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Purpose: This paper describes a novel circuit for intraoperative analysis with ICBCT in maxillofacial surgery. The aim is to establish guidelines, define indications, and conduct an analysis of the implementation of the circuit for intraoperative comparison of surgical outcomes in relation to 3D virtual planning in maxillofacial surgery., Methods: The study included 150 maxillofacial surgical procedures. Intraoperative actions involved fluoroscopy localization, intraoperative CBCT acquisition, segmentation, and superimposition, among other steps. Surgical times due to intraoperative superposition were measured, including time required for ICBCT positioning and acquisition, image segmentation, and comparison of 3D surfaces from the surgical planning., Results: Successful intraoperative comparison was achieved in all 150 cases, enabling surgeons to detect and address modifications before concluding the surgery. Out of the total, 26 patients (17.33%) required intraoperative revisions, with 11 cases (7.33%) needing major surgical revisions. On average, the additional surgical time with this circuit implementation was 10.66 ± 3.03 min (n = 22)., Conclusion: The results of our research demonstrate the potential for performing intraoperative surgical revision, allowing for immediate evaluation, enhancing surgical outcomes, and reducing the need for re-interventions., Competing Interests: Declarations. Conflict of interest: Authors declare no conflict of interest. Ethical approval: This study was approved by the Ethics Committee and conducted in compliance with the Helsinki Declaration (Fortaleza, Brazil, October 2013). The informed consent was requested when necessary., (© 2024. CARS.)

Introduction: High-risk neuroblastoma, particularly in the relapse/refractory (R/R) setting, poses unique challenges to obtaining the representative-quality tissue that is mostly required for molecular analysis. This study explores the use of 123I-MIBG radioguided surgery to access complex locations of MIBG-positive neuroblastoma as a tool to overcome the difficulties associated with repeated surgeries in these patients., Methods: This study is a retrospective review of all patients with R/R neuroblastoma and MIBG-uptaking lesions who underwent radioguided surgery between February 2020 and 2023 at SJD Barcelona Children's Hospital. The Europrobe 3.2 gamma probe was used to identify neuroblastoma tissue in the operating room., Results: Ten patients were identified. Radioguided surgery was useful in all patients. One patient with previous multiple operations developed an entero-cutaneous fistula with posterior full recovery. Mean surgical time was 111.7 min. The gamma probe identified 100% of neuroblastoma lesions which were all completely removed (123I-MIBG-SPECT/CT negative post-surgery). Pathology and molecular studies could be successfully performed in all samples., Conclusions: 123I-MIBG radioguided surgery proved effective in obtaining viable tissue from difficult-to-access sites in high-risk relapsed neuroblastoma.

