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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 34  |  Issue : 2  |  Page : 115-116

Sigmoid volvulus in a child: An uncommon presentation of Hirschsprung disease


Department of Pediatric Surgery, King George's Medical University, Lucknow, Uttar Pradesh, India

Date of Submission17-Apr-2020
Date of Acceptance08-Sep-2020
Date of Web Publication25-Jan-2021

Correspondence Address:
Anand Pandey
Department of Pediatric Surgery, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jms.jms_27_20

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  Abstract 


Sigmoid volvulus (SV) is a rare cause of intestinal obstruction in childhood, and it is very uncommon below the age of 20 years and above 70 years. In childhood, SV comprises 4% of all intestinal volvulus. Hirschsprung disease (HD) is a developmental disorder of the enteric nervous system, which is characterized by the absence of ganglion cells in the myenteric and Meissner plexus; rarely, SV may complicate HD. We treated one such patient. Being extremely rare, this association is being reported with a review of the relevant literature.

Keywords: Hirschsprung disease, intestinal obstruction, sigmoid volvulus


How to cite this article:
Kumar A, Shandilya G, Pandey A, Gupta A. Sigmoid volvulus in a child: An uncommon presentation of Hirschsprung disease. J Med Soc 2020;34:115-6

How to cite this URL:
Kumar A, Shandilya G, Pandey A, Gupta A. Sigmoid volvulus in a child: An uncommon presentation of Hirschsprung disease. J Med Soc [serial online] 2020 [cited 2021 Mar 2];34:115-6. Available from: https://www.jmedsoc.org/text.asp?2020/34/2/115/307897




  Introduction Top


Sigmoid volvulus (SV) is a rare cause of intestinal obstruction in childhood, and it is often associated with other medical disorders.[1],[2] SV is very uncommon below the age of 20 years and above 70 years.[3] In childhood, SV comprises 4% of all intestinal volvulus. Clinical diagnosis in children is often difficult.[4]

Hirschsprung disease (HD) is a developmental disorder of the enteric nervous system, which is characterized by the absence of ganglion cells in myenteric and Meissner plexus.[4] The typical clinical presentation of HD is abdominal distension with delayed passage of the first meconium. Mismanaged patients may present with toxic megacolon or pneumoperitoneum. Rarely, HD may be complicated by SV. We treated one such patient. Being extremely rare, this association is being reported with a review of the relevant literature.


  Case Report Top


A 3-year-old male child was admitted in the emergency department with complaints of abdominal distension, inability to pass flatus and feces, and on and off bilious vomiting for the last 5 days. On examination, he was severely dehydrated and lethargic. His abdomen was distended and visible bowel loop present. The digital rectal examination had no significant finding. X-ray abdomen and pelvis anteroposterior erect view was obtained, which showed multiple air and fluid levels. The patient was planned for emergency laparotomy.

He was resuscitated with intravenous (IV) fluid and IV antibiotics covering Gram-positive, negative, and anaerobe organisms. Exploratory laparotomy revealed SV with 270° counterclockwise rotation [Figure 1]a and [Figure 1]b at the sigmoid colon mesentery axis. On the clockwise de-rotation of volvulus, a transition zone was noticed with the collapsed distal segment and dilated proximal segment. After completion of exploration, divided sigmoid colostomy was made, along with a serial biopsy of the colon from the colostomy site, transition zone, and beyond it. The postoperative period was uneventful, and stoma becomes functional on the 2nd postoperative day.
Figure 1: (a) Intraoperative picture showing sigmoid volvulus. (b) Sigmoid colon after de-rotation

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Histopathology examination (HPE) report showed the absence of ganglion cells in rectosigmoid with adequate ganglion cells at the colostomy site. Hence, the diagnosis of HD was confirmed. As of now, the patient is waiting for definitive surgery.


  Discussion Top


SV is an infrequent clinical entity in children.[1] It occurs due to the rotation of a redundant sigmoid loop around a narrow, elongated mesentery, which leads to reversible or irreversible vascular compromise.[2] On the contrary, HD is frequently seen in our setup. It is dominant in male children in a ratio of 4:1.[1] SV, as a complication of HD, is extremely rare. Its prevalence has been reported to be 0.66%. However, among children presenting with SV, 18% of children have coexisting HD.[5]

The usual presentation of HD is abdominal distension and failure to pass meconium in neonatal age and chronic constipation at a late age.[6] There were missing in this patient. It appears that attendants failed to notice these complaints. Besides this, probably due to volvulus, DRE was noninformative as the air might have been above the level of obstruction. Because of no definite history and normal DRE, our provisional diagnosis was mechanical obstruction, most likely due to band obstruction. After exploratory laparotomy, our diagnosis was changed to HD complicated by SV. Patients of SV with HD present in more acute conditions than SV without HD.[5] X-ray evaluation may not be able to diagnose SV; hence, the possibility of a diagnosis of SV with HD is unlikely.[7],[8] This limitation was noted by us also.

The management of SV, due to any reason, has been described as conservative or surgical. If a diagnosis of SV is made and patients have been stable, sigmoidoscopy or contrast enema through the rectal tube has been advocated.[7],[8] If the obstruction is severe, the patient may need urgent laparotomy. In our patient, there was no evidence of SV on X-ray, and our diagnosis was band obstruction. In this condition, we proceeded to exploratory laparotomy. We made the diagnosis intraoperatively, which was confirmed after the HPE report.

To conclude, SV with HD is an extremely uncommon clinical association. It may have a varied presentation. Any case of SV in pediatric age must have histological confirmation of adequate ganglion cells in the colon before proceeding with definitive management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their 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.
Stehr W, Cynthia A. Gingalewski. Other Causes of Intestinal Obstruction. In: Coran AG, Krummel TM, Laberge J, Shamberger RC, Caldamone AA, editors. Pediatric Suregry. 7th ed. Philadelphia PA: Elsevier Saunders; 2012. p. 1127-34.  Back to cited text no. 1
    
2.
Ranjan A, Jain V, Sharma S, Gupta DK. Sigmoid volvulus: an uncommon complication of Hirschsprung's disease. BMJ Case Rep. 2016;2016:bcr2016214693.  Back to cited text no. 2
    
3.
Rajsiddharth B, Patlolla SR, Reddy BS, Sriramoju S, Kumar PB, Maripeddi K. A Clinical Study of Sigmoid Volvulus. Int J Sci Stud 2016;3:158-162.  Back to cited text no. 3
    
4.
Tannouri S, Hendi A, Gilje E, Grissom L, Katz D. Pediatric colonic volvulus: A single-institution experience and review. J Pediatr Surg 2017;52:1062-6.  Back to cited text no. 4
    
5.
Zeng M, Amodio J, Schwarz S, Garrow E, Xu J, Rabinowitz SS. Hirschsprung disease presenting as sigmoid volvulus: A case report and review of the literature. J Pediatr Surg 2013;48:243-6.  Back to cited text no. 5
    
6.
Langer JC. Hirschsprung Disease. In: Coran AG, Krummel TM, Laberge J, Shamberger RC, Caldamone AA, editors. Pediatric Suregry. 7th ed. Philadelphia PA: Elsevier Saunders; 2012. p. 1266-78  Back to cited text no. 6
    
7.
Ito E, Ohdaira H, Suzuki N, Yoshida M, Suzuki Y. Percutaneous endoscopic sigmoidopexy for sigmoid volvulus: A case report. Int J Surg Case Rep 2015;17:19-22.  Back to cited text no. 7
    
8.
Atamanalp SS. Sigmoid volvulus. Eurasian J Med 2010;42:142-7.  Back to cited text no. 8
    


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