BACKGROUND:Spinal anesthesia is an alternative to general anesthesia in infants. A caveat of spinal anesthesia in infants is short block duration. Clonidine is a common adjunct that prolongs spinal anesthesia. The mechanisms by which clonidine prolongs spinal anesthesia are unknown. Infants under spinal anesthesia appear in a sleep-like state. We hypothesized that infants receiving bupivacaine spinal anesthesia with clonidine may exhibit more sleep spindles on the electroencephalogram (EEG). METHODS:We obtained intraoperative frontal EEG recordings in 73 infants under spinal anesthesia. We compared EEG spectral features of bupivacaine and bupivacaine + clonidine spinal anesthesia using nonparametric multitaper spectral analysis. A recently developed switching state-space modeling approach was then applied to extract and compare spindle features in bupivacaine versus bupivacaine + clonidine spinal anesthesia. We then applied the same model to compare younger versus older infants. RESULTS:There was no difference in the power spectra and sleep spindle detection probability between bupivacaine and bupivacaine + clonidine spinal anesthesia ( P = .51). We found age-related EEG changes in both bupivacaine and bupivacaine + clonidine spinal anesthesia independent of clonidine. Increasing age was associated with decreased spectral power from 0 to 0.6 Hz (median difference -2.9 dB, 95% CI [-5.3, -0.5,]) and increased power from 2 to 15 Hz (median difference 3.4 dB, 95% CI [1.5, 5.2]). Increasing age was also associated with increased spindle strength (R 2 = 0.323, F(2,67) = 15.98, P < 0.001). These EEG findings mirror those found in infants under physiologic sleep. CONCLUSIONS:Our findings suggest that low-dose clonidine does not impact sleep spindle properties in the EEG of infants under spinal anesthesia. The EEG of infants under spinal anesthesia demonstrate age-related changes that mirror quiet physiologic sleep. In addition, the presence of intrathecal clonidine has no effect on the age-related changes in the EEG pattern. Clonidine is an adjunct for spinal anesthesia in infants that appears to prolong anesthetic duration without affecting their EEG patterns of physiologic sleep.

Myhre syndrome is an increasingly diagnosed ultrarare condition caused by recurrent germline autosomal dominant de novo variants in SMAD4. Detailed multispecialty evaluations performed at the Massachusetts General Hospital (MGH) Myhre Syndrome Clinic (2016-2023) and by collaborating specialists have facilitated deep phenotyping, genotyping and natural history analysis. Of 47 patients (four previously reported), most (81%) patients returned to MGH at least once. For patients followed for at least 5 years, symptom progression was observed in all. 55% were female and 9% were older than 18 years at diagnosis. Pathogenic variants in SMAD4 involved protein residues p.Ile500Val (49%), p.Ile500Thr (11%), p.Ile500Leu (2%), and p.Arg496Cys (38%). Individuals with the SMAD4 variant p.Arg496Cys were less likely to have hearing loss, growth restriction, and aortic hypoplasia than the other variant groups. Those with the p.Ile500Thr variant had moderate/severe aortic hypoplasia in three patients (60%), however, the small number (n = 5) prevented statistical comparison with the other variants. Two deaths reported in this cohort involved complex cardiovascular disease and airway stenosis, respectively. We provide a foundation for ongoing natural history studies and emphasize the need for evidence-based guidelines in anticipation of disease-specific therapies.

