Incidence of adhesive small bowel obstruction after surgery for colorectal cancer in Sweden 2007-2016.

AIM: Population-based data on incidence and risk factors of adhesive small bowel obstruction (SBO) are limited. The aims of this study were to assess the risk of SBO and SBO surgery after bowel resection for colorectal cancer (CRC) and to assess whether this risk is modified by minimally invasive surgery (MIS) and radiotherapy in a retrospective national study. METHODS: CRCBaSe, a nationwide register linkage originating from the Swedish Colorectal Cancer Register, was used to identify Stage I-III CRC patients who underwent resection in 2007-2016, with follow-up throughout 2017. Matched CRC-free comparators (1:6) were included as a reference of SBO and SBO surgery incidence. The association between MIS and preoperative radiotherapy and the incidence rate of SBO was evaluated in adjusted multivariable Cox regression models. RESULTS: Among 33 632 CRC patients and 198 649 comparators, the 5-year cumulative incidence of SBO and SBO surgery was 7.6% and 2.2% among patients and 0.6% and 0.2% among comparators, with death as a competing risk. In all patients, MIS was associated with a reduced incidence of SBO (hazard ratio [HR] 0.7, 95% CI 0.6-0.8) and SBO surgery (HR 0.5, 95% CI 0.3-0.7). In rectal cancer patients, radiotherapy was associated with an increased incidence of SBO (HR 1.6, 95% CI 1.4-1.8) and SBO surgery (HR 1.7, 95% CI 1.3-2.3). DISCUSSION: Colorectal cancer surgery is associated with a marked increase in risk of SBO, compared with the general population. The incidence is further increased if open surgery or radiotherapy is performed.

Economic Consequences of Surgery for Adhesive Small Bowel Obstruction: A Population-Based Study.

Background and Aims: Most patients develop adhesions after abdominal surgery, some will be hospitalized with small bowel obstruction (SBO), and some also require surgery. The operations and follow-up are expensive, but recent data of costs are scarce. The aim of this study was to describe the direct costs of SBO-surgery and follow-up, in a population-based setting. The association between cost of SBO and peri- and postoperative data was also studied. Methods: In a retrospective cohort study, all patients (n = 402) operated for adhesive SBO in Gävleborg and Uppsala counties (2007-2012) were studied. The median follow-up was 8 years. Costs were calculated according to the pricelist of Uppsala University Hospital, Uppsala, Sweden. Results: Overall total costs were €16.267 million, corresponding to a mean total cost per patient of €40,467 during the studied period. Diffuse adhesions and postoperative complications were associated with increased costs for SBO in a multivariable analysis (P < 0.001). Most costs, about €14 million (85%), arouse in conjunction with the SBO-index surgery period. In-hospital stay was the dominating cost, accounting for 70% of the total costs. Conclusion: Surgery for SBO generates substantial economic burden for healthcare systems. Measures that reduce the incidence of SBO, the frequency of postoperative complication, or the length of stay have the potential to reduce this economic burden. The cost estimates from this study may be valuable for future cost-benefit analyses in intervention studies.

Complications and adverse effects related to surgical and medical treatment in patients with inflammatory bowel disease in a prospectively recruited population-based cohort.

BACKGROUND: Medical adverse effects and surgical complications have been reported during treatment of patients with inflammatory bowel diseases (IBDs). There is however a shortage of studies describing these in the same cohort of patients. AIM: To describe medical adverse effects and surgical complications in a prospectively followed population-based cohort of patients followed for at least 10 years. METHODS: All newly diagnosed patients with ulcerative colitis (UC) and Crohn's disease (CD) in the county of Uppsala between 2005 and 2009 were prospectively followed. At the end of 2019, the medical notes were scrutinised and all medical adverse effects and postoperative surgical complications were registered. RESULTS: A total of 330 patients with UC and 153 patients with CD in all age groups were included in the cohort. Four hundred and forty-two of these (91.5%) could be followed for 10 years or until death. One hundred and twenty-two patients (26.9%) experienced one or more adverse effects during the pharmacological treatment, and 25 of these could be classified as serious. Fifty-seven malignancies were diagnosed during the observation time. Surgery was performed in 16/330 UC and 33/153 CD patients. Frequency of early postoperative complications was 31% for UC patients and 36% for CD patients. Most complications were minor but two patients were re-operated, two needed intensive care and one patient died postoperatively. CONCLUSIONS: Adverse effects related to medical therapy were experienced by approximately every fourth patient, and by every third patient that was operated.

