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Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome: a 25-year experience.

OBJECTIVE: To analyze the outcome of children with short bowel syndrome (SBS) who required long-term parenteral nutrition (PN). STUDY DESIGN: Retrospective analysis of children (n=78) with SBS who required PN >3 months from 1975 to 2000. STATISTICS: univariate analysis, Kaplan-Meier method, and Cox proportional regression model were used. RESULTS: We identified 78 patients. Survival was better with small bowel length (SBL) >38 cm, intact ileocecal valve (ICV), intact colon, takedown surgery after ostomy (all P <.01), and primary anastomosis (P <.001). PN-associated early persistent cholestatic jaundice (P <.001) and SBL of <15 cm (P <.01) were associated with a higher mortality. Intestinal adaptation was less likely if SBL <15 cm (P <.05), ICV was removed, colonic resection was done (both P <.001), >50% of colon was resected (P <.05), and primary anastomosis could not be accomplished (P <.01). Survival was 73% (57), and 77% (44) of survivors had intestinal adaptation. CONCLUSIONS: SBL, intact ICV, intestinal continuity, and preservation of the colon are important factors for survival and adaptation. Adaptation usually occurred within the first 3 years. Need for long-term PN does not preclude achieving productive adulthood. Patients with ICV even with <15 cm of SBL and patients with SBL >15 cm without ICV have a chance of intestinal adaptation.

Adaptation, Physiological↗

Is there a role for surgery beyond colonic aganglionosis and anorectal malformations in children with intractable constipation?

PURPOSE: The aim of this study was to assess benefit of surgery in the treatment of childhood constipation in children without aganglionosis or anorectal malformations. METHODS: Retrospective chart review and follow-up questionnaire of 19 children (10 girls, age 7.6 +/- 3.9 years) who underwent surgery after colonic and anorectal manometry had documented abnormal motility. Children at the time of manometric evaluation had symptoms of intractable constipation a mean of 5.1 +/- 2.5 years. Follow-up questionnaires were administered to caregivers a mean of 11.4 +/- 9.3 months after surgical intervention. RESULTS: Proximal colonic abnormalities were seen in 2 patients, abnormalities involving the distal colon and rectosigmoid region in 13 patients, pancolonic abnormalities in 2 patients, and incomplete relaxation of the internal anal sphincter in 2 patients. Surgery led to increase in frequency of bowel movements per week (8.1 v 1.9; P <.005), decrease in soiling episodes per week (2.20 v 4.7; P <.01), and decrease in daily use of medications for constipation (0.8 v 2.3; P <.05). Adverse events included skin breakdown at site of ostomy (9%), fecal incontinence after pull-through procedure (13%), and persistent constipation (4.5%). Parents felt that the symptoms after surgery were completely resolved in 89% of patients. CONCLUSIONS: Surgery may be beneficial in the management children with chronic intractable constipation and documented abnormalities in motility.

Anastomosis, Surgical↗

Total colonic Hirschsprung's disease: a 28-year experience.

PURPOSE: The aim of this study was to review outcomes after surgical treatment of total colonic Hirschsprung's disease (TCH). METHODS: Twenty-five records of patients with TCH treated between 1974 and 2002 were reviewed. Follow-up data were collected using a standardized questionnaire. Objective functional outcome was assessed using a scoring system. RESULTS: Twenty patients had aganglionosis of the colon and distal ileum, 5 of whom had a more extensive condition. One of these 5 patients underwent an endorectal pull-through (ERPT), 1 underwent intestinal transplantation, and 3 died. Four of the remaining 20 patients underwent a primary ERPT, 16 received a stoma as neonates followed by ERPT in 12, and a Martin-Duhamel procedure or Swenson's operation in 3 (median age, 10.5 months); 1 remains with an ostomy. Postoperative complications included enterocolitis (55%), anal stricture (25%), and perineal excoriation (20%). Mean follow-up were 17.5 years (+/-11.1 years). Eighty-nine percent were free of recurrent enterocolitis. Frequency of bowel movements is 1 to 5 per day in 82% of the patients, 18% have 6 or more bowel movements per day. Occasional soiling is noted in 40% (one third of those requiring nighttime diapers). Overall functional outcome was good in 83%. Those patients with the longest follow-up periods had the best stooling scores (P = .04). CONCLUSIONS: Surgical treatment of TCH is associated with a number of complications including recurrent enterocolitis and anal strictures. Long-term outcome is quite favorable.

