Anatomical Gynecological Anomalies in Girls Born with Anorectal Malformations: A Retrospective Cohort Study of 128 Patients.

STUDY OBJECTIVE: In girls born with an anorectal malformation (ARM), anatomical gynecological anomalies (GA) may be present and might need treatment. Therefore, the aim of this study was to provide an overview of GA in girls born with ARM in our cohort. Additionally, diagnostic timing and methods for GA were assessed. METHODS: A retrospective mono-center study was performed from January 2000 to December 2022. All patients assigned female at birth were eligible for inclusion. GA were classified according to ESHRE/ESGE classification. Outcomes were the number of girls with GA with subsequent screening methods, factors associated with GA, and GA requiring treatment. Uni- and multivariable logistic regression analyses were performed to identify the association between baseline characteristics and the presence of GA. RESULTS: In total, 128 girls were included, of whom 30 (24.1%) had additional GA, with vaginal anomalies being present most often (n = 17). Fifty-six patients (43.8%) underwent full screening, and this number improved over time (37.7% before 2018 vs 72.7% after 2018; P = .003). Thirteen of 30 patients (43.3%) required surgical treatment for their GA, without the occurrence of postoperative complications. CONCLUSION: Additional GA were present in almost a quarter of the girls born with an ARM, with vaginal anomalies most often identified. Despite GA being most often found in patients with cloacal malformations, these anomalies were also identified in patients with other ARM types. Surgical treatment was required in almost half of the girls with GA. Therefore, this study emphasizes the importance of screening for GA in patients with an ARM, regardless of the ARM type.

Potential benefits of routine cystoscopy and vaginoscopy prior to reconstructive surgery in patients with an anorectal malformation.

PURPOSE: First, to assess the number of patients with anorectal malformations (ARM) in whom additional urological and/or gynecological anomalies were identified through routine screening with cysto- or vaginoscopy prior to reconstructive surgery. Second, to assess potential procedure-related complications. METHODS: Retrospective mono-center cohort study, including all ARM patients born between January 2019 and December 2022. Routine screening consisted of cystoscopy for male patients, with the addition of vaginoscopy for female patients. Chi-square was used to compare the screening percentages over time. RESULTS: In total, 38 patients were included, of whom 27 (71.1%) underwent cystoscopy ± vaginoscopy, without the occurrence of complications. Nine of 13 females (69.2%) underwent cysto- and vaginoscopy and 18 of 25 males (72.0%) underwent a cystoscopy. The percentage of patients that underwent these procedures improved over the 2 time periods (50.0% in 2019-2020 vs 90.0% in 2021-2022, p = 0.011). In 15 of 27 patients (55.6%) that underwent cystoscopy ± vaginoscopy, additional anomalies were found that were not identified through physical examination or US-kidney. CONCLUSIONS: In 56% of the patients that underwent cysto- ± vaginoscopy, additional anomalies were identified that were not with imaging studies or physical examination. This study emphasizes the potential benefit of routine cysto- and vaginoscopy in the diagnostic work-up of children with ARM. LEVEL OF EVIDENCE: III.

Spinal cord anomalies in children with anorectal malformations: a retrospective cohort study.

PURPOSE: First, to assess the number of spinal cord anomalies (SCA), specifically tethered spinal cord (TSC) in patients with anorectal malformations (ARM), identified with spinal cord imaging (i.e. spinal cord US and/or MRI). Second, to report outcomes after TSC treatment. METHODS: A retrospective mono-center study was performed. All ARM patients born between January 2000 and December 2021 were included. Screening for SCA consisted of spinal cord US and/or MRI. Radiology reports were scored on presence of SCA. Data were presented with descriptive statistics. RESULTS: In total, 254 patients were eligible for inclusion, of whom 234 (92.1%) underwent spinal cord imaging. In total, 52 (22.2%) patients had a SCA, diagnosed with US (n = 20, 38.5%), MRI (n = 10, 19.2%), or both US and MRI (n = 22, 42.3%), of whom 12 (23.5%) with simple, 27 (52.7%) intermediate, and 12 (23.5%) complex ARM types. TSC was identified in 19 patients (8.1%), of whom 4 (21.1%) underwent uncomplicated neurosurgical intervention. CONCLUSIONS: SCA were present in 22% of ARM patients both in simple, as well as more complex ARM types. TSC was present in 19 patients with SCA, of whom 4 underwent uncomplicated neurosurgical intervention. Therefore, screening for SCA seems to be important for all ARM patients, regardless of ARM type. LEVEL OF EVIDENCE: Level III.