Treatment and outcome of ulcerative colitis during the first 10 years after diagnosis in a prospectively followed population-based cohort.

BACKGROUND AND AIMS: There is a shortage of studies evaluating the effect of prevalent use of immunomodulators (IMMs) and biologicals on the clinical course of ulcerative colitis (UC) during 10 years. The aim of the present study is to report the use of drugs and surgery as well as mortality in a population based setting. METHODS: Between 2005 and 2009, we identified 330 patients in all ages (3-86 years) with an incident diagnosis of UC in the County of Uppsala, Sweden. They were followed prospectively and the medical notes were retrospectively analysed with special reference to the use of drugs, surgery and mortality. RESULTS: Median follow-up was 11.2 years (inter-quartile range 10.2-12.7). Out of the 330 patients, 298 (90.3%) could be followed for at least 10 years or until death. The cumulative exposure to different drugs was as follows: 5-ASA 96.6%, steroids 73.3%, IMMs 35.4% and biologicals 11.4%. Fourteen patients (4.6%) needed a colectomy during the observation time. Overall mortality in 10 years was 7% (23/330) whereof three patients died as a consequence of the disease or its treatment. Three patients (0.9%) were diagnosed with colonic cancer of whom two also had sclerosing cholangitis. CONCLUSIONS: A frequent use of IMMs and biologicals during 10 years, can result in a low need for colectomy without increased mortality compared to previous reports.

Clinical course of Crohn's disease in a population-based cohort in Uppsala County followed for 10 years.

BACKGROUND AND AIMS: There is a shortage of studies describing the outcome of patients with Crohn's disease (CD) where both biological therapy and immunomodulators (IMMs) have been available. The aim of the present study was to describe the clinical course of the disease, the use of drugs, the need for surgery and mortality in a prospectively recruited population-based cohort of patients followed for 10 years. METHODS: All patients diagnosed with CD in the County of Uppsala in Sweden 2005-2009 were prospectively recruited and followed until the end of 2019. The medical notes were scrutinised and relevant information collected. RESULTS: One hundred and fifty-four patients covering all age groups were diagnosed with CD and 145 (94.2%) could be followed for 10 years or until death. Nine patients were lost to follow up. The following drugs were used: 5-ASA 83%, steroids 84%, IMMs 69% and biologicals 23%. The proportion of penetrating disease increased from 9.7 to 14.5%. Primary bowel resections were performed in 22% of the patients, and none of these had any secondary surgery because of recurrent or progressive disease during the observation time. Twelve patients (7.8%) died during the follow up, and one of these because of a small bowel carcinoma. CONCLUSIONS: In the present study, the clinical course of CD was similar to previous reports during the first year after diagnosis, but the following years were considerably more stable with moderate increase of intestinal damage and totally a low frequency of surgery and no repeated surgery.

Acute severe attacks of ulcerative colitis in a population-based cohort: epidemiology, treatment and outcome.

Background: Although the criteria for acute severe ulcerative colitis have been stable for decades, the epidemiology for this condition has rarely been described in a population-based setting.Aim: To describe the appearance, treatment and outcome of acute severe ulcerative colitis in a population-based cohort of ulcerative colitis diagnosed between 2005 and 2009.Methods: Between 2005 and 2009, all patients diagnosed with ulcerative colitis in the County of Uppsala, a geographical area consisting of an average 318,000 individuals, were included in a cohort that was followed until the end of 2019. Three hundred and thirty patients covering an age interval of 3-86 years, were included. The medical records of the patients were scrutinized with regard to clinical course, drugs prescribed for IBD, hospitalization and surgical interventions. Patients that had left the health care area were contacted through letters except in case of emigration outside the country.Results: Forty-nine patients experienced a first severe attack (14.8%), and six of these were operated (12.2%). Fifteen patients suffered a second or third attack, and one more was operated. One elderly lady died postoperatively from sepsis.Conclusions: Acute severe attacks of ulcerative colitis appeared early after diagnosis and if surgery was escaped, there was no increased need for surgery in case of a new severe attack later.

