Superior mesenteric artery syndrome and nutcracker syndrome

Abstract

A 15-year-old asthenic boy (38 kg, 160 cm, body mass index 14.8) presented with postprandial discomfort, nausea, and vomiting for 1 month. In addition, there were complaints of painless gross hematuria and left flank pain. Urinalysis revealed numerous isomorphic erythrocytes. Epigastralgia and distended abdomen were noted on physical examination. The boy had a history of anorexia and rapidly lost 17 kg during a 3-month period. Psychiatrists had made the diagnosis of depression and anorexia nervosa. An abdominal ultrasound revealed a reduced aortomesenteric angle and an engorged left renal vein (Supplementary A). Abdominal computed tomography later disclosed a hugely dilated stomach along with dilated duodenum with abrupt vertical cutoff at the level of the third part of duodenum caused by its compression between the aorta and the superior mesenteric artery (SMA) (Fig. 1), consistent with SMA syndrome. In addition, it also revealed entrapment of the left renal vein in the fork between the aorta and SMA (Fig. 2), which is compatible with nutcracker syndrome, and explained his non-glomerular hematuria and left flank pain (Supplementary B). His rapid weight loss led to depletion in mesenteric fat, thus resulting in a decreased aortomesenteric distance (2.8 mm), a sharp aortomesenteric angle (5°), and simultaneous development of nutcracker and SMA syndromes. Initial nasogastric decompression and antacid and motility agents were administered. Following conservative management wherein enteric and parenteral nutrition and psychotherapy were provided, the patient gained weight and experienced complete relief of symptoms, and the patient's follow-up urinalysis was normal.Figure 2Computed tomography revealed dilatation in the left renal vein (arrows); it was compressed by the aorta and the superior mesenteric artery (arrow head).View Large Image Figure ViewerDownload Hi-res image Download (PPT) The pathogenesis of nutcracker and SMA syndromes is a narrow aortomesenteric angle and a short distance between the SMA and aorta, usually resulting from the loss of mesenteric fat due to low body mass index or rapid weight loss. The abnormal anatomy leads to the upper compression of left renal vein and lower obstruction of duodenum.1Ananthan K. Onida S. Davies A.H. Nutcracker syndrome: an update on current diagnostic criteria and management guidelines.Eur J Vasc Endovasc Surg. 2017 Jun; 53: 886-894Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar, 2Shi Y. Shi G. Li Z. Chen Y. Tang S. Huang W. Superior mesenteric artery syndrome coexists with Nutcracker syndrome in a female: a case report.BMC Gastroenterol. 2019; 19: 15Crossref PubMed Scopus (5) Google Scholar, 3Merrett N.D. Wilson R.B. Cosman P. Biankin A.V. Superior mesenteric artery syndrome: diagnosis and treatment strategies.J Gastrointest Surg. 2009; 13: 287-292Crossref PubMed Scopus (177) Google Scholar Gross hematuria is attributed to the rupture of thin-wall varices into the collecting system because of elevated pressure on the left renal vein. Conservative treatment aimed at regaining weight is suggested for most patients. Surgical intervention should be considered only when symptoms are severe or persistent.1Ananthan K. Onida S. Davies A.H. Nutcracker syndrome: an update on current diagnostic criteria and management guidelines.Eur J Vasc Endovasc Surg. 2017 Jun; 53: 886-894Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar, 2Shi Y. Shi G. Li Z. Chen Y. Tang S. Huang W. Superior mesenteric artery syndrome coexists with Nutcracker syndrome in a female: a case report.BMC Gastroenterol. 2019; 19: 15Crossref PubMed Scopus (5) Google Scholar, 3Merrett N.D. Wilson R.B. Cosman P. Biankin A.V. Superior mesenteric artery syndrome: diagnosis and treatment strategies.J Gastrointest Surg. 2009; 13: 287-292Crossref PubMed Scopus (177) Google Scholar None. The following is the Supplementary data to this article: Download .docx (.62 MB) Help with docx files Multimedia component 1