OBJECTIVE: We present a case series of Right Ventricle (RV) rehabilitation after Starnes in patients with Ebstein anomaly, applying the Cone repair of the tricuspid valve (TV) to achieve biventricular or 1.5-ventricle physiology.
METHODS: This is a retrospective database analysis from two institutions in North America. We included all consecutive cases of Cone repair after the Starnes procedure. The data are expressed as median and interquartile range (IQR).
RESULTS: Eleven patients underwent RV rehabilitation between 2019 and 2023 after initial Starnes palliation with a median age of 27 months (IQR 20.5). All were critically ill before their Starnes procedure; four were on extracorporeal membrane oxygenation. Before the Cone repair, the median pre-operative regurgitant velocity at the Starnes patch was 1.65 m/s (IQR 1.3). During the Cone procedure, nine patients required a concomitant PV repair, of which three required a transannular monocusp patch. Four patients were successfully rerouted to a two-ventricle repair, and seven patients with a previous Glenn achieved 1.5 ventricle circulation. There was no heart block and no deaths. Seven patients had trivial regurgitation in a median follow-up of eleven months (IQR 21.5), three mild and one moderate tricuspid regurgitation. There was no significant TV stenosis; all patients had good functional status at the last follow-up despite severe RV dysfunction in one patient.
CONCLUSIONS: After the Starnes procedure, the Cone repair allowed RV rehabilitation, resulting in trivial or mild TV regurgitation sustained in midterm follow-up. The Starnes procedure is a reproducible technique that no longer commits patients to lifetime single ventricle physiology.

UNASSIGNED: Anterior cervical discectomy and fusion (ACDF) is a common cervical procedure with more than 137,000 cases in the United States (U.S.) each year. Historically, ACDF has been a relatively safe procedure despite encountering vital anatomical structures that can risk serious complications. One particularly dangerous sequela of ACDF is the risk of lacerating the vertebral artery (VA). While VA injuries are rare (0.5% of cases), it is crucial for surgeons to prevent this potentially deadly complication with thorough knowledge of VA anatomy. The VA is commonly protected within the transverse foreman; however, anomalies can exist with the artery potentially being found more medial or proximal within the surgical site than expected. The purpose of this article is to report an anomaly found in a cadaveric specimen, where the VA courses within 2 mm of an ACDF plate.
UNASSIGNED: A 66-year-old male with a past medical history of coronary artery disease passed away due to complications of a myocardial infarction. During cadaveric dissection, it was discovered that the donor had undergone a previous 3-level (C4-C7) ACDF procedure for an unknown reason. Under further examination, the left VA was observed to take an anomalous medially tracking course before eventually entering the transverse foramina of C5. Left anterior tubercles were absent at the level of C5 and C6 with prominent osteophytes found on the anterior tubercles/transverse foramina of the contralateral side.
UNASSIGNED: There are no studies published on the anatomy of VA variants and their implications on ACDF procedures. ACDF was found to have complication rates in 13.2% of cases. VA variants are observed in up to 20% of the U.S. population and VA injury in ACDF procedures has a rate near 0.5%. With anatomic variations leading to a higher risk of injury, surgeons are required to take further precautionary steps before operating including ordering computed tomography angiography or magnetic resonance angiography films. Understanding the detailed anatomy of the VA and its variants is critical. Using the perspectives of orthopedic spine surgeons, this study supplements the literature on potential VA anomalies encountered in ACDF procedures.