Mechanisms of adhesive small bowel obstruction and outcome of surgery; a population-based study.

BACKGROUND: This study aims to describe the mechanisms of adhesive small bowel obstruction (SBO) and its morbidity, mortality and recurrence after surgery for SBO in a defined population. METHOD: Retrospective study of 402 patients (240 women, median age 70 years, range 18-97) who underwent surgery for SBO in the Uppsala and Gävleborg regions in 2007-2012. Patients were followed to last note in medical records or death. RESULT: The cause of obstruction was a fibrous band in 56% and diffuse adhesions in 44%. Early overall postoperative morbidity was 48 and 10% required a re-operation. Complications, intensive care and early mortality (n = 21, 5.2%) were related to age (p < 0.05) and American Society of Anesthesiologist's class (p < 0.01). At a median follow-up of 66 months (0-122), 72 patients (18%) had been re-admitted because of SBO; 26 of them underwent a re-operation. Previous laparotomies (p = 0.013), diffuse adhesions (p = 0.050), and difficult surgery (bowel injury, operation time and bleeding, p = 0.034-0.003) related to recurrent SBO. The cohort spent 6735 days in hospital due to SBO; 772 of these days were due to recurrent SBO. In all, 61% of the cohort was alive at last follow-up. Late mortality was related to malignancies, cardiovascular disease, and other chronic diseases. CONCLUSIONS: About half of patients with SBO are elderly with co-morbidities which predispose to postoperative complications and mortality. Diffuse adhesions, which make surgery difficult, were common and related to future SBO. Overall, nearly one-fifth of patients needed re-admission for recurrent SBO. Continued research for preventing SBO is desirable. TRIAL REGISTRATION: The study was registered at ClinicalTrials.gov (NCT03534596, retrospectively registered, 2018-05-24).

Injectable bulking treatment of persistent fecal incontinence in adult patients after anorectal malformations.

BACKGROUND/PURPOSE: Injectable bulking therapy has emerged as a treatment for fecal incontinence (FI), however there are no studies including adult patients with anorectal malformations (ARM). This study aimed to evaluate non-animal stabilized hyaluronic acid with dextranomer (NASHA/Dx) for the treatment of adult ARM patients with persistent FI. METHODS: Seven adults with ARM and incontinence to loose stool at least once weekly and without rectal or mucosal prolapse were treated with anal NASHA/Dx injection. They were evaluated preoperatively, at 6 and 18 months with a bowel function questionnaire and a 2-week bowel diary as well as FIQL and SF-36 quality of life questionnaires. RESULTS: Before treatment, the mean number of incontinence episodes over 2 weeks was 20.7 (median 16, range 8-52). At 6 months, the corresponding figures were 5.3 (median 4, range 0-19, p = 0.018), and at 18 months the figures were 4.3 (median 2, range 1-20, p = 0.018). An improved physical function in SF-36 from 74.3 at baseline to 86.4 at 6 months was noted (p = 0.04). No serious adverse events occurred. CONCLUSIONS: NASHA/Dx is a promising treatment option for selected adult patients with persistent FI after ARM. Longer follow up of larger patient series and studies on patients in adolescence is needed. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level IV.

Long-Term Outcome after Dynamic Graciloplasty for Treatment of Persistent Fecal Incontinence in Patients with Anorectal Malformations.

PURPOSE: Dynamic graciloplasty (DGP) has been used to treat severe fecal incontinence since the 1980s. Previous studies have shown an inferior outcome in patients with anorectal malformations (ARMs). Our experience has been that DGP has been appreciated by ARM -patients. The objective of the study was to evaluate the long-term outcome of DGP in our patients with ARM compared with patients with other underlying conditions. MATERIALS AND METHODS: Twenty-three patients operated with DGP at our institution from 1996 to 2010 were sent validated bowel function and quality of life questionnaires. Eighteen of 23 responded. Seven had ARM and 11 had other etiologies of fecal incontinence. The mean follow-up time was 11.6 years (range, 5-17). RESULTS: Four of 7 of the patients with ARM and 8 of 11 of patients with other etiologies used their implants at follow-up. The Miller incontinence score was slightly higher for patients with ARMs, but they had less constipation and higher Fecal Incontinence Quality of Life (FIQL)- and 36-Item Short Form Health Survey (SF-36) scores. None of the differences were statistically significant. CONCLUSION: This study cannot confirm earlier reports in which DGP has an inferior outcome in patients with ARM. We therefore believe that the procedure should remain a treatment option for selected patients.

