The Importance of Fertility Preservation in the Transgender Population.

OBJECTIVE: To call to attention the often-overlooked aspect of pediatric transgender care: the importance of fertility preservation prior to instituting gender-affirming therapy. The transgender population has long been marginalized by society. Societal stigmata, fear to seek care, and dearth of provider knowledge regarding transgender health issues has caused disparities to widen. Gender affirming procedures and hormone therapy affect the long-term reproductive potential of transgender individuals. While cost concerns and insurance coverage regarding oncofertility is a prominent area of discussion, the transgender community is often excluded. METHODS: Sixteen genetically XY females, followed by their multidisciplinary transgender care teams, were interested in starting hormone therapy due to impending onset and/or progression of puberty. Their physicians were aware of fertility struggles after undergoing hormone therapy and therefore referred to urology. Sperm cryopreservation via open gonadal biopsy, testicular tissue cryopreservation (TTC), and semen sample (when age/maturity-appropriate) were discussed. Though requiring surgery, biopsy/TTC relieves patients of the psychological impact of semen sample production. RESULTS: Under IRB approval, 15 patients (median age 12 years, range 10-16 years) underwent TTC (Figure 1). One patient (aged years) opted for semen sample. All patients had success with spermatogonial stem cells cryopreserved for future patient use. CONCLUSIONS: With more individuals beginning medical and surgical therapy at a younger age, fertility preservation discussions are essential but often overlooked, depriving these individuals the joy of becoming a biological parent. TTC can be safely done in pediatric populations, though research is necessary to expand beyond current experimental stage of tissue-development.

Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

And then there was one incision. First single-port pediatric robotic case series.

BACKGROUND: In the past two decades, technology has advanced to augment an already minimally-invasive approach in laparoscopic surgery. Robotic-assisted laparoscopic platforms have now evolved to its 4th-generation product: a single-port system, first cleared through the FDA for urologic procedures last year. A single, 2.5 cm incision allows for placement of a port that admits a fully-wristed camera as well as three fully-wristed instruments, all controlled by the surgeon at the console. OBJECTIVE: We sought to document the feasibility of the single-port (SP) robotic platform in the first clinical series of pediatric patients, reporting use of this system for dismembered pyeloplasty and Mitrofanoff. Secondary aims were to report intraoperative details and perioperative outcomes. STUDY DESIGN: Seven patients underwent surgery using the da Vinci SP Surgical System (Intuitive Surgical, Sunnyvale, CA). Six patients, two girls and 4 boys, were diagnosed with ureteropelvic junction obstruction and underwent SP robotic-assisted dismembered pyeloplasty while one male patient with neurogenic bladder underwent SP robotic-assisted Mitrofanoff procedure. Patient's ages ranged from 22 months to 14 years. A 2.5-cm incision was made within the Pfannenstiel line in HIdES fashion for the pyeloplasties, while the previous gastrostomy tube site was used for the Mitrofanoff. Through this incision a 25-mm multichannel port was placed. The 12 × 10-mm articulating robotic camera and two 6-mm articulating robotic instruments were utilized. RESULTS: All surgeries were completed successfully through the single port without intraoperative complications, need for separate ports, or conversion. Median operative time was 120 min, and all patients were dismissed in less than 24 h, taking only acetaminophen and ibuprofen for pain control. There was no issue with instrumentation in older patients; however, shorter working distance in the 22-month-old pyeloplasty limited wristing of the instruments. CONCLUSIONS: We report the first cases utilizing the SP robotic platform in children. Despite their smaller size and limited workspace, we had no issues with instrument clashing or triangulation in older patients, completing the procedures in a similar timeframe as multiport robotic platforms. Use of the SP platform is not recommended if working distance will be < 10 cm from the end of the port as instrument movement is prohibitive. The HIdES approach of placing the port in the Pfannenstiel line gave additional working distance and kept the incision below the swimsuit line for excellent cosmesis (Figure 1). Further study with additional cases will compare this approach with standard multiport robotics to analyze and compare operative data and outcomes.

Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study.

