Pediatric Rectal Prolapse

One classification of rectal prolapse divides the entity into true prolapse (protrusion of all layers of the rectum) and procidentia (herniation of only the mucosa). However, this classification is confusing and nonspecific and therefore, fallen out of use.

The most used classification for rectal prolapse was described in 1971 by Altemeier et al, who divided the entity into the following types[4] :

Type I Protrusion of redundant mucosa, termed false prolapse; usually associated with hemorrhoids

Type II Intussusception without sliding hernia of the cul-de-sac; it occupies the rectal ampulla but does not continue through the anal canal; the most common symptom is fecal incontinence, but solitary ulcers in the anterior rectal mucosa can be seen

Type III Complete, full-thickness rectal wall prolapse, associated with a sliding hernia of the Douglas pouch. It is the most frequent type.

Types II and III can be further subdivided into three degrees[5] :

First degree prolapse includes the mucocutaneous junction. The length of the protrusion from the anal verge usually is greater than 5cm.

Second degree prolapse occurs without involvement of the mucocutaneous junction. The length of the protrusion from the anal verge usually is between 2 and 5 cm.

Third degree prolapse is internal concealed or occult, and does not pass through the anal verge.

For the particular case of rectal prolapse occurring after the surgical correction of an anorectal malformation,[6]  the entity can also be classified as:

Minimal, when the rectal mucosa was visible at the anal verge with Valsalva manoeuvre

Moderate, when there was a protrusion of the rectal mucosa inferior to 5 mm without the Valsalva manoeuvre; and 

Evident, when the mucosal protrusion was greater than 5 mm without the Valsalva manoeuvre

The entity can also be categorized as circumferential,when it affects the rectum for 360o and hemi-circumferential, when the rectum is affected for less than 180o.[6]  Most patients (77%) with rectal prolapse presenting after anorectoplasty can be successfully managed with conservative treatment.[6]

The anal canal extends cephalad from the anal verge to the anorectal ring. The rectum extends from this point to the sacral promontory.

Upon histologic examination, the anal canal consists of mucosa, submucosa, and 2 muscular layers: the internal anal sphincter (IAS), which is a continuation of the circular muscle of the rectum, and [3] the external anal sphincter (EAS), which lies outside the IAS as an elliptic cylinder, continuous with the puborectalis muscle superiorly. The surgical anal canal includes this entire muscular sphincter mechanism (see the images below).

The many and varied procedures described for the treatment of rectal prolapse attempt to create a fixation of the anorectal mucosa to the submucosa, or the rectal wall to perirectal tissues.

Children are predisposed to rectal prolapse due to anatomic considerations: A pelvic floor defect with levator ani muscle diastasis and a deep endopelvic fascia represent the pathophysiology of the disease. Patients with rectal prolapse have lost the normal semi-horizontal rectal position; they also have weak muscle insertions to the pelvic walls and sacrum, an abnormally deep Douglas pouch and Houston’s valves absence in approximately 75% of infants younger than 1 year of age.[7]  A redundant rectosigmoid and weaker / wider anal sphincter are common.

The normal resting tone of the anal sphincter decreases in response to rectal distention. In 1962, Porter found that patients with rectal prolapse have a profound and lengthy response and weakened tone of the levator ani muscles.[8]  Whether this is a causative factor or a secondary finding is not clear, since the prolapse begins above the pelvic musculature. Most recently, information has suggested that rectal prolapse can be developed as a result of circumferential intussusception of the upper rectum and rectosigmoid colon.[7]

Rectal prolapse has been associated with a myriad of conditions, including:

• Increased intraabdominal pressure due to straining (as often occurs in toilet training and constipation)
• Parasitic disease (the most common cause of rectal prolapse in developing countries)
• Neoplastic disease
• Malnutrition (loss of ischiorectal fat pad) Worldwide, this is possibly the most common condition associated with pediatric rectal prolapse; the loss of ischiorectal fat reduces perirectal support
• Ulcerative colitis
• Ehlers-Danlos syndrome
• Meningomyelocele
• Pertussis
• Surgical repair of anorectal malformation
• Fecal incontinence and diarrhea ^{[9, 10]}
• Chronic constipation  ^[11]
• Neuromuscular disorders
• Mental challenge  ^[12]
• Poor sacral root innervation (Spina bifida)
• Bladder or cloacal exstrophy  ^[13]
• Scleroderma  ^[14]
• Hirschsprung disease (especially in ultrashort aganglionic segment, which acts as subocclusion, favoring prolapse)  ^[15]
• Rectal polyps (the polyp acts as a leading point)  ^[16]
• Cystic fibrosis  ^[17]
• Shigellosis in neonates  ^[1]
• Diarrheal disease (Infection by organisms that increases bowel motility)  ^[7]

Cystic fibrosis (CF) deserves special attention as it may cause rectal prolapse in children. In the past, rectal prolapse was described in up to 20% of individuals with cystic fibrosis. However, current reports estimate an incidence of 3% of children with the disease.[18, 19]  Potential mechanisms include bulky bowel movements, coughing paroxysms, and undernutrition. It is most frequently seen in toddlers, but it can occur at any age (triggered by cough). Clinical clues to cystic fibrosis include oily, malodorous, or floating stools; poor growth; wheezing or other respiratory symptoms; and digital clubbing. The absence of respiratory symptoms and normal findings upon physical examination do not necessarily exclude this diagnostic possibility. Sweat chloride test should be performed in order to rule out cystic fibrosis.

Most cases of childhood rectal prolapse occur in children younger than 4 years, with the highest incidence during the first year of life. Anatomic considerations related to this early presentation include the vertical course of the rectum along the straight surface of the sacrum, a relatively low position of the rectum in relation to other pelvic organs, increased mobility of the sigmoid colon, relative lack of support by the levator ani muscle, loose attachment of the rectal mucosa to the underlying muscularis, and absence of Houston valves, seen in about 75% of infants.

