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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 3  |  Issue : 1  |  Page : 26-29

Rare presentation of congenital gut malrotation as adult cecal volvulus


1 Department of General Surgery, GMC, Rajouri, Jammu and Kashmir, India
2 Department of Anesthesiology, GMC, Rajouri, Jammu and Kashmir, India

Date of Submission15-Jul-2020
Date of Decision12-Aug-2020
Date of Acceptance18-Aug-2020
Date of Web Publication02-Oct-2020

Correspondence Address:
Dr. Mudassir Ahmad Khan
Department of General Surgery, GMC, Rajouri, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJCS.IJCS_22_20

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  Abstract 

Incomplete or faulty rotation and fixation of the gut during 5th–11th week of the fetal life results in intestinal malrotation. Cecal volvulus is a rare (1%–1.5%) cause of intestinal obstructions in adults. However, it accounts up to 25%–40% of all volvuluses involving the colon. Cecal volvulus due to malrotation presenting as an intestinal obstruction in an adult is very rare, and only few cases are reported in the literature. We present a case report of a 33-year-old adult male who presented to the emergency with a 2-day history of pain abdomen and vomiting with no significant past history. Per abdomen examination revealed a tender lump in the right paraumblical region. The diagnosis of intestinal obstruction was made on clinical and X-ray features. Emergency laparotomy was done. However, to our surprise, we found that the patient is having malrotated gut with cecal volvulus with the features of intestinal obstruction. The cecal volvulus was carefully de-torted, and right hemicolectomy with hand-sewn end-to-side ileotransverse anastomosis was done. Postoperatively, the patient behaved well and was discharged on the 8th postoperative day.

Keywords: Cecal volvulus, cecopexy, gut malrotation, intestinal obstruction, right hemicolectomy


How to cite this article:
Khan ZA, Khan MA, Shah GA. Rare presentation of congenital gut malrotation as adult cecal volvulus. Indian J Colo-Rectal Surg 2020;3:26-9

How to cite this URL:
Khan ZA, Khan MA, Shah GA. Rare presentation of congenital gut malrotation as adult cecal volvulus. Indian J Colo-Rectal Surg [serial online] 2020 [cited 2020 Dec 3];3:26-9. Available from: https://www.ijcrsonweb.org/text.asp?2020/3/1/26/297099


  Introduction Top


The normal human embryological process during the development of the small and large gut involves the steps of physiologic herniation of the primitive digestive tube, followed by the return of midgut loop into the peritoneal cavity from the umbilical herniation with simultaneous 180° counterclockwise rotation around the superior mesenteric pedicle. And finally, the midgut gets fixed along with the continuation of counterclockwise rotation, normally up to 270°. Incomplete or faulty rotation and fixation of the gut during 5th–11th week of the fetal life results in intestinal malrotation. One of the modes of presentation of intestinal malrotation in early childhood is the cecal volvulus. Cecal volvulus is a rare (1%–1.5%) cause of intestinal obstructions in adults. However, it accounts up to 25%–40% of all volvuluses involving the colon.[1] Usually, the underlying causes of cecal volvulus are idiopathic or congenital bands. The frequent precipitating factors are the conditions that impair emptying of the colon due to any chronic or sub-acute mechanical obstruction, postoperative paralytic ileus, pregnancy with cecal dislocation, abdominal surgery requiring mobilization of the right colon, and high residual diet.[2] Cecal volvulus due to malrotation presenting as an intestinal obstruction in an adult is very rare, and only few cases are reported in the literature. Cecal volvulus is of two types. The common type is axial rotation of the cecum and the ileum around the mesentery and the less common form is cecal bascule, where the cecum folds upon itself.[3] Treatment of cecal volvulus depends upon the clinical condition of the patient at the time of presentation and intraoperative findings.


