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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 50  |  Issue : 4  |  Page : 150-152

Segmental dilatation of the ileum


Division of Pediatric Surgery, Department of Surgery, Chi-Mei Medical Center, Tainan, Taiwan

Date of Submission23-Oct-2016
Date of Decision11-Jan-2017
Date of Acceptance19-Jan-2017
Date of Web Publication19-Jul-2017

Correspondence Address:
Tune-Yie Shih
901, Tzong-Huang Road, YongKang, Tainan 71004
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_47_17

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  Abstract 

A 2-year-old boy was sent to the emergency department with the chief problem of abdominal pain for 1 day. He was just discharged from the pediatric ward with the diagnosis of mycoplasmal pneumonia and paralytic ileus. After initial examinations and radiographic investigations, midgut volvulus was impressed. An emergency laparotomy was performed. Segmental dilatation of the ileum with volvulus was found. The operative procedure was resection of the dilated ileal segment with anastomosis. The postoperative recovery was uneventful. The unique abnormality of gastrointestinal tract – segmental dilatation of the ileum, is described in details and the literature is reviewed.

Keywords: Midgut volvulus, paralytic ileus, segmental dilatation of the ileum


How to cite this article:
Shih TY. Segmental dilatation of the ileum. Formos J Surg 2017;50:150-2

How to cite this URL:
Shih TY. Segmental dilatation of the ileum. Formos J Surg [serial online] 2017 [cited 2019 Jun 18];50:150-2. Available from: http://www.e-fjs.org/text.asp?2017/50/4/150/211086




  Introduction Top


Segmental dilatation of the intestine is a rare congenital disorder in children as a cause of intestinal obstruction. This disease was first reported by Swenson and Rathauser in 1959 in a clinical situation mimicking Hirschsprung's disease.[1],[2],[3],[4],[5],[6] Thereafter, about 60 cases had been reported in the literature.[1] Most of the cases did not have the definite diagnosis before operation. Although this disease entity was well established, its pathogenesis and etiology are unknown. We report a case of segmental dilatation of ileum with a satisfactory surgical result.


  Case Report Top


A 2-year-2-month-old boy, without major congenital abnormality, was sent to the emergency service with the chief problem of abdominal pain for 1 day. He was just discharged from the pediatric ward 2 days ago, after a 5-day's hospitalization for paralytic ileus and mycoplasmal pneumonia [Figure 1]a showing unremarkable small bowel gas pattern and prominent colon gas in plain abdominal film]. Tracing the past history, the patient was delivered through normal spontaneous delivery with gestational age 39 weeks and birth weight 2900 g. He was noted to have intermittent bloody stool after birth and was diagnosed and treated as allergy to milk. The physical examination revealed a thin boy (body length 89 cm and body weight 11.8 Kg) with acute-ill looking, having diffuse abdominal tenderness without peritoneal sign. The bowel sounds were hypoactive. Digital rectal examination revealed fresh blood. The blood tests showed white count 6600/mm 3, Hb 13.9 g/dL, platelet count 522,000/mm 3, and C-reactive protein <0.2 mg/L. The stool routine showed no occult blood, parasite ovum, or rotavirus antigen. The plain abdominal film revealed a large stool-filling intestinal segment in the right side of the abdomen [Figure 1]b, which was interpreted as stool retention in colon. Since the pain looked severe and was not alleviated by enema, an abdominal computed tomography (CT) was done, which showed inverted superior mesenteric vein/superior mesenteric artery relationship [Figure 2]a with whirl sign and dilated bowel loop in the right side of the abdomen [Figure 2]b, suggesting midgut volvulus. Thus, an emergency operation was performed. In the operation, we found a well-demarcated dilated ileal segment, filled with fecal material, 15 cm in length, 6 cm in diameter, 40 cm proximal the ileocecal valve, with markedly enlarged mesenteric lymph nodes [Figure 3]. The ileal segment was anchored by adhesion in the right side of the abdominal cavity, producing a 360° unstrangulated clockwise twist of a small intestinal loop about 1 m in length. Otherwise, malrotation of midgut with mobile ascending colon in epigastric area was also found. After reduction of the intestinal twist, we resected the dilated ileal segment and made an end-to-end anastomosis. The resected specimen revealed no obstructive lesion at its proximal and distal ends and unremarkable intestinal mucosa without an ulcer on its surface. The postoperative recovery was smooth. The pathology revealed eroded mucosa with focal hemorrhage and congestion, no ectopic gastric mucosa, and there were normal ganglion cells in bowel wall. During a 2-year's follow-up, the patient was free of abdominal pain or bloody stool.
Figure 1: (a) Plain abdominal film in antecedent admission. (b) Plain abdominal film in this admission

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Figure 2: (a) Inverted superior mesenteric vein/superior mesenteric artery relationship (arrow). (b) Whirl sign (arrow) and dilated bowel loop in the right side of the abdomen

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Figure 3: A well-demarcated dilated ileal segment with enlarged mesenteric lymph nodes

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  Discussion Top


Segmental dilatation of the ileum is an unique and rare cause of intestinal obstruction in children although it may be found in adolescents and adults. This disease was also called idiopathic localized dilatation of the ileum, ileal dysgenesis, segmental megaileum, and giant Meckel's diverticulum.[1] This form of idiopathic dilatation of intestine may involve ileum, colon, jejunum, and duodenum (in sequence of case numbers), and most cases had single lesion with the dilated segment around 10–20 cm in length.[2],[3],[4],[5],[6] However, involvement of a very long segment or multifocal dilatations have been reported.[7],[8],[9]

The characteristic features of segmental dilatation of the ileum are localized aperistaltic dilatation of a well-defined segment of the ileum with abrupt transition to a normal bowel proximally and distally, without mechanical blockage or deficient neuromuscular architecture.[1],[2],[3] Ectopic gastric mucosa has been reported present in the lining of the dilated ileal segment and was postulated to be the cause of intestinal bleeding,[3],[4] although in most cases, a gross ulceration was not found.

