Infant prostatic Rhabdomyosarcoma: A diagnostic and therapeutic challenge.

The following case report describes a case of prostatic rhabdomyosarcoma in a 6-month-old male who presented with urinary retention and constipation. MRI showed a prostatic mass that was displacing the rectum and bladder, leading to bladder outlet obstruction. A suprapubic tube was placed for urinary diversion and a transvesical approach was used for tissue diagnosis. Biopsy confirmed the diagnosis of prostatic rhabdomyosarcoma. Patient underwent chemotherapy regiment with VAC (vincristine, actinomycin D and cyclophosphamide) and subsequently ifosfamide and doxorubicin. Eventually, due to tumor progression, the patient underwent a radical cystoprostatectomy with pelvic lymph node dissection and ileal conduit.

Open versus robot-assisted laparoscopic ureteral reimplantation: Hospital charges analysis and outcomes at a single institution.

PURPOSE: Robot-assisted laparoscopic extravesical ureteral reimplantation has previously been described as a viable minimally invasive option to open surgery. However, concerns for robotic surgery have been raised owing to assumed higher costs and heterogeneous clinical outcomes. We hypothesized that similar hospital charges and clinical outcomes occur when comparing open and robotic cases in matched cohorts. MATERIALS AND METHODS: Open and robotic reimplantation cases from 2013 to 2015 for primary vesicoureteral reflux were matched by age using 1:1 nearest neighbor matching. The matched cohorts were analyzed and compared for their direct itemized hospital charges per surgical case, complications, and clinical outcomes. RESULTS: There were 38 patients in each group after age-matching the 135 patients. Operating room charges were higher for the robotic group compared to the open group (p=0.002), whereas pharmacy and laboratory costs were lower for the robotic group. However, there were no significant differences in total overall charges between the open and robotic groups with cystoscopy or without cystoscopy (p=0.345, p=0.533), since the median hospital stay length was shorter for the robotic group (p<0.001). Clinical success rates were identical for the two groups (open 94.8% vs robotic 94.8%). There were also no significant differences in number of complications between the two cohorts. CONCLUSIONS: This is the first age-matched study comparing hospital charges and clinical outcomes of pediatric open and robotic reimplantation. While operating room charges were higher for the robotic cohort, lower hospitalization charges led to comparable overall hospital charges, as well as equivalent clinical outcomes for both cohorts. LEVEL OF STUDY: Level III (Retrospective comparative study) TYPE OF STUDY: Retrospective Study.

Does the use of 5 mm instruments affect the outcomes of robot-assisted laparoscopic pyeloplasty in smaller working spaces? A comparative analysis of infants and older children.

INTRODUCTION: Pediatric robot-assisted laparoscopic (RAL) pyeloplasty has become a viable minimally invasive surgical option for ureteropelvic junction obstruction (UPJO) based on its efficacy and safety. However, RAL pyeloplasty in infants can be a challenging procedure because of the smaller working spaces. The use of the larger 8 mm instruments for these patients instead of the 5 mm instruments is common because of the shorter wrist lengths. OBJECTIVE: We hypothesized that the use of 5 mm instruments for RAL pyeloplasty in infants with smaller working spaces will have comparable perioperative parameters and surgical outcomes in comparison with older children with larger working spaces. STUDY DESIGN: We compared the perioperative parameters and surgical outcomes of RAL pyeloplasties performed by a single surgeon in infants and non-infant pediatric patients over a 2 year period. All of the procedures were performed using an 8.5 mm camera and 5 mm robotic instruments. Patient demographics, operative times, perioperative complications, hospital pain medication usage, hospital length of stay, and treatment success rates were compared between the two groups. RESULTS: A total of 65 pediatric RAL pyeloplasties were included in the study (16 infants and 49 non-infants, Table). There were no significant differences in gender, laterality, proportion of re-do pyeloplasty, or preoperative hydronephrosis grade between the two groups. All procedures were performed without conversion to open surgery or significant perioperative complications. There were no differences in segmental operative times (total operative time, console time, port placement time, time for dissection to UPJO, and anastomosis time), hospital pain medication usage, and hospital length of stay between the two groups (p > 0.05 for all comparisons). The treatment success rates were 93.8% (15/16) and 100% (49/49), respectively (p = 0.08). DISCUSSION: We present the first comparative study of infant and non-infant pediatric RAL pyeloplasty using 5 mm robotic instruments. An advantage of the current study is the use of a single surgeon's experience to compare RAL pyeloplasty outcomes in infants with those of older children, a group in which RAL pyeloplasty has already been shown to be efficacious and safe. Operative tips for infant RAL pyeloplasty are also provided. CONCLUSIONS: RAL pyeloplasty is a safe and effective surgical modality even in infants, with comparable perioperative parameters and outcomes as those in older children. The use of 5 mm instruments in infants does not affect outcomes and offers the potential for improved cosmesis.