UNASSIGNED: To help radiologists examine the growing number of computed tomography (CT) scans, automatic anomaly detection is an ongoing focus of medical imaging research. Radiologists must analyze a CT scan by searching for any deviation from normal healthy anatomy. We propose an approach to detecting abnormalities in axial 2D CT slice images of the brain. Although much research has been done on detecting abnormalities in magnetic resonance images of the brain, there is little work on CT scans, where abnormalities are more difficult to detect due to the low image contrast that must be represented by the model used.
UNASSIGNED: We use a generative adversarial network (GAN) to learn normal brain anatomy in the first step and compare two approaches to image reconstruction: training an encoder in the second step and using iterative optimization during inference. Then, we analyze the differences from the original scan to detect and localize anomalies in the brain.
UNASSIGNED: Our approach can reconstruct healthy anatomy with good image contrast for brain CT scans. We obtain median Dice scores of 0.71 on our hemorrhage test data and 0.43 on our test set with additional tumor images from publicly available data sources. We also compare our models to a state-of-the-art autoencoder and a diffusion model and obtain qualitatively more accurate reconstructions.
UNASSIGNED: Without defining anomalies during training, a GAN-based network was used to learn healthy anatomy for brain CT scans. Notably, our approach is not limited to the localization of hemorrhages and tumors and could thus be used to detect structural anatomical changes and other lesions.

Background In this study, we aim to report our single-center experience with laparoscopic pediatric urological surgeries. We aim to determine the feasibility of various urological and urogenital laparoscopic procedures and the tricks that increase surgical success. Methodology Data from 98 patients who underwent laparoscopic urological and/or urogenital procedures for diagnostic and therapeutic purposes in our clinic between June 2018 and February 2023 were retrospectively analyzed. All surgeries were performed by the same surgical team. Laparoscopic procedures included orchidopexy, gonadectomy, vaginoplasty, hysterectomy, pyeloplasty, nephrectomy/partial nephrectomy, ureteroneocystostomy, bladder diverticulum excision, renal cyst excision, proximal ureter stone removal, oophorectomy, ovarian detorsion, oophoropexy, and lymph node excision for diagnostic purposes. The surgical planning of the patients was based on the decisions of the pediatric nephrology, pediatric endocrinology, and pediatric oncology departments and the multidisciplinary council. Demographic characteristics of the patients, surgical indications, and intraoperative data, as well as postoperative pathological diagnoses and complications, were recorded. All patients underwent a transperitoneal approach. The duration of the operation was obtained from anesthesia records and defined as the time from the beginning of the surgical incision to the closure of the skin incision. Results Of the patients, 54 were males and 44 were females. The median age was 7.8 years. No complications other than grade 1 according to the Clavien-Dindo classification were observed in our patients. As different types of surgeries were analyzed, the mean operative duration was estimated. Conclusions The laparoscopic method should be performed by surgeons experienced in advanced surgeries in pediatric urology. It is critical to consider the difference in the size of pediatric patients in preparation for laparoscopic surgery to minimize technical and ergonomic problems. We believe that each surgery has its specific tricks and that these should be a part of laparoscopy training. Moreover, developing and sharing this information would be very useful for pediatric urologists.

BACKGROUND: Crossed fused renal ectopia (CFRE) is a common congenital anomaly where one kidney is positioned abnormally on the opposite side of the midline, often fused with the other kidney. However, single ureter draining crossed fused renal ectopia is a rare occurrence.
METHODS: Here, we report a case of crossed fused renal ectopia with a single ureter in a 46-year-old Nepali male who presented with history of lithuria. Computed tomography revealed that the left kidney was situated on the right side and fused with the right kidney. The renal pelvises of both kidneys were fused, and a single ureter, located on the right side, was draining both kidneys into the bladder. The patient was advised to have regular follow-ups.
CONCLUSIONS: Crossed fused renal ectopia with a single ureter represents a rare renal anomaly. Asymptomatic patients can typically be managed conservatively. Regular follow-up is recommended to monitor renal function, calculus formation, infections, and malignant changes.

