It is defined by the presence of blood or red blood cells in the urine. These elevated pressures may cause poor swallowing of both solid and liquid foods. Ultimately it may be associated with substantial morbidity and even lifethreatening events. Nutcracker syndrome occurs more frequently in women by the 3rd or 4th decades, although cases have been reported in children. Nutcracker esophagus refers to having strong spasms of your esophagus. Nutcracker syndrome ncs, which is the compression of left renal vein lrv between superior mesenteric artery sma and aorta, is such a condition. This buildup, which is a reaction to foods, allergens or acid reflux, can inflame or injure the esophageal tissue. Hematuria definition of hematuria by medical dictionary. Many patients with nutcracker esophagus do not have any symptoms at all, as esophageal manometry studies done on patients without symptoms may show the same motility findings as nutcracker esophagus. Patients with nutcracker esophagus present with severe chest pain leading that can simulate the pain of a heart attack. Nutcracker esophagus may also be associated with metabolic syndrome. The most common clinical manifestations of nutcracker syndrome are left flank pain, pelvic pain, hematuria and gonadal varices. Alport syndrome is a genetic condition characterized by kidney disease, hearing loss, and eye abnormalities. It has been postulated that the mechanism that produces the hematuria is an increase in left renal vein pressure, which may cause minute rupture of thinwalled veins.
On the other hand, there is an appreciable risk of malignancy in older patients eg, over age 35 years with. Left renal vein hypertension, also called nutcracker phenomenon or nutcracker syndrome, is a rare vascular abnormality responsible for gross hematuria. Nutcracker syndrome, hematuria nutcracker syndrome refers to the compression of the left renal vein between the. Unique nutcracker phenomenon involving the right renal artery. Nutcracker esophagus, or hypertensive peristalsis, is a disorder of the movement of the esophagus characterized by contractions in the smooth muscle of the esophagus in a normal sequence but at an excessive amplitude or duration. It results in left renal venous hypertension, and the subsequent development of venous varicosities of the renal pelvis, ureter, and gonadal vein. But in nutcracker esophagus, the contractions are severe and strong leading to pain in chest and difficulty in swallowing. However, it seems to be related to an issue with the muscle function and thickness of the esophagus.
There are different therapeutic options for the treatment of nutcracker syndrome, such as open surgery, endovascular treatment, or conservative treatment. Heartburn this is a burning sensation under the breastbone. Jun 14, 2012 nutcracker syndrome ncs is an uncommon cause of hematuria. 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. It can trigger chest pain, difficulty swallowing and a feeling that food is stuck in your throat. Nutcracker syndrome is defined as compression of the left renal vein between the superior mesenteric artery and the aorta and is associated with urinary abnormalities, fig 8a. Nutcracker esophagus or hypertensive peristalsis is an esophageal motility disorder, where there is abnormal peristalsis or movement of the esophagus. Left renal venous hypertension from extrinsic left renal vein compression should be considered in the differential diagnosis of hematuria, especially in.
Nutcracker esophagus is a form of esophageal spasm. This condition is associated with gastroesophageal reflux disease in many patients. Nutcracker syndrome associated with severe anemia and mild. Recurrent hematuria caused by nutcracker syndrome sciencedirect. 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. The patient has also nutcracker syndrome, which is the compression of. Loin pain hematuria syndrome lphs causes severe, unexplained loin pain and blood in the urine. Nutcracker syndrome is a rare cause of haematuria caused by compression of the.
The exact cause of nutcracker esophagus is unknown. This condition is primarily diagnosed with manometry with high intraesophageal pressure and normal peristalsis. Patients who suffer motility disorders commonly experience two primary symptoms. Hematuria american english or haematuria british english is a clinical sign. Jul 29, 2016 in this paper a case of 12yearold girl with rare association of nutcracker and alport syndrome is presented who admitted for persistent severe microscopic hematuria, recurrent gross hematuria, occasional vomiting, loin pain and positive family history of microscopic hematuria associated with hearing deficit. At the upmc heart and vascular institute, our surgeons in the division of vascular surgery are experts in treating nutcracker syndrome. Nov 09, 2016 nutcracker syndrome treatment options. Children with iga nephropathy, familial benign hematuria, or alport syndrome can have macroscopic hematuria at the time of, or 1 or 2 days following, an upper respiratory tract infectiona phenomenon. We finally went to mayo clinic and she was diagnosed there. Blood or red blood cells can enter and mix with urine at multiple anatomical sites.