Quantifying the Additional Difficulty of Pediatric Robot-Assisted Laparoscopic Re-Do Pyeloplasty: A Comparison of Primary and Re-Do Procedures.

BACKGROUND: Re-do pyeloplasty after failed open or laparoscopic ureteropelvic junction (UPJ) obstruction correction can be a challenging procedure because of scar formation at the previous anastomosis site and decreased vascularity of the ureter. This study compared the perioperative parameters for pediatric robot-assisted laparoscopic (RAL) primary and re-do pyeloplasties with an emphasis on the intra-operative parameters. MATERIALS AND METHODS: We compared the perioperative parameters of pediatric RAL procedures performed by a single surgeon at a tertiary care children's hospital for both primary ureteropelvic junction obstruction (UPJO) and recurrent UPJO after a previous open or laparoscopic procedure over 2013-2015. The operative time was subdivided as total operative time, console time, port placement time, dissection time to UPJ, and anastomosis time. RESULTS: A total of 65 pediatric RAL pyeloplasty procedures for UPJO were performed (55 primary and 10 re-do pyeloplasties) during the study period. The console times were 43.3% longer for re-do pyeloplasties than for primary pyeloplasties (133.0 ± 30.7 versus 92.8 ± 24.0 minutes, respectively, P < .01). The re-do cases had longer operative times, especially for UPJ exposure (52.2 ± 21.0 versus 28.0 ± 14.0 minutes, P < .01). There were no conversions to open surgery or significant perioperative complications. There was no difference in hospital pain medication usage and hospital length of stay between the 2 groups. The treatment success rates were 98.2% (54/55) and 100% (10/10), respectively. CONCLUSIONS: RAL re-do pyeloplasty is associated with significantly longer operative times as compared with primary pyeloplasties, especially during the exposure of the UPJ, but it is overall a safe and effective surgical modality for persistent/recurrent UPJO in children. As surgeons are increasingly asked for more accurate predictions of operative time lengths when scheduling cases, this information can be helpful for surgeons when scheduling these cases and with counseling families.

Special Considerations in Pediatric Kidney Transplantation.

Universally accepted as the treatment of choice for children needing renal replacement therapy, kidney transplantation affords children the opportunity for an improved quality of life over dialysis therapy. Immunologic and surgical advances over the last 15 years have improved the pediatric patient and kidney graft survival. Unique to pediatrics, congenital genitourinary anomalies are the most common primary diseases leading to kidney failure, many with urological issues. Early urological evaluation for post-transplant bladder dysfunction and emphasis on immunization adherence are the mainstays of pediatric pretransplant and post-transplant evaluations. A child's height can be challenging, sometimes requiring an intra-abdominally placed graft, particularly if the patient is <20 kg. Maintenance immunosuppression regimens are similar to adult kidney graft recipients, although distinctive pharmacokinetics may change dosing intervals in children from twice a day to thrice a day. Viral infections and secondary malignancies are problematic for children relative to adults. Current trends to reduce/remove corticosteroid therapy from post-transplant protocols have produced improved linear growth with less steroid toxicity; although these studies are still ongoing, graft function and survival are considered acceptable. Finally, all children with a kidney transplant need a smooth transition to adult clinics. Future research in pertinent psychosocial aspects and continued technological advances will only serve to optimize the transition process. Although some aspects of kidney transplantation are similar in children and adults, for instance immunosuppression and immunosuppressive regimens, and rejection mechanisms and their diagnosis using the Banff criteria, there are important differences this review will focus on and which continue to drive innovation.

Urologic Considerations in the Separation of Thoracoomphalopagus Conjoined Twins.

Conjoined twins are seen in approximately 1/500,000 live births, and therefore surgical management of urologic anomalies in conjoined twins has not been extensively reported. Various degrees of sharing of the urinary tracts and genitalia can be seen in different types of conjoined twins. Detailed preoperative imaging, including magnetic resonance imaging, computed tomography, and voiding cystourethrogram, is essential to define the anatomy and planning of a successful separation. We describe the urologic presentation, evaluation, and treatment of thoracoomphalopagus conjoined twins.

