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Malignant potential in intestinal juvenile polyposis syndromes.

BACKGROUND: Unlike familial polyposis coli, where the premalignant nature of adenomatous polyps is well established, the cancer risk in juvenile polyposis has generally been considered not increased. METHODS: This study reviews all cases of juvenile polyposis reported in the English language to date to assess the occurrence and prognosis of carcinoma in the gastrointestinal tract. RESULTS: A total of 218 patients met the inclusion criteria. Mean age at diagnosis was 18.5 years (range: 9 months to 67 years). No gender preference was identified. The most common presenting symptom was chronic anemia, followed by acute gastrointestinal bleeding, rectal prolapse of polyp, protein-losing enteropathy, and intussusception. A family history of juvenile polyposis could be established in approximately 50% of patients, and associated congenital malformations were detected in 15%. Ninety-nine patients underwent 138 gastrointestinal operations: 121 colorectal, 12 gastric, and 5 small intestinal procedures. The development of a gastrointestinal carcinoma was reported in 36 cases (17%). Mean age at diagnosis of carcinoma was 35.5 years (range: 4-60 years). Most malignancies were located in the distal colon and rectum, with only one case of gastric and one case of duodenal carcinoma. Tumor stage at diagnosis was usually advanced, with poor survival figures. CONCLUSIONS: This study shows that juvenile polyposis syndromes carry a more significant risk of carcinoma than generally appreciated. Therefore, more intense endoscopic surveillance may be warranted, and definitive surgical options should often be considered in these syndromes.

Adolescent↗

Surgical correction of anal incontinence.

Seventy-six operative procedures for anal incontinence performed at the Lahey Clinic Medical Center between 1964 and 1985 were reviewed. Etiologic factors, findings on preoperative physical examination, and functional results are reported for 61 procedures in the four categories of simple anterior reefing, anterior reefing with perineal body reconstruction and anoplasty skin closure, posterior proctopexy, and Dacron Silastic sling insertion. In women with anterior sphincter defects, combining anoplasty skin closure and deep external sphincter plication gives superior functional results over superficial reefing, especially when there is attenuation of the rectovaginal septum and perineal body. The posterior proctopexy is most useful in patients with intact external sphincters and incontinence without recognizable cause or after abdominal repair of rectal prolapse.

Adolescent↗

Overlapping sphincteroplasty for acquired anal incontinence.

When defects of the anal sphincter are caused by trauma, surgical correction can be successful even in long-standing cases. At the University of Minnesota, we used overlapping sphincteroplasty in 79 patients with fecal incontinence from 1952 to 1982. There were 62 women and 17 men. Ages ranged from 17 to 68 years. Incontinence had been present from three weeks to 40 years and had been caused by childbirth, previous anorectal surgery, trauma or rectal prolapse. Following overlapping sphincteroplasty, there was one postoperative death and 13 complications. Complications included temporary difficulty in voiding, excessive bleeding, abscess formation, fecal impaction, and hematoma. Seventy-six of the 78 surviving patients were followed for an average of 35 months. Results ranged from excellent to poor with only one failure. From our experience it was concluded that several factors were important for good surgical results. 1) The patient must have intact neuromuscular bundle with detectable voluntary sphincter contraction. 2) If a primary repair has failed, a minimum duration of three months should elapse before overlapping sphincteroplasty is attempted. 3) Scar tissue from the severed muscles should not be excised. 4) The internal and external sphincter muscles should not be separated. 5) A temporary concomitant colostomy is not necessary.

Adolescent↗

Balloon topography. A simple method of evaluating anal function.

Balloon topography was developed to simultaneously measure anal canal pressure, anal canal length, and anorectal angle. It is performed using a cylindrical flexible balloon placed into the anal canal and rectum and filled with liquid radiopaque contrast dye under low pressure. The pressure of the dye inside the balloon is controlled by the investigator, and does not vary with changes in balloon volume. The shape of the balloon within the anal canal is recorded using fluoroscopy and plain radiopaques. The patient receives less radiation than he would receive during a single contrast barium enema. We have performed the test on 27 subjects including a normal control, rectal prolapse patients, and incontinent patients. Early results demonstrate the usefulness of the test in examining anal sphincter and pelvic floor function in maintaining fecal continence in health and disease. The test measures multiple aspects of anopelvic function simultaneously that previously required separate investigations.

Aged↗

Clinical and manometric assessment of gracilis muscle transplant for fecal incontinence.

