The Ileoileal intussusception due to a tubular duplication in a child: A case report

Abstract Intussusception is a surgical emergency that may result in the perforation of the intestinal wall if not immediately treated. Pathologic lead points, such as intestinal duplication, are present in 2.2%–15% of the cases.We describe a 4‐year‐old girl with a necrotic ileoileal intussusception diagnosed with a rare tubular ileal duplication.


| INTRODUCTION
Intussusception is one of the most common causes of intestinal obstruction. It occurs when a portion of the gastrointestinal tract gets telescoped into another. It can occur in children aged 6 months to 2 years, and the most common type is idiopathic ileocolic intussusception. [1][2][3][4][5][6] It formerly had significant mortality and morbidity rates, but with advancements in diagnosis and efficient treatment, a good outcome was achieved. 2 Ischemia and perforation of the intestinal wall are probable in cases where treatment is delayed, with an unfavorable prognosis. 3 A triad of vomiting, a palpable abdominal mass, and the passage of currant-jelly stools are the typical signs that raise clinical suspicion of intussusception. 3 Pathologic lead points such as Meckel's diverticulum, intestinal duplication, benign polyps, malignant lymphoma, Peutz-Jeghers syndrome, and hamartoma are present in 2.2%-15% of cases. [7][8][9] The preferred therapy for noncomplicated intussusception is radiological reduction, which has a 10%-60% failure rate and necessitates surgery in failed situations. 3 Small intestinal duplication is a rare congenital malformation characterized by a tubular or cystic structure that shares its muscular wall and blood supply with the adjacent intestine, primarily the ileum. [10][11][12][13] This study describes a 4-year-old girl with a complicated ileoileal intussusception caused by a tubular ileal duplication.

| History
A four-year-old Afghan refugee girl was admitted to the pediatric ward with acute abdominal pain as her main complaint and no prior medical or surgical history. The symptoms started as intermittent abdominal pain (every 60 min with a duration of 20 min) in the periumbilical and hypogastric areas 3 days ago, accompanied by two episodes of bilious vomiting (last night and 1 hour before admission) and one episode of currant-jelly stool defecation with no sign of hematochezia. Her complaints were temporarily relieved by taking anticholinergics and antiemetics as self-treatment.

| Physical examination
In general appearance, she was an ill child, and physical examination revealed an abdominal distention with a palpable tender mass in the periumbilical area.
Ultrasonography confirmed an ileoileal invagination as a doughnut sign with obstructive findings, free abdominopelvic fluid, and no vascular flow in the invaginated loop in Doppler investigations.

| Treatment plan
We performed urgent surgery following fluid resuscitation and the administration of preoperative broad-spectrum antibiotics due to the patient exhibiting symptoms of peritonitis. The stomach was decompressed using a nasogastric tube while the patient was under general anesthesia. A twisted ileoileal invaginated section was observed at 20 cm from the ileocecal valve during exploratory laparotomy ( Figure 1A). First, we attempted to untwist the volvulus, and then, by milking from the distal area, we released the 20 cm invaginated section. Eventually, we found a 10 cm necrotic area as the small intestine duplication ( Figure 1B). We decided to perform excision and anastomosis surgery due to the total thickness necrosis and the inability to recover the invaginated part's circulation. The patient was discharged after 96 hours without complications or notable events. Histopathological findings confirmed pan-necrosis in the resected specimen ( Figure 2).

| DISCUSSION
Intussusceptions are typically idiopathic, and only 2.2%-15% have pathologic lead points like Meckel's diverticulum (the most common type) or intestinal duplication. [7][8][9] Most pathologic lead points present as ileoileocolic intussusceptions, and generally, signs and symptoms are similar; however, due to the large variety of lesions or intestinal malformations, presentations might vary and are frequently nonspecific. Pathologic lead points should be considered, especially in children over 5 years with multiple recurrences or failure of the enema technique for reduction. 3 We recommended that under suspicious circumstances, a primarily negative ultrasonography investigation should never exclude a second repeat ultrasonography.
Duplications of the small intestine are common in the ileum and can be cystic or tubular. They differ from Meckel's diverticulum in that they are mostly linked to the mesenteric region of the intestine and consist of two parts as follows: an outer layer of smooth muscle and an inner lining of the gastrointestinal epithelium. 2,10,[12][13][14] As many children with intussusceptions caused by pathologic lead points may present with nonclassic symptoms, a specific diagnosis must be determined by diagnostic examination using radiologic imaging. The best options for confirming intussusception caused by a duplication cyst or lymphoma in the hands of an experienced examiner are ultrasonography and reduction enema, but less so for a Meckel's diverticulum. [2][3][4][5][7][8][9] Treatment of a stable patient with intussusception begins with an enema reduction in the emergency department without contraindications such as perforation or shock signs. 3 Our case presented with the classic symptoms of intussusception, and the ultrasonography findings boosted the suspicion. We identified the tubular type of ileal duplication at the operation, which caused ileoileal intussusception. In the literature, we found a few cases of children with ileoileal intussusception secondary to tubular ileal duplication. 5,15

| CONCLUSION
Small intestine duplication should be considered in the differential diagnosis of ileoileocolic or ileoileal intussusception, especially in children over 5 years with failed reduction attempts. Moreover, surgical treatment is imperative for all symptomatic cases.