Superior Mesenteric Artery Angle Research Articles

The superior mesenteric artery angle in diagnosis of nutcracker syndrome: a systematic review and meta-analysis.

Nutcracker syndrome (NCS) can be caused by narrowness of the superior mesenteric artery (SMA) angle. Nevertheless, the cut-off value of the SMA angle is controversial and variable. Therefore, the present study evaluated the optimal SMA angle to maximize diagnostic performance for NCS diagnosis by conducting a meta-analysis. We comprehensively searched the English literature related to the diagnosis of NCS from the perspective of SMA (from the date of database inception to June 2022). The accuracy of an SMA angle less than 41° in the diagnosis of NCS was evaluated by calculating the pooled sensitivity (SEN), pooled specificity (SPE), positive likelihood ratio (LR+), negative likelihood ratio (LR-), summary receiver operating characteristic (SROC) curve and area under the curve (AUC) value. The I2 test and meta-regression analysis were used to assess heterogeneity and sources of heterogeneity, respectively. Publication bias was assessed using Deeks' funnel plot asymmetry test. Six studies (526 patients) met the inclusion criteria. SEN and SPE were 0.94 (95% confidence interval (CI) 0.80-0.99) and 0.85 (95% CI 0.65-0.94), respectively. The LR + value was 6.0, and the LR- value was 0.07, revealing that SMA angles less than 41° exhibited an excellent ability to help confirm or exclude NCS. Additionally, SROC curves showed that the AUC of SMA angles less than 41° for the diagnosis of NCS was 0.96, indicating that SMA angles less than 41° have good efficacy for helping to diagnose NCS. This study explored the diagnostic efficacy of the cut-off value of the SMA angle by meta-analysis. According to the high SPE and SEN results, SMA angles less than 41° have good efficacy in facilitating NCS diagnosis.

Isolated superior mesenteric artery dissection with concomitant vascular aberrancy: Extremely rare cases and clinical implications.

Vascular aberrancy of superior mesenteric artery (SMA) may contribute to the occurrence of SMA dissection. However, there is no direct evidence to support this hypothesis. Etiology, natural history, classification, and treatment options of ISMAD are still in controversial at some degree. We also review the current understanding of ISMAD based on our results. Out of 57 patients, 2 cases of isolated superior mesenteric artery dissection (ISMAD) which concomitant with replaced common hepatic artery with SMA origin, are first reported. Two patients have no any typical etiological factors, such as atherosclerosis, hypertension, long-term smoking, and connective tissue disease. The contrast-enhanced computed tomography and (or) angiography showed concomitant SMA aberrancy. They have 81.2°, 132.7° SMA angle, respectively. After conservative treatment of 4, 6days, respectively, these 2 patients were discharged smoothly. Vascular aberrancy may be a new identified risk factor for ISMAD. Even in ISMAD cases with vascular aberrancy, conservative treatment still can be used as first line therapy.

Exploring celiac trunk parameters in median arcuate ligament syndrome: A CT study.

To assess anatomical variations in the celiac trunk (Ct) in patients with Median Arcuate Ligament Syndrome (MALS) using computed tomography (CT). The primary objectives were to investigate the celiac trunk angle (CtA), origin level, length (CtL), and their relationships with the superior mesenteric artery (SMA) in MALS patients. Additionally, the study intended to evaluate gender differences in these parameters and explore correlations between variables. Retrospectively, reports of abdominal CT scans taken between January 2018, and Sepmtember 2021, in the hospital image archive were screened vey two observers independently for MALS diagnosis. Parameters such as CtA, CtL, Ct-SMA distance, SMA angle (SMAA), and median arcuate ligament thickness (MALT) were measured. Statistical analyses were conducted using SPSS software. Among the 81 patients (25 females, 56 males), significant differences were observed in MALT between genders (p = 0.001). CtA showed a negative correlation with CtL and Ct-SMA (p < 0.001), and a positive correlation was found between CtL and Ct-SMA (p = 0.002). CtL was measured as 25mm for the all group. Origin levels of Ct and SMA were evaluated in comparison to vertebral levels. Ct-SMA distance was relatively shorter (9.19mm) compared to the literature. SMAA findings were consistent with normal population values. This study provided valuable insights into the anatomical parameters of the Ct ans SMA in MALS patients. Despite some differences compared to normal population parameters, no evidence supported the hypothesis of a superiorly placed Ct contributing to MALS.