With anterior nutcracker syndrome, the left renal vein is compressed between the aorta and the superior mesenteric artery. The nutcracker esophagus is defined by high amplitude peristaltic contractions in the distal esophagus and can be associated with dysphagia andor chest pain. In this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome. Nutcracker phenomenon refers to compression of the left renal vein, most commonly between the aorta and the superior mesenteric artery, with impaired blood outflow often accompanied by distention of the distal portion of the vein. Some people may not have symptoms, while others develop severe and. It refers to the compression of the left renal vein between the aorta and superior mesenteric artery. Dsyphagia this is characterized by the sensation of food getting stuck under the breast bone. Savino, phd c left renal vein hypertension, also called nutcracker phenomenonor nutcracker syndrome,is a rare vascular. Management of renal nutcracker syndrome by retroperitoneal. Nutcracker syndrome diagnosed with 3dimensional computed tomography angiography sang hoon choi, jin sup kim, tae seoup shin, yong seong lee, hyung joo kim, nak gyeu choi from the department of urology, hallym university college of medicine, seoul, korea we report a case of nutcracker syndrome diagnosed with 3dimensional. In women, the hypertension in the left gonadal vein can also cause increased pain during menses. Now i am doing good to get down yogurt and pudding.
Jun 22, 2017 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. Normally when you swallow, the esophagus contract and help the food to move forward. The compression causes renal vein hypertension, leading to hematuria which can lead to. Minimally invasive treatments for venous compression syndromes. My daughter has nutcracker syndrome testicular disorders. Nutcracker syndrome ncs is a syndrome caused by compression of the left renal vein lrv, between the abdominal aorta and the superior mesenteric artery, resulting in hypertension of the lrv and.
Nov 14, 2017 nutcracker esophagus is a rare condition. Nutcracker syndrome servier phlebolymphologyservier. Nutcracker syndrome ncs is caused by a compression of the left renal vein between the aorta and the superior mesenteric artery sma. It is in the same category of esophageal motility disorders as jackhammer esophagus and diffuse esophageal spasms. Nutcracker syndrome accompanying with superior mesenteric. Renal vein entrapment the nutcracker syndrome brandon t. We report a rare cause if a 12yearold boy who presented with a history of frequent intermittent episodes of painless constant. Rare causes of hematuria associated with various vascular. In the absence of clinical symptoms, renal vein compression is referred to as nutcracker phenomenon or. An anatomical framework is helpful in developing a comprehensive differential diagnosis. Some people may not have symptoms, while others develop severe and persistent symptoms. The incidence of nutcracker esophagus in all patients is uncertain.
Hence, patients with unknown causes of unilateral hematuria and flank pain should undergo further studies such as ultrasonography, ct, mri, mra, angiography, or venography to clarify this possibility. Hypertensive esophageal peristaltic contractions nutcracker esophagus are among the most common reported findings in clinical manometric studies 2. We report a rare cause if a 12yearold boy who presented with a history of frequent intermittent episodes of painless constant haematuria. Pumphrey5, lincoln patel2 and balamurugan sankarapandian1 1tarrant nephrology associates, fort worth, texas, usa. Nutcracker syndrome, also called renal nutcracker syndrome is a rare vascular condition. It embraces an extended nonpathognomonic spectrum of symptoms that imply a difficult diagnosis. Kirby sebro 1, lester goetz 1, satyendra persaud 2 1 division of surgical sciences, university of the west indies, st augustine, trinidad and tobago. Spontaneous resolution of hematuria in children with nutcracker syndrome has been reported following an increase in body mass index. It causes difficulty swallowing, or dysphagia, to both solid and liquid foods, and can cause significant chest pain.
Most patients may have symptoms for many years and nondiagnostic evaluations before accurate. Nutcracker syndrome definition of nutcracker syndrome by. It is a rare cause of hematuria, which results from the rupture of congested renal veins into the collecting system. Feb 28, 2018 nutcracker syndrome i pelvic congestion syndrome i update. Nutcracker syndrome consists of haematuria originating from the left collecting system secondary to compression of the left renal vein, between the superior.
The entrapment of the left renal vein is not easily. Nutcracker esophagus symptoms, diagnosis and treatment. Superior mesenteric artery syndrome accompanying with. The most common symptoms of nutcracker syndrome can include. Nutcracker esophagus or hypertensive peristalsiscauses. The nutcracker syndrome ncs results most commonly from the compression of the left renal. Almost all affected individuals have blood in their urine hematuria, which indicates abnormal functioning of the kidneys. Management and diagnosis of nutcracker syndromea case report. Nutcracker esophagus is a motility disorder of esophagus. It is called as anterior ncs if left renal vein lrv is entrapped between aorta and superior mesenteric artery. Nutcracker phenomenon and nutcracker syndrome ncbi. Hematuria that is not explained by an obvious underlying condition eg, cystitis, ureteral stone is fairly common.