Surgery is a crucial component of pediatric cancer treatment, but conventional methods may lack precision. Image-guided surgery, including fluorescent and radioguided techniques, offers promise for enhancing tumor localization and facilitating precise resection. Intraoperative molecular imaging utilizes agents like indocyanine green to direct surgeons to occult deposits of tumor and to delineate tumor margins. Next-generation agents target tumors directly to improve specificity. Radioguided surgery, employing tracers like metaiodobenzylguanidine (MIBG), complements fluorescent techniques by allowing for detection of tumors at a greater depth. Dual-labeled agents combining both modalities are under development. Three-dimensional modeling and virtual/augmented reality aid in preoperative planning and intraoperative guidance. The above techniques show great promise to benefit patients with pediatric tumors, and their continued development will almost certainly improve surgical outcomes., (© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Introduction: Pediatric oncology patients use totally implantable venous access devices (TIVADs) to enable central venous access. Anxiety, pain and/or discomfort are common despite anesthesia. Virtual reality (VR) is a non-pharmacological approach that may reduce pain and anxiety in these patients. We aimed to assess the use of VR for reducing anxiety/pain in patients with TIVADs while facilitating the task of healthcare providers when accessing a TIVAD., Methods: patients 4-18 years old with a TIVAD were prospectively randomized to an intervention group (IG) or a control group (CG). In the IG, VR goggles (Oculus Quest 2, Meta Platforms ® , Menlo Park, CA, USA) were used displaying a relaxing video in the Raja Ampat environment (Ecosphere app from Phoria ® ) while the TIVAD was accessed. The CG was managed as per standard of care. Satisfaction and pain were measured by FPS-R and STAIC scales, respectively., Results: this is the report of a prospective, randomized (60 per group)-unblinded-, single institution study of 120 pediatric oncology patients enrolled from January to April 2022. Median ages for IG and CG were 9.22 and 10.52 years, respectively. Satisfaction was higher in the IG (4.80) compared to the CG (3.92), p ≤ 0.0001. Regarding pain, mean FPS-R scores were 1.79 for the CG and 0.83 for the IG. Significantly different scores were found in the 12 to 18 years group, p ≤ 0.05. The healthcare professionals index of satisfaction was high (4.50 mean Likert score) for the IG compared to accessing the TIVAD without VR (3.73 mean Likert score)., Conclusion: The use of VR helped reduce pain and/or discomfort in pediatric oncology patients, mainly in the older age group as they can better interact with VR. Healthcare providers were satisfied with the help of VR for TIVAD management.

Cancer poses significant emotional challenges for children and adolescents, despite improvements in survival rates due to new therapies. However, there is growing concern about the long-term effects, including fertility issues. This review examines recent advancements and future directions in fertility preservation within a pediatric population subjected to oncological therapies. Worldwide, there is variability in the availability of fertility preservation methods, influenced by factors like development status and governmental support. The decision to pursue preservation depends on the risk of gonadotoxicity, alongside factors such as diagnosis, treatment, clinical status, and prognosis. Currently, options for preserving fertility in prepubertal boys are limited compared to girls, who increasingly have access to ovarian tissue preservation. Adolescents and adults have more options available, but ethical considerations remain complex and diverse.

Neuroblastoma presents with two patterns of disease: locoregional or systemic. The poor prognostic risk factors of locoregional neuroblastoma (LR-NB) include age, MYCN or MDM2-CDK4 amplification, 11q, histology, diploidy with ALK or TERT mutations, and ATRX aberrations. Anti-GD2 immunotherapy has significantly improved the outcome of high-risk (HR) NB and is mostly effective against osteomedullary minimal residual disease (MRD), but less so against soft tissue disease. The question is whether adding anti-GD2 monoclonal antibodies (mAbs) benefits patients with HR-NB compounded by only soft tissue. We reviewed 31 patients treated at SJD for HR-NB with no osteomedullary involvement at diagnosis. All tumors had molecular genetic features of HR-NB. The outcome after first-line treatment showed 25 (80.6%) patients achieving CR. Thirteen patients remain in continued CR, median follow-up 3.9 years. We analyzed whether adding anti-GD2 immunotherapy to first-line treatment had any prognostic significance. The EFS analysis using Cox models showed a HR of 0.20, p = 0.0054, and an 80% decrease in the risk of relapse in patients treated with anti-GD2 immunotherapy in the first line. Neither EFS nor OS were significantly different by CR status after first-line treatment. In conclusion, adding treatment with anti-GD2 mAbs at the stage of MRD helps prevent relapse that unequivocally portends poor survival.