BACKGROUND: Testicular descent is a physiological process regulated by many factors. Eventually, disturbances in the embryological/fetal development path facilitate the occurrence of scrotal hernia, a congenital malformation characterized by the presence of intestinal portions within the scrotal sac due to the abnormal expansion of the inguinal ring. In pigs, some genes have been related to this anomaly, but the genetic mechanisms involved remain unclear. This study aimed to investigate the expression profile of a set of genes potentially involved with the manifestation of scrotal hernia in the inguinal ring tissue.
RESULTS: Tissue samples from the inguinal ring/canal of normal and scrotal hernia-affected male pigs with approximately 30 days of age were used. Relative expression analysis was performed using qPCR to confirm the expression profile of 17 candidate genes previously identified in an RNA-Seq study. Among them, the Myosin heavy chain 1 (MYH1), Desmin (DES), and Troponin 1 (TNNI1) genes were differentially expressed between groups and had reduced levels of expression in the affected animals. These genes encode proteins involved in the formation of muscle tissue, which seems to be important for increasing the resistance of the inguinal ring to the abdominal pressure, which is essential to avoid the occurrence of scrotal hernia.
CONCLUSIONS: The downregulation of muscular candidate genes in the inguinal tissue clarifies the genetic mechanisms involved with this anomaly in its primary site, providing useful information for developing strategies to control this malformation in pigs and other mammals.

OBJECTIVE: We investigated the postoperative renal function in persistent cloaca (PC) patients who underwent posterior sagittal anorecto-urethro-vaginopalsty (PSARUVP) and factors influencing the renal functional outcomes.
METHODS: A questionnaire survey was distributed to 244 university and children\'s hospitals across Japan. Of the 169 patients underwent PSARUVP, 103 patients were enrolled in the present study. Exclusion criteria was patients without data of renal prognosis.
RESULTS: The present study showed that renal anomalies (p = 0.09), vesicoureteral reflux (VUR) (p = 0.01), and hydrocolpos (p = 0.07) were potential factors influencing a decline in the renal function. Approximately half of the patients had a normal kidney function, but 45.6% had a reduced renal function (Stage ≥ 2 chronic kidney disease: CKD). The incidence of VUR was significantly higher in the renal function decline (RFD) group than those in the preservation (RFP) group (p = 0.01). Vesicostomy was significantly more frequent in the RFD group than in the RFP group (p = 0.04). Urinary tract infections (p < 0.01) and bladder dysfunction (p = 0.04) were significantly more common in patients with VUR than in patients without VUR. There was no association between the VUR status and the bowel function.
CONCLUSIONS: Prompt assessment and treatment of VUR along with bladder management may minimize the decline in the renal function.

A patient was delivered at 26 weeks (about 6 months) gestation via an emergency caesarian section. A patent ductus arteriosus (PDA) and atrial septal defect (ASD) were discovered during an echocardiogram 13 days after birth. The patient had catheter-based closure of the PDA and ASD. During a routine echocardiogram to check device placements, it was discovered that there was dilation of the superior vena cava (SVC), and it was suspected that a thrombus was present. Computed tomography angiography (CTA) was completed to better define SVC anatomy and flow acceleration. The CTA demonstrated that there was a double innominate vein.

OBJECTIVE: To examine the clinical characteristics and auditory performance of patients with CHARGE syndrome following cochlear implantation (CI), as well as the prognostic factors affecting auditory outcomes.
METHODS: Retrospective cohort.
METHODS: Tertiary academic center.
METHODS: A retrospective chart review was performed in patients with CHARGE syndrome who underwent CI from 2007 to 2022. The category of auditory performance (CAP) score was used to assess the CI outcomes, and factors that may affect the speech outcomes were also evaluated.
RESULTS: In 14 children with CHARGE syndrome, 22 CIs were performed, 6 unilaterally and 8 bilaterally. The mean age at CI was 25.9 months (range: 10-62). All patients had ear abnormalities and developmental delays, and cochlear nerve deficiency (CND) was present in all ears. At the last follow-up (mean: 49.6 months), the mean CAP score improved significantly compared to the preoperative measure (from 0.36 ± 0.81 to 3.21 ± 1.70, P = .001), with 6 patients (42.9%) achieving a CAP score of 4 points or higher. However, between the unilateral and bilateral CI groups, the final CAP score or change in CAP score was similar. Factors including age, coloboma, and CND did not significantly affect speech outcomes (all P > .05).
CONCLUSIONS: Even though CHARGE syndrome features challenging anomalies, CI can be conducted safely and can offer effective contribution to significant speech improvement. Patients with CHARGE syndrome should be given the opportunity to undergo CI to maximize their audiological progress.

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