ABBREVIATIONS
CSP
cold snare polypectomy
EBD
endoscopic balloon dilation
EIT
electroincision therapy
FAP
familial adenomatous polyposis
HSP
hot snare polypectomy
IBD
inflammatory bowel disease
IPAA
ileal pouch-anal anastomosis
NEC
necrotizing enterocolitis
PSARP
posterior sagittal anorectoplasty
QoL
quality of life
UC
ulcerative colitis
INTRODUCTION
Children are in a continual state of change. Their physiology evolves, their capacity for recovery is great, and they are full of potential. In contrast to adults, the diseases that afflict infants and children have a greater dependency on their age and stage of growth and development. The conditions and underlying pathophysiology that affect children under 1 year of age can be dramatically different from children older than 5 to 6 years of age. Paired with this constant mode of change is the unperturbed protoplasm of the neonate. While fetal physiology plays a crucial role in the health and disease of the neonate, the ramifications of genetic contributions begin to be realized at birth during the transition to extra-uterine life. Diseases of the newborn comprise a greater proportion dictated by its genomic architecture and the role of external and environmental influences become increasingly prominent for the rest of its life. Pediatrics, in large part, focuses on this evolution of underpinning genetic components influencing and reacting to environmental factors ( Fig. 41.1 ).
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Gastrointestinal diseases manifest these core principles of pediatric medicine: (1) “the die is cast” in congenital malformations in colorectal disorders and monogenic immune dysregulation, and (2) environment influencing the development and disruption of the mucosal immune system in inflammatory bowel disease (IBD) and necrotizing enterocolitis (NEC).
Growth and Development
Children are full of potential. When we intervene in pediatrics, we must preserve and foster the realization of that potential and avoid limiting it. A critical outcome in children is growth, including statural and skeletal growth, brain growth and cognitive development, and the growth of viscera. Depending on the area of the bowel affected, there is often a great capacity for an increase in intestinal length following surgical intervention. This is especially salient in congenital and neonatal conditions. Unfortunately, longitudinal studies are challenging and uncertainties in growth potential are common. Children tend to be resilient; however, and all approaches in children should account for adaptability and plasticity. To foster this growth, comprehensive nutritional care is a top priority in children with digestive disorders, including dedicated dietitians. As children progress through pubertal development into adolescence and adulthood, reproductive capacity and psychosocial maturation become central. Endoscopic and surgical interventions in IBD and colorectal disease must factor in the possible risks to reproductive health and the impacts on psychosocial development.
Development of Immune System and Microbiome
Our understanding of the role of the gut microbiome in human homeostasis is emerging, and subtle perturbations have been found to influence the development, progression, and resolution of disease. Newborn infants have been believed to be born with mostly sterile intestinal contents with colonization initiated at the time of delivery, although recent evidence demonstrates bacteria in meconium, hypothesized to result from swallowed amniotic fluid during gestation. , After birth, the development of the complexity and richness of gut microbial composition is affected by many factors, including mode of delivery, gestational age, and diet. Vaginal birth exposes infants to the microbes within the birth canal while children that are born by C-section have initial colonization of their gut with more skin-associated bacteria. Preterm infants display divergent microbial communities as compared to healthy full-term infants, and resultant microbial dysbiosis is hypothesized to contribute to increased risk for complications such as NEC and late-onset sepsis. Formula-fed infants have a more rapid maturation of their microbiome toward an adult-like composition while breast-fed infants are dominated by the beneficial Bifidobacteria and fewer microbes that are associated with intestinal inflammation. , Lastly, many other environmental influences such as antibiotic use, prolonged time in the hospital, and medications can also have significant effects on the development of the microbiome.
