Concomitant intussusception and appendicitis "appendi-sception" in children: A case report and review of literature

Conceptualization: Bhawesh Bhattarai, Sujan Paudel, Prajjwol Luitel, Asim Shrestha, Bibek Poudel, Dinesh Koirala. Patient Management: Bhawesh Bhattarai, Dinesh Koirala. Writing – original draft: Bhawesh Bhattarai, Sujan Paudel, Prajjwol Luitel, Asim Shrestha, Bibek Poudel, Dinesh Koirala. Writing – review & editing: Bhattarai, Sujan Paudel, Prajjwol Luitel, Asim Shrestha, Bibek Poudel, Dinesh Koirala. Visualization and Supervision: Bhawesh Bhattarai, Sujan Paudel, Prajjwol Luitel, Asim Shrestha, Bibek Poudel, Dinesh Koirala.

Written informed consent was obtained from the patient's parents for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Since this is a case report and review of literature, our Institutional Review Board of Institute of Medicine, Tribhuvan University has waived the requirement for ethical approval.

This study received no funding.

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Sujan Paudel.

It's crucial to acknowledge that the secondary causes may be involved in the intussusception, particularly in cases not responding to initial management. CT scan should be done in such cases to rule out other differentials and find secondary causes of intussusception. Co-existing intussusception and appendicitis can be present in children which can be successfully managed with appendectomy and manual reduction in the same setting. Therefore, this case highlights the value of considering multiple potential diagnoses when faced with complex presentations, especially in pediatric patients who may not clearly articulate their symptoms. The following is the supplementary data related to this article. Supplementary Video 1 Supplementary Video 1

