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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 196-198

Cecal duplication: An unusual presentation


Department of Neonatology and Radiology, Emirtaes Specialty Hospital, Dubai Health Care City, Dubai, United Arab Emirates

Date of Submission02-Feb-2021
Date of Decision29-Jul-2021
Date of Acceptance08-Aug-2021
Date of Web Publication31-Aug-2021

Correspondence Address:
Dr. Monika Kaushal
Department of Neonatology and Radiology, Emirtaes Specialty Hospital, Dubai Health Care City, Dubai
United Arab Emirates
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_40_21

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  Abstract 

Background: Enteric duplication cysts are rare congenital malformations that are most commonly found in the ileum and ileocecal junction, followed by the esophagus, stomach, and duodenum. Although most present in the 1st 2 years, they are often difficult to diagnose as both presentation and radiological findings can be variable and nonspecific. Clinical Description: We present the case report of an 11-month-old child who presented with nonbilious vomiting and diarrhea. There was no fever, abdominal distension, blood in stools, or crying on defecation. A provisional diagnosis of acute gastroenteritis was kept and management started as per the standard protocol. Management: A series of investigations were carried out when the child did not improve and developed bilious vomiting. Ultrasound findings were suggestive of an ileal duplication cyst which was confirmed by magnetic resonance imaging. The child then underwent surgery where cecal duplication cyst was confirmed, which was previously missed due to a malpositioned cecum. Conclusion: Gastrointestinal duplication cysts are rare developmental anomalies that may present as an acute abdomen in a young child. The possibility of the duplication cyst should be kept as a differential diagnosis in a child presenting with unexplained vomiting, abdominal distension, or pain. Early suspicion, investigation, and intervention help in faster recovery with minimal complications.

Keywords: Cecal duplication, diarrhea, vomiting


How to cite this article:
Kaushal M, Kaushal A, Sasidharan K, Gupta S. Cecal duplication: An unusual presentation. Indian Pediatr Case Rep 2021;1:196-8

How to cite this URL:
Kaushal M, Kaushal A, Sasidharan K, Gupta S. Cecal duplication: An unusual presentation. Indian Pediatr Case Rep [serial online] 2021 [cited 2021 Sep 26];1:196-8. Available from: http://www.ipcares.org/text.asp?2021/1/3/196/325099

Intestinal duplications are rare congenital malformations that are encountered in one out of 4000–5000 births.[1] They are found most commonly in the ileum and ileocecal junction (53%), followed by the esophagus, stomach, and duodenum.[2] Colonic duplications are infrequent, accounting for 13% of total cases.[2] These develop during the embryonic growth of the gastrointestinal tract during the 1st 6–8 weeks of the development. Only a minority are diagnosed prenatally (20%–30%), but the most common age of presentation is during infancy (70%).[3] Clinical presentation and radiological findings are variable and nonspecific, leading to delayed diagnosis. Only a quarter are diagnosed preoperatively.[3] Clinical manifestations depend on the location, size, and type.

A cecal duplication cyst usually presents with abdominal distension followed by vomiting. It is rare for a cecal duplication cyst to present with the features of acute gastroenteritis. This case offers an atypical presentation of a cecal duplication cyst due to its abnormal location that was identified intraoperatively.


  Clinical Description Top


An 11-month-old boy presented to our emergency department with 36-h history of persistent vomiting and one episode of loose stools. Before coming to us, his parents had taken him to another health-care facility, where he had been prescribed antiemetics and oral rehydration solution (ORS) and sent home. However, the vomiting continued and the frequency of watery stools increased. The vomiting was nonbilious and nonprojectile. The child did not cry while defecating. There was the absence of blood or mucus in the stools and no bleeding per rectum. There was no history of fever or symptoms suggestive of acute intestinal obstruction such as abdominal distension, discomfort, and obstipation. The child had an adequate urine output. Child was not lethargic though the activity was reduced than normal.

On general examination, vitals were stable with no signs of dehydration. Anthropometric measures showed stunting with other growth parameters within normal range (weight: 8.28 kg [0–2 standard deviation (SD)], length: 68.5 cm [-2SD-3SD], and weight for length 0- +1SD). There was no pallor, icterus, or lymphadenopathy. Per abdomen examination did not reveal any abdominal distension or tenderness on palpation. Bowel sounds were normal, and no mass could be palpated per abdomen. A per rectal examination was performed to look for currant jelly stools, and the result was normal. Other systemic examinations were within normal limits.