Persistent fecal incontinence into adulthood after repair of anorectal malformations.

PURPOSE: Persistent fecal incontinence beyond childhood is common in ARM patients. The aim of this study was to analyze a consecutive series of adult patients with persistent incontinence, establish the causes, and evaluate whether further treatment could be offered. METHODS: Forty-four adult ARM patients with reported incontinence were invited. Eighteen patients (11 males, median age 40.5 years, range 18-50 years) accepted and underwent clinical examination, rectoscopy, and 3D-ultrasound. Five had previously been treated with secondary surgery to improve continence. RESULTS: Seventeen of the 18 patients had abnormal findings at examination. Eight patients had obstruction of the reconstructed anus. Eleven patients had sacral deformities. Nine patients had a defect in the external anal sphincter and nine patients could not contract the sphincter on demand. Five patients had significant prolapse of mucosa. In one patient, the neoanus was totally misplaced, one patient had a rectovaginal fistula, and one patient had short bowel syndrome due to several small bowel resections. Ten patients were offered conservative and five surgical treatment. CONCLUSIONS: This case series of adults shows that a majority of the patients can be offered further treatment. This indicates a need for structured follow-up of ARM patients into adulthood.

Glucagon-Like Peptide-1 Inhibits Prandial Gastrointestinal Motility Through Myenteric Neuronal Mechanisms in Humans.

Context: Glucagon-like peptide-1 (GLP-1) secretion from l-cells and postprandial inhibition of gastrointestinal motility. Objective: Investigate whether physiological plasma concentrations of GLP-1 inhibit human postprandial motility and determine mechanism of action of GLP-1 and analog ROSE-010 action. Design: Single-blind parallel study. Setting: University hospital laboratory. Participants: Healthy volunteers investigated with antroduodenal manometry. Human gastric and intestinal muscle strips. Interventions: Motility indices (MIs) obtained before and during GLP-1 or saline infusion. Plasma GLP-1 and glucagon-like peptide-2 (GLP-2) measured by radioimmunoassay. Gastrointestinal muscle strips investigated for GLP-1- and ROSE-010-induced relaxation employing GLP-1 and GLP-2 and their receptor localization, and blockers exendin(9-39)amide, Lω-nitro-monomethylarginine (L-NMMA), 2',5'-dideoxyadenosine (DDA), and tetrodotoxin (TTX) to reveal target mechanism of GLP-1 action. Main Outcome Measures: Postprandial gastrointestinal relaxation by GLP-1. Results: In humans, food intake increased MI to 6.4 ± 0.3 (antrum), 5.7 ± 0.4 (duodenum), and 5.9 ± 0.2 (jejunum). GLP-1 administered intravenously raised plasma GLP-1, but not GLP-2. GLP-1 0.7 pmol/kg/min suppressed corresponding MI to 4.6 ± 0.2, 4.7 ± 0.4, and 5.0 ± 0.2, whereas 1.2 pmol/kg/min suppressed MI to 5.4 ± 0.2, 4.4 ± 0.3, and 5.4 ± 0.3 (P < 0.0001 to 0.005). In vitro, GLP-1 and ROSE-010 prevented contractions by bethanechol and electric field stimulation (P < 0.005 to 0.05). These effects were disinhibited by exendin(9-39)amide, L-NMMA, DDA, or TTX. GLP-1 and GLP-2 were localized to epithelial cells, GLP-1 also at myenteric neurons. GLP-1R and GLP-2R were localized at myenteric neurons but not muscle. Conclusions: GLP-1 and ROSE-010 inhibit postprandial gastrointestinal motility through GLP-1R at myenteric neurons, involving nitrergic and cyclic adenosine monophosphate-dependent mechanisms.

Outcome in adults with anorectal malformations in relation to modern classification - Which patients do we need to follow beyond childhood?

