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.

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.

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.

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.

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.

Robotic-assisted surgery approach in a biliary rhabdomyosarcoma misdiagnosed as choledochal cyst.

Rhabdomyosarcoma is a soft tissue malignant tumor affecting 1% of children from 0 to 14 years. Preoperative imaging may not always be diagnostic for hepatobiliary rhabdomyosarcoma and differential diagnosis with choledochal cyst (CC) could be difficult. We report a case of 2-years-old girl with a strange CC pattern of presentation. A grapelike lesion involving the choledochal and biliary ducts was easily and completely resected by robotic assisted surgery. Since no previous reports were available about oncologic safety of robotic approach, the porto-enterostomy was performed in open surgery. On histologic examination, the specimen revealed a botryoidembryonal rhabdomyosarcoma affecting both the common bile duct and the common hepatic duct. One year postoperatively the child is safe of tumor relapse. Robotic approach seems to be safe and advantageous to obtain a radical excision of the tumor at the porta hepatis, even in case of misdiagnosed malignant lesion mimicking a CC.

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.

Assessment of treatment response in hepatocellular carcinoma: a review of the literature.

Hepatocellular carcinoma (HCC) has a high incidence all over the world. Even if the primary end point of treatment of HCC is survival, radiological response could be a surrogate end point of survival, and could have a key role in clinical management. Since 1950 several radiological response criteria have been applied; however, it was not until 2000 that specific criteria for HCC were introduced by the European Association for the Study of the Liver (EASL), and these were then standardized in 2010 with the development of the modified Response Evaluation Criteria for Solid Tumors (mRECIST) for HCC. The purpose of this brief review is to compare data in literature regarding the application and the performance of mRECIST in clinical practice, and to discuss unclear and open issues.

Treatment of hepatocellular carcinoma: present and future.

Hepatocellular carcinoma is a major health problem. It is the sixth most common cancer worldwide and the third most common cause of cancer-related death. Despite the availability of several treatment opportunities, diagnosis is still made in an advanced phase, limiting application of most therapeutic choices that currently are based on the Barcelona Clinic Cancer Liver Classification and include surgical resection, orthotopic liver transplantation and ablative methods for very early and early disease, arterial chemoembolization for intermediate stages and systemic therapy with sorafenib for advanced hepatocellular carcinoma. Thanks to novel advancements in knowledge of molecular pathogenesis of this tumor, many new systemic agents and locoregional treatments are in different stages of clinical development and they represent an important promise of further improvements in patients' survival.

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.

Adrenal masses in children: the role of minimally invasive surgery.

The development of laparoscopic surgery has extended its uses to include adrenalectomy in children and in adults. Because conventional adrenalectomy requires a large incision, minimally invasive surgery offers a less aggressive solution in some selected cases. Twenty-nine adrenal masses in 26 children were treated using adrenalectomy between 1994 and 2004 (12 were treated laparoscopically, the remaining 17 with open surgery). Minimally invasive procedures were limited to the removal of small localized adrenal tumors and to biopsies. Although this approach must be limited to operations on lesions presumed to be benign, preoperative criteria for nonmalignancy are often difficult to define. Indications can be expanded to include to stage I neuroblastoma. There seems to be no age and weight limit. The technique applied varies in accordance with anatomy and the surgeon's experience: minimally invasive adrenalectomy, in our experience, was preferentially performed through a lateral retroperitoneal approach. Laparoscopic adrenalectomy can be used if the selection of cases is rigorous and the operations are performed by well-trained laparoscopic surgeons.

WAGR syndrome: is the 'R' always justified?

Although mild-to-moderate intellectual disability is usually considered part of WAGR syndrome (Wilms' tumour (WT), Aniridia, Genital abnormalities, and metal Retardation, due to 11p13 deletion) the neuropsychological profile of the syndrome is little reported in the literature. We report about a 12-year-old boy presenting with WAGR syndrome (WT, right complete aniridia, bilateral cryptorchidism, interstitial deletion involving band 11p13) but with no mental retardation. An in-depth clinical evaluation revealed no behavioural or social problems and the child's neuropsychological profile was found to be within the normal range for all abilities and functions investigated (with the exception of an impulsive cognitive style and some difficulties in academic skills). This case underlines the importance of in-depth neuropsychological evaluation that includes not only IQ measurement, but also examination of attention and academic skills, in order to establish the complete phenotypical profile of WAGR patients, rather than labelling them as learning disabled (i.e. mental retardation).

Urethral polyp in a 1-month-old child.

Urethral polyps are a rare finding in children, particularly in the very young. They are suspected by the presence of various clinical signs such as obstruction, voiding dysfunction and haematuria. There is an association with other urinary tract congenital anomalies. They are usually benign fibro-epithelial lesions with no tendency to recur and are treated by surgical ablation, fulguration or laser therapy. We report a 1-month-old boy with an antenatally diagnosed left ectopic pelvic kidney, postnatal urinary tract infection and no clinical signs of obstruction. Voiding cystourethrography to exclude vesico-ureteric reflux showed a trabeculated bladder and a mobile filling defect in the posterior urethra. Owing to its large size, cystotomy was necessary to remove the polyp successfully.

Bladder papilloma in a 12-year-old girl.

Epithelial bladder tumours are rare in childhood. A case of bladder papilloma in a 12-year-old girl is reported. Emphasis is given to the important role of ultrasound in diagnosis. Endoscopic treatment was successful and no evidence of recurrence was found at 5-year follow-up. Exposure to a chemical environmental factor (volatile shoe adhesives) is suggested as a possible cause of the tumour.