Anastomosis, Surgical↗

Complications associated with global endometrial ablation: the utility of the MAUDE database.

OBJECTIVE: To investigate the number and type of complications associated with global endometrial ablation using public-access governmental databanks. METHODS: MEDLINE (PubMed) and the US Food and Drug Administration Manufacturer and User Facility Device Experience (MAUDE) databases were searched for entries for the four US Food and Drug Administration-approved global endometrial ablation devices. RESULTS: Traditional MEDLINE and bibliography searches yielded reports of two cases of hemorrhage, one case of pelvic inflammatory disease, 20 cases of endometritis, two cases of first-degree skin burns, nine cases of hematometra, and 16 cases of vaginitis and/or cystitis. A search of the US Food and Drug Administration MAUDE database yielded reports of 85 complications in 62 patients. These included major complications: eight cases of thermal bowel injury, 30 cases of uterine perforation, 12 cases in which emergent laparotomy was required, and three intensive care unit admissions. One patient developed necrotizing fasciitis and eventually underwent vulvectomy, ureterocutaneous ostomy, and bilateral below-the-knee amputations. One of the patients with thermal injury to the bowel died. CONCLUSION: Use of the US Food and Drug Administration MAUDE database is helpful in identifying serious complications associated with global endometrial ablation not yet reported in the medical literature.

Databases, Factual↗

True prevalence of urinary incontinence among female nursing home residents.

OBJECTIVES: Urinary incontinence is a significant problem in nursing home residents in the United States. Estimates of its prevalence have often been based on data from medical records obtained at nursing home admission. To measure the sensitivity of this method of defining the prevalence of urinary incontinence among female nursing home residents, we examined data from a clinical survey. METHODS: We analyzed data from the National Nursing Home Survey, which collected information from nursing homes for each resident concerning admission diagnoses, presence of an indwelling Foley catheter or ostomy, need for assistance from equipment or personnel in using the toilet, and difficulty controlling urination. RESULTS: Residents' medical records revealed a very low rate of admission diagnoses of incontinence. However, clinical queries revealed a high prevalence of bladder dysfunction. More than one half of all female nursing home residents were reported to have "difficulty controlling urination," and more than one half needed assistance in using the toilet. CONCLUSIONS: Although only 1% to 2% of nursing home residents have a diagnosis of urinary incontinence, the true prevalence of bladder dysfunction in this group is much greater. The sharp divergence of National Nursing Home Survey data from published studies on the prevalence of incontinence in nursing homes highlights the limitations of using administrative data to study the epidemiology of bladder dysfunction.

Aged↗

Morbidity of rectosigmoid resection and primary anastomosis in patients undergoing primary cytoreductive surgery for advanced epithelial ovarian cancer.

