Wilkie syndrome or superior mesenteric artery syndrome is a rare cause of duodenal obstruction in pediatric and adult age groups. Its manifestations are due to compression of the third portion of the duodenum between the aorta and the superior mesenteric artery. We present one case of a 29-year-old female with a recent history of epigastric pain and severe weight loss due to duodenal obstruction from superior mesenteric syndrome. She was treated conservatively with jejunostomy tube feeding to regain loss of weight. Our case is unique in that the patient has also nutcracker syndrome.
Key words: duodenal obstruction; loss of weight; megaduodenum; nutcracker syndrome; superior mesenteric artery syndrome; Wilkie syndrome
The differential diagnosis of duodenal obstruction is broad. One of its rare causes is Wilkie syndrome or superior mesenteric artery syndrome. Wilkie syndrome is arteriomesenteric obstruction of the duodenum that is characterized by compression of the third portion of the duodenum by the superior mesenteric artery as it passes over this portion of the duodenum. Vascular compression of the duodenum is a rare condition that presents with profound nausea and vomiting, abdominal distention, weight loss, and post-prandial epigastric pain. Epigastric pain varies from intermittent to constant depending on the severity of the duodenal obstruction. Weight loss often starts before the onset of other symptoms. This report describes a female adult who was admitted to our hospital with this diagnosis and was managed conservatively using jejunostomy tube feeding. The patient was found incidentally to have nutcracker syndrome due to left renal vein compression between the superior mesenteric artery and the aorta. The patient continued to have normal renal function and she was managed conservatively. The purpose of this case report is to discuss the diagnosis and conservative management of a patient with both Wilkie syndrome and nutcracker syndrome.
A 29-year-old female was transferred to our hospital for management of duodenal obstruction. Her past surgical history was notable for resection of a brain stem ganglioma, with residual dysarthria and general debility. Three years previously, she was treated for malnutrition and pneumonia following a laminectomy. The previous summer, she complained of early satiety and sharp post-prandial epigastric abdominal pain occurring half an hour after eating associated with anorexia and significant weight loss of 20–30 pounds. The abdominal pain was relieved with lying on the left side or on her back. Endoscopic gastroduodenoscopy (EGD) revealed erythematous gastropathy. Two months later, she was admitted with large bilious emesis and worsening of her epigastric pain. The patient denied any urinary symptoms. An abdominal computed tomography (CT) scan and an upper gastrointestinal Gastrografin study demonstrated compression of the third portion of the duodenum by the overlying superior mesenteric artery. It also revealed an opacified renal collecting system with contrast material but there was no obvious opacification of the ureters or bladder. EGD was repeated and confirmed a high-grade focal extrinsic obstruction of the mid-third portion of the duodenum that was not present 2 months previously. The patient was transferred to our hospital for further evaluation.
Physical examination revealed a cachectic female with a height of 170 cm and weight of 39.9 kg. The abdomen was soft and non-tender with normal bowel sounds, but a fluid wave was present suggesting ascites.
Laboratory studies with a reference range revealed mild anemia with hemoglobin of 11.1 g/dl (12.0–15.5 g/dl), normal leukocyte count of 4.9 × 109/l (3.5–10.5 × 109/l), normal serum urea nitrogen of 12 mg/dl (6–21 mg/dl), creatinine of 0.3 mg/dl (0.6–1.1 mg/dl), serum sodium of 146 mmol/l (135–145 mmol/l), serum potassium of 4.7 mmol/l (3.6–4.8 mmol/l), alanine aminotransferase of 20 U/l (8–43 U/l), alkaline phosphatase of 93 U/l (37– 98 U/l) and albumin of 3.3 g/dl (3.5–5.0 g/dl). Prealbumin and urine analysis were not checked. An abdominal CT confirmed extrinsic compression of the duodenum associated with an unusual inferior location of the superior mesenteric artery (SMA) (Figures 1 and 2). It also revealed diffuse ascites and bilateral pleural effusions. There was impingement of the left renal vein by the SMA with multiple venous collaterals indicative of nutcracker syndrome (Figure 3). An EGD was obtained and revealed bile-induced gastropathy but no overt duodenal obstruction at the site of the SMA.
In view of normal renal function with normal blood pressure, no further intervention was necessary for treating her nutcracker syndrome. The patient’s massive weight loss with loss of intraabdominal fat was felt to be a major contributing factor to the unusual vascular compression of the duodenum by the overriding SMA. The patient was treated conservatively through enteral nutrition support with a jejunostomy feeding tube in order to gain weight and this was effective in reducing her epigastric abdominal pain.
