Urological Outcomes in Post-Pubertal Patients With Myelomeningocele: A Single-Center Retrospective Study.

BACKGROUND: Recent decades have seen changes in the urological treatment of myelomeningocele (MMC). We aimed to evaluate the urological outcomes in post-pubertal patients and to clarify associations with walking status, hydrocephalus, and sex. METHODS: A retrospective study of 103 MMC patients at their final pediatric urological control. Urological procedures, the necessity for Clean Intermittent Catheterization (CIC) and anticholinergic medication, the state of continence, renal ultrasound findings, and serum creatinine values were assessed. RESULTS: The median age of the patients was 18 years (IQR 16.7-19.6), with 51 (49.5%) being female. Renal function was preserved in all but of one, who presented with mild hydronephrosis. 38 patients walked without assistance, 46 used wheelchairs. Most patients (93%) utilized CIC, and 83% had interventions for overactive or poorly compliant bladder, including anticholinergic medication (47%), Botox treatments (35%), or bladder augmentation (36%). Nearly half (45%) had undergone bladder neck procedures. Continence status revealed 55% fully continent, 18% were rarely incontinent, and 26% were incontinent daily, with most episodes limited to droplet leakage. Incontinence was not associated with the ambulatory status, hydrocephalus, or sex (p = 0.08, >0.99, and 0.07 respectively). CONCLUSIONS: Renal function was effectively maintained with our treatment strategy; however, daily incontinence episodes occurred in one out of four patients, with an additional 18% experiencing occasional rare incontinence episodes. Incontinence, when present, was mostly mild. We found no association between patient characteristics, treatment approach, and continence. Emphasizing incontinence treatment becomes a mainstay in future studies. LEVEL OF EVIDENCE: IV.

Management of sphincter insufficiency in patients with neurogenic bladder and bladder augmentation.

PURPOSE: To investigate the need and efficacy of treatment of bladder neck procedures in patients with neurogenic bladder and augmentation. METHODS: The hospital database was reviewed for patients undergoing enterocystoplasty because of neurogenic bladder during 1990-2019. Diagnoses of patients as well as frequency, type, and efficacy of treatment of sphincter insufficiency were evaluated. RESULTS: Thirty-seven of 87 patients (43%) underwent surgery because of sphincter insufficiency. The median age at bladder augmentation was 11.9 years (IQR 8.5-14.8), and at the last control, 21.8 years (IQR 18.9-31.1). Bladder neck injections (BNI) were performed for 28 patients, fascial sling operation for 14 patients, and bladder neck closure (BNC) was done for five females. Full continence was achieved in 10/28 (36%) patients with one or repeat BNIs and 9/14 (64%) with sling operation. The outcome of BNIs and sling operations was similar in both sexes. All five female patients with BNC became continent. At the end of follow-up, 64 (74%) patients were dry, 19 (22%) had occasional incontinence episodes, and 4 (5%) had daily incontinence episodes necessitating pads. CONCLUSIONS: Treatment of sphincter insufficiency is challenging in patients with bladder augmentation and neurogenic disease. Only 74% of our patients became fully continent despite treatments for sphincter insufficiency.

Additional surgery in patients with bladder augmentation.

INTRODUCTION: Bladder augmentation is an option to protect kidneys and treat incontinence in frequent neurogenic and congenital non-neurogenic disorders. However, patients may need also other procedures to treat incontinence or to aid catheterizations. In addition, the patients are prone to complications. OBJECTIVE: To evaluate complications or additional surgeries in augmentation cystoplasty according to the etiology of the bladder dysfunction. STUDY DESIGN: Data on additional surgical procedures related to bladder augmentation were collected from 128 patients who had undergone enterocystoplasty between 1990 and 2019, with a median age of 10.1 years (IQR 7.2-12.8) and a follow-up time of 11.8 years (IQR 5.7-18.6) after augmentation. RESULTS: Eighty-three patients had a neurogenic disease, and 45 patients had a non-neurogenic disease. Bladder neck operations were performed either at the time of or after augmentation for 62 patients (48.4%), more commonly in the non-neurogenic than neurogenic group <0.01. In addition, continent stoma operations in 56 (36.8%) patients were more common in the non-neurogenic group (p < 0.01). Ten patients received kidney transplantation (four in the neurogenic, six in the non-neurogenic group); seven of these patients originally had dysplastic kidneys, two developed nephrological kidney disease, and one had hydronephrosis and renal insufficiency at the time of diagnosis of neurogenic bladder. Surgical problems resulting from bladder augmentation were treated in 28 patients (21.8%). The risk for surgical treatment because of complications was 4%, 17%, 24%, 27% and 27% at 1, 5, 10, 15 and 20 years follow up respectively. Complications were more common in patients in non-neurogenic than in patients with neurogenic group (p < 0.01). However, the greater proportion of continent stomas in non-neurogenic group explained the difference (Fig.). The most frequent complication requiring surgery during follow-up was bladder stones, which occurred in 24 (18.8%) patients. The first stone was treated at a median of 3.3 years (range 0.3-14.2) after augmentation. Stones were more common in patients with a continent stoma than without (p < 0.01). Other indications for surgical treatment were as follows: obstructive ileus in five patients, reduced bladder capacity in three patients, bladder perforation in two patients and lobulated bladder with infections in one patient. CONCLUSIONS: The risk for surgical treatment was about 27% in 20-year follow-up after bladder augmentation, because of augmentation related complications. The most prevalent complication was bladder stone, that was mainly associated with continent stomas. Continent stomas were most prevalent in patients with non-neurogenic diagnosis.