Patient-Reported Outcomes for Robot-Assisted Laparoscopic Extravascular Renal Vein Stent Placements for Nutcracker Syndrome.

Introduction: Nutcracker phenomenon is the compression of the left renal vein between the superior mesenteric artery (SMA) and the abdominal aorta. Nutcracker syndrome refers to the presence of nutcracker phenomenon with symptoms. Between 2016 and 2022, we performed 18 robot-assisted laparoscopic extravascular renal vein stent (RALERVS) placements. We sought to assess patient-reported outcomes of RALERVS placement by a single institution. Methods: We performed a single-center retrospective review of 18 patients with a minimum of 3 months follow-up. Symptoms were assessed utilizing a questionnaire conducted with a 5-point Likert scale at a minimum of 3 months postoperatively. Five on the Likert scale was severe while 1 was none. Primary study outcomes compared pre- and postoperative patient-reported symptom scores. Results: Twelve out of 18 patients responded to the survey. The average length of time from date of operation to completion of survey was 2.6 years. Average age of the cohort was 36 years with a mean BMI of 19.4 kg/m2. There was only one man. Mean operative time was 137 minutes and mean estimated blood loss was 12 mL. Mean preoperative SMA angle was 19° and mean postoperative SMA angle was 36°. Patients reported that flank pain, abdominal pain, nausea, headaches, back pain, pelvic pain, and early satiety improved (p < 0.05). Ten of 12 respondents would recommend RALERVS for nutcracker syndrome. Conclusion: RALERVS demonstrates an effective treatment for nutcracker syndrome. Patients reported improved symptoms at 3 months postoperatively across multiple domains. Further studies need to be conducted to assess long-term durability of the extravascular renal vein graft.

Effect of left renal vein compression stenosis on functional status of left kidney in patients with pelvic venous disorders

ObjectiveWe evaluated the effect of left renal vein (LRV) compression stenosis on the functional state of the left kidney in patients with pelvic venous disorders (PeVDs). MethodsWe examined 162 female patients with PeVD and diagnosed LVR compression stenosis using duplex ultrasound (DUS) in 40. Patients with clinical manifestations of PeVD (n = 26) had symptoms and signs of pelvic venous congestion but without pain in the left flank of the abdomen, typical for nutcracker syndrome (NS). The remaining patients were asymptomatic (n = 14). The parameters measured with DUS included the angle of the superior mesenteric artery (SMA) with the aorta, the LRV diameter (Dhilum/Dstenosis) ratio, and LRV velocity (Vstenosis/Vhilum) ratio. All the patients underwent laboratory testing (complete blood count, urinalysis, and biochemical blood testing) and dynamic renal scintigraphy to assess the secretory and evacuation functions of the renal tubular system. ResultsThe laboratory tests revealed no abnormalities, including no hematuria or proteinuria, in either group. The Dhilum/Dstenosis and Vstenosis/Vhilum ratios varied from 2.8 to 5.2 and from 2.9 to 8.3, respectively, and did not differ between the symptomatic and asymptomatic patients. All 40 patients with LRV compression stenosis were diagnosed with left gonadal vein reflux with a mean duration of 4.7 ± 0.6 seconds and 2.2 ± 0.6 seconds in the symptomatic and asymptomatic patients, respectively (P = .005). Eight patients had signs of NS on DUS, including five in the symptomatic group (SMA angle, 34.8° ± 2.7°; Dhilum/Dstenosis ratio, 5.2 ± 0.2; and Vstenosis/Vhilum ratio, 5.7 ± 0.4) and three in the asymptomatic group (SMA angle, 35° ± 2.8°; Dhilum/Dstenosis ratio, 5; and Vstenosis/Vhilum ratio, 5 ± 0.5). The groups did not differ significantly in the DUS parameters. Scintigraphy did not reveal any cases of secretory or evacuation dysfunction of the left kidney, including in the patients with DUS signs of NS. The maximum uptake time, elimination half-life, and effective renal plasma flow were within the normal ranges. ConclusionsLRV compression stenosis without hematuria has no significant effects on the functional state of the left kidney, irrespective of the disease severity. In patients with PeVDs, dynamic renal scintigraphy provides an objective assessment of left kidney function.