BACKGROUND: We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy (CE) in a multi-institutional study. METHODS: We performed a cross-sectional study of people with CE and covered variants managed at five participating institutions. Those with <1 year follow-up or born with variants without hindgut involvement were excluded. Primary outcomes were methods of urinary and bowel management. Urinary management included: voiding via urethra, clean intermittent catheterizations (CIC), incontinent diversion and incontinent in diaper. Bowel management included: intestinal diversion (colostomy/ileostomy) and pull-through (with/without MACE). We evaluated three age groups: children (<10 years), older children (10 to <18) and adults (≥18). We assessed if management varied by age, institution or time (born≤2000 vs. >2000). RESULTS: A total of 160 patients were included (40% male). Median follow-up was 15.2 years (36% children, 22% older children, 43% adults). While 42% of children were incontinent in diapers, 73% of older children and adults managed their bladder with CIC, followed by incontinent urinary diversion (21%) (p < 0.001, Table). CIC typically occurred after augmentation (88%) via a catheterizable channel (89%). Among older children and adults, 86% did not evacuate urine per urethra and 28% of adults had an incontinent urinary diversion. No child or adult voided per urethra. Age-adjusted odds of undergoing incontinent diversion was no different between institutions (p = 0.31) or based on birthyear (p = 0.08). Most patients (79%) had an intestinal diversion, irrespective of age (p = 0.99). Remaining patients had a pull-through, half with a MACE. The probability of undergoing bowel diversion varied significantly between institutions (range: 55-91%, p = 0.001), but not birth year (p = 0.85). SUMMARY: We believe this large long-term data presents a sobering but realistic view of outcomes in CE. A limitation is our data does not assess comorbidities or patient-reported outcomes. Rarity of volitional urethral voiding in CE forces the question of whether is a potentially unachievable goal. We advocate thoughtful surgical decision making and thorough counseling about appropriate expectations, distinguishing between volitional voiding and urinary and fecal dryness. CONCLUSIONS: In this long-term, multi-institutional study of patients with CE, 94% of older children and adults manage their bladder with incontinent diversion or CIC. Nearly 80% of patients, regardless of age, have an intestinal diversion. Given that no patients were dry and voided via urethra and 86% of older patients do not evacuate urine per urethra, these data bring into question what functional goals are achievable when performing reconstructive surgery for these patients.

Imaging of pre- and post-cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pediatric intraperitoneal malignancy.

Although rare, pediatric peritoneal carcinomatosis does occur in primary abdominopelvic tumors. Additionally, peritoneal carcinomatosis has been described to occur as metastatic disease where the primary tumor is outside the abdominopelvic cavity. Where amenable, cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) can be beneficial in disease management. However, favorable outcomes are predicated on specific tumor histology as well as proper patient selection, which significantly relies on preoperative imaging. This review gives a comprehensive, up-to-date summary on pediatric peritoneal carcinomatosis pre-surgical evaluation; where imaging is beneficial and limited; pediatric radiologists' role in helping to quantify disease; and how we, as pediatric radiologists, can help the surgeons and oncologists in the selection of patients for cytoreductive surgery and HIPEC.

Spinal anesthesia in infants undergoing urologic surgery duration greater than 60 minutes.

