Genital Malformations in Children With VACTERL - Has Time Come to Include "G" in the Acronym?

BACKGROUND: Genital malformations are frequently diagnosed in patients with VACTERL, but are currently not included in the acronym. This study aimed to analyze the frequency of genital anomalies in patients with esophageal atresia (EA) and/or anorectal malformation (ARM), with a subgroup analysis of children fulfilling the VACTERL criteria. METHOD: This was a cross-sectional retrospective analysis of two prospectively collected registries of patients operated on for ARM and EA between 2012 and 2022 at a specialized national center. Children were screened routinely for malformations according to the VACTERL acronym. RESULTS: A total of 174 children were included in the study. VACTERL was diagnosed in 60 children (34%), while 114 children (66%) were defined as non-VACTERL. Genital malformations were diagnosed in 38% (23/60) of the children with VACTERL, and in 11% (13/114) of the children without VACTERL (p < 0.001). The presence of genital malformations correlated linearly with the number of diagnosed component features (CFs). In boys with VACTERL, the most common genital malformation was undescended testes present in 10/27 (21%) compared to 1/71 (1%) in non-VACTERL boys (p < 0.001). Müllerian duct anomalies were found in 26% of girls with VACTERL vs. 7% in non-VACTERL girls (p < 0.05). CONCLUSION: There was a higher frequency of genital malformations in patients with VACTERL emphasizing the importance of genital assessment for these patients. We propose VACTERL-G as an extension of the current acronym aiming to reduce the risk of long-term morbidity due to delayed diagnosis of reproductive anomalies.

Orchidopexy for undescended testis-rate and predictors of re-ascent.

PURPOSE: This study aimed to investigate the rate of re-ascent requiring re-operation after primary orchidopexy and to investigate eventual differences between the inguinal and scrotal approach as well as other potential predictors for re-ascent. METHODS: A retrospective cohort study of children treated for undescended testis (UDT) with orchidopexy between 2018 and 2022 was conducted. The primary outcome was re-ascent requiring re-operation, and the secondary outcome was atrophy rate. Independent variables were age, underlying conditions, side, surgical approach, operation time, bilaterality, congenital/ascended UDT, presence of scrotal hypoplasia, presence of a patent processus vaginalis, division of external oblique, and suture of the testis. Univariate and logistic regression were used to evaluate differences between groups and risk for re-ascent. RESULTS: A total of 662 testes in 554 patients were included. Re-operation occurred in 6% (7% with inguinal approach, 3% with scrotal approach, p = 0.04). Re-operation was associated with younger age, congenital UDT, and inguinal approach, but neither of these variables remained significant in multivariate analyses. Atrophy occurred in one testis. CONCLUSION: The rate of re-ascent was 6% and the atrophy rate was 0.15%. A larger study may find predictors for re-ascent but with very low absolute risk. The lower rate of re-ascent with the scrotal approach is probably due to selection bias.

National survey of physicians in Swedish child health centres finds insecurity and unawareness regarding management of undescended testicles.

AIM: To investigate underlying factors for previously reported shortcomings in child health centres' (CHC) referral process of boys with undescended testicles. METHODS: A total of 386 physicians working at Swedish CHCs were surveyed regarding their knowledge about undescended testicles and their clinical management. Multivariate regression analyses were performed to identify risk factors of non-adherence to guidelines and self-reported lack of clinical skills. RESULTS: The overall knowledge of the health benefits of undescended testicle surgery was high (89%), while two-thirds were unaware of surgery being recommended <1 year of age. One-fifth of respondents had never received guidance on examination techniques. Male gender (adjusted odds ratio [aOR] 0.51, 95% confidence interval [95% CI] 0.31-0.86), education in paediatrics (aOR 0.37, 95% CI 0.18-0.76) and more experience (aOR 0.02, 95% CI 0.01-0.09) significantly decreased the risk of unfamiliarity with examinations. More experience decreased the risk of stating the incorrect indications for undescended testicle surgery (aOR 0.17, 95% CI 0.03-0.95) and finding examinations difficult (aOR 0.22, 95% CI 0.07-0.72). Medical education outside Nordic countries was a risk factor for unawareness of guidelines (aOR 2.06, 95% CI 1.21-3.51). CONCLUSION: The knowledge and confidence level of the study population varied widely. The results indicate a need for further theoretical and practical education among Swedish CHC physicians.