Congenital Internal Jugular Phlebectasia: An Anomaly Still Poorly Recognized.

Congenital internal jugular phlebectasia (CIJP) is a rare condition characterized by congenital dilatation of the vein without tortuosity that becomes more evident during straining as a lateral neck mass. CIJP often remains undiagnosed from a few months to several years after the onset of the swelling. It is frequently asymptomatic although symptomatic cases have been occasionally reported. We present the case of a healthy 7-year-old boy with a lateral neck mass, triggered by the Valsalva maneuver. Neck ultrasound (US) showed right internal jugular axial ectasia, increasing during the Valsalva maneuver; contrast computed tomography (CT) scan confirmed a fusiform dilatation of the right internal jugular vein. Due to the lack of symptoms, we treated our patient conservatively. At 5 years of follow-up, the patient is still asymptomatic, with no evidence of complications or thrombosis. Due to its self-limiting nature, treatment for asymptomatic cases of CIJP should be conservative, providing a follow-up with both clinical and US annual evaluations.

Elaboration and development of a realistic 3D printed model for training in ultrasound-guided placement of peripheral central venous catheter in children.

BACKGROUND: Simulation for training is becoming a trend topic worldwide, even if its applications are commonly limited to adulthood. Ultrasound-guided procedures require practice and experience-especially in the pediatric field, where the small size of the involved anatomical structures poses major problems. In this context, a realistic 3D printed pediatric phantom for training of the ultrasound-guided placement of peripheral central venous catheters in children was developed. MATERIALS AND METHODS: Starting from Computed Tomography scans of an 8 years-old girl, her left arm was virtually reconstructed-including bones, arteries, and veins-through a semi-automatic segmentation process. According to preliminary results, the most suitable 3D printing technologies to reproduce the different anatomical structures of interest were selected, considering both direct and indirect 3D printing techniques. Experienced operators were asked to evaluate the efficacy of the final model through a dedicated questionnaire. RESULTS: Vessels produced through indirect 3D printing latex dipping technique exhibited the best echogenicity, thickness, and mechanical properties to mimic real children's venous vessels, while arteries-not treated and/or punctured during the procedure-were directly 3D printed through Material Jetting technology. An external mold-mimicking the arm skin-was 3D printed and a silicone-based mixture was poured to reproduce real patient's soft tissues. Twenty expert specialists were asked to perform the final model's validation. The phantom was rated as highly realistic in terms of morphology and functionality for the overall simulation, especially for what concerns vessels and soft tissues' response to puncturing. On the other hand, the involved structures' US appearance showed the lower score. CONCLUSIONS: The present work shows the feasibility of a patient-specific 3D printed phantom for simulation and training in pediatric ultrasound-guided procedures.

46, XY Disorders of Sexual Development: a case report and theoretical framework.

BACKGROUND AND AIM: Disorders of sexual differentiation (DSD) with karyotype 46,XY include gonadal developmental differences such as complete gonadal dysgenesis, partial gonadal dysgenesis, testicular regression and ovotesticular sexual differentiation disorder, differences in androgen synthesis or action, such as androgen synthesis deficiency, androgen action deficits, LH receptor deficiency, AMH synthesis or action deficits, and other conditions such as severe hypospadias, cloaca estrophy, etc. Methods: A 17 years-old girl came to our attention for hirsutism, clitoral hypertrophy, primary amenorrhea, and bilateral mammary hypoplasia. According to clinical features and anamnesis, the diagnosis of 46, XY DSD was made. For diagnostic purposes, she underwent an extensive genetic analysis, hormone dosage and instrumental examinations. After a clitoridoplasty and hormone replacement treatment, the patient performs appropriate multidisciplinary follow-up and regular psychotherapy. RESULTS: The clinical case reported falls, according to the recent classification developed by the Chicago Consensus, within the scope of DSD with karyotype 46, XY. About 160 cases of patients with 17ß-HSD3 deficiency, diagnosed at a mean age of 12 years, are described in the literature, most of them coming from Western Asia and Europe and only three cases from Eastern Asia. Clinically, about 30% of patients showed virilization, 20% clitoromegaly, ambiguous genitalia, inguinal/labial mass, 16% primary amenorrhea, and 5% absence of mammary development, features that are partly traced in the case described here. CONCLUSIONS: This case underscores the complexity of managing individuals with DSD. Having acquired the concept that irreversible surgery should be avoided, except in cases where failure to do so would determine health risks, the primary objective of the medical decision lies in meeting conditions aimed at harmonious sexual identification, especially regarding sexual activity and fertility, involving a team of experienced professionals (psychologists, pediatricians, surgeons, endocrinologists, radiologists), capable of promptly identifying suggestive clinical signs.