INTRODUCTION: Spinal anesthesia (SA) has been safely utilized in infants. There are limited data regarding the safety and efficacy of SA in pediatric urologic surgery lasting ≥60 min. We outlined the perioperative course for infants undergoing single-injection 0.5% plain bupivacaine SA-only for urologic procedures lasting ≥60 min. OBJECTIVE: To characterize the safety and efficacy of SA for urologic surgery in infants lasting ≥60 min. METHODS: We reviewed our prospectively maintained database of infants undergoing SA for urologic procedures lasting ≥60 min from May 2018 to March 2021. Patients received preoperative intranasal dexmedetomidine, some received intranasal fentanyl, and all patients received lidocaine cream applied preoperatively over the lumbar spine. Oral sucrose on a pacifier was provided as needed, and the patient's arms were swaddled for the procedure. Success was defined as no conversion to general anesthesia. Time points for start/end of spinal injection, procedure duration, wheels in/out of operating room (OR), and discharge were collected. RESULTS: Of 245 cases conducted with SA during the study period, 76 (31%) infants underwent surgery lasting ≥60 min. Of these, 73 (96%) were successfully completed with SA alone. In the 3 cases converted to general anesthesia, 2 (67%) required mask anesthesia after 96 and 169 min (for the last <10 min of surgery), and one was converted to intubation before start of surgery. Median patient age was 6 (IQR 5-7) months, and median procedure length was 95 (IQR 75-120) minutes. Following initial preoperative intranasal dexmedetomidine ± fentanyl, at least one additional dose of IV sedative was given in 27 (36%) cases at a median time of 90 (IQR 60-120) minutes into surgery. Following closure, patients exited the OR after a median 10 (IQR 8-12) minutes and subsequently discharged after spending a median of 73 (IQR 61-96) minutes in recovery. DISCUSSION: We describe pediatric urologic surgical cases lasting ≥60 min that employed single-injection intrathecal bupivacaine alone without adjunct intrathecal agents. In this report, SA was safely utilized in infants undergoing urologic procedures lasting at least 60 min, with about 40% of patients receiving additional IV dexmedetomidine and fentanyl. Non-medication measures (swaddling, oral sucrose) were important for maximizing patient comfort. Communication between surgeon and anesthesia as cases progress is key to maintaining adequate anesthesia. CONCLUSION: A single-injection bupivacaine-only spinal anesthesia approach for urologic surgery lasting over an hour and up to 3 h is safe and effective in infants. Selecting appropriate candidates for SA should be a joint decision between the surgeon and the anesthesiologist.

Collateral Damage From Fetal Interventions: Increasing the Complexity of Complex Patients.

Fetal interventions are often key to fetal survival and growth; however, they can often have complications causing significant morbidity and mortality. This case highlights not only a complication of fetal surgery, but also a very unusual diagnosis. We present the case of a male fetus who was diagnosed with urethral atresia and subsequently underwent 2 vesicoamniotic shunt placements. At birth, he was diagnosed with Megacystis Microcolon Intestinal Hypoperistalsis Syndrome and was noted to have rectovesical and vesicocutaneous fistulae likely iatrogenically created from shunt placement. While fetal interventions are often required, a multidisciplinary team approach is often necessary as complications occur.

Validation of the modified Bosniak classification system to risk stratify pediatric cystic renal masses: An international, multi-site study from the pediatric urologic oncology working group of the societies for pediatric urology.

BACKGROUND: Pediatric cystic renal lesions are challenging to manage as little is known about their natural course. A modified Bosniak (mBosniak) classification system has been proposed for risk stratification in pediatric patients that takes ultrasound (US) and/or computed tomogram (CT) characteristics into account. However, literature validating this system remains limited. OBJECTIVE: To determine if the mBosniak classification system correlates with pathologic diagnoses. The hypothesis is that mBosniak classification can stratify the risk of malignancy in children with renal cysts. STUDY DESIGN: Patients treated for cystic renal masses with available imaging and pathology between 2000 and 2019 from five institutions were identified. Clinical characteristics and pathology were obtained retrospectively. Characteristics from the most recent US, CT, and/or magnetic resonance imaging (MRI) were recorded. Reviewers assigned a mBosniak classification to each scan. mBosniak scores 1/2 were considered low-risk and 3/4 high-risk. These groups were compared with pathology (classified as benign, intermediate, malignant). Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (+LR), and negative likelihood ratio (-LR) were calculated to assess this categorization as a screening tool to guide surgical intervention. Agreement between imaging modalities was also explored. RESULTS: 99 patients were identified. High-risk imaging findings were correlated with malignant or intermediate pathology with a sensitivity of 88.3%, specificity of 84.6%, PPV of 89.8%, NPV of 82.5%, +LR of 5.7, and -LR of 0.14. The sensitivity for detecting malignant lesions only was 100%. There was substantial agreement between US/CT (n = 55; κ = 0.66) and moderate agreement between US/MRI (n = 20; κ = 0.52) and CT/MRI (n = 13; κ = 0.47). DISCUSSION: The mBos classification system is a useful tool in predicting the likelihood of benign vs. intermediate or malignant pathology. The relatively high sensitivity and specificity of the system for prediction of high-risk lesions makes this classification applicable to clinical decision making. In addition, all malignant lesions were accurately identified as mBosniak 4 on imaging. This study adds substantial data to the relatively small body of literature validating the mBosniak system for risk stratifying pediatric cystic renal lesions. CONCLUSIONS: Pediatric cystic renal lesions assigned mBosniak class 1/2 are mostly benign, whereas class 3/4 lesions are likely intermediate or malignant pathology. We observed that the mBosniak system correctly identified pathology appropriate for surgical management in 88% of cases and did not miss malignant pathologies. There is substantial agreement between CT and US scans concerning mBos classification.

