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Study Objective: Infants with genital development considered atypical for assigned female sex may undergo feminizing genitoplasty (clitoroplasty and/or vaginoplasty) in early life. We sought to identify factors associated with parent/caregiver decisions regarding genitoplasty for their children with genital virilization., Design: Longitudinal, observational study SETTING: Twelve pediatric centers in the United States with multidisciplinary differences/disorders of sex development clinics, 2015-2020 PARTICIPANTS: Children under 2 years old with genital appearance atypical for female sex of rearing and their parents/caregivers INTERVENTIONS/OUTCOME MEASURES: Data on the child's diagnosis and anatomic characteristics before surgery were extracted from the medical record. Parents/caregivers completed questionnaires on psychosocial distress, experience of uncertainty, cosmetic appearance of their child's genitalia, and demographic characteristics. Urologists rated cosmetic appearance. For 58 patients from the study cohort with genital virilization being raised as girls or gender-neutral, we compared these data across 3 groups based on the child's subsequent surgical intervention: (i) no surgery (n = 5), (ii) vaginoplasty without clitoroplasty (V-only) (n = 15), and (iii) vaginoplasty and clitoroplasty (V+C) (n = 38)., Results: Fathers' and urologists' ratings of genital appearance were more favorable in the no-surgery group than in the V-only and V+C groups. Clitorophallic length was greater in the V+C group compared with the V-only group, with substantial overlap between groups. Mothers' depressive and anxious symptoms were lower in the no-surgery group compared with the V-only and V+C groups., Conclusions: Surgical decisions were associated with fathers' and urologists' ratings of genital appearance, the child's anatomic characteristics, and mothers' depressive and anxious symptoms. Further research on surgical decision-making is needed to inform counseling practices., (Copyright © 2022. Published by Elsevier Inc.)

Objective: To report on our experience performing office-based pediatric urologic procedures. We hypothesize that office-based interventions are safe and effective for children, avoiding unnecessary risk and cost associated with general anesthesia., Methods: We retrospectively identified patients undergoing office-based interventions from 2014 to 2019, including lysis of penile or labial adhesions, division of skin bridges, meatotomy and excision of benign lesion. Success was defined as a completed attempt in the office. Failure includes any unsuccessful office attempts. Complications include 30-day ED visits/readmissions and recurrent skin bridge post division of skin bridge., Results: We identified 1326 interventions: 491 lyses of penile adhesions (37%), 320 division of skin bridges (24%), 128 lyses of labial adhesions (10%), 348 meatotomies (26%), and 39 excisions of benign lesions (3%) [Table 1]. There was a >95% success rate reported in every procedure with an overall complication rate of 0.6%. Excision of benign lesion had 100% success rate. ED visits within 30 days are rare (0.2%), and no patients required admission after their procedure [Table 2]. The rate of recurrence was highest following lysis of labial adhesions (13.3%). Of the 54 patients who underwent retreatment, very few required general anesthesia (n = 6)., Conclusion: Office-based urologic interventions in children are well tolerated with excellent safety and efficacy. Complications and recurrence are universally low. Ultimately, 99.5% of this cohort was managed under local anesthetics, thereby avoiding the risks of anesthesia use in the pediatric population., (Copyright © 2022 Elsevier Inc. All rights reserved.)

A 32-week estimated gestational age female infant had elevated systolic blood pressure at birth and a palpable left abdominal mass. Radiologic evaluation revealed a left upper pole renal mass. The renin level was significantly elevated. Left nephrectomy confirmed a diagnosis of congenital mesoblastic nephroma. The postoperative renin level normalized along with the blood pressure. Histologic analysis identified renin production from either the mesoblastic nephroma or secondarily from compression or ischemia. Mesoblastic nephroma should be considered as part of the differential diagnosis for hypertension in the young. The renin concentration and a renal ultrasound scan should be obtained as part of the evaluation.

An infant born at 38 weeks' gestation with ambiguous genitalia had a prenatal 45X karyotype but an enlarged phallus on an ultrasound scan at 31 weeks' gestation. The newborn examination demonstrated penoscrotal hypospadias with chordee and two gonads palpable in the scrotum with a right hydrocele. Ultrasound showed a saccular structure containing debris behind the bladder. The postnatal karyotype was revealed to be 45X/46XY, with a pseudodicentric Y chromosome. Cystoscopy/genitography identified a uterus and a right fallopian tube, which were removed along with a dysgenetic right gonad. Biopsy of the descended left gonad revealed rare germ cells. The final diagnosis was 45X/46XY male pseudohermaphroditism with testicular dysgenesis. One should be aware of possible chromosomal mosaicism and combine the prenatal karyotype with the ultrasound genital findings to formulate an intersex differential diagnosis.