You have accessJournal of UrologyCME1 May 2022MP08-17 SPINAL ANESTHESIA FOR INFANT OUTPATIENT GENITAL SURGERY Jack Elder, Andrew Chen, Jacquelin Peck, Christine Mai, Diana Liu, David August, and Chang A. Liu Jack ElderJack Elder More articles by this author , Andrew ChenAndrew Chen More articles by this author , Jacquelin PeckJacquelin Peck More articles by this author , Christine MaiChristine Mai More articles by this author , Diana LiuDiana Liu More articles by this author , David AugustDavid August More articles by this author , and Chang A. LiuChang A. Liu More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002530.17AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Spinal anesthesia (SA) avoids the need for endotracheal intubation in general anesthesia (GA), reduces the risk of adverse airway events and avoids intraoperative narcotic administration. We hypothesized that SA is a safe alternative to GA in healthy infants with genital anomalies in the ambulatory setting. METHODS: A prospective IRB-approved database for pediatric patients undergoing urologic surgery under SA was created. Typically, patients <10 months and <10 kg are selected for SA. EMLA cream is applied to the L3-L5 interspace for 30 minutes. Patients receive intrathecal plain 0.5% bupivacaine 1 mg/kg without additional sedatives. Clonidine 1 µg/ml is added to extend the duration of the block if the procedure is anticipated to last >75 minutes. Patients are soothed with a sugar solution and pacifier. Patients receive a caudal block following the procedure for postoperative pain relief. Patient age, gestational age, weight, time to place spinal anesthetic, spinal medication(s) used, surgical procedure, procedure length and peri-anesthetic events were recorded. RESULTS: A total of 68 patients were included (Table 1). Mean patient age was 5 months and mean weight was 7.3 kg. Average time for placement of SA was 10.4 ± 2.5 minutes. Clonidine was administered in 42/68 (61.8%), and combined spinal/caudal anesthesia was employed in 8/68 (11.8%). Operative time was >60 minutes in 53 (78%). Of the 68 cases, 1 was converted to GA (1.5%) due to intolerance of laparoscopic insufflation. Adjunctive intravenous sedation was required in 14 cases. Average procedure time was significantly higher among cases performed with combined spinal/caudal catheter (139 vs 73 mins, p <0.001). Intraoperative apnea occurred in 7 patients (5 received clonidine, 2 did not, p=0.285), of whom 4 required manual stimulation while none required further intervention. There were no anesthetic events requiring post-operative management. All patients were brought awake and ready to feed to the PACU and discharged after feeding. CONCLUSIONS: SA is a safe and effective alternative to GA in young infants undergoing outpatient genital surgery. SA reduces the need for invasive airway placement, improves OR efficiency, and shortens PACU time. Surgical time can be prolonged by use of adjuncts such as clonidine and combined spinal/caudal catheter. Source of Funding: None © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e132 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Jack Elder More articles by this author Andrew Chen More articles by this author Jacquelin Peck More articles by this author Christine Mai More articles by this author Diana Liu More articles by this author David August More articles by this author Chang A. Liu More articles by this author Expand All Advertisement PDF downloadLoading ...

PURPOSE:Ascending testes have been documented to be descended in the scrotum within the first year of life and then reascended. The aim of this study was to investigate to what extent the fertility potential was impaired in boys with such testes compared to the fertility potential of boys with late referral congenital cryptorchidism.MATERIALS AND METHODS:A total of 153 consecutive boys underwent bilateral orchiopexy at age 2 to 7 years (median 3.9) between 2011 and 2018. Of the patients 67 were diagnosed with bilateral ascended testes and 86 with late referral bilateral congenital cryptorchidism. We assessed serum levels of inhibin B and gonadotropins and histological parameters, number of germ cells per tubule cross-section and number of type A dark (Ad) spermatogonia per tubule cross-section. All values were compared to our normal material.RESULTS:Number of germ cells per tubule cross-section of boys with ascended testes (median 0.50, range 0 to 2.29) was not significantly higher compared to boys with congenital cryptorchidism (median 0.37, range 0 to 2.57; p=0.11). Mean number of germ cells per tubule cross-section was below normal range in 40 boys with ascending testes (60%) vs 57 boys with late referral congenital cryptorchidism (66%, p=0.40). Biopsies absent of Ad spermatogonia were noted in 31% of boys with ascending testes (21 of 67) vs 34% of boys with congenital cryptorchidism (29 of 86, p=0.76). Serum levels of inhibin B and gonadotropins did not differ between the 2 groups.CONCLUSIONS:The fertility potential of boys with bilateral ascended testes was impaired to almost the same level as that of boys with bilateral congenital cryptorchidism and should therefore be surgically corrected as soon as the diagnosis of ascended testes is settled.