Surfaceome Profiling of Rhabdomyosarcoma Reveals B7-H3 as a Mediator of Immune Evasion.

Novel therapeutic strategies are needed for the treatment of rhabdomyosarcoma (RMS), the most common soft-tissue sarcoma in children. By using a combination of cell surface proteomics and transcriptomic profiling of RMS and normal muscle, we generated a catalog of targetable cell surface proteins enriched in RMS tumors. Among the top candidates, we identified B7-H3 as the major immunoregulatory molecule expressed by RMS tumors. By using a large cohort of tissue specimens, we demonstrated that B7-H3 is expressed in a majority of RMS tumors while not detected in normal human tissues. Through a deconvolution analysis of the RMS tumor RNA-seq data, we showed that B7-H3-rich tumors are enriched in macrophages M1, NK cells, and depleted in CD8+-T cells. Furthermore, in vitro functional assays showed that B7-H3 knockout in RMS tumor cells increases T-cell mediated cytotoxicity. Altogether, our study uncovers new potential targets for the treatment of RMS and provides the first biological insights into the role of B7-H3 in RMS biology, paving the way for the development of next-generation immunotherapies.

Improving Operating Room Efficiency Using the Six Sigma Methodology.

OBJECTIVE: To help improve operating room efficiency in a tertiary care facility, Six Sigma analysis was utilized. Six Sigma is a technique driven by data, methodology, and a philosophy of continuous improvement to help create a system in which 99.99966% of products are free from defects. This process helps to reduce variability and waste in production, while increasing quality and decreasing cost of the final product. METHODS: The steps of define, measure, analyze, improve, and control (DMAIC) methodology were used. Variables measured included close-to-cut time, patient-out to patient-in time, and room utilization. Statistical analysis was conducted, while an individual was sent to shadow various levels of operating room personnel to help recognize possible shortcomings in the system, including communication and coordination errors. RESULTS: Baseline results were recorded for each surgical specialty to help determine the specific needs of each OR team. Changes in workflow, such as staggered start anesthesia times, were recommended for each level of personnel based on statistical analysis and observation. Results were followed for several years with initial data showing success of the process. However, ongoing construction and resistance to change has prevented further review. CONCLUSION: While Six Sigma can be a valuable tool, it is essential to have a proper leadership team that includes all stake holders. The process requires horizontal and vertical leadership as well as buy-in from all individuals affected by the process. The synergy of these factors is key for success.

Single-port robotic Mitrofanoff in a pediatric patient.

INTRODUCTION: Pediatric surgery began with single-incision flank surgery and has evolved to multi-port laparoscopic and robotic approaches. Recent technological advances with the single-port (SP) robot have allowed for transition back to single-incision surgery. METHODS: A 14-year-old paraplegic male with T2 spinal injury presented with neurogenic bladder and increasing difficulty performing clean intermittent catheterization thus the decision was made to perform the first SP robotic Mitrofanoff procedure in a pediatric patient. The SP platform has one 2.5 cm, 4-channel port, a 12 × 10 mm articulating camera, and 6 mm multi-wristed instruments. DISCUSSION: The SP robotic Mitrofanoff was completed successfully without issues with space, triangulation or articulation. There is, however, loss of insufflation with use of laparoscopic instruments as the seal on the port is difficult to maintain. The single-port robot has been successfully utilized in seven patients: six underwent dismembered pyeloplasty and one underwent Mitrofanoff with a median operative time of 120 min and estimated blood loss of <25 cc. Postoperatively, no patients required opioid pain medications, and all were discharged in <24 h without complications. CONLUSIONS: Single-port robotic surgery is feasible in pediatric patients, but patient selection is key. Future development of the platform is needed to widen application to smaller patients.