OBJECTIVES: Studies from the colorectal literature have shown that factors associated with anastomotic leak after colorectal resection include long surgical time (>2 h), multiple blood transfusions, and short distance to the anal verge. The aim of this study was to assess the morbidity associated with en bloc resection of ovarian carcinoma with low anterior resection and anastomosis in patients undergoing primary cytoreductive surgery for advanced disease. METHODS: We performed a retrospective chart review of all patients who had undergone primary cytoreduction for advanced epithelial ovarian cancer with rectosigmoid resection followed by low rectal anastomosis between January 1994 and June 2004. Patient characteristics, operative details, and postoperative complications were extracted from patients' charts. RESULTS: Seventy patients met the above criteria and form our study group. The median age was 59 years (range, 25-82). There were 52 stage IIIC (74%) and 18 stage IV (26%) cancers. The median operating time was 315 min (range, 120-750) and the median estimated blood loss was 1200 ml (range, 250-8000), with 53 (76%) patients requiring blood transfusion. Twenty-eight patients (40%) underwent major upper abdominal procedures other than omentectomy, and 14 patients (20%) underwent a second bowel resection. Twelve patients (17%) underwent a protective ileostomy while the remainder (83%) did not. Of the 58 patients with no ostomy, the only complications associated with the resection and anastomoses were a pelvic abscess in 3 patients (5%) and an anastomotic leak requiring diverting colostomy in 1 patient (1.7%). Of the 12 patients who had protective ileostomies, 3 (25%) had complications related to their ileostomy short-bowel syndrome requiring early reversal, incarceration of the prolapsed loop requiring surgical correction, and prolapse corrected electively at the time of second-look surgery. CONCLUSIONS: In women undergoing primary cytoreductive surgery, the morbidity associated with en bloc resection of ovarian carcinoma with low rectosigmoid resection and anastomosis without protective ileostomy was acceptably low, with an anastomotic leak rate of less than 2%. Protective ileostomy is not always necessary and should be used selectively.

Adult↗

Reoperation in patients with the short bowel syndrome.

The clinical courses of 53 adult patients with the short bowel syndrome (SBS) were evaluated to determine the incidence of and indications for reoperation. Mesenteric vascular disease (23 patients) and malignancy/irradiation (18 patients) were the most frequent causes of resection. Early reoperation was necessary in nine (17%) patients, primarily for intestinal complications. Twenty (53%) of the 38 patients leaving the hospital required a later abdominal procedure during the mean follow-up of 30 months (range: 2 to 108 months). Three (33%) of nine patients with ulcer disease had gastric resection. Six (21%) of 28 patients at risk for cholelithiasis developed symptoms. Four of these patients underwent cholecystectomy, and three others had a prophylactic cholecystectomy. Ten patients underwent ostomy closure or formation. Intestinal disease necessitated stricturoplasty (three), serosal patch (one), minimal resection (three), or takedown of an ileal conduit (one). Twenty-four (63%) of 38 patients with SBS received home total parenteral nutrition for a mean of 22 months (range: 2 to 105 months). Eleven patients required more than 1 vascular access procedure, and 4 had more than 3 procedures. Patients with the SBS frequently require reoperation for intestinal conditions, cholelithiasis, peptic ulceration, and vascular access. Prophylactic cholecystectomy and strategies for preserving intestinal length are important considerations in these patients.

Adult↗

Edgar J. Poth Memorial Lecture. Surgical aspects of the short-bowel syndrome.

BACKGROUND: Surgeons are frequently confronted with patients with the short-bowel syndrome. Important surgical issues are maintaining intestinal continuity, treating complications, and performing procedures to improve intestinal function. METHODS: A comprehensive review of the English language literature and the author's own experience were employed to make recommendations about surgical management of the short-bowel syndrome. CONCLUSION: At the time of initial resection, ostomy formation is often prudent. The decision to restore continuity at a later time should balance anticipated functional outcome against potential complications. Several surgical strategies can be employed at reoperation in these patients to minimize further loss of intestine. Prophylactic cholecystectomy should be considered because of the increased risk of cholelithiasis. Gastric hypersecretion rarely requires operative therapy. Surgical therapy for the short-bowel syndrome includes procedures to slow intestinal transit, optimize intestinal function, and increase intestinal surface area. The choice of operation is influenced by intestinal remnant length and caliber and its function. Only a small proportion of patients are candidates for nontransplant procedures, of which intestinal lengthening is most efficacious. Intestinal transplantation, either alone or combined with the liver, is emerging as the most promising therapy in short-bowel syndrome.

Humans↗

Multiple intestinal atresia with apple peel syndrome: successful treatment by five end-to-end anastomoses, jejunostomy, and transanastomotic silicone stent.