The Importance of Screening for Additional Anomalies in Patients with Anorectal Malformations: A Retrospective Cohort Study.

BACKGROUND: In children with anorectal malformations (ARM), additional anomalies can occur within the VACTERL-association. Routine screening is of great importance for early identification and potential treatment. However, uniformity in screening protocols is lacking and only small cohorts have been described in literature. The aim of this study was to assess and describe a unique large cohort of ARM patients who underwent VACTERL screening in the neonatal period. METHODS: A retrospective mono-center cohort study was performed. Included were all neonates born between January 2000 and December 2020 who were diagnosed with ARM and screened for additional anomalies. Full screening consisted of x-ray and ultrasound of the spine, cardiac and renal ultrasound, and physical examination for limb deformities, esophageal atresia, and ARM. Criteria for VACTERL-classification were predefined according to the EUROCAT-definitions. RESULTS: In total, 216 patients were included, of whom 167 (77.3%) underwent full VACTERL-screening (66% in 2000-2006 vs. 82% in 2007-2013 vs. 86% in 2014-2020). Median age at follow-up was 7.0 years (IQR 3.0-12.8). In 103/167 patients (61.7%), additional anomalies were identified. Some 35/216 patients (16.2%) fulfilled the criteria of a form of VACTERL-association. In 37/216 patients (17.1%), a genetic cause or syndrome was found. CONCLUSIONS: The majority of ARM patients underwent full screening to detect additional anomalies (77%), which improved over time to 86%. Yet, approximately a quarter of patients was not screened, with the potential of missing important additional anomalies that might have severe consequences in the future. Forms of VACTERL-association or genetic causes were found in 16% and 17% respectively. This study emphasizes the importance of routine screening. LEVEL OF EVIDENCE: III.

Foreskin healing after distal hypospadias repair: Does stenting affect the outcome?

BACKGROUND: Distal penile hypospadias account for about 70% of all cases of hypospadias. There is a variety of operative techniques that could be performed when foreskin reconstruction is an option. The urethral stent is left in the urethra to prevent complications. OBJECTIVES: To determine whether the duration of stenting influences the healing of foreskin after distal hypospadias repair. MATERIAL AND METHODS: Data from 2 institutions was retrospectively analyzed. Inclusion criteria were as follows: 1) a modified meatal advancement glanuloplasty without tubularization of the urethral plate, 2) foreskin reconstruction and 3) follow-up - 12 months. All other types of reconstruction and re-do procedures were excluded. The period of urethral stenting was determined intraoperatively depending on the surgeon's preferences. Mean age at operation was 23.3 months. The cohort was divided into 3 groups. In Group I (G-I), no catheter was left or it was removed the next day after surgery. In Group II, the catheter was left for more than 5 days. In those 2 groups, the surgery was done by different surgeons. Group III consisted of 35 patients who had a stent for <2 days, and the procedure was performed by the same surgeon. The χ2 with Yates's correction and Pearson's χ2 tests were used for the statistical analysis. RESULTS: Overall, 11 patients had foreskin dehiscence and needed re-do surgery. None of the patients required operation because of foreskin stenosis. Complications occurred in 3 out of 33 patients (9%) in Group I, 2 out of 27 in Group II (7.4%) and 6 out of 35 in Group III (17%). There was no statistically significant difference between Groups I and II (p = 0.8144), nor between Groups I and III (p = 0.5344). In the non-parametric Pearson's χ2 test, no significant difference was found in such grouped data (p = 0.4239). CONCLUSIONS: Prolonged urethral stenting does not reduce the risk of a re-do foreskin surgery after hypospadias repair.

Orchidopexy for bilateral undescended testes: A multicentre study on its effects on fertility and comparison of two fixation techniques.

To evaluate fertility potential after orchidopexy for bilateral undescended testis and compare two surgical fixation techniques for effect on fertility. Men older than 22 years who had either tunica albuginea orchidopexy (TAO) or "no-touch" technique (NTO) in childhood for bilateral undescended testis (BUDT) were selected. Participants filled out a questionnaire followed by physical examination, had testicular ultrasound, blood sample and semen analysis. Statistical testing was performed using general linear modelling. Sixty-seven out of 166 individuals responded. Forty-nine completed the questionnaire, and nine (18.3%) reported having fathered children. Thirty-six showed up for further examination, 26 had TAO and 10 NTO. Impaired hormonal spermatogenesis regulation (34.6% vs. 20%), higher subfertility rate (46% vs. 20%) and lower means of motile spermatozoa (58.1 × 106 spz vs. 177.9 × 106 spz) were observed in the TAO versus the NTO group; none of these were statistically significant. Four (15.4%) of the TAO and two (20%) of the NTO group have azoospermia. Although the operation technique did not have a significant impact on fertility, unfavourable outcomes were more common after surgery involving the tunica albuginea of the testis. Larger sample sizes are needed to ascertain whether the trends favouring the NTO technique are of any significance.