The intimate coevolution of the microbiome with their mammalian hosts has resulted in an interlocked symbiotic relationship with the microbiota contributing to essential host functions, and any intestinal dysbiosis may interrupt this homeostasis, leading to disease. In NEC, reduced microbial diversity and increased potentially pathogenic microorganisms are common. In IBD, it is very likely that perturbations in codevelopmental pathways with important immune-microbe interactions contribute to pathogenesis. Recently, molecular analyses from the multicenter prospective pediatric Crohn’s disease RISK cohort identified pathogenic pathways involved in mucosal immune system interface with luminal bacteria. , Antibiotics have long been a mainstay in the treatment of intestinal inflammation, and it is likely that the changes in the microbial composition have beneficial effects. Relatedly, early antibiotic use in childhood has a clear association with autoimmune diseases, including IBD. Additionally, the surgically altered bowel may provide insight into the luminal environment that leads to dysbiosis and intestinal inflammation. Stasis may be a relevant factor, with sites of inflammation often proximal to anastomoses: ulcers in Crohn’s disease and pouchitis in ileal pouch-anal anastomosis (IPAA).
Sedation and Anesthesia in Children
Infants and children have unique physiology, distinct risk factors, and pertinent psycho-socio-developmental considerations for perioperative and periprocedural management. For pediatric surgical procedures, anesthesia care should include appropriate staff, equipment, and facilities. This encompasses pediatric anesthesiologists, advanced practice nurses, pediatric nursing, pediatric airway equipment and resuscitation medications and supplies, pediatric postanesthesia care unit, and accessible pediatric intensive care unit. In all of these care contexts, expertise in pediatric advanced life support is paramount. Pediatric airways are smaller and more compliant with an increased risk of obstructive complications from mucous plugging. Additionally, children have a higher incidence of reactive airways and are often in the midst of or recovering from respiratory viral illnesses. Children with congenital heart disease and neuromuscular disorders require special attention and children with developmental disorders are generally at increased risk of anesthesia-related complications. Sedation in children, in contrast to adults, aims to provide not only analgesia and anxiolysis but also behavioral modification to ensure the safe completion of the procedure. The developmental level of the children affects their understanding and ability to cooperate in the context of the procedure. While moderate and deep sedation may be sufficient for endoscopic procedural sedation in adults, children are more prone to progression through deep sedation into general anesthesia due to their variable response to medical agents. Sedation for pediatric endoscopy in children thus requires dedicated expertise and, although in some pediatric hospital systems, endoscopists, hospitalists, or intensivists provide procedural sedation, it appears to be most often provided by pediatric anesthesia. , Interventional endoscopy in children should be exclusively under pediatric anesthesia care.
ENDOSCOPIC MANAGEMENT IN CHILDREN
Endoscopic Polypectomy in Children
Cold snare polypectomy (CSP) has emerged as the preferred technique over hot snare polypectomy (HSP) in adult practice for smaller polyps (diameter 5–7 mm or less). CSP is associated with a reduced risk of bleeding and perforation and has comparable ascertainment of tissue for histopathologic assessment; however, it has not been studied in children. Larger polyps, greater than 5 to 7 mm in diameter, are often resected by snare electrocautery HSP, and this is the most common approach in children, especially in the context of the greater proportion of pedunculated and highly vascular juvenile polyps. With these pedunculated polyps, positioning is crucial, aiming for the polyp to be located in the 5- to 7-o’clock position, where the snare will enter from the instrument channel. Adequate visualization is necessary to ensure that the polyp head is encircled, followed by cinching down around the stalk. In contrast to adults, in which snare close down to the base of the stalk may be indicated to ensure complete resection of adenomatous tissue, in children with juvenile polyps, performing the transection close to the head of the polyp will help limit bowel wall injury and provide a more accessible field in the case of postpolypectomy bleeding. Injection of saline or epinephrine to facilitate lift is an important option and may reduce the risk of bleeding.
