Persistent Sciatic Vein Research Articles

Klippel-Trénaunay Syndrome: Spectrum and Management

To describe a series of 252 patients with Klippel-Trénaunay syndrome (KTS), a rare congenital malformation characterized by the triad of capillary malformations, atypical varicosities or venous malformations, and bony or soft tissue hypertrophy usually affecting one extremity. We reviewed the clinical characteristics and findings in 136 female and 116 male patients with KTS who underwent assessment at Mayo Clinic Rochester between January 1956 and January 1995. In addition, management options are discussed. Capillary malformations (port-wine stains) were found in 246 patients (98%), varicosities or venous malformations in 182 (72%), and limb hypertrophy in 170 (67%). All three features of KTS were present in 159 patients (63%), and 93 (37%) had two of the three features. Atypical veins, including lateral veins and persistent sciatic vein, occurred in 182 patients (72%). Operations performed in 145 patients with KTS included epiphysiodesis, stripping of varicose veins or venous malformations, excision of vascular malformations, amputations, and debulking procedures. Most patients with KTS should be managed conservatively. The clearest indication for operation is a leg length discrepancy projected to exceed 2.0 cm at skeletal maturity, which can be treated with epiphysiodesis in the growing child. If a functioning deep vein system is present, removal of symptomatic varicosities or localized superficial venous malformations in selected patients can yield good results.

Persistent sciatic vein: Diagnosis and treatment of a rare condition

Persistent sciatic vein (PSV) is a rare anomaly most often associated with Klippel-Trenaunay Syndrome (KTS). Magnetic resonance imaging (MRI) and extended use of phlebography have increased the frequency of diagnoses. To evaluate our experience, we reviewed patients in whom PSV was diagnosed at our institution. MRIs and phlebograms of all patients with KTS seen since 1985 were reviewed, and patients with PSV were identified. Forty-one of 186 patients with KTS underwent MRI. PSV was diagnosed in 20 of the 41 (48.8%). In addition, one other patient without KTS had PSV. Thirteen patients (61.9%) were female. Patient age ranged from 5 to 71 years, with a median age of 22 years. PSVs were present in the entire thigh and buttock of eight patients (38.1%), the upper thigh and buttock of six patients (28.6%), and the lower thigh of seven patients (33.3%). Six patients (28.6%) had anorectal arteriovenous malformations with heavy bleeding. Five patients (23.8%) had pulmonary embolization. Two patients (9.5%) died. Amputation was required in one (4.8%). Nineteen patients (90.5%) underwent no specific surgery for PSV. Two patients (9.5%) underwent successful excision of PSVs. Excision of PSV appears to be rarely indicated. It may be indicated for localized symptoms in patients whose condition and anatomy permit. PSV may be a marker for patients with more extensive arteriovenous malformations and for those at high risk for rectal bleeding and pulmonary embolization. Prognosis is related to the associated vascular malformations. MRI and extended use of phlebography should increase the frequency of diagnoses. Treatment rationales should evolve.