Wilkie's syndrome may be due to poor motility

Abstract

Fig. 1. The arrow points to a giant ganglion within the muscularis mucosa. Within the insert, the arrowhead points to a single ganglion cell within the submucosa (haematoxylin and eosin stain).

Superior Mesenteric Artery Syndrome: Diagnosis and Treatment Strategies

“…24 Certainly, patients with motility disorders such as intestinal neuronal dysplasia type B and familial megaduodenum may have similar clinical features of SMA syndrome. However, there is little evidence, either pathological or physiological, to support this postulate.…”

Recalling Superior Mesenteric Artery Syndrome

“…Conventional barium studies still play an important role for diagnosis and the classic but unspecific picture is that of a dilated proximal duodenum with an abrupt termination of the barium column in the third portion. This radiographic appearance might also be seen in cases of megaduodenum (intestinal pseudo-obstruction) [10]. The following strict radiographic criteria have been established for diagnosis of the superior mesenteric artery syndrome: (i) dilatation of the first and second parts of the duodenum, with or without gastric dilatation, (ii) abrupt vertical and oblique compression of the mucosal folds, (iii) antiperistaltic flow of contrast medium proximal to the obstruction, (iv) delay in transit of 4–6 h through the gastroduodenal region, and (v) relief of obstruction in a prone, knee-chest or left lateral decubitus position [1, 5].…”

“…In rare cases, the syndrome may be acute with rapid evolving upper intestinal ileus [34, 55]. Whereas patients that underwent scoliosis surgery often show symptoms within the first postoperative week [27, 42, 43] and burn patients after 2 weeks [31], other patients suffer from non-specific symptoms for months [10, 56] to years [1, 7, 8, 17]. …”

“…The complex of clinical signs reflecting duodenal obstruction includes postprandial epigastric pain, nausea, vomiting, anorexia and weight loss and has been termed aortomesenteric artery compression [1], arteriomesenteric duodenal compression [2], duodenal vascular compression [3], superior mesenteric artery [4,5,6], Wilkie’s [7, 8] or cast syndrome [9]. In contrast, megaduodenum characterizes a dilatation of the duodenum without primary or secondary duodenal stenosis which has been ascribed to intestinal myopathy and which can be familial [10,11,12]. In fact, the existence of the superior mesenteric artery syndrome has been controversial because of confusion with other causes of megaduodenum [13].…”

Superior mesenteric artery syndrome presented with postprandial bilious vomiting successfully treated with open duodenojejunostomy in a resource-poor setting

“…Conventional barium studies still play an important role in diagnosis and the classic but unspeci c picture is that of a dilated proximal duodenum with an abrupt termination of the barium column in the third portion. This radiographic appearance might also be seen in cases of megaduodenum (intestinal pseudo-obstruction) (11). The following strict radiographic criteria have been established for the diagnosis of superior mesenteric artery syndrome: (I) dilatation of the rst and second parts of the duodenum, with or without gastric dilatation, (II) abrupt vertical and oblique compression of the mucosal folds, (III) antiperistaltic ow of contrast medium proximal to the obstruction, (IV) delay in transit of 4-6 h through the gastroduodenal region, and (V) relief of obstruction in a prone, knee-chest or left lateral decubitus position (10).…”