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Segmental absence of intestinal musculature

Nariman A Nawar, Phyllis R Sawyer
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...
October 17, 2016: American Journal of Case Reports
Shirish S Nandedkar, Kamal Malukani, Ekta Patidar, Renu Nayak
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.
September 2015: International Journal of Applied and Basic Medical Research
Daniel Abensur Athanazio, Mateus T Amaral Rocha, Angela P Mattos, Tereza Cristina Medrado Ribeiro, Ney Cristian Boa Sorte, Luiz Antonio R Freitas, Luciana R Silva
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...
April 2015: Fetal and Pediatric Pathology
Shirish Nandedkar, Kamal Malukani, Prasann Bandi, Renu Nayak
No abstract text is available yet for this article.
November 2014: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
James S Davis, Mark L Ryan, John M Shields, Juan E Sola, Eduardo A Perez, Holly L Neville, Maria M Rodriguez
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...
August 2012: Journal of Pediatric Surgery
Omar Aldalati, Cordelia Phelan, Hassan Ibrahim
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...
2009: BMJ Case Reports
V Procházka, T Svoboda, O Soucek, Z Kala
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...
November 2010: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
Monika Dzieniecka, Adriana Grzelak-Krzymianowska, Andrzej Kulig
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.
2010: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
Chiara Oretti, Rossana Bussani, Augusta Janes, Sergio Demarini
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...
August 2010: Journal of Pediatric Surgery
A Chaloner, A Rao, E D Al-Chaer, B Greenwood-Van Meerveld
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...
February 2010: International Journal of Developmental Neuroscience
I Faruk Ozguner, Cagri Savas, Meltem Ozguner, Ozden Candir
BACKGROUND/PURPOSE: The aim of this study was to investigate the possible etiologic factors of small bowel atresia and to detect the prognostic role of adequate resection and tapering in postoperative morbidity and mortality. METHODS: Intestinal resection specimens were obtained from 10 patients with jejunoileal atresia and 3 control subjects without any gastrointestinal disease. Intestinal specimens taken from 2-cm and 4-cm proximal sides of atresia, atretic segment, and 1-cm and 2-cm distal sides of atresia were stained with Masson trichrome and H&E...
August 2005: Journal of Pediatric Surgery
Sushil Budhiraja, T S Jaiswal, Rajeev Sen
An unusual case of segmental defect of ileal musculature with terminal ileal atresia and ventricular septal defect is reported. Clinically the patient was diagnosed as ileal atresia. On exploration, there was type I atresia at ileocaecal junction with attendant ileal dilatation and an adjoining 5 cm x 4 cm segment resembling segmental dilatation of ileum, about 15 cm proximal to ileocaecal junction. Pathological examination of segmentally dilated ileum revealed focal absence of circular and longitudinal muscle coat without any evidence of necrosis or inflammation...
February 2004: Indian Journal of Pediatrics
Z Pavlovský, B Habanec, M Hermanová, I Penka, Z Kaplan
Herein we describe a case of 33-year old woman repeatedly affected by incomplete ileus. Primary segmental absence of lamina muscularis propria has been found in this patient. Histological examination revealed areas of small intestine with total absence of muscularis propria followed by areas of the intestinal wall characterised by normal histological structure. No necrosis, inflammation, and fibrosis supporting the secondary origin of this lesion was found.
April 2003: Ceskoslovenská Patologie
N Morikawa, S Namba, Y Fujii, Y Sato, K Fukuba
A case of intrauterine intestinal volvulus without malrotation is presented, and 9 other cases from the world literature are reviewed. Bloody diarrhea, bluish discoloration of the abdominal wall (Cullen's sign), and a tense abdomen in neonates suggest midgut volvulus requiring prompt surgical management. On pathological examination, the neck of the twisted bowel in this case showed a half-circle defect of the intestinal muscle. This suggests that a segmental defect of the intestinal musculature could be secondary to volvulus...
October 1999: Journal of Pediatric Surgery
T Fu, X Cui, X Wang, Z Fu
A full-term baby boy with a segmental defect of the ileal musculature associated with terminal ileal and biliary atresia is presented. The newborn had a dilated loop of the ileum 30 cm proximal to the ileal atretic site. Pathological study results showed absence of the intestinal musculature with relatively intact mucosa. Foci of recent muscular necrosis were found in the lesion. One month later, relaparotomy was performed because of persistent jaundice and hepatic duct atresia was confirmed. Segmental defect of the intestinal musculature associated with ileal atresia and biliary atresia has not been reported in the literature...
March 1998: Journal of Pediatric Surgery
O Tawfik, B Newell, K R Lee
No abstract text is available yet for this article.
February 1998: Digestive Diseases and Sciences
C Darcha, T Orliaguet, O Levrel, D Pezet, P Lointier, J Chipponi, P Dechelotte
We report a case of segmental defect of colonic muscularis propria revealed by perendoscopic perforation in a 64-year-old woman. Segmental absence of intestinal musculature is well documented in new-borns and infants and is more frequent in small bowel. It is characterized by localised absence of muscularis propria without fibrous scar. The remaining layers of the bowel wall are intact. The pathogenesis of this lesion is discussed.
March 1997: Annales de Pathologie
S F Huang, J Vacanti, H Kozakewich
Two cases of premature newborns with a segmental defect of the intestinal musculature are presented. The clinical diagnoses were intestinal obstruction and perforation, respectively. Emergency laparotomy was performed. In the first case, this showed a dilated loop of ileum and proximally dilated small bowel. The second case had ileal perforation with marked pneumoperitoneum. Pathological examination showed multifocal partial or complete absence of the muscularis propria, with relative preservation of the remaining components of the bowel wall in both cases...
May 1996: Journal of Pediatric Surgery
D Wurtzel, R F Nicosia, A Yoskovitch, A B Zubrow
We describe a rare case of a viable preterm infant who developed a spontaneous localized perforation of the small bowel caused by a segmental absence of the intestinal musculature and associated with vascular ectasia. The findings may support a causal relationship between absence of the intestinal muscle coat and a vascular network disorder which may be responsible for the gastroenteric defect.
January 1996: Journal of Maternal-fetal Medicine
D W McCarthy, S Qualman, G E Besner
Absent intestinal musculature is a rare entity of uncertain etiology. The diagnosis is confirmed histologically by segmental absence of the intestinal muscularis. The remaining layers of the bowel wall are completely intact, and notably absent are significant inflammation and hemorrhage. The authors report two cases of segmental absence of intestinal musculature presenting as perforations. There was gross or microscopic evidence of diverticula formation at sites of perforation and absent muscle. Based on the anatomic evidence in these specimens and a review of the surgical literature, we propose that the etiology is based on embryologic diverticuli...
November 1994: Journal of Pediatric Surgery
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