Several predisposing factors have been identified, chronic constipation and straining being most common (52%). Other causes include diarrhea (15%),[9]  rectal parasites, [20]  malnutrition,[7]  neuromuscular and pelvic nerve disorders, myelomeningocele, bladder and cloacal exstrophy, Hirschsprung disease, behavioral and psychological disorders,[21]  high anorectal malformations, [22]  cystic fibrosis, chronic respiratory infections and cough,[23]  lymphoid hyperplasia, rectal polyps, and shigellosis.[24]  Rectal prolapse has also been described in a case of Clostridium difficile –associated pseudomembranous colitis in a child.[22]

Hill et al. evaluated 39 patients for associated behavioral/psychiatric disorders. Of these, they identified 21 (54%) children with one or more behavioral or psychological disorders (ADHD, anxiety disorder, depression, encopresis/stool withholding). They concluded there’s a high rate (over 50%) incidence of behavioral and psychological disorders in older (3-18 years) children presenting with rectal prolapse. However, there is scant published literature identifying a high rate of BPD in prolapsed children.[25]

Broden and Snellman demonstrated by cineradiography, that the entity implies a circumferential intussusception of the rectum, with its origin three inches above the anal margin.[26]

In adults, rectal prolapse is six times more common in females than in males. 75% of patients have history of constipation, which stretches the pelvic floor and the anal sphincter mechanism, predisposing them to the disease.

In children, incidence is higher during the first year of life, after which it becomes increasingly infrequent. It is slightly more common in boys than in girls and usually occurs between infancy and 4 years of age.

Rentea and St. Peter, proposed the following risk factors for rectal prolapse [7] :

• Chronic constipation                                28%
• Neurologic or anatomic conditions           24%
• Diarrheal disease                                     20%
• No underlying cause                                17%
• Cystic Fibrosis                                         11%

As mentioned before, the cystic fibrosis group deserves special interest. An earlier review of CF revealed that 23% of patients with CF experienced rectal prolapse and that 78% of these patients experiencing rectal prolapse before the diagnosis of CF. This led to the recommendation that CF should be considered in a child with rectal prolapse of unknown etiology.[1]

Most cases reduce spontaneously. Otherwise, venous stasis, edema, and ulceration ensure. Longstanding or frequent recurrent prolapse episodes lead to proctitis.

Approximately 10% of patients who experience rectal prolapse as children continue to be symptomatic into adulthood. Over 90% of children who prolapsed during the first 3 years of life, respond to conservative treatment by age 6. It is important to achieve this early because the more episodes of rectal prolapse, especially, those cases that do not reduce spontaneously or have difficult reduction, demonstrate a lesser response to conservative management.[27]  Spontaneous resolution is much less likely in children who develop their first episode of prolapse after age 4 years. After a surgical rectopexy, continence is achieved in 92% of patients. Resective procedures demonstrate lower recurrence rates.

Recovery of continence after surgery is not immediate, it may take up to a year.

Nwako et al reported a 100% success rate with the Lockhart-Mummery procedure, which involves packing the presacral space with gauze through a posterior approach, with excision of the prolapsed mucosa.[24]

Hight et al recommend linear cauterization of the rectal mucosa, with a 98% success rate in 72 patients.[28]

 

Rectal prolapse presents as a red ring of rectal mucosa protruding through the anus after straining (see the image below). It is often associated with tenesmus and mucus or bloodstained clothing. Constipation is present in 25-50% of individuals; up to 75% of patients have fecal incontinence.

Sarmast et al reported prolapse of a mass (96%), bleeding after defecation (36.6%), diarrhea (23.9%), prolapsed rectum (14.1%), and constipation (6%) as the most common signs and symptoms in their cohort.[29]

Prolapse initially occurs with defecation and straining, but as the pelvic floor musculature becomes laxer, it can recur with the mildest straining, in upright position, or even spontaneously at rest. Most cases reduce spontaneously; however, the parents (or patient) may need to manually reduce the prolapsed bowel.

History of neonatal stooling problems or cases of cystic fibrosis in family members should be sought. The clinician should ask about excessive straining due to constipation or diarrhea (most common), prolonged toilet sitting with hips and knees flexed, and operative correction of imperforate anus. Inquire about history of the following:

• Surgical correction of anorectal malformation (ARM)
• Ehlers-Danlos Syndrome
• Hirschsprung disease
• Congenital megacolon
• Polyps
• Pneumonia
• Pertussis
• Malnutrition/anorexia
• Myelomeningocele
• Parasitic infection
• Rectal neoplasm and rectal duplication cyst have also been reported  ^{[30, 26]}  

Patients with rectal prolapse frequently have an associated dysfunction, either fecal incontinence (50%) or constipation (15-65%). Excessive pushing during defecation induces mucosal injury of the anterior rectal wall, which may lead to a solitary rectal ulcer [31] .

Rectal prolapse is not uncommon after the surgical correction of anorectal malformations (27%) [6] , being more frequent in patients with "high" ARM´s [32] . These authors also found that the severity of the malformation, muscle quality, associated vertebral or spinal anomaly, and postoperative constipation, were factors associated with the development of rectal prolapse[32] .

Frequently, physical examination findings are normal. Parents provide a history of a dark or bright red mass protruding from the child’s anus, although the child appears to be pain free or in minimal discomfort.

Because most prolapses spontaneously reduce before arrival for evaluation, a brief examination of the patient in a sitting or squatting position and observation for recurrence of prolapse is recommended. (Other positions, such as jackknife or left lateral decubitus are frequently inadequate to reproduce the prolapse). Images taken by the caretaker during an acute episode aid in confirming the diagnosis.

The prolapsed rectum is a pouting, swollen rosette. In false or mucosal (partial) prolapse, the mucosa shows radial folds at the anal junction, and it is usually quite small, whereas a full thickness or complete prolapse has circular folds (see the images below). If the prolapse is present upon examination, feeling the prolapsed mucosa between fingers allows the examiner to distinguish between mucosal and full thickness rectal prolapse.

A prolapsed rectal polyp appears as a plum-colored mass that does not involve the entire anal circumference. Digital examination can also distinguish prolapse from rectal intussusception. In the case of prolapsed intussusception, a finger can be passed into the space between the anal wall and the protruding mass. With rectal prolapse, inserting a finger into this space is not possible. The triad of abnormal perineal descent, enterocele, and recto-rectal intussusception which progresses to recto-anal intussusception before becoming a full thickness rectal prolapse, is not necessarily seen in all patients, but must always be considered prior to surgical intervention [31] .

