Gastroparesis is a frequent expression of CIPOS. Bowel decompression by a gastrostomy is often required. Repeated acute episodes of bowel obstruction and chronic intestinal distension require bowel decompression by using nasogastric suction. The placement of a venting gastrostomy is of great benefit in avoiding the recurrent placement of nasogastric tubes. When surgery is required, a gastrostomy may be performed during the same surgical procedure. If a gastrostomy is not surgically placed, percutaneous endoscopic gastrostomy tube (GT) placement is easily achieved in these children. Since enteral feeding should always be preferred to using parenteral nutrition (PN), intragastric administration of feeding may be achieved by the GT as continuous or bolus enteral tube feeding. Gastric decompression by repeated and even permanent gastric drainage is not always sufficient. Surgical procedure such as pylorotomy or gastroenterostomy may be discussed with caution according to the risk of overloading a poorly motile intestine without any clear functional benefit and clinical improvement.

In neonates and young infants, intestinal obstruction may last several weeks requiring total parenteral nutrition (TPN) with subsequent complications including catheter-related sepsis and liver disease [4]. Enterostomy may offer the chance to restart intestinal transit allowing feeding and reducing the need for PN.

In some patients, attacks of intestinal obstruction are frequent and/or life threatening. Chronic bowel dilatation impairs intestinal motility creating a vicious circle which increases intraluminal bacterial overgrowth with the subsequent risk of intestinal translocation, enterotoxin release, and liver disease [41, 42]. Enterostomy should be performed to bypass the functional obstruction and obtain digestive decompression.

The location of the enterostomy is a matter of debate. In cases of obvious megacystis microcolon syndrome, a terminal ileostomy is certainly required. Otherwise, we do recommend performing a terminal ileostomy and avoiding a colostomy whatever the clinical presentation or histopathologic pattern. It is important to consider the so-called ileocecal brake as the segment that should be short-circuited. In our experience, all patients who first underwent a colostomy went on to have formation of a terminal ileostomy or jejunostomy. In some case, a venting ileostomy using the Santulli or Bishop-Koop procedure may be attempted with the aim of maintaining colonic function and avoiding diversion colitis (DC) (see below).

The outcome after ileostomy or jejunostomy varies according to the location of the enterostomy and to the disease itself. The literature does not provide any evidence of a histopathology-related prognosis even if the survey reported by Henyeke et al. suggested worse prognosis of myopathies and that they all need ileostomies [32]. However, much fewer than 50 % of patients improve after ileostomy by being weaned from PN. In our opinion, enterostomy, as distal as possible, is the most logical approach. Terminal ileostomy usually enables transit to resume and leads to a major long-term reduction in obstructive episodes. We currently perform an ileostomy to obtain durable intestinal autonomy and PN weaning, with the future plan to do a total or subtotal colectomy with ileorectal or ileosigmoid pull-through [33].

A paper by Irtan et al. reported stomal prolapse in children with chronic intestinal pseudo-obstruction as a frequent complication [43]. Twenty-two out of 34 (65 %) CIPOS children referred to their center between 1988 and 2008 had a stoma and were compared with 22 other children referred for another pathology necessitating a stoma. The incidence of stomal prolapse in CIPOS children was 45 % vs. 9 % in non-CIPOS children (p = 0.01). Prolapse occurred between the first postoperative day and the tenth postoperative month, with a median of 2 months. Surgical management was required in 60 %, with an intestinal necrosis rate of 20 % leading to intestinal resection. The authors did not identify particular risk factors favoring stomal prolapse.

