Segmental absence of intestinal musculature

BACKGROUND AND AIMS: Perforation during esophageal endoscopic submucosal dissection (ESD) typically results from electrical damage. However, there are cases in which perforation occurs due to segmental absence of intestinal musculature (SAIM) without iatrogenic muscular injury. We investigated the occurrence rate and clinical course of SAIM during esophageal ESD. METHODS: We conducted a retrospective review of esophageal ESDs performed between 2013 and 2019 in 10 centers in Japan...

INTRODUCTION: Ehlers-Danlos syndromes (EDS) comprise a rare variety of genetic disorders, affecting all types of collagen. Herein, we describe a case of the vascular type of EDS, with coexisting segmental absence of intestinal musculature, while simultaneously performing a narrative review of the existing literature. CASE PRESENTATION: A 23-year-old male patient with a history of multiple abdominal operations due to recurrent bowel perforations and the presence of a high-output enterocutaneous fistula was admitted to our surgical department for further evaluation and treatment...

BACKGROUND: Segmental absence of intestinal musculature (SAIM) is a partial defect of the intrinsic muscular layer of the intestinal tract. In this report, we describe a case of perforation of the sigmoid colon due to SAIM accompanied by vascular Ehlers-Danlos syndrome (vEDS), which was successfully treated by surgical therapy. CASE PRESENTATION: A male in his 30 s was being followed up for vEDS diagnosed by genetic testing. He had undergone two major vascular surgeries, abdominal aortic artery revascularization and thoracic endovascular aortic repair for a residual dissection and enlarging abdominal aortic aneurysm...

INTRODUCTION AND IMPORTANCE: Segmental absence of the muscularis propria intestinalis (SAIM) is a rare pathology characterized by a partial or complete absence of the intestinal musculature. Diagnosis requires histological confirmation, and treatment includes urgent laparotomy, resection of affected areas, and end-to-end anastomosis or creation of stomas. The work has been reported in line with the SCARE guideline criteria. CASE REPORT: We present the case of a 31-week preterm newborn with prenatal diagnosis of polyhydramnios and non-immune fetal ascites...

BACKGROUND: Segmental Absence of Intestinal Musculature (SAIM) is a rare condition associated with intestinal obstruction and perforation. Colostomy fistula as a presentation of SAIM and their association with anastomotic failure have not been previously reported. This case report aimed to raise awareness of this unique manifestation and its potential implications. CASE PRESENTATION: A 58-year-old male with a history of type 2 diabetes, hypertension, and lumbar hernia presented with diarrhea...

No abstract text is available yet for this article.

Patients with vascular Ehlers-Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there is a lack of a muscularis propria layer in the intestinal wall. Although typically documented in the literature in neonates or adults, it can be seen in children of other ages. This is a case report of a patient who exhibits both rare entities, which has not been described in the literature to date...

Segmental absence of the intestinal musculature (SAIM) can cause intestinal perforation in adults. However, its prevalence and clinicopathologic features have not been well-described. This study aimed to determine the prevalence of SAIM-associated perforation and characterize its clinicopathologic features. We retrospectively examined 109 cases of intestinal perforation that underwent surgical resection from January 2009 to December 2019. SAIM was defined as the complete absence of the muscularis propria without extensive inflammation and fibrinous exudation around the perforation...

No abstract text is available yet for this article.

BACKGROUND: Segmental absence of intestinal musculature (SAIM) is a rare cause of intestinal obstruction and perforation due to partial or complete defects in the intestinal muscularis propria in neonates and is occasionally observed in adulthood. CASE PRESENTATION: The first case of small intestinal stenosis derived from SAIM, which was difficult to differentiate from Crohn's disease (CD), is reported. A 4-year-old girl presented with abdominal pain, anemia, and a positive fecal occult blood test...