Urologic Outcomes of Children With Hemorrhagic Cystitis After Bone Marrow Transplant at a Single Institution.

OBJECTIVE: To analyze clinical outcomes and the risk factors associated with genitourinary (GU) morbidity and mortality in children who present with hemorrhagic cystitis (HC) after bone marrow transplant (BMT). METHODS: A retrospective chart review of patients with HC who had undergone BMT at a single pediatric hospital from 2008 to 2015 was conducted. Demographic data, severity of hematuria, HC management, and mortality were analyzed. Bivariate analysis and binary logistic regression were performed to identify risk factors. RESULTS: Out of 43 patients who met inclusion criteria, 67.4% were male with a median age at BMT of 10.2 years (interquartile range 5.8-14.6). Percutaneous nephrostomy catheters were inserted in 5 patients for urinary diversion. All-cause mortality was 32.6% (N = 14). Intravesical retroviral therapy (P <.001), HC grade (P <.001), total Foley time (P <.001), total gross hematuria time (P <.001), total days hospitalized (P = .012), and days to most improved hematuria (P = .032) were associated with significant GU morbidity on bivariate analysis. On multivariable analysis, days to most improved hematuria was associated with significant GU morbidity odds ratio of 1.177 (1.006-1.376) (P = .042). Status of percutaneous nephrostomy was not associated with increased mortality (P = .472); however, in the multivariate model, BK viremia (P = .023), need for renal dialysis (P = .003), and presence of Foley catheter (P = .005) were associated with increased mortality. CONCLUSION: Children with HC after BMT fall in a very high-risk category with high mortality and significant GU morbidity. The presence of a Foley catheter, need for dialysis, and BK viremia are associated with increased mortality.

Vacuum-assisted Closure of a Vesicocutaneous Fistula in a Pediatric Patient After Bladder Cystoplasty.

A vesicocutaneous fistula is an abnormal communication from the bladder to the skin (Pritts et al, 2001). Recently, wound vacuum-assisted closure (VAC) has been used to facilitate fistula closure. There are no reports of using VAC to help fistula closure in the pediatric population. We present a case of an adolescent patient who develops a vesicocutaneous fistula after bladder augment cystoplasty and was treated with VAC only.

A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma.

Cystic partially differentiated nephroblastoma (CPDN) is a rare multicystic renal tumor along the spectrum of cystic nephroma and cystic Wilms' tumor. There have only been two previously reported cases of bilateral CPDN in the literature. We present here a case of bilateral CPDN vs cystic Wilms' tumor treated with neoadjuvant and adjuvant chemotherapy in addition to a bilateral partial nephrectomy. We also review the relevant literature regarding CPDN in an effort to aid in diagnosis and management of these rare cystic renal tumors.

Comparison of different extraction sites used during laparoscopic radical nephrectomy.

PURPOSE: Laparoscopic radical nephrectomy is commonly performed for renal tumors that are not amenable to nephron sparing treatment. A number of techniques for intact specimen extraction are used. The development of incisional hernias from the extraction site is a known but infrequent delayed complication. We analyzed different extraction sites and risk factors for such hernias. MATERIALS AND METHODS: We retrospectively analyzed a cohort of patients undergoing laparoscopic radical nephrectomy with intact specimen extraction through 3 sites. Patients and operation specific parameters were included with particular attention to factors predisposing patients to incisional hernia, including chronic obstructive pulmonary disease, diabetes mellitus, chronic steroid use and a high body mass index. RESULTS: A total of 181 nephrectomies were performed in 175 patients and 175 kidneys (96.7%) had malignancy. Mean tumor size was 4.9 cm. Mean followup was 28.8 months. Extraction was done from a lower quadrant site in 55 patients (31.4%), from the umbilical site in 58 (33.2%) and from a paramedian site in 62 (35.4%). Patients with paramedian and lower quadrant extraction sites were older (p = 0.016), and had a higher body mass index (p = 0.001) and greater specimen weight (p = 0.003). In 4 patients an incisional hernia developed. An incisional hernia was significantly associated with the paramedian extraction site (p = 0.015). CONCLUSIONS: Incisional hernias may occur as a delayed complication of laparoscopic radical nephrectomy. This complication most commonly develops at the extraction site. In patients with a high body mass index using a paramedian extraction site is a significant risk factor for incisional hernia formation.