Children with more than five polyps in the colon should undergo genetic testing in addition to careful histopathologic examination of all identified polyps. Hereditary polyposis syndromes encompass a spectrum of genetically based phenotypes with oncogenic potential. Diagnosis in children is often either through genetic testing based on a family member’s condition or during the evaluation for rectal bleeding. The underlying genetic defect, which is autosomal dominant in the majority of these conditions, can drive the management, factoring in the known natural history and predicted clinical course. Hereditary polyposis syndromes include familial adenomatous polyposis (FAP), attenuated FAP, Peutz–Jeghers syndrome, juvenile polyposis syndrome, and PTEN hamartoma syndrome. In children with a first-degree relative with adenomatous polyposis or more than five juvenile polyps diagnosed early in life, it is generally accepted that colonoscopic surveillance for adenoma begins at 10 to 12 years of age. The average age of detection of polyp in FAP is 16 years. Once adenomas are detected, the recommendation is to proceed with colectomy, due to the near inevitable progression to colorectal cancer.
Endoscopic Balloon Dilation of Strictures in Children
The most common application of endoscopic balloon dilation (EBD) in children is for esophageal strictures, primarily for peptic injury, esophageal atresia/tracheoesophageal fistula, eosinophilic esophagitis, and caustic ingestions. The use of EBD in pediatric IBD is less common, but the reported use is promising and appears to resemble the safety and efficacy in adults. , The role of EBD in children with colorectal anastomotic strictures remains to be clarified. Given the frequency of recurrent esophageal strictures in children, intralesional steroids and topical mitomycin C have both been used with variable success. , In adults with IBD, intralesional steroids have not impacted the rate or time to recurrent stricture and the limited data in children is equivocal. , In children, EBD should be performed in facilities with ready availability of pediatric and/or colorectal surgery, given the risk of perforation. MR enterography will provide the best opportunity for characterization of the stricture in the ileum and colon prior to endoscopic intervention, although the length is often overestimated. For IPAA and anorectal strictures barium defecography is preferred. Attempting EBD in strictures greater than 5 cm in length or the setting of multiple strictures is not advised in adults or children. Through-the-scope balloon dilation with controlled radial expansion and the use of the rule of three, in which dilation is limited to going up three levels, are guiding practices in pediatrics.
Endoscopic Stricturotomy and Strictureplasty in Children
Endoscopic electroincision therapy (EIT) is emerging in pediatric endoscopy with a scant description of IBD and colorectal disease. EIT for endoscopic stricturotomy and endoscopic strictureplasty (stricturotomy followed by endoscopic clipping) has a demonstrated role in addressing intestinal strictures in IBD in adults. In this technique, an endoscopic needle-knife or insulated tip knife with monopolar electrocautery settings is directed with precision to the fibrotic areas of the stricture to release tension and open the internal lumen. The area of cut and resultant mucosal injury is under the precise control of the endoscopist, providing the opportunity to ensure the cut addresses the most important areas of the stricture and avoid areas of potential complication, such as perforation. For example, in strictures of IPAA, the knife is directed to the posterior wall to avoid complications of the anterior wall, such as rectovaginal fistula. In EBD, the area of mucosal injury and depth of tear depends upon the integrity of the tissue itself, with areas most prone to perforation preferentially affected. This limits the degree to which the endoscopist can address the stricture, with the extent determined by the area most at risk of complication, rather than targeting the most relevant portions of the stricture. Due, in part, to this control, the rate of perforation is lower in EIT compared to EBD in adults with IBD with superior efficacy. However, endoscopic stricturotomy does have a 4% to 10% rate of delayed bleeding. The application of endoscopic clips (to complete strictureplasty) and instillation of D50 solution are used in cases determined to have a higher risk of bleeding.
In children, EIT has shown efficacy in refractory esophageal strictures albeit with a higher perforation rate as compared to EBD, which may be attributable to selection bias. EIT has also been utilized for duodenal strictures from the duodenal web; however, with few reported cases the complication rates, most notably perforation, are unclear. The application of EIT in pediatric IBD and colorectal disease remains limited. In our experience, this has been successfully utilized for IPAA stricture, anastomotic stricture following ileocecal resection in Crohn’s disease, and anastomotic strictures resulting from short bowel syndrome. A crucial aspect of the decision-making process in endoscopic therapy for children is the alternative approach to address the underlying defect. In many circumstances, the risks posed by therapeutic endoscopy, while certainly higher than diagnostic endoscopy, are favorable in comparison to the risks associated with the alternative surgical option. These options must be factored in collectively with transparent multidisciplinary and family discussions. It is imperative to have an exit strategy and prepare for possible complicated results.