  Case Report Top


This is a case report of a 33-year-old young healthy adult male who presented to the emergency with a 2-day history of pain abdomen and vomiting with no significant past history. There was no history of constipation and fever. Per abdomen examination revealed a tender lump in the right paraumblical region. Except for the presence of neutrophilia, the baseline investigations were normal while his ultrasonography abdomen showed distension of gut loops. X-ray abdomen (standing) showed the presence of coffee-bean appearance type dilatation of the large gut on the right side with concavity toward the left upper quadrant [Figure 1]. The diagnosis of intestinal obstruction was made on clinical and X-ray features. In view of clear-cut diagnosis of acute intestinal obstruction and the need for emergent laparotomy, the computed tomographic scan of abdomen was not done.
Figure 1: X-ray abdomen of the patient showing features of cecal volvulus

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Emergency laparotomy was done. However, to our surprise, we found that the patient is having malrotated gut with cecal volvulus with features of intestinal obstruction. Actually, it was a Type-1 malrotation. The cecal volvulus had formed the obstructed closed loop with gross dilatation of the loop involving the cecum and the ascending colon [Figure 2]. The DJ flexure was on the right side of the superior mesenteric vessels and the lumbar spine. The whole of the small gut was packed on the right side of the abdominal cavity, while the large gut was on the left side [Figure 3]. The cecum and ascending colon were redundant, while the transverse colon was retroperitonalized. The cecal volvulus was carefully de-torted. However, due to the preischemic and patchy hemorrhagic changes [Figure 2] in the obstructed and grossly dilated, loosely redundant caeco-ascending colon loop and the probability of future recurrences, the decision of right hemicolectomy instead of cecopexy was taken. Hence, the right hemicolectomy with hand-sewn end-to-side ileotransverse anastomosis (double layer using 2-0 and 3-0 Vicryl sutures) was done with the placement of pelvic drain [Figure 4]. Postoperatively, the patient behaved well. Liquid orals were started on the 2nd postoperative day (POD) and the patient moved bowels on the 3rd POD. Drain removed on the 7th POD and the patient discharged on the 8th POD. The patient is still on follow-up and doing well.
Figure 2: Gross dilatation of cecum and ascending colon loop with ischemic patches

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Figure 3: Cecal volvulus with axial torsion

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Figure 4: Right hemicolectomy specimen

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  Discussion Top


The cecum measures 6 cm in length and 7.5–8 cm in width and is the proximal most portion of the colon. The cecum is covered by the visceral peritoneum without any distinct mesentery and has limited mobility.[4] However, sometimes, the cecum may have the extended mobility, predisposing it to volvulus. The pathogenesis of cecal volvulus is mainly attributed to the redundancy of the cecum due to long right colonic mesentery. In its frequency of occurrence, among the large bowel volvuluses, the cecal volvulus is the second most common cause after the sigmoid volvulus.

According to the Stringer classification, there are three forms of malrotation; Type 1 – Nonrotation, Type 2 – Duodenal malrotation, and Type 3 – duodenal plus cecal malrotation.[5] The patient discussed here was a case of Type 1 malrotation (nonrotation) and had malpositioned bowel loops with malfixation of the mesentery. The vast majority (64%–80%) of intestinal malrotation cases present during the neonatal period only, and up to 90% present within the 1st year of life.[6] Adult presentation of intestinal malrotation is very rare and accounts for only 0.2%–0.5% of cases.[7],[8],[9] Cecal volvulus in an adult usually presents as a case of distal small bowel obstruction with acute onset of abdominal pain and vomiting; however, it may sometimes contribute to the unusual clinical presentations of acute appendicitis.[10]

Treatment and prognosis of cecal volvulus largely depend upon the intraoperative findings such as viability of the gut, degree of gut distension due to obstruction, and degree of redundancy and fixation of various parts of the gut. However, surgery is the only definite treatment of the cecal volvulus. In cecal volvulus due to malrotation, most of the times, the surgery may limit to the de-torsion of the cecal volvulus with cecopexy plus–minus Ladd's procedure.[2],[11],[12],[13] Rarely, the gut resections such as right hemicolectomies are needed.[14],[15],[16] Arulmolichelvan et al.[11] managed a case of cecal volvulus due to intestinal malrotation in a 23-year-old young patient who presenting as postoperative (postpercutaneous nephrolithotomy) intestinal obstruction by laparotomy with de-rotation with cecopexy.

