We provided a more specific classification of ileocecal duplications and found that ileocecal duplications at specific locations were prone to intussusception and influenced the choice of surgical procedure. Moreover, our findings also demonstrate that ileocecal resection did not seem to affect children’s growth and defecation patterns at mid-term follow-up.
Ileocecal duplication is a particular entity, a highly rare enteric duplication that usually presents symptoms or complications in infancy and childhood [12, 13]. Ileocecal duplication was considered more common than cecal duplication, which shared a common blood supply and wall with the ileum for a few centimeters from the ileocecal valve and compressed the cecum . However, we believe that ileocecal duplication could be distinguished from ileum duplication and includes cecal duplication and cases of duplication at the ileocecal junction because all of these cases are located in the ileocecal region, have the same blood supply (the ileocolic artery) and can present with the same symptoms and complications . Some previous studies on other diseases have demonstrated that resection of the ileocecal region has adverse effects [15, 16]. However, the available literature on ileocecal duplication is sparse, and the literature mainly consists of case reports. No controlled study about the different surgical procedures for ileocecal duplication has been reported.
A review of our data shows that similar to other types of enteric duplication, except for the prenatal ultrasonography findings, vomiting, abdominal pain, and abdominal distension were the main symptoms in ileocecal duplication; however, the rates of complications in ileocecal duplication, including intussusception and intestinal obstruction, were significantly higher than the rate in the other types [2, 5, 14]. Moreover, we noted that intussusception occurred only in ileocecal duplications at specific locations, including ileal intraluminal, ileocecal valve, and cecal intraluminal types. Two possible ways can explain this phenomenon. First, according to the classification diagram (Fig. 1, A, C, D), the lesion in the above three types can act as a pathologic lead point for intussusception [17, 18]. In contrast, the other two types cannot. Second, in the ileocecal valve and cecal intraluminal types, bowel thickening may also show the typical target signs of intussusception on ultrasound, which is not a true intussusception [13, 19]. For intestinal obstructions, space-occupying cystic lesions and the compression effects resulting from mucus secretion and accumulation may be the basic pathogenesis, which will affect the normal process of food passing through the ileocecal valve . Similar to a previous study, abdominal ultrasound was performed for all of the patients in our study and had high accuracy for a preoperative diagnosis [9, 21]. Whenever there is a cystic lesion in the right lower abdomen on ultrasound in a case of intussusception or intestinal obstruction in a child, the first differential diagnosis should be enteric duplication, including ileum duplication, which is the most common, or ileocecal duplication [22, 23].
Notably, the location of the cystic lesions in ileocecal duplication will affect the choice of the surgical procedure; the proportions of patients who had cyst excision without ileocecal resection performed in the ileal extraluminal, ileal intraluminal, and cecal extraluminal types were 91.7%, 78.0%, and 100.0%, respectively, which were significantly higher than those in the patients with the ileocecal valve and cecal intraluminal types. In addition, the choice of surgical procedure also depends on the surgeon’s experience and any complications that develop, such as intestinal necrosis . In our study, 40% of the patients were admitted to our center as an emergency, suggesting that the condition of these children was severe, and the patients needed to have surgery performed as soon as possible. When the children’s conditions are complicated with intussusception for a long time, intestinal necrosis may occur, further leading to shock or even death . Some surgeons preferred to choose the relatively simple ileocecal resection and ileocolostomy to prevent complications, such as delayed intestinal perforation .
With the improvement in the perioperative management and surgical techniques, especially when considering that ileocecal duplication is a benign congenital malformation, an increasing number of studies have described excision of duplication cysts in order to preserve the ileocecal region with laparoscopy or laparoscopic assistance [6, 9, 27]. Comparing the perioperative clinical data between the patients undergoing the two different surgical procedures in our center in the past 12 years, we noted that although repair of the bowel or ileocecal valve was sometimes needed during the cyst excision, compared with the traditional ileocecal resection and ileocolostomy procedures, these procedures did not significantly increase the operative time or blood loss. Furthermore, these patients recovered more quickly after surgery, manifested by an earlier oral intake and a shorter hospital stay. This technique retains the important function of the ileocecal valve in childhood and is consistent with the concept of enhanced recovery after surgery . We also observed that the mean age of the patients undergoing ileocecal resection and ileocolostomy was significantly younger than those undergoing cyst excision. This observation implies that younger children with ileocecal duplication tend to have more severe symptoms and complications, such as dehydration, and the surgical procedures for these patients tend to be more conservative, even including an ileostomy [8, 23]. Therefore, cyst excision without cecal resection is a safe and feasible option for ileocecal duplication, and ileocecal resection and ileocolostomy are recommended for patients with severe complications, including dehydration, intestinal perforation, and a prolonged intussusception.
Furthermore, in our study, ileocecal resection did not increase the incidence of postoperative complications or affect the growth of children with ileocecal duplication. At the same time, in contrast to previous literature, a high proportion of the patients did not have postoperative diarrhea . The patients undergoing ileocecal resection and ileocolostomy had a higher incidence of dry stool with a mean follow-up time of 8.4 (0.6–10.8) years. This finding might be related to the fact that ileocecal resection without an extensive ileal resection did not affect bile acid absorption [15, 30]. Moreover, we hypothesized that an ileocecal valve resection could lead to colon bacterial overgrowth and colonization of the ileum, thereby increasing the ability of the digestive tract to absorb water, resulting in dry stool at mid-term follow-up [16, 31].
This study was a retrospective analysis of a single institution and still had limitations. The primary limitation was the retrospective nature of the design. The short-term postoperative defecation patterns in the patients undergoing cecal resection and ileocolostomy could not be acquired and compared with other studies. In addition, our study did not compare the follow-up results with normal children, so it was impossible to know whether cecal resection affects the patients’ long-term postoperative defecation, which is the focus of our future study. Finally, we will test the intestinal flora of the patients enrolled in our study to confirm our hypothesis.
Authors’ contributions JY and YC conception and design of the work. JY and WL conducted data collection and performed data analysis. JY, WL, JY, CD, and TL contributed to data interpretation. JY, WL, and YC assisted in drafting the manuscript. All authors critically revised the manuscript and read and approved the final manuscript for submission.