Complications of rectal prolapse include:

• Incarceration: Entrapment of the prolapsed intestine making it irreducible; it may lead to strangulation of the prolapsed segment.
• Strangulation and gangrene: When the prolapse is not reduced in a timely fashion, the resulting edema further precludes its reduction; the impaired blood flow to the mucosa endangers the viability of the prolapsed segment, mandating emergency resection.
• Ulceration and hemorrhage: Trauma over the exposed mucosa produces ulceration, bleeding, and mucous discharge; occurs in about 12% of patients; treatment involves correction of straining and defecation habits.
• Prolapse rupture: Excoriation of the mucosa can perforate the prolapsed intestine; urgent surgery is indicated
• Incontinence: Observation for 6-12 months is appropriate because it is likely to resolve spontaneously.
•  Cancer risk: No clear correlation between colorectal tumors and rectal prolapse has been established; however, a study in adults demonstrated a 4.2 fold increase in the relative risk for rectal cancer in patients with rectal prolapse  ^[33]

The primary care physician should initially approach rectal prolapse as a symptom rather than a specific disease entity and should always search for an underlying disorder. Anatomic causes such as Hirschsprung disease and history of imperforate anus repair should be sought. Inquire about a history of constipation, diarrhea, parasitic infections, polyps, or anal stenosis.

High-resolution ultrasonography and magnetic resonance imaging (MRI) provide excellent depiction of the pelvic anatomy and are helpful to illustrate functional changes. Contrast enema, proctosigmoidoscopy, video defecography, anal manometry, electromyography, and anal endosonography may also be useful.

Sweat chloride test

Since the potentially disastrous consequences of missing the diagnosis of cystic fibrosis, and because of the improved prognosis associated with early diagnosis and institution of treatment, the sweat chloride test is indicated in all patients who present with rectal prolapse without an underlying anatomic abnormality. In the era of newborn screening for cystic fibrosis, 3.6% of patients with rectal prolapse had cystic fibrosis, and conversely, 3.5%, of patients with cystic fibrosis have rectal prolapse. Earlier studies quoted a higher incidence of rectal prolapse in children with cystic fibrosis, often occurring in approximately 20% of cases, usually between 6 months and 3 years of age [1] .

Stool evaluation for ova and parasites

Rectal prolapse has been associated with Escherichia coli 0157:H7 infection; antibiotic-associated colitis; Entamoeba histolytica infection; and Giardia, Salmonella, Shigella, and Trichuris infection. Consider a workup for other associated illnesses in the appropriate setting as clinically indicated.

Dynamic MRI provides a high resolution, real time integrated structural assessment on morphological and functional display of the pelvic anatomy [35, 36] . The first reports of MRI in the study of gastrointestinal tract were reported in the late 1980’s and early 1990’s, which assessed gastric emptying [35] . For children, the advantages of dynamic MRI over other modalities are that it is non-invasive, without exposure to ionizing radiation, provides excellent soft tissue resolution, and allows visualization of the anterior, middle and posterior pelvic compartments. Limitations include the supine positioning and evaluation of defecation under non-physiological conditions[35, 36] .

MRI can be used both for a multicompartmental dynamic assessment of the pelvic floor, which is referred to as dynamic MRI defecography [37] , and for the anatomic analysis of the internal anal sphincter (IAS) and external anal sphincter (EAS), which is referred to as static MRI[38] . The dynamic sequence consist of rest, squeeze, straining (pre rectal filling), and defecation (post rectal filling) [39] .

Considering MRD versus fluoroscopy, MRD has a better detection rate for structural abnormalities than fluoroscopy [40] . According to previous studies, fluoroscopic defecography would have been more accurate at identifying enterocele and internal prolapse [36] .

It must be emphasized, that etiology and natural history of an internal prolapse, especially rectal intussusception, requires special preoperative imaging assessment [36] . However, there is no evidence to demonstrate that the dMRI abnormalities shown for adults can be interpreted in the same way for children [35] . Actually, there is no significant advantage of MRD when attempting to detect abnormalities of the anterior or middle compartments, compared to clinical examination. Dvorkin et al. recommended the use of MRD when planning surgery; similarly, Attenberg et al. concluded that MRD did have an impact on treatment strategy decision-making. Kaufman et al. found that MRD had been particularly useful when used in the context of follow-up for patients after pelvic floor reconstruction. Finally, Ramage et. al concluded that MRD with a full evacuatory phase remains as a valid study, forming part of the diagnostic work-up in the management of multi-compartment pelvic floor dysfunction. As well, Li, Jiang, Peng, and Yang, suggest that MR defecography is an excellent tool to better understand the complex anatomy and function of the pelvic floor, aiding the surgeon in preoperative planning and selecting the surgical procedure of choice, specifically in case of multi-compartment problems[39, 40] .

Sigmoid intussusception rarely presents as rectal prolapse in pediatric patients. In 1990, Ashcraft et al highlighted the importance of preoperative diagnosis for prevention of inappropriate initial treatment and postoperative recurrence.[41] In their series of 46 patients, two children required subsequent sigmoid resection. Preoperative contrast enema with a defecating view revealed a coiled-spring appearance typical of sigmoid intussusception. It is also useful in identifying polyps or other leading points.

Defecography or video defecography is a dynamic radiologic test performed with contrast during voluntary evacuation of the rectum to asses the anorectal function at rest and during defecation [37] . Video defecography of the contrast-filled rectum during defecation can be used to identify rectal prolapse or intussusception, or to disclose significant pathology (enterocele, rectocele, sigmoid intussusception) and thereby guide surgical treatment [42, 43, 44] . It also allows understanding the pathophysiology of defecation disorders in children.

The main indication for defecography on Mugie et al. study was long-lasting severe constipation. Other indications on the same study were intractable fecal incontinence and rectal prolapse [37] .

