Segmental absence of intestinal musculature with metachronous bowel perforations in an infant

Oyachi, Noboru; Suzuki, Takeyuki; Emura, Takaki; Obana, Kazuko; Nemoto, Atsushi; Naito, Akira; Oyama, Toshio

doi:10.1016/j.epsc.2017.10.016

Cited by 5 publications

(5 citation statements)

References 11 publications

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“…2 3 4 5 In some cases, a segmental absence of intestinal musculature may cause spontaneous bowel perforation, intestinal obstruction, or intussusception. 6 The resection of the affected segment usually leads to complete recovery, but the clinical polymorphism and the lack of specificity of diagnostic imaging may point to the difficulty to have a complete preoperative picture of the disease. 7 8…”

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confidence: 99%

Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria

Sergi

Hager

2019

AJP Rep

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Background and Aim Congenital segmental intestinal dilatation (CSID) is a neonatal condition with unclear etiology and pathogenesis. Typically, the newborn with CSID presents with a limited (circumscribed) bowel dilatation, an abrupt transition between normal and dilated segments, neither intrinsic nor extrinsic perilesional obstruction, and no aganglionosis or neuronal intestinal dysplasia. We aimed to review this disease and the long-term follow-up at the Children's Hospital of the Medical University of Innsbruck, Tyrol, Austria. Study Design Retrospective 25-year review of medical charts, electronic files, and histopathology of neonates with CSID. Results We identified four infants (three girls and one boy) with CSID. The affected areas included duodenum, ileum, ascending colon, and sigmoid colon. Noteworthy, all patients presented with a cardiovascular defect, of which two required multiple cardiac surgical interventions. Three out of the four patients recovered completely. To date, the three infants are alive. Conclusion This is the first report of patients with CSID and cardiovascular defects. The clinical and surgical intervention for CSID also requires a thorough cardiologic evaluation in these patients. CSID remains an enigmatic entity pointing to the need for joint forces in identifying common loci for genetic investigations.

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confidence: 99%

Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria

Sergi

Hager

2019

AJP Rep

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“…SAIM in adults was first reported by Emanuel et al 9 in 1967 as a cause of neonatal intestinal obstruction, and SAIM in newborns is a well-characterized entity related to acute abdomen. [9][10][11][12][13][14][15] In contrast, the number of adult SAIM cases that have been reported thus far is limited, with only 16 available in the English literature (summarized in Table 5 and Fig. 5).…”

Section: Discussionmentioning

confidence: 99%

“…Segmental absence of the intestinal musculature (SAIM), also known as segmental muscular defect of the intestine, was first reported as a cause of neonatal intestinal obstruction by Emanuel et al9 in 1967. Subsequently, SAIM has become a distinctive disease entity when dealing with an acute abdomen in newborns 10–15…”

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confidence: 99%

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Prevalence and Clinicopathologic Features of Intestinal Perforation Caused by Segmental Absence of the Intestinal Musculature in Adults

Tsuyuki

Satou

Takahara

et al. 2021

American Journal of Surgical Pathology

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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. SAIM was the second most frequent cause of perforation (26 cases: 24%), the most frequent cause being related to diverticulitis (39 cases: 36%). The most common site was the sigmoid colon (12 cases: 46.2%). The younger group (aged below 65 y) exhibited more frequent perforation of the upper segments of the gastrointestinal tract (from the duodenum to the descending colon) than the older group (65 y and above) (P=0.0018). No patients developed recurrence. The most common gross features were well-defined circular or small punched-out lesions, and the histologic features were complete absence of the muscularis propria and absence of hemorrhage and necrosis around the area of perforation. The characteristic features of SAIM were unique and their prevalence was higher than previously reported. The precise recognition of SAIM can aid in understanding the cause of perforation and avoiding further unnecessary examinations.

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“…During laparotomy, the affected segment is thin and transparent - it may present as perforation or segmental dilation [ 28 ]. Prenatal ultrasound could also help to suspect disease in the neonatal period - in three cases, including ours, polyhydramnios was observed in prenatal ultrasound scans [ 7 , 29 ] and in one case they visualized transitory dilation of the intestine before birth [ 30 ] however, these findings are not specific to SAIM.…”

Section: Introductionmentioning

confidence: 99%

Segmental absence of intestinal musculature in newborn: Case report

Vargova,

Abecia Martinez,

Escartín Villacampa

et al. 2023

International Journal of Surgery Case Reports

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Segmental absence of intestinal musculature with metachronous bowel perforations in an infant

Cited by 5 publications

References 11 publications

Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria

Congenital Segmental Intestinal Dilatation: A 25-Year Review with Long-Term Follow-up at the Medical University of Innsbruck, Austria

Prevalence and Clinicopathologic Features of Intestinal Perforation Caused by Segmental Absence of the Intestinal Musculature in Adults

Segmental absence of intestinal musculature in newborn: Case report

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