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35 Chronic Constipation
273
Fig. 35.1 Algorithm for chronic constipation
Initial evaluation would include assess­ment for causes of secondary constipation, including medications, metabolic disorders, neurologic dysfunction, endocrinopathies, and psychiatric disorders. In regards to drug history, it is important to note the temporal relationship between starting a particular drug and the onset of constipation. Rectal bleeding, abdominal pain, inability to pass atus, vomiting, and unexplained weight loss
are warning signs and symptoms that warrant evaluation for malignancy. After a complete assessment of secondary causes, one can bet­ter assess for primary causes of constipation.
B. Physical
A thorough general physical exam may pro­vide some insight into the systemic causes of constipation. An abdominal exam can iden­tify abdominal wall abnormalities, previous surgical scarring, and abdominal tenderness.
274
Rectal examination is very useful in this set­ting since it can be used to provide informa­tion regarding distal anorectal pathologies and assess pelvic oor function.
• The external evaluation of the anus allows the physician to detect anal ssures, hem­orrhoids, cutaneous and mucosal lesions, and pelvic oor weakness. Evidence of pelvic oor descent, rectal prolapse, and anal stenosis can be seen on visual inspec­tion. If rectal prolapse is suspected, the patient can be asked to Valsalva while sit­ting on a commode.
• During the digital rectal examination, the patient is assessed for rectal tenderness, mucosal abnormalities, sphincter func­tion, and gross or occult bleeding via stool guaiac. A quick pelvic oor evaluation can be performed by having the patient Kegel and Valsalva with the examining digit in the anal canal. Rectocele or vagi­nal prolapse can be assessed at this time as well.
• If external visualization and digital exam are insufcient to make a denitive diagnosis, anoscopy can be used to visualize anal canal to evaluate for mucosal lesions, internal hemorrhoids, and anal ssures.
C. Diagnostic Studies
Labs
Basic bloodwork can be used to promptly identify hematologic, endocrine, and meta­bolic disorders. A complete blood count can be used to assess for anemia due to gross or occult rectal blood loss. Thyroid function tests can be ordered if a patient exhibits constipation with signs of hypothyroidism. Electrolyte abnor­malities associated with constipation, such as hypokalemia and hypercalcemia, can be evalu­ated with serum chemistry tests. Laboratory blood tests are important to rule out these sys­temic causes of constipation during the initial evaluation of the patient.
Radiology
Radiographic tests assess the general con­dition of the colon in the setting of constipa­tion by providing a baseline evaluation of the colonic anatomy.
P. I. Denoya and S. K. Abbas
Fig. 35.2 Abdominal plain lms are usually the rst line in imaging to detect signicant stool retention in the colon and areas of distension such as megacolon. It can identify fecal impaction, bowel obstruction, and fecaliths. Plain lms can also be used to monitor bowel cleansing in patients with fecal retention
• Abdominal plain lms are usually the rst line in imaging to detect signicant stool retention in the colon and areas of disten­sion such as megacolon. It can identify fecal impaction, bowel obstruction, and fecaliths. Plain lms can also be used to monitor bowel cleansing in patients with fecal retention (Fig.35.2).
• Barium and gastrogran studies are dynamic studies that provide information to evaluate anatomical causes of constipation. Contrast studies can give insight into distal colorectal conditions that contribute to luminal nar­rowing. Examples include congenital condi­tions, such as Hirschsprung disease, as well as obstructing colon cancer, intermittent volvulus, and colonic strictures.
Endoscopy
Endoscopy is the best initial test for evalua­tion of anatomical abnormalities as a cause of constipation. Available endoscopic modalities to identify colonic occlusions and strictures include rigid or exible sigmoidoscopy and
35 Chronic Constipation
275
colonoscopy. Endoscopic studies are used to evaluate inammatory bowel disease, masses, malignancies, strictures, diverticular disease, and post-surgical anatomic abnormalities. Diagnostic colonoscopies should be priori­tized in patients with constipation and at high risk of colorectal malignancies. These include patients who are over 50years old and have had no precious colon cancer screening, younger patients with a positive family history of colon cancer, and patients who present with alarm features of malignancy.