Six cases of gracilis muscle transplant for fecal incontinence are reported. The causes of fecal incontinence included previous anal operation, idiopathic incontinence, and rectal prolapse. All patients had had a previous operation for fecal incontinence. Postoperative sepsis developed at the operative site in five patients despite a defunctioning colostomy in two. Functional results of the operation were poor in all patients and a colostomy has now been raised in all cases. The operation was not associated with any objective improvement in resting or voluntary component pressure.

Adult↗

Biofeedback in the management of partial anal incontinence.

Fifty patients with anal incontinence were treated by biofeedback as an office procedure. This took the form of an intra-anal plug, containing two ring electrodes and connected to an electromyometer. In four or less sessions, patients were instructed in sphincter contraction and extraneous muscle relaxation. All have been followed for at least one year, and in 74 per cent there has been satisfactory restoration of continence. The method was particularly successful in post-surgical (anal) patients, with the exception of those with "keyhole deformity" after anal fistulectomy. In nonsurgical patients, results were excellent in the idiopathic and postradiation groups, but were poor in patients with incontinence due to rectal prolapse or to neurogenic causes. The simplicity and portability of this method make it a valuable aid in the office management of anal incontinence.

Adult↗

Balloon sphincterography. Clinical findings after 200 patients.

There are two muscular mechanisms of fecal continence. The anal sphincter squeezes the anal canal, thus lengthening it and increasing its resistance. The puborectalis kinks the distal rectum, preventing the transmission of intra-abdominal pressures into the anal canal. Balloon sphincterography simultaneously records the shape of the anal canal and distal rectum and measures the strength of the puborectalis and anal sphincter muscles. This allows the physician to evaluate the function of these important muscles in patients with symptomatic defecation disorders such as constipation, incontinence, and rectal prolapse. A cylindrical balloon is connected by a hose to a fluid reservoir filled with liquid barium. The deflated balloon is placed into the anal canal and inflated by raising the fluid reservoir in increments. Fluoroscopy visualizes the balloon's shape and video records the results. Quantitative sphincterogram measurements in patients with defecation disorders include (the three measurements in each category refer respectively to incontinent patients [N = 87], prolapse patients without incontinence [N = 26], and constipated patients [N = 65]); anorectal angle (degrees + S.D.): 114 + 28, 103 + 18, 95 + 19; anal canal length (mm + S.D.): 33 + 11, 38 + 10, 39 + 10; squeeze pressure (cm H2O + S.D.): 68 + 23, 80 + 16, 91 + 22, and opening pressure (cm H2O + S.D.): 52 + 25, 67 + 22, 81 + 24. The method is useful in identifying specific defects, such as paradoxic puborectalis contractions, that can cause constipation, and injuries to the sphincters that can cause incontinence. In over 280 patients with a wide variety of defecation disorders, sphincterography has yielded information not available by standard manometric techniques. It augments the findings of defecography.

Anal Canal↗

Diffuse juvenile polyposis of the colon: a premalignant condition?

Juvenile polyps are a common cause of rectal bleeding in the pediatric age group. These polyps are usually solitary, but occasionally polyposis can be identified by barium-enema or colonoscopy examinations. The benign nature of a single polyp or even scattered polyposis in childhood has been supported by several authors. A case of diffuse juvenile polyposis of the colon with associated adenomatous polyps of the colon in a 17-year-old who presented with iron deficiency anemia and rectal prolapse is discussed. This combination of diffuse juvenile polyposis and adenomatous polyps is an uncommon entity which is left to represent a premalignant lesion that warrants appropriate therapy. A review of the literature concerning the cause, diagnosis, and treatment of juvenile polyps is also discussed.

Adolescent↗

Quality of life of stoma patients: temporary ileostomy versus colostomy.