Development of Late Continence in Bladder Exstrophy and Epispadias Patients.

OBJECTIVE: To evaluate to what extend urinary continence develops during puberty in patients with classic bladder exstrophy (CBE) and epispadias. METHODS: The operative database was reviewed for surgical procedures and urinary continence in all 65 CBE and epispadias patients born in 1976-2005. Excluded were 2 patients who had insufficient data for evaluation. RESULTS: Thirty-five patients with volitional voiding were incontinent at the age of 10 years, 27 had daily incontinence, and 8 had rare incontinence. Sixteen patients (46%) became later fully continent without any major surgery (except Deflux injections in 3 patients) or CIC treatment. Among these 16 fully continent patients there were 7/8 with rare incontinence, 9/23 with daily incontinence (P = .04); 3/9 males with CBE, 10/13 males with epispadias, 3/5 females with CBE, and 0/4 females with epispadias (P = .25 between sex and P = .48 between diagnosis). Of the 15 patients remaining incontinent, 10 had daily and 5 had rare incontinence episodes. The latest control was at the median age of 19 years (interquartile range 17-21 years). Twenty-five patients (40% of the whole material) were fully continent (11/38 (29%) with CBE and 14/25 (56%) with epispadias, P = .04) and 8 (13%) had rare incontinence with volitional voiding (1/38 [3%] with CBE and 7/25 [28%] with epispadias). CONCLUSION: Almost half of the incontinent CBE and epispadias patients with volitional voiding achieved continence after the age of 10 without major surgery. Prognosis for pubertal development of continence was best in patients with rare incontinence and in males with epispadias.

Effect of Pediatric Testicular Torsion on Testicular Function in the Short Term.

PURPOSE: To evaluate short-term testicular outcome after torsion in children. METHODS: Fifty-four children and adolescents were evaluated after 6 months of the operation for testicular torsion. Testicular volume was measured and circulating Inhibin B, FSH, LH and testosterone levels were checked. RESULTS: Delay from the onset of symptoms to surgery was shorter in the orchidopexy group (n = 47), than in the orchiectomy group (n = 7, p = 0.001). In the orchidopexy group, the median volume of the affected testis was 83% (IQR 43-104) of the contralateral testis (p = 0.002). The plasma hormone levels in orchidopexy and orchiectomy groups were: 148 ng/l (IQR 108-208) vs. 129 ng/l (IQR, 123-138, p = 0.269) for Inhibin B; 4.5 IU/L (IQR2.6-6.9) vs. 11.7 IU/L (IQR 4.3-12.8, p = 0.037) for FSH; 2.9 IU/L (IQR 1.3-3.7) vs. 4.8 (IQR 3.0-5.6, p = 0.066) for LH; and 13.6 nM (IQR 6.5-18.0) vs. 14.5 nM (IQR 6.7-15.9, p = 0.834) for testosterone. The association between FSH, LH as well as testosterone levels was most clear with the volume of the contralateral testis (Rho = 0.574, p < 0.001, Rho = 0.621, p = 0.001 and Rho 0.718, p < 0.001 respectively). CONCLUSIONS: Testicular function is mainly dependent on the volume of contralateral testicle after testicular torsion. However, testis preserving surgery tends to maintain better function than orchiectomy. TYPE OF STUDY: Retrospective review. LEVEL OF EVIDENCE: III.