The printing and manufacturing of anatomical 3D models has gained popularity in complex surgical cases for surgical planning, simulation and training, the evaluation of anatomical relations, medical device testing and patient-professional communication. 3D models provide the haptic feedback that Virtual or Augmented Reality (VR/AR) cannot provide. However, there are many technologies and strategies for the production of 3D models. Therefore, the aim of the present study is to show and compare eight different strategies for the manufacture of surgical planning and training prototypes. The eight strategies for creating complex abdominal oncological anatomical models, based on eight common pediatric oncological cases, were developed using four common technologies (stereolithography (SLA), selectie laser sinterning (SLS), fused filament fabrication (FFF) and material jetting (MJ)) along with indirect and hybrid 3D printing methods. Nine materials were selected for their properties, with the final models assessed for application suitability, production time, viscoelastic mechanical properties (shore hardness and elastic modulus) and cost. The manufacturing and post-processing of each strategy is assessed, with times ranging from 12 h (FFF) to 61 h (hybridization of FFF and SLS), as labor times differ significantly. Cost per model variation is also significant, ranging from EUR 80 (FFF) to EUR 600 (MJ). The main limitation is the mimicry of physiological properties. Viscoelastic properties and the combination of materials, colors and textures are also substantially different according to the strategy and the intended use. It was concluded that MJ is the best overall option, although its use in hospitals is limited due to its cost. Consequently, indirect 3D printing could be a solid and cheaper alternative.

Surgeons use different medical devices in the surgery, such as patient-specific anatomical models, cutting and positioning guides, or implants. These devices must be sterilized before being used in the operation room. There are many sterilization processes available, with autoclave, hydrogen peroxide, and ethylene oxide being the most common in hospital settings. Each method has both advantages and disadvantages in terms of mechanics, chemical interaction, and post-treatment accuracy. The aim of the present study is to evaluate the dimensional and mechanical effect of the most commonly used sterilization techniques available in clinical settings, i.e., Autoclave 121, Autoclave 134, and hydrogen peroxide (HPO), on 11 of the most used 3D-printed materials fabricated using additive manufacturing technologies. The results showed that the temperature (depending on the sterilization method) and the exposure time to that temperature influence not only the mechanical behavior but also the original dimensioning planned on the 3D model. Therefore, HPO is a better overall option for most of the materials evaluated. Finally, based on the results of the study, a recommendation guide on sterilization methods per material, technology, and clinical application is presented., Competing Interests: The authors declare no conflict of interests., (Copyright:© 2023, Valls-Esteve A, Lustig-Gainza P, Adell-Gomez N, et al.)

Pediatric surgical oncology is a technically challenging field that relies on CT and MRI as the primary imaging tools for surgical planning. However, recent advances in 3D reconstructions, including Cinematic Rendering, Volume Rendering, 3D modeling, Virtual Reality, Augmented Reality, and 3D printing, are increasingly being used to plan complex cases bringing new insights into pediatric tumors to guide therapeutic decisions and prognosis in different pediatric surgical oncology areas and locations including thoracic, brain, urology, and abdominal surgery. Despite this, challenges to their adoption remain, especially in soft tissue-based specialties such as pediatric surgical oncology. This work explores the main innovative imaging reconstruction techniques, 3D modeling technologies (CAD, VR, AR), and 3D printing applications through the analysis of three real cases of the most common and surgically challenging pediatric tumors: abdominal neuroblastoma, thoracic inlet neuroblastoma, and a bilateral Wilms tumor candidate for nephron-sparing surgery. The results demonstrate that these new imaging and modeling techniques offer a promising alternative for planning complex pediatric oncological cases. A comprehensive analysis of the advantages and limitations of each technique has been carried out to assist in choosing the optimal approach.

