Megaduodenum Resulting From Absence of the Para-Sympathetic Ganglion Cells in Auerbachʼs Plexus

Megaduodenum with Duodenal Diospyrobezoars

“… 4 5 Idiopathic megaduodenum, also known as congenital megaduodenum, is defined as a huge dilatation confined to the duodenum without mechanical obstruction. 6 Zhang et al 7 reported data on four patients with idiopathic megaduodenum. All four patients were children under the age of 12 years and had undergone operation for relieving symptoms of abdominal distension, nausea, vomiting, and malnutrition.…”

Tapering duodenoplasty and gastrojejunostomy in the management of idiopathic megaduodenum in children

“…It was first described by Rokitansky in 1863 [1]. The first clinical report appeared in the literature by Melchior in 1924 [4].…”

“…Five surgical procedures have been documented in the literature and clinical practice: side-to-side gastrojejunostomy or duodenojejunostomy [1,7], side-to-side gastrojejunostomy plus tapering duodenoplasty, side-to-side gastrojejunostomy plus pyloric division with closure of the duodenal stump, tapering duodenoplasty plus Roux-en-Y duodenojejunostomy [15,16], and tapering duodenoplasty with pylorus division and closure of the duodenal stump plus Roux-en-Y or end-to-side gastrojejunostomy (Figs. 3B and 4).…”

“…It is defined as a massive dilatation confined to the duodenum without mechanical obstruction. It is also called congenital megaduodenum [1]. Patients with megaduodenum often present with recurrent abdominal distention, nausea, vomiting, diarrhea, malnutrition, and dystrophia [2].…”

Wilkie's syndrome may be due to poor motility

“…However, there are reports of motility disorders in patients, such as ours, who present with the classic features of Wilkie's syndrome. Barnett and Wall 3 found replacement of the myenteric plexus with a profusion of neural sheath cells in a resected megaduodenum; and, Eaves and Schmidt 4 reported smooth muscle degeneration around an apparently normal myenteric plexus in tissue from an index patient who had close relatives with megaduodenum who were asymptomatic. In 1966, Hearn 5 commented that motility disorders of the duodenum ‘must be rare, but might be shown, by more frequent biopsy of the distal duodenum at surgery, to be less uncommon than hitherto realized’.…”