The defecography has the advantage of displaying pathological sequences in a simulated defecation in a conscious patient, being a well-tolerated test. Disadvantages include radiation exposure (0.63 mGy to 2.09 mGy), and a considerable false negative rate on rectal prolapse and related disorders (83.3%).  In younger patients, lack of cooperation is another issue [37] . Koivusalo, Pakarinen, Rintala and Seuri recommended the dynamic defecography in patients 10 years of age or older with atypical symptoms, or if rectal ulcer is suspected. It is considered unnecessary for full thickness rectal prolapse [45, 46, 47] . Whenever MRI defecography is available it should be preferred instead of fluoroscopic defecography due to its lack of ionizing radiation exposure [37] .

Endoscopic evaluation is useful to rule out polyps in patients with recurring rectal prolapse or history of rectal bleeding. It allows for tissue samples and identifies a leading point in the case of intussusception.

Anal manometry

The clinical and cost benefits of routine preoperative anal manometry, pudendal nerve motor latency, and colonic transit are unclear. Anal manometry shows low resting pressure; patients with coexisting fecal incontinence also have low squeezing pressures. However, after surgery, the resting pressure or sphincter length may not change or improve. However, squeeze pressure may improve.[46]

Electromyography

Electromyography has provided insights into the pathogenesis of fecal incontinence; however, it has no place in the clinical workup of this entity, and hardly any research has been done so far. Abnormalities can be found in patients with rectal prolapse, but these results do not predict continence after rectopexy.[46]

Anal endosonography

It may show asymmetry and thickening of the internal anal sphincter and submucosa. Demonstration of a sphincteric defect might be useful if a sphincter reconstruction is being considered.[46]

Patients who present with a prolapsed rectum should undergo prompt manual reduction. If edema is already present, sustained, gentle pressure should be applied to reduce swelling and permit reinsertion of the rectal tissue through the anal orifice. Conservative management is appropriate in selected patients. Treatment should be directed to the underlying cause, since once treated, conservative management is usually successful. Cases of difficult reduction and patients with recurrent episodes are less likely to respond to conservative measures. The benefit of using biofeedback in patients with chronic straining or paradoxical contractions of the anal sphincters is yet to be elucidated. For many years, case series and expert opinions proposed biofeedback as a first choice treatment option for fecal incontinence. Most case series reported positive outcomes in over 70% of patients [48] .

The author recommends a six-month trial of conservative management in all patients under age four. Surgical treatment is reserved for patients with neuromuscular disorders or anatomic malformations. Also, for patients who do not improve with conservative management or patients with complicated rectal prolapse (eg, damage to the rectal tissue by repeated prolapse, recurrence after injection sclerotherapy or, frequent, difficult to reduce prolapses, painful episodes, ulceration, or significant rectal bleeding) [27] .

In patients with cystic fibrosis, initiation of adequate pancreatic enzyme replacement usually results in cessation of rectal prolapse.

Some authors have reported excellent results with injection sclerotherapy [49, 50, 51] .

Internal rectal prolapse (IRP)

IRP refers to a full-thickness intussusception of the rectum during defecation.[45] Radiographically, different grades have been proposed, from low-grade (recto-rectal intussusception) to high-grade (rectoanal intussusception) rectal prolapse. This kind of prolapse may lead to outlet obstruction and/or fecal incontinence. IRP plays an important role in the pathophysiology of obstructive defecation, which refers to the inability to empty the rectum satisfactorily during defecation.

Surgical correction for IRP is possible through a transabdominal or transanal approach. Commonly performed procedures include laparoscopic ventral rectopexy (LVR) and stapled transanal rectal resection (STARR). LVR corrects the intussusception of the rectum and reinforces the rectovaginal septum with a mesh, which suspends the rectum and vaginal vault to the sacral promontory. During the STARR procedure, a stapled resection of the redundant rectal wall is performed. The optimal procedure is difficult to select since no comparative studies exist to date.[42]

When the prolapse is present at the time of examination, reduction should be promptly performed before the onset of edema. Parents should have gloves and lubricant at home, and should be taught how to reduce the prolapse at home promptly.

The prolapsed bowel may be grasped with lubricated gloved fingers and pushed back in with gentle steady pressure. If the bowel has become edematous, firm steady pressure for several minutes may be necessary to reduce the swelling and allow reduction. Digital rectal examination should always follow this procedure to verify complete reduction. If the prolapse immediately recurs, it may be reduced again and the buttocks taped together for several hours.

Prompt reduction is critical. The prolapsed mucosa becomes edematous as a result of lymphatic obstruction. If the process is not reversed at this stage, venous obstruction ensues, aggravating the edema (see the image below) eventually leading to arterial obstruction with subsequent necrosis. Once the bowel becomes necrotic, emergency surgical resection is necessary. An acutely strangulated rectal prolapse should be covered with warm moist towels, and the patient should be brought to the emergency department immediately.

When reduction is difficult due to edema, topical table sugar may be used as an osmotic aid to help decrease the edema and facilitate reduction, thereby obviating emergency surgical intervention.[43, 44] This allows the patient to be treated electively under stable conditions.[44] The sugar exerts a mild osmotic power over the prolapsed mucosa, helping the edema to slowly resolve, which permits a nontraumatic reduction and prevents complications.

Sugar does not irritate the mucosa like other substances (eg, salt). The author has used sugar to reduce edematous prolapsed bowel in 12 patients. The edema resolves by as much as 50% in 30-90 minutes. The entire prolapsed mucosa must be covered with sugar, and the process can be repeated up to 3 times. This also improves the microcirculation in the affected segment, making it more favorable for any surgical procedure.

Conservative management should be the first approach since it may prove useful in over 90% of children. It is aimed at treating the cause and reducing straining. It should be attempted for one year before surgical management is chosen.

In patients with diarrhea and constipation, rectal prolapse usually resolves when the stool pattern returns to normal. Therefore, constipation should be aggressively managed. Constipation is treated with dietary modification (total dose per day is 5 g of fiber plus an additional 1 g for each year of age; dose for adults is 20 g once or twice daily) and stool softeners (eg, polyethylene glycol) to reduce straining, or osmotic laxatives. These have been shown to prevent recurrence. Adequate fluid intake should be ensured.

Infectious diarrhea or parasitic infestation should be appropriately treated.

Further management should focus on parental reassurance and education. Instruction on how to reduce a prolapse may prevent repeated presentations to the emergency department.

