Journal of Pediatric Urology最新文献

Introduction: There is a paucity of research regarding transition to adult services within pediatric and adolescent urology. Several recent articles have discussed the barriers in transitioning urologic patients from pediatric to adult health care, but empiric data that may drive intervention are lacking. This study proposes to begin to address this gap in literature and to provide information that may lead to improved understanding of how best to support transition in urologic care.

Objectives: 1) to identify modifiable and non-modifiable factors related to transition readiness as measured by Transition Readiness Assessment Questionnaire (TRAQ) scores in a congenital urologic population and 2) to evaluate the relationships between TRAQ scores (a validated questionnaire measuring transition readiness) and scores measuring anxiety levels related to transition (using an adapted, non-validated questionnaire).

Study design: This is a cross-sectional study of adolescent and young adult patients with complex congenital urologic diagnoses. Subjects were electronically administered the validated TRAQ and a study-developed ADHERENT survey, which assesses anxiety and worry surrounding transition. Regression models for the outcomes of the TRAQ and ADHERENT scales were developed to assess multivariable associations with variables of clinical importance.

Results: The youngest subgroup (14-17 years of age) compared to the oldest subgroup (21-25 years of age) had significantly lower TRAQ scores [regression estimate = 12.3 (95 % CI: 2.9, 21.7), p = 0.010]. Additionally, single participants versus those in a stable relationship had significantly lower TRAQ scores [estimate = 8.7 (95 % CI: 1.9, 15.4), p = 0.012]. The Spearman correlation coefficient between TRAQ and ADHERENT scores was 0.52 (p = <0.001), indicating a positive, moderate relationship between the two measures, suggesting more readiness correlated with less anxiety.

Discussion: This study found that age, higher education, and stable relationship status were associated with higher measures of transition readiness. There was a correlation found between more transition readiness and less anxiety surrounding transition. This finding can be used to inform future research and emphasizes the need for multidisciplinary support throughout the transition process.

Conclusion: Early discussion of transition of care and education around transition readiness are not the only solution to improving transition success. The second phase of ADHERENT seeks to understand the patient experience and to include adolescents and young adults in shaping effective healthcare transition strategies.

Introduction: The modified staged repair, or Toronto approach to reconstruct classic bladder exstrophy, involves bladder neck (BN) tailoring and bilateral ureteral reimplantation during primary closure, and later epispadias repair using external corpora rotation and a rotational penile skin flap. It aims to incorporate the advantages of complete primary repair while minimizing risks of upper tract deterioration and penile ischemia and improve cosmetic appearance of the genitalia. We present long-term outcomes for our initial patient series.

Methods: All patients with initial operation between 2000 and 2014 were reviewed. Data on demographics, continence, erectile and ejaculatory function, cosmetic appearance of the genitalia, and upper tract status were collected.

Results: Twelve male and four female patients were identified, with median follow-up of 12.7 (IQR 10.9-15.4) and 12.5 years (IQR 10.6-15.6), respectively. Full continence (voiding with no leaks, dry periods ≥3 h) was achieved in two of 12 males and two of four females. Five of 12 males and all four females had dry periods longer than 1 h. Nine of 12 males and all females attained volitional voiding. Three of 12 males and one of four females underwent additional continence procedures. None have undergone augmentation cystoplasty or bladder neck closure. Of seven males with preliminary sexual function data, all experienced erections, straight in five, with recurrent dorsal curvature and ventral curvature in one patient each. Four of seven ejaculate and none have attempted penetrative intercourse. All seven males reported satisfactory cosmetic appearance despite a subjectively shorter penis. Although transitory dilations of the ureters were seen immediately post op, none had scarring, hydronephrosis, or febrile urinary tract infections at latest follow-up. One patient had an eGFR on the upper range of CKD 2, while the rest of the cohort had eGFR ≥ 90 mL/min/1.73m². Mean bladder capacity on ultrasound was 145 mL for males and 97 mL for females.

Conclusion: The present data suggests that the modified staged repair of exstrophy (Toronto approach) is associated with acceptable continence outcomes while minimizing escalation to augmentation cystoplasty and bladder neck closure. Most patients void volitionally and stay dry for 1-3 h, but few are fully continent or able to remain dry for 3 h or more. No patients in the cohort had CKD3 or worse, and none had hydronephrosis or history of febrile urinary tract infections. Most males experience ejaculation and straight erections. There was no glanular or corporal tissue loss.

Introduction: Females born 46,XX with the severe malformations of cloacal exstrophy (CE) and cloacal anomalies (CA) have high rates of mullerian abnormalities leading to outflow tract obstruction (OTO) of menses, invasive surgeries, and/or chronic pain. This study aims to discuss long-term surgical outcomes of mullerian structures in a single major institution with a high volume of CE and CA patients.

Methods: A prospectively maintained database was reviewed for CE and CA patients. The patient database was received for CE and CA female patients. Data on mullerian anatomy at birth, hormone suppression, and surgical procedures were evaluated.

Results: 91 females (46XX, 17 CE and 74 CA) were included with median age of 14.5 [0.17, 30.5] years. Of those, 68.1 % had duplicated uteri. Vaginal anatomy was duplicated in 52.7 % with 18.7 % having complete atresia. 23.1 % of patients underwent a hysterectomy (41.2 % CE, 18.9 % CA). Hysterectomy was performed prior to menarche in 52.4 % and post menarche in 47.6 %. Hormone suppression was used in 24.2 % (22/91) of the entire cohort, with 45.2 % (10/22) of those patients proceeding to hysterectomy. In those with hormone suppression alone (54.5 %, 12/22), either vaginoplasty to relieve obstruction or hysterectomy of obstructed horn is planned for the future once the patient is ready to participate in surgical discussion. All hysterectomies were performed on patients with duplicated uteri. Reasons for pre-menarchal hysterectomy included nonfunctional or noncommunicating uteri, little chance of safe pregnancy, and/or family desire for minimal surgical intervention. Post-menarchal hysterectomy reasons included pelvic pain secondary to hematocolpos, desire to discontinue hormone suppression and/or need for vaginostomy, and/or vesicouterine fistula.

Conclusion: This study demonstrates high rates of surgical removal of mullerian structures in females with CE and CA. Further study would be beneficial for early identification of CE and CA patients at risk of undergoing hysterectomy while maintaining fertility potential in those with low risk of mullerian complications.