Colon Transit Studies
Colon transit studies are most useful in evaluating patients with infrequent defecation. Specically, these studies are indicated for patients with chronic constipation in order to differentiate between slow and normal colonic transit after they have failed conservative man­agement with diet changes and laxatives. The following tests measure the colonic transit time which is dened as the time it takes for stool to pass through the colon.
• Radiopaque marker studies measure the colonic transit time by following the passage of radiopaque markers as they travel through the gut as monitored by serial abdominal radiographs. Routinely, the patient will swallow a capsule with 24 markers on day 1 and is followed by abdominal x-ray up to day 5 (after 120 h). The patient should abstain from laxatives during the duration of the test. The test is considered abnormal if there is retention of more than ve markers on day 5. Based on the pattern of marker movement within the colon, patients can have normal colonic movement, slow transit constipation if markers are retained in the right or transverse colon, or outlet obstruc­tion (dyssynergia) as markers progress nor­mally through proximal colon and stagnate in the sigmoid colon and rectum.
• Wireless motility capsule studies are useful for assessing regional transit time (gastric emptying, small bowel transit, colon transit) and whole gut transit time. The patient swal­lows a capsule that is used to measure the pH and pressures of the gut as it travels through
the digestive tract. The patient wears a wire­less receiver until the capsule is expelled. Wireless motility capsule studies are well tol­erated, has good compliance, and avoids the risks of radiation exposures. These studies are more expensive and augment additional ndings of motility dysfunction. Sensitivity and specicity are similar when comparing these two methods.
Motility Studies
Motility studies are done to evaluate defe­catory disorders that involve rectal sensation and compliance, internal and external anal sphincter and puborectalis function, and motil­ity patterns during defecation. They can also be used to assess the response to biofeedback therapy as discussed later in this chapter.
• Endorectal ultrasound is an imaging modal­ity that allows one to assess submucosal lesions, anatomical sphincter defects, and the presence of stulae and deep abscesses in the distal anorectal region. An ultra­sound probe is introduced into the anal canal, providing a circumferential image of the rectal mucosa and perirectal tissues.
• Anorectal manometry is performed by placing a balloon with pressure sensors in the rectum and measuring the intrarectal pressure and external sphincter pressure as the patient attempts to evacuate the manom­eter. Normally, there is an increase in intra­rectal pressure and a decrease in external sphincter pressure during defecation. In patients with dyssynergia, there is a para­doxical increase in external sphincter pres­sure during defecation. Rectal sensation and compliance may also be assessed. The rectoanal inhibitory reex (RAIR) should be tested, to evaluate for possible Hirschsprung disease. RAIR will be abnor­mal in Hirschsprung but also in patients with prior coloanal anastomosis or other surgery involving the anorectal canal.
• Balloon expulsion tests can be used as a simple ofce screening for defecatory dysfunction. In this test, a water lled bal­loon is placed in the rectum to stimulate stool and the patient is told to expel the
276
P. I. Denoya and S. K. Abbas
balloon. The test is normal if the balloon is expelled in less than one minute and is abnormal if it takes more than two minutes to expel the balloon. Balloon expulsion tests should be used in addition to other more rigorous tests of anorectal function.
Defecography
Defecography evaluates for anorectal obstruction due to mechanical lesions and physiologic muscular dysfunction. The test is done by placing barium paste in the patient’s rectum and instructing the patient to bear down while sitting on a radiopaque commode. During evacuation, the barium paste is moni­tored by uoroscopy. The test can be modied to involve MRI in order to evaluate the global pelvic oor anatomy, sphincter morphology, and the dynamic motion during defecation. MRI is more expensive and in most instances is performed in the supine position, which is not as physiologic as a sitting defecogram. However, uoroscopic defecography is becoming less available in many locations and is being replaced by MRI defecography. MRI has the added advantage of evaluating the ante­rior and middle pelvic compartments simulta­neously with the posterior compartment, and avoids radiation exposure.