The type of toilet that the child uses is also important; use of an adult toilet contributes to rectal prolapse because the buttocks are in a dependent position and the feet are unsupported. Using a special child’s toilet or using a step to support the feet can be a useful adjunct to treatment. In some patients, switching from a “potty” chair to an adult commode may help prevent recurrence. Time spent on the toilet should also be limited to minimize straining.

Biofeedback training can be used to teach children how to tighten and relax their perianal muscles in order to pass bowel movements more efficiently, however, there is no evidence that biofeedback training adds any benefit to conventional treatment in the management of childhood constipation. Must be considered when the child has subtle dyssynergia characteristics during defecography [52, 40] .

Surgery is infrequently required for rectal prolapse. However, if the prolapse persists after an adequate trial of medical therapy, surgical intervention may be required. Age, duration of conservative management, and frequency of recurrence should be taken into consideration. Pain, excoriations, and rectal bleeding are considered indications for surgical treatment.

When the prolapse cannot be reduced or if necrosis is already present, emergency surgical resection is indicated.[46] If a recurrent prolapsed rectum is successfully reduced in the emergency department, surgery is scheduled within the next two weeks to allow the edema to subside before the procedure.

The main purpose of surgical treatment for rectal prolapse is correction of the prolapsed rectum and recovery and prevention of the associated defecation dysfunction. Therefore, when selecting surgical methods, the surgeon should understand the exact causative factors and anatomical variations.[53]

As mentioned before, three out of four patients presenting with rectal prolapse after the surgical correction of an ARM, can be successfully treated with conservative management. However, in such patients, it has been observed that prolapses presenting before colostomy closure are more likely to require surgical repair than children with prolapses diagnosed after colostomy closure (50 vs 16 %)[6] . Thus, the presence of rectal prolapse before colostomy closure seems to be predictive of the need for surgical repair.

The only absolute contraindication for surgery is poor general condition that precludes a major operation.

A great deal of debate surrounds the optimal surgical management of rectal prolapse. Currently, more than 130 operative procedures for the treatment of rectal prolapse are recognized. In 2008, a Cochrane Database Review of studies available in adult literature found that all reparative procedures have similar clinical outcomes with various degrees of risk.[47]

No single surgical procedure is appropriate for all patients with rectal prolapse[48] ; each has disadvantages and carries some risk of recurrence. No clear-cut indication for any procedure is known, and no consensus has been reached on the operation of choice for a particular patient. Personal experience of the surgeon is a major determinant.

Choosing between abdominal and perineal Surgery:

Since recurrence rates after abdominal repair are generally lower, this approach is commonly suggested. It is important to emphasize that the abdominal procedure is suggested for physically fit and stable patients. Concomitant rectopexy also has lower recurrence rates.[47]

Altemeier et al described their perineal approach in 1971. They used anterior closure of the pelvic diaphragm and transanal resection of the prolapsed segment, with primary end-to-end anastomosis.[4]  It is typically performed for rectal prolapse with a length greater than 3-4 cm.

In an Altemeier perineal rectosigmoidectomy, a full-thickness circumferential incision is made in the prolapsed rectum about 1-2 cm from the dentate line (see the image below). The hernia sac is entered, and the prolapse is delivered. The mesentery of the prolapsed bowel is serially ligated until no further redundant bowel can be pulled down. The bowel is transected and either hand-sewn to the distal anal canal or stapled with a circular stapler. Before anastomosis, some surgeons plicate the levator ani muscles anteriorly, which may help improve continence.

Ripstein and Lauter addressed the problem by suspending the rectum via an abdominal approach.[49]

Ashcraft and Holder reported their experience with posterior repair in 46 children over a period of 17 years, with satisfactory resolution in 42 patients.[54] Three of the failures were attributed to sigmoid intussusception. Such outcomes highlight the importance of distinguishing this condition from true rectal prolapse preoperatively.

Surgical treatment can be accomplished either transanally (perineal approach) or transabdominally. In general, transanal approaches have lower morbidity, whereas abdominal approaches have lower recurrence rates. Laparoscopic repair provides rectal fixation equal to that achieved through open procedures, with less morbidity.

Abdominal repairs involve mobilization of the rectum and fixation to the anterior sacral wall, which can be achieved with sutures or with prosthetic material. Most abdominal procedures can be safely and effectively perform either via an open or laparoscopic approach[55] . Fixation with prosthesis carries a higher risk of stenosis and obstruction. A sling prosthesis should be tailored to the individual patient, taking growth into consideration. In general, resection rectopexy has an acceptable recurrence rate (2-8%), but it is associated with the added morbidity of a colorectal anastomosis.

Injection sclerotherapy is a valuable initial procedure. It is well tolerated and efficacious. Success rates range from 80-100%. Sclerotherapy is cheaper and less invasive than surgery. It might be a good alternative in the management of rectal prolapse in patients with HIV/AIDS[50] or other poor surgical candidates.[51]

In spite of this, some pediatric surgeons are reluctant to inject sclerosing agents into a child’s anorectum, out of concern for possible induction of fibrosis and the potential long-term risk for carcinogenesis.

Injection can be done with phenol in oil, isotonic sodium chloride, 50% Dextrose solution, or ethyl alcohol. Longitudinally injecting the sclerosing agent in each of the four quadrants, promotes scarring and adherence of the mucosa to the rectal wall. This, stabilizes the rectum; each of these materials has its advantages and complications. It elicits an inflammatory reaction in the submucosal and perirectal tissues, resulting in fibrosis, with subsequent cessation of the prolapse. It can be performed in an outpatient setting, with no need for bowel preparation.

The patient is placed in the lithotomy or left lateral position under general anesthesia. A 20-gauge spinal needle is introduced through the anal mucosa via a proctoscope or is externally introduced 2-3 cm from the anal margin, with a guiding finger in the anal canal, to a point several centimeters above the dentate line. The sclerosant is circumferentially injected into the submucosal and perirectal space as the needle is withdrawn. To prevent, necrosis, bleeding, or stenosis, care should be taken to avoid injecting the sclerosing agent into the mucosa.

Patients undergoing sclerosant injection are discharged the same day with simple analgesics and stool softeners.

