Reconsidering Diagnosis, Treatment, and Postoperative Care in Children with Cloacal Malformations.

Cloacal malformations are among the most complex types of anorectal malformation and are characterized by the urological, genital, and intestinal tracts opening through a single common channel in the perineum. Long-term outcome is affected by multiple factors, which include anatomical variants of the malformation itself, associated anomalies, and the surgical approach. Reconsidering these variables and their influence on "patient important" function might lead to strategies that are more outcome-driven than focused on the creation of normal anatomy. Key outcomes reflect function in each of the involved tracts and the follow-up needed should therefore not only include the classical fields of colorectal surgery and urology but also focus on items such as gynecology, sexuality, family-building, and quality of life as well as other psychological aspects. Involving patients and families in determining optimal treatment strategies and outcome measures could lead to improved outcomes for the individual patient. A strategy to support delivery of personalized care for patients with cloacal malformations by aiming to define the best functional outcomes achievable for any individual, then select the treatment pathway most likely deliver that, with the minimum morbidity and cost, would be attractive. Combining the current therapies with ongoing technological advances such as tissue expansion might be a way to achieve this.

Two versus five days of antibiotics after appendectomy for complex acute appendicitis (APPIC): study protocol for a randomized controlled trial.

BACKGROUND: Acute appendicitis is one of the most common indications for emergency surgery. In patients with a complex appendicitis, prolonged antibiotic prophylaxis is recommended after appendectomy. There is no consensus regarding the optimum duration of antibiotics. Guidelines propose 3 to 7 days of treatment, but shorter courses may be as effective in the prevention of infectious complications. At the same time, the global issue of increasing antimicrobial resistance urges for optimization of antibiotic strategies. The aim of this study is to determine whether a short course (48 h) of postoperative antibiotics is non-inferior to current standard practice of 5 days. METHODS: Patients of 8 years and older undergoing appendectomy for acute complex appendicitis - defined as a gangrenous and/or perforated appendicitis or appendicitis in presence of an abscess - are eligible for inclusion. Immunocompromised or pregnant patients are excluded, as well as patients with a contraindication to the study antibiotics. In total, 1066 patients will be randomly allocated in a 1:1 ratio to the experimental treatment arm (48 h of postoperative intravenously administered (IV) antibiotics) or the control arm (5 days of postoperative IV antibiotics). After discharge from the hospital, patients participate in a productivity-cost-questionnaire at 4 weeks and a standardized telephone follow-up at 90 days after appendectomy. The primary outcome is a composite endpoint of infectious complications, including intra-abdominal abscess (IAA) and surgical site infection (SSI), and mortality within 90 days after appendectomy. Secondary outcomes include IAA, SSI, restart of antibiotics, length of hospital stay (LOS), reoperation, percutaneous drainage, readmission rate, and cost-effectiveness. The non-inferiority margin for the difference in the primary endpoint rate is set at 7.5% (one-sided test at ɑ 0.025). Both per-protocol and intention-to-treat analyses will be performed. DISCUSSION: This trial will provide evidence on whether 48 h of postoperative antibiotics is non-inferior to a standard course of 5 days of antibiotics. If non-inferiority is established, longer intravenous administration following appendectomy for complex appendicitis can be abandoned, and guidelines need to be adjusted accordingly. TRIAL REGISTRATION: Dutch Trial Register, NTR6128 . Registered on 20 December 2016.

Urological and sexual outcome in patients with Hirschsprung disease: A systematic review.