Ileostomy for proximal diversion as a preferred option over colostomy has been a recent topic of interest. Our study evaluated the quality of life (QOL) of patients with a temporary ileostomy and compared it with that of patients with a temporary colostomy. The QOL of 25 patients with an ileostomy (median age 42 years, range 22-76 years) was compared with that for 25 patients with a colostomy (median age 44 years, range 18-70 years). Indications for a stoma were rectal carcinoma, trauma, inflammatory bowel disease, anastomotic leak, or incontinence following an operative procedure for rectal prolapse. The study was conducted at a median of 8 weeks (range 6-16 weeks) for ileostomy patients and of 9 weeks (range 5-17 weeks) for colostomy patients following stoma creation. A self-administered structured questionnaire was used, with responses obtained for 10 QOL questions on a visual analog rating scale (0-100 mm); they were graded good (71-100), satisfactory (31-70), or poor (0-30). Altogether, 22 (88%) patients with an ileostomy, compared with 16 (64%) patients with a colostomy, were able to purchase their stomal appliances ( p = 0.09, chi(2): NS). Effluent was tolerable in 18 (72%) patients with an ileostomy compared with 7 (28%) patients with a colostomy ( p = 0.002, chi(2)). Appetite was not affected in any of the patients with an ileostomy (100%), compared with 64% of patients with a colostomy ( p = 0.002, chi(2)), travel by public transport 32% compared to 28% with colostomy (NS), dress in 20% compared to 24% with colostomy (NS), and daily activities 28% compared to 24% with colostomy (NS). Moreover, 68% with an ileostomy did not have a problem with hygiene compared with 40% with a colostomy (NS); 95% with an ileostomy abstained from sexual activity compared with 81% with a colostomy ( p = 0.21, chi(2): NS). Both ileostomy and colostomy resulted in significant QOL impairment. However, with ileostomy, the effluent was more tolerable, had less of an impact on personal hygiene, and preserved the appetite compared with colostomy. There were no differences in travel, dress, daily chores, or sexual activity between the two groups.

Adolescent↗

Burst strength of laparoscopic and open hernia repair.

BACKGROUND: There are few reports of overall strength of laparoscopic and open incisional hernia repair. METHODS: After anesthesia, a 2-inch circular defect was made in the abdominal wall of 28 female swine. Gore-Tex DualMesh Biomaterial (W. L. Gore & Associates, Flagstaff, AZ) was used for all repairs. Sixteen animals underwent open repair and 12 underwent laparoscopic repair. Burst strength was detected within 2 weeks and at 6 weeks by euthanizing the animals and insufflating the abdominal cavity with water while measuring the intraabdominal pressure until it could no longer be pressurized. RESULTS: Three events occurred after insufflation: rupture around patch (R), dissection from insufflation or pressure monitoring sites (D), or rectal prolapse (P). Failure after open early repair occurred at 289 (range 219-388) mmHg with 7-R, 1-P and late 289 (196-343) mmHg with 1-R, 6-P. Failure after laparoscopic early repair occurred at 259 (191-388) mmHg with 4-R, 1-P, 1-D and late 291 (140-330) mmHg with 2-R, 1-P, 3-D. Late groups were less likely to rupture. CONCLUSION: Both hernia repairs are durable at early and late periods. Tissue ingrowth adds to repair strength. We could not show that one repair was stronger than the other. Nonetheless, laparoscopic repair tended to degrade by dissection, which was our highest pressure event.

Abdominal Wall↗

Multimedia article. Laparoscopic treatment of chronic sigmoid volvulus in a young adult.

INTRODUCTION: Sigmoid volvulus is responsible for 8% of all intestinal obstructions. The most frequent presentation is in the elderly, with it occurring exceptionally in young people. Surgical resection is mandatory to prevent recurrence. Laparoscopic maneuvers in the long and distended bowel are difficult, and not much experience with these procedures has been reported. MATERIALS AND METHODS, AND RESULTS: A 21-year-old man with antecedents of constipation had two episodes of rectal prolapse, and one episode of acute volvulation treated with decompressive endoscopy. A laparoscopic exploration was performed for definitive treatment. Transanal intubation with a large-bore tube permitted deflation of the bowel. A deep Douglas's pouch was observed with a mobile sigmoid loop that intussuscepted the rectum. A proctosigmoidectomy including the 5 cm of the upper rectum was performed without incident. CONCLUSION: Laparoscopic management of suboclusive colonic volvulus is feasible. Intraopertive transanal intubation permits deflation the loop and facilitates manipulation.

Adult↗

Post-dysenteric hemolytic uremic syndrome in children during an epidemic of Shigella dysentery in Kwazulu/Natal.

