Simultaneous second-stage Fowler-Stephens Orchiopexy and microvascular testicular autotransplantation in a patient with prune belly syndrome with cryptorchidism.

We present a case of simultaneous second-stage Fowler-Stephens Orchiopexy (FSO) with microvascular testicular autotransplantation for cryptorchidism and in a patient with prune belly syndrome. At 5 months old, the patient underwent laparoscopic bilateral first-stage FSO with the right testicle located 1 cm from the liver and the left slightly more caudal. An ultrasound on postoperative Day 72 following second-stage FSO and microvascular autotransplantation showed patent testicular vasculature. Our experience shows that this combination technique is safe and effective to supercharge the testicle and augment collateral vessels if clinical suspicion for monotherapy failure is high.

Intra-operative Urodynamics: Is the Test an Accurate Representation of the Lower Urinary Tract in Children?

OBJECTIVE: To compare intraoperative UDS results with UDS in the postoperative care unit (PACU) to assess the accuracy and efficacy of intraoperative UDS in children who cannot tolerate ambulatory urodynamic evaluation. METHODS: Pediatric patients undergoing intraoperative UDS at a single institution were enrolled over a 5-year time period (1/2013-8/2018). Urodynamics were performed in the operating room under general anesthesia, then in the PACU after recovery from anesthesia. Electromyographic (EMG) activity during filling, bladder compliance, cystometric bladder capacity (CBC), detrusor overactivity, presence of urinary leak, leak point pressure (LPP), and pressure specific volumes (PSV) at 10, 20, 30, and 40 cm water were compared between studies. RESULTS: Nineteen patients underwent urodynamic evaluation under general anesthesia and met inclusion criteria. Ten patients (52.6%) underwent 2 filling cycles while awake in PACU, resulting in a total of 48 urodynamic studies available for subsequent analysis. Intraoperative urodynamic studies were more likely to have decreased EMG activity during filling (P=<.01), normal compliance (P <.01), and a lower detrusor LPP (P = .03) compared to UDS performed after recovery from anesthesia. Detrusor overactivity was less frequently observed intraoperatively (P <.001) and involuntary detrusor contractions were lower in magnitude than those observed in the PACU. Twelve of the 19 (63%) children had detrusor overactivity that was present only on the UDS in PACU and not intra-operatively. CONCLUSIONS: The results of urodynamic testing performed under general anesthesia should be interpreted with caution, as pediatric patients appear to have improved bladder compliance, lower detrusor LPP and decreased detrusor overactivity when under anesthesia. For this reason, it is preferable to utilize ambulatory urodynamic evaluation to guide patient management and treatment.

Review of Outcomes of Low Verses Standard Pressure Pneumoperitoneum in Laparoscopic Surgery.

BACKGROUND: Carbon dioxide pneumoperitoneum during laparoscopy changes cardiorespiratory physiology and contributes to post-op pain. We studied outcomes before and after implementing low-pressure pneumoperitoneum QI project. METHODS: Forty-two patients were insufflated at standard pressures (15 mmHg) while 41 were insufflated using low (8-12 mmHg) during laparoscopic procedures. These variables were obtained from the patient chart: pain scores, intravenous morphine milligram equivalents (MME), peak inspiratory pressures (PIP), end-tidal CO2 (EtCO2), surgery duration, and patient demographics. The study was conducted after IRB approval. RESULTS: Low-pressure pneumoperitoneum is feasible and the surgeon can increase to 10-12 mmHg as needed. The mean post-op IV MME was significantly decreased in the low-pressure group (11.75 ± 10.41) compared to the standard pressure group (17.36 ± 18.1) (t-test, P = .047). Mean peak inspiratory pressures during insufflation were significantly higher for procedures conducted at standard pressure (31.40 ± 4.82) compared to the 8 mmHg (24.68 ± 4.19) and 12 mmHg (27.33± 3.85) low pressure groups (one-way ANOVA, P < .0001). During insufflation, there was a significant increase in the average EtCO2 in the standard pressure group (42.07 ± 5.60) compared to the 8 mmHg low pressure group (37.59 ± 5.05) (ANOVA, P = .0096). Constant flow insufflation was more likely to be performed at low pressure than demand mode (58% v. 33%). CONCLUSION: Low pressure pneumoperitoneum decreases PIP pressure and CO2 absorption evidenced by lower ETCO2 intra-operatively. Patients have significant improvement in postoperative pain evidenced by decreased narcotics needed. Low pressure pneumoperitoneum using a constant flow insufflator is safe and results in improved patient outcomes.

Clinical outcomes of COVID-19 in patients with sickle cell disease and sickle cell trait: A critical appraisal of the literature.