BACKGROUND/PURPOSE: Knowledge about the functional outcome in adults with anorectal malformations is essential to organize structured transition to adult care for this patient group. The aim of this study was to investigate the functional outcome and quality of life in adults with anorectal malformations characterized according to the Krickenbeck classification. METHODS: Of 256 patients diagnosed with anorectal malformations at our institution in 1961-1993, 203 patients could be traced and were invited to participate in the study. One hundred and thirty-six patients replied (67%) and were compared with one hundred and thirty-six population based sex and age-matched controls. Patients and controls were evaluated with both a validated questionnaire as well as a study-specific questionnaire to assess bowel function. SF-36 was used for quality of life. Outcome in nine incontinence-related parameters, 10 constipation-related, 6 urogenital function-related, and 13 quality of life parameters were assessed in the patients and compared to the outcome of controls as well as to the type of anorectal malformations according to the Krickenbeck classification. RESULTS: The ARM-patients had an inferior outcome (P<0.05) for all incontinence parameters, 8 of 10 parameters for constipation, 2 of 6 for urogenital function and 7 of 13 quality of life parameters. Patients with rectobulbar and vestibular fistulas had the worst statistical outcome but patients with cloaca and rectoprostatic/bladder-neck fistula had worse outcome in absolute numbers. Forty-four patients (32%) reported incontinence of stool at least once a week and 16 (12%) had a permanent colostomy. CONCLUSIONS: The functional outcome and quality of life in adults with anorectal malformations are closely related to the type of malformation. A large proportion of the patients have persistent fecal incontinence, constipation and sexual problems that have a negative influence on their quality of life. Structured multidisciplinary follow-up of adults with anorectal malformations by pediatric and colorectal surgeons, as well as urologists and gynecologists is therefore advocated.

Clinical course of Crohn's disease during the first 5 years. Results from a population-based cohort in Sweden (ICURE) diagnosed 2005-2009.

OBJECTIVE: The aim of the study was to describe the medical treatment, change in phenotype, need for surgery and IBD-associated mortality during the first 5 years after diagnosis. MATERIAL AND METHODS: Patients diagnosed with Crohn's disease including all age groups in the Uppsala healthcare region in the middle of Sweden 2005-2009 were included in the study. Medical notes were scrutinised and patients contacted. Out of 269 patients, 260 (96.3%) could be followed for 5 full years or until death. RESULTS: The following drugs were used: 5-ASA 66.7%, systemic steroids 76.4%, antimetabolites 56.7% and anti-TNF 20.3%. Described with the Montreal classification, the proportion with inflammatory behaviour decreased from 78.1% to 74.0% from diagnosis to end of the observation, patients with stricturing behaviour increased from 13.0% to 15.4% and patients with penetrating behaviour increased from 8.9% to 10.6%. After the first year, 12.4% had been treated with intestinal resection or colectomy, a figure that increased to 14.8 after 5 years. Two patients suffered an IBD-related death. CONCLUSIONS: Compared to similar patient cohorts, the present study demonstrates that although the course of Crohn's disease seems difficult to change during the first year after diagnosis, the following years up to 5 years shows a more benign course than has usually been described earlier.

Low colectomy rate five years after diagnosis of ulcerative colitis. Results from a prospective population-based cohort in Sweden (ICURE) diagnosed during 2005-2009.

OBJECTIVE: The medical treatment of ulcerative colitis (UC) has seen a change towards a more active attitude during recent years, including both the use of more traditional drugs as well as new biological substances. In this epidemiological study we have evaluated the results of modern treatment of UC in a population-based cohort of patients including all age groups, with regard to relapse rate, colectomy and IBD-associated mortality. MATERIAL AND METHODS: Patients diagnosed with UC in the Uppsala health care region in the middle of Sweden during 2005-2009 were included in the study. Out of 524 patients, 491 (93%) could be followed for five full years or until death. RESULTS: Nineteen patients (3.9%) had died and two of these deaths could be attributed to UC (one postoperative death and one colonic carcinoma). The following drugs were used by the patients during the study period: 5-ASA (91%), systemic steroids (66%), immunomodulators (IMM), mainly thiopurines (26%) and anti-TNF (11%). During the observation period, 74% experienced at least one relapse and 5.3% were subjected to colectomy. Among patients <17 years at diagnosis, colectomy was performed in two (4.8%). CONCLUSIONS: Five years after diagnosis of ulcerative colitis, 5.3% had been subjected to colectomy and two patients (0.38%) had died because of the disease.

Neuropeptide S inhibits gastrointestinal motility and increases mucosal permeability through nitric oxide.

