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W. Sandmann, T. Scholbach, B. Borowski

One stage Surgical Decompression for Simultaneous Complex Venous Congestion Disorders in Non-thrombotic Patients which are affected by Hypermobility Spectrum Disorders /HEDS

Objective

Patients which suffer from simultaneous presence of nutcracker and MAY-THURNER syndrome are mostly female and affected by inherited HSD/hEDS. Due to concomitant arterial – visceral compressions these individuals have lost weight continuously until break down of heath stability at the age of adolescence and above.

Methods

We report about 201 patients (female mean age 28,7 ys., male mean age 28,2 ys., which had undergone preoperative USD and subsequently CTA. 168 patients presented with NCS (anteaortic LRV 159, retroaortic LRV 9) and with MTS simultaneously. n=37 patients presented with neurological issues (6 left side monopareses, 31 left leg senso motoric weakness). All patients underwent decompression of LRV and LIV applying extravenous ringenforced PTFE tube graft protection during one operation. N=13 patients (6,4 %) which had failed success of stenting elsewhere (NCS 4, MTS 9) and were decompressed as well. 33 patients had undergone either decompression for one vein or arterio visceral syndrome simultaneously.

All grafts were fixed by nonabsorbable stay sutures according to a meticulously designed protocol. Intra. op. photography was used for anatomy and control of graft position. CTA / MRI was performed at dismission to control the operative results.

Results

In five patients (2,8 %) the LRV and LIV could not be decompressed and had the veins to be replaced by artificial grafts due to significant malformation (retroaortic n= 3). In only one patient the LIV PTFE ring enforced graft had collapsed. The mother was pressing her children, resulting in renewal of the graft. In all other patients, successful decompression of NCS and for MTS was encountered while the graft diameter remained stable. Significant Improvement based on survey was reported in (male i93 %), (female in 82 %). All elsewhere stented patients reported stable improvement after our surgery. All patients being admitted because of paresis / monopareses had left the hospital neurologically normal, walking on their feet. No infection nor death was encountered despite these complex cases.

Conclusions

We have developed an approach to decompress the LRV for nutcracker syndrome according to the technic described by R. Barnes in 1988 (JVS). We have developed that technic further to be applied for LIV in patients with MTS and NCS at one stage surgery. Although stenting in these patients instead of surgery seems very attractive, but these hEDS/HSD patients have complex health issues and surgery is the safer way to follow.