Effectiveness of Articulating Linear Stapler for Total and Partial Laparoscopic Splenectomy in Children.

Purpose: We performed a retrospective study to determine the effectiveness and feasibility of articulating linear stapler in laparoscopic total splenectomy (LTS) and laparoscopic partial splenectomy (LPS), focusing on technical laparoscopic skills that could help pediatric surgeons to avoid intra- and postoperative complications. Methods: Retrospective cohort study of children younger than 18 years who underwent laparoscopic spleen surgery between January 2008 and March 2020. Age, sex, indication for surgery, operative time (OT), intra- and postoperative complications, and postoperative length of hospital stay (LHS) were analyzed. Data from parenchymal resection and vessels sealing techniques were obtained. Results: Thirty patients, 19 LTS and 11 LPS, were included. The mean age of the patients was 10.9 years, and 16 patients were male and 14 were female. For hematologic diseases, LTS was the elective surgery, associated with cholecystectomy in 5 cases. LPS was the common procedure for splenic cysts. The stapler was used in LTS to close the hilum vessels and in LPS for parenchymal resection. No statistically significant differences in OT were observed comparing LTS and LPS. Two conversions occurred in LTS; none in LPS. The mean LHS was 6 days in both groups. No recurrence or major complications appeared in both groups at 1-12 years of follow-up. In particular for LPS, there are no relapse of cyst neither reduction in splenic function. Conclusions: This study shows the effectiveness, feasibility, and safety of mechanic stapler in splenic surgery both for hilum vessels sealing and for parenchymal resection. The use of this device can reduce risk of hemorrhagic recurrences or major surgical complications improving the safety of the operation.

Robotics-Assisted Pediatric Oncology Surgery-A Preliminary Single-Center Report and a Systematic Review of Published Studies.

AIM: The use of robotics-assisted surgery in oncology has been proved effective and safe in adults. Despite these results, the use of robotics has been rarely reported for pediatric oncology. Our review aims to evaluate the safety and feasibility of robotics-assisted surgery in this field, analyzing our experience and performing a systematic review of the most recent studies. METHODS: We reviewed all patients affected by an oncological disease who underwent a robotics-assisted procedure at our institute. We performed a systematic review of the literature from 2012 to 2021 on the subjects. FINDINGS: A total of 14 patients underwent robotics-assisted tumor resection. Eleven procedures (median age 13.2-years old) were carried out in children with adnexal lesions (seven tumor excision and four ovariectomies). Histological diagnosis was mature teratoma (six), serous papillary cystadenofibromas of the fallopian tube (two), ovarian serous cystadenoma (one), ovarian mucinous cystadenoma (one), and ovarian seromucinous cystadenoma. The median length of stay was 2 days. No recurrences or complications at a median follow-up of 2.1-years were observed. A 5-year-old girl underwent a complete posterior resection of a type 3 sacrococcygeal tumor with a robotics-assisted approach for the dissection of a possible intraabdominal residual component of the lesion. No intra- and postoperative complications were recorded. Complete excision of a recurrent differentiating neuroblastoma of the left para-renal region was performed on a 9-year-old girl. An idiopathic anaphylactic shock occurred 1 day after the procedure. At 9 months' follow-up, no local recurrences of the lesion were observed. Overall, we reported no conversion to open surgery. Lastly, a robotic excision of a growing left superior mediastinal intermixed ganglioneuroblastoma was performed on an 8-year-old girl with no postoperative complications. Follow-up was uneventful (7 months). In the literature, the rate of complications ranges from 0 to 28%, mainly related to difficult dissection and impaired anatomy. Conversion is reported in 5% of all oncological procedures, due to more invading tumors and altered anatomical features. No robotics-related complications were reported. CONCLUSION: Robotics-assisted surgery in pediatric oncology has proven to be feasible. Nevertheless, its use should be limited to selected cases and performed by highly trained oncological surgeons. Preparation and patient positioning, alongside a correct port placement, are crucial to carrying out these procedures. Further innovations in robotics may allow a wider application of this technology in pediatric oncology.

Intracavitary electrocardiography-guided positioning of central vascular access device can spare unnecessary ionizing radiation exposure in pediatric patients.