The success and complication rates of such treatments reported in the literature differ for each sclerosing agent. Possible complications include injury to nerves, injury to surrounding tissue, and possible carcinogenic effects.

In Spain, Ibanez et al used fibrin adhesive in patients aged one month to eight years[56] ; they reported no postinjection complications and found that adequate sclerosis was achieved in less than 24 hours.

In Egypt, Fahmy and Ezzelarab treated 130 children with rectal prolapse aged six months to 12 years, who underwent injection with 98% ethyl alcohol (group 1), phenol in almond oil 5% (group 2), or dextranomer and hyaluronic acid injectable gel (Deflux; group 3); they found that submucosal injection resulted in no mortality and varying morbidity.[57] Deflux had the lowest complication rate. Phenol in almond oil 5% had a high complication rate and should not be used. Alcohol is inexpensive and should be considered an alternative to Deflux.

Follow-up in this study was two months to three years.[57] In group 1, the recurrence rate was 11%; two patients had mucosal sloughing, and a girl developed a rectovaginal fistula. In group 2, 18% had abscesses and mucosal sloughing and two developed perianal fistula. In group 3, two patients had immediate postoperative prolapse that spontaneously resolved. No patients had mucosal ulceration or abscess formation, and none had recurrence on follow-up.

In Turkey, Abes and Sarihan used 15% saline solution as sclerosing agent in 16 children with rectal prolapse[58] ; they found that prolapse ceased in 93.7% of the patients after the first injection, only one patient required a second injection, and no complications occurred. The investigators concluded that 15% saline is preferred over other sclerosing agents because of a high cure rate, the safety of the procedure, ease of injection, and lack of complications.

Zganger et al published their 30-year experience in Croatia, using cow milk as a sclerosing agent. Their study included 86 children with rectal prolapse treated with cow milk injection sclerotherapy. Treatment was successful in 95.3% (82 children) of patients. They reported recurrent rectal prolapse in four (4.7%) patients, which subsequently underwent surgical treatment. Seventy-two percent of patients were younger than 4 years of age (62 children), whereas the remaining 24 patients were older (28%). Up to three applications may be needed. For children who needed operative treatment, the Thiersch procedure was performed without complications. They conclude that injection sclerotherapy with cow milk is a simple and effective treatment method for rectal prolapse in children, with minimal complications.[59]

For procedures performed through an abdominal approach or those needing bowel resection, bowel preparation and prophylactic antibiotics should be used. The patient is discharged once bowel function is restored.

Thiersch operation

The Thiersch, or sling, procedure uses synthetic materials to create a perianal sling to support the rectum. It has a success rate of about 90%. This procedure is a good choice for children since it can be done with self-absorbing sutures to provide temporary relief of symptoms until the base pathology is managed. It is considered a palliative procedure since it does not cure the prolapse itself. In adults, it is associated with high recurrence rates.

Perianal sutures are subcutaneously placed as a cerclage (see the image below). The principle is to create a mechanical barrier to contain the prolapse and provoke an inflammatory response on the perirectal tissues to generate a fibrous ring rather than a toneless sphincter.

Modifications involving the use of knitted polypropylene mesh (Marlex mesh) or other nonabsorbing materials have been described (see the image below).[60] Patients should be followed until the wire is removed. Advantages include simplicity, effectiveness, and safety. Disadvantages are poor tolerance, rigidity, breakage, and infection. Infection has always been described as local and superficial, although a case of infection spreading to the scrotum was described by Saleem and Al-Momani.[61]  Chauhan et al described a modification by placing three Thiersch sutures circumferentially along the anal canal in a recurrent case of rectal prolapse.[62]

 

Lockhart-Mummery Operation

Mesh gauze packing is placed temporarily (for 8-10 days) in the retrorectal space to promote adhesions that stabilize the rectum. Success rates are very high (nearly 100%)

Cauterization

In this procedure, the prolapsed rectum is circumferentially cauterized in a linear fashion extending to the submucosa. This produces perirectal inflammation and scarring that prevents further prolapse. The success rate is approximately 80%.[63]

Abdominal rectopexy

Abdominal rectopexy (75% success rate) can be safely performed either laparoscopically or through an open approach. In this procedure, the perirectal tissues are attached to the presacral fascia to assure correct anatomic positioning and tissue adherence. Fixation can be done with direct sutures or with prosthetic material, such as polypropylene mesh (Ripstein procedure; see the first and second images below) or an Ivalon sponge (see the third image below).

This procedure has a high success rate for prolapse control (approximately 75%), and incontinence is improved in 60% of patients, although as many as 60% of patients have postoperative constipation. It might be wise to close the peritoneum over the mesh to isolate it from the abdominal cavity.

Ekehorn rectopexy

Ekehorn rectopexy involves placing a mattress suture in the rectal ampulla from inside the rectum, through the lowest part of the sacrum and out through the skin, where it is tied externally.[64] The suture is left in place for 10 days. This leads to local inflammation, which causes adhesions between the rectal wall and perirectal tissues (sacrorectopexy). The procedure takes only a few minutes and is reportedly 100% effective.[65]

Delorme's procedure

The mucosa and part of the underlying rectal muscle are excised, and the rectum is then plicated with polydioxanone sutures towards the anal canal.

Long-term results are not satisfactory, with a recurrence rate of 17%.[66] This repair has been used in children with recurrent prolapse and has the advantage of not entering the abdomen.[67] The observation that recurrence and complication rates may be lower in younger and medically fit patients suggests that the Delorme repair does not necessarily have to be restricted specifically to older, medically unfit patients.[68]

Transanal Endorectal Approach with modified Delorme's Procedure

This technique involves a Delorme’s procedure modified by longitudinally suturing the muscular cuff to complete the plication. De la Torre et. al concluded the modified Delorme’s procedure was effective for the treatment of idiopathic rectal prolapse with no recurrences or complications[67] . Being this a perineal technique, it avoids the risk of nerve injury that exists for transabdominal methods [67] .

Perineal resection

Mikulicz first described perineal resection in 1889.[4] Perineal rectosigmoidectomy with rectopexy, correction of the pelvic floor (plication of the puborectalis muscles), and coloanal anastomosis is promising and could be a good approach for pediatric patients with intractable prolapse and redundant sigmoid. It has been successfully performed using stapling devices for the resection and reconstruction of colonic continuity.[69] This technique avoids the abdominal approach, with its obvious complications.

