Rodrigo L.P. Romao

Timing and nature of reconstructive surgery for disorders of sex development - introduction.

The ideal timing and nature of surgical reconstruction in individuals with Disorders of Sex Development (DSD) is highly controversial. Despite the increasing number of publications on this topic, evidence-based recommendations still cannot be made. However it is generally accepted that optimal care for DSD requires an experienced multidisciplinary team. This means that surgical decisions are now made within the context of a multidisciplinary team and all members of the team - and not just specialist surgeons - may be called upon to discuss choices for surgery with patients and parents. To do this well, every clinician in the team should have an understanding of the range of techniques available for genital surgery, the risks and benefits of procedures and the controversies surrounding timing of surgery. The aim of this paper is to give an overview of the variety of surgical procedures in current use and in what situation a particular technique would be indicated. The short-term risks and benefits are described and where available long-term outcome data is discussed. To date, discussions surrounding genital surgery have been led primarily by surgeons. Some non-surgical clinicians have expressed unease about decision making in genital surgery but have felt ill equipped to comment on an area with which they are unfamiliar. This review gives a detailed explanation of current surgical practice offered in a specialized center for DSD and such information should facilitate a more balanced discussion.

Delayed primary serial transverse enteroplasty as a novel management strategy for infants with congenital ultra-short bowel syndrome.

BACKGROUND Congenital ultra-short bowel syndrome (USBS) is a challenging problem with a poor outcome. We report a new management approach for USBS infants that attempts to optimize gut growth potential. METHODS We report five neonates with USBS in whom no correction was performed at primary surgery except placement of a gastrostomy (G) tube. Sham feeds were started with intermittent G-tube clamping to induce bowel dilatation/growth. Serial fluoroscopy was done until bowel caliber reached 5 cm. STEP was performed and continuity established to the colonic remnant. Small bowel length (SBL) and enteral caloric intake were tabulated. RESULTS Patients were born with a mean residual SBL of 19 ± 7.6 cm (14.8% of expected). Median duration of sham feeds prior to STEP was 108 (range 27-232)days. Mean SBL at STEP was 47 ± 12.1cm, which increased post-STEP to 70 ± 12.7 cm (a mean increase of 296% from birth, representing 36.4% ± 13.1% of expected gut length). With a median follow-up time of 20 months (range 8-28), 4/5 achieved >50% enteral calories and have normal liver function. One has undergone liver transplantation. CONCLUSIONS In USBS patients, delayed surgical correction with sham feeds accelerates gut growth, optimizing potential for autologous reconstruction. This approach may offer greater opportunity for intestinal adaptation than traditional options.

Renal function in patients with Wilms tumor

Survival in patients with Wilms tumor (WT) is excellent compared with other pediatric malignancies and adult renal tumors. Treatment-related long-term morbidity and mortality in WT survivors is an area of increasing concern. Renal dysfunction is an example of one of the most feared long-term issues observed in these survivors. Direct toxicity from chemotherapy and radiation as well as direct nephron ablation from surgical treatment (nephrectomy) renders the kidney susceptible to a multitude of problems over time in patients with WT. In this article, we review the existing literature pertaining to renal function in these patients. Incidence rates, causes, and methods to mitigate renal dysfunction are presented in 3 distinct clinical situations: sporadic unilateral WT, syndromic unilateral WT, and bilateral WT. We also offer a critical lens on the current role of nephron-sparing surgery as a means to preserve renal function in these patients. Finally, we discuss potential avenues for refining renal function preservation in patients with WT in the future. We conclude that: (1) renal function in pediatric cancer survivors must be carefully ascertained prospectively using methods that allow diagnosis of mild cases (rather than focus solely on extreme cases represented by the development of end-stage renal disease), (2) every effort should be made to recognize subtle features of predisposition syndromes to avoid syndromic cases from being misclassified and treated as sporadic, (3) molecular stratification for disease aggressiveness as well as multifocality and renal dysfunction will be very important to tailor treatment and balance survival with preservation of renal function, and (4) the role and potential benefits of nephron-sparing surgery deserves careful exploration under well-designed protocols.

