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[biomedres]

Gastric Outlet Obstruction as an Aggravation of Peutz- Jeghers Syndrome: Case Report - BJSTR Journal

Gastric Outlet Obstruction as an Aggravation of Peutz- Jeghers Syndrome: Case Report by Marcelo Zamorano* in Biomedical Journal of Scientific & Technical Research

https://biomedres.us/fulltexts/BJSTR.MS.ID.006160.php

The Peutz-Jeghers Syndrome (PJS) is a rare autosomal dominant disease characterized by macules of mucocutaneous pigmentation and hamartomatous intestinal polyps. Some signs of the PJS appear in childhood, usually with abdominal pain. In some cases, patients can develop complications. In this article, we report a case of a patient with PJS that reached the emergency room after presenting a concomitant gastric outlet obstruction (GOO). The patient underwent through an endoscopic polypectomy, followed by a laparotomy and an intraoperative enteroscopy. The symptoms were controlled and the extraction of hamartomatous intestinal polyps were achieved, culminating with an intestinal segment resection with muscle atrophy and posterior gastrojejunostomy. There is evidence of PJS cases related to other conditions that developed abnormal scenarios such as biliary obstruction or cecal appendix polyps. However, the association between PJS and GOO and a concomitant delimited intestinal aperistalsis has not been reported. Thus, this case report shows the possible association between the PJS and GOO, whose cause is the intraluminal occupation of the duodenum by polyps and the synergy of the symptoms given by the atrophy of the jejunum muscle. 

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[mcatmemoranda]

The question asked where the degeneration of inhibitory neurons is located. I know Auerbach's plexus is between the circular and longitudinal smooth muscle layers. But I still got it wrong. :( The inhibitory neurons (Auerbach's plexus) are in the muscularis externa. Inhibitory neurons release VIP and NO to relax the LES. In pts who have achalasia, these neurons are missing, so they can't relax their LES.

This patient has achalasia, as confirmed by the narrowing of the gastroesophageal junction (“bird’s beak” appearance) seen on barium swallow test, i.e., the upper gastrointestinal series. This disease presents with difficulty swallowing, regurgitation of non-digested food, chest pain and weight loss. Manometry can be used to confirm the diagnosis and will show abnormal contractions of esophageal muscles, absence of peristalsis, and absence of relaxation of the lower esophageal sphincter (LES) upon swallowing. Failure of relaxation of the lower sphincter is due to a loss of function of the myenteric (Auerbach’s) plexus, which is composed of non-adrenergic, non-cholinergic, inhibitory ganglion cells. These cells are located between the longitudinal and circular layers of muscularis externa in the gastrointestinal tract and are responsible for coordinating motility of the entire alimentary canal. In patients with achalasia, there is a decrease in ganglion cell density in Auerbach's plexus resulting in aperistalsis of the esophagus and incomplete relaxation of the LES. The majority of cases are idiopathic, however, secondary causes include malignancies (most commonly adenocarcinoma of proximal stomach) and Chagas’ disease. There is no cure for achalasia. Management includes lifestyle modifications (excessive chewing of food, sleep with trunk elevated, avoid eating before bed), medical therapy (anti-muscarinics, sublingual nitroglycerin, long acting nitrates, calcium channel blockers), botulinum toxin injections, and surgical intervention (balloon dilatation, myometry).

Bottom Line: Intrinsic innervation is fundamental to coordinating gastrointestinal motor activity. Auerbach’s plexus, also called the myenteric plexus, is a neural network branching between longitudinal and circular muscle layers of the gastrointestinal tract and allows for coordinated movement along the tract. Loss of these neural cells results in achalasia, which commonly presents with chest pain, heart burn, dysphagia, and regurgitation.

#achalasia #Auerbach #Auerbachs plexus #myenteric plexus

[deltaconsulting]

Achalasia also known as esophageal achalasia, achalasia cardiae, cardiospasm, and esophageal aperistalsis is a rare disorder that makes it difficult for food and liquid to pass into the stomach.

[englischdeutschubersetzung]

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[englischdeutschubersetzung]

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[englischdeutschubersetzung]

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