Intermittent ureteropelvic junction obstruction, or Dietl crisis, is a rare entity with sparse reports in published literature. Establishing the diagnosis is challenging given its intermittent nature. We report a case of Dietl crisis, focusing on ultrasound (US) and magnetic resonance urography (MRU) findings in a 7-year-old boy with recurrent episodes of colicky abdominal pain prompting multiple visits to the emergency department. Severe left hydronephrosis was visualized on US during one episode with complete resolution on follow-up US. MRU demonstrated severe left hydronephrosis with delayed calyceal transit time, time-to-peak enhancement, and excretion. There was no aberrant blood vessel. Surgical pyeloplasty provided complete symptomatic resolution. MRU can be a valuable tool in eliciting and dynamically confirming the diagnosis of Dietl crisis.

Panel Thomas F. Kolon, MD (Chair), Children’s Hospital of Philadelphia Raymond and Ruth Perelman School of Medicine at the University of Pennsylvania Philadelphia, PA Peter A. Lee, MD, Penn State College of Medicine Hershey, PA Linda A. Baker, MD, Children’s Medical Center J. Pavilion Dallas, TX Julia S. Barthold, M.D., FAAP (Vice Chair), duPont Hospital for Children Wilmington, DE Laurence S. Baskin, MD, ULSF Children’s Hospital San Francisco, CA Cheryl G. Baxter, MSN, RN, CPNP Nationwide Children’s Hospital NAPNAP Columbus, Ohio C.D. Anthony Herndon, MD, FAAP,FACS University of Virginia Pediatric Urology Charlottesville, VA Earl Y. Cheng, MD Lurie Children’s Hospital of Chicago Chicago, IL Gregory E. Tasian, MD, MSc, MSCE Center for Pediatric Clinical Effectiveness Children’s Hospital of Philadelphia Philadelphia, PA Carl J. Seashore, MD, FAAP University of North Carolina Chapel Hill, NC

Vesicoureteral reflux (VUR) affects approximately 1% of children. VUR predisposes an individual to upper urinary tract infection (UTI) (that is, pyelonephritis). Repeated episodes of pyelonephritis can result in renal scarring (reflux nephropathy), hypertension, impaired somatic growth, renal insufficiency, end-stage renal disease, and complications during pregnancy. VUR can be managed medically or surgically.

Case diversity and degree of complexity vary among newly trained pediatric urologists. The urologist with the greatest case diversity never performed 45% of the 51 analyzed CPT codes, while the one with the least case diversity never performed 90% of the codes. Male surgeons performed more operations, particularly those of minimal and significant complexity. The variability in operative experience reinforces the importance of continuing education and mentorship after completion of fellowship.

