|Year : 2020 | Volume
| Issue : 1 | Page : 71-73
Caecal duplication cyst: A rare disease with variable presentation and its management in the era of laparoscopy
Rahul Saxena, Manish Pathak, Arvind Sinha
Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
|Date of Submission||19-Aug-2018|
|Date of Acceptance||13-Oct-2018|
|Date of Web Publication||20-Dec-2019|
Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur - 342 005, Rajasthan
Source of Support: None, Conflict of Interest: None
Duplication cysts of the intestine are a rare congenital anomaly that can occur anywhere throughout the length of intestine and can be either cystic or tubular. Around two dozen cases of caecal duplication cyst (CDC) are reported in the literature with variable presentation. We describe three cases of CDC who presented at variable age and with variable presentation. None of the imaging study could confirm the site of the duplication, preoperatively. All patients underwent laparoscopy-assisted resection and anastomosis. All patients are free of any complications at median follow-up of 24 months.
Keywords: Caecal duplication cyst, intestinal obstruction due to caecal duplication cyst, perforated caecal duplication cyst
|How to cite this article:|
Saxena R, Pathak M, Sinha A. Caecal duplication cyst: A rare disease with variable presentation and its management in the era of laparoscopy. J Min Access Surg 2020;16:71-3
|How to cite this URL:|
Saxena R, Pathak M, Sinha A. Caecal duplication cyst: A rare disease with variable presentation and its management in the era of laparoscopy. J Min Access Surg [serial online] 2020 [cited 2020 Jan 29];16:71-3. Available from: http://www.journalofmas.com/text.asp?2020/16/1/71/249444
| ¤ Introduction|| |
Duplication cysts of the intestine are a rare congenital anomaly that can occur anywhere throughout the length of intestine and can be either cystic or tubular. The most common site is ileum, only 13% of all gastrointestinal duplications arise from the colon, and those arising from caecum are very rare. Around two dozen cases of caecal duplication cyst (CDC) are reported in the literature. Patients may present at any age, but 80% present in the first 2 years of life. The clinical presentation is variable depends on the location, size and presence of ectopic gastric mucosa. Patients can present with abdominal mass, intussusceptions, gastrointestinal haemorrhage, abdominal pain mimicking appendicitis and chronic or acute intestinal obstruction.
| ¤ Case Reports|| |
We are describing three patients of CDC who have presented at different ages with different complaints.
A 2-month-old female child presented with fever, loose motion and abdominal distension and dehydration, shock and prerenal acute kidney injury with altered sensorium. She also had one episode of seizure outside hospital. X-ray abdomen had dilated bowel loops.
On ultrasound, a well-defined cystic lesion showing double-wall sign is seen in the right side of the abdomen anterior to the right kidney measuring 2.3 cm × 1.8 cm. No communication with the adjacent bowel loops was noted. The lesion shows internal echogenic debris within. Another similar lesion was seen inferior to the kidney measuring 2.67 cm × 1.8 cm with internal septation and echogenic debris within. Contrast-enhanced computed tomography was suggestive of bilobed cystic lesion in the mesentery abutting the jejunal loops likely bilobed enteric duplication cyst. Diagnostic laparoscopy identified CDC at the mesenteric border which was dissected and delivered by right transverse incision [Figure 1]a and [Figure 1]b. Ileocaecal segment was resected and an end-to-end anastomosis was done. Biopsy suggested CDC lined with columnar epithelium. The postoperative period was uneventful, and the child is thriving well in 6-month follow-up.
|Figure 1: (a) Laparoscopic view of CDC (b) ileocecal segment was delivered out by small transverse incision; CDC held by babcock's forceps (c) ileocecal segment after resection in case 2 (d) Perforated cecal duplication cyst of case 3; (*Cecal Duplication cyst)|
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An 18-year-old boy presented with abdominal pain for 2 days associated with non-bilious vomiting. On examination, there was pain and tenderness in the right hypochondrium and epigastrium. Ultrasound detected a lobulated hypoechoic area 40 × 47 × 47 with thick debris seen in the right hypochondrium-epigastric region. On diagnostic laparoscopy, CDC was identified at the mesenteric border that was dissected and delivered out by transverse abdominal incision. The ileocaecal junction with CDC was resected, and side-to-side stapled anastomosis was done [Figure 1]c. Biopsy confirmed the diagnosis of inflamed CDC lined with small intestine mucosa. The patient recovered well and is asymptomatic in the follow-up of 2 years.