Endoscopic Management of GI Bleeding From Endoscopic Intervention
The risk of postendoscopic bleeding requiring medical attention in children is estimated to be 0.1% to 0.5% based on large multicenter cohorts and is more likely to occur following therapeutic procedures, including polypectomy. , The approach to GI bleeding is similar in children as compared to adults with the most relevant difference being the restriction of the working channel to accommodate therapeutic devices. In children weighing <10 kg, in which a neonatal (ultrathin) gastroscope is indicated for upper endoscopy and some colonoscopy, the working channel of 2.0 mm will limit therapeutic devices to injection needle, argon plasma coagulation probe, bipolar probe, and some grasping devices. For children weighing >10 kg the use of a pediatric gastroscope and pediatric colonoscope with working channels >2.8 mm will accommodate the range of therapeutic devices. Prior to endoscopic intervention, it is important to anticipate bleeding as a complication and recognize these possible limitations in pediatric patients. The techniques of injection of hemostatic agents (epinephrine, saline), thermocoagulation, and hemostatic clipping, in addition to banding ligation for variceal bleeding, all apply in pediatric cases. ,
Endoscopic Management of Intestinal Perforation From Endoscopic Intervention
There are several proactive measures for endoscopic procedures with increased perforation risk, and these are especially important in children. As with all interventional procedures, the parents/caregivers of the child need to have a full understanding of the risks involved and the alternate approaches. Carbon dioxide insufflation has become commonplace in pediatric endoscopy and should be utilized during high-risk procedures. If perforation is recognized during an endoscopic procedure, carbon dioxide should be employed, if not already in use, to minimize the effect of intraperitoneal pressure and foster postprocedural absorption. The area must be cleared by suction and limited irrigation to the extent feasible to reduce the risk of peritonitis (or mediastinitis). In children, there are less stringent parameters of bowel preparation adequacy, owing to the challenges of laxative administration in children and the common diagnostic categories. For therapeutic procedures, however, attention to bowel preparation may mitigate the complications of perforation. Colonic perforation can occur during diagnostic endoscopy due to mechanical trauma from forceful advancement and looping. Furthermore, the success of therapeutic interventions by endoscopy depends upon secure positioning. Therefore, careful and experienced advancement of the colonoscope is essential to minimize the risk of perforation in the endoscopic intervention of the lower GI tract. The right colon and second portion of the duodenum are specific areas of thin wall that deserve special attention.
In addition to limiting exacerbating factors of insufflation and cleansing the lumen, the decision to proceed with endoscopic management of perforation should coincide with the experience and preparedness of the endoscopist and supporting team. Prompt surgical consultation is the standard in children. Broad-spectrum antibiotics should be initiated during the procedure. Techniques of through-the-scope clips and over-the-scope clips should be used in accordance with the experience of the endoscopist.
SURGICAL MANAGEMENT IN CHILDREN
Surgical Resection, Diverting Ostomy, and Anastomosis in Children
The pediatric conditions that often require intestinal resection and anastomosis in infants and children include NEC, intestinal atresia, Hirschsprung’s disease, anorectal malformations, IBD, and polyposis syndrome. The indications for each depend on the disease state, but a unifying principle in childhood disease, as compared to adulthood, is the uncertainty of long-term outcomes and the potential for changing conditions with the growth and development of the body and gastrointestinal tract. For example, in severe medically refractory colitis in IBD, great care is undertaken to determine whether the child has Crohn’s disease or ulcerative colitis (UC). Despite best efforts, many children with a diagnosis of UC will later be characterized as Crohn’s disease from inception and the proportion of children with Crohn’s colitis (compared to UC) is higher than in adults. Decision making around surgical intervention for medically refectory IBD colitis must take these factors under consideration that often leads to a higher rate of ileal diversion and staged colectomy, pouch creation, and re-anastomosis. In some respects, it benefits the surgeon to give the disease process time to declare itself in children, to avoid worse complications. Children with a diagnosis of UC who undergo restorative proctocolectomy and IPAA prior toage 8 years of age, who then ultimately reveal Crohn’s disease physiology, are at great risk of complicated fistulizing complications of the pouch. , While the implications of prolonged ileal diversion are relevant, especially for the growing and developing child and adolescent, the benefits of ensuring optimal pouch construction must be considered.
