament [19-211. In about 30 to 40% of people the deep femoral artery is a lateral branch of the com- mon femoral artery, while in 5% a medial origin is described [4]. The normally large caliber of this vessel can be explained by its development. In many higher vertebrates this vessel leaves the ex- ternal iliac artery to the posterior side of the upper thigh and supplies the strong hip extensor muscles

[22]. In humans these muscles have lost most of their original size and function due to the erect position. A large deep femoral artery is an impor- tant source of collaterals when the superficial fem- oral artery is occluded [23]. An overview of the most important abnormalities of the deep femoral artery is displayed in Table I.

Bilateral congenital aplasia of the deep femoral arteries has not been described before to our knowl- edge. However, in four patients aplasia with a

TABLE I.-Congenital anatomical varieties of the deep femoral artery

Anomalv Author Bilateral aplasia - Unilateral aplasia Young [3], Nicholson [14],

Bilateral doubled artery Ruge [l] Unilateral doubled artery Hepburn [17], Bluntschli [2] Bilateral anomaly of origin Ruge [l], Bluntschli [2],

Lang [4], Lippert [18], Lipshutz [25]

Unilateral anomaly of Ruge [l], Bluntschli [2], origin Lang [4], Lippert 1181,

Lioshutz 1251

Anger [24]

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normal contralateral side has been described [ 1,14,24].

ACKNOWLEDGMENTS

We thank Mrs. V. Bergmann for the excellent technical assistance in preparing the figures and Mrs. Schierz for the skillful help in the preparation of the photographic material.

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