Naxitamab is an anti-GD2 antibody approved for the treatment of relapsed/refractory HR-NB. We report the survival, safety, and relapse pattern of a unique set of HR-NB patients consolidated with naxitamab after having achieved first CR. Eighty-two patients were treated with 5 cycles of GM-CSF for 5 days at 250 μg/m 2 /day (-4 to 0), followed by GM-CSF for 5 days at 500 μg/m 2 /day (1-5) and naxitamab at 3 mg/kg/day (1, 3, 5), on an outpatient basis. All patients but one were older than 18 months at diagnosis and had stage M; 21 (25.6%) pts had MYCN-amplified (A) NB; and 12 (14.6%) detectable MRD in the BM. Eleven (13.4%) pts had received high-dose chemotherapy and ASCT and 26 (31.7%) radiotherapy before immunotherapy. With a median follow-up of 37.4 months, 31 (37.8%) pts have relapsed. The pattern of relapse was predominantly (77.4%) an isolated organ. Five-year EFS and OS were 57.9% (71.4% for MYCN A) 95% CI = (47.2, 70.9%); and 78.6% (81% for MYCN A) 95% CI = (68.7%, 89.8%), respectively. EFS showed significant differences for patients having received ASCT ( p = 0.037) and pre-immunotherapy MRD ( p = 0.0011). Cox models showed only MRD as a predictor of EFS. In conclusion, consolidation with naxitamab resulted in reassuring survival rates for HR-NB patients after end-induction CR.

Background : Pre-surgical simulation-based training with three-dimensional (3D) models has been intensively developed in complex surgeries in recent years. This is also the case in liver surgery, although with fewer reported examples. The simulation-based training with 3D models represents an alternative to current surgical simulation methods based on animal or ex vivo models or virtual reality (VR), showing reported advantages, which makes the development of realistic 3D-printed models an option. This work presents an innovative, low-cost approach for producing patient-specific 3D anatomical models for hands-on simulation and training. Methods : The article reports three paediatric cases presenting complex liver tumours that were transferred to a major paediatric referral centre for treatment: hepatoblastoma, hepatic hamartoma and biliary tract rhabdomyosarcoma. The complete process of the additively manufactured liver tumour simulators is described, and the different steps for the correct development of each case are explained: (1) medical image acquisition; (2) segmentation; (3) 3D printing; (4) quality control/validation; and (5) cost. A digital workflow for liver cancer surgical planning is proposed. Results : Three hepatic surgeries were planned, with 3D simulators built using 3D printing and silicone moulding techniques. The 3D physical models showed highly accurate replications of the actual condition. Additionally, they proved to be more cost-effective in comparison with other models. Conclusions : It is demonstrated that it is possible to manufacture accurate and cost-effective 3D-printed soft surgical planning simulators for treating liver cancer. The 3D models allowed for proper pre-surgical planning and simulation training in the three cases reported, making it a valuable aid for surgeons.

The goals of this work were to identify factors favoring patient-derived xenograft (PDX) engraftment and study the association between PDX engraftment and prognosis in pediatric patients with Ewing sarcoma, osteosarcoma, and rhabdomyosarcoma. We used immunodeficient mice to establish 30 subcutaneous PDX from patient tumor biopsies, with a successful engraftment rate of 44%. Age greater than 12 years and relapsed disease were patient factors associated with higher engraftment rate. Tumor type and biopsy location did not associate with engraftment. PDX models retained histology markers and most chromosomal aberrations of patient samples during successive passages in mice. Model treatment with irinotecan resulted in significant activity in 20 of the PDXs and replicated the response of rhabdomyosarcoma patients. Successive generations of PDXs responded similarly to irinotecan, demonstrating functional stability of these models. Importantly, out of 68 tumor samples from 51 patients with a median follow-up of 21.2 months, PDX engraftment from newly diagnosed patients was a prognostic factor significantly associated with poor outcome (p = 0.040). This association was not significant for relapsed patients. In the subgroup of patients with newly diagnosed Ewing sarcoma classified as standard risk, we found higher risk of relapse or refractory disease associated with those samples that produced stable PDX models (p = 0.0357). Overall, our study shows that PDX engraftment predicts worse outcome in newly diagnosed pediatric sarcoma patients., (© 2021 The Authors. The Journal of Pathology: Clinical Research published by The Pathological Society of Great Britain and Ireland & John Wiley & Sons, Ltd.)