Short-term Complications After Pyeloplasty in Children With Lower Urinary Tract Anomalies.

OBJECTIVE: To investigate whether children with lower urinary tract (LUT) anomalies are at greater risk for postoperative complications after laparoscopic pyeloplasty stented with a double-J catheter (JJC). MATERIALS AND METHODS: Prospectively collected data of laparoscopic pyeloplasty (LP) performed between 2006 and 2015 were analyzed. Inclusion criteria are (1) toilet-trained child and (2) unilateral dismembered pyeloplasty stented with a JJC done by the same surgeon. Our pyeloplasty protocol includes cystoscopy and retrograde pyelography. JJC is left in for 3weeks. Asymptomatic patients with infravesical LUT anomalies (a-LUTA) and those with history of LUT symptoms (LUTS) were identified. Any short-term complication was classified according to Clavien-Dindo. Fisher's exact test was used for statistical analysis. RESULTS: Fifty-four children (mean 9.8 years) were included. Ten of 54 patients had LUTS. In 4 of those 10, anatomical infravesical anomaly was found during cystoscopy. Accidental urethral anomaly was found in 11 patients (a-LUTA). The control group (CG) consisted of 33 patients. Postoperative hospital stay ranged from 1 to 8 days (mean 2 days). Overall complication rate was 8 of 54 (14%). Grade 1 complications occurred in 3 patients in the CG. Five patients had grade 3 complications (2 needed replacement of bladder catheter, and 3 had diversion of the upper tract). Those problems occurred in 1 of 10 patients with LUTS and 3 of 11 patients with a-LUTA compared to 1 of 33 in the CG. This difference was statistically significant (P < .05). CONCLUSION: Careful history should be taken in toilet-trained children before pyeloplasty. If any infravesical abnormality is discovered, internal diversion should probably be avoided. Special attention must be paid to bladder function in the postoperative period.

Laparoscopic burch colposuspension in children: technical challenges and primary results.

OBJECTIVE: To present the primary results of laparoscopic, Burch-type colposuspension (LBC) in children. PATIENTS AND METHODS: LBC was performed in eight children (mean age, 14 years) with urinary incontinence after failed extensive urotherapy program. In all patients, bladder neck (BN) incompetence was proven by clinical observation, perineal ultrasound (US), and video-urodynamic study (V-UDS). The LBC was performed with patients in the lithotomy position by means of three 5-mm ports. The anterior wall of the vagina, lateral to the BN, was mobilized, and the vaginal wall was bilaterally lifted and sutured to Cooper's ligament, resulting in elevation and fixation of the BN. A catheter was left for 4 days. The mean operation time was 101 minutes (range, 56-150 minutes), and the follow-up time was >6 months. RESULTS: The postoperative period was uneventful for all patients. Shortly after the procedure, 5 patients (62.5%) were dry, and 1 patient improved (12.5%). After 6 months, 3 patients (37.5%) were dry, and 2 (25%) improved. In 3 wet patients, control US and V-UDS focused on the BN revealed hypermobility and persistent flat vesicourethral angle (VUA) in 1 patient, hypermobility with a good restoration of the VUA in 1 patient, and good fixation with good restoration of the VUA in 1 patient. CONCLUSIONS: LBC is a challenging procedure in children because of the small operation field. This procedure can be considered to cure refractory stress urinary incontinence in children with BN insufficiency when noninvasive methods have failed.

Intestinal malrotation and volvulus in adult life.

INTRODUCTION: Midgut volvulus due to intestinal malrotation is a rare cause of intestinal obstruction when occurring in adult life. This paper documents the difficulties in reaching an early diagnosis. PRESENTATION OF CASE: We describe the case of an 85-year-old man with non-specific abdominal complaints for 20 years, who presented with sudden onset central abdominal pain. An acute median laparotomy under general anaesthesia was performed during which a 360° clockwise rotation of the small bowel around the mesenteric pedicle of the superior mesenteric artery and vein was found. DISCUSSION: Malrotation is considered any deviation of the normal rotation of the midgut in embryological development, causing intermittent episodes of gastrointestinal obstruction or acute events of midgut volvulus. Although mainly a paediatric diagnosis, some cases do present in adult life. Radiologic investigations include: upper gastrointestinal contrast studies, Doppler sonography and a contrast enhanced CT of the abdomen. If a true malrotation is diagnosed or found by coincidence, a Ladd's procedure is advised, even if the patient is asymptomatic. There is no proven surgical strategy for preventing the recurrence of small bowel volvulus in case of malfixation of the midgut. CONCLUSION: Non-specific recurrent abdominal complaints in adults of any age should raise suspicion of the possibility of a midgut malrotation or malfixation with or without intermittent volvulus. This case highlights the importance of diagnosis in an early stage.