Paternity, erectile function, and health-related quality of life in patients operated for pediatric testicular torsion.

INTRODUCTION: Spermatic cord torsion (SCT) may lead to organ loss and can potentially influence fertility. Long-term effects of SCT are not fully investigated. OBJECTIVE: The purpose was to evaluate paternity rates in adults who have had SCT in childhood and to compare the results to those of a control population. The secondary purposes were to compare paternity rates after testis-preserving surgery with those after orchiectomy and to evaluate erectile function and health-related quality of life (HRQoL). STUDY DESIGN: Questionnaires concerning paternity, erectile function (International Index of Erectile Function [IIEF]-5 questionnaire), and HRQoL (15D questionnaire) were mailed to 74 men who had been treated for SCT and to 92 controls treated for testicular appendage torsion in 1977-1995 and who were currently older than 30 years. RESULTS: Thirty-five of the 74 (47%) patients with SCT and 58 of the 92 (63%) controls responded. A same-aged control was selected for each patient with SCT. The median age at investigation was 41 (interquatile range [IQR]: 36 to 46) years in the SCT group and 41 (IQR: 38 to 46) years in the control group (p = 0.81). The paternity rate was 23 of 35 (66%) in the SCT group and 26 of 34 (76%) in the control group (p = 0.43). Nine percent of patients and controls suffered from infertility. Of the 30- to 50-year-old patients with SCT, 9 of 16 (56%) had children after orchiectomy, and 13 of 16 (81%), after detorsion (p = 0.25). Significant or moderate erectile dysfunction (IIEF-5 total score <12) was observed in 3 of 32 (9%) patients and in 1 of 35 (3%) controls (p = 0.34). Erectile dysfunction was similarly rare in both the orchidopexy and orchiectomy group. Total HRQoL scores were similar in the SCT and control groups (p = 0.69) as well as in patients with orchidopexy and orchiectomy (p = 0.50). DISCUSSION: Paternity, erectile function, or HRQoL was not impaired in the general level in the patients with SCT in comparison with controls. Both the modes of treatment, orchiectomy or detorsion, had no significant impact on the results. However, the results cannot be generalized to the individual level. The limitations were a small sample size and inability to investigate maternal factors to the paternity. However, the results are encouraging for the patients and families. CONCLUSION: Paternity rate and HRQoL were similar in patients with SCT and controls. The type of surgery (orchiectomy vs. detorsion) did not affect paternity rates statistically. Moderate or significant erectile dysfunction was rare in both groups.

Clinical findings in prepubertal girls with inguinal hernia with special reference to the diagnosis of androgen insensitivity syndrome.

OBJECTIVE: Complete androgen insensitivity syndrome (CAIS) is a rare disease. However, there is a higher rate of CAIS in girls with inguinal hernia. The aim of this study was to estimate the incidence of CAIS in girls with inguinal hernia and to find a proper screening test for CAIS in these girls. MATERIAL AND METHODS: A total of 109 consecutive girls attending the University Hospitals of Turku and Tampere, Finland, for inguinal hernia repair between January 2003 and December 2007 participated in the study. After induction of anaesthesia, vaginal length was measured with a small, lubricated Hegar dilator. During hernia repair, tension was placed on the round ligament to identify the contents of the hernial sac. The karyotype of all patients was measured, with identification of the Y-chromosome from a buccal mucosa swab sample. Vaginal length measurements were plotted against age and standards for vaginal length in prepuberty were established. RESULTS: Four patients were found to have very short vaginas, with one CAIS patient confirmed as having a 46XY karyotype from the verification of the Y-chromosome from buccal mucosa. The other three patients with abnormal vaginal length were karyotypically normal XX girls and had visible ovaries, fallopian tubes or round ligament, which suggests an error in the measurements. Unlike all the other operated girls, the CAIS patient lacked a round ligament. CONCLUSIONS: The incidence of CAIS in girls undergoing hernia repair was 1%. The CAIS patient had a significantly shorter vagina than girls with normal karyotype. Vaginal length is a useful additional clinical tool in screening girls for karyotyping CAIS, especially if abnormalities in the round ligament and contents of the hernial sac are suspected. It is recommended that ovaries and fallopian tubes are searched for if the round ligament is not found to be normal during the hernia operation. If no ovaries or fallopian tubes are found, consent for karyotyping should be sought in cases where the vaginal length is <4 cm in girls older than 4 years and <3 cm in younger girls.