We report 81 of 107 cases of hemolytic uremic syndrome (HUS), admitted between July 1994 and February 1996, following an outbreak of Shigella dysenteriae type 1 dysentery in Kwazulu/Natal. All patients, excluding 1, were black with a mean age of 38 months (range 1-121); 50 (61.7%) were males. The mean duration of dysentery was 11.3 days (range 1-41) and HUS 15 days (range 1-91). Most patients had acute oliguric renal failure (90.1%), 42 (51.6%) required peritoneal dialysis. Complications included encephalopathy 30 (37.0%), convulsions 12 (14.8%) and hemiplegia 2 (2.3%), gastrointestinal perforation 8 (9.9%), protein losing enteropathy 26 (32.1%), toxic megacolon 4 (4.9%), rectal prolapse 5 (6.2%), hepatitis 11 (13.6%), myocarditis 5 (6.2%), congestive cardiac failure 3 (3.7%), cardiomyopathy 3 (3.7%), infective endocarditis 1 (1.2%), septicemia 15 (18.5%), disseminated intravascular coagulation 17 (21%). Leukemoid reactions were found in 74 (91.3%) patients, hyponatremia in 56 (69.1%), and hypoalbuminemia in 67 (82.7%). Stool culture for Shigella dysenteriae type I was positive in only 7 (8.6%) patients; Shiga toxin assays were not performed. Outcome was as follows: recovery 32 (39.5%), impaired renal function 8 (9.9%), chronic renal failure 26 (32.1%), end-stage renal disease 1 (1.2%), and death 14 (17.3%) patients.

Child↗

Nonfamilial juvenile polyposis coli in a child: report of a case.

Juvenile polyposis coli (JPC) is an uncommon condition, manifesting as hamartomatous gastrointestinal polyposis with potential malignancy. This report describes a 15-month-old girl who was diagnosed to have nonfamilial JPC accompanied by macrocephaly, clubbed fingers, and mental retardation. Radiography of the colon by a barium enema and total colonoscopy demonstrated numerous colonic polyps. A barium meal study did not show any abnormality in the stomach, duodenum, or small intestine. She died at 6 years of age from hemorrhagic shock due to massive lower gastrointestinal bleeding associated with a rectal prolapse. The related literature is reviewed, and the treatments and complications of JPC in children are also discussed. We emphasize that family members of patients diagnosed with juvenile polyposis should be questioned and undergo appropriate examinations of the entire intestine from the stomach to the rectum.

Adenomatous Polyps↗

Associations of defecography and physiologic findings in male patients with rectocele.

This study evaluated the incidence and physiological findings in male patients with rectoceles. All defecographic studies were evaluated by a single colorectal surgeon. After diagnosis of rectocele in male patients, the patient's history, symptoms, and physiologic tests (anal manometry, pudendal nerve terminal motor latency [PNTML], assessment and electromyography [EMG]) were studied. A prominent rectocele was defined as one that did not empty during defecography and was associated with outlet obstructive syndrome. Forty (17%) rectoceles were diagnosed in 234 male patients with evacuatory disorders who underwent defecography. Rectoceles were anterior in 19 (48%) and posterior in 21 (52%) patients. The main complaint was constipation with difficult defecation in 33 (83%), followed by rectal pain in 5 (13%), rectal prolapse in 1 (3%), and incontinence in 1 (3%). Previous prostatic surgery had been performed in 16 (40%) patients. The mean age and duration of symptoms were 72.4 years (range, 30-88) and 10.3 years (range, 0.5-70), respectively. Excessive straining during evacuation was noted in 73%, unilateral or bilateral pudendal neuropathy in 24.5%, paradoxical puborectalis contraction in 49% and abnormal EMG in 11% of patients. Higher resting pressures with a mean 3.9 cm high pressure zone were noted in 29% of patients. The accompanying findings in defecography were, non-relaxing or partially relaxing puborectalis muscle (66%), perineal descent (65%), intussusception (23%), and sigmoidocele (15%). None of the patients underwent surgery for rectocele alone. In conclusion, rectocele is uncommon in males; it rarely appears as an isolated dysfunction as it is often associated with functional disorders of the pelvic floor. There is a frequent association between rectocele and prostatectomy. Clinical significance and therapeutic strategy remain unknown.

Adult↗

The utility of pudendal nerve terminal motor latencies in idiopathic incontinence.