Vesicoscopic bladder neck procedure in children: what we have learned from the first series.

OBJECTIVE: To assess the results of endoscopic bladder neck procedure on the anterior bladder wall in children. MATERIALS AND METHODS: Surgery is done in the lithotomy position using three 5-mm ports. The bladder is insufflated with CO2. A U-shaped incision is made around the bladder neck. A mucosal strip is tabularized around a 12 French catheter and covered with the second layer of mucosa. Twenty procedures were performed on 18 patients (mean age, 9.8 years), and the follow-up period was >1 year (mean, 34 months). Mean operation time was 149 minutes. Twelve patients had neurogenic lower urinary tract dysfunction. Fifteen patients failed earlier bladder neck surgery, predominantly fascia sling suspension. Preoperatively, all patients had low detrusor leak point pressure. Clean intermittent catheterization (CIC) was resumed through the urethra in 11 patients and through a stoma in 6 patients. RESULTS: Two patients needed conversion because of CO2 leakage. Six patients were dry, and 4 improved in the short term (3-6 months). After 1 year of follow-up, 2 patients were dry, and 6 improved. In the long term, 1 (9%) out of 11 patients who were catheterized through the urethra was dry, and 3 of the 11 patients (27%) improved. Of the 6 patients with a CIC stoma, 1 (17%) was dry, and 3 (50%) improved. CONCLUSIONS: Endoscopic bladder neck surgery is, for most patients, a minor operation, but the long-term results are disappointing. The construction of a continent channel for CIC can improve the outcome when anterior bladder neck plasty is performed.

Efficacy and safety of urethral de-obstruction in boys with overactive bladder complaints.

OBJECTIVE: To gain insight into the efficacy and safety of urethral de-obstruction in boys with overactive bladder (OAB) complaints refractory to conservative treatment. MATERIALS AND METHODS: All boys, older than 5 years, referred in 2009 for OAB complaints were included, n = 180. Nine had abdominal or penile pain as predominant complaint. 82% were tertiary referrals after unsuccessful conservative treatment with antimuscarinic medication and/or urotherapy for OAB. In 121, urethral obstruction was urodynamically proven or seriously suspected, and they underwent urethrocystoscopy with relief of obstruction, when present. Average duration of unsuccessful conservative pre-treatment in this group of patients was 1.2 years. Postoperative results, in terms of relief of complaints, were analyzed. Safety was assessed by analyzing those patients who had a secondary transurethral procedure in the same year, or in the 3 years after primary treatment. RESULTS: Of 106 boys with OAB, urge incontinence or therapy-resistant bedwetting, after de-obstruction 33 became free of complaints and 39 showed significant improvement, totaling 72 (68%); dry after additional urotherapy 11 (10%); no change 21 (20%). Nine boys had de-obstruction because of penile or abdominal pain, with 5 completely cured after the procedure. Follow-up treatment was cognitive training in 39, temporary anticholinergic treatment in 26 and CIC in 2 cases. Recurrence of obstruction was seen in 10% during the 3-year follow-up period. CONCLUSION: After failure of conservative therapy, one should actively look for any urethral obstruction as underlying cause of OAB. For such patients, urethral de-obstruction is highly effective, with only a few minor late complications resulting in recurrence of obstruction.

Detrusorectomy reduces the need for augmentation and use of antimuscarinics in children with neuropathic bladders.