Rectal Biopsy
Rectal biopsy is done to assess for areas of reduced or absent ganglionic activity within the colon. Although this test is done more often in neonates and infants with delayed meconium passage, it may be done for adults with suspected undiagnosed Hirschsprung dis­ease. In infants and young children, a suction biopsy is performed by a device that applies a suction cup to the wall of the colonic mucosa, then introduces a knife to dissect a sample of the mucosa and submucosa for histological analysis of ganglionic cells. Alternatively, a transanal biopsy without suction may be done in older children and adults. The biopsy should be done about 2 cm proximal to the dentate line to ensure obtaining rectal mucosa and sub­mucosa. A standard colonoscopic biopsy for­ceps should not be used as submucosa is needed for the diagnosis.
Management
D. Medical Management
Initial Management
Initial treatment for constipation in adults involves patient education regarding dietary changes and appropriate bowel habits.
• Patients should increase their ber intake to 20–35g per day. Potential side effects include increased bloating, mild abdomi­nal distension, and increased atulence.
• Patients should also increase their uid intake to the recommended eight glasses of water per day. Furthermore, caffeine drinks and diuretics should be avoided since decreased available water in the body can contribute to the development of constipation.
• In addition, patients are instructed to attempt defecation during periods of increased colonic activity, such as after meals and in the morning.
• Patients with obstructed defecation may benet from placing their feet on a stool or box while defecating on the toilet to raise the knees above the hips to simulate squatting.
Pharmacologic Treatment
Besides lifestyle changes, pharmacologic therapy may be used to augment initial con­servative management by softening stool to facilitate defecation.
• Bulk-forming laxatives are natural or syn-
thetic polysaccharides/cellulose derivatives
that absorb water and increase fecal mass,
increasing the frequency of defecation and
softening the consistency of stools. These
stool softeners must be used on a long-term
basis since it takes time for water to be pas-
sively absorbed into the stool. These
include agents containing psyllium or other
“ber supplements.”
• Surfactants work by lowering the surface
tension of feces, allowing water to be pas-
sively absorbed to soften the stool.
• Osmotic agents are nonabsorbable materi-
als that draws water into the colon via the
osmotic effect. This increases intestinal
35 Chronic Constipation
277
water secretion which softens stool and increases stool frequency. This includes agents containing polyethylene glycol.
• Stimulants act by altering electrolyte trans­port in the intestinal mucosa and by stimu­lating the intestinal motor activity, increasing intestinal secretion and motility. This category includes medications con­taining senna or bisacodyl.
• Lubiprostone is a C1C-2 intestinal epithe­lial chloride channel activator that increases intestinal uid secretion.
• Linaclotide is a guanylate cyclase agonist that increases cGMP, anion and uid secre­tion, and intestinal motor activity, similar in action to stimulant laxatives.
• Misoprostol is a prostaglandin analog that promotes intestinal motility.
The decision regarding a specic laxative depends on patient tolerance and physician pref­erence. Other methods of medical management include suppositories and botulinum toxin injec­tions. These options are usually saved until the constipated patient fails the previously described medical management. Glycerin or bisacodyl sup­positories act by liquefying stool to bypass any defecatory dysfunction. Botulinum toxin is used rarely for patients with pelvic oor dysfunction by injecting a small amount of toxin in the puborectalis muscle, allowing the muscle to relax and relieving the defecatory dysfunction. This method require repeated treatments in order to prevent recurrence of constipation.