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Appendicitis as a lead point in intussusception is exceptionally rare and adds complexity to both diagnosis and treatment. Few cases have been reported in pediatric population (< 18 years), we performed search of literature in PUBMED till June 2, 2024 for cases of intussusception with appendicitis in pediatrics age group with Search strategy (“appendicitis”[Title/Abstract]) AND (“intussusception”[Title/Abstract]) AND (“pediatric”[Title/Abstract]). ( Table 1 ). Table 1 Patients' clinical characteristics. Table 1 s.n. Author Year Age(M: month, Y: year) Duration (days) Preoperative examination USG findings AR findings CT findings Initial treatment final treatment Diagnosis of intussusception made via 1 Chang et.al [ 11 ] 2011 30 M 0.5 AR, USG, CT 1st USG: target sign, 2nd USG: appendicolith Distended bowel loops and a paucity of rectal gas Appendicolith, multiple loculated fluid collections PR Lap app USG 2 Kee et.al [ 8 ] 2015 38 M 2 AR, CT Na Mild generalized ileus II with diffuse thickened wall of the appendix trapped within Na Open app, MR CT 3 Aybay et.al [ 12 ] 2016 8 Y 14 USG and CT 1st USG: target sign, 2nd USG: appendix invaginating into the cecum Na Target sign on the cecal level HR App CT 4 Lauren Marjon [ 6 ] 2018 1 Y 1 USG, air enema radiograph Ileocolic intussusception along with Multiple lymph nodes and fat and nonvisualization of appendix Paucity of bowel gas Na PR Lap app, MR USG 5 Tameshwar K Algu [ 13 ] 2020 8 M 3 USG Sausage-shaped mass and free fluid in the abdominal cavity Na Na Na RLH with a primary end-to-end anastomosis USG 6 Cocomazzi et.al [ 4 ] 2021 27 M 3 USG, Barium enema Typical target sign with mild fluid collection and reactive lymph nodes around the invaginated segment Ileocolic intussusception at transverse colon Na HR Lap app, MR Barium enema 7 Lee et al. [ 14 ] 2021 1st case: 35 M 2 AR, USG, CT Multicentric, bowel-in-bowel mass appearance Oval-shaped haziness and localized bowel gas II with diffuse thickened wall of the appendix trapped within 3 PR Open app, MR AR 2nd case: 35 M 1 AR, CT Na Paucity of bowel gas Bowel in bowel appearance PR No surgery CT 8 Hurtado et.al [ 15 ] 2022 11 Y 7 CT Na Na Dilated fluid-filled appendix with uncomplicated appendicitis Na Lap app with partial cecectomy CT 9 Samantha et al. [ 16 ] 2022 25 M 14 USG 1st time: ileocolic intussusception, 2nd time: intussusception comprised of inverted appendix Na Na 3 times PR Lap app, MR USG 10 Kimura et al. [ 7 ] 2022 17 Y 2 CT, Colonoscopy Na Na Target sign, swollen appendix and appendicolith Na Lap ileocecal resection CT and colonoscopy AR: Abdominal radiography, USG: ultrasound, CT: Computed Tomography, HR: Hydroreduction, PR: Pneumoreduction, Lap: laparoscopic, app: appendectomy, MR: manual reduction, RLH: Right limited hemicolectomy. Patients' clinical characteristics. AR: Abdominal radiography, USG: ultrasound, CT: Computed Tomography, HR: Hydroreduction, PR: Pneumoreduction, Lap: laparoscopic, app: appendectomy, MR: manual reduction, RLH: Right limited hemicolectomy. We found 10 studies published between 2011 and 2022, reporting 11 cases of patients with mean age of 5 years (range 8 months to 17 years), 7(63.63 %) were male ( Table 1 ). All the studies were case report. The most common presenting symptoms were abdominal pain (72.7 %) followed by vomiting (54.5 %) and fever (18.18 %). The average duration of symptoms was 4.5 days (range 12 h to 2 weeks). The most common physical examination finding was tenderness of abdomen (81 %) followed by hypoactive bowel sound, guarding and abdominal distension each comprising 18.18 %. 5 cases (45.4 %) didn't response to the initial treatment (hydroreduction and/or pneumoreduction). In 8(72.7 %) cases the diagnosis of appendicitis was made prior to the surgery. All cases except 1 underwent Surgery (Appendectomy 72.7 %, 9 % ileocecal resection, 9 % right limited hemicolectomy). Appendiceal intussusception is believed to result from abnormal peristalsis caused by intraluminal or intramural irritants in the appendix. These irritants include fecoliths, foreign bodies, lymphoid hyperplasia, parasite in children and endometriosis implants, and appendiceal neoplasms such as mucoceles, carcinoid tumors, adenocarcinoma and metastasis in adults. Intussusception and acute appendicitis often present with similar symptoms, including vomiting, abdominal pain, and irritability [ 13 ]. There are some characteristic symptoms helpful for differentiation between them; young age (<4 years), intermittent colicky periumbilical pain, currant jelly-like stool, target sign in USG favors intussusception while symptoms like migratory right lower quadrant pain favors acute appendicitis. In many cases, if one condition is suspected, then the other may be overlooked as in our case initially [ 8 ]. However, in cases of intussusception not responding to initial management secondary causes should be looked for. The classic radiographic finding of appendiceal intussusception is a finger-like intraluminal filling defect in the cecum accompanied by the absence of filling in the appendix [ 9 ]. In our case the diagnosis of intussusception was primarily based on clinical presentation and confirmed by USG, which revealed the characteristic bowel-in-bowel loop. We did not perform CT scan in our case due to typical clinical and USG findings of intussusception and assuming most cases of ileocolic intussusception in young infants are not associated with a leading point [ 8 ]. Thus, in our case the underlying cause of the intussusception, appendicitis, was not identified until the exploratory laparotomy which was later confirmed histologically. This scenario is similar to prior case studies [ 4 , 6 , 13 ] which discovered appendicitis incidentally during surgery for intussusception. This emphasizes the necessity of keeping a broad differential diagnosis and considering secondary causes in intussusception when initial management strategies, such as hydroreduction, fails. Hydroreduction and/or Pneumoreduction was performed in 6 cases (54.54 %) as initial management out of which 3 cases (50 %) were successful. Appendectomy was performed in 72.7 % of the cases [ 4 , 6 , 8 , 11 , 12 , [15] , [16] , [17] ]. In our case, we started with hydroreduction initially but since it was unsuccessful, we performed appendectomy.