Mucosal plication with anal encircling

In mucosal plication with anal encircling, Teflon tape is routed relatively deep outside the external anal sphincter (EAS).[70] Clinical results show a recurrence rate of 0-31%, with no mortality and almost no serious complications.

Levator repair and posterior suspension

Levator repair with posterior suspension is performed via a posterior sagittal approach. Nwako reported a 100% success rate with the Lockhart-Mummery procedure, which involves packing the presacral space with gauze through a posterior approach and excision of the prolapsed mucosa.[24] Hight et al recommend linear rectal cauterization of the anorectal mucosa; they had 98% success in 72 patients.[28]

Closed rectosacropexy

In Egypt, Lasheen described a technique of closed rectosacropexy for management of rectal prolapse in children.[71] The technique simply involves passing several U-shaped sutures into the sacral fascia, through stab incisions made in the skin posterior to the anus, into the sacral fascia, then into the wall of the rectum, down to the anal canal, and out through the stab incisions. The strands of the suture are tied subcutaneously through the stab incisions.

This operation was successfully performed in 42 children (mean age, 3.5 years) who had recurrent rectal prolapse for 3-5 months. None of the children had any further recurrence or specific complications during follow-up of 1-3 years.

Virtually every type of open transabdominal approach for rectal prolapse has been tried laparoscopically. Laparoscopic rectopexy with suture fixation of the rectum to the sacral promontory, with or without segmental colectomy as necessary, using an umbilical camera port and two laterally placed working ports. The recto-sigmoid colon is mobilized and dissected below the peritoneal reflection, after which the mesentery is taken with the Ligasure, and the redundant sigmoid colon removed. The anastomosis can be performed with an EEA stapler. Following anastomosis, the rectum is secured to the presacral fascia with several non- absorbable braided permanent sutures.

Gomes-Ferreira et al[72] , described the laparoscopic modified Orr-Loygue mesh rectopexy is a simple, reproducible, and efficient technique, for the surgical treatment of non-resolving recurrent complete rectal prolapse in children. To avoid postoperative constipation, they state important to perform a tension-free rectopexy, achieved by suspending rather than fixing the redundant rectosigmoid with a mesh.

Some researchers have shown adequate results in laparoscopic repair of rectal prolapse in children, even as an outpatient procedure, thus it promises to become the criterion standard for the management of full thickness rectal prolapse in children. The rate of surgical complications is 0-3%, and the recurrence rate ranges from 0-10%. Regarding complications, recurrence rate, and correction of the associated rectal dysfunction, its effectiveness is comparable to that of an open approach [72, 73, 74, 75, 76, 36] .

Lesser rates of adhesions formation can be expected. It is associated with less postoperative pain and shorter hospital stay with excellent cosmesis[76] .

Current laparoscopic surgical techniques include suture rectopexy, stapled rectopexy, posterior mesh rectopexy, and resection of the sigmoid colon with colorectal anastomosis with or without rectopexy [77, 78, 79] .

According to Kairaluoma et al, the main advantages of a laparoscopic approach are a shortened hospital stay and reduced intraoperative blood loss[74] . The recurrence rate is not increased in the short term. Less postoperative pain, better cosmetic results, faster recovery of bowel function and introduction of diet have also been reported [77, 78, 79] .

Koivusalo et al performed eight laparoscopic sacrorectopexies with good results [80] . They reported two patients with postoperative constipation. It appears that patients have less constipation and incontinence if the lateral rectal ligaments can be preserved; however, this requires further analysis.

D’Hoore and Penninckx described a laparoscopic ventral rectal colpopexy technique for the repair of rectal prolapse and enterocele [78] .

Delaney reported 109 laparoscopic repairs in adults [75] . Hospital length of stay was three days (compared with six days for open surgery), and recurrence rates were 8% for laparoscopic surgery compared with 5% for open surgery. The procedure is described as follows:

1. The presacral space is entered, and the rectum is mobilized

2. A precut mesh is passed down a port and tacked to the sacral promontory in the midline

3. The edges are then sutured to the lateral mesorectal tissue for support

4. In patients having a resection (those with slow intestinal transit and severe constipation), the upper rectum is transected with an endoscopic stapler and pulled out through a small left lower quadrant muscle-splitting incision

5. The resection is completed, and the anvil of a circular stapler is inserted in the proximal bowel before it is returned to the abdominal cavity

6. The anastomosis to the rectal stump is performed before the lateral mesorectal tissue is sutured to the promontory.

Saxena et al successfully treated a 22-month-old girl by using laparoscopic simple suture rectopexy with 5mm instruments and placement of 3-0 nonabsorbable sutures on either side of the rectum to secure it to the presacral fascia [79] . No blood loss occurred, and the procedure was completed without complications. The child was followed up for 24 months, with good results. Similar results were seen in a study by Awad et al that evaluated laparoscopic suture rectopexy on 20 pediatric patients who required laparoscopic suture rectopexy. The study found that the median length of hospital stay was one day and only one patient had full thickness recurrence [76] .

Gomes–Ferreira et al described a modified Orr-Loygue mesh rectopexy technique for recurrent, nonresolving prolapse. The technique involves excision of the Douglas pouch and tension free suspension of the rectum using a nonabsorbable prerectal mesh [72] .

G Stonelake, Gee, McArthur and Jester described for the first time, the use of laparoscopic protack rectopexy in children, adopting the principles of suture rectopexy, with full rectal mobilization from the sacral promontory to the pelvic floor, followed by fixation of the lateral mesorectal peritoneal ligaments to the sacral promontory. On the study, seven consecutive patients with full thickness rectal prolapse underwent laparoscopic Protack rectopexy. The mean age was 9 years, with a male to female ratio of 6:1. Etiologies for rectal prolapse were idiopathic constipation, cystic fibrosis, postoperative anorectal malformation, and constipation in association with autism and learning difficulties. Mean operative time was 82 min. There were no complications and there have been no recurrences reported at a median follow-up of 17 months (range 10-38 months). The immediate complication reported was pain, and in this pediatric series they added gabapentin for postoperative pain control [81] .