We kept a provisional diagnosis of acute gastroenteritis with and started the child on intravenous fluid, antiemetics, zinc, and ORS along with feeding. At 18 h of admission, the child had an episode of bilious vomiting, following which further investigations were planned to rule out obstruction.


  Management and Outcome Top


Baseline complete blood count, C-reactive protein, renal function test including serum electrolytes, and liver function test were normal (hemoglobin: 11.5 mg/dl, total leukocyte count: 12100 cells/cumm, platelet count: 5.14lakh/cumm, C-reactive protein: 3.5 mg/dl, blood urea: 21.6 mg/dl, serum creatinine: 0.36 mg/dl, serum sodium: 137 mg/dl, and serum potassium: 4.7 mg/dl), and a routine stool test to look for adenovirus and rotavirus was negative.

A radiographic examination of the abdomen revealed large, dilated bowel loops. The ultrasound of abdomen showed a thick-walled cystic lesion in the right hypochondrium [Figure 1], with a typical gut signature sign of the intestinal wall. Magnetic resonance imaging of the abdomen was done as it provides excellent soft-tissue resolution and anatomical orientation. It confirmed the presence of a well-defined lobulated cyst at the level of hepatic flexure [Figure 2]. These findings suggested an ileal duplication cyst due to its location and presentation.
Figure 1: Ultrasonography abdomen showing a thick-walled cyst in the right hypochondrium close to the inferior margin of the liver at the level of the right kidney measuring 4.3 cm × 2.4 cm × 2.5 cm in size

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Figure 2: Coronal, axial and sagittal view of abdomen showing a well-defined lobulated cyst in the right hypochondrium, adjacent and medial to the inferior margin of liver at the level of the hepatic flexure

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Surgery was planned, and preoperative examination under anesthesia revealed a mass in the hypochondrium. We could not feel the mass on routine palpation as it was deep, and the child was not very cooperative before giving anesthesia. Intraoperatively, the cecum was noted to be enlarged, mobile, and subhepatic in position, with a spherical cyst attached to the cecal wall and filled with serous fluid. The cecal cyst was compressing the adjacent stomach and intestines, causing proximal dilatation. There was no associated malrotation. The cecum with the cyst, appendix, and terminal ileum was resected, followed by end-to-end anastomosis. Histopathological analysis confirmed the diagnosis of a cecum duplication cyst based on the presence of a common single outer muscular layer and an innermost lining of the gastrointestinal mucosa. Following an uneventful postoperative period of 10 days, the child was discharged. Other structural anomalies were not found in a more in-depth evaluation of other systems. The child has been in follow-up for a year and has remained well.


  Discussion Top


The essential features of an enteric duplication cyst include a well-developed smooth muscle coat; mucosal lining found within some portion of the alimentary tract; and attachment or communication with any segment of the alimentary tract.[1] Although rare (13%), colonic duplications are primarily associated with the transverse colon and are uncommon in cecum accounting for 0.4% of all gastrointestinal duplications.[2] Only 20 such cases had been reported in the literature.[4] The etiopathogenesis of these malformations is still unknown. However, the various hypotheses include splitting of the notochord during in utero development, perseverance of fetal enteric diverticula, vascular occlusion in the intrauterine period, and the failure of recanalization of the intestine. Enteric duplications may be cystic, tubular, or mixed. Cystic colonic duplications are usually not associated with other anomalies, whereas tubular colorectal duplications are often associated with genitourinary, vertebral, and other structural anomalies.

Up to 80% of duplications are identified before 2 years of age. They can mimic intussusception (waxing-waning abdominal pain, vomiting, and passage of red currant stools); we found four case reports of cecal duplication mimicking intussusception[5] or malrotation/volvulus (abdominal pain and distension with vomiting and obstipation). A duplication cyst can cause recurrent episodes of abdominal pain and vomiting in infancy. Proximal intestinal lesions usually present with vomiting as an early symptom. In contrast, distal lesions present with abdominal distension and constipation. Vomiting usually does not occur. Colonic duplications tend to present later in life compared to other sites with symptoms of distal intestinal obstruction.[6] In our case, nonbilious vomiting was the only symptom that led to further evaluation for the proximal intestinal lesions. Even the radiological investigations suggested the same. Distal intestinal involvement was identified only intraoperatively. The atypical presentation of symptoms despite being located in the large bowel was due to the cecal malposition. The cecum was mobile and subhepatic, which when distended, had led to compression of the stomach, leading to the vomiting.

