Composite Functional and Quality of Life Outcomes in Adults Operated for Cloacal Malformation.

INTRODUCTION: Composite long-term outcomes of cloacal malformations remain unclear. We aimed to evaluate bowel and bladder control, sexual function, reproductive health, and quality of life in adulthood. METHODS: A Nordic multi-center cross-sectional observational study evaluating the outcome of adult patients with cloacal malformations was performed. Patient characteristics were retrieved from case records. Established questionnaires were sent to the patients to evaluate bowel- bladder- and sexual function and quality of life. RESULTS: Thirty-four of 48 (70%) eligible patients with median age 28 years (18-45) responded. Eight (24%) patients had a common channel >3 cm. Imaging-confirmed spinal and sacral abnormalities were present in 4 and 17 patients respectively. 85% (n = 29/34, four patients with permanent urinary diversions after bladder neck closure excluded) reported no urinary leakage without physical activity or urge to urinate. Three patients had a permanent enterostomy. 42 % (n = 11/26) reported acceptable bowel function according to Bowel Function Score (with eventual ongoing bowel management). The median Profile of Female Sexual Function Score was below normative values. Quality of life was comparable to the reference population, but thirty percent scored within the distress domains. Eight patients (24%) had been pregnant (15 pregnancies), resulting in seven live births, but the need of assisted reproduction techniques (40%, n = 6/15) and miscarriage (40%, n = 6/15) was common. CONCLUSIONS: Adequate spontaneous bowel control was rare, while most patients were dry for urine without additional procedures. Cloacal malformation also have a negative impact on sexual function health related quality of life and reproductive health. Long-term follow-up is crucial, not only regarding bowel and bladder function, but also for sexual and reproductive function, which may be important negative factors for health-related quality of life. LEVEL OF EVIDENCE: Level IV.

Psychosocial outcome in adult men born with hypospadias.

INTRODUCTION: Hypospadias, which is a surgically treated congenital malformation of the male urethra, may have a negative impact on quality of life. This aspect has previously been subject to limited research. This study examined the long-term psychosocial outcome of a large cohort of adult males born with hypospadias. OBJECTIVE: The purpose of this case-control study was to assess a possible negative influence on the psychosocial outcome in adult males with hypospadias. STUDY DESIGN: Males with hypospadias treated in Sweden and aged ≥18 years old participated in this follow-up study. Age-matched men and university students were recruited as controls. The participants answered a questionnaire designed to reflect the subjective quality of life, social factors, need of support and follow-up, and the perceived impact of the disease upon upbringing. It also looked at the validated Psychological General Well-Being (PGWB) questionnaire and Relationship Questionnaire (RQ). RESULTS AND DISCUSSION: A total of 167 patients (median age 34 years, 63% distal, 24% mid, and 13% proximal hypospadias) and 169 controls (median age 33 years) participated in the study. Patients had their first operation at 4 years of age (median) and the median follow-up time was 29 years following the first surgery. Men with hypospadias had a comparable total quality of life level with a mean total PGWB score of 82 (normal range 78-83) compared with 85.6 in controls. Scores on wellbeing and vitality were lower, even if the differences were small. Hypospadias did not affect marital status, presence of children in the family, frequency of employment or experience of bullying. These men more often lived at home with their parents (P=0.001) and had a lower level of education (P=0.004), even if the educational level in both patients and controls was high compared with the general Swedish population. Patients with proximal hypospadias were shorter compared with controls (P=0.003), which was consistent with the prenatal growth restriction associated with hypospadias. The group with proximal hypospadias expressed a greater need for medical (45.5%) follow-up compared with mid (28.2%) and distal (18.1%) cases (P=0.001). Patients with proximal hypospadias tended to avoid close relationships because of fear of being hurt. CONCLUSIONS: The findings suggested that patients treated for hypospadias have a good HRQoL, can be expected to have a normal psychosocial life, and marry and have children. Repeated follow-up and psychological support during childhood/adolescence is however of great importance for patients with more proximal hypospadias.