PURPOSE: There is a paucity of recent evidence regarding long-term urological and sexual outcomes following surgery for Hirschsprung disease (HD). We aimed to undertake a systematic review of all HD literature to define these outcomes. MATERIALS AND METHODS: A systematic literature search was conducted on studies from 1966 to 2014. Relevant articles were assessed for urological/sexual operative complications and functional sequelae. Studies were analysed in qualitative (Rangel score) and quantitative syntheses. RESULTS: Initially 257 reports were assessed, with 24 studies were eligible for inclusion (1972-2014). Mean study quality was 16.5 ± SD 4.8 (range 6-23), indicating overall fair/poor quality. Ten studies (1021 patients) reported operative complications, with ureteric/urethral/vaginal injury occurring in seven (0.7%) patients. In three studies, the primary outcome was urological functional assessment. From 17 studies, 52/2546 patients (2.0%) had reported urinary incontinence. In infants, absent spontaneous erections post-operatively was reported in 3/203 patients (1.5%, 5 studies); of these 3, parents did not note spontaneous erections pre-operatively either. In older patients, erectile dysfunction occurred in 6/498 (1.2%) males. Other sexual outcomes were reported in 10 studies, with 5/10 studies (416 patients) reporting no erectile dysfunction. In the other studies reports ranged from non-specified sexual dysfunction in one study to diverse sexual related problems in nine (7.8%) of their patients in another. CONCLUSIONS: Urological/sexual outcomes are rarely reported after HD surgery (24 studies over 42 years). Study quality is usually poor and a large proportion of the studies are more than 30 years old. In the majority of series it is unclear whether urological and sexual function impairments were not present or if they were not assessed. Prospective reporting of urological/sexual outcome is required, in particular in the era of new surgical techniques/approaches to HD.

Colectomy in pediatric ulcerative colitis: A single center experience of indications, outcomes, and complications.

BACKGROUND/PURPOSE: There is a paucity of data on outcomes and complications of colectomy for pediatric ulcerative colitis (UC). This study reports the experience of a regional center for 18years. METHODS: Patients were identified from a prospective database and data obtained by note review. Median height/weight-SDS were calculated preoperatively and postoperatively. Data are expressed as median values (range). RESULTS: 220 patients with UC (diagnosed <17years) were identified, and 19 (9%) had undergone colectomy. Age at diagnosis was 11.6years (1.3-16.5), and 42% of patients were male. Time from diagnosis to surgery was 2.2years (0.1-13.1). All patients had failed maximal medical therapy. Fifteen patients had urgent scheduled operation, and 4 had emergency procedures, with 2 for (11%) acute-severe colitis (1 Clostridium difficile colitis) and 2 for acute-severe colitis with toxic dilatation. All initial procedures were subtotal-colectomy with ileostomy. Nine patients (47%) had early complications (during initial admission), 7 (37%) requiring reoperation. Six (32%) had late complications, with 5 requiring laparotomy. No patients had both early and late complications. Height-SDS was -0.27 before surgery and -0.23 (maximal follow-up). Weight-SDS was 0.32 and 0.05 (maximal follow-up). CONCLUSION: Approximately 1/11 children with UC required colectomy during childhood. Half of patients had acute complications, and 1/3 of patients required another operation during their first admission. 1/3 of patients developed late complications.

Antenatally diagnosed right-sided stomach (dextrogastria): A rare rotational anomaly.

AIM: Antenatal detection of right-sided stomach (dextrogastria) is rare, and its significance in regards to intestinal rotation is unclear. We aimed to review all cases of antenatally-diagnosed dextrogastria in our regional fetal medicine unit over 10years. METHODS: A retrospective case-note review of patients identified from a prospectively-maintained database was performed. RESULTS: Twenty cases of antenatally-diagnosed dextrogastria were identified from 2004 to 2014. There were 8 terminations and 1 intra-uterine death. One patient has no post-natal information obtainable. Ten infants were live-born, and 2 died secondary to cardiac disease in the neonatal period. All had significant cardiac/vascular anomaly on postnatal assessment, including the 3 neonates in whom dextrogastria was the only antenatal finding. Two neonates developed bilious vomiting and underwent Ladd's procedure. Operative findings were dextrogastria/malrotation in both. A third child had gastro-oesophageal reflux, and contrast demonstrated stable duodenal/midgut position. This child has not developed symptoms attributable to malrotation and not undergone surgery. All 3 of these infants had asplenia or polysplenia and were managed with antibiotic prophylaxis/immunisation. Five children in the series were not investigated for malrotation and have not come to surgical attention (one is known to be asplenic). CONCLUSION: Antenatally-detected dextrogastria, even if apparently isolated, was always associated with postnatal significant cardiovascular anomaly, splenic abnormality or situs inversus. This may be important for antenatal counselling. We currently recommend postnatal echocardiography and splenic assessment, but reserve GI investigation/intervention for symptomatic malrotation owing to potential significant cardiac comorbidity.