Background: Li-Fraumeni Syndrome (LFS) is a cancer predisposition syndrome characterized by the early-onset of multiple primary cancers which can occur at different moments (metachronous onset) or, more rarely, coincidentally (synchronous onset). Here we describe a previously unreported patient with presentation of synchronous Wilms tumor and Choroid plexus papilloma, leading to the diagnosis of a Li-Fraumeni Syndrome (LFS)., Case Presentation: A 6-year-old girl without previous complains presented with abdominal pain. Abdominal US and MRI showed a left renal tumor with subcapsular hematoma. Due to mild headaches, the diagnostic workup included a brain MRI that unexpectedly identified a large left parietal lobe tumor. Histopathological analysis determined the diagnosis of classic Wilms tumor and choroid-plexus papilloma (CPP), respectively. Both neoplasms showed intense nuclear p53 immunostaining associated with the pathogenic TP53 mutation c.844C > T (p.Arg282Trp). Our patient and her father shared the same heterozygous germline TP53 mutation, confirming the diagnosis of familiar Li-Fraumeni syndrome in the girl. The treatment was tailored to simultaneous tumor presentations., Conclusions: LFS has been associated with Choroid plexus carcinoma (CPC), but rarely with CPP as in our patient. That suggests that it may be advisable to consider the possibility of analyzing TP53 mutation, not only in all patients with CPC, but also in some patients with CPP, especially when histological or clinical evidences point out to perform this study. The dissimilar presentation of LFS among our patient's father, not having so far any neoplasia diagnosed, while her daughter presented precociously with two simultaneous different tumors, could be related to possible effects of modifier genes on the underlying mutant p53 genotype.

Background: The aim of this study was to test the feasibility and utility of developing patient-derived orthotopic xenograft (PDOX) models for patients with malignant peripheral nerve sheath tumors (MPNSTs) to aid therapeutic interventions in real time., Patient & Methods: A sporadic relapsed MPNST developed in a 14-year-old boy was engrafted in mice, generating a PDOX model for use in co-clinical trials after informed consent. SNP-array and exome sequencing was performed on the relapsed tumor. Genomics, drug availability, and published literature guided PDOX treatments., Results: A MPNST PDOX model was generated and expanded. Analysis of the patient's relapsed tumor revealed mutations in the MAPK1, EED , and CDK2NA/B genes. First, the PDOX model was treated with the same therapeutic regimen as received by the patient (everolimus and trametinib); after observing partial response, tumors were left to regrow. Regrown tumors were treated based on mutations (palbociclib and JQ1), drug availability, and published literature (nab-paclitaxel; bevacizumab; sorafenib plus doxorubicin; and gemcitabine plus docetaxel). The patient had a lung metastatic relapse and was treated according to PDOX results, first with nab-paclitaxel, second with sorafenib plus doxorubicin after progression, although a complete response was not achieved and multiple metastasectomies were performed. The patient is currently disease free 46 months after first relapse., Conclusion: Our results indicate the feasibility of generating MPNST-PDOX and genomic characterization to guide treatment in real time. Although the treatment responses observed in our model did not fully recapitulate the patient's response, this pilot study identify key aspects to improve our co-clinical testing approach in real time., Competing Interests: Conflict of interest statement: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s), 2020.)