PURPOSE: Pudendal nerve terminal motor latency testing has been used to test for pudendal neuropathy, but its value remains controversial. We sought to clarify the relationship of pudendal nerve terminal motor latency to sphincter pressure and level of continence in a cohort of patients with intact anal sphincters and normal pelvic floor anatomy. METHODS: We reviewed 1,404 consecutive patients who were evaluated at our pelvic floor laboratory for fecal incontinence. From this group, 83 patients had intact anal sphincters on ultrasound and did not have internal or external rectal prolapse during defecography. These patients were evaluated by pudendal nerve terminal motor latency testing, a standardized questionnaire, and anorectal manometry, which measured resting and squeeze anal pressures. Incontinence scores were calculated by using the American Medical Systems Fecal Incontinence Score. Values were compared by using the Fisher's exact test and Wilcoxon's rank-sum test; and significance was assigned at the P < 0.05 level. RESULTS: 1) Using a 2.2-ms threshold, 28 percent of patients had prolonged pudendal nerve terminal motor latency unilaterally and 12 percent bilaterally. 2) At a 2.4-ms threshold, 18 percent of patients had prolonged pudendal nerve terminal motor latency unilaterally and 8 percent bilaterally. 3) Bilaterally prolonged pudendal nerve terminal motor latency was significantly associated with decreased maximum mean resting pressure and increased Fecal Incontinence Score, but not decreased maximum mean squeeze pressure, at both 2.2-ms and 2.4-ms thresholds. 4) Unilaterally prolonged pudendal nerve terminal motor latency was not associated with maximum mean resting pressure, maximum mean squeeze pressure, or fecal incontinence score at either threshold. CONCLUSIONS: The majority of incontinent patients with intact sphincters have normal pudendal nerve terminal motor latency. Bilaterally but not unilaterally prolonged pudendal nerve terminal motor latency is associated with poorer function and physiology in the incontinent patient with an intact sphincter.

Aged↗

Anorectal Disease.

Most symptomatic internal hemorrhoids, grade 1 through 3, can be treated successfully with office-based procedures. Anorectal suppurative diseases must be treated surgically. Control of sepsis with subsequent fistula surgery as necessary is the goal. New nonoperative methods of anal fissure therapy are directed at reducing anal sphincter pressures. These methods have shown significant reduction in the need for sphincterotomy--a proven surgical technique with some risk of impaired continence. Surgery, using an advancement flap and partial internal sphincterotomy, remains the primary treatment for anal stenosis. Solitary rectal ulcer remains a difficult problem to manage medically and surgically. Multiple surgical techniques can effectively treat rectal prolapse. A minimal technique using Silastic wrap (Wright Medical Technologies; Arlington, TX), perineal resection (Altemeier procedure), and sigmoidectomy-rectopexy, or Ripstein suspension, has been the most favored method in selected patients.

Journal Article↗

Surgical Therapy for Colitis Cystica Profunda and Solitary Rectal Ulcer Syndrome.

The initial treatment for patients with colitis cystica profunda is aimed at re-education of bowel habits to avoid straining. Patients are instructed to spend the minimum time on the commode. A high-fiber diet with bulk laxatives is recommended. If fiber does not work, polyethylene glycol solutions and surface-active stool softeners such as docusate sodium are tried. Conservative therapy resolves most symptoms. Biofeedback is offered to patients who fail dietary therapy. For patients with associated rectal prolapse, an operation designed to correct the intussusception is considered. For full-thickness prolapse (procidentia) a mucosal resection (Delorme) or perineal proctectomy (Altemeier) is recommended. Surgical treatment of internal (occult) prolapse is problematic. If attempted, a resection and suture rectopexy has the most support. In the absence of procidentia, transanal excision may be used in selected patients. If other measures fail, a mucosal sleeve resection with coloanal pull-through or a diverting colostomy may be considered.

Journal Article↗

Genitourinary prolapse and joint hypermobility in women.

OBJECTIVE: To determine whether joint hypermobility, a clinical marker for connective tissue abnormalities, is associated with genital prolapse. METHODS: One hundred seven women were recruited from a university gynecology clinic. Subjects were examined in the standing and nonstraining positions for cystocele, rectocele, and uterine or vault prolapse. The degree of prolapse was graded 0-3. A separate investigator, blinded to the results of the gynecologic examination and using accepted criteria, evaluated each subject for joint hypermobility. RESULTS: Clinical joint hypermobility was found in 39 of 107 (36%) study patients. Subjects with joint hypermobility had a significantly higher prevalence of cystocele (33 of 37 [89%] versus 40 of 69 [58%], P = .001), rectocele (32 of 38 [84%] versus 33 of 69 [48%], P = .0002), and uterine or vault prolapse (25 of 38 [66%] versus 20 of 69 [29%], P = .0002) compared to women with normal joint mobility, respectively. No differences in the prevalence of stress incontinence were found between the two groups. CONCLUSION: Women with joint hypermobility have a significantly higher prevalence of genital prolapse compared to women with normal mobility, which suggests an underlying connective tissue abnormality as one etiology of pelvic relaxation.

Adult↗