The association of apple peel bowel with multiple intestinal atresias is a rare event. The authors report a case of a 2.200-g newborn affected by this uncommon condition. The interruptions of small bowel continuity were treated successfully with 5 end-to-end anastomoses and with the construction of 2 ostomies. A gastroduodenal silicone tube (Vygon S.A., Ecouen, France) was used as an intraluminal stent.

Abnormalities, Multiple↗

Vesicointestinal fissure revisited.

This complicated anomaly of bladder exstrophy, intestinal fistula, and various degrees of colonic atresia with imperforate anus is readily recognizable. The long-term result of conventional management has been disappointing. Fecal and urinary incontinence have been unavoidable and a source of disappointment to patients and parents. An alternative method of total management is detailed. This variation of management may result in earlier and more complete social acceptability of the child so afflicted. At as young an age as possible, the vesicointestinal connection is detached and the bladder defect as well as the intestinal opening closed. The short colon is freed and transected at its midpoint. The right half of the colon is brought out through a separate wound as a colostomy. The distal half of the divided colon is brought out as mucous fistula on the left. This distal colonic segment is the proposed future colonic loop for urinary diversion to be fashioned in the first year of life. The exstrophied bladder is removed at a later date. Epispadias in the male may be repaired later. A Pediatric Ostomy Club has been organized to include a pediatric stomal therapist and involved nurses and physicians. The group gives the parents advice and moral support. We believe that this approach will permit the child to go to school and carry on relatively normal activity and to be socially acceptable. An overview of our total experience in the management of 25 patients with vesicointestinal fistula details the result in 9 surviving patients.

Anus, Imperforate↗

Total intestinal aganglionosis: a new technique for prolonged survival.

Total small bowel aganglionosis is uniformly fatal; and prolonged nutritional treatment for the resulting severe short bowel syndrome in the absence of a therapy designed to achieve a functional bowel length has not been warranted. We report an operative technique, long segment small bowel myectomymyotomy, which has produced a functioning length of intestine capable of supporting ever increasing amounts of enteral nutrition. A term female was noted to have neonatal intestinal obstruction, and two laparotomies proved total colonic and near-total small bowel aganglionosis. At 2 months of age reoperation was done and the aganglionosis was proved to extend to 7 cm below the ligament of Treitz. From this transition zone to 10 cm distally, a myectomy was done removing a 1 cm wide length of seromuscular tissue to the level of the submucosa. From the distal end of the myectomy, another 40 cm of bowel received an antimesenteric border myotomy cutting to the submucosal level followed by spreading of the cut surface to a width of 1 cm. This left the patient with 55 to 60 cm of small bowel from the ligament of Treitz to the end of the myotomy at which point an end ostomy was created. The remainder of the small bowel was excised and the colon exteriorized as a mucous fistula. The patient was continued on total parenteral nutrition alone for ten days at which time small volume enteral feeds were introduced. By 5 months of age, 25% of calories were enteral; by 6 months, 33% of calories were enteral; and by 8 months, 45% of intake was enteral.(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗

Chemically induced bowel denervation improves survival in short bowel syndrome.

This study evaluates the effect of chemically induced bowel denervation on survival, weight gain or loss, transit time, and d-xylose absorption in rats following 80% small bowel resection. Forty-three male Sprague-Dawley rats (150 g) underwent 80% midsmall bowel resection and anastomosis. Twenty rats were short bowel controls (group I). In 23 rats (group II), a 2.0 cm segment of jejunum proximal to the anastomosis was denervated by application of 0.1% benzalkonium chloride (BK) for 30 minutes. Ten additional rats underwent sham laparotomy without bowel resection. Five remained untreated (group III) and in 5 (BK) denervation was added (group IV). Bowel denervation was confirmed by histologic study in all (BK) rats. Weight and daily food and water intake were measured for 30 days and the groups compared. Weight in group I was 43.8 +/- 52.9 g, group II 95.0 +/- 50.1, (P less than .005), group III 177 g, and group IV 175 g. Food intake was greater in group I than II (P less than .05) and was similar to groups III and IV. Water intake calculated as animal weight (g)/mL H2O ingested was lowest in group I (P less than .05). Mortality was 30% in group I (6/20) and only 8.6% in group II (2/23). No deaths were observed in unresected controls (III and IV). Twenty-four additional rats were evaluated for d-xylose absorption and transit time by bringing out a loop enterostomy 10.0 cm from the Ligament of Treitz. Twelve rats were ostomy controls (group V).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

The use of povidone iodine in neonatal bowel surgery.