Since gerd is likely to be the cause, i recommend that you take steps to deal with that. Pelvic congestion in women, or varicose veins in the lower abdomen that may cause pain and heaviness in the pelvis or genital area. In this paper a case of 12yearold girl with rare association of nutcracker and alport syndrome is presented who admitted for persistent severe microscopic hematuria, recurrent gross hematuria, occasional vomiting, loin pain and positive family history of microscopic hematuria associated with hearing deficit. It belongs to a group of conditions related to abnormal movement and function of the esophagus, known as motility disorders. Instead, blood can flow backwards into other veins and cause them. People with alport syndrome experience progressive loss of kidney function. Aug 04, 2011 does anyone have thisi need help i have pain and nausea everyday i dont even want to eat anymore. In conclusion, nutcracker syndrome is a rare cause of hematuria and is easily missed by routine diagnostic methods. Nutcracker syndrome and loin pain hematuria syndrome youtube. The nutcracker syndrome results most commonly from the compression of the left renal vein between the abdominal aorta and superior mesenteric artery, although other variants exist. In eosinophilic esophagitis eosinofillik uhsofuhjietis, a type of white blood cell eosinophil builds up in the lining of the tube that connects your mouth to your stomach esophagus. Although the pain is very distressing, nutcracker esopahgus is not a serious condition.
Nutcracker esophagus, also known as hypertensive peristalsis, is a benign condition and one of the motility disorders of the esophagus where the patient has contractions in the smooth muscles of the esophagus, which occur for excessive duration or amplitude 180 mm hg. Livingston2, vijay kalaria3, wilder diazcalderon4, john a. Evaluation of asymptomatic microscopic hematuria in adults. Liver, portal vein pv and vertebra are notated for anatomic reference. The blood may be easily seen in the urine or may only be seen under a microscope. We present here a 6yearold girl with episodic macroscopic hematuria who was diagnosed as ncs. Nov 01, 20 nutcracker syndrome ncs is uncommonly diagnosed as a cause of hematuria. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine. Chest pain experienced by most patients with nutcracker esophagus, chest pain may feel the same as the pain produced by a heart attack. I will also be going into detail about my next steps in my journey since i was. In nutcracker syndrome, the renal vein coming from your left kidney becomes compressed and blood cant flow through it normally.
Know the causes, symptoms, treatment and pathophysiology of nutcracker esophagus or hypertensive peristalsis. Hypercontracting nutcracker esophagus radiology reference. Etiology and evaluation of hematuria in adults uptodate. She had surgery, which is left renal vein transposition, where. Nutcracker syndrome ncs is characterized by impeded outflow from the left. Loin pain hematuria syndrome genetic and rare diseases. Its also known as jackhammer esophagus or hypercontractile esophagus. Apr 03, 2018 nutcracker esophagus, also known as hypertensive peristalsis, is a benign condition and one of the motility disorders of the esophagus where the patient has contractions in the smooth muscles of the esophagus, which occur for excessive duration or amplitude 180 mm hg. Nutcracker esophagus is a condition associated with very high pressures greater than 180 mm hg that happen in the esophagus during the swallowing process. Currently, there is no published data to indicate an increased risk for esophageal cancer in patients with nutcracker esophagus. Nutcracker syndrome is a rare phenomenon, with few cases described.
Renal nutcracker syndrome genetic and rare diseases. The most commonly reported symptom of nutcracker syndrome is hematuria. Nutcracker phenomenon definition of nutcracker phenomenon. She had surgery, which is left renal vein transposition, where her symptoms resolved for about two weeks. Gross haematuria in patients with nutcracker syndrome nefrologia. The symptoms of nutcracker esophagus can be alarming chest pain so severe that it can be mistaken for a heart attack and the feeling that food is stuck under the breastbone. Nutcracker syndrome ncs is characterized by impeded outflow from the left renal vein lrv into the inferior vena cava ivc due to extrinsic lrv compression. Hypercontracting nutcracker esophagus is a motility disorder of the esophagus. The nutcracker syndrome ncs is the clinical equivalent of. Eosinophilic esophagitis symptoms and causes mayo clinic. Evaluation of asymptomatic microscopic hematuria in adults this is a corrected version of the article that appeared in print. Nutcracker syndrome european heart journal cardiovascular.