Use of tubularized bovine pericardium in left renal vein transposition for nutcracker syndrome

Nutcracker syndrome is an extrinsic compression of the left renal vein by the superior mesenteric artery anteriorly and aorta posteriorly, resulting in hallmark manifestations of hematuria, proteinuria, and flank and/or pelvic pain. This report illustrates the case of a patient with a history of left flank pain and intermittent gross hematuria every 2 weeks. The patient denies any pelvic pain or gastrointestinal or lower extremity symptoms. Urinalysis revealed red blood cells, but no infection was noted. The cystoscopy findings were normal. Computed tomography urography showed left renal vein and duodenal compression between the aorta and superior mesenteric artery with a narrow aortic–superior mesenteric artery angle. The patient underwent left renal vein transposition to the distal inferior vena cava via a transabdominal approach. The left renal vein was transected at the inferior vena cava; however, the length was too short to create a tension-free anastomosis. Thus, a bovine pericardium sheet was tubularized and used as an interposition graft. The patient recovered well and is symptom-free. Left renal vein transposition is a well-described surgical technique in the treatment of nutcracker syndrome. The use of a vein or prosthetic graft has been described. This case demonstrates the use of an alternate conduit for reconstruction.

PD31-07 PATIENT REPORTED OUTCOMES AFTER ROBOTIC ASSISTED LAPAROSCOPIC EXTRAVASCULAR RENAL VEIN STENT PLACEMENT FOR NUTCRACKER SYNDROME

You have accessJournal of UrologyCME1 Apr 2023PD31-07 PATIENT REPORTED OUTCOMES AFTER ROBOTIC ASSISTED LAPAROSCOPIC EXTRAVASCULAR RENAL VEIN STENT PLACEMENT FOR NUTCRACKER SYNDROME Jacques Farhi, Hal Kominsky, Mohannad Awad, and Jeffrey Cadeddu Jacques FarhiJacques Farhi More articles by this author , Hal KominskyHal Kominsky More articles by this author , Mohannad AwadMohannad Awad More articles by this author , and Jeffrey CadedduJeffrey Cadeddu More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003324.07AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Nutcracker phenomenon is the compression of the left renal vein between the superior mesenteric artery (SMA) and the abdominal aorta. Nutcracker syndrome refers to the presence of nutcracker phenomenon with symptoms including hematuria, intermittent left flank pain, early satiety, nausea, vomiting, pelvic congestion syndrome, and dyspareunia. Treatment of nutcracker syndrome includes weight gain, vascular transposition, renal autotransplant, nephrectomy, endovascular renal vein stent placement, or extravascular renal vein stent placement. Between 2016 and 2022, we performed 18 robotic assisted laparoscopic extravascular renal vein stent placement. We sought to assess patient reported outcomes of robotic assisted laparoscopic extravascular renal vein stent placement by a high-volume robotic surgeon at a single institution. METHODS: We performed a single-center retrospective review of 18 patients with a minimum 3 month follow up. Symptoms were assessed utilizing a questionnaire conducted with a five-point Likert scale at 3 months. Five on the Likert scale was severe while a one was none. Primary study outcomes compared pre-operative patient reported symptom scores versus post-operative patient reported symptom scores. RESULTS: 12 out of 18 patients responded to the survey. Average age of the cohort was 36. Average BMI was 19.4. There was only one male. Mean operative time was 136 minutes and mean estimated blood loss was 12. Mean pre-operative SMA angle was 19 and mean post operative SMA angle was 36. Patients reported that flank pain, abdominal pain, nausea, headaches, back pain, pelvic pain, and early satiety improved (p<0.05) (Figure 1). 8 respondents would strongly recommend the surgery for nutcracker syndrome. CONCLUSIONS: Robotic assisted laparoscopic extravascular renal vein stent placement demonstrates an effective treatment for nutcracker syndrome. Patients reported improved symptoms at 3 months post operatively across multiple domains. Further studies need to be conducted to assess long-term durability of robotic assisted laparoscopic extravascular renal vein stent placement. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e903 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Jacques Farhi More articles by this author Hal Kominsky More articles by this author Mohannad Awad More articles by this author Jeffrey Cadeddu More articles by this author Expand All Advertisement PDF downloadLoading ...