BACKGROUND Segmental absence of intestinal musculature is a well described entity in premature infants. It presents with peritonitis, bowel perforation, and obstruction. The diagnosis is based on pathologic observation of absence of intestinal musculature. Researchers hypothesized that this entity is a result of a vascular accident during embryogenesis. However, segmental absence of intestinal musculature is no longer limited to the pediatric population. Recently, a few cases have been described in adults with and without significant vascular diseases...

Segmental absence of intestinal musculature (SAIM) is a rare cause of intestinal obstruction and/or perforation seen in neonates, and rarely in adults. We present a case of SAIM in a 48-year-old male, who presented with acute abdominal symptoms and was repeatedly explored because of recurrence of abdominal symptoms.

Segmental absence of the intestinal musculature is a rare cause of acute abdomen most likely associated with intestinal perforation and sepsis in neonates and adults. We present a case of a 10-year-old boy who developed acute abdomen and was treated with right hemicolectomy and partial resection of the ileum. The ileum showed a 20-cm-long stenotic segment showing prominent thinning of the intestinal wall. This case occurred in an older child, which appears to be rare when comparing the literature. The bowel defect was also the largest described to date, indicating the potential effect of an atonic segment leading to obstructive disease...

No abstract text is available yet for this article.

BACKGROUND: Segmental absence of the intestinal musculature (SAIM) is a known but clinically rare entity involving partial or complete absence of the intestinal muscularis propria. Clinical presentation is consistent with peritonitis, and treatment involves an emergent laparotomy, bowel resection, and reanastamosis or possible ostomy creation. Diagnosis results from histopathologic evaluation of the resected intestinal specimen. Most of the publications are case reports. METHODS: We retrospectively reviewed all surgical pathology files at a tertiary pediatric hospital from 2003 to 2010, identifying children who were noted to have absence of intestinal musculature on pathology...

Segmental absence of intestinal musculature (SAIM) is a rare entity of uncertain aetiology. A case of SAIM in an adult is presented, and three other adult cases of SAIM are reviewed. Our case concerns a middle-aged man who underwent a Whipple's procedure for a suspected neoplasm in the head of the pancreas. At surgery, a redundant segment of proximal jejunum with multiple large diverticula was incidentally noted and resected. On histological examination, the small bowel segment showed focal extreme thinning of the muscularis propria, focally amounting to complete absence...

Segmental absence of intestinal musculature (SAM) may, rarely, present with symptoms of ileus or intestinal perforation. Most commonly, the disorder presents with signs of intestinal disorders in newborns. Colonic perforations are then rare in adulthood. In this study, the authors present a case of a young female patient, operated for right abdominal pains when she was 30 weeks pregnant. Although no advanced appendicitis was confirmed, appendectomy was performed. Five days later, surgical revision was indicated for signs of septic condition and abdominal pain...

Congenital defect of the small intestine muscular layer is rare cause of spontaneous bowel perforation or obstruction in premature infants. Etiology is still unknown. The authors report one case of segmental absence of small bowel muscular layer in preterm born infant. Some ideas concerning the pathogenesis of this entity and review of the literature is presented.

Defect of the intestinal musculature is a rare condition. It may cause intestinal perforation or obstruction. It manifests itself mainly in the neonatal period and usually affects preterm infants. We describe one such case, which was first diagnosed as a spontaneous isolated intestinal perforation. Emergency laparotomy was performed and showed multiple perforations, with accompanying peritonitis and ascites. Pathologic examination showed partial or complete absence of the musculature, particularly of the inner circular layer, with fibrous tissue in the regions of missing muscle, and abnormal vasculature...

Previous studies have shown that early life trauma induced by maternal separation or colonic irritation leads to hypersensitivity to colorectal distension in adulthood. We tested the hypothesis that repetitive colorectal distension in neonates leads to abnormalities in colonic permeability and smooth muscle function in the adult rat. In neonatal rats, repetitive colorectal distension was performed on days 8, 10, and 12. As adults, stool consistency was graded from 0 (formed stool) to 3 (liquid stool). Colonic tissue was isolated for histology and myeloperoxidase levels...