Percutaneous endoscopic cecostomy or colostomy (PEC ) is increasingly proposed as an alternative to surgery to treat CIPOS and relapsing sigmoid volvulus [44–47]. Cecostomies or even sigmoidostomies have been used to administer antegrade enemas when intractable constipation appears to be the prominent symptom. A few reports are available both in children and adults describing the indications, complications, and outcomes. A retrospective, single-center study involving eight adults was reported by Lynch et al. [46]. Six patients had CIPOS and two had chronic constipation. The use in seven of eight cases resulted in clinical improvement with reduction of intestinal obstruction episodes and improved feed tolerance. One patient suffering chronic constipation required surgical removal of the percutaneous endoscopic cecostomy tube at 4 days for fecal spillage resulting in peritonitis despite successful tube placement. Removal of the cecostomy tube occurred in three of six cases of pseudo-obstruction (the other three remain in place). In the other patient with chronic constipation, clinical improvement occurred, but the patient died of underlying illness 21 days after placement. A case of acute stercoral peritonitis was reported [47]. At laparotomy, the colostomy flange was embedded in the abdominal wall, but no pressure necrosis was found at the level of the colonic wall. This complication was likely related to inadvertent traction of the colostomy tube. Percutaneous endoscopic cecostomy is considered by some authors as a viable alternative to surgically or fluoroscopically placed cecostomy in a select group of patients with recurrent colonic pseudo-obstruction or chronic intractable constipation.

In children whom a decompression ileostomy has produced relief, but there is diffuse disease, the urge to reestablish connection with the defunctioned limb of the bowel should be resisted as this will only result in further episodes of obstruction. In other words, performing an ileostomy and closing it because of clinical improvement results in the patient undergoing two surgical procedures without resolution of the primary issues. This should be avoided. Conversely, in patients in which clear improvement from ileostomy is observed, with PN weaning and at least 2 years follow-up on enteral tube feeding or oral feeding without exacerbations, total colectomy and ileorectal anastomosis with the Duhamel procedure may be considered. In our experience, two thirds of the patients who underwent this procedure remain off PN for a long period of time [33].

In the past, many patients underwent multiple surgical procedures. Unnecessary abdominal surgery in children with CIPOS should be avoided because they bear the risk of prolonged postoperative ileus and developing adhesions, creating a diagnostic problem each time there is a new obstructive episode. Mechanical obstruction should be considered in patients with an enterostomy who continue to present with exacerbations of bowel obstruction. In an earlier study involving only seven patients, surgery was performed as a treatment 21 times with a mean of three procedures per patient [30]. This is similar to other data reported. In one study, 67 surgical procedures were performed in 22 patients [8], and in another study involving 105 pediatric infants and children, 71 patients underwent surgery during their illness, with 217 surgical procedures [31]. An ostomy was the most performed procedure. Surgery may cause adhesions, so interpretations of postoperative obstructive episodes are difficult. Exploratory laparotomy for obstruction should be performed only when a clear mechanical obstruction has been demonstrated which remains very difficult to assess. Signs of peritonitis, extreme dilatation, and pain in association with specific episodes of obstruction point more toward mechanical rather than functional obstruction, and a laparotomy may be required to relieve it.

Patients with CIPOS or chronic intractable constipation (CIC ) may develop anatomical obstruction such as colonic volvulus, with presenting symptoms mimicking those of underlying pseudo-obstruction. Patient records of eight children with colonic volvulus were retrospectively reviewed [48]. The mean age at presentation with colonic volvulus was 13.2 ± 5.05 years. All patients presented with worsening of abdominal distension and pain. The mean duration of symptoms of colonic volvulus before seeking medical help was 4.2 days (range 1–7 days). Water-soluble contrast enema was the single most useful investigation for confirming the diagnosis. All patients required surgery. There was no mortality associated with colonic volvulus. Clinicians should be vigilant and include volvulus in the differential diagnosis of the acute onset of abdominal distension and pain in patients with CIPOS and CIC. Delay in diagnosis can result in bowel ischemia and perforation.

Some patients, in whom there is segmental bowel dilatation but no evidence of mechanical obstruction, have been reported to benefit from segmental resections or to have improved following placement of a jejunostomy tube within the dilated loop [49, 50]. In our experience, the use of this jejunostomy button device for daily intermittent bowel decompression can effectively improve bowel function allowing decreased PN intake. However, one should consider the quality of life (QOL) of a child with three tubes and, for most of the time, a central line (Fig. 50.2).

