A mother and her childs journey to find answers.
Compressions are common in those with connective tissue disorders like Hypermobility Spectrum and Hypermobile Ehler’s Danlos. Many who have these conditions are not diagnosed. If you are bendy and flexible, you may be affected by a connective tissue issue. When the connective tissue is weak, organs can droop, causing compression of abdominal arteries. It can cause NDPH-style pain. Note that there are other abdominal compressions, including median arcuate ligament syndrome (MALS) and Superior Mesenteric Artery Syndrome (SMAS). I am only covering Nutcracker and May Thurner because these are the two compressions documented that can be a cause of NDPH. Note there are great Facebook support groups if you want to learn more about these conditions. For Nutcracker, join Renal Nutcracker Support Group (https://www.facebook.com/groups/1452002075077453), and for May Thurner Syndrome (https://www.facebook.com/groups/28440704811). Given the difficulty in diagnosis, missed diagnosis is very common. If you suspect you may have one of these compression syndromes, get the testing done, join the appropriate Facebook group for the compression you have, and send your imaging to the specialists listed in those groups. This is how to find doctors that know these rare diseases and know how to fix them.
Nutcracker Syndrome (NCS):
Nutcracker Syndrome is compression of the left renal vein by the superior mesenteric artery. This syndrome got its name because it looks like a nutcracker squeezing the left renal vein. It blocks blood at the left kidney. Most vascular surgeons think NCS presents with only pelvic symptoms. This is very wrong. This compression can cause blood in the urine and other pelvic symptoms, but it depends on how the body handles the backed-up blood. The body often grows collateral veins to drain the blood. These collateral veins can grow into the pelvic area, spine, and adrenal gland. If they grow into the spine, this is where the backed-up blood is dumped. Symptoms: NCS can cause high intracranial pressure, NDPH-style headaches, POTS, and CSF leaks (low cranial pressure) left flank pain, back pain, abdominal pain, nausea, vomiting, pain when eating, varicose veins in the pelvic area, blood in urine, frequent UTIs, and more. Testing for NCS includes a Renal Duplex Doppler Ultrasound, CT or MR Angiogram, Venogram, and a Renal Hilar Block. Treatment can be a renal vein bypass (not always great for those with hEDS or connective tissue disorders, but it depends on what the specialist thinks), an auto kidney transplant where they move the left kidney out of the compression to the right side, or a nephrectomy. It is recommended to NEVER allow a stent in the renal vein for NCS because stents migrate easily, and this will cause vein damage that is difficult to impossible to repair. https://rarediseases.info.nih.gov/…/renal-nutcracker…
May Thurner Syndrome (MT):
MT is compression of the iliac vein which can cause a lot of leg pain, headaches, abdominal pain, etc. When the iliac vein is compressed, the body can grow collateral veins to move the backed-up blood. In some cases, these collateral veins can grow into the tailbone area. Symptoms: Headache, restless leg, leg warmth and swelling, nausea, vomiting, cysts around the tailbone if there is a collateral vein growing into it, blood clots, etc. Testing for MT includes a CT or MR Angiogram and a venogram. Treatment is typically a stent in the artery. https://my.clevelandclinic.org/…/17213-may-thurner…