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 Table of Contents  
CASE SERIES
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 20-23

Case series of acute abdomen with midgut malrotation presenting in adulthood


Department of Surgical Gastroenterology, GMKMCH, Salem, Tamil Nadu, India

Date of Submission11-Jun-2021
Date of Decision01-Sep-2021
Date of Acceptance12-Oct-2021
Date of Web Publication01-Jan-2022

Correspondence Address:
A Sivasankar
Department of Surgical Gastroenterology, GMKMCH, Salem, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ghep.ghep_36_21

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  Abstract 


Introduction: Intestinal malrotation (IM) is a congenital anomaly due to partial or complete failure of the 270° counter-clockwise rotation of the midgut around the superior mesenteric vessels in 5th to 12th week of fetal life. Midgut malrotation usually presents as an emergency in the 1st month of life or within infancy. Adult cases are rarely reported and usually present with chronic nonspecific symptoms. Here, we present a case series of acute abdomen with midgut malrotation presenting in adulthood that came to the Department of Surgical Gastroenterology of a tertiary hospital in Salem, Tamil Nadu. Case Summary: Four patients presented with acute abdominal complaints, diagnosed, and proceeded with emergency laparotomy. Three patients had abdominal abnormalities unrelated to coexisting IM, and the other one showed sequelae of IM with Ladd's band and obstruction. Except the first patient, all other patients improved well postoperatively and were discharged without complications. Conclusion: IM should be considered in differential diagnosis of acute abdomen and bowel obstruction in adults. IM may be encountered as an incidental imaging/laparotomy finding or be the cause of acute abdomen.

Keywords: Acute abdomen, bowel obstruction, Ladd's band, midgut malrotation


How to cite this article:
Katheresan V, Ramamurthy C, Kumar DK, Kesavan B, Ponchidambaram M, Sivasankar A. Case series of acute abdomen with midgut malrotation presenting in adulthood. Gastroenterol Hepatol Endosc Pract 2022;2:20-3

How to cite this URL:
Katheresan V, Ramamurthy C, Kumar DK, Kesavan B, Ponchidambaram M, Sivasankar A. Case series of acute abdomen with midgut malrotation presenting in adulthood. Gastroenterol Hepatol Endosc Pract [serial online] 2022 [cited 2022 Aug 18];2:20-3. Available from: http://www.ghepjournal.com/text.asp?2022/2/1/20/334698




  Background Top


Intestinal malrotation (IM) is a congenital anomaly due to partial or complete failure of the rotation of the midgut around the superior mesenteric vessels in fetal life.[1] Incidence of malrotation is extrapolated from postmortem studies at 1 in 6000 live births.[2] Majority of malrotation cases present in the 1st month of life and 90% within the 1st year.[3]

Traditionally, adult presentation is considered to be very rare accounting for only 0.2% to 0.5% of cases.[4],[5] However, some studies suggest, malrotation may be more common in adulthood than previously considered.[6],[7]

Here, we present a case series of acute abdomen with midgut malrotation that came to the Department of Surgical Gastroenterology of a tertiary hospital in Salem, Tamil Nadu.


  Case Reports Top


Case report 1

A 32-year-old female patient was admitted to Surgical Gastroenterology Department with abdominal pain and vomiting for 2 days. Her medical history was unremarkable. Blood pressure (BP), pulse, and fever were measured as 80/60 mmHg, pulse 122/min, and 38°C, respectively. Examination revealed abdominal distension and diffuse guarding. Rectal examination revealed formed stool without blood. Computed tomography (CT) scan showed features of superior mesenteric artery (SMA) thrombosis, bowel ischemia, pneumatosis intestinalis, perfusion defect at the right lobe segment 8/5 of the liver, and features of nonrotation [Figure 1]. Laparotomy and exploration revealed necrosis involving 50 cm from duodenojejunal flexure (DJ flexure) to 20 cm from ileocecal junction (IC junction). Bowels were packed with warm pads for 10 min; some of the dusky segments were turned to pink. Small bowels which remained necrotic were resected, and proximal jejunostomy and distal ileal mucous fistula with appendicectomy were performed. Second-look laparotomy after 24 h revealed distal ileal mucous fistula which was healthy but 20 cm from DJ flexure including jejunostomy site of small bowel showed frank necrosis. Ischemic bowel segments were resected, and refashioning of jejunostomy was done. Postoperatively, the patient was intensively managed with inotropes, carbapenem group of antibiotics with parenteral hyperalimentation. Despite the above measures, the patient died on postoperative day (POD) 4 due to acute respiratory distress syndrome and multi-organ failure.
Figure 1: Bowel gangrene due to superior mesentric artery thrombosis