Feasibility Study of Vascularized Composite Urinary Bladder Allograft Transplantation in a Cadaver Model.

PURPOSE: The purpose of this study was to establish the feasibility of performing a urinary bladder vascularized composite allograft transplantation for either bladder augmentation or neobladder creation. MATERIALS AND METHODS: Six adult cadavers were studied. Cadavers were excluded for any previous pelvic surgery, radiation, vascular surgery or history of pelvic malignancy. An intravascular colored silicone and barium mixture was injected and both computerized tomography scans and gross dissections were performed. Contrast enhanced computerized tomography imaging was used to delineate urinary bladder vascular anatomy variability. Bladders were explanted en bloc from 2 cadavers with bilateral vascular pedicles based on the external iliac vessels and "transplanted" to replicate a bladder transplant. RESULTS: Contrast enhanced 3-D-computerized tomography reconstructions and cadaver dissections revealed distal vascular variability with proximal blood supply based primarily on the internal iliac artery. Urinary bladder vascularized composite allograft transplantation was successfully performed during 2 mock transplants with the vascular anastomosis done to the recipient external iliac artery and vein. CONCLUSIONS: Urinary bladder vascularized composite allograft transplantation is technically and anatomically feasible. This procedure may obviate the use of intestinal segments for bladder reconstruction in select patients. A phase 1 clinical trial is in progress.

Consult and procedure incidence outcomes following establishment of a fertility preservation program for children with cancer.

PURPOSE: Fertility is a quality of life outcome adversely affected by cancer therapy. Many childhood cancer patients, however, are not offered options to preserve their fertility. Providers acknowledge difficulty discussing impaired fertility to patients due to lack of knowledge of available options. Our objective was to review the impact of a pediatric multidisciplinary fertility preservation program on providers' fertility preservation counseling and discussion of options. METHODS: A retrospective medical chart review was conducted for pediatric cancer patients prior to and following program establishment. Fertility preservation discussions, consults, and incidence were noted. Following filtering and stratification, 198 and 237 patients were seen prior to and following program establishment, respectively. RESULTS: Following program establishment, provider-patient discussions of impaired fertility (p = 0.007), fertility preservation consults (p = 0.01), and incidence of fertility preservation procedures (p < 0.001) increased among patients. Furthermore, the number of patients who received fertility preservation consults after receiving gonadotoxic treatment decreased (p < 0.001). This trend was particularly noted in pre-pubertal and female patients, for whom fertility preservation options are limited without an established program. CONCLUSION: The establishment of a formal program greatly improved access to fertility preservation consults and procedures in children with cancer.

Robot-Assisted Laparoscopic Urachal Excision Using Hidden Incision Endoscopic Surgery Technique in Pediatric Patients.