Necrotizing enterocolitis is a pathophysiologic condition primarily of premature neonates that involves infectious, inflammatory, and ischemic contributions leading to bowel damage and sepsis. In this patient population, the related and often overlapping scenario of intestinal perforation may occur, and the decision to proceed with intraperitoneal drainage or laparotomy has long been debated. There may be a benefit in neurological outcomes for laparotomy, but there is also an advantage for future bowel length in those who receive a drain. The care of NEC requires the diligence and experience of the neonatal intensive care unit. Comprehensive management of cardiopulmonary and neurodevelopmental progress and recovery is crucial to ensuring optimal outcomes. In addition, surgical resection of the bowel in premature neonates typically results in small bowel syndrome, a state of intestinal failure in which an insufficient surface area of the bowel is available for adequate nutrient and fluid absorption. This requires long-term parenteral nutrition with a dedicated multidisciplinary team. Long-term complications include intestinal failure associated with liver disease, central line-associated bloodstream infection, and venous thromboembolism. The progression or recurrence of these complications may lead to the indication for intestinal and/or liver transplantation.
With the near-inevitable risk of colorectal cancer in FAP, colectomy is a crucial preventive measure and is often undertaken in the teenage years. The decision to proceed with ileorectal anastomosis and IPAA is based upon rectal and colonic adenoma burden with IPAA favored for rectal adenoma >20 and colonic adenoma >1000. Rarely, proctocolectomy with ileostomy is needed.
Surgical Stricturoplasty in Children
Stricturing behavior in Crohn’s disease is a major source of morbidity and is often refractory to medical interventions. The diagnosis of Crohn’s disease in the younger population also often leads to recurrent need for surgical operations. As such, preservation of bowel and bowel length should be balanced with the need to address the mechanical complications of strictures. This is commonly done with stricturoplasty, with other surgical options including resection and bypass. There are many factors that go into the decision for stricturoplasty, as opposed to resection, but indications include multiple strictures with diffuse involvement of the small bowel, previous extensive resections of the small bowel, duodenal strictures, and strictures at previous anastomoses. Contraindications for stricturoplasty are perforation, malnutrition, and multiple strictures over a short length of bowel.
There are many different types of stricturoplasties described. The most commonly performed stricturoplasty for strictures <10 cm is the Heineke–Mikulicz. This involves making a longitudinal incision through the stricture and then closing the enterotomy in a transverse manner. Longer strictures may require different techniques including Finney or Michelassi stricturoplasties.
Short-term complications from stricturoplasties are generally low; however; studies comparing to bowel resection have shown conflicting long-term results. Resection does seem to offer an advantage to the long-term need for further interventions, however, it does have a higher complication rate. The most common complications from stricturoplasty are re-stricturing or continued mechanical obstruction. Other complications include leaks, abscesses, bleeding, and fistulas.
Prolapse Surgery in Children
Prolapse is a common complication for ostomies that are performed in infants and children. It is more common among those done in the small bowel although can also be seen in colostomies, especially if done using the transverse colon. The extremely mobile mesentery and lack of attachments in children lead to an increased rate of prolapse. In addition, most ostomies are required to be done while the bowel is dilated. As the bowel shrinks down, these stomas are at increased risk of prolapse and parastomal hernias. As the majority of ostomies are temporary, this will often lead to the takedown of the ostomy and re-anastomosis earlier than planned. However, if this is not doable, there have been many different techniques that have been tried. Intestinal dysmotility may also predispose to prolapse in children and preoperative manometric confirmation may inform the surgical approach.