A literature search revealed six cases of malrotation associated with enteric duplication. All of them were jejunal and ileal duplications that presented with the symptoms of upper intestinal obstruction in the newborn period.[7] Somuncu et al. reported malrotation and jejunal duplication in a 2 years old who had intermittent vomiting.[8] Although the cecum was mobile and subhepatic in this case, the duodenojejunal flexure was in the proper position that ruled out malrotation. We found only one similar case in the literature in a newborn with intestinal duplication and a malfixed right colon.[9] Kibayashi et al. also reported a similar case in an infant who died with complications from a duplication cyst, after being misdiagnosed as gastroenteritis.[10]

Detection and diagnosis of enteric cysts can be difficult, as their symptoms vary significantly and can reflect a broad spectrum of gastrointestinal conditions, medical and surgical. In our case, we were unable to appreciate a mass clinically, probably due to its deep location. Atypical surgical malformations can present with gastroenteritis. In this case, the character of vomitus was initially nonbilious, presumably because the cecal duplication cyst was abnormally high and compressed the stomach. It is possible that the transgressions in intestinal contraction and the increase in the intestinal content led to the obstruction of the intestine, causing bilious vomiting later. Ultrasound is the most used investigative approach for abdominal imaging in children. The classical sonographic feature is a double wall or muscular sign (innermost hyperechoic mucosa and external hypoechoic muscularis propria) called “gut signature sign.” MRI provides excellent soft-tissue resolution and anatomical orientation and helps to establish the correlation between the cyst and adjacent structures before surgery.

Surgical excision is recommended even in asymptomatic cases. A classic laparotomy approach is the modality of choice, but there are reports of successful, less invasive techniques such as laparoscopy.[10] Adjacent normal bowel requires resection in addition to the cyst, as they share the same muscular wall and blood supply. In this case, we removed the terminal ilium, appendix, and cecum due to the presence of a common muscular wall and vascular supply. When the structures have an independent blood supply, they can be resected in isolation. The resection should be complete; otherwise, there is a chance of recurrence. Our case highlights a unique point of proximal intestinal obstruction secondary to a cecal duplication cyst due to the abnormal location of the cecum.



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.
Chang HC, Huang SC, Chen TC, et al. Y-shaped colonic duplication: Report of a case and literature review. Chang Gung Med J 2011;34:43-7.  Back to cited text no. 1
    
2.
Sharma S, Yadav AK, Mandal AK, et al. Enteric duplication cysts in children: A clinicopathological dilemma. J Clin Diagn Res 2015;9:C08-11.  Back to cited text no. 2
    
3.
Atalar MH, Cankorkmaz L, Ozer H, et al. A huge duplication cyst of the ileum. Pol J Radiol 2013;78:70-3.  Back to cited text no. 3
    
4.
Ijaz L, Husnain M, Malik SI, et al. Cecal duplication cyst presenting as acute intestinal obstruction in an infant. APSP J Case Rep. 2011;2:11.  Back to cited text no. 4
    
5.
Verma S, Bawa M, Rao KL, et al. Caecal duplication cyst mimicking intussusception. BMJ Case Rep 2013;2013:bcr2012008056.  Back to cited text no. 5
    
6.
Bakir B. Colonic duplications: Clinical presentation and radiologic features of five cases. Eur J Radiol 2007;61:490.  Back to cited text no. 6
    
7.
Macutkiewicz C, Carlson GL. Acute abdomen: Intestinal obstruction. Surgery (Oxford) 2005;23:208-12.  Back to cited text no. 7
    
8.
Somuncu S, Cakmak M, Caglayan E, et al. Intestinal duplication cyst associated with intestinal malrotation anomaly: Report of a case. Acta Chir Belg 2006;106:611-2.  Back to cited text no. 8
    
9.
Rahul SK, Upadhyaya VD, Kumar B. Malrotation and midgut volvulus associated with asymptomatic duplication cyst of jejunum. APSP J Case Rep 2016;7:33.  Back to cited text no. 9
    
10.
Kibayashi K, Sumida T, Shojo H, et al. Unexpected death due to intestinal obstruction by a duplication cyst in an infant. Forensic Sci Int 2007;173:175-7.  Back to cited text no. 10
    


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