Renal nutcracker syndrome genetic and rare diseases. Nutcracker syndrome ncs or left renal vein lrv entrapment is rare and may be caused by compression of the vein between the aorta and the superior mesenteric artery sma. The presence of hematuria, proteinuria, and pain albeit atypically right sided made it nutcracker syndrome. Ovarian vein embolization for the treatment of pelvic congestion syndrome. Before envisaging ovarian embolization, type ii insufficiency, according to the classification described by greiner et al.
She had surgery, which is left renal vein transposition, where. Although aggressive treatment in the early stages offers the best prognosis. A therapeutic option in nutcracker syndrome and ovarian. Embolization of the ovarian and iliac veins for pelvic congestion syndrome page 1 of 6. Embolization of the ovarian and iliac veins for pelvic congestion syndrome page 3 of 6. Mesoaortic compression of the left renal vein nutcracker syndrome produces both obstruction and reflux, resulting in symptoms of pelvic congestion. Duplex ultrasound is firstline imaging for all patients presenting with signs or symptoms of pelvic venous insufficiency. Nutcracker syndrome complicated by left renal vein thrombosis. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and aa with some imagination appear to be a nutcracker crushing a nut the renal vein. This syndrome results most commonly from the compression of the left renal vein between the abdominal aorta aa and superior mesenteric artery sma, although other variants exist. The left ovarian vein drains most often into the left renal vein. The term syndrome refers to a group of signs and symptoms primarily occurring together, characteristic of a specific condition.
Nutcracker syndrome is an easily missed cause of hematuria in children. Nutcracker syndrome is an underdiagnosed occurrence that can also result in ovarian vein insufficiency. Multidetector ct of vascular compression syndromes in the. Pelvic venous congestion syndrome pcs or pelvic venous insufficiency incompetence is similar to the condition of varicose veins in the legs. The underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous. Some people may not have symptoms, while others develop severe and. When symptomatic, such compressions can result in a variety of uncommon syndromes in the abdomen and pelvis, including median arcuate ligament syndrome mals, maythurner syndrome, nutcracker syndrome, superior mesenteric artery sma syndrome, upj obstruction, ovarian vein syndrome ovs, and other forms of ureteral compression.
Pelvic venous insufficiency pvi is defined as venous reflux in the ovarian vein ov, internal iliac vein iiv, or parauterine veins. The mild nutcracker syndrome can be witnessed in children during the changes their body undergoes due to growth. Pelvic congestion syndrome pelvic venous incompetence. Anticoagulation was started and three weeks later, cta showed complete disappearance. We present a patient whose complaint of flank pain led to the diagnosis of a renal vein thrombosis. The left ovarian or spermatic vein empties into the left renal vein and is an additional site of venostasis in. Despite advances in treatment, the survival rate has risen only slightly since 1950. Female ovarian vein insufficiency clinical indications. In this article, we refer to this heterogeneous group of. Patients with pelvic insufficiency typically have varied symptoms that are nonspecific that makes diagnosis difficult. The objective is to assess the inferior vena cava ivc. Type 3 patients are diagnosed with gonadal vein obstruction due to a mesenteric aortic compression of the left renal vein nutcracker syndrome.
Meissner the nutcracker syndrome is characterized by entrapment and compression of the left renal vein between the abdominal aorta and the superior mesenteric artery sma figure 1. Nutcracker phenomenon ncp, also known as left renal vein entrapment, is characterized by impeded outflow from the left renal vein lrv into the inferior vena cava ivc due to extrinsic lrv compression, often accompanied by demonstrable lateral hilar dilatation and medial mesoaortic narrowing schematic representation of ncpnutcracker syndrome ncs in figure 1. Duplex ultrasound investigation in pelvic congestion. It forms a rich anastomotic venous plexus with the paraovarian, uterine, vesical, rectal and vulvar venous plexus. The prevalence of nutcracker syndrome is unknown, and the diagnostic criteria vary 1517. Pelvic congestion syndrome radiology reference article. The nutcracker syndrome ncs results most commonly from the compression of the left renal vein lrv between the abdominal aorta aa and superior mesenteric artery sma, although other variants exist. Primary pelvic insufficiency includes congenital or acquired ovarian vein incompetence from nonobstructive causes. Our protocol for transabdominal pelvic vein duplex ultrasound. Pelvic congestion syndrome and ovarian vein reflux.
However, obstruction of the gonadal outflow may produce similar symptoms. Some people may not have symptoms, while others develop severe and persistent symptoms. Symptoms of pelvic venous congestion chronic pelvic pain, dyspareunia, dysuria, and dysmenorrhea have been attributed to massive gonadal reflux. Renal nutcracker syndrome ncs is a condition that occurs when the left renal vein the vein that carries blood purified by the left kidney becomes compressed. The nutcracker syndrome is a vascular disorder, but the signs and symptoms are mainly urological or gynecological. Dilated left gonadal vein and pelvic varices indicate left renal vein lrv ouflow obstruction by the superior mesenteric artery sma i ordered a renal duplex and a ct venogram for procedural planning. I just recently had a major surgery for something called nutcracker syndrome. Nutcracker phenomenon ncp, also known as left renal vein entrapment, is characterized by impeded outflow from the left renal vein lrv into the inferior vena cava ivc due to extrinsic lrv compression, often accompanied by demonstrable lateral hilar dilatation and medial mesoaortic narrowing schematic representation of ncp nutcracker syndrome ncs in figure 1. It is a commonly missed and potentiallytreatable cause of chronic abdominopelvic pain. Imaging appearance and nonsurgical management of pelvic.
