During this study, we demonstrated that 95% (37 of 39 patients) of patients with HD successfully underwent SSPT surgery following our preoperative management of HD. Cases that could be managed without performing emergent enterostomy during the neonatal period were managed by irrigation until radical surgery was performed. Although some cases of TCA required enterostomy, parents could easily perform preoperative management of patients with HD, allowing for SSPT surgery for cases including long-segment HD and TCA; hence, this method should be selected first for HD cases.
Pull-through surgery is the standard surgical method for HD cases, although there is no consensus regarding the superior HD treatment method. However, we adopted the single-stage laparoscopic trans-anal pull-through modified Swenson procedure for short-segment and long-segment HD  and laparoscopic restorative proctocolectomy with an ileal-J-pouch anal canal anastomosis for TCA and extensive aganglionosis . No specific advantages of radical surgery during the immediate neonatal period have been demonstrated [7, 10, 14]. Furthermore, radical surgery is usually performed electively within 2 to 3 months if the condition of the infant is stable and the bowel is decompressed [12, 15]. SSPT surgery is associated with significantly lower readmission and re-operation rates than multi-stage pull-through surgery ; furthermore, the patients in the multi-stage surgery group had worse conditions. The presence of an ileostomy for a long period of time could cause complications such as high output of the stoma and peristomal skin excoriation [14, 16]. The European Paediatric Surgeons’ Association Survey reported that 67% of the members performed delayed pull-through surgery [15, 17]. Regarding delayed pull-through surgery, preoperative management was essential to prevent HAEC and decompress bowel distension until the time of radical surgery.
For short-segment HD, GE use was first selected at our institution owing to its ease of use. A previous study showed that GE use only relieved obstruction in approximately 80% of HD cases [16, 18]. However, only 43% (13 of 30 cases) of short-segment HD cases were successfully managed by GE use only during this study, and 57% (17 of 30 cases) of short-segment HD cases required irrigation by an indwelling tube. Rectal irrigation at home was feasible and effective for HD cases, allowed delayed pull-through surgery [17, 19], and contributed to decreasing perianal excoriation, anastomotic site strictures and leakage, HAEC, and incomplete continence . We also adopted home rectal irrigation via the insertion of an indwelling tube each time irrigation was performed as previously described because it had been reported that rectal irrigation could effectively decompress the bowel in approximately 75% of HD cases [10, 14]. However, long-segment HD and further extended agangolionosis cases were not suitable for rectal irrigation [17, 19] because an indwelling tube could not be effective unless the tip of the tube was placed in the dilated bowel. Only short-segment HD cases were successfully managed using irrigation with an indwelling tube inserted each time irrigation was performed during this study. Moreover, some short-segment HD cases additionally required irrigation using an indwelling tube. If an indwelling tube was deeply inserted in the dilated bowel, then this irrigation technique could contribute to bowel decompression. However, blinded deep insertion and irrigation could cause perforation  and are very dangerous procedures.
The novel point of this study is that preoperative management allowed for SSPT surgery, even for TCA cases. The descriptions of our preoperative management protocol and fixation of the tube could aid in their application by other institutions. Although rectal irrigation at home was feasible and effective for patients with short-segment HD, it was not suitable for patients with long-segment HD or further extedend aganglionosis. Bowel distension that could not be decompressed by rectal irrigation required enterostomy. Enterostomy could be complicated by electrolyte disturbances, stoma prolapse, or peristomal skin excoriation . However, we consider enterostomy too invasive for managing long-segment HD. Indwelling tube irrigation effectively decompressed the dilated bowel of patients with HD, including long-segment HD and TCA. During this study, 100% (four of four patients) of long-segment HD patients and 60% (three of five patients) of TCA patients who underwent tube placement for irrigation were successfully managed, thus allowing for SSPT surgery. Two cases of TCA were unresponsive to indwelling tube irrigation and required ileostomy. It was only natural that irrigation using an indwelling tube placed in the bowel was unable to effectively wash-out the terminal ileum. Hence, cases of aganglionosis extending to the terminal ileum were theoretically unresponsive to total colon irrigation.
The effect of irrigation on long-segment HD and TCA should be considered. All long-segment HD cases were successfully managed by irrigation, and preoperative HAEC occurred only in one out of four patients. Theoretically, tube irrigation decompressed bowel distension until the ascending colon. Unlike long-segment HD, whether the irrigation tube in the ascending colon can decompress bowel distention with TCA is questionable. During this study, three cases were successfully managed by irrigation, whereas the other two sustained abdominal distension despite irrigation and required ileostomy. Two cases of aganglionosis of the entire colon and 38 cm and 20 cm of the terminal ileum were unresponsive to irrigation, suggesting that the irrigation tube in the ascending colon was unable to decompress the terminal ileum more than 20 cm from ileocecal valve. Three cases of aganglionosis of the entire colon and ≤ 5 cm of the terminal ileum was successfully managed by irrigation, meaning that aganglionosis until approximately 5 cm of the terminal ileum could be managed by irrigation. However, slight distension of the abdomen continued in these cases before surgery, and parents appropriately performed irrigation repeatedly when they noticed abdominal distention. Although preoperative HAEC only occurred in one patient, successful irrigation management for TCA depends on the aganglionic distance from the ileocecal valve and the parents’ efforts; therefore, irrigation management is not necessarily effective for all TCA cases.