Superior mesenteric artery syndrome coexists with nutcracker. The sma frequently rises at an acute angle from the aorta, but can have a low or lateral origin, and the distance between the sma and the aorta at the level of the lrv is reduced, as shown by fu. Sma syndrome, or aortomesenteric compression, occurs when an abnormally acute angle is formed between the abdominal aorta and the sma, causing an extrinsic compression of the third. The velocity of the lrv flow proximal to the narrowing was 23 cmsec with an increase to 350 cms at the narrowing. 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. This rare, potentially lifethreatening syndrome is typically caused by an angle of 625 between the aa and the sma, in. Imaging findings and clinical features of abdominal vascular. Superior mesenteric artery syndrome smas is a digestive condition that occurs when the duodenum the first part of the small intestine is compressed between two arteries the aorta and the. When the angle becomes too acute, compression of either structure. 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.
Imaging findings and clinical features of abdominal. Superior mesenteric artery sma syndrome is a rare cause of gastrointestinal obstruction, caused by external compression of the third part of the duodenum by the sma. Mar 22, 2018 the aortasma angle was measured at 15. At the upmc heart and vascular institute, our surgeons in the division of vascular surgery are experts in treating nutcracker syndrome. Nutcracker syndrome is characterized by a set of signs and symptoms secondary to compression of the left renal vein lrv either in the acute anatomic angle between the abdominal aorta aa and the origin of the superior mesenteric artery sma known as anterior ncs 2,3,4,5 or in a retroaortic position between aa and vertebral column, which. A diagnosis of nutcracker syndrome was made figures 3 and 4.
Superior mesenteric artery syndrome project gutenberg self. 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. Using a definition of sma angle nutcracker syndrome, and using a dd definition of nutcracker and superior mesenteric artery sma syndromes are rare conditions where the left renal vein or duodenum may be compressed by an unusually acute angle between the sma and aorta. Superior mesenteric artery sma syndrome is a rare, potentially lifethreatening gastrovascular disorder characterized by a compression of the third and final portion of the duodenum by the abdominal aorta aa and the overlying superior mesenteric artery. The specific causes of nutcracker syndrome can vary. Superior mesenteric artery syndrome radiology reference. We retrospectively measured sma angles, left renal vein lrv distance, and duodenal distance dd in 205 consecutive pediatric abdominal ct. Diagnosis and surgical treatment of nutcracker syndrome. The normal angle between the sma and the abdominal aorta is. Sma syndrome is also known as wilkies syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arteriomesenteric occlusion. It should not be confused with nutcracker syndrome which can be an.
This narrowing of the left renal vein can lead to increase venous pressure within the renal circulation and promote development of varices of the renal. In nutcracker syndrome, the renal vein coming from your left kidney becomes compressed and blood cant flow through it normally. If clinical symptoms are mild, conservative treatment is implemented. A differential diagnosis of haematuria following a motor.
The nutcracker syndrome should be considered as an uncommon cause of haematuria, particularly when a left sided varicocoele is also present. A narrow aortomesenteric angle causes compression or entrapment of the. Superior mesenteric artery sma syndrome, also known as wilkie syndrome, is a rare acquired vascular compression disorder in which acute angulation of the superior mesenteric artery sma results in compression of the third part of the duodenum, leading to obstruction. Axial contrastenhanced ct of the abdomen demonstrated compression of the left renal vein between the superior mesenteric artery sma and the aorta as the left renal vein crosses midline figure 1.
Kirby sebro 1, lester goetz 1, satyendra persaud 2 1 division of surgical sciences, university of the west indies, st augustine, trinidad and tobago. A young female presented to the emergency department following a motor vehicle collision. This rare, potentially lifethreatening syndrome is typically caused by an angle of 625 between the aa and the sma, in comparison to the normal range of 3856, due to. Nutcracker syndrome is characterized by a set of signs and symptoms secondary to compression of the left renal vein lrv either in the acute anatomic angle. 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. Superior mesenteric artery syndrome and the nutcracker. It has been referred to by a variety of other names, including cast syndrome, wilkie syndrome, arteriomesenteric duodenal obstruction, and chronic duodenal ileus. 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 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. Nutcracker syndrome due to leftsided inferior vena cava.