BACKGROUND: Most hospital protocols-including those of our own institute-require the use of radiography to validate tip position in every central vascular access device placement. This study evaluated whether unnecessary ionizing radiation exposure could be spared in the pediatric population when intracavitary electrocardiography is used to guide catheter placement. MATERIAL AND METHODS: Retrospective study of intracavitary electrocardiography-guided central vascular access device placements in our pediatric surgery department between 2013 and 2018. We evaluated the operating time, success in positioning the catheter, and accuracy of final tip position. We also assayed the effects of catheter type and of catheter access point on operating time, success, accuracy, and complications. We applied the chi-square test for statistical analysis. RESULTS: In total, 622 interventions of central vascular access device placements were evaluated; 340 intracavitary electrocardiography-guided central vascular access device placements were included in the study. The electrocardiography method successfully positioned the tip of the catheter in 316/340 (92.94%) of placements. Where intracavitary electrocardiography placement was successful, radiography confirmed accuracy of tip position in 314/316 (99.41%) of placements. CONCLUSION: When electrocardiography-guided positioning is uneventful and a valid P-Wave pattern is seen, postprocedure radiograph imaging for verification is unnecessary. Any effort should be made to upgrade hospital policies according to evidences and newest guidelines to spare pediatric patients harmful exposure to radiation by limiting the use of radiography only to selected cases.

Low penetrance COL5A1 variants in a young patient with intracranial aneurysm and very mild signs of Ehlers-Danlos syndrome.

Spontaneous cervical artery dissection (CeAD) is a major cause of ischemic stroke in young adults, whose genetic susceptibility factors are still largely unknown. Nevertheless, subtle ultrastructural connective tissue alterations (especially in the collagen fibril morphology) are recognized in a large proportion of CeAD patients, in which recent genetic investigations reported an enrichment of variants in genes associated with known connective tissue disorders. In this regard, COL5A1 variants have been reported in a small subset of CeAD patients, with or without classical Ehlers-Danlos syndrome (cEDS) features. We investigated a 22-year-old patient with intracranial aneurysm and mild connective tissue manifestations reminiscent of EDS. Whole-exome sequencing identified two COL5A1 missense variants in trans configuration: NM_000093.5:c.[1588G>A];[4135C>T], NP_000084.3:p.[(Gly530Ser)];[(Pro1379Ser)]. Functional assays demonstrated a significant decrease of collagen α1(V) chain expression in both heterozygous parents compared to control cells, and an additive effect of these two variants in the proband. Interestingly, both parents manifested very subtle EDS signs, such as atrophic scars, recurrent bone fractures, colonic diverticulosis, varicose veins, and osteoarthritis. Our findings emphasize the involvement of COL5A1 in the predisposition to vascular phenotypes and provide novel insights on the c.1588G>A variant, whose functional significance has not been definitely established. In fact, it was previously reported as both "disease modifying", and as a biallelic causative mutation (with heterozygous individuals showing subtle clinical signs of cEDS). We speculated that the c.1588G>A variant might lead to overt phenotype in combination with additional genetic "hits" lowering the collagen α1(V) chain expression below a hypothetical disease threshold.

Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

INTRODUCTION: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. METHODS: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. RESULTS: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

Juxta-articular Myxoma of the Hip: A Rare Pediatric Tumor.

Juxta-articular myxoma (JAM) is a very rare myxoid tumor in pediatric age. JAM is frequently located at large joints, particularly in the knee, and most lesions present as palpable swelling sometimes associated with pain or tenderness. Only three cases of pediatric JAM have been reported in the literature to date. We describe a case of JAM in a 12-year-old boy, characterized by several unique aspects related to age, site of onset, size, and associated symptoms.

Juvenile Granulosa Cell Tumor of the Testis: Prenatal Diagnosis and Management.

Prepubertal primary testicular tumors account for ∼1% of all pediatric solid tumors. We report a new case of prenatal diagnosis of juvenile-type granulosa cell tumor (JGCT). A fetal ultrasound performed at the 38th week of gestation for suspected nonvertex presentation identified a left multilocular septated cystic testicular mass, suggestive for JGCT. At birth, a painless left scrotal mass was detected. Ultrasound re-evaluation excluded torsion of the testis. Tumor markers and abdominal ultrasound were normal for age. Inguinal exploration revealed a cystic mass beneath the tunica albuginea that had replaced all the normal parenchyma. Since organ-sparing surgery was thus not feasible, an orchiectomy was performed and diagnosis of JGCT was confirmed. At 7-year follow-up, the child presented an uneventful outcome. Our case shows that neonatal JGCT, which has an intrauterine genesis, can be diagnosed prenatally by ultrasound in the last weeks of pregnancy.