Volumetric Analysis of Progressive Remodeling of Isolated Mesenteric Artery Dissection Treated by Conservative Therapy

PurposeTo characterize remodeling of conservatively treated isolated mesenteric artery dissection (IMAD) using 3-dimensional (3D) volumetric analysis. Material and MethodsPatients with Type I/II (classification of Yun) treated by conservative therapy between January 2018 and January 2020 were prospectively included. Semiautomatic morphological analysis of the superior mesenteric artery (SMA) included volumetric measurements of the true lumen (TL), false lumen (FL), and overall lumen (OL) and 3D aortomesenteric angles from computed tomography angiography data at admission (T0), 1 month (T1), and 12 months (T12). The SMA morphology of patients with IMAD (n = 15, mean age 53 years ± 7; 87% men) was also compared with that of control individuals (n = 51, mean age 56 years ± 4; 94% men). ResultsA significant reduction in OL volume was observed (P <.001), whereas TL volume remained stable (P =.23). The TL/OL volume ratio significantly increased over time (P =.001) from 53% at T1 to 78% at T12. Aortomesenteric 3D angles at 2, 4, and 6 cm from the ostium showed a progressive decrease toward values observed in the control group (P =.013, P =.002, and P =.027, respectively). At T12, 5 patients (33%) had complete remodeling, and aneurysmal change was observed in 2 patients (<20 mm). Smoking and SMA angle at a distance of 6 cm from the ostium (T0) were the only factors affecting remodeling negatively at T12. ConclusionsOne-year remodeling in IMAD followed an overall decrease in OL volume related to a decrease in FL volume. Smokers and patients with larger SMA angles at baseline showed poorer remodeling. Spontaneous arterial remodeling in IMAD might favor conservative therapy.

The Superior Mesenteric Artery Angle and Abdominal Main Vessel Diameters in Normovolemic Children: For Practical Sonographic Evaluation.

Knowledge of the normal size of important abdominal vessels is important for determination of hydration status and intravascular volume. Knowing the normal value of superior mesenteric artery (SMA) exit angle from the aorta (SMA angle) is necessary for the diagnosis of clinical pathologies caused by SMA compression. The aim of this study was to determine the normal SMA angles and normal diameters of the main abdominal vascular structures of pediatric participants on ultrasonography (USG). Between January 2019 and July March 2019, the USG examinations obtained from 210 participants (age range, 1-214 years; mean age, 83.5 [SD, 5.5] months; females 51%, males 49%) with normal abdominal examinations were prospectively evaluated. The participants were divided into 5 groups according to age. Body mass index values of the participants were kept equal. The diameters of the abdominal vascular structures increased with age, and the statistical differences among the age groups were significant. In all groups, no statistically significant difference was observed between sexes (P > 0.05). The SMA angle was between 17° and 58° (mean, 37.8° [SD, 8.5°]). A statistically significant difference was observed between the age groups in terms of SMA angle (P < 0.05). No statistically difference was found between sexes (P > 0.58-0.912). In our study, we think that the measurements obtained by USG provide normal values of the diameters of the abdominal main vessels and SMA angle. These values can be used as a reference for interpreting hydration status, vascular diameter assessment, and SMA compression syndromes.

The utility of magnetic resonance angiography in children with nutcracker syndrome

Background/aim The presented study aimed to evaluate the utility of magnetic resonance angiography (MRA) in the pediatric population with nutcracker syndrome.Materials and methods Patients with suggestive clinical symptoms and laboratory findings and got the diagnosis of nutcracker syndrome with Doppler ultrasonography between January 2011–2019 were included in the study. In addition, children who had renal MRA due to hypertension were evaluated as the control group. MRA images of all patients were examined retrospectively by three radiologists at different levels of experience, and the superior mesenteric artery angle, aorta-mesenteric distance, left renal vein diameter both in the regions of aorta-mesenteric, and renal hilum were recorded. ResultsForty-five patients diagnosed with nutcracker syndrome were included in the study. The mean age of patients was 12 (4–16) and 30 (67%) were female. As the control group, 25 patients with hypertension who had MRA were included and they had a mean age of 12 (1–18) and 19 (76%) were male. The mean superior mesenteric artery angle was 26.5 ° (16–73 ± 12) in the patient group and 57.8 ° (25–139, ± 33) in the control group (p < 0.001); the mean aorta-mesenteric distance was 3.3 mm (1.7–6.5, ± 1.1) in the patient group and 8 mm (3.4–32, ± 5.9) in the control group (p < 0.001). MRA measurements of three radiologists were consistent with each other.ConclusionMRA imaging can be applied as an alternative diagnostic method for Doppler ultrasonography and multidetector CT examinations by radiologists with different experience levels in pediatric patients with nutcracker syndrome.

Value of magnetic resonance imaging indices of left renal vein entrapment in the diagnosis of nutcracker syndrome in children.