The goals of treatment are the immediate and adequate resuscitation, quick radiological confirmation of the diagnosis followed by emergent surgery to de-tort the intestinal torsion, decompress the obstruction, and fix the cecum in the right lower quadrant in case the gut is healthy and not ischemic. The redundant cecum may be fixed either by creating a pocket in the right parietal peritoneum and suturing the cecum to the peritoneum or by using tube cecostomy. However, if strangulation and gut ischemia have already occurred at the time of laparotomy, then right hemicolectomy may be the definitive procedure.


  Conclusion Top


This case highlights the importance of suspecting preoperatively the concomitant occurrence of gut malrotation in a case of cecal volvulus.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Rabinovici R, Simansky DA, Kaplan O, Mavor E, Manny J. Cecal volvulus. Dis Colon Rectum 1990;33:765 9.   Back to cited text no. 1
    
2.
Pramod S, Kumar G. Rare presentation of acute intestinal obstruction: Cecal volvulus with malrotation. J Case Rep 2019;9:33-5.  Back to cited text no. 2
    
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Luca S, Pemberton JH. Embryology and anatomy of the colon. In: Shackelford's Surgery of the Alimentary Tract. Vol. 2, 6th ed. Saunders Elsevier, 2007; USA. P-1862.  Back to cited text no. 3
    
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Ballantyne GH, Brandner MD, Beart RW Jr., Ilstrup DM. Volvulus of the colon. Incidence and mortality. Ann Surg 1985;202:83-92.  Back to cited text no. 4
    
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Huseyin K Bektasoglu, Ufuk O Idiz, Mustafa Hasbahceci, Erkan Yardimci, Yurdakul D Firat, Oguzhan Karatepe, et al. Midgut malrotation causing intermittent intestinal obstruction in a young adult. Case Reports in Surgery, Volume 2014, Article ID 758032, 3 pages. http://dx.doi.org/10.1155/2014/758032 [PMCID: PMC4054901].  Back to cited text no. 5
    
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Torres A, Ziegler M. Malrotation of the intestine. World J Surg 1993;17:326-31.  Back to cited text no. 7
    
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Adrian T. Fung, David E. Konkin, and Zaheer S. Kanji. Malrotation with midgut volvulus in an adult: A case report and review of the literature. Journal of Surgical Case Reports, 2017;5, 1–3. doi: 10.1093/jscr/rjx081.[PMCID: PMC5441244].  Back to cited text no. 8
    
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Low S, Ngiu C, Sridharan R, Lee Y. Midgut malrotation with congenital peritoneal band: A rare cause of small bowel obstruction in adulthood. BMJ Case Rep. Volume 2014, issue-January 2014. Available from: [PMCID: PMC3948007]  Back to cited text no. 9
    
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Pinto A, Di Raimondo D, Tuttolomondo A, Fernandez P, Caronia A, Lagalla R, et al. An atypical clinical presentation of acute appendicitis in a young man with midgut malrotation. Radiography 2007;13:164-8.  Back to cited text no. 10
    
11.
Arulmolichelvan A, Arjun S, Ashok M. Cecal volvulus associated with intestinal malrotation presenting as postoperative intestinal obstruction. Med Princ Pract 2012;21:389-91.  Back to cited text no. 11
    
12.
Yadav DK, Khanna K, Jain V, Amat Us Samie. Caecal volvulus with intestinal malrotation: need for caecopexy? BMJ Case Rep; Volume 2017, issue-January 2017; [doi: 10.1136/bcr 2017222247].  Back to cited text no. 12
    
13.
Ferreira MS, Simões J, Folgado A, Carlos S, Carvalho N, Santos F, et al. Recurrent midgut volvulus in an adult patient – The case for pexy? A case report and review of the literature. Int J Surg Case Rep 2020;66:91-5.  Back to cited text no. 13
    
14.
Butterworth WA, Butterworth JW. An adult presentation of midgut volvulus secondary to intestinal malrotation: A case report and literature review. Int J Surg Case Rep 2018;50:46-9.   Back to cited text no. 14
    
15.
Singh S, Das A, Chawla AS, Arya SV, Chaggar J. A rare presentation of midgut malrotation as an acute intestinal obstruction in an adult: Two case reports and literature review. Int J Surg Case Rep 2013;4:72-5.  Back to cited text no. 15
    
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Sözen S, Güzel K. Intestinal malrotation in an adult: Case report. Turk J Trauma Emerg Surg Case Rep 2012;18:280-2.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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