Neuropeptide S (NPS) receptor (NPSR1) polymorphisms are associated with enteral dysmotility and inflammatory bowel disease (IBD). This study investigated the role of NPS in conjunction with nitrergic mechanisms in the regulation of intestinal motility and mucosal permeability. In rats, small intestinal myoelectric activity and luminal pressure changes in small intestine and colon, along with duodenal permeability, were studied. In human intestine, NPS and NPSR1 were localized by immunostaining. Pre- and postprandial plasma NPS was measured by ELISA in healthy and active IBD humans. Effects and mechanisms of NPS were studied in human intestinal muscle strips. In rats, NPS 100-4,000 pmol·kg(-1)·min(-1) had effects on the small intestine and colon. Low doses of NPS increased myoelectric spiking (P < 0.05). Higher doses reduced spiking and prolonged the cycle length of the migrating myoelectric complex, reduced intraluminal pressures (P < 0.05-0.01), and increased permeability (P < 0.01) through NO-dependent mechanisms. In human intestine, NPS localized at myenteric nerve cell bodies and fibers. NPSR1 was confined to nerve cell bodies. Circulating NPS in humans was tenfold below the ∼0.3 nmol/l dissociation constant (Kd) of NPSR1, with no difference between healthy and IBD subjects. In human intestinal muscle strips precontracted by bethanechol, NPS 1-1,000 nmol/l induced NO-dependent muscle relaxation (P < 0.05) that was sensitive also to tetrodotoxin (P < 0.01). In conclusion, NPS inhibits motility and increases permeability in neurocrine fashion acting through NO in the myenteric plexus in rats and humans. Aberrant signaling and upregulation of NPSR1 could potentially exacerbate dysmotility and hyperpermeability by local mechanisms in gastrointestinal functional and inflammatory reactions.

Posterior sagittal anorectoplasty results in better bowel function and quality of life in adulthood than pull-through procedures.

BACKGROUND/PURPOSE: The short-term outcome of posterior sagittal anorectoplasty (PSARP) procedure has been reported to be better than after abdominoperineal or abdominosacroperineal (AP) procedures. This study aimed to investigate the long-term functional outcome and quality of life after PSARP in adulthood and compare with the outcome after AP procedures. METHODS: Twenty-four patients operated with PSARP at the Department of Pediatric Surgery, Uppsala, Sweden, from 1984 to 1993 were identified. They were compared with 20 patients that underwent AP pull-through procedures from 1974 to 1983. The patients were sent validated bowel function and quality of life (SF-36) questionnaires. Sixteen PSARP (median age 21, five females) patients and fourteen AP patients (seven abdominosacroperineal and seven abdominoperineal pull-throughs, median age 32, seven females) responded and were included in the study. RESULTS: The median Miller incontinence score was 1 (range 0-13) in the PSARP group and 10 (range 3-16) in the pull-through group (P=0.0042). The use of underwear protection and oral loperamide was significantly less frequent in the PSARP group (P=0.0096 and 0.0021 respectively). The SF-36 scores of Vitality, Mental health and Mental Cluster Scale were higher in the PSARP group (P=0.0291, 0.0500, 0.0421 respectively). CONCLUSIONS: PSARP results in superior bowel function and better quality of life in adulthood compared with AP procedures for the repair of anorectal malformations.

Comparison of different magnetic resonance imaging sequences for assessment of fistula-in-ano.