Perioperative Nutritional Management in Congenital Perineal and Vestibular Fistulas: A Systematic Review.

BACKGROUND: Surgical advancements have led to improved outcomes for children with congenital anorectal malformations with vestibular and perineal fistulas. However, the effect of perioperative nutritional management is debated and guidelines have not yet been established. OBJECTIVE: The study aims to give an overview of available published evidence, regarding the impact of different perioperative nutritional management protocols on surgical outcome. METHODS: A systematic literature review was conducted using PubMed, Embase, Cochrane Library, and CINAHL databases. All original articles concerning perioperative nutrition in children with vestibular and perineal fistulas were included. Methodological quality was assessed with the Rangel score. Included studies were subdivided into two groups: early enteral nutrition and prolonged fasting with or without parenteral nutrition. RESULTS: The database search resulted in 768 publications. Nine studies were eligible for inclusion. Wound complications were present in 56 of the 1,557 patients (4%) in whom this was assessed, and were more frequently seen in the prolonged fasting group (2 vs. 10%, p < 0.0001). Regarding the long-term outcome, constipation (grade II-III) was seen in 4% of the early feeding group, compared with 13% in the prolonged fasting group (p < 0.0001). CONCLUSION: This systematic review presents an overview of studies reporting on perioperative nutritional management in children with perineal and vestibular fistulas. Although study quality is low and study heterogeneity may also influence our results, early enteral feeding seems to be the preferable postoperative feeding strategy. Both early wound complications as well as long-term complications, in terms of clinically relevant constipation, seem to be lower in the early enteral feeding group. However, a prospective randomized, multicentered trial should be initiated to draw definitive conclusions regarding this matter.

Effects of reconstructive surgery on bladder function in patients with anorectal malformations.

PURPOSE: Bladder dysfunction is common in patients with anorectal malformations and can be congenital or acquired as a consequence of surgery. We investigated the effects of surgical correction of anorectal malformations on bladder function. MATERIALS AND METHODS: The charts of all 341 patients who underwent surgery at our center between 1990 and 2010 were retrospectively analyzed for preoperative and postoperative videourodynamics. A total of 52 patients were eligible for study inclusion. Each assessment was scored according to International Children's Continence Society standards. RESULTS: Urodynamic study indicated normal bladder function preoperatively in 36 patients (69%) and postoperatively in 37 (71%). Median bladder emptying efficiency and relative bladder capacity changed significantly after posterior sagittal anorectoplasty. Bladder function according to International Children's Continence Society standards did not change postoperatively in 43 patients (83%). In 1 of 4 patients with deterioration of bladder function the deterioration could be attributed solely to surgery. Clinical outcome was available in 38 patients and showed complete urinary continence with spontaneous voiding in 24 (63%). Seven of 25 patients (28%) with preoperative videourodynamics indicating normal bladder function demonstrated dysfunctional voiding at latest followup. CONCLUSIONS: Urodynamic and clinical outcomes after anorectal malformation repair are good, with 63% of patients being continent of urine. Urodynamic studies are of limited value in preoperative settings in these patients. Current techniques of reconstructive surgery for anorectal malformations seem to preserve bladder function in the majority of patients.

Urogenital function after cloacal reconstruction, two techniques evaluated.