The ostomy sites of four premature infants undergoing bowel reanastomosis were lavaged with a 10% solution of povidone iodine to reduce local bacterial contamination and the risk of anastomosis dehiscence. There was a significant decrease in serum thyroxin from 112 (+/- 11) mumol/L to 90 (+/- 33) mumol/L 24 hours following surgery (P less than .05), but no change in serum thyroid stimulating hormone (TSH). The total serum iodine rose from 1.5 (+/- 0.05) mumol/L before surgery to 61.6 (+/- 46.4) mumol/L 24 hours later and urinary iodine excretion was 60 times the preoperative value in the second 24 hours following surgery. Both the hormonal and biochemical indices returned to normal 2 weeks following surgery except for total serum iodine, which remained slightly elevated. Povidone iodine is a safe and effective antiseptic agent. However, as it may cause transient suppression of thyroid function in neonates, thyroid status should be tested in all such infants at approximately 2 weeks following repeated or widespread use.

Humans↗

Long-term morbidity in total colonic aganglionosis.

BACKGROUND/PURPOSE: No surgical treatment for total colonic aganglionosis (TCA) clearly has been proven superior. To identify clinical criteria associated with long-term functional outcome, a 28-year retrospective institutional review of this entity from 1969 through 1996 inclusive was undertaken. METHODS: Total colonic aganglionosis (TCA), defined here as aganglionosis extending from the anus to at least the ileocecal valve but no further than 50 cm proximal to the ileocecal valve, was identified in 29 infants and children. Appropriate leveling ileostomy was performed in 28 of 29 patients, and definitive surgical reconstruction was performed in 26 of 29. Three groups were identified based on the definitive surgical repair performed: group 1, construction lacking or incorporating a short ganglionic-aganglionic common channel (modified Soave or modified Duhamel, n = 8); group II, construction of an extended common channel (Martin-Duhamel, Martin-Soave; n = 6); and group III, all others including an intermediate-length common channel (n = 13). RESULTS: Functional outcome at extended follow-up (mean, 6.6 +/- 5.6 years; range, 0.7 to 23) was determined based on survival, long-term ostomy requirements, growth, major complications, continence, and enterocolitis and bowel movement frequency. Although long-term functional outcome was deemed satisfactory in six of seven patients in group I, function was satisfactory in none of six group II patients. Group III results were intermediate (satisfactory in 6 of 13). CONCLUSIONS: Acceptable long-term outcome was most frequent in TCA patients whose definitive repair did not incorporate an extended ganglionic-aganglionic common channel. The use of extensive lengths of aganglionic bowel to maximize fluid absorption is frequently met with substantial morbidity.

Analysis of Variance↗

Intestinal stoma complications in immunocompromised children.