Several refluxmd members have asked about a motility disorder known as nutcracker esophagus. One of the major causes of nutcracker esophagus is gastroesophageal reflux. A 21yearold chinese woman was admitted with a sevenmonth history of unilateral severe hematuria. Nutcracker esophagus hypertensive peristalsis is a condition associated with poor swallowing of both solid and liquid foods due to very high pressures in the esophagus during the swallowing process. The name derives from the fact that, in the sagittal plane andor transverse plane, the sma and aa appear to be a nutcracker crushing a nut. Nutcracker syndrome can be treated, and treatments depend on your age, anatomy, and symptoms. Clinically, nutcracker syndrome is characterized by intermittent hematuria with or without left flank or abdominal pain. Jan 24, 2019 in this video i will be describing the similarities between nutcracker syndrome and loin pain hematuria syndrome.
I have been suffering with chest pain, throat pain and food and pills hanging up in my throat since january. Nutcracker esophagus is one of several motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. A rare and potentially under diagnosed cause of haematuria. They said my colon doesnt function properly so thats what they think is causing it but i also have a compressed renal vein which can cause nutcracker syndrome. Gross hematuria is defined by the presence of an increased number of red blood cells rbcs in the urine that is visible to the naked eye. The frequency and length of pain episodes can vary.
What is nutcracker esophagus hypertensive peristalsis. A 6yearold girl was admitted to the hospital with intermittent hematuria of 2 weeks. Nutcracker esophagus digestive disorders gastroenterology. Nutcracker syndrome diagnosed with 3dimensional computed. Another clinical study has shown that nutcracker syndrome is a frequent finding in varicoceleaffected patients and possibly, nutcracker syndrome should be routinely excluded as a possible cause of varicocele and pelvic congestion. Posterior ncs occurs when the persistent posterior branch of fetal periaortic vascular ring gets compressed between aorta and vertebral body. Chest pain experienced by most patients with nutcracker esophagus, chest pain may feel the same as the pain produced by a heart attack dsyphagia this is characterized by the sensation of food getting stuck under the breast bone heartburn this is. The nutcracker syndrome ncs is a rare cause of haematuria. She was diagnosed in april 2009 after over a year and a half of trying to figure out what was wrong. Dysphagia difficulty with swallowing or chest pain, which is described as noncardiac chest pain due to its origin in the esophagus. Urine may be red, bloody, or colacolored gross hematuria with oxidation of blood retained in the bladder or not visibly discolored microscopic hematuria. The underlying anatomic anomaly, first described by gant in 1937, can lead to left renal venous. Hematuria is red blood cells rbcs in urine, specifically 3 rbcs per highpower field on urine sediment examination.
Nutcracker syndrome refers to the compression of the left renal vein between the aorta and the superior mesenteric artery, which results in elevated left renal vein pressure and possible collateral vein development. The phenomenon is attributable to the idiopathic decrease in the angle between the aorta and the superior mesenteric artery with consequent compression of the left renal vein. Isolated hematuria merck manuals professional edition. Although an uncommon occurrence in children, it is nevertheless a disturbing finding for both the affected child and hisher family. Posterior nutcracker syndrome with left renal vein. Microscopic examination of a urine specimen can reveal red blood cells not evident to the naked eye. Nutcracker syndrome caused by compression of the left renal vein between the abdominal aorta and the superior mesenteric artery is a rare anatomopathological condition.
I have to all but pulverize my meds to get them down. Furthermore, the venous return from the left gonadal vein returning to the left renal vein is blocked, thus causing testicular pain. In some cases, nutcracker syndrome may not need treatment, especially for patients who are. 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. Extrinsic compression of the left renal vein lrv between the superior mesenteric artery sma and the aorta ao is known as nutcracker syndrome. Hematuria should be from the left ureteric orifice only 4. Nutcracker syndrome an overview sciencedirect topics. Symptoms are often aggravated by physical activity and commonly include hematuria, pain or gonadal vein syndrome, varicocele, orthostatic proteinuria, and. The nutcracker syndrome domenico russo, md, roberto minutolo, phd, vittorio iaccarino, md, micheleandreucci, phd, alfredo capuano, md, and francescoa. In many such patients, particularly young adult patients, the hematuria is transient and of no consequence 1. Hematuria, renal nutcracker syndrome, superior mesenteric artery. The nutcracker phenomenon ncp and the nutcracker syndrome ncs.