The symptoms caused by the dilated ileal segment are related to functional obstruction, volvulus, and blood loss. In the neonatal period, the symptoms and signs of acute obstruction are more common.[6] In elder infant, children, and adults, the obstruction tends to be intermittent,[3],[4] and chronic anemia can be caused by long-term gross or occult intestinal bleeding.[5]

Segmental dilatation of the ileum is frequently associated with omphalocele, malrotation of midgut, and Meckel's diverticulum,[1],[2],[3],[4],[5] but the etiology of this disorder remains unknown. A number of hypotheses have been proposed, including primitive neuromuscular dysfunction, extrinsic compression, intrauterine strangulation, and disturbance during the division of the notocord from the endoderm.[1],[2],[3],[4],[5],[6]

The diagnosis of segmental dilatation of the ileum is essentially made by radiographic findings or at laparotomy. Patients present vomiting, abdominal pain, abdominal distension, bloody stool, or chronic anemia, which call for medical attention. An abdominal mass may be palpated. In several cases the antenatal ultrasound demonstrated cystic or hypoechoic lesions in the fetal abdomen, but the definite diagnosis was made only after postnatal contrast study of gastrointestinal tract or during laparotomy.[10] In plain radiography, segmental dilatation of the ileum typically presents as a solitary well-demarcated dilated bowel loop with loss of valvulae conniventes and no significant air–fluid level. In addition, there is no evidence of complete obstruction distal to the dilated bowel segment,[1] except in association with volvulus. Small bowel series and CT scan demonstrate a sharply demarcated dilated bowel loop with pooling of contrast medium.[1],[2],[3],[4],[5],[6] However, in most cases, because of rarity of this disease entity, a definite diagnosis was not made until laparotomy, with the preoperative diagnosis of duplication, ileus, intestinal atresia, Hirschsprung's disease, etc. Several more common entities mimic the radiographic appearance of segmental dilatation of the ileum, including aneurysmal dilatation in lymphoma, cavitating neoplasms, sacculation in scleroderma, postoperative change, intestinal duplications, dilatation secondary to obstruction, and scarring in inflammatory disease.[4] Thus, a careful history taking, physical examination, and detailed analysis of the image findings help make the correct diagnosis.

Segmental dilation of the ileum is cured by resection of the dilated segment with anastomosis.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10] If it is an incidental finding at laparotomy, a complete resection was recommended.[3] In cases of involvement of a significant length or multiple segments, a bowel-preserving surgery (plication, tapering, tubularization) had been suggested.[8],[9]

This 2-year-2-month-old boy presented intermittent intestinal bleeding as the initial manifestation of segmental dilatation of the ileum. An episode of ileus, subsiding spontaneously, 1 week anteceded to the attack of volvulus. The definite diagnosis was made at laparotomy, and segmental resection of the dilated ileum gave a satisfactory surgical result.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lee JE, Lee SW, Hwang JY. Segmental dilatation of the ileum: A report of two cases. Acta Radiol 2006;47:525-8.  Back to cited text no. 1
    
2.
Basaran UN, Sayin C, Oner N, Celtik C. Segmental intestinal dilatation associated with omphalocele. Pediatr Int 2005;47:227-9.  Back to cited text no. 2
    
3.
Kobayashi T, Uchida N, Shiojima M, Sasamoto H, Shimura T, Takahasi A, et al. Segmental dilatation of the ileum covered almost entirely by gastric mucosa: Report of a case. Surg Today 2007;37:1102-4.  Back to cited text no. 3
    
4.
Shah AD, Kovanlikaya A, Beneck D, Spigland N, Brill PW. Segmental dilatation of the ileum in a healthy adolescent. Pediatr Radiol 2009;39:1350-3.  Back to cited text no. 4
    
5.
Sjolin S, Thoren L. Segmental dilatation of the small intestine. Arch Dis Child 1962;37:422-4.  Back to cited text no. 5
    
6.
Rathod KJ, Mohd Z, Kanojia R, Rao KL. Segmental ileal dilatation: An unsuspected cause of neonatal intestinal obstruction. Trop Gastroenterol 2012;33:143-6.  Back to cited text no. 6
    
7.
Kaiser M, Castellani C, Singer G, Marterer R, Ratschek M, Till H. Huge congenital segmental dilatation of the sigmoid colon in a neonate: A “rarity to meet” and a “challenge to treat”. Case Rep Pediatr 2016;2016:9685307.  Back to cited text no. 7
    
8.
Ragavan M, Arunkumar S, Balaji N. Segmental dilatation of near total colon managed by colon preserving surgery. APSP J Case Rep 2012;3:18.  Back to cited text no. 8
    
9.
Mirza B, Bux N. Multiple congenital segmental dilatations of colon: A case report. J Neonatal Surg 2012;1:40.  Back to cited text no. 9
    
10.
Mboyo A, Aubert D, Massicot R, Destuynder O, Lassauge F, Lorin A. Antenatal finding of intestinal obstruction caused by isolated segmental jejunal dilatation: A case report. J Pediatr Surg 1996;31:1454-6.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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