BackgroundNutcracker syndrome (NCS) is also known as left renal vein (LRV) compression syndrome. Magnetic resonance imaging (MRI) can better depict the anatomical structure of soft tissues in the area of compression. Diagnosis of NCS using MRI in adults is not uncommon. However, there are few studies on the diagnosis of NCS using MRI in children. Therefore, we conducted this study to evaluate the clinical value of the MRI indices of the LRV in the diagnosis of NCS in children.MethodsThis study was a single-center retrospective analysis. One hundred seventy-four patients with suspected NCS were enrolled from January 2017 to July 2020. The inclusion criteria for suspected NCS were mainly based on clinical symptoms or signs, laboratory examinations and imaging reports. Other diseases that may cause hematuria and/or proteinuria were excluded. We grouped the patients based on the diagnostic criteria for NCS into a nutcracker group and a control group. The receiver operating characteristic (ROC) curve was constructed for evaluating the value of MRI indices in the diagnosis of NCS.ResultsThe majority of NCS patients presented with orthostatic proteinuria (OP) (67.2%), followed by hematuria (55.2%), abdominal pain (19.0%), and left flank pain (15.5%). The areas under the curve (AUCs) for the superior mesenteric artery (SMA) angle, beak sign, and compression ratio (CR) in the diagnosis of NCS were 0.870, 0.895, and 0.878, respectively, and the best cutoff values of the SMA angle and CR were 36.8 and 3.99, respectively. The specificities of SMA angle <36.8°, beak sign, CR >3.99, SMA angle <36.8° and beak sign, SMA angle <36.8° and CR >3.99, and beak sign and CR >3.99 were 82.5%, 93.8%, 93.8%, 97.9%, 95.9% and 97.9%, respectively.ConclusionsChildren with SMA angles less than 36.8°, beak signs and CR greater than 3.99 should be highly suspected of having NCS. Among these parameters, “beak sign” showed the highest diagnostic accuracy by MRI, and the combination of any two of the above parameters achieved a higher specificity than the single parameters.

Measurement of the Angle Between the Superior Mesenteric Artery and the Aorta: Correlation with Body Mass Index

Objective: To discover the mean figures of the distance and angle between the superior mesenteric artery (SMA) and the aorta in four standard acknowledged body mass index (BMI) classifications in an ordinary populace and for the two genders, to work with a computed tomography CT based finding of Superior mesenteric artery syndrome (SMAS). Methods: A retrospective cross-sectional study was performed on 338 (157 female, 181 male) patients who were referred for abdominal CT examination. Age, sex, weight and height of all patients were noted, and the BMI was calculated. The distance between the SMA and aorta were measured, as was the angle. Pearson and Spearman correlation coefficients were used to correlate distance and BMI category, angle and BMI category, anteroposterior abdominal distance and SMA angle with aortomesenteric distance. Results: The mean BMI of patients in our study was 27.95 kg/m2 while the mean SMA angle, mean anteroposterior abdominal wall distance and mean aortomesenteric distance was 58.74 degrees, 224.35 cm, and 18.98 cm, respectively. The results showed positive correlation of BMI and anteroposterior abdominal distance with SMA angle and distance. This means if BMI increases, SMA angle and aortomesenteric distance will also increase, and vice versa. This same relationship was also valid for anteroposterior abdominal distance with aortomesenteric angle and distance. Conclusion: Our study helps in stating the values of aortomesenteric distance and angle for different BMI categories that can be used in CT-based diagnosis of SMAS in a South Asian population. The values we found for different BMI categories can be used by radiologists as a reference standard.

Isolated superior mesenteric artery dissection: CTA features and clinical relevance.

To determine the detailed computed tomography angiography (CTA) features of isolated superior mesenteric artery dissection (ISMAD), aiming to improve the current understanding of ISMAD. We retrospectively analyzed 57 patients with confirmed diagnoses of ISMAD with CTA from June 2011 to June 2018 in our hospital. The superior mesenteric artery (SMA) angle, entry site of dissection, and features of both false and true lumen were analyzed individually. In general, middle-aged men with atherosclerosis (42.1%), hypertension (35.3%), or long-term smoking (33.3%) were the main populations. 80.7% of patients can be discharged successfully after conservative treatment. 87% of patients had a right (or almost right) SMA angle, and the entry site of dissection in 90% of patients occurred in the curved segment of the SMA. As a polymorphic disease, we can use the coded CTA features to delineate individual cases, including cases beyond the presenting classification systems. The diameter of the true lumen is the only different CTA feature between the conservative treatment group and the interventional treatment group (p = 0.000). The re-entry site (p = 0.501), thrombosis in the false lumen (p = 0.135), the distance between the entry site of dissection and SMA root (p = 0.133), and SMA branch involvement (p = 0.322) had no difference between the 2 groups. Based on CTA analysis, the bending area, especially in the SMA with right or nearly right SMA angle, is the most vulnerable anatomical region for the occurrence of ISMAD. The diameter of true lumen is the determined CTA feature for the decision of treatment. CTA should be recommended as a standardized diagnostic and classification tool for ISMAD.