Intestinal Duplication and Hirschsprung's Disease: An Extremely Rare and Misleading Combination.

Hirschsprung's disease and, more rarely, intestinal duplication can both cause intestinal obstruction in neonates. The simultaneous occurrence of these two diseases is reported in only two studies, and in both cases, intestinal duplication was an incidental finding, as it had not determined clinical intestinal occlusion. This paper reports a unique case of coexistence of the two conditions, with both causing intestinal obstruction, delayed appropriate, and definitive surgical treatment.

Extracellular vesicles derived from mesenchymal cells: perspective treatment for cutaneous wound healing in pediatrics.

AIM: We evaluated the effects of the intradermal injection of extracellular vesicles (EVs) derived from adipose stem cells (ASC-EVs) and bone marrow cells (BM-EVs) in an experimental cutaneous wound repair model. METHODS: Mesenchymal stem cells (MSCs) were in vitro expanded from adipose (ASC) or BM tissues (BM-MSC) of rabbits. EVs were separated from the supernatants of confluent ASC and BM-MSCs. Two skin wounds were induced in each animal and treated with MSC or EV injections. Histological examination was performed postinoculation. RESULTS: EV-treated wounds exhibited a better restoration compared with the counterpart MSC treatment. ASC-EV-treated wounds were significantly better than BM-EVs (p = 0.036). CONCLUSION: EV topical inoculation provides restored architecture during cutaneous wound healing and represents a promising solution for regenerative medicine in children.

Mesenchymal stromal cells for cutaneous wound healing in a rabbit model: pre-clinical study applicable in the pediatric surgical setting.

OBJECTIVE: Mesenchymal stromal cells (MSCs) expanded in vitro have been proposed as a potential therapy for congenital or acquired skin defects in pediatrics. The aim of this pre-clinical study was to investigate the effects of intradermal injections of MSC in experimental cutaneous wound repair comparing allogeneic and autologous adipose stem cells (ASCs) and autologous bone marrow-mesenchymal stromal cells (BM-MSCs). METHODS: Mesenchymal stromal cells were in vitro expanded from adipose and BM tissues of young female New Zealand rabbits. MSCs were characterized for plastic adhesion, surface markers, proliferation and differentiation capacity. When an adequate number of cells (ASCs 10 × 10(6) and BM-MSCs 3 × 10(6), because of their low rate of proliferation) was reached, two skin wounds were surgically induced in each animal. The first was topically treated with cell infusions, the second was used as a control. The intradermal inoculation included autologous or allogeneic ASCs or autologous BM-MSCs. For histological examination, animals were sacrificed and wounds were harvested after 11 and 21 days of treatment. RESULTS: Rabbit ASCs were isolated and expanded in vitro with relative abundance, cells expressed typical surface markers (CD49e, CD90 and CD29). Topically, ASC inoculation provided more rapid wound healing than BM-MSCs and controls. Improved re-epithelization, reduced inflammatory infiltration and increased collagen deposition were observed in biopsies from wounds treated with ASCs, with the best result in the autologous setting. ASCs also improved restoration of skin architecture during wound healing. CONCLUSION: The use of ASCs may offer a promising solution to treat extended wounds. Pre-clinical studies are however necessary to validate the best skin regeneration technique, which could be used in pediatric surgical translational research.

Robotic-assisted surgery for excision of an enlarged prostatic utricle.

INTRODUCTION: Prostatic utricle is a rare malformation arising from incomplete regression of the Müllerian ducts. Diagnosis is easily made but management may be challenging. The minimally invasive approach has so far been considered the gold standard for surgical treatment. Many endoscopic and surgical procedures have been described for removal, but to date only few cases of robot-assisted procedures have been mentioned in the literature and there are no reports of redos. PRESENTATION OF THE CASE: We report the case of a giant prostatic utricle cyst successfully treated with robotic-assisted surgery two years after an unsuccessful first attempt at laparoscopic excision. No relapse was found at one year follow up. DISCUSSION: We were able to excise a retro-vescical structure in spite of adhesions caused by the previous surgery and the very large size of the diverticulum thanks to the high magnification and 3-D visualization available in robotic assisted laparoscopy. CONCLUSION: Robot assisted laparoscopy should be considered an advantageous technique for the treatment of prostatic utricle.

Five millimetre-instruments in paediatric robotic surgery: Advantages and shortcomings.