Biofeedback Therapy
Patients with dyssynergia can be treated
with biofeedback therapy, which uses behav­ioral training to correct inappropriate pelvic oor muscle and sphincter contraction. Biofeedback therapy uses electromyography or manometry to provide feedback on sphinc­ter muscle function during defecation to allow the patient to adjust pelvic oor muscle contraction. Other techniques include the use of an inatable balloon in order to simulate the passage of stool. The balloon is inated until the patient feels the urge to defecate and the patient attempts to expel the balloon.
Biofeedback therapy helps relieve dyssyner­gic constipation without the use of laxatives.
E. Surgical Management
Manual and Endoscopic Disimpaction
Surgical options are available for patients that fail conservative medical management of constipation. These procedures are mainly for those suffering from slow transit constipation and rectal outlet obstruction. Patients with fecal impaction may undergo disimpaction, which involved manual fragmentation of stool fol­lowed by mineral oil enema for stool softening and colonic lubrication. If the constipation con­tinues despite disimpaction, a water- soluble contrast enema may be indicated to assess for obstructions and proximal impactions. Flexible or rigid sigmoidoscopy can be used to fraction­ate the proximal impactions followed by either warm water enemas or polyethylene glycol solutions for bowel cleansing.
Surgeries for STC
There are several procedures for patients with slow transit constipation.
• The most radical procedure is an abdominal colectomy with ileorectal anastomosis. The entire colon is resected at the proximal rec­tum with an ileorectal anastomosis. Other options include leaving a segment of colon proximal to the rectum such as an ileosig­moid anastomosis, to decrease risk of an anastomotic leak and diarrhea. It is extremely important to rule out pelvic oor dysfunction prior to colectomy, as this may lead to continued constipation, possibly requiring an ileostomy.
• A segmental colectomy can be performed for patients that have a small dysmotile colonic segment, such as adult Hirschs­prung disease.
• In patients who have slow transit consti­pation and are unable to tolerate a colec­tomy can opt to have an antegrade colonic enema. This procedure, done rarely in adults, creates an appendicos­tomy with a valve mechanism to allow for catheterization of the appendix for enema uid.
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P. I. Denoya and S. K. Abbas
Surgeries for Rectal Outlet Obstruction
• Patients with rectal outlet obstruction may suffer from rectal intussusception, recto­cele, or rectal prolapse.
• Rectocele repair is indicated when the rectocele is 3cm or more in depth, defe­cography demonstrates signicant bar­ium trapping, or the patient requires digitation for adequate evacuation. Several options for rectocele repair include transvaginal posterior colorrha­phy, transanal posterior repair, transperi­neal repair, or transabdominal repair.
• Rectopexy is indicated for patients with a complete rectal prolapse. This procedure involves mobilizing the rectum and secur­ing it to the sacrum, therefore reducing the prolapse. Additionally, a sigmoid colon resection may be added in patients who have underlying constipation, but is not necessary in the nonconstipated patient. Pexy may be done with sutures or with mesh. In addition, there are several resec­tion techniques for rectal prolapse that can be offered for elderly patients that cannot tolerate abdominal surgery.
– Mucosal proctosigmoidectomy (Delor-
me’s procedure) is specic mucosal prolapse and small rectal prolapse, and involves excising the mucosa of the prolapsed rectum in order to plicate the underlying muscle wall and reduce the rectum back into the pelvis.
– Perineal proctosigmoidectomy (Alte-
meier procedure) is indicated for patients with complete rectal pro-
lapse who wish to avoid abdominal surgery. In this transanal procedure, the prolapsed rectum is incised, the redundant colon is resected, and then the colon and distal rectum are anastomosed.
– Finally, the stapled transanal rectal
resection (STARR) procedure is offered for elderly patients with recto­celes or intussusceptions. This opera­tion uses two circular staplers to perform a circumferential transanal resection of the rectum, effectively resecting the rectal outlet obstruction.

Suggested Reading

Agachan F, Chen T, Pfeifer J, Reissman P, Wexner SD.
A constipation scoring system to simplify evaluation and management of constipated patients. Dis Colon Rectum. 1996;39(6):681–5.