Individuals with sickle cell disease (SCD) and sickle cell trait (SCT) have many risk factors that could make them more susceptible to COVID-19 critical illness and death compared to the general population. With a growing body of literature in this field, a comprehensive review is needed. We reviewed 71 COVID-19-related studies conducted in 15 countries and published between January 1, 2020, and October 15, 2021, including a combined total of over 2000 patients with SCD and nearly 2000 patients with SCT. Adults with SCD typically have a mild to moderate COVID-19 disease course, but also a 2- to 7-fold increased risk of COVID-19-related hospitalization and a 1.2-fold increased risk of COVID-19-related death as compared to adults without SCD, but not compared to controls with similar comorbidities and end-organ damage. There is some evidence that persons with SCT have increased risk of COVID-19-related hospitalization and death although more studies with risk-stratification and properly matched controls are needed to confirm these findings. While the literature suggests that most children with SCD and COVID-19 have mild disease and low risk of death, some children with SCD, especially those with SCD-related comorbidities, are more likely to be hospitalized and require escalated care than children without SCD. However, children with SCD are less likely to experience COVID-19-related severe illness and death compared to adults with or without SCD. SCD-directed therapies such as transfusion and hydroxyurea may be associated with better COVID-19 outcomes, but prospective studies are needed for confirmation. While some studies have reported favorable short-term outcomes for COVID-19 patients with SCD and SCT, the long-term effects of SARS-CoV-2 infection are unknown and may affect individuals with SCD and SCT differently from the general population. Important focus areas for future research should include multi-center studies with larger sample sizes, assessment of hemoglobin genotype and SCD-modifying therapies on COVID-19 outcomes, inclusion of case-matched controls that account for the unique sample characteristics of SCD and SCT populations, and longitudinal assessment of post-COVID-19 symptoms.

Elements Selection for Transcostal HIFU Refocusing Method: Simulation Study.

Treatment of tumors in organs obscured by the ribs is a challenge for high-intensity focused ultrasound (HIFU) array. The ribs absorb the ultrasound beam's energy causing the temperature of the ribs to rise, the beam to be distorted, and the focal heat deposition to be limited. The challenges presented by the ribs motivated the development of the limited power deposition (LPD) refocusing algorithm, which is capable of limiting the power deposition over the ribs while maximizing the energy deposition at the focus, thus achieving an efficient and safe ablation. In this article, a new iterative sparse LPD (ISLPD) approach that provides similar focal heating to previously developed methods while reducing the number of transducers excited during the HIFU treatment is discussed. It will be possible to conduct processes, such as imaging and motion tracking in parallel with the HIFU treatment by using the elements no longer used by the refocusing technique. The approach removes transducer elements by using an iteratively reweighted penalty matrix to achieve a more sparse solution over the course of multiple rounds of sparsity induction by harshly punishing the use of elements that previously had low excitation magnitudes. The semidefinite relaxation (SDR) method is used as a means of relaxing the nonconvex constraints into convex form and induces sparsity using the one-norm squared as a convex surrogate for the zero-norm. A 1-MHz spherical phased-array focused on a target in an inhomogeneous medium is simulated to test the algorithms' efficacy. The propagation of the waves as they travel from the array toward the target was modeled using a finite-difference time-domain propagation model. Temperature simulations that utilized the inhomogeneous bioheat transfer equation (BHTE) were used to determine the temperature rise profile within the region of interest (ROI). These simulation results illustrate the benefits of the optimization-based approach proposed in this article over the ray-tracing (shadowing) method for element selection.

Chemoradiotherapy-induced Cytodifferentiation in Bladder/Prostate Rhabdomyosarcoma With Genetic Downregulation of Myogenin and MyoD1 Gene Expression: A Case Study and Review of the Literature.

Rhabdomyosarcoma is the most common sarcoma diagnosed in childhood and adolescence, arising from the bladder/prostate in only 5%-10% of cases. Treatment-induced cytodifferention of tumor cells into mature rhabdomyoblasts has been reported following chemoradiation and is thought to suggest a more favorable outcome. We report a case of embryonal rhabdomyosarcoma of the bladder/prostate that exhibited extensive cytodifferentiation with downregulation of myogenin and MyoD1 gene expression in rhabdomyoblasts following treatment with chemoradiation therapy. The downregulation of myogenin and MyoD1 expression in rhabdomyoblasts following chemoradiation treatment has not previously been described in the literature and its significant remains uncertain.

Intermediate Follow-up and Management of Previously Reported Malignant Peripheral Nerve Sheath Tumor of the Penis.

Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerve fibers and primarily occur in the setting of neurofibromatosis (NF1). MPNST arising from the penis is very rare and may require mutilating surgery to achieve surgical cure. We previously reported a case of MPNST involving the penis in a 14-month-old boy treated with neoadjuvant chemotherapy, total penectomy, and adjuvant radiation. Here we report intermediate follow-up of the same patient, describe his subsequent genitourinary reconstruction, and discuss management dilemmas that arise following treatment of penile MPNST.