Preserving bowel length with a transluminal stent in neonates with multiple intestinal anastomoses: a case series and review of the literature

BACKGROUND/PURPOSE In neonatal surgery, preserving small bowel length is important to avoid short bowel syndrome. Our aim was to assess the outcomes of intraluminal stenting of neonatal multiple intestinal anastomoses. METHODS We conducted a retrospective review of 9 patients (5, single institution; 4, published literature) who received multiple anastomoses stented by a silicon tube. Demographics, surgical anatomy and complications, nutritional outcomes, and follow-up were reviewed. RESULTS Diagnosis was multiple intestinal atresias in 8 patients and necrotizing enterocolitis in 1. A silicon catheter entered either the mucous fistula (5 patients received a jejunostomy/mucous fistula) or a proximal opening on the dilated bowel and was threaded through viable segments of the bowel. The bowel ends were approximated. Stent was externalized in 7 patients. Final mean small bowel length was 63.9 cm. All complications (3 patients, leak/stricture) required surgery. Mean time to stent removal, feeds initiation, and parenteral nutrition (PN) discontinuation was 31.2 days, 17.3 days, and 159 days, respectively. Only 1 patient remains on PN (mean follow-up, 25.4 months). CONCLUSIONS Multiple intestinal anastomoses stenting is an excellent technique to avoid short bowel syndrome in the setting of multiple viable segments of gut, such as type IV intestinal atresia or necrotizing enterocolitis. Both our experience and the published literature show no mortality and PN-free survival.

The transition of young adults with lifelong urological needs from pediatric to adult services: An international children's continence society position statement

Children with urinary tract disorders managed by teams, or individual pediatricians, urologists, nephrologists, gastroenterologists, neurologists, psychologists, and nurses at some point move from child‐centered to adult‐centered health systems. The actual physical change is referred to as the transfer whilst the process preceding this move constitutes transition of care. Our aims are twofold: to identify management and health‐service problems related to children with congenital or acquired urological conditions who advance into adulthood and the clinical implications this has for long‐term health and specialist care; and, to understand the issues facing both pediatric and adult‐care clinicians and to develop a systems‐approach model that meets the needs of young adults, their families and the clinicians working within adult services.

Fertility preservation options for children and adolescents with cancer

INTRODUCTION Cancer in children and adolescents has seen a stark rise in survival rates in the last decades; overall survival in excess of 80% can be reasonably expected for many newly diagnosed patients with malignancies in this age group. Survivorship has unfolded several specific issues faced by these patients, including fertility concerns. Hence, fertility preservation efforts have been discussed and undertaken with increased frequency. METHODS In this article, the authors provide a broad overview of the current recommendations surrounding fertility preservation in this patient population. Reasons to offer fertility preservation, target groups for interventions, and methods available based on age group and gender are discussed in detail. RESULTS The medical literature and patient advocates strongly support a discussion about fertility preservation at the time of diagnosis; the risk of infertility is real and parents and families wish to be informed about it. In postpubertal males, sperm-banking is relatively straightforward and should be attempted by most newly diagnosed patients, ideally before commencement of treatment. Cryopreservation of testicular tissue in higher-risk prepubertal males is feasible, but still experimental. Female fertility preservation is more complex, requires more invasive procedures, and can delay initiation of treatment due to the requirement for hormone stimulation of follicles prior to harvesting. CONCLUSIONS Oncofertility initiatives in children and youth are still in their early days and will continue to expand; urologists should be prepared to offer counselling and interventions when appropriate to this growing vulnerable population.

Urological Follow-up in Adult Spina Bifida Patients: Is There an Ideal Interval?