Introduction: Although laparoscopic urachal excision in children has been well reported, there are limited data on a robot-assisted surgery (RAS) approach. The hidden incision endoscopic surgery (HIdES) technique is an established method of eliminating visible scars following a number of RAS urologic procedures. We report our experience of using a robotic approach to treat urachal anomalies in children, and we present the first description of utilizing the HIdES port configuration for this procedure. Materials and Methods: We retrospectively reviewed pediatric patients who underwent resection of a urachal remnant at our institution from 2013 to 2018. Surgical techniques were either the traditional open approach (OA) or RAS. HIdES trocar placement configuration was employed in all robotic cases. Patient demographics, perioperative data, pathology reports, and outcomes were abstracted and compared. Results: Twenty-three patients underwent a urachal remnant resection in the study period (RAS: 14 patients vs OA: 9 patients). RAS patients were older (8.5 vs 2.0 years, p = 0.031) and weighed more than OA patients (36.1 vs 13.9 kg, p = 0.063). Median operative time for RAS was longer than OA operative time (136 vs 33 minutes, p < 0.01). Fewer RAS patients were outpatient compared with OA (7.1% vs 66.7%, p < 0.01), but with a median length of stay of 1 day (0-1 day). Two patients (14.3%) in the RAS group experienced postoperative complications within 1 week of the procedure compared with 1 (11.1%) in the OA group. There was no significant difference in blood loss, postoperative narcotic requirements, or duration of follow-up between both groups. Conclusion: RAS is a safe and feasible alternative to open surgery for urachal anomalies. Complete excision can be achieved by using HIdES port configuration, allowing for excellent cosmetic outcomes that are superior to traditional surgical scars without limitation to essential surgical ergonomics.

Fertility Preservation: A Tale of Two Testicles.

OBJECTIVE: The transgender population has long been marginalized by society. Societal stigmata, fear to seek care, and dearth of provider knowledge regarding transgender health issues has caused disparities to widen. The purpose of this case study is to call to attention the often-overlooked aspect of transgender care: the importance of fertility preservation prior to undergoing therapy. METHODS: 13 and 16-year old genetically XY patients presented to a tertiary care facility for gender affirmation. Both self-identified as female since a young age and successfully socially transitioned. Impending onset and/or progression of puberty prompted patients to seek hormonal therapy. Fortunately, physicians in transgender clinic were aware of fertility struggles after undergoing hormone therapy and referred for consultation. RESULTS: Sperm cryopreservation via open gonadal biopsy, tissue cryopreservation, and semen sample were discussed. Though invasive, biopsy relieves patients of the psychological impact of sample production and is indicated in pubertal immaturity. After further discussion with patients and parents, the 13-year-old decided to undergo testicular biopsy while the 16-year old opted for semen sample. Both patients had success and their genetic material was cryopreserved for future assisted reproduction. CONCLUSION: Gender affirming procedures and hormone therapy affect the long-term reproductive potential of transgender individuals. While cost concerns and insurance coverage regarding oncofertility is a prominent area of discussion, the transgender community is often excluded. With more individuals beginning medical and surgical therapy at a younger age, fertility preservation discussions are essential but often overlooked, depriving these individuals the joy of becoming a biological parent.

Implementation of a spinal anesthesia and sedation protocol that reliably prolongs infant spinal anesthesia: Case series of 102 infants who received spinal anesthesia for urologic surgery.

BACKGROUND: The use of spinal anesthesia in infants is seeing resurgence as an alternative to general anesthesia. AIMS: Our primary aims are to describe our institution's experience introducing a spinal anesthesia and sedation protocol for infants undergoing urologic surgery, to describe methods of improving prolonged anesthesia, and to describe the failure rate of spinal anesthesia in these patients. Sedation was provided for some infants with intranasal dexmedetomidine ± fentanyl. METHODS: This is a retrospective case series examining infants aged 1-<14 months who received spinal anesthesia for circumcision, orchiopexy, orchiectomy, hypospadias repair, or epispadias repair. The electronic medical record was reviewed and compared with unmatched historical controls who received general anesthesia. RESULTS: A total of 230 patients underwent a urologic procedure; 102 patients received spinal anesthesia and 128 received general anesthesia. Length of surgical time with spinal anesthesia ranged from 4 to 189 minutes. The hospital length of stay was shorter in the spinal anesthesia group (median [IQR] of 5.3 hours [4.3, 7.2]) compared to the general anesthesia group (17.1 hours [15.6, 17.5]).The median bupivacaine dose was 0.75 mg/kg [0.67, 0.85]. There was one case in which cerebral spinal fluid was unable to be obtained, and one case that required conversion to general anesthesia after surgery had started. There were no cases of apnea, bleeding, infection, or neurologic compromise. CONCLUSIONS: We describe the successful implementation of an infant spinal anesthesia and sedation protocol and a technique that uniquely provides prolonged surgical anesthesia with a low failure rate. We also report shorter anesthesia time, surgical time, and recovery room length of stay in patients who received spinal anesthesia compared to general anesthesia.