OBJECTIVE: Current surgical techniques for cloacal reconstruction are posterior sagittal anorecto vagino urethroplasty (PSARVUP) and posterior sagittal anorectoplasty (PSARP) with total urogenital mobilization (TUM). The aim of this study was to explore the results of reconstructive cloaca surgery in the Netherlands and evaluate urogenital function after PSARVUP and TUM. PATIENTS AND METHODS: Medical records from five pediatric surgical departments in the Netherlands were studied for patients with cloacal malformations treated between 1985 and 2009. Forty-two patients were eligible, and patients with short common channels were categorized into PSARVUP and TUM groups. Groups were compared using Fisher's exact test. RESULTS: Median age at time of surgery was 9 months (range 1-121). In 24 patients (57%) a PSARVUP was done, in 18 patients (43%) TUM. Median follow-up was 142 months (range 15-289). At follow-up spontaneous voiding was seen in 29 patients (69%). Clean intermittent catheterization (CIC) was needed in 14 patients (33%); a urinary diversion was created in 10 patients (24%). In total 32 patients (76%) were dry with no involuntary loss of urine per urethra. Recurrent urinary tract infections were seen in 23 patients (55%). When comparing PSARVUP and TUM groups in our series of patients with short common channels, there were no differences in urological outcome. Normal menstruation was present in 11 of the 20 patients who reached puberty (55%). CONCLUSION: Urogenital functional outcome after reconstructive surgery for cloacal malformations was similar in PSARVUP and TUM groups in patients with short common channels. A thorough urological follow-up is needed to establish the long-term bladder function and urinary incontinence results to prevent long-term risks of recurrent UTI. Albeit without differences between PSARVUP and TUM groups, 45% of the patients present with abnormal or absent menstruations. Gynecological follow-up is mandatory in all patients with cloacal malformations 6 months after the first sign of puberty.

Early versus late reconstruction of cloacal malformations: the effects on postoperative complications and long-term colorectal outcome.

INTRODUCTION: Patients with a cloacal malformation generally undergo reconstructive surgery within the first years of life. However, the ideal age for surgery has rarely been mentioned. The aim of this study was to report differences in outcome between early (<6 months) and late repair of cloacal malformations. METHODS: Charts of patients with a cloacal malformation treated in 5 pediatric surgical centers between 1985 and 2009 were retrospectively studied for associated anomalies, postoperative complications, and colorectal and urological outcome. RESULTS: Forty-two patients were eligible for this study, giving a mean exposure of less than 1 patient yearly per center. Forty-five percent of the patients had a short common channel (>3 cm), and 14% had a long common channel. Length of common channel was missing in 41% of the patients. Median age of the cloacal reconstruction was 9 months (range 1-121 months). Twelve patients (29%) underwent an early surgical repair (within the first 6 months of age; median 3 months), and 30 (71%) patients underwent a late repair (after 6 months of age; median 14 months). Eighteen postoperative complications (<30 days) had been documented in 15 patients (35%), with significant more perineal wound dehiscences in patients with an early repair (42% vs. 10%, p=0.031). There were no differences in complication rate between patients with short and long common channels. Mean follow-up was 142 months (range 15-289). At the last follow-up, 10 patients (24%) had voluntary bowel movements. Fourteen patients (33%) had complaints of soiling, 25 (60%) were constipated, with no differences between the early and late repair groups. Patients in the late repair group as well as the group of patients with a short common channel were more frequently able to void spontaneously. CONCLUSIONS: Postoperative complications are common in patients with cloacal malformations. Early repair is associated with more wound dehiscences, however, without affecting long-term functional outcome. All centers had limited annual exposure of less than 1 patient. In these clinical settings, ideal age of cloacal reconstruction seems to be between 6 and 12 months. In general, centralized care for these complex malformations may be the crucial factor for reducing postoperative complications and better long-term outcome.

Long-term follow-up of functional outcome in patients with a cloacal malformation: a systematic review.

