Dear friends,

Please forgive my very heavy presentation but it looks like the case deserves your attention.

A 9 year old patient presented to our outpatient department with a “varicose vein” on the left thigh, extending across the pubic region towards the right thigh (see pictures below).

On examination, the child was a healthy-looking 9 year old.
Asymmetry of the face was evident, with the maxillary region of the left side swollen and erythematous. There was also bilateral scleral melanosis.
The trunk was normal except for an erythematous region over the lower sternum, and midline posteriorly extending from T6 – T11 levels. There was no visible nor palpable deformity of the spinous processes.
Both upper limbs were normal, and of equal length and circumference.
The left leg was 64 cm in length, while the right leg measured 62cm.
A difference of 3.8 cm in circumference was noted at mid-thigh level, and a difference of 2.3 cm in cicumference in calf diameter, with the left side of greater circumference.
The child walked with a short-limbed gait as a result of the discrepancy in length between the two lower limbs.

Abdominal examination, and that of the chest were unremarkable.

Fundoscopy was normal bilaterally, and examination of the nervous system revealed no abnormalities. The child appeared to be of normal intellect, although a history was obtained of poor performance at school.

On examination of the abnormal vessel, it appeared to be continuous with the left great saphenous vein, extending across the pubis, and apparently communicating with the deep venous circulation in the right thigh. Flow was clinically from left to right, and there were no bruits audible.
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Doppler ultrasonography of the right leg revealed significant turbulence and high velocity flow in the right common femoral vein. High systolic velocity was also discovered in the right common femoral artery.

CT angiography revealed absence of the left common iliac vein, with the abnormal vein apparently draining into the right external iliac (see pictures below).
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There was no evidence of persistence of the posterior cardinal vein.

The findings were discussed at our departmental academic meeting, and the following points were brought up for further consideration, with cognizance taken of our situation in a developing nation:

- Is there an isolated absence of the left common iliac vein?
- Is there a co-existent arterial abnormality?
- What path should further investigations take? Would magnetic resonance angiography be indicated?
- Does this pathology necessitate further expensive and invasive investigation? Would such investigation be of benefit to the patient or of purely academic benefit?
- Does this condition mandate surgery? Of what nature, and to what effect?

1. Rev Hosp Clin Fac Med Sao Paulo 2001
Sep-Oct;56(5):159-62Related Articles, Links
Persistence of the embryonic lateral marginal vein: report of two cases.
Rojas Martinez R, Puech-Leao P, Guimaraes PM, Netto BM.
Department of Vascular Surgery, Hospital das Clinicas, Faculty of
Medicine, University of Sao Paulo, Brazil.

PURPOSE: Congenital venous malformations of the lower limbs represent a
particular challenge for the vascular surgeon. Persistence of fetal
veins is a rare malformation, and the most common is the persistence of
the lateral marginal vein usually observed in patients with
Klippel-Trenaunnay Syndrome. The persistence of this embryonic vein as
an isolated venous malformation without the other characteristics of the
Klippel-Trenaunnay Syndrome has not yet been reported. This paper
describes two cases. METHODS: Two patients, a 17-year-old male patient
and a 16-year-old female, have had since their birth a large venous
trunk in the lateral aspect of the right leg and thigh. The limbs
underwent duplex scanning and phlebography. The surgical removal of the
lateral marginal vein was performed. RESULTS: Surgical treatment
resulted in very good functional and aesthetic results. Follow-up at 26
months showed no evidence of varicose vein recurrence. CONCLUSIONS: To
achieve good results, surgical intervention may be indicated in cases of
orthopedic deformity, hemorrhage, symptomatic, and unaesthetic lesions.

2. J Vasc Surg 2002 Aug;36(2):396-400Related Articles, Links
Persistent sciatic vessels, varicose veins, and lower limb hypertrophy:
an unusual case or discrete clinical syndrome?
Parry DJ, Aldoori MI, Hammond RJ, Kessel DO, Weston M, Scott DJ.
Department of Vascular Surgery, Huddersfield Royal Infirmary.
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Persistent sciatic artery is a rare congenital anomaly with a high
incidence rate of aneurysmal degeneration and risk of
thromboembolization or rupture. Despite a number of recognized
associations, the presence of coexistent venous anomalies is extremely
rare. We present the case of a 27-year-old woman with atypical
left-sided varicose veins and soft tissue hypertrophy. Imaging showed
persistence of both sciatic artery and vein. Whether these anomalies are
an incidental finding or represent a discrete clinical syndrome remains
unclear. We emphasize that unusual distribution varicose veins may be
associated with underlying persistent sciatic vessels and recommend
formal duplex scan assessment for these anomalies.