Sexuality and fertility in men with hypospadias; improved outcome.

The aim of this study was to investigate sexual function and fertility in adult men born with hypospadias. Patients born with hypospadias, age-matched controls, and a group of circumcised men completed a questionnaire constructed to reflect their psychosexual situation and fertility. Core gender identity, sexual orientation, and gender role behavior was also assessed. 167 patients [63% distal, 24% mid shaft and 13% proximal, mean age 34 (19-54) years], 169 controls from the general population [mean age 33 (19-48) years] and 47 controls circumcised because of phimosis (mean age 26 [19-44]) participated and completed the questionnaire. There were no differences in having a partner, reported fertility, age at sexarche (mean age 17.8), number of sex partners or sexual interest between the patients and controls. More patients than controls reported anejaculation. Reported glanular sensitivity was lower in hypospadias patients and circumcised controls compared with non-circumcised controls. The odds of being satisfied with their sexual life increased with a higher penile perception score in patients (OR = 1.54, p = 0.01). There was no association with penile length. Sexual orientation, core gender identity and gender role behavior were sex-typical in both patients and controls. Patients with proximal hypospadias had a lower reported fertility, experienced anejaculation more often, and were less satisfied with their sexual life. Men born with hypospadias have a good long-term outcome concerning sexual function and fertility. Men born with proximal hypospadias have a more impaired outcome concerning both sexual function and fertility. As satisfaction with genital appearance is important for sexual life satisfaction, clinical, and psychological follow-up into adulthood is especially important in boys born with proximal hypospadias.

Intertwin discordance in umbilical venous volume flow: a reflection of blood volume imbalance in twin-to-twin transfusion syndrome.

OBJECTIVES: To compare umbilical venous volume flow (UVVF) between donor and recipient twins in twin-to-twin transfusion syndrome (TTTS) using an index that is independent of gestational age and to correlate changes in this index with outcome following endoscopic laser surgery. METHODS: UVVF was calculated in 84 cases of TTTS by multiplying the umbilical vein cross-sectional area at its entry into the fetal abdomen by time averaged blood velocity. All cases were classified according to the Quintero staging system. The ratio between UVVF in recipients and donors (R/D-UVVF) in each pair of twins was calculated before and 48 h after laser treatment, and changes in R/D-UVVF were correlated with pregnancy outcome. Intraobserver and interobserver UVVF measurement reliability was assessed in 19 singletons, and 13 donor and recipient twins before laser treatment using the intraclass correlation coefficient (ICC). RESULTS: Twenty-five, 32, 21 and six cases presented as Quintero Stages 1, 2, 3 and 4, respectively, at a median gestational age of 20 (range, 15-26) weeks. Intraobserver and interobserver ICC for UVVF measurement in twins were 0.97 and 0.67, respectively. UVVF was a median of 2.13-fold (range, 0.3-19-fold) higher in recipients than in donors (137 mL/min vs. 64 mL/min, P < 0.001) and increased with gestational age (r = 0.58, P < 0.001 for recipients, r = 0.62, P < 0.001 for donors). From 68 cases in which R/D-UVVF could be measured 48 h following laser surgery, cases with a favorable outcome showed a significant decrease in R/D-UVVF from a median of 1.97 to 1.27 (P < 0.01) and cases with recurrent TTTS (n = 6) did not (decrease in R/D-UVVF from a median of 2.32 to 2.19, P = 0.17). Using a cut-off of < 30% reduction of R/D-UVVF, 66% of the cases with recurrence could be predicted, and the odds ratio for recurrence was 3.13 (95% CI, 0.52-18.29). A significant UVVF imbalance between recipient and donor twins was found in cases with Quintero Stages 1-3 but not in those with Stage 4. CONCLUSIONS: In TTTS, UVVF is significantly higher in recipients than in their donor cotwins and the R/D-UVVF seems adequately to indicate the flow imbalance between twins, regardless of gestational age. A decrease in R/D-UVVF could be predictive of a favorable evolution following laser treatment.