Bharucha AE, Dorn SD, Lembo A, Pressman A.American
Gastroenterological Association medical posi­tion statement on constipation. Gastroenterology. 2013;144(1):211–7.
Corman M, Nicholls RJ.Corman’s colon and rectal sur-
gery. Lippincott Williams & Wilkins; 2012.
Denoya P, Sands DR. Anorectal physiologic evalu-
ation of constipation. Clin Colon Rectal Surg. 2008;21(2):114–21.
Hussain ZH, Everhart K, Lacy BE. Treatment of
chronic constipation: prescription medications and surgical therapies. Gastroenterol Hepatol (N Y). 2015;11(2):104–14.
Levitt MA, Mathis KL, Pemberton JH.Surgical treatment
for constipation in children and adults. Best Pract Res Clin Gastroenterol. 2011;25(1):167–79.
Steele SR, Hull TL, Read TE, etal. The ASCRS text-
book of colon and rectal surgery. New York: Springer; 2016.
Part IV
Rectal

Retrorectal Tumors

EricG.Weiss andLuanneForce
36
Refer toAlgorithm inFig. 36.1
A. Presentation
Retrorectal tumors encompass a heteroge­neous group of tumors located in the presa­cral, or retrorectal space. The boundaries of the retrorectal space are the presacral fascia posteriorly, the rectum anteriorly, the rectal stalks, iliac arteries and ureters laterally, Waldeyer’s fascia inferiorly and the perito­neal reection superiorly. Lesions may be categorized as congenital, neurogenic, osse­ous, or miscellaneous. These are rare tumors, accounting for 1/40,000 hospital admis­sions. Most often, a retrorectal tumor will present as an incidental nding on imaging for another cause approximately 50% of the time. In patients that are symptomatic, the symptoms are often vague. Low back pain, leg pain or vague rectal pain may be present. There may also be compression of the sacral nerves that might present as incontinence, or pain that radiates to the buttocks and/or legs. Alternatively, these may present with signs of acute or chronic infection, as an abscess or stula to the perirectal or perianal space.
E. G. Weiss (*) Cleveland Clinic Florida, Weston, FL, USA e-mail: weisse@ccf.org
L. Force University of Miami, Miami, FL, USA

Evaluation

B. History and Physical Examination
A detailed history and physical examination is important when evaluating patients with retro­rectal tumors. These tumors are rare, often presenting with vague signs and symptoms, so a high level of suspicion must be maintained. The patient may present with a palpable rectal mass and be completely asymptomatic. Other signs/symptoms may include back pain, peri­neal pain, constipation, and vague gyneco­logic complaints in women. Neurologic complaints secondary to nerve impingement may also be present, including lower extrem­ity pain. Infection of the lesion may present as chronic sinuses, recurrent pilonidal infections, drainage into the rectum or vagina. Some female patients may present after a difcult vaginal delivery secondary to vaginal canal obstruction. Patients may have vague com­plaints for years without a diagnosis. Physical examination should include a thorough inspection of the perineal skin and soft tissue, looking for any chronic sinuses or stulae. A skin dimple may be observed posterior to the anus. A soft, smooth extrarectal mass may be palpated on digital rectal exam.