Background/purpose: To identify factors that dim the efficacy of ultrasound guided saline enema (USGSE) and to design a mathematical model for predicting the probability of success of USGSE., Methods: Retrospective review of patients admitted with the diagnosis of ileocolic intussusception from 2009 to 2014. Demographics, clinical and sonographic data were reviewed., Results: 116 first episodes of ileocolic intussusceptions. 109 USGSE attempts were analyzed. Composite reduction rate was 77%. A significant relationship was found between initial location of the intussusception, free peritoneal fluid (OR=0.329, 95% CI: 0.124-0.875), negative Doppler signal and sonographic signs of intestinal occlusion and unsuccessful USGSE. Initial location beyond the splenic angle was an independent risk factor for USGSE failure (OR=0.053, 95% CI: 0.005-0.534). A predictive model based on onset of symptoms, free peritoneal fluid and intussusception location was a reliable tool for prediction (AUC 0.63, 95% CI: 0.53-0.817). Assuming that a patient with less than 75.3% chance of USGSE success is going to fail, we would identify more than 80.9% of the real failures., Conclusions: This predictive model could be a filter selection for the patients at risk of USGSE failure and therefore candidates to further imaging investigations or referral to a surgical unit., Level of Evidence: III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: In animal models of congenital diaphragmatic hernia (CDH), tracheal occlusion (TO) has induced maturation of both airway spaces and vascular structures. Airway and vascular response to TO are assumed to occur in parallel. This study aims to describe and measure the relationship between airway and vascular maturation induced by TO., Methods: A rabbit model of CDH on gestational day (GD) 23 and TO on GD 28 (term = GD 31) has been used. Two study groups have been defined: DH (diaphragmatic hernia) and TO (DH treated with TO). Animals were collected on GD 30 and blood flow data of the pulmonary artery (pulsatility index (PI) and fractional moving blood volume) were ultrasonographically measured. Lung morphometry consisted of measurements of radial alveolar count (RAC) and arterial muscular thickness., Results: Animals in the DH group (n = 9) had the worst hemodynamic parameters; their lungs were hypoplastic and had the thickest arterial muscular layer. Animals in the TO group (n = 10) had all these effects reversed. There were no correlations among hemodynamic, airway, and vascular parameters, except for RAC and PI (r = -0.528, P = 0.043)., Conclusion: Airway and vascular maturation after TO appear to be uncorrelated effects. TO could trigger several pathways that separately regulate airway and vascular responses.

Purpose: We retrospectively analyzed our institutional incidence of hemorrhagic cystitis, identified risk factors, and examined associations of risk factors with disease severity and genitourinary complication rates., Materials and Methods: We reviewed charts of all consecutive pediatric patients treated from 1986 to 2010. We analyzed demographics, underlying diagnosis and treatment data to assess risk factors for hemorrhagic cystitis. We also correlated disease severity scores with clinical predisposing factors, and performed univariate and multivariate analyses to examine associations between risk factors and outcomes., Results: Hemorrhagic cystitis was observed in 97 of 6,119 children (1.6%), most of whom (75%) had severity scores of II or III. Mean ± SD age was 12.2 ± 6.3 years for patients with hemorrhagic cystitis and 10.5 ± 7 years for patients without hemorrhagic cystitis (p = 0.017). On univariate analysis increased risk of hemorrhagic cystitis was significantly associated with age greater than 5 years, male gender, cyclophosphamide or busulfan chemotherapy, bone marrow or peripheral blood stem cell transplantation, pelvic radiotherapy and underlying diagnoses of rhabdomyosarcoma, acute leukemia and aplastic anemia. On multivariate analysis age greater than 5 years, allogeneic bone marrow or peripheral blood stem cell transplantation and pelvic radiotherapy were significantly associated with increased risk of hemorrhagic cystitis. Older age, late onset hemorrhagic cystitis, positive urine culture for BK virus and bone marrow or peripheral blood stem cell transplantation were associated with greater disease severity. Patients with higher severity scores more frequently experienced bladder perforation, hydronephrosis, overall hemorrhagic cystitis complications, and increased creatinine and blood urea nitrogen levels during followup., Conclusions: Older age, previous bone marrow or peripheral blood stem cell transplantation and BK virus in the urine are risk factors for hemorrhagic cystitis and are associated with a higher severity score. Higher severity scores are associated with increased rates of genitourinary complications and renal impairment., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: To analyze if the laparoscopic pyeloplasty is as effective as the open procedure in the ureteropelvic junction obstruction in patients under 2 years of age., Patients and Methods: Pyeloplasties performed in children under 2 years of age between 2007 and 2010. Weight, pre and postoperative renal pelvis and calices diameter, operating time, hospital stay and complications were analyzed., Results: We found no statistic differences in weight, complications, or pre and postoperative renal pelvis and calices diameter. However, we found differences in hospital stay and operating time (p<0.05)., Conclusions: Laparoscopic pyeloplasty in children under 2 years of age is a good alternative technique for ureteropelvic junction obstruction, although operating time are still long.

