A combined approach of robot-assisted laparoscopic pyeloplasty and flexible endoscopy to treat concomitant ureteropelvic junction obstruction and calyceal stones in children: Technical considerations and review of the literature.

The management of children with concomitant ureteropelvic junction (UPJ) obstruction and calyceal stones remains challenging. The various treatment options available for pediatric nephrolithiasis may require multiple sessions, and the techniques by themselves are not designed for simultaneous correction of UPJ obstruction. Recently, success in combining robot-assisted laparoscopic pyeloplasty (RALP) and flexible endoscopy has been reported by multi-institutional studies to treat children with concomitant UPJ obstruction and renal stones. Given the paucity of technical details of this novel approach in the existing literature, we herein report our techniques to treat two girls aged 6 and 10 years who had concomitant UPJ obstruction and multiple stones in mid- and lower poles calyces. Three robotic ports were used without any assistant ports. A flexible endoscope, either a cystoscope or a single-use ureteroscope, was introduced via the undocked epigastric port to perform nephroscopy and stones removal after the renal pelvis was opened. The rest of the RALP was completed in the usual manner. Technical modifications were employed to facilitate the flexible endoscope to examine the entire calyceal system. Both patients underwent successful surgical procedures by the combined approach without any intra- or post-operative complications. Three and 14 stones were removed from each of the patients respectively. Postoperative investigations demonstrated successful correction of UPJ obstruction and complete stone clearance in both patients. A combined approach of RALP and flexible endoscopy is a safe and effective technique to treat concurrent UPJ obstruction and calyceal stones in children.

Surgery in Chinese children affected by 45,X/46,XY disorders of sex development: A 20-year experience in a single center.

BACKGROUND: Children affected by 45,X/46,XY mosaicism have a wide spectrum in their phenotypes. Although surgery has a role in management, there is no universally agreed strategy. METHODS: We conducted a retrospective review in a tertiary center on the surgical management and its long-term outcomes in children with the karyotype 45,X/46,XY or its variants over a 20-year study period. Patients were divided into 4 groups based on their phenotypes. RESULTS: 22 children(female=13, male=9) were included in the study, and were grouped as I)female phenotype(n = 11), II)ambiguous external genitalia(n = 2), III)undervirilized male with hypospadias(n = 6), and IV)normal male phenotype(n = 3). 19/22(86%) underwent gonadal surgeries(bilateral gonadectomy=15; unilateral gonadectomy=1; testicular biopsy=3) and  8/22(36%) underwent genitalia reconstructive surgeries(hypospadias repair=6; feminizing surgery=2). Gonadal germ cell tumors were detected in 55%, 50% and 50% of Group I, II and III respectively. 3/6 patients required reoperations for complications after hypospadias repairs. None of the patients had recurrence of germ cell tumors after gonadectomy. All the patients who had reached late adolescence or adulthood maintained their genders as their sex of rearing. CONCLUSIONS: Majority of 45,X/46,XY children benefit from timely gonadal surgery while genitalia reconstructive surgery can be considered in selected patients. Surgical management can be strategized by patients' phenotypes.

Comparing Robot-Assisted Laparoscopic Pyeloplasty vs. Laparoscopic Pyeloplasty in Infants Aged 12 Months or Less.

Objective: To investigate the outcomes of minimally invasive approach to infants with ureteropelvic junction (UPJ) obstruction by comparing the two surgical modalities of robot-assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP). Methods: We conducted a retrospective review of all consecutive infants aged ≤12 months who underwent either LP or RALP in a single institution over the period of 2008-Jul 2020. We included primary pyeloplasty cases that were performed by or under the supervision of the same surgeon. Results: Forty-six infants (LP = 22; RALP = 24) were included with medians of age and body weight at 6 months (2-12months) and 8.0 kg (5.4-10 kg), respectively. There was no difference between the two groups in the patients' demographics and pre-operative characteristics. All infants underwent LP or RALP successfully without conversion to open surgery. None had intraoperative complications. Operative time (OT) was 242 min (SD = 59) in LP, compared with 225 min (SD = 39) of RALP (p = 0.25). Linear regression analysis showed a significant trend of decrease in OT with increasing case experience of RALP(p = 0.005). No difference was noted in the post-operative analgesic requirement. RALP was associated with a shorter hospital length of stay than LP (3 vs. 3.8 days; p = 0.009). 4/22(18%) LP and 3/24(13%) RALP developed post-operative complications (p = 0.59), mostly minor and stent-related. The success rates were 20/22 (91%) in LP and 23/24 (96%) in RALP (p = 0.49). Conclusions: Pyeloplasty by minimally invasive approach is safe and effective in the infant population. RALP may have superiority over LP in infants with its faster recovery and a more manageable learning curve to acquire the skills.

A standardized technique of laparoscopic placement of peritoneal dialysis catheter with omentectomy and closure of patent processus vaginalis: A 3-in-1 minimally invasive surgical approach in children.