Background: Robotic pyelolithotomy (RPL) and robotic nephrolithotomy (RNL) may be utilized for treating kidney stones as an alternative to percutaneous nephrolithotomy or flexible ureteroscopy. Objective: To describe the techniques of RPL and RNL, and present multi-center outcome data for patients undergoing these procedures. Design, setting, and participants: This study was a retrospective analysis of 27 patients undergoing RPL and RNL at five tertiary academic institutions between 2008 and 2014. Surgical procedure: RPL and RNL without use of renal ischemia. Measurements: We assessed stone clearance by visual assessment and postoperative imaging. We also examined other factors, including complications (Clavien grade), estimated blood loss, operative time, and length of stay. Results and limitations: Twenty-seven patients underwent 28 procedures for a mean renal stone size of 2.74 cm (standard deviation: 1.4, range: 0.8-5.8). The mean stone volume was 10.2 cm(3). RPL accounted for 26 of these procedures. RNL was performed in one patient, while another underwent combined RPL-RNL. Indications included failed previous endourological management (13), staghorn calculi (five), gas containing stone (one), calyceal diverticulum (one), complex urinary tract reconstruction (two), and patient preference (four). The mean patient age was 35.6 yr and mean body mass index was 25.5 kg/m(2). Mean operative time/console times were 182 min and 128 min, respectively. The mean estimated blood loss was 38 ml. The mean length of stay was 1.7 d. There was no significant change in preoperative and postoperative serum creatinine levels. The overall complication rate was 18.5% (Clavien 1 = 3.7%; 2 = 7.4%; 3b = 7.4%). The complete stone-free rate was 96%. Conclusions: RPL and RNL are safe and reasonable options for removing renal stones in select patients. In particular, RPL allows the removal of stones without transgressing the parenchyma, reducing potential bleeding and nephron loss. Patient summary: The robotic approach allows for complete removal of the renal stone without fragmentation, thereby maximizing chances for complete stone clearance in one procedure. Published by Elsevier B. V. on behalf of European Association of Urology.

Objective Transient urinary retention has been recognized as a complication of bilateral ureteroneocystostomy (UNC), when performed extravesically. The objective of this study was to review a collective surgeons’ experiences of unilateral extra- and unilateral and/or bilateral intra-vesical ureteral reimplanation, where urinary retention greater than 6 weeks, or what we have termed, “prolonged urinary retention” (PUR), occurred. Materials and methods We retrospectively reviewed charts to identify PUR after any open or robotic reimplant, other than bilateral extravesical, between 1998 and 2015 as reported by five surgeons. Results During the review period, ten cases were documented where PUR was encountered. Bilateral Cohen reimplants (5), unilateral extravesical open reimplant with ureteral tapering (3), unilateral Cohen reimplant (1) and unilateral extravesical robotic reimplant with tapering (1) were associated with PUR. Younger males predominated (70%). The mean age at operation of the patients was 3.1 years. Eventually 7/10 patients were able to void normally, with periods ranging from 6 weeks to 8 years. The remaining three patients are still unable to void more than 5 years after UNC. A majority of the samples (6/10) were suspected to have bowel and bladder dysfunction (BBD ) , but neurologically all were normal. Conclusion PUR can occur as a potential complication following any type of UNC and is associated with the risk of significant morbidity, including permanent urinary retention. Patients and caregivers should be counseled accordingly.

In 1997, the American Urological Association (AUA) published the Guideline on the Management of Primary Vesicoureteral Reflux in Children. Since that time there has been an expanding body of literature involving not only the evaluation and the management of vesicoureteral reflux (VUR) but also the role of screening in its management. For this reason, the AUA appointed a Panel of experts to update the 1997 document and elected to expand its scope to include guidelines for the screening of siblings of children with vesicoureteral reflux (VUR) and of neonates/infants with prenatally diagnosed hydronephrosis. A literature search, review of the evidence, and data extraction from the relevant clinical studies and case series were performed. Extracted data underwent meta-analysis to determine the outcomes related to five topics: 1) management of children over one year of age with VUR; 2) evaluation and management of infants with VUR; 3) management of children with VUR and Bladder and Bowel Dysfunction (BBD); 4) screening of siblings and offspring of patients with VUR; and 5) screening of neonates and infants with prenatal hydronephrosis. This document summarizes the guideline statements derived from a meta-analysis. Additional chapters (1-5) provide a detailed summary of each of these topics.