OBJECTIVE To establish the time to development of urological issues over time in adult spina bifida (SB) patients. MATERIALS AND METHODS This is a retrospective study of adult patients attending a multidisciplinary adult SB clinic from 2000 to 2013. Patient age, sex, number of clinic visits, and length of follow-up were recorded. For each unique visit, presence of symptoms, type of urological issue (if any), and time lapsed since last appointment were obtained. The interval between the development of urological issues was assessed using a time-to-event analysis. RESULTS One hundred twenty-three patients (46% male, 54% female, median age 26.8years) were followed for a median of 48 months, contributing to 586 unique clinic visits. Urological issues were identified in 109 patients (88.5%) during 267 visits (46%), and of those 21% were asymptomatic. In symptomatic patients, the median time to present with a urological issue was 12 months. Among the asymptomatic cases, 12%, 23%, and 34% had developed a urological issue at 12, 24, and 36 months of follow-up, respectively. Eighty-one percent of the urological issues seen in the clinic required some form of treatment or intervention. The treatment or intervention in 56% of asymptomatic urological issues was surgery. CONCLUSION Most adult SB patients with urological issues are symptomatic by 2 years of follow-up; however, over time the proportion of asymptomatic patients with urological issues rises steadily, reaching a worrisome 34% at 3 years. Closer follow-up seems warranted.

Clinical Outcomes of the Upper Urinary Tract after Ureteral Clipping for Treatment of Low Functioning or Nonfunctioning Renal Moieties

Purpose: A growing body of evidence suggests that it is safe to ligate the ureter of poorly functioning renal moieties during renal transplantation. We present clinical outcomes and data on hydronephrosis progression in pediatric cases associated with ectopic ureters and obstructive ureteroceles. Materials and Methods: We prospectively collected data for 35 consecutive patients (23 females and 12 males) who underwent ureteral clipping between February 2011 and August 2016. Patients were divided into 4 groups consisting of 1) duplex system with ectopic ureter (45.7%), 2) duplex system with a large ureterocele (11.4%), 3) other duplex system (8.6%) and 4) single system kidneys (34.3%). Patients were followed for clinical outcomes and hydronephrosis trends. Comparisons included preoperative and postoperative anteroposterior diameter, maximal ureteral diameter and ureterocele size. Results: Median age at surgery was 59 months (IQR 11 to 120, range 5 to 216). Median ± SD operative time was 108.9 ± 31.1 minutes (range 20 to 180) and median length of stay was 7.5 hours (IQR 6 to 19, range 5 to 336). Immediate resolution of urinary incontinence was observed in all 16 ectopic ureter cases. After a median ± SD followup of 20.8 ± 13.8 months (IQR 8.5 to 30, range 6 to 50) 97.2% of the patients remained asymptomatic. No significant differences were observed between initial and last anteroposterior diameter measurements except in group 1 (p = 0.001). All ureteroceles demonstrated a significant decrease in median ± SD size after clipping (from 2.7 ± 0.41 to 0.53 ± 0.92 cm, p = 0.003). Pyonephrosis developed in 1 patient, who underwent laparoscopic nephrectomy. Conclusions: Ureteral clipping appears to be a reasonable, safe and effective option for pediatric patients in the reported settings, with the potential to be simpler and quicker than extirpative or reconstructive procedures.

Canadian Urological Association-Pediatric Urologists of Canada (CUA-PUC) guideline for the diagnosis, management, and followup of cryptorchidism

Cryptorchidism is one of the most common congenital anomalies in males, characterized by inability to palpate the testicle in the expected normal anatomical position (i.e., within its respective hemi-scrotum). It represents an abnormality of testicular descent and development associated with long-term concerns, including infertility, hypogonadism, and development of neoplasms.

Urinary tract infection in children

In children less than two years of age, a fever (> 39°C) without an apparent source is the most common sign of urinary tract infection (likelihood ratio 4.0).[1][1] Abnormal or strong urinary odour has poor predictive accuracy (i.e., sensitivity 43%, specificity 48%; Appendix 1, available at [www.