Duodenum compression by the SMA was first described in 1861 by Von Rokitansky. Since then, it has acquired various names such as Cast syndrome due to its increased incidence in patients requiring orthopedic casting and Wilkie syndrome in reference to a series of 75 cases reported by Dr Wilkie with chronic duodenal ileus.1–3 SMA syndrome is more common in females than males, with a predominance in the mid-30 to mid-40 age groups, but it can occur in older children and adolescents.4 Although rare, it should always be considered in the differential diagnoses of duodenal obstruction (Table 1). The entity of SMA syndrome has been controversial. It may be over-diagnosed in some patients due to a similarity with other causes of duodenal obstruction.5 Radiological imaging studies may also show discordant findings. Moreover, after surgical correction, some symptoms such as vomiting persist, while others like weight loss may improve.
Causes of SMA syndrome
SMA syndrome has been associated with postorthopedic spine surgeries (e.g. scoliosis).7 It is more common in thin individuals due to loss of the duodenal fat pad. Neurological injury, as in our patient, is a common risk factor. In one series of SMA syndrome in children, neurological injury such as paraplegia or cerebral palsy was present in 50% of patients.4 Various mechanisms are hypothesized associated with the anatomical relation of the duodenum to neighboring structures. The duodenum is suspended by its attachment to the ligament of Treitz as it crosses the angle formed between the aorta and SMA. Therefore, an unusual low origin of the SMA, compression of the duodenum due to peritoneal adhesions, or high insertion of the duodenum at the ligament of Treitz will lead to narrowing of the aorto-mesenteric angle leading to entrapment of the third portion of duodenum between the SMA and the aorta.8 The narrowing of the angle can be aggravated by depleted mesenteric fat from severe loss of weight.
Symptomatology varies between acute onset to a gradually progressive course of early satiety and post-prandial abdominal pain. Typical features include pain aggravated by a supine position and relieved by prone or left lateral decubitus, or even by a knee–chest position. Other symptoms include bilious vomiting with loss of appetite and weight, which can occur with advanced obstruction.9 These patients might present with manifestations of electrolyte imbalance or with gastrointestinal obstructive complications including massive gastric dilatation with gastric wall pneumatosis and portal venous gas.10 A physical examination is non-specific with findings including a thin body build, abdominal distension or high-pitched bowel sounds and signs of intestinal obstruction.
This is a difficult diagnosis to make based on clinical symptoms and signs which are mostly non-specific. Radiological imaging studies are needed to look for SMA syndrome or to confirm its presence. A plain abdominal radiograph may be an initial study to look for dilated gastric or proximal duodenal dilatation. A barium upper gastrointestinal series study or hypotonic duodenography might demonstrate the cessation of flow of barium from the duodenum to the jejunum.11 An EGD or CT scan of the abdomen could be helpful confirmatory studies.12 These modalities may reveal extrinsic compression of the third portion duodenal obstruction and narrow angle between the aorta and SMA. There is a case report about using endoscopic ultrasound for diagnosing SMA syndrome in children.1
In the majority of cases, conservative management to treat electrolyte imbalance and provide adequate nutrition using either enteral or parenteral feeding will result in weight gain and improved symptoms.14 In addition, proper positioning of the patient after eating, especially in the left lateral decubitus, prone, or even knee-to-chest position may be of benefit. Surgery can be considered when conservative measures fail. Surgical options include simple release of the ligament of Treitz, or through reconstructive approaches such as open or laparoscopic duodenojejunostomy or Roux-en-Y reconstruction.15,16 However, there is little long-term data to support the success of surgery to relieve symptoms, except in refractory emesis after the failure of all conservative measures.6,17 Another surgical approach is through infrarenal transposition of the SMA. This approach is done to avoid dumping syndrome through disruption of the physiologic intestinal continuity due to small-bowel bypass-surgical procedures.15
Description of nutcracker syndrome
Our patient presents with another rare compression syndrome of the left renal vein (LRV). Nutcracker syndrome is due to LRV compression between SMA and abdominal aorta. It was described first in 1937 by Grant18 with the first venography done by Chait, et al.19 The nutcracker syndrome leads to elevated renal venous pressure with formation of varices in the renal pelvis and ureter. Complications include consequent hematuria, proteinuria and left flank pain that were absent in our patient. Diagnosis is obtained after excluding other causes of hematuria. Imaging studies such as ultrasonography, magnetic resonance angiography, and computed tomographic angiography, as well as measuring pressure gradients between the inferior vena cava and LRV during venography are helpful in confirming this diagnosis. Surgical options may be performed in symptomatic patients. These procedures include nephropexy, transluminal balloon angioplasty with stenting,20 renal vein bypass, transposition of LRV, mesoaortic transposition, and renal autotransplantation.
Wilkie syndrome and nutcracker syndrome are rare compression syndromes. The diagnosis and treatment of Wilkie syndrome is controversial. The initial management should include gastric andduodenal decompression, treatment of fluid and electrolyte imbalance as well as reversal of weight loss using enteral and parenteral feeding. Surgery may be appropriate in some patients who continue to have debilitating symptoms after a good trial of conservative therapy.
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