Undescended testis (UDT) is the most common disorder of sexual development in boys and affects 3.5% of male newborns. Although approximately half of newborn UDTs descend spontaneously, some boys develop an ascending testis later in childhood. Recent guideline recommendations advocate orchiopexy by 18 months of age to maximize potential for fertility and perhaps reduce the risk for testicular carcinoma in the future. For palpable testes, a standard inguinal approach is appropriate. However, the prescrotal approach is often effective for low inguinal testes and reduces surgical time and patient discomfort with an equivalent success rate in boys with an ascending testis. Some advocate monitoring until adolescence to determine whether the testis will spontaneously descend into the scrotum, but data do not support this approach. Instead, prompt orchiopexy is recommended. In boys with a nonpalpable testis, approximately 50% are abdominal or high in the inguinal canal and 50% are atrophic, typically in the scrotum. Routine inguinal/scrotal ultrasound is not recommended, although in an older boy who is overweight, it is appropriate. If the patient has contralateral testicular hypertrophy, scrotal exploration is appropriate, and removal of the testicular remnant and contralateral scrotal orchiopexy to prevent future contralateral testicular torsion is recommended. In most cases, diagnostic laparoscopy is advised to determine whether the testis is abdominal. For the abdominal testis, there are numerous treatment options. If the testis is mobile or a peeping testis just distal to the internal inguinal ring, standard one-stage laparoscopic or open orchiopexy should be attempted using the Prentiss maneuver. If the testicular vessels are short or the testis is not mobile, a two-stage Fowler-Stephens orchiopexy is appropriate. The second stage can be performed laparoscopically or open. Another option is microvascular testicular autotransplantation, which is a technically demanding procedure. Surgical results of abdominal orchiopexy are highly variable, short term, and highly subjective. Prospective clinical trials with follow-up into adolescence and adulthood are necessary to assess the success of various surgical approaches.

ObjectivesTo examine temporal trends in inpatient testicular torsion (TT) treatment and testicular loss (TL), and to identify risk factors for TL using a large nationally representative paediatric cohort, stratified to established high prevalence TT cohorts (neonatal TT [NTT]; age <1 years) and adolescent TT (ATT; age 12–17 years).MethodsBoys (age ≤17 years, n = 17 478) undergoing surgical exploration for TT were identified within the Nationwide Inpatient Sample (1998–2010). Temporal trends in inpatient TT management (salvage surgery vs orchiectomy) and TL were examined using estimated annual percent change methodology. Multivariable logistic regression models were used to identify risk factors for TL.ResultsTeaching hospitals treated 90% of boys with NTT, compared with 55% with ATT (P < 0.001). Of boys with NTT, 85% lost their testis, compared with 35% with ATT (P < 0.001). Inpatient management of NTT declined during the study period, from 7.5/100 000 children in 1998 to 3/100 000 in 2010 (estimated annual percent change −4.95%; P < 0.001). The decrease was similar but less dramatic in ATT. TL patterns did not improve. In adjusted analyses, for NTT, orchiectomy was more likely at teaching hospitals. For ATT, orchiectomy was more likely in children with comorbidities (odds ratio 5.42; P = 0.045), Medicaid coverage or self‐pay (P < 0.05) and weekday presentation (P = 0.001). Regional or racial disposition was not associated with TL.ConclusionsThere has been a gradual decrease in inpatient surgical treatment for both NTT and ATT, presumably as a result of increased outpatient and/or non‐operative management of these children. Concerningly, TL patterns have not improved; targeted interventions such as parental and adolescent male health education may lead to timely recognition/intervention in children at‐risk for ATT. We noted no regional/racial disparities in contrast to earlier studies.