OBJECTIVE: To assess bladder behaviour and long-term outcome after detrusorectomy in children. MATERIAL AND METHODS: Between 1990 and 2001, 49 detrusorectomies were performed (mean follow-up: 9.6 years) in children with neuropathic bladders (mean age at surgery: 9.8 years). Urodynamic study (UDS) was done before surgery (pre-UDS) and after surgery (UDS-1 and UDS-2). Bladder behaviour was assessed as good, fair or poor depending on the volume and intravesical pressure. In all patients, oxybutynin and clean intermittent catheterization were used preoperatively. In 24 patients, good bladder compliance and capacity were seen before detrusorectomy. RESULTS: Good and fair outcomes were observed in 35 (71%) patients at 1 year and in 39 (79%) patients 6 years after detrusorectomy. In 30 (60%) patients, there was hardly any difference between the first and second follow-up. In 9 (18%) patients, formal bowel bladder augmentation was necessary: in 6 (12%) because of poor compliance and in 3 because of small bladder volume and incontinence. Seven patients improved during follow-up, 5 of them after resuming oxybutynin. In 11 patients, oxybutynin could be stopped, and in 2 the dosage could be reduced to once daily. CONCLUSIONS: The good short-term results of detrusorectomy generally remain unchanged at long-term follow-up. Detrusorectomy can reduce the need for antimuscarinics, and the need for formal bladder augmentation in selected cases.

The Malone antegrade continence enema procedure: the Amsterdam experience.

BACKGROUND: The Malone antegrade continence enema (MACE) procedure has been previously described as a safe and effective option for the treatment of children with chronic defecation disorders when maximal medical therapy and conventional treatment have failed. PURPOSE: To evaluate clinical success, complications, and quality of life of children with chronic defecation disorders with a MACE stoma. METHODS: A retrospective analysis of 23 patients who underwent the construction of a MACE stoma was performed. Preoperative and postoperative data were evaluated. A specific questionnaire was used to assess patient satisfaction. RESULTS: A significant increase was found in defecation frequency (1.0 [range, 0-4] pretreatment vs 5.5 [range, 0-28] posttreatment per week; P < .006) and a significant decrease in fecal incontinence frequency (10 [range, 0-14] pretreatment vs 0 [range, 0-14] posttreatment per week; P < .034). Postoperative complications of the MACE procedure were fecal leakage (43%), wound infection (52%), and stomal stenosis (39%). A total of 86% of the patients were satisfied with the results of the Malone stoma (n = 21). CONCLUSIONS: The MACE procedure is an effective treatment in children with intractable defecation disorders. Postoperative complications are, however, not uncommon. Further refinement of the technique focused to reduce the complication rate is necessary to expand the application of this approach.

Anterior or posterior sagittal anorectoplasty without colostomy for low-type anorectal malformation: how to get a better outcome?

BACKGROUND/PURPOSE: Usually, anorectal malformations (ARM) are treated in 2 or 3 stages for fear of disturbed wound healing and subsequent damage to the anal sphincter complex. The aim of this study was to assess the feasibility, safety, advantages, and follow-up of an anterior or posterior sagittal anorectoplasty in low-type ARM (rectoperineal or rectovestibular), performed without colostomy. MATERIALS AND METHODS: Prospective collection of data regarding demographics, VACTERL (Vertebral-, Anal-, Cardiac-, Tracheo-Esophageal-, Renal-, Limb malformations) screening, perioperative measurements, surgeons, and complications. RESULTS: In 35 consecutive children (13 boys, 22 girls), repair of a low-type ARM was performed without colostomy. There were 13 boys and 10 girls with a rectoperineal and 12 girls with a rectovestibular fistula. The median age at operation was 4 months (range, 0-73 months); 34% being performed in the newborn period. Seventeen children had one or more other congenital anomaly. Preoperatively, all patients had rectal washouts with oral and rectal neomycin, and perioperative antibiotics, either 24 h (prophylaxis) or for 2 to 5 days. An anterior or posterior sagittal anorectoplasty was performed. Postoperatively, 9 children had no enteral feeding and total parenteral nutrition (TPN). All children had postoperative anal dilatations according to the Peña scheme. Two children (both with rectoperineal fistula) had a wound abscess; in the first child (with renal insufficiency), a colostomy was performed and in the other child a successful correction of the anoplasty was done. In 7 children (4 rectoperineal, 3 rectovestibular fistulae), the anus eventually healed after minor wound dehiscence. There was 1 anal stricture, after a median follow up of 14 months (range, 1-84 mo). After therapeutic antibiotics (2-5 days), 11% (2/18) had some degree of wound infection, versus 41% (7/17) after either no antibiotics or after prophylactic antibiotics (24 hours). Patients with TPN did not seem to profit with regard to wound healing and one patient experienced a central line related sepsis. At last follow-up, 12 children needed regular laxatives and/or enemas. Anal dilatations were well accepted above 6 months, and a trend was seen towards less need for laxatives when dilatations were continued longer. CONCLUSION: Repair of a low-type ARM without colostomy, with therapeutic antibiotics, and followed by a long period of postoperative anal dilatations has low morbidity and good outcome, which does not seem to be improved with TPN.