Use of Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in Pediatric Sarcoma for Maximal Oncologic Control.

Few centers worldwide have trialed cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in pediatric rhabdomyosarcoma. This case involves a 5-year-old boy with gross hematuria who was found to have an 8 cm pelvic mass, likely arising from the prostate and extending along the bladder wall. Excisional biopsy revealed undifferentiated fusion negative sarcoma. The mass demonstrated reduction in size with chemotherapy and photon radiation therapy. He presented to our institution for delayed primary excision, and underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy using cisplatin. Follow-up imaging 15 months postoperatively demonstrates no evidence of disease.

Full thickness genital burns independently increase the odds of death among pediatric burn patients.

BACKGROUND: There are limited published data characterizing pediatric burn patients with genital burns (GB). OBJECTIVE: Assess prevalence of GB in pediatric burn patients and analyze clinical characteristics including predictors of mortality. STUDY DESIGN: We queried American Burn Association's National Burn Repository to identify all pediatric burn patients who presented to North American burn centers over a 10-year period. We excluded all patients aged ≥18 years and patients with unknown sex, race, and/or mortality. We also excluded subsequent encounters for patients with multiple visits. Demographic and clinical characteristics were compared between patients with and without GB. Univariable and multivariable logistic regression analyses were performed to identify predictors of mortality. RESULTS: Among 38 211 pediatric burn patients, 1244 (3.3%) suffered from second- or third-degree GB. Patients who suffered from third-degree GB (GB3) were significantly older than patients who suffered from second-degree GB (GB2) or patients without GB. Of the patients, 32.3% were aged 0-2 years. Scalding was the most common mechanism of injury for pediatric GB patients at 73.8%. Compared to non-GB patients, GB patients had significantly higher total body surface area (TBSA) burned (16.5% vs 7.0%), higher rates of associated inhalation injury (4.1% vs 2.6%), longer length of stay (LOS) (14.3 days vs 6.7 days), higher rates of urinary tract infection (UTI) (13.0% vs 2.8%) and sepsis (14.1% vs 2.3%), and higher mortality (3.5% vs 0.7%) (P < 0.0001 for all). The differences were more pronounced for the subset of patients who suffered from GB3 (TBSA 43.5%, associated inhalation injury 19.9%, LOS 42.9 days, 21.3% UTI, 33.3% sepsis, and 19.3% mortality). On multivariable analysis, the presence of GB3, TBSA, non-white ethnicity, and the presence of associated inhalation injury were significant predictors of mortality. Only 4.5% of pediatric GB patients underwent genital surgery, with the majority consisting of excision, reconstruction, or repair of the penis, vulva, or perineum. No patient required orchiectomy or suprapubic catheter placement. DISCUSSION: This is the largest study to date of pediatric GB patients. A minority of pediatric burn patients present with GB. However, when they occur, GB are associated with significantly worse clinical outcomes. Importantly, the presence of GB3 is an independent predictor of mortality in pediatric burn patients. CONCLUSION: The presence of GB appears to be a strong marker of severe burn injury. Pediatric GB patients need to be carefully assessed and aggressively managed for additional injuries, complications, surgical needs, and mortality risk.

Endoscopic-assisted robotic pyelolithotomy: a viable treatment option for complex pediatric nephrolithiasis.