BackgroundThe American Academy of Pediatrics (AAP) Task Force on Circumcision has called for the development of standards of trainee proficiency in regards to evaluation and technique for neonatal clamp circumcision (NCC). At the present time, there is no standardized or general consensus on patient selection for NCC. An improved method to evaluate newborns for NCC is an important first step in this process. Therefore, the authors collaborated to identify criteria useful in the evaluation of newborns for suitability for NCC, and for assessment of success after NCC and have named it "Checklist Assessment for Neonatal Clamp Circumcision Suitability."MethodsA national multi-institutional collaboration was created to obtain consensus on objective criteria for use in determining patient suitability for NCC, and for assessing post-circumcision success outcomes. Criteria included elements from detailed medical history, bedside physical examination, and post-circumcision follow-up. Patients desiring NCC were enrolled consecutively and prospectively. The Checklist was followed to determine which newborns were suited to NCC, and NCC was done in those cases. The patients' caretakers were given post-circumcision care instructions and a follow-up appointment. Post circumcision, the Checklist was followed to determine if the procedure resulted in a successful circumcision or if there were complications.ResultsA total of 193 cases were enrolled prospectively and consecutively from January 2014 through October 2014. The mean age was 15 days (1-30 days). Of those 193 patients, 129 (67%) were deemed suitable for circumcision and underwent NCC. Post-circumcision assessment showed a 100% success rate with no complications. A total of 64 (23%) cases were deemed unsuitable for NCC because at least one checklist criterion was not satisfied, most commonly: penile torsion (n = 25), chordee (n = 19), and penoscrotal webbing ( n = 19).DiscussionUse of the Checklist in the present study has demonstrated a method of patient screening resulting in a 100% success rate with no complications. A high proportion of patients (33%) was identified as unsuited for NCC; however, the patient population consisted of newborn males referred to pediatric urology, and thus does not represent the general population, which is expected to have a lower proportion of unsuited patients. Regardless, the Checklist has the potential to enhance the decision-making process for both urologic and non-urologic care providers.ConclusionsThe use of the "Checklist Assessment for Neonatal Clamp Circumcision Suitability" assessment tool improves identification of patients unsuited for NCC and thereby potentially decreases the likelihood of circumcision-related complications.

BackgroundIn the United States, both pediatric urologists (PUROs) and general pediatric surgeons (GPSs) perform nephrectomies in children, with PUROs performing more nephrectomies overall, most commonly for benign causes. GPSs perform more nephrectomies for malignant causes. We questioned whether the same trends persisted for partial nephrectomy.ObjectiveWe hypothesized that PUROs performed more partial nephrectomies for all causes, including malignancy. Our primary aim was to characterize the number of partial nephrectomies performed by PUROs and GPSs. We also compared short-term outcomes between subspecialties.Study designWe analyzed the Pediatric Health Information System (PHIS), a database encompassing data from 44 children's hospitals. Patients were <= 18 years old and had a partial nephrectomy (ICD-9 procedure code 554) carried out by PUROs or GPSs between 1 January, 2004 and June 30, 2013. Queried data points included surgeon subspecialty, age, gender, 3M (TM) All Patient Refined Diagnosis Related Groups (3M (TM) APR DRG) code, severity level, mortality risk, length of stay (LOS), and medical/surgical complication flags. Data points were compared in patients on whom PUROs and GPSs had operated. Statistical analysis included the Student t test, chi-square test, analysis of covariance, and logistic regression.ResultsResults are presented in the table. While PUROs performed the majority of partial nephrectomies, GPSs operated more commonly for malignancy. For surgeries performed for non-malignant indications, PURO patients had a shorter LOS and lower complication rate after controlling for statistically identified covariates. There was no difference in LOS or complication rate for patients with malignancy.DiscussionA Pediatric Health Information System study of pediatric nephrectomy demonstrated PUROs performed more nephrectomies overall, but GPSs performed more surgeries for malignancy. The difference was less dramatic for partial nephrectomies (63% GPS, 37% PURO) than for radical nephrectomies (90% GPS, 10% PURO). PUROs performed more partial nephrectomies for benign indications (94% PURO, 6% GPS) at an even greater rate than nephrectomies (88% PURO, 12% GPS). As a national database study, there are a number of inherent limitations: applicability of results to non-participating hospitals, possibility of inaccurate data entry/coding, and lack of data points that would be relevant to the study.ConclusionsWhile most partial nephrectomies in the United States are performed by PUROs, GPSs perform the majority of surgeries for malignancy. There is no difference in LOS or complication rate undergoing nephron-sparing surgery for malignant disease; however, PUROs had a shorter LOS and lower complication rate when operating for benign diseases.