Gonadal vein transposition for the treatment of nutcracker syndrome. Patients may present with hematuria, left flank pain, dyspareunia, and vaginal or abdominal wall varicose veins. Nutcracker syndrome is a relatively rare condition that occurs when the vein running from the left kidney becomes compressed between the abdominal aorta and the superior mesenteric artery. Venous coil embolization is used when there are varicose veins in the pelvis that are causing significant pain pelvic congestion syndrome. We finally went to mayo clinic and she was diagnosed there. Pelvic congestion syndrome some prefer pelvic venous insufficiency 9 is a condition that results from retrograde flow through incompetent valves in ovarian veins. Pelvic congestion syndrome, also known as pelvic vein incompetence, is a long term condition in women believed to be due to enlarged veins in the lower abdomen. Two to three trunks form a single ovarian vein at the l4 interspace. Nutcracker syndrome is a variation of nutcracker phenomenon or renal vein entrapment syndrome, in which the arteries near the kidney compress the left renal kidney vein. Some patient can develop pelvic varicose veins due to compression of the left renal vein nutcracker syndrome or from compression of the iliac veins causing blood to flow through.
Variations are common in the number of trunks double or triple and in the mode of termination. Type 4 patients have gonadal vein insufficiency that is characteristic of pcs. Venous coil embolization vein specialists of the carolinas. The nutcracker syndrome in a venous or even broader scope, vascular compression syndromes is based upon decreased space between the superior mesenteric artery and the aorta in the area of the left renal vein. A diagnosis of nutcracker syndrome was made and a left renal. My daughter has nutcracker syndrome testicular disorders. Vulvovaginal varicosities and pelvic congestion syndrome. The condition may cause chronic pain, such as a constant dull ache, which can be worsened by standing. In most cases, compression of the left renal vein is between the abdominal aorta the main artery in the abdomen and the superior mesenteric artery, which brings blood. These patients may have lower extremity varicosities that originate in the pelvis and may also have associated hypogastric. Portal hypertension and acquired inferior vena cava syndrome may be related to the development of secondary pelvic venous congestion 2, 5, 6. Imaging diagnosis, treatment approaches, and therapeutic issues m. Embolization of the ovarian and iliac veins for pelvic.
Renal vein entrapment the nutcracker syndrome brandon t. The renal vein assimilates a nut in a nutcracker caught between two stronger objects. It is characterized by left renal vein entrapment between the abdominal aorta and the superior mesenteric artery causing renal venous hypertension. Pelvic congestion syndrome and its relationship to varices. Nutcracker syndrome as an unusual cause of postprandial.
Nutcracker syndrome wikimili, the free encyclopedia. In addition, venous outflow obstruction from anatomic variants, such as compression of the left renal vein by the superior mesenteric artery nutcracker syndrome may result in engorgement of the left ovarian vein. Most of the varicose veins are located around the uterus and ovaries and sometimes the bladder and rectum. Pelvic venous congestion is a commonly overlooked condition that can be severely painful and debilitating for many women.
Whereas a fairly large body of data regarding transcatheter. Mri and venographic aspects of pelvic venous insufficiency. The right ovarian vein drains directly into the inferior vena cava and occasionally into the right renal vein. Venography revealed compression of the left renal vein, extensive pelvic varices, and significant engorgement of her left ovarian vein.
In this case, abdominal computed tomography angiography was helpful in diagnosing the nutcracker syndrome complicated by the renal vein thrombosis. The nutcracker syndrome ns may lead to insufficient perirenal collaterals as well as incompetence of the left ovarian vein with consecutive ovarian vein insufficiency. Pelvic congestion syndrome is recognized when the diameter of the ovarian vein measures greater than 5 mm by transvaginal sonography and is visually tortuous2. Nutcracker syndrome is a vascular compression disorder that refers to the compression of the left renal vein most commonly between the superior mesenteric artery sma and aorta, although other variations can exist 1 this can lead to renal venous hypertension, resulting in rupture of thinwalled veins into the collecting system with resultant hematuria. Nutcracker syndrome nutcracker syndrome describes the compression of the left renal vein between the aorta and the superior mesenteric artery fig 1, which causes clinical symptoms such as hematuria, proteinuria, and flank pain.
A case report of a young girl with recurrent hematuria. Nutcracker syndrome is a rare entity in which compression of the left renal vein lrv, usually by the overlying superior mesenteric artery sma, results in renal venous congestion and reflux in the left ovarian vein lov. Nutcracker syndrome ncs is symptomatic unilateral renal venous hypertension due to compression of the left renal vein between the superior mesenteric artery and aorta anterior ncs or between the aorta and spine posterior ncs. The syndrome is characterized by a complex of ymptoms with substantial variations, the diagnosis is difficult and. The term pelvic congestion syndrome specifically refers to the condition first described by louis alfred richet in 1857 and characterized by chronic, dull pelvic pain, pressure, and heaviness that persist for more than 6 months with no other cause. Prevalence and management of ovarian vein insufficiency in the presence of leg venous insufficiency. Pelvic congestion syndrome pcs is caused by varicose veins in and around the pelvic organs and therefore the symptoms include the uterus, vagina, bladder and bowel. The reflux may or may not result in increased venous engorgement i.
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