Optimization of transcostal phased-array refocusing using sparse semidefinite relaxation method.

Treating tumors in organs shadowed by the ribs is a challenge for high intensity focused ultrasound (HIFU). The ribs absorb energy from the ultrasound beams causing their temperature to rise, while also distorting the beams, and limiting the target heat deposition. Accordingly, it is necessary to devise focusing methods that address the difficulties presented by the ribs. In this paper, a new approach that reduces total power deposition on the region of interest (ROI) by removing transducer elements is introduced. The method builds on the limited power deposition (LPD) method, which took advantage of the semidefinite relaxation (SDR) method to relax nonconvex constraints into convex form. The method introduced in this paper induces sparsity using the one-norm squared. The results of using this method to focus a 1-MHz spherical phased array on a target in an inhomogeneous medium are presented and compared to the ray tracing (shadowing) approach [1]. A finite-difference time domain propagation model was used to model the wave propagation to the target. Temperature simulations that utilized the inhomogeneous bioheat transfer equation (BHTE) illustrate the advantages of the induced sparsity LPD method. Together, these simulation results illustrate the advantages of using optimization based on sparsity inducing techniques over the shadowing approach.

Anterior urethral valve associated with posterior urethral valves: report of 2 cases and review of the literature.

Anterior urethral valve (AUV) associated with posterior urethral valves (PUVs) is an extremely rare congenital urologic anomaly resulting in lower urinary tract obstruction. We present our experience with 2 children with concomitant AUV and PUV as well as a literature review. The clinical presentation of concomitant AUV and PUV is variable. Successful endoscopic management can result in improvement in renal function, reversal of obstructive changes, and improvement or resolution of voiding dysfunction.

Malignant peripheral nerve sheath tumor of the penis: a case report and review of the literature.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas that arise from peripheral nerve fibers and are derived from Schwann cells, perineural cells, or fibroblasts. MPNST is an aggressive neoplasm in which local recurrence is common and complete excision of the mass should be the goal of surgery. We report a case of MPNST involving the penis in a 14-month-old boy. This is only the second reported case of penile MPNST without evidence of neurofibromatosis 1 and the first of which to occur in a patient this young.

An institutional review of splenic artery aneurysm in childbearing-aged females and splenic artery aneurysm rupture during pregnancy. Is screening justified?

OBJECTIVE: To determine the prevalence of splenic artery aneurysm (SAA) in women of childbearing age and the incidence of peripartum rupture to ascertain a possible benefit of screening this potentially high-risk population. METHODS: Patients diagnosed with SAA over a 6-year period were collected from a single institutional medical records database. Inclusion criteria included female gender and age between 15 and 49 years. The number of abdominal computed tomography studies performed on our study population during the study period was compared to the number of detected SAAs. The number of deliveries at our institution during the study period was compared to the number of peripartum SAA ruptures. The resultant data were used to calculate the prevalence of SAA in childbearing-aged females and the incidence of SAA rupture during pregnancy. RESULTS: The prevalence of SAA in childbearing-aged females and incidence of rupture during pregnancy were less than 0.1%. CONCLUSION: Radiologic screening of all childbearing-aged females is not warranted, but identification of those at greater risk of harboring an asymptomatic SAA, along with the early institution of treatment according to current guidelines, may prevent maternal and fetal mortality in the rare event of SAA rupture during pregnancy.

Technique for preservation of penile skin in genital reconstruction: free graft to the scrotum.

OBJECTIVE: To describe a new technique to preserve the penile epithelium with good cosmetic results when additional surgery is a significant possibility. Complex genital reconstruction can require multiple procedures. METHODS: Seven patients underwent grafting of the penile epithelium to the scrotum during genital reconstruction to bank the tissue for potential future use. The graft size was 8-40 mm in the maximal length. RESULTS: The median age at surgery was 8.1 months. The median interval to the last follow-up examination after surgery was 13.5 months. All 7 patients demonstrated excellent wound healing with good cosmetic results. CONCLUSION: In patients with complex genital anomalies, nonhair-bearing epithelium is a valuable asset. At times, the paucity of available local tissue necessitates autografting with nongenital epithelium. The need for nongenital tissue can be reduced if excess penile skin is banked for potential use at a later date. We describe a technique in which the penile skin is preserved for potential future reconstruction using free grafting to the scrotum.

Pediatric urolithiasis: clinical predictors in the emergency department.