Patients suffering from CIPOS clearly benefit from home parenteral nutrition (HPN ) to maintain adequate nutritional status and general health [51]. However, permanent and severe intestinal dysmotility can seriously disturb the QOL to the point of making it intolerable [52, 53]. Subtotal enterectomy [54, 55] and bilateral thoracoscopic splanchnicectomy have been proposed in severe CIPOS [56]. A retrospective study of eight patients with end-stage CIPOS maintained on HPN and suffering from chronic occlusive symptoms refractory to medical treatment underwent extensive small bowel resection preserving less than 70 cm of total small bowel and less than 20 cm of ileum [55]. The jejunum was anastomosed either to the ileum or to the colon. Six patients were completely relieved from obstructive symptoms. Two patients needed a second operation to remove the residual ileum because of recurrent symptoms. Both were significantly improved and there was no postoperative death. All patients experienced a significant improvement in their QOL. Near-total small bowel resection appears to be a safe and effective procedure in end-stage CIPOS patients, refractory to optimal medical treatment.

The implantation of gastric or intestinal pacemakers aimed at improving motility constitutes a promising investigational approach in patients with severe motility disorders. The use of gastric electrical stimulation has been shown to significantly improve nausea and vomiting not only in patients with diabetic gastroparesis but more recently also in three adult patients with familial and one with postsurgical CIPOS with disabling nausea and vomiting [57]. The weekly vomiting frequency decreased from 24 before implantation of the gastric pacemaker to 6.9 after 12 months. The clinical response was unrelated to the presence of, or improvement in, delayed gastric emptying in these patients. Although placements of the electrotrodes along the anterolateral surface of the stomach was successful in most patients by laparoscopic implantation, the procedure was not without risk since the electrodes caused ileus necessitating explantation and short intestinal resection [57, 58].

Diversion colitis (DC) is the inflammation of the excluded segment of the colon in patients undergoing ostomy. Long-lasting terminal ileostomy for CIPOS may result in DC [59]. We already experienced this rare complication requiring, most of the time, a total colectomy. DC may be an indolent inflammatory nidus and a potential cause for repeated bacteremia, abdominal pain, and bleeding. DC has been reported in 11 of 14 children, 4 months to 7 years after surgical diversion of the colon for CIPOS [60]. The children had complained of diffuse, poorly localized abdominal pain and a history of bloody stools in three children. Both colonoscopy and biopsies showed a nonspecific acute and chronic inflammation and/or nodular lymphoid hyperplasia. Authors failed to show any correlation between the duration of the colonic diversion and the severity of the colitis. Usual histology of the bypassed segment is characterized by diffuse follicular lymphoid hyperplasia; lamina propria expansion by plasma cells, lymphocytes, and some neutrophils; cryptitis; reactive epithelium; and mucin depletion. Crypt abscesses, aphthous ulcers, mild architectural distortion, and Paneth cell metaplasia may be noted in the most severe cases [61]. DC may be an inevitable consequence of colonocyte nutrient deficiency and may be superimposed by a second insult, such as a low-grade pathogen. Treatment modalities usually include surveillance for paucisymptomatic patients, restoration of bowel continuity for severely symptomatic cases, and the use of short-chain fatty acid (SCFA) enemas in selected cases. It is suggested that a change in colonic microbiota may lead to colitis; however, direct evidence for this disease progression is poorly established. A study involved 48 patients: 26 DC patients and 22 in the control group [62]. Differences were observed between the two groups in the levels of Staphylococcus (p = 0.038), Enterococcus (p < 0.001), Klebsiella (p < 0.001), Pseudomonas (p = 0.015), Lactobacillus (p = 0.038), the presence of anaerobes (p = 0.019), and Bifidobacterium (p < 0.001). A significant correlation between the severity of colitis and bacterial composition was only observed for Bifidobacterium (p = 0.005, correlation coefficient = −0.531). In case of CIPOS, according to the risk of infection, and the need of permanent ostomy, total colectomy is, in general, required. Otherwise, Santulli or Bishop-Koop procedure may be attempted with the aim of restoring partial colonic function and resuming DC.