AIM: To assess agreement between different forms of T2 weighted imaging (T2WI), and post-contrast T1WI in the depiction of fistula tracts, inflammation, and internal openings with that of a reference test. METHODS: Thirty-nine consecutive prospective cases were enrolled. The following sequences were used for T2WI: 2D turbo-spin-echo (2D T2 TSE); 3D T2 TSE; short tau inversion recovery (STIR); 2D T2 TSE with fat saturation performed in all patients. T1WI were either a 3D T1-weighted prepared gradient echo sequence with fat saturation or a 2D T1 fat saturation [Spectral presaturation with inversion (SPIR)]. Agreement for each sequence for determination of fistula extension, internal openings, and the presence of active inflammation was assessed separately and blindly against a reference test comprised of follow-up, surgery, endoscopic ultrasound, and assessment by an independent experienced radiologist with access to all images. RESULTS: Fifty-six fistula tracts were found: 2 inter-sphincteric, 13 trans-sphincteric, and 24 with additional tracts. The best T2 weighted sequence for depiction of fistula tracts was 2D T2 TSE (Cohen's kappa = 1.0), followed by 3D T2 TSE (0.88), T2 with fat saturation (0.54), and STIR (0.19). Internal openings were best seen on 2D T2 TSE (Cohen's kappa = 0.88), followed by 3D T2 TSE (0.70), T2 with fat saturation (0.54), and STIR (0.31). Detection of inflammation showed Cohen's kappa of 0.88 with 2D T2 TSE, 0.62 with 3D T2 TSE, 0.63 with STIR, and 0.54 with T2 with fat saturation. STIR, 3D T2 TSE, and T2 with fat saturation did not make any contributions compared to 2D T2 TSE. Post-contrast 3D T1 weighted prepared gradient echo sequence with fat saturation showed better agreement in the depiction of fistulae (Cohen's kappa = 0.94), finding internal openings (Cohen's kappa = 0.97), and evaluating inflammation (Cohen's kappa = 0.94) compared to post-contrast 2D T1 fat saturation or SPIR where the corresponding figures were 0.71, 0.66, and 0.87, respectively. Comparing the best T1 and T2 sequences showed that, for best results, both sequences were necessary. CONCLUSION: 3D T1 weighted sequences were best for the depiction of internal openings and active inflammatory components, while 2D T2 TSE provided the best assessment of fistula extension.

Long-term outcome after free autogenous muscle transplantation for anal incontinence in children with anorectal malformations.

PURPOSE: Patients with high anorectal anomalies are often incontinent after reconstruction, particularly with the older forms of surgical treatment, that is, anorectal pull-through or Stephen's operations. In 1974, a new treatment for anal incontinence in children was introduced at the Akademiska Hospital: free autogenous muscle transplantation (FAMT) to the perirectal area. All the patients receiving FAMT were totally incontinent before the procedure and had no rectal sensitivity. The aim of this study was to evaluate the long-term functional outcome of this procedure. METHODS: Twenty-two patients (17 males) operated on with FAMT below the age of 15 years were identified through records. One of the patients had died, and 2 were not available for follow-up. The remaining 19 were sent a validated bowel function questionnaire, and 15 (78.9%) of 19 patients responded (12 males). These 15 patients were compared with 15 patients with the same sex, age, and a similar malformation from our patient database. RESULTS: At follow-up, after an average of 30 years postoperatively, 2 of 15 patients with FAMT had a stoma compared with 3 of 15 in the control group. The Miller incontinence score had a mean of 6.2 (median, 6; range, 0-15) in the FAMT group and 3.7 (median, 4; range, 0-12) in the control group. All patients in both groups could sense stool, and 11 of 13 patients in the FAMT group could distinguish between feces and flatus. CONCLUSIONS: The patients with FAMT had a slightly inferior anorectal function compared with the controls. Considering they were all totally incontinent before FAMT, we conclude that FAMT has an acceptable effect 30 years postoperatively. Therefore, we find that FAMT could be an alternative for anorectal malformation patients who are totally incontinent.

Acquired haemophilia A and Kaposi's sarcoma in an HIV-negative, HHV-8-positive patient: a discussion of mechanism and aetiology.

BACKGROUND: Acquired haemophilia A (AHA) is a rare bleeding disorder caused by an imbalance in the immune system leading to the production of factor VIII antibodies. In half of the cases, the underlying cause is not known. CLINICAL HISTORY: We report on a patient with AHA and Kaposi's sarcoma (KS), which is caused by the human herpes virus 8 (HHV-8). The patient presented with appendicitis and developed several severe post-operative haemorrhages. He spent 3 months in intensive care due to long and difficult infections. While recuperating on the ward, the patient developed KS in the lower extremities. He had a positive HHV-8 infection. DISCUSSION/CONCLUSION: Due to its latency and replication in the lymphoid system, HHV-8 is an ideal candidate for causing an imbalance in the immune system in susceptible patients. Our conclusion is that AHA was caused or prompted by the HHV-8 infection. Since HHV-8 viral infection is often subclinical, viral testing might be an important tool in acquired haemophilia diagnostics even when viral symptoms are absent.