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Case report 2

A 50-year-old male presented with acute severe epigastric and right hypochondriac pain for 1 day. He was febrile with BP 110/70 mmHg and pulse rate of 110/min. On examination, his epigastrium and right hypochondriac region were tender. Total white blood cell count was 15,300/μL. His serum electrolytes, liver enzyme, and amylase levels were normal. Abdominal ultrasound (US) showed minimal free fluid at Morison's pouch; hence sealed perforated peptic ulcer was suspected. CT revealed a retrocecal thickened tubular structure at the right hypochondriac region, located subhepatically, extending cranially from the base. We revised our diagnosis to acute appendicitis with subhepatic location. Emergency laparotomy was done which showed findings of incomplete rotation type of malrotation, cecum was located in subhepatic region with inflamed appendix, and Ladd's peritoneal band [Figure 2] was present. Ladd's band is released between the cecum and the lateral abdominal wall and also between the duodenum and distal ileum. Then, the cecum is mobilized, and Ladd's procedure is performed followed by appendicectomy. Postoperative period was uneventful. The patient was discharged on POD 8.
Figure 2: Inflamed appendix in subhepatic region

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Case report 3

A 22-year-old female presented with complaints of intermittent cramping abdominal pain and bilious vomiting for 4 days. There was a past history of recurrent vomiting with pain abdomen since childhood. On examination, the patient was thin built, with a weight of 40 kg, a pulse rate of 98/min, and BP 100/70 mmHg. The abdomen was mildly distended, soft with normal bowel sounds with no peritoneal signs. Laboratory investigations were normal. CT showed features of nonrotation, large, and small bowel loops on the left and right side of abdomen, respectively, with dilated bowel loops. The patient did not show any improvement after 48 h of conservative measures, diagnosed as malrotation with obstruction and proceeded with exploration. Laparotomy showed that the cecum and appendix were on the left side of the abdomen, and the DJ flexure was on the right side. There was a narrow jejunocolic isthmus held with a band of fibro-fatty tissue from the colon across to the left side of the abdomen, and there was no evidence of strangulation or gangrene [Figure 3]. Ladd's procedure proceeded by releasing the band, and the ascending and transverse colon were freed. Then, the cecum was separated from the jejunum to widen the jejunocolic isthmus, and appendicectomy was performed. Postoperatively, the patient improved well and discharged on POD 7.
Figure 3: Intestinal malrotation present with obstruction

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Case report 4

A 60-year-old male presented with acute abdominal pain for 3 days and history of alcohol abuse. There was no history of trauma, loose stools, vomiting, fever, and melena. On examination, BP was 90/60 mmHg, pulse rate 110/min, abdomen mildly distended; diffuse tenderness and guarding present. X-ray erect abdomen shows air under diaphragm. Without delay, emergency laparotomy was done, and intraoperatively, duodenal (DU) perforation with malrotation of the gut was found [Figure 4]. Due to hemodynamic instability, live omental patch closure with appendicectomy was done without addressing the malrotation. Postoperatively, the patient improved well and discharged but lost subsequent follow-up for definitive surgical procedure.
Figure 4: Intestinal malrotation present with hollow viscus perforation

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  Discussion Top


Midgut malrotation is defined as failure of the midgut to rotate 270° anticlockwise in its return from the umbilical cord during fetal development which occurs between 5th and 12th week.[8] Nonrotation of duodenojejunal limb, followed by normal rotation and fixation of cecocolic limb, results in duodenal obstruction by abnormal mesenteric bands, the Ladd bands that extend from the cecum across anterior duodenum.[9]

Majority of patients present acutely with bilious vomiting to the emergency in infancy(75 %) or within the first 1 year of life (90 %).[10] Adult patients usually present with chronic recurrent abdominal pain and vomiting, hematemesis, weight loss, early satiety, constipation, and intermittent diarrhea.[11] Among this, recurrent abdominal pain is the most common presenting symptom in adults present with malrotation.[12]

IM in adults is rarely suspected based on the clinical history and physical findings alone. Diagnosis is confirmed by imaging studies or at laparotomy in most of the time. Upper gastrointestinal barium series detects malrotation by failure of DJ flexure to cross the midline and lying below the level of the duodenal bulb. CT findings include abnormal relationship between the SMA and superior mesenteric vein (SMV) like SMA, SMV in vertical or left right inverse relationship, but this abnormal orientation of SMA-SMV axis alone does not confirm the diagnosis as it can also be seen in patients without IM. Other CT findings include whirlpool signs, i.e., swirling appearance of bowel and mesentery twisted around the SMA axis, duodenal obstruction, congestion of mesenteric vasculature, are other supporting evidence of malrotation.[13] In our first case, SMA thrombosis was the cause for bowel gangrene with IM as an incidental finding. There was no evidence of midgut volvulus which is a major complication of IM due to clockwise rotation of bowel around SMA axis because of narrow mesenteric attachment.