Clinico-radiological correlation of nutcracker syndrome: a single centre experience.

The term nutcracker syndrome (NS) refers to the compression of left renal vein between the aorta and the superior mesenteric artery (SMA) causing renal venous hypertension. The symptoms vary from asymptomatic hematuria to severe pelvic congestion. The purpose of this study is to evaluate the clinical characteristics of patients with NS and the correlation between clinical and laboratory findings with Doppler sonographic measurements. Sixty-three patients were evaluated (19 boys and 44 girls) since January 2004 to January 2014. The mean age of the patients was 12.21 ± 3.21 years (range 7-17). Hematuria and proteinuria disappeared during the follow up of 9/63 patients. These nine patients showed statistically significant difference in upright SMA angles (p= 0.035). Doppler sonographic evaluation is a non-invasive method for diagnosis NS. Upright SMA angle measurement is more efficient than supine SMA angle measurement on clinicoradiological correlation.

Wilkie syndrome. Case report

Abstract Background Wilkie syndrome, also referred as superior mesenteric artery syndrome, is an unusual cause of a proximal small bowel obstruction. It is characterised by the compression of the duodenum in its third portion due to a narrowing of the space between the superior mesenteric artery and the aorta. Its presentation symptoms are consistent and include the obstruction of the proximal small bowel. However, the physical and laboratory findings are non-specific. Nevertheless, many imaging methods are useful for its diagnosis. The management of this condition varies between observation and surgery, depending on each particular case. Clinical case The case is presented of a 19-year-old male who began with acute, intense abdominal pain, nausea, vomiting, and diarrhoea. On examination, he had abdominal wall rigidity and hyperesthesia. Imaging studies were requested, revealing a decreased superior mesenteric artery angle, a shortening of the aortic mesenteric distance, and a decrease in the calibre of the third duodenal portion, all findings concomitant with Wilkie syndrome. Conservative treatment was applied and the patient was discharged without complications. Conclusions Wilkie syndrome continues to be an unknown condition to the general practitioner, and the underdiagnosis of this condition may put a patient at risk of serious complications. A high index of suspicion is required to reach a diagnosis. Early treatment should give a good outcome most of the time.

Candida-Induced Emphysematous Gastritis and Wilkieʼs Syndrome

Destruction of protective gastric mucosa can predispose to Emphysematous gastritis (EG), a rare and lethal entity. Co-occurrence of candida induced EG with gastric perforation and superior mesenteric artery (SMA) syndrome is extremely rare. A 21-year-old African American man with poorly controlled type 1 diabetes mellitus presented with diffuse abdominal pain, nausea, and vomiting. He was cachectic, afebrile, with RR 45/min, pulse 130/min, BP of 80/45 mmHg, with dry mucous membranes, and diffuse abdominal tenderness. Laboratory data showed blood glucose 55 mg/dL, Cr 2 mg/dL, WBC of 19,100/microliter, lactate 5.2 mmol/L, with normal lipase level. He was started on 5% dextrose-normal saline with bicarbonate and meropenem. CT abdomen showed markedly distended stomach with gas bubbles in gastric wall and fluid filled loops of proximal small bowel. Nasogastric suction revealed dark colored aspirate. General surgery recommended explorative laparotomy for possible small bowel obstruction. Laparotomy showed 60% gastric necrosis with proximally dilated and distal compressed duodenum with a narrow SMA angle. This suggested EG with Wilkie's (SMA) syndrome. Partial gastrectomy, and Roux-en-Y procedure with a duodenojejunostomy were done. Gastrectomy specimen showed mural necrosis with abundant fungi consistent with Candida. While hepatectomy specimen showed focal nodular hyperplasia. Blood cultures grew Candida albicans. The patient was treated with micafungin and sepsis resolved. EG is characterized by gas within stomach wall due to invasion by microbes like Streptococci, Escherichia coli, Enterobacter, Clostridium welchii, and Candida. Predisposing factors are alkali/acid ingestion, alcohol abuse, immunosuppression, prior abdominal surgery and diabetes (as in our case). EG presents as acute abdomen with fever, chills, hematemesis and decreased bowel sounds. Laboratory findings include lactic acidosis and leukocytosis. Diagnosis is suggested by CT abdomen showing gas bubbles in the stomach wall that are unaffected by positional change. Complications include gastric infarction, gastric perforation (as seen in our case) with high mortality. Prompt management requires fluid resuscitation; antibiotic therapy to cover gram negative bacteria and anaerobes with de-escalation based on nasogastric aspirate, blood, and stomach wall specimen cultures. Deterioration despite optimal medical management and gastric perforation are the indications for emergent surgery.