A 12-year-old boy presented with abdominal pain, fever and vomiting for 2 days. O/E tenderness, guarding and rigidity were present in the right lower abdomen. Ultrasound was suggestive of perforated appendix with periappendiceal loculated collection of volume 34cc and extending up to the subhepatic region. On diagnostic laparoscopy, perforated CDC with localised collection was identified with infarction of adjacent caecum. After laparoscopic dissection ileocaecal junction and right colon, it was delivered out by right transverse incision and right hemicolectomy was done and ileotransverse anastomosis was done [Figure 1]d. On biopsy, perforated CDC was identified with variable lining epithelium, including gastric mucosa, stratified columnar epithelium, and metaplastic squamous epithelium. Postoperative recovery was uneventful, and the patient is well in 3-year follow-up.
| ¤ Discussion|| |
Enteric duplication cyst is rare congenital anomaly that can occur anywhere from the mouth to the anus. They involve small intestine most commonly where the order of occurrence is ileum, jejunum and duodenum. Only 13% of duplication are present in colon. The caecum is rare site for duplication cyst. Oudshoorn in his review of 362 cases of duplication cysts found only 16 patients of CDC. Patients with CDC can present at any age but up to 80% of patients do so within 2 years of age. Our one patient was infant 2 of our patients presented very late in the second decade of life. CDC. About 45 cases of CDC have been described in previous literature, and it is more common in females. It can be tubular or spherical in shape. Spherical type is more common and all of our patients had spherical CDC. Presenting feature may differ depending on site, size and morphology of CDC. The common symptom and signs are vomiting, abdominal mass, abdominal distension, pain abdomen, blood in stools and constipation.
The presentation is variable, and it may present as intestinal obstruction in neonates and infants. Case 1 of our series presented with sepsis and recurrent intestinal obstruction. Thus, CD should be kept as important differential while dealing with neonates with intestinal obstruction and palpable abdominal mass such as mesenteric, ovarian or choledochal cysts. The presentation may mimic intussusception or can act as lead point for intussusception.,, It may mimic present with peritonitis as our case 2 and as perforated appendix as our case 3. Duplication cysts may have malignant transformation, and cases of adenocarcinoma from cyst wall have been reported.,
Ultrasound is most common modality used for the diagnosis, and the 'double-wall' sign is highly suggestive of enteric duplication with high specificity and positive predictive value whereas CT and MRI are less commonly used., Two of our cases (case 1 and 2) were diagnosed on ultrasound, but the specific site was not identified, and although the findings were confirmed in detail on CT, the exact site was not located which was done on diagnostic laparoscopy. Case 3 was diagnosed as perforated appendix, which was diagnosed to have CDC on diagnostic laparoscopy.
Duplication cysts are closely associated with some portion of gastrointestinal tract, have a well-developed smooth muscle layer and epithelial lining of gastrointestinal tract and share blood supply with associated bowel. In two of our patients, CDC was lined with columnar epithelium, and ectopic gastric mucosa was present in one patient.
Treatment is segmental surgical resection of small cystic or short tubular duplication along with adjacent intestine, with a primary end-to-end anastomosis. If the lesion is long and tubular and cannot be excised to preserve length of bowel, other options include enucleation, marsupialisation and mucosal excision.,, In two of our patients, we identified and dissected CDC laparoscopically and exteriorised the segment for resection and end-to-end anastomosis. The patient with perforated CDC was converted to open procedure to manage the peritoneal collection and perform hemicolectomy.
| ¤ Conclusion|| |
CDC may have variable presentation at different ages. CDC is to be considered in the differential diagnosis in neonates and children who present with acute or subacute intestinal obstruction. The most common imaging modalities to diagnose duplication cysts are ultrasonography and CT abdomen, but identifying the exact location of cyst is still difficult. Laparoscopy plays a vital role in the diagnosis, identifying the site, dissection and possibly definitive management. Complete surgical resection is treatment of choice, and early intervention can prevent extreme morbidities such as sepsis and perforation peritonitis.
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.
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Conflicts of interest
There are no conflicts of interest.
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