In the second case, there was subhepatically located cecum with inflamed appendix and IM as a cause for emergency surgery. Typical clinical and CT findings of acute appendicitis are not seen in IM due to altered anatomy. Appendicitis should be ruled out if there is focal inflammation anywhere in the abdomen along with abnormal SMA/SMV relationship.[14]

In the third case, bowel obstruction is due to malrotation and Ladd's band itself. The surgery of choice is Ladd's procedure where the bands are sectioned, mesentery base widened, and intestinal repositioning done.

In the fourth case, acute abdomen is due to DU perforation with chance finding of malrotation. Although IM is a chance finding, surgical intervention is a better option as it has least morbidity and prevents future life-threatening consequences.

This case series depicts IM is more often encountered as a chance finding than by clinical diagnosis. It is also an eluding diagnosis in managing the cases of acute abdomen and with recurrent abdominal pain.


  Conclusion Top


IM should be considered in the differential diagnosis of acute abdomen and bowel obstruction and also with chronic vague abdominal symptoms in adults. IM may be encountered as an incidental imaging/laparotomy finding or be the cause of acute abdomen.

Acknowledgment

I also express my sincere gratitude to all the study subjects for their participation and co-operation in the study.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sahu SK, Raghuvanshi S, Sinha A, Sachan PK. Adult intestinal malrotation presenting as midgut volvulus: Case report. J Surg Arts 2012;5:18-21.  Back to cited text no. 1
    
2.
Biko DM, Anupindi SA, Hanhan SB, Blinman T, Markowitz RI. Assessment of recurrent abdominal symptoms after Ladd procedure: Clinical and radiographic correlation. J Pediatric Sug 2011;46:1720-5.  Back to cited text no. 2
    
3.
Emanuwa OF, Ayantunde AA, Davies TW. Midgut malrotation first presenting as acute bowel obstruction in adulthood: A case report and literature review. World J Emerg Surg 2011;6:22. [doi: 10.1186/1749-7922-6-22].  Back to cited text no. 3
    
4.
Fung AT, Konkin DE, Kanji ZS. Malrotation with midgut volvulus in an adult: A case report and review of the literature. J Surg Case Rep 2017;2017:rjx081.  Back to cited text no. 4
    
5.
Low SF, Ngiu CS, Sridharan R, Lee YL. Midgut malrotation with congenital peritoneal band: A rare cause of small bowel obstruction in adulthood. BMJ Case Rep 2014;2014:bcr2013202690.  Back to cited text no. 5
    
6.
Moldrem AW, Papaconstantinou H, Broker H, Megison S, Jeyarajah DR. Late presentation of intestinal malrotation: An argument for elective repair. World J Surg 2008;32:1426-31.  Back to cited text no. 6
    
7.
Durkin ET, Lund DP, Shaaban AF, Schurr MJ, Weber SM. Age-related differences in diagnosis and morbidity of intestinal malrotation. J Am Coll Surg 2008;206:658-63.  Back to cited text no. 7
    
8.
Arunachalam R. Malrotation and abdominal pain: A diagnosis eluding the unprepared mind. Indian J Gastroenterol 2015;34:415-7. [doi.org/10.1007/s12664-015-0615-y].  Back to cited text no. 8
    
9.
Alemany VS. Adult midgut malrotation: A case report. J Surg Proce Case Rep 2020;1:1-5.  Back to cited text no. 9
    
10.
Pumberger W, Kargl S. Malposition of the intestine malposition malrotation volvulus “midgut volvulus”. Eur Surg 2012;44:237-47.  Back to cited text no. 10
    
11.
Jadhav RR, Pai VD. Adult intestinal malrotation presenting as recurrent vomiting: A case report. Indian J Surg 2015;77:162-3.  Back to cited text no. 11
    
12.
Prasad GR, Rao JV, Fatima H, Shareef HM, Shah A, Satyanarayana G. Malrotation of midgut in adults, an unsuspected and neglected condition – An analysis of 64 consensus confirmed cases. Indian J Gastroenterol 2015;34:426-30.  Back to cited text no. 12
    
13.
Pickhardt PJ, Bhalla S. Intestinal malrotation in adolescents and adults: Spectrum of clinical and imaging features. AJR Am J Roentgenol 2002;179:1429-35.  Back to cited text no. 13
    
14.
Ben Ely A, Gorelik N, Cohen-Sivan Y, Zissin R, Carpineta L, Osadchy A, et al. Appendicitis in adults with incidental midgut malrotation: CT findings. Clin Radiol 2013;68:1212-9.  Back to cited text no. 14
    


    Figures

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



 

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