C. Imaging Modalities
A variety of imaging modalities may be used to assess retrorectal tumors, including plain radiographs, CT scanning and
© Springer Nature Switzerland AG 2020 S. R. Steele etal. (eds.), Clinical Decision Making in Colorectal Surgery,
https://doi.org/10.1007/978-3-319-65942-8_36
281
282
E. G. Weiss and L. Force
Fig. 36.1 Algorithm for retrorectal tumors
MRI.Several “hallmarks” on imaging may suggest a certain tumor. A plain radiograph of the pelvis may show evidence of bony destruction, which may indicative of a chordoma or malignant lesion. Multiple areas of calcication could signify a tera­toma. A “Scimitar” sign consists of the sacral border appearing to be rounded with­out bony destruction, which is seen in sacral meningocele. MRI has been shown in mul­tiple series, such as Glasgow and col­leagues, to be superior to CT to evaluate retrorectal masses. MRI can delineate the size, location of the tumor, and any invasion into surrounding structures and provides better contrast enhanced image. Features of the lesion on MRI imaging may give some clue as to the etiology and origin of the tumor. The presence of fat within the lesion on MRI may be indicative of a teratoma, dermoid cyst, liposarcoma, lipoma, myelo­lipoma or extramedullary hematopoiesis. Non-fatty containing solid lesions may be
categorized as cystic or solid. Solid masses include nerve sheath tumors (e.g., schwan­noma or neurobroma), myxoma, metasta­sis or lymphoma. If there is evidence of sacral invasion, the lesion may be a sacro­coccygeal chordoma or another osseous tumor. Non-fat containing cystic masses can be categorized as tailgut cysts, cystic ham­artomas, rectal duplication cyst, epidermoid cyst, anterior sacral meningocele, or extra­mucosal mucinous adenocarcinomas.
D. Endoscopy
Endoscopy has a limited role in evaluating presacral tumors. Sigmoidoscopy or colo­noscopy should be performed to exclude a primary rectal neoplasm. In the absence of a primary rectal cancer, endoscopy can be used to visualize the rectal mucosa and determine if the rectum is involved by the mass to aid with preoperative planning. Even when the mucosa is not directly involved, a smooth bulging may be seen related to mass effect of the tumor on the overlying rectal wall.
ab
36 Retrorectal Tumors
283
Endoanal ultrasound may be performed, but some difculty in interpretation is often present, as these lesions are rare and the endosonographer may not be familiar with the different appearances identied. Since these tumors are rare, there is limited experi­ence with ultrasound for evaluation, and often better evaluated by CT or MRI.
E. Percutaneous Biopsy
There is some debate in the literature about the importance and usefulness of preopera­tive biopsies of presacral masses. Many authors claim that there is a limited role for preoperative biopsy given the accuracy of MRI imaging. In addition, there have been some reports of seeding of the biopsy tract, which may lead to increased morbidity of subsequent procedures, as the tract or other organs that are in the path of biopsy may also need resection. Furthermore, biopsy of a meningocele may lead to fatal meningitis. Proponents for biopsy indicate that tissue diagnosis is more accurate and concordant with postoperative pathologic results in comparison with MRI alone. In addition, the presence of a malignant tumor may indicate a need for neoadjuvant therapy prior to sur­gical resection. Preoperative biopsy may guide therapeutic options for inoperable tumors or prior to operative intervention. A large, unresectable chordoma may benet
from high dose radiation therapy as a pallia­tive option. Retrorectal sarcomas may also benet from adjuvant treatment. GIST tumors may occur in the presacral space, which are amenable to treatment with ima­tinib. In general, biopsy should be individu­alized towards the goal of the biopsy. If therapy in addition to surgery is initially warranted, and pathological diagnosis is required, then biopsy may be performed. Moreover, if neoadjuvant therapy may allow for tumor shrinkage (e.g., GIST), the biopsy may be warranted. “Straightforward” lesions that are amenable to upfront surgical resec­tion typically do not require biopsy.

Risk Assessment

Pathology: Four Tissue Types
F. Congenital
Congenital tumors are the most common sub­type of retrorectal tumors, accounting for 50–70% of all patients. Developmental cysts are the most common congenital retrorectal tumors (Fig. 36.2). Majority of patients are female. These cysts may contain cells from any developmental cell type. Developmental cysts could be dermoid, epidermoid, tailgut or teratomas. Epidermoid cysts contain a squa-
Fig. 36.2 (a) CT scan image of an epidermoid cyst. (b) Sagittal view. (Courtesy of Dr. Laurence Sands)