OBJECTIVE: The objective of this study was to identify factors that predict the presence of urolithiasis detected with unenhanced computed tomography (UCT) in children. METHODS: A retrospective study of all subjects <21 years of age who presented to the emergency department at Akron Children's Hospital and underwent UCT of the abdomen between January 2002 and December 2005 was performed. Demographic, clinical, diagnostic, treatment, and disposition data were abstracted by using a standardized form. Univariate and logistic regression analyses of factors associated with urolithiasis were performed. RESULTS: A total of 339 eligible patients were identified, with 110 cases of urolithiasis detected with UCT for 95 individual patients. The mean age of the study patients was 14.4 years; 72 patients (66%) were female. In 17 cases (15%) of urolithiasis, initial urinalysis results were negative for blood. Fifty-seven stones (51.8%) were ureteral, 26 (23.6%) were renal, and 4 (3.6%) were in the bladder. Among children who did not have a stone identified through UCT, 23 cases (10%) of potentially significant, alternative diagnoses were identified. A history of urolithiasis, a history of nausea and vomiting, the presence of flank pain on examination, and >2 red blood cells per high-power field in urine microscopy were positively associated with urolithiasis. A history of fever or dysuria and costovertebral angle tenderness on physical examination were inversely associated with urolithiasis on UCT scans. CONCLUSIONS: UCT plays an important role in the diagnostic evaluation of children with flank pain. Approximately 15% of children with urolithiasis do not have hematuria.

Role of positional instillation of contrast cystography in the algorithm for evaluating children with confirmed pyelonephritis.

OBJECTIVES: To assess the utility of positional instillation of contrast (PIC) cystography in detecting vesicoureteral reflux (VUR) in patients with renal scarring from recurrent febrile urinary tract infections that standard voiding cystourethrography and nuclear cystogram imaging failed to reveal. METHODS: Between June 2004 and November 2004, a total of 5 pediatric patients with recurrent febrile urinary tract infections and radiologic evidence of upper tract involvement were examined with PIC cystography. All patients had at least one previous negative standard reflux study (voiding cystourethrography or nuclear cystography). RESULTS: All 5 patients showed VUR on PIC cystography. Unilateral reflux was detected in 3 patients, and 2 patients had bilateral VUR. CONCLUSIONS: The PIC cystogram should be integrated into the algorithm for diagnosing patients with recurrent febrile urinary tract infection, who do not exhibit VUR on standard imaging modalities. The morbidity associated with undiagnosed VUR, as demonstrated by this group of patients, suggests that permanent renal damage may be prevented by early diagnosis and treatment.

Automated bladder scan urine volumes are not reliable in complex neonatal cases.

PURPOSE: We investigate the accuracy of urine volumes obtained by an automated bladder scan in complex neonatal cases. MATERIALS AND METHODS: Automated bladder scan determinations of urine volumes were obtained by neonatal intensive care unit nursing staff in 10 patients with myelodysplasia and cloacal exstrophy. Urine volumes were then immediately obtained by straight catheterization. Correlation between the scan and catheter volumes was then evaluated across and within cases. RESULTS: There was low correlation between automated bladder scan volume and catheter volume across and within cases (0.037 +/- 0.37) and (0.188 +/- 0.12), respectively. Using a cutoff of 20 cc 25% of significant volumes were missed. The 95% confidence interval from these data indicates that a significant volume is missed 7% to 25% of the time. CONCLUSIONS: We urge clinicians to exercise caution in the use of automated bladder scanners for determination of urine volumes in complex neonatal intensive care unit cases.

Spermatic cord torsion with preserved testis perfusion: initial anatomical observations.

PURPOSE: Despite the high sensitivity and specificity of scrotal ultrasonography, there continue to be reports of missed torsion. These "false-negative" scans are attributed to technical factors and intermittent torsions. We hypothesize that patients with specific anatomical configurations maintain testicular blood flow for prolonged periods, and, therefore, will have flow on ultrasound despite concurrent torsion. MATERIALS AND METHODS: Patient charts and scrotal ultrasounds were reviewed to identify patients younger than 18 years who underwent scrotal exploration between January 1998 and January 2003 for acute scrotum. Patients who underwent radiological evaluation before scrotal exploration were the main focus of this study. Operative reports were reviewed for specific anatomical details. RESULTS: A total of 61 patients underwent scrotal exploration for acute scrotum during the study period. Of these patients 14 had torsion confirmed intraoperatively and a preoperative scrotal ultrasound available. Four of these 14 patients had normal testicular blood flow on ultrasound but had testicular torsion confirmed intraoperatively. Operative findings suggest that these patients have specific anatomical characteristics. CONCLUSIONS: Testis perfusion can be maintained for a prolonged period in the presence of testicular torsion. Anatomical variability may account for differences in the duration of viability of the torsed testis. A high index of suspicion must be maintained to avoid missing the diagnosis of testicular torsion in these challenging cases.