Nutcracker Syndrome combined with Superior Mesenteric Artery Syndrome in a Pediatric Patient: A Case Report

Abstract

Nutcracker syndrome is a phenomenon that the left renal vein (LRV) is pressed between the superior mesenteric artery (SMA) and the aorta. Clinical characteristics include gross or microscopic hematuria, orthostatic proteinuria, abdominal pain, and back pain. It occurs due to LRV squeezing caused by narrowed aortomesenteric angle. SMA syndrome is a disease that the third part of the duodenum is prone to intestinal obstruction by narrowed angle between the SMA and the abdominal aorta. Clinical symptoms include postprandial abdominal distension, epigastric pain, nausea, and vomiting. SMA syndrome and nutcracker syndrome have common features that result from narrowed aortomesenteric angle. However, it is very rare for both syndromes to occur simultaneously, so the two syndromes are regarded as separate diseases. This is a report on a case of nutcracker syndrome with SMA syndrome in a child who presented gross hematuria, recurrent abdominal pain and vomiting. To our knowledge, nutcracker syndrome simultaneous with SMA syndrome has not been previously reported in pediatric patient, especially with an exhibition of gross hematuria. This case suggests that the simultaneous presence of SMA syndrome with the same pathogenesis needs to be considered when nutcracker syndrome is suspected in pediatric patients with hematuria.

Superior mesenteric artery syndrome mimicking gastric outlet obstruction: a case report and a literature review

“…Patients may experience relief when lying prone, where gravity contributes to duodenal compression 4 . The symptoms are relieved when the patient is in the knee-to-chest position because it reduces the small-bowel mesenteric tension at the aortomesenteric angle 10 . A Hayes maneuver in which pressure is applied below the umbilicus in cephalad and dorsal directions elevates the root of the small-bowel mesentery and may also relieve obstruction temporarily 3 .…”

Superior Mesenteric Artery Syndrome and Nutcracker Syndrome as the Presentation of Crohn’s Disease in a Young Patient: A Case Report and Review of Literature

“…Wong et al (14) described the use of esophagogastroduodenoscopy as part of the evaluation in a pediatric patient with an uncertain etiology of SMA until a final diagnosis of CD was made. Conservative treatment with bowel decompression, hydration, and nutritional support are the mainstays of management for patients with upper gastrointestinal obstruction caused by SMAS and NCS (2,(5)(6)(7)(8)(9). Feeding management focuses on safe weight gain while monitoring for refeeding syndrome.…”

“…Proteinuria, observed in NCS, often requires treatment with an ACE inhibitor (9). Most pediatric patients with SMAS and NCS have complete resolution of symptoms with medical management alone (2,(5)(6)(7)(8)11,16). If medical management fails, surgery may then be considered (2,9).…”

“…SMAS has been described in patients with Crohn's disease (CD) (14,15), and both SMAS and NCS were described in pediatric patients (5)(6)(7)(8)16). However, none of the reports included patients with both syndromes as the initial presentation of CD.…”

Superior mesenteric artery syndrome: A review of the literature

“…10 The literature discusses cases of weight loss from anorexia nervosa being associated with SMA syndrome and even the likelihood of delayed diagnosis of these entities in males because of a common gender bias of eating disorders. 11,12 In pediatrics, the reported co-occurrence of SMA syndrome and Nutcracker syndrome is relatively rare, [13][14][15] but it is not unexpected that they would occur together. Both conditions are due to compression of structures at similar anatomical levels within the abdomen.…”