Objective: Lower urinary tract tumours are uncommon in paediatrics. Transitional cell carcinoma of the bladder (TCCB) is rarely found in the first two decades of life and is exceptional under 10 years of age. The present series aimed to expand the number of reported cases in the literature., Patients and Methods: In 1984-2007, six patients (four male, two female), aged 6, 9, 12, 13, 14 and 17 years, were treated at our centre. Clinical presentation was macroscopic haematuria in five and pyelonephritis in one. Physical examination, laboratory analysis, ultrasound and cystoscopy were performed before surgical treatment in all patients. Follow up was by clinical and ultrasound assessment., Results: Neither physical examination nor laboratory analysis revealed any significant abnormalities, but ultrasound showed exophytic intravesical lesions. Surgical resection was performed endoscopically. Histological studies showed grade I TCCB in all cases. The immediate postoperative period was uneventful and long-term follow up did not reveal recurrence., Conclusion: Despite its low incidence in children, TCCB must be suspected in the event of macroscopic haematuria. Ultrasound followed by cystoscopy are the ideal diagnostic tools for visualization of these tumours. Endoscopic resection proved effective in all the present cases. Follow up must be clinical with periodic ultrasound evaluation. Urine cytologic examination is ineffective. Periodic cystoscopy is indicated only in cases of clinical or ultrasonographic suspicion of recurrence., (Copyright © 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To report our experience of laparoscopic heminephroureterectomy (Hnu) in pediatric patients with duplex anomalies, in comparison to open surgery., Patients and Methods: Retrospective review of data from patients who underwent Hnu from 2005 to 2008 was performed. The patients were divided into two groups: laparoscopic (LHnu) and open surgery (OHnu). Laparoscopic surgery was performed by transperitoneal approach in majority of cases. Open surgery was performed by retroperitoneal approach in all cases., Results: Group LHnu: nine patients (8 females, 1 male) with median age of 14 months (range 3-205). Transperitoneal approach was performed in eight patients. Mean operative time was 182 min (CI 95% 146-217). No conversion to open surgery was necessary and there were no complications. Mean hospital stay was 2.44 days (CI 95% 1.37-3.52). Group OHnu: eight patients (3 females, 5 males) underwent nine heminephrectomies at median age of 6.9 months (range 1-12). Mean operating time was 152 min (CI 95% 121-183). There were no complications and mean hospital stay was 4.38 (CI 95% 2.59-6.16) days. Statistical analysis showed no statistically significant difference (P>0.05) in operating time between groups while mean hospital stay was significant (P=0.021)., Conclusion: The laparoscopic approach is feasible, safe, reduces hospital stay, does not increase operating time and has better cosmetic results. We believe this should be the first option for heminephrectomy., (Copyright © 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: In oncology practice, angioembolization has been reported for tumor reduction before surgery, treatment of life-threatening conditions, and for palliative care. Nevertheless, the overall experience with angioembolization for the treatment of tumors is limited. We report our experience in 7 nonvascular solid pediatric tumors., Materials and Methods: A retrospective review was carried out of medical records from pediatric patients (0-18 years) with solid nonvascular tumors who underwent angioembolization in the last 5 years at our institution., Results: Seven patients underwent embolization: 2 neuroblastomas, 1 metastatic paraganglioma, 1 hepatoblastoma, 1 myofibroblastic tumor, 1 osteosarcoma, and 1 undifferentiated sarcoma. The reason for angioembolization was preparation for surgery (3), treatment of a life-threatening event (1), or palliative care (3). Each case is presented and discussed. The outcome was subsequent complete surgical resection in 3 cases, tumor vanished in 1 case, symptom control was achieved in 1, and the other 2 patients improved their survival and quality of life, however, died of disease progression., Conclusions: Tumor angioembolization may enter the treatment algorithm for selected patients who have to face difficult or unwarranted surgical procedures or have diseases where conventional therapies have failed.