In younger infants and children, the prolapse can often be reduced. In older children, the ostomy can be tacked, revised, or addressed with mesh, although mesh is generally avoided in the younger population. Techniques should begin with prevention and meticulous surgical construction of the ostomy. During the initial construction, attention to the size of the bowel and match to the opening, careful suturing to the fascia and skin, as well as the choice of the segment of the bowel are all important factors.
Ileal Pouch Surgery in Children
Construction of the ileal pouch is beneficial to two primary populations in pediatrics: UC and FAP. Even during the era of biologic therapy, 13% to 14% of children with newly diagnosed UC undergo colectomy within 5 years of diagnosis. Restorative proctocolectomy with IPAA, often in stages, is the preferred surgical intervention for medically refractory pediatric UC. There are two common sequences: a two-stage surgical procedure, with subtotal proctocolectomy and pouch creation and a loop ileostomy to defunctionalize the pouch, followed by a reversal of the ileostomy; and a three-stage procedure, in which a subtotal colectomy precedes a completion proctectomy with pouch creation and loop ileostomy and then reversal of the ileostomy. In children, it is more common to extend the time between these stages.
The maintenance of intestinal continuity without the need for permanent ileostomy improves the quality of life (QoL); however, a variety of structural, inflammatory, or functional conditions of the pouch may occur, including leaks, strictures, fistulae, pouchitis, cuffitis, de novo Crohn-like disease of the pouch, and irritable pouch syndrome. However, our understanding of the incidence and risk factors for these conditions in children is limited. Preoperative cumulative corticosteroid use, , younger age at surgery, and higher clinical severity at initial diagnosis have been associated with the development of pouchitis. The surgical volume of the surgeon and center is likely associated with the risk of procedural complications and may impact the development of pouch disorders. Furthermore, more than half of pediatric colectomies occur in centers performing fewer than two per year. The incidence of pouch disorders and pouch failure in children appears to resemble that in adults; , however, recent studies have suggested that outcomes are actually worse in children. Rates of pouchitis in children range from 31.5% to 67% and the incidence of de novo Crohn-like disease might be higher than in adults.
In FAP, as discussed above, IPAA is performed when adenoma burden is high and outcomes are generally excellent in comparison to IPAA for IBD. The rate of pouch disorders in FAP is lower than in IBD, especially inflammatory conditions. , Use of laparoscopy, compared to open surgery, is increasing in children, and single-stage IPAA at the time of proctocolectomy for FAP may have advantages, but with possible increased risk of anastomotic leak and need for reoperation. In FAP, following proctocolectomy with IPAA, the risk of adenoma remains and endoscopic surveillance of the anal transition zone and pouch is necessary.
The risks of infertility and pregnancy-related outcomes in females and reduced sexual function in men are understudied in children who undergo pouch surgery, and the available research in adults is incomplete. At present, these risks should be under consideration and further study is necessary.
Surgical Approach to Anorectal Malformations and Cloacal Deformities
Anorectal malformations (ARMs) are a congenital disease where the anus is not formed within the anal sphincters. This encompasses a wide range of congenital abnormalities from anal stenosis to a cloaca where the urogenital tract and rectum—all exit through a single orifice. ARMs are usually classified by their location and described as either high, intermediate, or low anomalies. High lesions are those in which the rectum ends above the levator complex and low lesions are below the levator musculature. These lesions may or may not have a fistula to their respective urogenital structures such as the urethra, vagina, or bladder neck. Approximately 50% to 60% of patients have an associated anomaly within the VACTERL association (vertebral, anorectal, cardiac, tracheoesophageal, renal, or limb). These associated abnormalities often determine the timing of surgical repair.