Nutcracker and SMA syndromes: What is the normal SMA angle in children?

ObjectiveThe 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. We measured the SMA angle to define the normal range in children. MethodsWe retrospectively measured SMA angles, left renal vein (LRV) distance, and duodenal distance (DD) in 205 consecutive pediatric abdominal CT. Total and visceral intra-abdominal fat at the level of the umbilicus were also assessed. ResultsMean SMA angle was 45.6±19.6° (range 10.6–112.9°), mean LRV distance was 8.6±3.9mm (range 2.0–28.6mm) and mean DD was 11.3±4.8mm (range 3.6–35.3mm). There was a significant but weak correlation between %visceral fat volume (%VF) and SMA angle (R=0.30; p<0.001), LRV distance (R=0.37, p<0.001) and DD (R=0.32; p<0.001). ConclusionThere is a wide range of SMA angle, LRV and DD in normal children, which correlated weakly with visceral fat volume. Using a definition of SMA angle <25° would diagnose 9.3% of asymptomatic children with nutcracker syndrome, and using a DD definition of <8mm would diagnose 20% with SMA compression. Our findings suggest exercising caution when attributing these rare syndromes to an absolute SMA angle.

Wilkie syndrome: report of a case

Wilkie syndrome is an unusual form of high gastrointestinal obstruction resulting from compression of the duodenum between the abdominal aorta and the superior mesenteric artery (SMA). The conditions that cause this syndrome can be classified into five categories: severe wasting diseases, severe injuries, diseases, deformity or trauma to the spine, dietary disorders and posoperative state. The symptoms include nausea, vomiting, distention postprandrial, epigastric pain and weight loss. Barium meal and arteriography were used as diagnostic tools, now CT-angiography is being used and shown higher diagnostic sensitivity. The diagnostic criteria are: dilated duodenum, compression of the duodenum by the SMA and aortomesenteric angle <20 degrees. Patients with acute syndrome often respond to conservative treatment (decompression, correction of dehydration and electrolyte imbalance and nutrition support). Most of the patients with chronic syndrome require surgical intervention. Duodenojejunostomy is the most effective surgical option, with a success rate of 90%.

Síndrome de Wilkie: a propósito de un caso

Wilkie syndrome is an unusual form of high gastroin- testinal obstruction resulting from compression of the duodenum between the abdominal aorta and the superior mesenteric artery (SMA). The conditions that cause this syndrome can be classified into five categories: severe wasting diseases, severe injuries, diseases, deformity or trauma to the spine, dietary disorders and posoperative state. The symptoms include nausea, vomiting, distention postprandrial, epigastric pain and weight loss. Barium meal and arteriography were used as diagnostic tools, now CT-angiography is being used and shown higher diagnostic sensitivity. The diagnostic criteria are: dilated duodenum, compression of the duodenum by the SMA and aortomesenteric angle <20 degrees. Patients with acute syndrome often respond to conservative treatment (decompression, correction of dehydration and elec- trolyte imbalance and nutrition support). Most of the patients with chronic syndrome require surgical inter- vention. Duodenojejunostomy is the most effective surgi- cal option, with a success rate of 90%. (Nutr Hosp. 2011;26:646-649) DOI:10.3305/nh.2011.26.3.5215

Could Superior Mesenteric Artery Angle Measurement Predict the Course of Renal Symptoms in Children With Nutcracker Syndrome?

Could Superior Mesenteric Artery Angle Measurement Predict the Course of Renal Symptoms in Children With Nutcracker Syndrome?