According to Stonelake et al, in the Protack rectopexy approach, dissection of mesorectum likely promotes adequate adhesions to be formed whilst the tacks successfully hold the rectum in place. In addition, the technique always involves division of the lateral peritoneal ligaments leaving enough peritoneum to tack it to the sacral promontory, which seems to be a factor in reducing recurrence rates, and avoids mesh-associated complications[81] .

Laparoscopic ventral mesh rectopexy (LVMR) shows good functional results, reduced postoperative constipation and low rates of morbidity and recurrence after LVMR. Robot-assisted technology (RVMR) is an alternative to conventional laparoscopy in rectopexy due to its technical advantages for the surgeon and potential benefits to the patient [36] .

The operation is performed according to the technique described by D’Hoore and Penninckx with patients placed in the deep Trendelenburg position using side docking with five trocar placements:

1. Cannula is placed at the umbilicus
2. Three ports are placed as described by D’Hoore and Penninckx: a 12 mm port in the right lower quadrant, a 5 mm port in the left lower quadrant and a 5 mm port in the right lateral abdominal wall 
3. The additional trocar was placed suprapubically to optimize the angle for a secure mesh fixation with tackers to the sacral promontory  ^[78]

Mean operation time was 125 +/- 27 min vs. 131 +- 25 min (p=0.52). Console time was 96 +/- 23 min during RVMR. Length of hospital stay was 2.2 +/- 1.5 and 2.5 +- 0.9 days in the RVMR and LVMR groups respectively (p=0.71). VAS pain score at two weeks was 2.9 +/- 1.8 in the RVMR group, and 2.7 +/- 1.4 in the LVMR group (p=0.69).

The results on Mäkelä-Kaikkonen et al study are generally consistent with previous reports showing that robot-assisted rectopexy can be performed safely, effectively and with short term outcomes similar to those achieved by laparoscopic procedures. It has also been demonstrated that the posterior compartment defects, external and internal rectal prolapses and rectoenteroceles were effectively corrected by ventral rectopexy at MR defecography [36] .

In current series, the complication rate was low (6%) and in accordance with other similar studies. However, we should take into consideration the surgeon’s expertise, also, MR defecography showed that both VMR and LVMR straighten the prolapsing and invaginating rectal wall, diminish rectocele and obliterate any associated enterocele. In the series, RVMR showed a trend toward less postoperative residual rectocele both in terms of amount and size than the LVMR, but the difference was not statistically significant [36] .

Radiofrequency ablation and plication

Gupta reported promising results with radiofrequency ablation and plication in the treatment of rectal prolapse, on the basis of a short operation time, a shorter hospitalization, less postoperative pain, faster wound healing, and a relatively low complication rate (9%, compared with a 23% complication rate for ligature and excision).[82] This procedure seems to be safe and effective.

Diathermy

Diathermy has also been used to treat mucosal prolapse; however, it is reported to be quite painful.[83] Further studies are needed.

Acupuncture

Some reports exist that describe successful management of rectal prolapse with acupuncture.[84]

Behavioral and/or physical therapy

A study highlighted the relationship between rectal prolapse in older children and behavioral/psychiatric disorders (BPD). The study reported that patients who had enrolled in a program of behavioral and/or physical therapy had reductions in frequency and severity of prolapse after initiating pelvic floor strengthening, behavior modification, and biofeedback.[21]

Complications of surgical treatment of rectal prolapse include the following:

• Postoperative pain

• Bleeding from the injection site

• Perirectal abscess formation

• Potential for damage to bladder neck or presacral nerve plexus

All of these are rare.

For sclerotherapy, some children may have 1 or 2 further episodes of prolapse in the days immediately after the injection before resolution. As many as 12% of patients require a second injection, and as many as 8% need 3 injections. Failure rates approach 15%.

Surgical consultation is recommended in patients who meet any of the following criteria:

• Recurrent prolapse with mucosal ulceration

• Failure to reduce the prolapse despite adequate sedation

• Recurrent rectal prolapse associated with severe pain and discomfort despite intensive medical treatment

• Failure of conservative management

• Full-thickness rectal prolapse in patients with myelomeningocele, exstrophy of the bladder, and postoperative changes after pull-through operations for imperforate anus and Hirschsprung disease

The prognosis for pediatric patients with rectal prolapse depends on the underlying etiology and should be approached as a symptom rather than as a disease on itself.

Evaluate the child for cystic fibrosis. Evaluate whether or not the child has exhibited signs of constipation or diarrhea. If sweat chloride test results are negative and the bowel regimen has been normal, consider proctosigmoidoscopy to rule out rectal polyps or lesions. Instruct parents how to manually reduce a prolapse and to seek immediate help from a healthcare provider if reduction fails.

There is no specific medical treatment for rectal prolapse, however, management of constipation, treatment of associated parasitic infections, and avoiding excessive strain are appropriate measures for the management of uncomplicated rectal prolapse.

Pancreatic enzyme replacement may be useful on CF patients with rectal prolapse with a 72% efficacy [1] .

Class Summary

In addition to dietary modification, stool softeners help to decrease bowel movement straining secondary to constipation.

Polyethylene glycol solution (MiraLAX, Dulcolax Balance)

Polyethylene glycol is an osmotic stool softener used for treatment of occasional constipation. In theory, there is less risk of dehydration or electrolyte imbalance with isotonic polyethylene glycol than with hypertonic sugar solutions. A laxative effect is generated because polyethylene glycol is not absorbed and continues to hold water by osmotic action through the small bowel and colon, resulting in mechanical cleansing.

Polyethylene glycol is supplied with a measuring cap marked to contain 17 g of laxative powder when filled to the indicated line. It may require 2-4 days (48-96 hours) to produce bowel movement.

Mineral oil (Fleet Mineral Oil Enema, Kondremul)

Mineral oil lubricates the intestine and facilitates the passage of stool by decreasing water absorption from the intestine.

Lactulose (Enulose, Generlac, Kristalose, Constulose)

Lactulose is an osmotic agent and ammonium detoxifying agent. It produces an osmotic effect in the colon that results in distention and promotes peristalsis.