There are two primary pathways of treatment, namely primary repair or delayed repair with colostomy. The determination of which pathway will be undertaken is dependent on a clear delineation of the anatomy. ARMs with “lower” anomalies such as rectoperineal fistula and rectovestibular fistulas can be repaired primarily in the neonatal period. This is done via posterior sagittal anorectoplasty (PSARP) in which the aberrant opening of the anus is transposed into the correct position within the sphincter complex. Most other ARMs will initially be treated with colostomy and PSARP at a later time. This may also be done for a low ARM with a life-threatening VACTERL anomaly such as severe structural cardiac disease. Cloacal abnormalities will also often require vaginostomy at their initial surgery as they often develop hydrocolpos that can obstruct the urinary tract. Again, depending on the distance from the rectum to the anal sphincters, laparoscopy may be required to address the intraabdominal structures or fistulas. Cloacal reconstruction should be done as part of a multidisciplinary team including pediatric urologists and gynecologists depending on the defect. Possible complications include acquired rectovaginal fistula, persistent fistula, recurrent fistula, anorectal mislocation, the remnant of the original fistula, mucosal prolapse, stricture, and dehiscence.
Surgical Approach to Hirschsprung Disease
Hirschsprung’s disease is a primary example of disrupted developmental biology with underlying, albeit incompletely understood, genetic contributions. The absence of ganglion cells in the rectum and, in some cases, more proximal colon is suspected to result from failure of neural crest cell migration, and a genetic mutation in rearranged during transfection (RET) is identifiable in a proportion of children. This leads to impaired peristalsis and intestinal obstruction with colonic dilation and characteristic radiographic findings of a transition zone between the distal aganglionic and proximal normally innervated bowel. Of the surgical techniques used—Duhamel, Soave, and Swenson—all attempt to bridge the normally innervated bowel across the aganglionic section and “pull through” functional bowel to the anal canal. The aganglionic segment is removed in each of these, with a limited defunctionalized portion left in Duhamel and muscular cuff left in Soave. Increasingly these techniques are performed in a single stage without the need for temporary colostomy, and there is generally high success for continent bowel function in the long term.
Early postsurgical complications include bleeding, infection, anastomotic leakage, and injury to surrounding structures. Severe anastomotic leakage will often require abdominal washout and colostomy. Late postsurgical complications include obstruction, fecal incontinence, recurrent or acquired aganglionosis, and recurrent Hirschsprung-associated enterocolitis. Obstruction should be evaluated with an exam under anesthesia, and, as necessary, contrast enema, to assess for anastomotic stricture and specific structural complications (e.g., spur in Duhamel, tight muscular cuff in Soave). Dilation or surgical revision can then be pursued. Structural problems such as twists may also occur. In the absence of an identifiable anatomic etiology, the persistent aganglionic segment must be excluded.
Surgical Management of Intestinal Perforation From Endoscopic Intervention
Perforation is a well-characterized risk of GI endoscopy, and this risk is increased when therapeutic interventions are undertaken ( Fig. 41.2 ). The estimated rates of perforation in children are 0.01% to 0.13% for all endoscopies and 1% for interventional procedures. , Experience in addressing perforation endoscopically in children is limited, and the availability of surgery to assess and perform repair, diversion, and/or resection is crucial, especially in interventional cases. An urgent, life-threatening complication—tension pneumoperitoneum—is of particular importance (see Fig. 41.2 ). In this condition, gas (air, CO 2 ) tracks into the peritoneal cavity leading to hemodynamic compromise. It most often occurs as a result of visceral perforation, although transmural passage of air may also lead to its development. As this air tracks into the peritoneal space, a valve mechanism is suspected to occur in which the omentum permits one-way passage, trapping it within the peritoneum. If enough air builds up, intraabdominal pressure rises and leads to abdominal compartment syndrome, which compresses the IVC and prevents adequate venous return with resultant cardiopulmonary compromise. The resultant clinical presentation will be hypotension, tachycardia, tachypnea, and acutely distended abdomen. With the endoscopist focusing on the intraluminal space, vital sign instability may be the first sign recognized by the anesthesiologist. Recognition and prompt clinical diagnosis are the most important steps to proceed with needle decompression. A Veress needle (or equivalent) is inserted inferior to the umbilicus followed by a gush of air and subsequent improvement in hemodynamic and cardiopulmonary status. Fewer than 20 cases have been reported in the literature and sites at risk of tension pneumoperitoneum include the esophagus, stomach, , duodenum, rectum, and ileal pouch.
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