PURPOSE: The study was designed to assess the utility and controversies surrounding the usage of 5-mm instruments in paediatric robotic surgery. Adequate, delicate instruments for surgery in very narrow spaces are still lacking. MATERIAL AND METHODS: Thirty children underwent elective abdominal robotic surgery. Working sites, assembly and operative time, hospital stay, advantages, complications and shortcomings are reported. RESULTS: Interventions were performed in the following anatomical sites: 11 upper abdominal, nine pelvic, ten renal procedures. The majority of procedures required two operative trocars. A 2-3 mm accessory port was necessary for operations in the renal area and upper abdomen. The ports had to be placed at least 3 cm from the costal margins and superior iliac spines and at an angle of at least 130° with respect to the camera trocar. This configuration allowed intra-corporal knotting, vessel ligation and dissection with instruments in the inverted position. Operative times and hospital stays were similar to those reported for 8 mm-instruments. CONCLUSION: The use of 5-mm instruments was advantageous in renal and pelvic sites. The benefits in upper abdominal surgery need further evaluation, particularly in patients weighing <10 kg. Smaller sized instruments with high endowrist dexterity would resolve the problems encountered in paediatric robotic-assisted surgery using 5-mm instruments.

Pediatric robotic pyeloplasty in patients weighing less than 10 kg initial experience.

AIM: To report the feasibility and safety of a robotic-assisted laparoscopic pyeloplasty (RALP) in patients weighing <10 kg. MATERIALS AND METHODS: Three patients weighing between 5 and 8 kg who were affected by severe congenital ureteropelvic junction obstruction, including a child with solitary kidney, were subjected to RALP. Three trocars were placed; sutures and pyeloplasty remodeling were performed with interrupted stitches. A double J stent was inserted through a 2-mm transparietal angiocatheter to protect the pyelic suture. RESULTS: The procedures were all completed within 90 minutes, the "docking" time requiring 20 minutes. The patients were discharged on postoperative day 2, without any complications. CONCLUSIONS: Comprehensive assessment of pyelic suture in a very narrow field with 2 operative instruments is feasible and safe. Robotic pyeloplasty provides all the advantages of mini-invasive surgery with the added advantage of higher magnification and excellent surgical navigation in very small spaces and on fragile infant tissues.

Intrascrotal lipoblastoma in a ten year old: case report and review of literature.

Lipoblastoma is a rare benign soft tissue tumor encountered almost exclusively in infancy and early childhood. The location of tumors varies, but most occur in the extremities, trunk, head and neck. Less frequently, lipoblastomas have been reported in the mediastinum, the retroperitoneum and the inguinal region. Only 7 cases of lipoblastoma in the scrotum have been reported so far in the English literature, with none of the patients older than 8. We report an intrascrotal lipoblastoma in a 10 year-old boy. The differential diagnosis is discussed with reference to the literature.

Robotic assisted surgery in pediatric gynecology: promising innovation in mini invasive surgical procedures.

STUDY OBJECTIVE: Robotic assisted surgery is not yet widely applied in the pediatric field. We report our initial experience regarding the feasibility, safety, benefits, and limitations of robot-assisted surgery in pediatric gynecological patients. DESIGN: Descriptive, retrospective report of experience with pediatric gynecological patients over a period of 12 months. SETTING: Department of Pediatric Surgery, IRCCS Policlinico San Matteo Foundation. PARTICIPANTS: Children and adolescents, with a surgical diagnosis of ovarian and/or tubal lesions. MAIN OUTCOME MEASURES: Robot assembly time and operative time, days of hospitalization, time to cessation of pain medication, complication rate, conversion rate to laparoscopic procedure and trocar insertion strategy. RESULTS: Six children and adolescents (2.4-15 yrs), weighing 12-55 kg, underwent robotic assisted surgery for adnexal pathologies: 2 for ovarian cystectomy, 2 for oophorectomy, 1 for right oophorectomy and left salpingo-oophorectomy for gonadal disgenesis, 1 for exploration for suspected pelvic malformation. Mean operative time was 117.5 ± 34.9 minutes. Conversion to laparatomy was not necessary in any of the cases. No intra- or postoperative complications occurred. CONCLUSION: Initial results indicate that robotic assisted surgery is safely applicable in the pediatric gynecological population, although it is still premature to conclude that it provides better clinical outcomes than traditional laparoscopic surgery. Randomized, prospective, comparative studies will help characterize the advantages and disadvantages of this new technology in pediatric patients.