A 44 year old Caucasian woman complained of very intense low back and pelvic pain episodes. She had two previous pregnancies with vaginal deliveries and a family history of acute myocardial infarction, stroke, and hypertension. A previous laparoscopy (Fig. 1A, B) revealed substantial pelvic and abdominal vascularisation on the left in the infundibulum and inferior vena cava, excluding the diagnosis of endometriosis.
On physical examination, the patient was 47 kg and her body mass index was below 18.5 kg/cm2. Two imaging tests (Fig. 1C, D) demonstrated compression of the LRV between the SMA and the aorta, suggesting reflux to the left ovarian vein, which was significantly dilated, and explaining the pelvic varices secondary to renal vein compression visible on laparoscopy.
Because the diagnostic hypothesis was pelvic congestion secondary to NCS, the chosen approach was intravascular stent placement (Zilver Vena stent, 16 mm x 60 mm, Cook Medical, US) and balloon angioplasty (ATB Advance, 14 mm x 40 mm, Cook Medical, US) (Fig. 2A, B). Doppler ultrasound (Fig. 3B) and post-operative computed tomography (CT) angiography confirmed that the device was properly placed and the LRV compression was resolved (Fig. 3A, C). The patient reported improvement of symptoms. Nevertheless, one month later, she returned because of intense pain episodes, similar to the previous ones. Doppler ultrasound and magnetic resonance angiography (Fig. 4A) confirmed that the stent was correctly placed, and a patent LRV suggested that the NCS was resolved. Because the patient continued to complain of pain, a new ultrasound was performed, this time by a different operator, to examine the various painful sites. This examination revealed extrinsic compression of the LRV proximal to the vena cava, now by the portal vein (Fig. 4B).
The patient underwent a second surgical procedure for diagnostic purposes to confirm the potential compression. The procedure was performed under local anaesthesia and, as the catheter and guide wire passed through the renal vein, the patient reported low back pain. Following sedation, an intravascular ultrasound (Fig. 5A) was performed, and measurement of intravascular LRV pressure before and after stent placement (Fig. 5B) showed pressure differences and extrinsic vein compression, suggestive of significant stenosis. Then, a second intravascular stent was placed (Zilver Vena stent, 16 mm x 60 mm, Cook Medical, US) using a telescoping technique.
The patient remained without pain for two weeks and was discharged from the hospital. Then, the pain returned. Doppler ultrasound (Fig. 6A), magnetic resonance imaging, and excretion urography revealed nephroptosis of the right kidney (Fig. 6B) that had been previously undetected (Fig. 3A). As the case was reassessed, the patient reported extreme joint mobility for the first time which, in association with the new image demonstrating nephroptosis, opened a new line of investigation. The patient scored 9 (maximum score) on the Beighton scale and met the criteria for EDS and hypermobility. She was referred to a team specializing in pain management with nerve block and to the genetics department, where the diagnosis of EDS was confirmed.