Malakoplakia is a rare chronic inflammatory disease that was originally described in the urinary bladder but can involve many other organs and soft tissues. It is believed to be caused by an alteration in the bacterial phagocytic system. Clinically, it is described as single or multiple tumors that can appear in any part of the body. Histologically, the presence of Michaelis-Gutmann bodies is pathognomonic. Malakoplakia in children is rare. Few pediatric cases in the urinary tract, kidney, or gastrointestinal tract have been published. We present a case of urinary and gastrointestinal malakoplakia in a 12-year-old girl.

Objective: To analyze the impact of in utero tracheal occlusion (TO) on lung tissue blood perfusion, as measured by fractional moving blood volume (FMBV) and conventional spectral Doppler, in a rabbit model of congenital diaphragmatic hernia (CDH)., Methods: In 50 fetal rabbits, a left CDH was surgically created at 23 days of gestational age (GA). At 28 days of GA, the surviving CDH fetuses were randomly assigned to undergo either TO (CDH+TO group) or a sham operation (CDH group). Twenty littermates, which were not operated on, served as internal normal controls. At 30 days of GA, lung perfusion estimated by FMBV and spectral Doppler of the proximal intrapulmonary artery were evaluated in the right lung during cesarean section. Doppler waveform analysis included the pulsatility index (PI), peak early diastolic reverse flow and peak systolic velocity., Results: Eleven CDH fetuses, 9 CDH+TO and 20 controls were suitable for the study. CDH fetuses showed a significantly higher PI [8.0 (SD 1.8) vs. 5.22 (SD 1.1), p < 0.001] and lower FMBV [13.5% (SD 4.6) vs. 23.0% (SD 2.1), p < 0.001] than the controls. In contrast, CDH+TO fetuses had a significantly lower PI [5.8 (SD 2.3) vs. 8.0 (SD 1.8), p = 0.015] and higher FMBV [27.6% (SD 7.1) vs. 13.5% (SD 4.6), p < 0.001] than CDH fetuses, with values similar to the controls. Peak early diastolic reverse flow and peak systolic velocity showed nonsignificant differences among the study groups. The lung to body weight ratio at necropsy correlated positively with lung FMBV (r = 0.60, p < 0.001) and negatively with the pulmonary artery PI (r = -0.48, p < 0.01)., Conclusion: Tracheal occlusion is consistently associated with increased lung tissue perfusion and decreased intrapulmonary impedance in a rabbit model of CDH.

Hepatocellular carcinoma (HCC) is a rare pediatric neoplasm exceptionally reported in infants and fibrolamellar hepatocarcinoma (FLC) a HCC variant. Controversy exists whether FLC has a better prognosis than classic HCC, although recent studies of children and young adults with FLC did not report a better outcome. We present a 4-month-old male infant without any related metabolic or infectious disease who developed a metastatic and multifocal FLC. Serum alpha-fetoprotein determinations were always normal. Induction chemotherapy using cisplatin and Adriamycin resulted in a partial response, however, refractory disease developed and regional metastasis precluded surgical resection. The child died from tumoral progression.

Median raphe cysts of the perineum are uncommon congenital lesions of the male genitalia. They can be found all the way from the distal penis and scrotum toward the perineum in a midline position. They are considered as congenital alterations in embryologic development. A case of a 6 year-old boy is presented. Review of the literature relevant to children was made regarding the embryologic, diagnostic, and treatment aspects. We believe it is important that adult and pediatric urologists recognize these lesions and their management to provide the appropriate information to the parents.