BACKGROUND/PURPOSE: When performing an urgent gastrointestinal operation on an immunocompromised child, the pediatric surgeon may have to decide between performing an intestinal anastomosis (and risk leakage or sepsis) or creating an intestinal stoma. This study evaluates the postoperative course of those patients treated with intestinal stomas and the long-term survival rate of such patients. METHODS: A 13-year retrospective review of immunocompromised children with intestinal stomas was performed. Patients were assessed as to their diagnosis, indication for surgery, stoma type, postoperative complications (within 30 days of surgery), ostomy-related complications, and survival. RESULTS: 19 stomas (8 ileostomies and 11 colostomies) were created in 18 patients. Six children had immunodeficiency disorders; 12 were immunosuppressed from chemotherapy treatment for cancer. Indications for surgery included infectious complications (n = 8); neoplasm-induced bowel obstruction, perforation, or invasion (n = 10); and Hirschsprung's disease (n = 1). Postoperative complications occurred in 13 cases (68%); two warranted reoperation. Four of six patients with neutropenia had serious postoperative infectious complications. Stoma complications occurred in 6 cases (32%); 1 required revision. All 3 patients in whom bleeding developed from their stoma site were thrombocytopenic. Nine of 18 patients (50%) died, yet no patient died of complications attributable to their stomas. Of the surviving 9 children, 6 underwent stoma takedown at a mean of 19 months after creation; 1 has a permanent colostomy, and 2 currently are undergoing chemotherapy. CONCLUSION: Although immunocompromised children who require intestinal stomas frequently die of their underlying illnesses and their stomas often produce considerable morbidity, stoma creation does not jeopardize their chance of survival.

Adolescent↗

Technique for endoscopy of urinary diversion conduits.

Endoscopic evaluation of bowel conduits used for urinary diversion can be a cumbersome and untidy endeavor. We present a simple technique that facilitates this type of examination using an ostomy bag connected to a urinary drainage bag.

Cystoscopy↗

Clinical evaluation of an external urine collection device for nonambulatory incontinent women.

Chronic urinary incontinence is a frequent complication of a variety of neurological diseases and is a major clinical problem among the aged. Urinary incontinence may be the pivotal factor that determines whether a patient requires long-term institutional care. For management of urinary incontinence in aged, nonambulatory women use of absorbent products may not prevent decubitus ulcers, while chronic use of indwelling urethral catheters results in bacteriuria and its complications. An effective external urine collection device for women, analogous in function to the condom catheter for men, may improve care by reducing complications, averting admissions to chronic care facilities and lowering medical costs. During 125 patient-days we evaluated 63 applications of an external urine collection device on 7 incontinent women in a nursing home. Each device was allowed to remain in situ for a maximum of 48 hours. Only 14 per cent of the devices required premature replacement due to unacceptable urine leakage. The median device wear time was 48 hours. The only adverse reactions observed were minimal erythema at 2 of 63 device removals and transient periurethral edema in 1 patient, which disappeared with continued use of the device. Thus, this device, exploiting an adhesive developed for ostomy appliances, was effective in maintaining patient dryness and was not associated with severe local reactions. These results appear to warrant clinical trials for extended periods in incontinent women.

Aged↗

Quality of life survey of urinary diversion patients: comparison of ileal conduits versus continent Kock ileal reservoirs.

There has been a recent marked increase in interest in continent urinary diversions. While considerable time has been spent on the technical aspects of these diversions the psychological impact has not yet been fully explored. We describe an extensive survey that was conducted among 100 consecutive adults (87 respondents) who had undergone urinary diversion via an ileal conduit and 100 consecutive adults (85 respondents) in whom a continent Kock ileal reservoir was created during the last 3 to 5 years at our university by the same surgeons. The Kock pouch patients were stratified further into 63 with primary diversion and 22 who underwent conversion from previous conduit diversions. The survey consisted of a questionnaire that included a social and sexual survey, the Beck Depressive Inventory, the Profile of Mood States and a physical impact study. The results revealed that all patients surveyed generally were satisfied with the diversions and they had adapted reasonably socially, physically and psychologically. The key to adaptation seemed to be a detailed, realistic preoperative education about the type of diversion used. Patients with ileal conduit diversions had the lowest expectations of the form of diversion as defined by the preoperative awareness of the need to wear an external ostomy appliance with its associated inconveniences and change in the external body image. Postoperatively, ileal conduit patients also had the poorest self images as defined by a decrease in sexual desire and in all forms of physical contact (sexual and nonsexual). The subset of patients who underwent conversion from conduit diversions to Kock pouches, however, were statistically the most satisfied, and they were the most physically and sexually active. We conclude that the Kock continent urostomy offers an important alternative to noncontinent forms of diversion.

Adaptation, Psychological↗