INTRODUCTION AND OBJECTIVE: Endourological and percutaneous approaches are the standard of care for treatment of pediatric urolithiasis. However, in certain situations, an endoscopic-assisted robotic pyelolithotomy (EARP) can be an acceptable alternative. Limited data exist on pediatric EARP; thus, the authors describe their experience. METHODS: Patient selection: The authors retrospectively analyzed the records of all robotic procedures performed at five institutions from 7/09-10/17 to identify patients who underwent EARP. The authors collected demographics data, indications, operative time, and postoperative complications. Stone composition was reported as the majority composition (≥50%), unless any uric acid or struvite was noted, and those stones were classified as such. TECHNIQUE: Through a traditional or hidden incision endoscopic surgery (HIdES) robot pyeloplasty approach, the authors are able to easily pass a flexible endoscope through a robotic trocar and into the renal collecting system to perform pyeloscopy or ureteroscopy. Stones were primarily retrieved via the pyelolotomy and, if indicated, treated with laser lithotripsy. RESULTS: The authors identified 26 patients who underwent EARP in 27 renal units. Median patient age was 12.2 years (interquartile range [IQR] 6.1-14.5 years), and body mass index was 17.5 kg/m2 (IQR 16.5-25.4 kg/m2). The median pre-operative dimension of the largest stone was 9.0 mm (IQR 5.8 mm-15.0 mm). Reasons for EARP: 21 (77.8%) concomitant pyeloplasty, four (14.8%) altered anatomy precluding other techniques, and two (7.4%) multiple large stones. Multiple stones were present in 20 renal units (74.1%). Stones were located in the renal pelvis in nine (33.3%), lower pole in 10 (37.0%), ureter in one (3.7%), and multiple locations in seven (25.9%). Hidden incision endoscopic surgery approach was used in 14 (51.9%), and the median operative time was 237.5 min (IQR 189.8-357.8 min) with a median length of stay 1.0 day (IQR 1.0-2.0 days). Stone composition included calcium oxalate in 14 (51.9%), calcium phosphate in five (18.5%), cysteine in two (7.4%), struvite in two (7.4%), and unknown in four (14.8%). Overall stone free status was 19 (70.4%); of the eight (29.6%) renal units with residual stones, four underwent ureteroscopy, two extracorporeal shockwave lithotripsy (ESWL), one spontaneously passed, and one underwent percutaneous nephrolithotomy (PCNL). After secondary treatment, final stone free rate was 96.3%. Complications included stent migration and admission for urosepsis. At a median follow-up of 12 months (IQR 6.2-19.2 months), five (18.5%) had stone recurrence. CONCLUSIONS: Endoscopic-assisted robotic pyelolithotomy is a reasonable treatment option for select pediatric patients with concomitant ureteropelvic junction obstruction and nephrolithiasis or pediatric patients with stones inaccessible by standard methods.

A Systematic Review of Complications of Minimally Invasive Surgery in the Pediatric Urological Literature.

PURPOSE: Minimally invasive surgery has been gaining momentum in pediatric urology and it is essential to analyze the unique complications pertinent to this approach. We conducted a systematic review to evaluate pediatric minimally invasive surgery complications in the published urological literature. MATERIALS AND METHODS: We searched PubMed®/MEDLINE® using relevant pediatric minimally invasive surgery terminology and applied specified eligibility criteria. The Clavien-Dindo classification scheme was used to categorize postoperative complications. For studies not using Clavien-Dindo, complications were recategorized into Clavien-Dindo grades. Primary outcome was frequency of grade III complications and conversions to open surgery. Covariates were surgery type (pyeloplasty, nephrectomy, partial nephrectomy, ureteral reimplantation and complex reconstruction) and surgical approach (laparoscopic, robotic assisted and/or laparoendoscopic single site). Proportions were compared using the chi-square test (α=0.05). RESULTS: Overall 123 studies met the inclusion criteria, reporting outcomes of 5,864 patients. About a third (35.8%) of studies used the Clavien-Dindo classification. Nephrectomy had a significantly lower frequency of grade III complications (1.18%) compared to pyeloplasty (3.64%), ureteral reimplantation (6.65%) and complex reconstruction (11.76%) (p <0.05). Complex reconstruction had a significantly higher frequency of grade III complications (11.39%) compared to all other analyzed surgeries (p <0.05). CONCLUSIONS: The rate of complications and open conversions varies by surgical procedure in pediatric urological minimally invasive surgery. Despite the existence of a standardized complication classification system, the majority of reviewed publications did not report complications in a standardized fashion. Our findings call for more robust studies in pediatric minimally invasive surgery and universal implementation of standardized complication reporting.