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CASE REPORT
Year : 2017  |  Volume : 44  |  Issue : 3  |  Page : 156-157

Segmental absence of intestinal musculature: A rare cause of intestinal obstruction in infants


Department of Pathology, JN Medical College, KLE University, Belgaum, Karnataka, India

Date of Web Publication14-Feb-2018

Correspondence Address:
Hema Basappa Bannur
Department of Pathology, JN Medical College, KLE University, Nehru Nagar, Belgaum - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jss.JSS_31_17

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  Abstract 

Segmental absence of intestinal musculature (SAIM) is a rare entity of unknown cause. Presenting features vary from intestinal obstruction, necrotizing enterocolitis, volvulus, and intussusception to spontaneous intestinal perforations. In majority of the cases, SAIM is a histological diagnosis. Here, we present a case of a 1-month female baby who presented with fever, abdominal distension, and vomiting. Resected segment of intestine histologically showed focal segmental absence of the muscularis propria. To the best of our knowledge, fewer than 50 cases of SAIM have been reported in the literature so far.

Keywords: SAIM, intestinal obstruction, necrotizing enterocolitis


How to cite this article:
Bannur HB, Suranagi VV, Davanageri R. Segmental absence of intestinal musculature: A rare cause of intestinal obstruction in infants. J Sci Soc 2017;44:156-7

How to cite this URL:
Bannur HB, Suranagi VV, Davanageri R. Segmental absence of intestinal musculature: A rare cause of intestinal obstruction in infants. J Sci Soc [serial online] 2017 [cited 2018 May 23];44:156-7. Available from: http://www.jscisociety.com/text.asp?2017/44/3/156/225504


  Introduction Top


Segmental absence of intestinal musculature (SAIM), a rare disease of uncertain pathogenesis, can be congenital or acquired. The age of presentation may vary from neonates to adulthood. It may affect the stomach, small intestine, or colon. Often, it manifests as intestinal obstruction or perforation.[1] SAIM is rarely diagnosed clinically, usually, it is a histological diagnosis where there is intact mucosa, submucosa, and serosa but segmental absence of the muscularis propria. In newborns, it resembles necrotizing enterocolitis (NEC), but it is distinguished from NEC by the absence of inflammation, hemorrhage, and necrosis.[2] Treatment is resection of the involved intestinal segment.


  Case Report Top


A 1-month female baby born to primi mother by lower segment cesarean section presented with fever, abdominal distension, and vomiting of 4 days duration. A clinical diagnosis of acute intestinal obstruction secondary to NEC was made. Peroperative adhesions were noted. A segment of small intestine was resected. We received small intestinal segment measuring 55 cm in length. Both the surgical ends appeared normal with central thinned out area. Microscopically, the thinned out are showed normal mucosa, submucosa, and serosa, but the absence of muscularis propria [Figure 1] and [Figure 2]. The rest of the intestine was mildly inflamed. Hence, the diagnosis of SAIM was given.
Figure 1: Small intestine showing absence of muscularis propria (H and E, ×100)

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Figure 2: Small intestine showing absence of muscularis propria (H and E, ×200)

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  Discussion Top


The first case of SAIM was described by Herbut in 1943.[3],[4] Emanuel et al. in 1967 described SAIM as a new entity causing intestinal obstruction.[5] Majority of the cases reported are in neonates, but it can present rarely in adults also. It is classified as primary idiopathic and secondary acquired. Acquired SAIM follows vasopressor use, nonsteroidal anti-inflammatory intake, or intestinal ischemia caused by hypoxia of prematurity.[6] The exact etiology and pathogenesis area not clear. Many attribute it to focal intrauterine ischemia of the gastrointestinal tract.[7] However, this theory cannot explain the intact mucosa, which is more vulnerable to ischemia. Huang et al. and Morikawa et al. have suggested that SAIM may be secondary to ischemic event, but because of the differences in the regenerative capacity of the mucosa and the muscle, the mucosa regenerates faster.[8],[9] Resection of the affected segment is curative.

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Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Alawadhi A, Chou S, Carpenter B. Segmental agenesis of intestinal muscularis: A case report. J Pediatr Surg 1989;24:1089-90.  Back to cited text no. 1
    
2.
Ahmed H, Al-Salem AH, Qureshi SS. Neonatal intestinal perforation secondary to congenital segmental agenesis of intestinal muscularis. Saudi Med J 1997;18:605-7.  Back to cited text no. 2
    
3.
Herbut PA. Congenital defect in a musculature of the stomach with rupture. Arch Pathol 1943;36:91-4.  Back to cited text no. 3
    
4.
Pereira S, Tortosa F, Gonclaves M. Segmental absence of intestinal musculature concerning a child with acute appendicitis. J Pediatr Surg 2015;3:207-10.  Back to cited text no. 4
    
5.
Emanuel B, Gault J, Sanson J. Neonatal intestinal obstruction due to absence of intestinal musculature: A new entity. J Pediatr Surg 1967;2:332-5.  Back to cited text no. 5
    
6.
Davis JS, Ryan ML, Shields JM, Sola JE, Perez EA, Neville HL, et al. Segmental absence of intestinal musculature: An increasingly reported pathology. J Pediatr Surg 2012;47:1566-71.  Back to cited text no. 6
    
7.
Stephens D, Arensman R, Pillai S, Alagiozian-Angelova V. Congenital absence of intestinal smooth muscle: A case report and review of the literature. J Pediatr Surg 2009;44:2211-5.  Back to cited text no. 7
    
8.
Huang SF, Vacanti J, Kuzakewich H. Segmental defect of the intestinal musculature of a new born: Evidence of acquired pathogenesis. J Pediatr Surg 1996;31:721-5.  Back to cited text no. 8
    
9.
Morikawa N, Namba S, Fujii Y, Sato Y, Fukuba K. Intrauterine volvulus without malrotation associated with segmental absence of small intestinal musculature. J Pediatr Surg 1999;34:1549-51.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

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