BACKGROUND: Omental wrapping is a common cause for catheter failure in children on peritoneal dialysis (PD). Previous studies are conflicting in the benefits of omentectomy. METHODS: We conducted a retrospective study comparing children who underwent PD catheter placement by a standardized laparoscopic three-in-one technique (lap3-in-1) from 2013 to 2018 versus a historical control cohort by open surgery without omentectomy. Lap3-in-1technique combined catheter placement with well-defined indication and extent of omentectomy, and closure of any patent processus vaginalis (PPV). RESULTS: There were 33 and 32 children in the lap3-in-1 and control cohorts respectively. 4/33(12.1%) in lap3-in-1 had reoperations for catheter failures which equated 1 reoperation per 144 catheter months. No reoperations were performed in lap3-in-1 cohort for omental wrapping or inguinal hernia, compared with 13/32 (41%; p < 0.001) and 5/32 (16%; p = 0.02) in the control cohort. Kaplan Meier survival curves showed significantly longer catheter life in the lap3-in-1 cohort (p < 0.001). In multivariate analysis by the COX proportional hazards model, the lap3-in-1 approach had significantly reduced risk of reoperation for catheter failure (HR 0.11; 95% CI: 0.04-0.31; p < 0.001). CONCLUSIONS: The lap3-in-1 technique is effective in selecting those children who would benefit from omentectomy, and avoiding a second operation for inguinal hernia which develops after PD. LEVEL OF EVIDENCE: Treatment study, level III.

The Hypospadias Phenotype With A Distal Meatus In The Presence Of Distal Penile Penoscrotal Angle Fixation.

OBJECTIVE: Hypospadias patients may present with the phenotype that features the migration of scrotum to distal penile shaft below a coronal/subcoronal meatus. Patients with this phenotype differ widely in the severity of the hypospadias and the complexity of the surgical repair. We aimed to investigate the operative findings and the outcomes of consecutive patients who presented with this phenotype. METHODS: We retrospectively reviewed the medical charts of 31 consecutive patients who underwent hypospadias repairs from January 2014 to May 2017, and the hypospadias was characterized by i) the external urethral meatus at coronal/subcoronal region, ii) scrotal skin encroaching distally resulting in fixation of penoscrotal angle at distal penile shaft, and iii) urethral plate below the glans on penile shaft invisible or barely visible. RESULTS: The median age at the time of surgery was 15.5 months (10-63). The division of corpus spongiosum was noted at distal penile shaft (n=2; 6.5%), mid-shaft (n=5; 16.1%) and proximal location (n=24; 77.4%). The median ventral curvature before degloving was 45 degrees (10-90). Twenty-eight and 3 patients underwent tubularized incised plate and 2-stage preputial flap repairs, respectively. Twenty-nine of 31 patients required cutback of the hypoplastic urethra. At the time of urethroplasty, 2 (6.5%), 7 (22.6%) and 22(70.9%) patients underwent distal, mid-shaft and proximal repairs, respectively. At a median follow-up of 30 months (14-50), 6 (19.4%) patients developed one or more complications (fistula=3; meatal stenosis=5). CONCLUSION: Patients affected by this particular phenotype likely require non-distal hypospadias repair with possibly higher complication rate and should be treated by surgeons with expertise in complex hypospadias repair.

Incidence and diagnoses of disorders of sex development in proximal hypospadias.

BACKGROUND: Evidence-based guidelines on evaluation of boys with proximal hypospadias for the possibility of a disorder of sex development (DSD) have yet to be developed. We aimed to investigate the incidence and diagnoses of DSD in patients with proximal hypospadias. METHODS: We retrospectively reviewed the records of consecutive boys who underwent proximal hypospadias repairs from 2006 to Sept 2017. Data collected included scrotal anomaly, testes position/palpability, micropenis, DSD investigations, and surgical techniques. RESULTS: 165 patients were eligible for the study. 14 (8.5%) were diagnosed to have DSD. The diagnoses were 46,XX testicular DSD [n = 1], 46,XY DSD [n = 7; partial gonadal dysgenesis (PGD) = 3; 5α-reductase type 2 deficiency = 3; 17α-hydroxylase deficiency = 1], Sex Chromosome DSD [n = 6; 45,X/46,XY PGD = 4; Klinefelter = 2]. 3/7 (43%) patients with PGD had gonadal germ cell neoplasms. Of the DSD patients, 6/14 (43%), 11/14 (79%) and 11/14 (79%) had undescended/impalpable testes, micropenis and penoscrotal transposition/bifid scrotum, respectively, significantly higher prevalence rates than those without DSD diagnosis (p-values <0.05). 10/14 (71.4%) DSD patients underwent 2-stage repair compared with 57/151 (37.7%) of others without DSD diagnosis (p = 0.01). CONCLUSIONS: Patients presenting with proximal hypospadias and one or more of the coexisting anomalies of micropenis, undescended/impalpable testes, and penoscrotal transposition/bifid scrotum should warrant DSD evaluation. Presence of bilaterally descended testes in scrotum does not preclude the possibility of DSD. LEVEL OF EVIDENCE: IV.