The incidence of sma syndrome has been found to be. Though sma syndrome and renal nutcracker syndrome share the same pathogenesis. Nutcracker syndrome ncs is characterized by impeded outflow from. Nutcracker syndrome accompanying with superior mesenteric. 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. On ct, the beak sign is an abrupt narrowing of the lrv between the aorta and sma, with proximal dilatation of the lrv. A case report of a young girl with recurrent hematuria. Ncs refers to a syndrome caused by significant compression of lrv between the abdominal aorta and sma, resulting in hypertension of the lrv and hematuria due to rupture of the thinwalled veins close to the calyceal fornices 10,11. Sma syndrome and nutcracker syndrome have common features that result from narrowed aortomesenteric angle. The term nutcracker syndrome can also be used for compression of the left ivc. Nutcracker syndrome combined with superior mesenteric artery. To our knowledge, there are few reports in the literature describing the coexistence of these two conditions and so we take this.
When the angle becomes too acute, compression of either structure can occur. Nutcracker syndrome refers to the compression of the left renal vein. Patients with lower body mass index may be at particular risk for nutcracker syndrome as a decrease in retroperitoneal fat is thought to reduce the aortomesenteric angle. An aortomesenteric angle between the superior mesenteric artery sma and aorta. The nutcracker and superior mesenteric artery sma syndromes are rare conditions where the left renal vein or duodenum may be compressed by an unusually acute angle between the sma and aorta. Superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction. Nutcracker syndrome symptoms, treatment, causes, surgery. Superior mesenteric artery syndrome is an uncommon cause of high gastrointestinal tract obstruction, and should be suspected when endoscopic examination does not reveal an intrinsic obstruction. Nutcracker syndrome is caused by compression of the left renal vein between the aorta and. The nutcracker and superior mesenteric artery sma syndromes are rare conditions where the left renal vein or duodenum may be compressed by an unusually acute angle between the sma and aorta, although the normal angle in children is unknown.
Doppler ultrasonography us has been commonly used for the diagnosis of ncs. Nutcracker syndrome servier phlebolymphologyservier. Superior mesenteric artery syndrome accompanying with. Nutcracker syndrome ncs refers to the clinical manifestations of ncp. The angle between the aa a and the sma b was less than 35 measured at c the third portion of the duodenum was compressed by the decreased aortomesenteric angle in the transverse plane. Feb 14, 2019 it is enveloped in fatty and lymphatic tissue and extends in a caudal direction at an acute angle into the mesentery. Nutcracker syndrome in the left renal vein may also occur following a decrease in the aortomesenteric angle. The duodenum and the left renal vein occupy the vascular angle made by the superior mesenteric artery and the aorta. Less often, the third portion of the duodenum courses in front of the lrv between the aorta and the sma. Current trends in the diagnosis and management of renal.
The normal superior mesenteric artery sma originates behind the neck of the pancreas at the level of the first lumbar vertebra, and usually creates an acute angle at its origin from the aorta. Ct imaging showing sma syndrome and nutcracker syndrome. The angle between the aa a and the sma b was less than 35 measured at c the third portion of the. It may be associated with the nutcracker phenomenon. In addition to an appropriate clinical history, ct findings indicating decreased aortomesenteric angle and a shortened aortomesenteric distance can suggest the diagnosis for both the superior mesenteric artery syndrome and accompanying nutcracker syndrome. Nutcracker syndrome leads to orthostatic proteinuria and varicocele, and symptoms relieved in supine position.