BACKGROUND: Reconstructive surgery is performed in patients with cloacal malformations to achieve anorectal, urological, and gynecological function. The aim of this study was to evaluate the functional outcome of cloacal malformation repair as reported in literature. METHODS: A systematic literature search was conducted according to PRISMA guidelines using PubMed, EMbase, and Web-of-Science. Records were assessed for the reporting of functional outcomes, which was divided into anorectal, urological, or gynecological function. Studies were used in qualitative (Rangel score) and quantitative syntheses. RESULTS: Twelve publications were eligible for inclusion. Voluntary bowel movements were reported in 108 of 188 (57%), soiling in 146 of 205 (71%), and constipation in 31 of 61 patients (51%). Spontaneous voiding was reported for 138 of 299 patients (46%). 141 of 332 patients (42%) used intermittent catheterization, and 53 of 237 patients (22%) had a urinary diversion. Normal menstruations were reported for 25 of 71 patients (35%). Centers with limited experience reported similar outcome compared to centers with more experience (≥1 patients/year). CONCLUSION: In this review we present functional outcome of the largest pooled cohort of patients with cloacal malformations as reported from 1993 to 2012. Functional disturbances are frequently encountered in anorectal, urological, as well as gynecological systems. Reporting of functional outcome in these patients should improve to increase knowledge about long-term results in patients with this rare malformation and to reach higher study quality. Especially, sacral and spinal anomalies should always be reported given their impact on functional outcome. Specialized care centers may be of great importance for patients with rare and complex conditions.

"This bicycle gives me a headache", a congenital anomaly.

BACKGROUND: [corrected] The combination of a presacral mass, a sacral bone deformity, and an anorectal malformation are also known as the Currarino triad or Currarino syndrome. The syndrome is associated with a very high rate of severe and intractable constipation and urinary incontinence. However, it can also result in less common complaints and symptoms. Although the syndrome is known since 1981 and the involved genes are clarified to a great extent, the diagnosis may be delayed or missed if unrecognized. CASE PRESENTATION: A 24-year old female presented with periodical headaches. She was born with an imperforate anus, absent rectum and colon, double bladder, and sacral defect. Soon after birth she underwent several surgical procedures for anorectal and bladder reconstructions. The patient now came to her pediatric urologist for urinary incontinence and mentioned severe headaches on the side, particularly when riding a bike. Finally, she solved her headache problem by stopping to ride her bicycle.On physical examination no abnormalities were found except the ileostomy that was present ever since soon after birth and her urinary incontinence. Blood tests showed no abnormalities. Additional MRI showed a large and previously not known anterior meningocele at the level of the sacrum. Surgical treatment consisted of closure of the dura by posterior approach. CONCLUSION: In this case report we describe the late discovery with an atypical presentation of an anterior meningocele in a young adult with urinary incontinence, a sacral defect, an anorectal malformation and headaches during bicycle riding. After surgical treatment of our patient the meningocele regressed. Three months after successful surgery she had no complaints and was able to ride a bike again.

Treatment of adults with unrecognized or inadequately repaired anorectal malformations: 17 cases of rectovestibular and rectoperineal fistulas.

STUDY OBJECTIVE: To analyze all cases of congenital rectovestibular and rectoperineal fistulas diagnosed and treated later in life, and to describe presenting complaints, treatment, and outcome. DESIGN: Retrospective cohort study. SETTING: Pediatric surgery departments of 3 major referral centers in the US and Europe. PARTICIPANTS: Seventeen women with untreated or inadequately treated rectovestibular or rectoperineal fistulas. INTERVENTIONS: Analyses of all eligible patients: charts were analyzed for the classification of the malformation, main complaints, continence, sexual function, indications for surgery, associated anomalies, surgical procedure, complications, and outcome. MAIN OUTCOME MEASURES: Patients' complaints, continence, constipation, and sexual function. RESULTS: Major complaints at time of diagnosis were fecal incontinence, and concerns for hygiene and cosmesis. All patients were repaired by a posterior sagittal approach. In all but 1 patient the complaints disappeared or improved after surgery. CONCLUSIONS: Anorectal malformations in females are congenital malformations mostly seen and treated in early childhood. If unrepaired or inadequately repaired the patient, when reaching adulthood, can suffer from significant morbidity. Surgical treatment is similar as in childhood and has an excellent clinical outcome.