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Pediatric intussusception is the leading cause of bowel obstruction in children under 2 years of age, with an estimated incidence of 1–4 cases per 1000 live births [ 1 ]. Intussusception with lead points in pediatric cases ranges from 2 % to 12 %, increasing to 57 % in patients older than four. In children, most cases are idiopathic and of the ileocolic type. Treatment often involves an air or contrast enema, which is successful in approximately 80 %–90 % of cases [ 2 , 3 ]. Acute appendicitis is the leading cause of emergency abdominal surgery in children, with a peak incidence of 25 cases per 10,000 between the ages of 10 and 17 years. However, it is relatively uncommon between 1 and 4 years of age [ 4 ]. Concurrent intussusception and appendicitis “appendi-sception” is an unusual condition, with an estimated incidence of 0.01 % [ 5 , 6 ]. This condition can affect individuals of any age and present with a variety of manifestations, ranging from an asymptomatic, acute abdomen to chronic symptoms [ 5 ]. Most reported cases have been in children, with a variable clinical presentation [ 7 , 8 ]. Because the clinical and radiographic findings can resemble those of more common diseases, the diagnosis is difficult to make preoperatively [ 9 ]. Adhering to the SCARE 2023 guidelines, we report a child with ileocolic intussusception with failed attempts of hydroreduction, eventually necessitating surgical intervention during which he was found to have concomitant appendicitis [ 10 ].

A 37-month-old boy presented to the emergency department with intermittent and colicky periumbilical abdominal pain that began 2 weeks ago, which was associated with drawing up his legs during episodes and passage of red currant jelly stool. He experienced multiple episodes of non-projectile, non-bilious vomiting for 3 days. He also had intermittent constipation for 2 weeks. The child was ill-looking with signs of dehydration. Vital signs were stable. The abdomen was soft, non-distended with mild tenderness in the periumbilical region and with presence of bowel sounds. No palpable mass was observed. Rectal examination revealed the presence of bloody jelly-like stool mixed with mucus. Rest of the systemic examinations were normal. Laboratory tests revealed anemia with hemoglobin of 8.4 g/dL (normal range: 11.5–15.5 g/dL). Abdominal Radiograph revealed dilated bowel loops suggestive of intestinal obstruction ( Fig. 1 ). Fig. 1 Abdominal X Ray (supine) showing dilated bowel suggestive of obstruction. Fig. 1 Abdominal X Ray (supine) showing dilated bowel suggestive of obstruction. Ultrasonography (USG) of the abdomen revealed a 2.5 cm × 2.5 cm heteroechoic lesion in the sub hepatic region with a bowel-in-bowel loop, a characteristic finding in intussusception ( Fig. 2 ). Fig. 2 Ultrasound of abdomen (Axial view) with multiple concentric hyperechoic and hypoechoic rings-target sign. Fig. 2 Ultrasound of abdomen (Axial view) with multiple concentric hyperechoic and hypoechoic rings-target sign. Given the clinical presentation and ultrasound findings, a clear diagnosis of intussusception was made. Hydro-reduction was attempted twice, with a 6-h interval between the attempts. Each attempt involved the use of a contrast enema under fluoroscopic guidance. Despite these efforts, the intussusception was completely non-reducible. Subsequently, the patient was taken for surgical intervention. During the exploratory laparotomy, an ileocolic intussusception was confirmed. On manual reduction of intussusception, inflamed appendix without perforation was found ( Fig. 3 ) (See supplementary video 1 for the operative procedure demonstrating intussusception along with appendicitis). An appendectomy was performed. Postoperatively, intravenous fluids, cefuroxime, metronidazole were continued. Perioperative course was uneventful and he was discharged on the fourth postoperative day. Histopathological examination confirmed the presence of suppurative appendicitis and he had no abdominal complaints on follow-up at one year. Fig. 3 Resected specimen of appendix. Fig. 3 Resected specimen of appendix.

No conflicts of interest were identified in the preparation of this case report.