In the majority of patients, the normal angle between the superior mesenteric artery and the aorta is between 38 and 65. Jan 23, 2019 ct imaging showing sma syndrome and nutcracker syndrome. Some people are born with certain blood vessel variations that can lead to symptoms of nutcracker syndrome. The term nutcracker phenomenon ncp refers to compression of the left renal. Renal nutcracker syndrome genetic and rare diseases. Nutcracker phenomenon is an uncommon condition characterized by compression of the left renal vein lrv between the abdominal aorta and the. Recommended protocol in instances of suspected nutcracker phenomenon.
The nutcracker phenomenon left renal vein lrv entrapment syndrome refers to compression of the lrv most commonly between abdominal aorta and superior mesenteric artery. Instead, blood can flow backwards into other veins and cause them. Sma syndrome is a rare condition where the third part of the duodenum is compressed between the sma and aa, leading to duodenal outlet obstruction. 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. Superior mesenteric artery syndrome genetic and rare. 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. Unusual manifestation of superior mesenteric artery. 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. Nutcracker syndrome, also called renal nutcracker syndrome is a rare vascular condition. Superior mesenteric artery syndrome project gutenberg. The nutcracker syndrome is an unusual problem where the left renal vein is narrowed as it passes between an angle formed between the abdominal aorta and the superior mesenteric artery. Multidetector ct of vascular compression syndromes in the. Superior mesenteric artery syndrome combined with renal nutcracker syndrome in a young male.
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. 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. A rare and potentially under diagnosed cause of haematuria. The term nutcracker phenomenon refers to the anatomic compression of the lrv that can occur between the sma and the. Superior mesenteric artery sma syndrome is a gastrovascular disorder in which the third and final portion of the duodenum is compressed between the abdominal aorta aa and the overlying superior mesenteric artery. Each type of compression is associated with specific clinical symptoms that constitute a rare disorder. A rare clinical entity that arises as a result of a hyperacute angle between the aorta and the sma is the nutcracker syndrome in which the left renal vein lrv is compressed between the abdominal aorta. Using a definition of sma angle angle was measured at 15.
Sma syndrome is triggered in supine position due to narrowed angle. There is a wide range of sma angle, lrv and dd in normal children, which correlated weakly with visceral fat volume. Superior mesenteric artery sma syndrome is a gastro vascular disorder in which the third and final portion of the duodenum is compressed between the abdominal aorta aa and the overlying superior mesenteric artery. Nutcracker syndrome the nutcracker syndrome ncsis a manifest variant of nutcracker phenomenon, renal vein entrapment syndrome, or mesoaortic compression of the left renal vein results most commonly from compression of left renal vein between the abdominal aortaaa and superior mesentric artery sma. Nutcracker syndrome ncs refers to a syndrome caused by significant compression of lrv between the abdominal aorta and sma, resulting in hypertension of the lrv and hematuria due to rupture of the. Nutcracker syndrome the nutcracker syndrome ncsis a manifest variant of nutcracker phenomenon, renal vein entrapment syndrome, or mesoaortic compression of the left renal vein results most.
She complained of left flank pain and was found to have haematuria. Sma transposition, together with division of fibrous bundle at the origin of the sma if present, is a safe and effective. Cooccurring superior mesenteric artery syndrome and. Nutcracker syndrome is caused by stenosis of the left renal vein lrv as it passes anteriorly to the aorta through the fork formed by both abdominal aorta aa and superior mesenteric artery sma. Normally, the angle between the sma and aa is approximately 450. Using a definition of sma angle 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. It occurs when arteries compress the left renal kidney vein, often causing intermittent hematuria blood in the urine. Nutcracker syndrome is characterized by a set of signs and symptoms secondary to compression of the left renal vein lrv either in the acute anatomic angle between the abdominal. Using a definition of sma angle nutcracker syndrome renal vein entrapment findings. Pain management specialists should include nutcracker syndrome in the differential when the symptoms indicate, and not assume atypical left flank pain is chronic myofascial or due to lower rib pathology.