Nutcracker syndrome in adolescent with perineal pain: An interesting case of an adolescent with perineal pain due to pelvic congestion from nutcracker syndrome with relief after balloon venoplasty and sclerotherapy

Abstract

Nutcracker phenomenon is the descriptor for a patient's anatomy whenever the left renal vein becomes compressed between the abdominal aorta and the superior mesenteric artery. Nutcracker syndrome is the terminology used when the nutcracker phenomenon is accompanied by symptoms including pain (abdominal, flank, pelvic), hematuria, and orthostatic proteinuria. Diagnosis can be made with Doppler ultrasound, venography, computed tomography, or magnetic resonance imaging. This case demonstrates some of the typical findings of nutcracker syndrome. The limited clinical features and interesting imaging findings, in addition to the young age of the patient, make this a notable case.

Keywords: Nutcracker syndrome; Pediatric; Pelvic congestion syndrome; Pelvic pain; Sclerotherapy; Venoplasty.

Fig. 1

(A-E) Axial T2 fast spin echo (FSE) demonstrating dilated paracervical and parauterine veins (A); axial T2 FSE with fat suppression normal study for comparison; (B) paracervical and parauterine venous enhancement following gadolinium (C); sagittal T2 FSE with fat suppression demonstrating the dilated paracervical and parauterine veins (D); sagittal T1 FSE with fat suppression demonstrating enhancement following gadolinium, although artifact distorts the superior aspect of the image (E).

Fig. 2

(A-D) Superficial longitudinal view of patient's left labial area demonstrating filling of paralabial vessels with valsalva maneuver (A) without valsalva, (B) with valsalva. Similar portrayal with transverse views (C) without valsalva, (D) with valsalva.

Fig. 3

(A, B) Axial computed tomography (CT) images with contrast demonstrating beak sign the left renal vein (A), sagittal CT with contrast demonstrating acute angle of superior mesenteric artery and abdominal aorta at roughly 39 degrees (B).

Fig. 4

(A, B) Images from the venogram demonstrating impression on the left common iliac vein by the right common iliac artery with minimal reflux into the left internal iliac vein (A). Left renal venogram shows left renal vein compression by the superior mesenteric artery (orange arrow) with evidence of collateral vessels (blue arrow) (B).

Fig. 5

(A-C) Images from left renal and gonadal venograms before sclerotherapy demonstrating reflux into retroperitoneal, lumbar, and gonadal veins (A), gonadal veins (B), and parauterine, perineal, and vulvar varices (C).

Fig. 6

(A-D) Pre- and postembolization of the distal left gonadal vein (A and B). Pre- and postpercutaneous sclerotherapy of the left vulvar varicosities (C and D) both demonstrating decreased aberrant and dilated vessels.