Growing teratoma syndrome in children and adolescents: Prevalence and surgical outcome.

INTRODUCTION: Patients affected by metastatic germ cell tumors may occasionally experience enlargement of masses with concurrent normalization of tumor markers during or after chemotherapy. This phenomenon is described as growing teratoma syndrome (GTS). The aim of the pre sent study is to assess the prevalence of GTS in the pediatric population and its implications in terms of surgical outcome. PATIENTS AND METHODS: The clinical notes of patients diagnosed with stage III and IV malignant germ cell tumors from January 2010 until December 2020 at our Institution were retrospectively reviewed. The prevalence of GTS, treatment strategies, survival, and outcome were analyzed. RESULTS: Thirty-three patients with high-stage malignant germ cell tumors were diagnosed in our institution in the analyzed period. Nine patients (28%) had radiologic evidence of enlargement of persistent masses with normal markers after chemotherapy; these patients were classified as GTS patients. All nine patients underwent resection of metastatic lymph nodes, and six had surgery on visceral metastases. In six patients, radical excision of all metastatic sites was achieved; five patients are alive and in complete remission, while one died because of peri-operative complications. Out of the three patients who could not achieve radical excision of the metastases, two died of progressive disease, and one is alive with progressive disease. CONCLUSIONS: Patients affected by GTS have a risk of progression of chemotherapy-resistant disease and death. Radical surgical excision is essential to achieve disease control and long-term survival.

The use of postoperative calibrations in Hirschsprung disease: a practice to reconsider?

PURPOSE: Daily postoperative anal dilations after endorectal pull-through for Hirschsprung disease (HD) are still considered a common practice. We analyzed the potential risks of this procedure and its effectiveness compared to a new internal protocol. METHODS: All infants (< 6 months of age) who underwent transanal endorectal pull-through between January 2021 and January 2023 were prospectively enrolled in a new postoperative protocol group without daily anal dilations (Group A) and compared (1:2 fashion) to those previously treated by postoperative anal dilations (Group B). Patients were matched for age and affected colonic tract. Patients with associated syndromes, extended total intestinal aganglionosis, and presence of enterostomy were excluded. Outcomes considered were: anastomotic complications (stenosis, disruption/leakage), incidence of enterocolitis, and constipation. RESULTS: Eleven patients were included in group A and compared to 22 matched patients (group B). There were no significant differences in the occurrence of anastomotic complications between the two groups. We found a lower incidence of enterocolitis and constipation among group A (p = 0.03 and p = 0.02, respectively). CONCLUSION: A non-dilation strategy after endorectal pull-through could be a feasible alternative and does not significantly increase the risk of postoperative anastomotic complications. Moreover, some preliminary advantages such as lower enterocolitis rate and constipation should be further investigated.

Hypospadias management in children with anorectal malformation: a multidisciplinary single center experience.

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.

Biliary atresia in preterm infants: a single center experience and review of literature.

Introduction: The diagnosis of biliary atresia (BA) remains challenging, and there is still uncertainty regarding the optimal time to perform a Kasai portoenterostomy (KPE). Little is known about the difficulties in the diagnosis and outcomes of BA in preterm infants (PBA). This study, which represents the first Italian report of preterm infants with BA, aims to describe a single-center experience of BA in preterm newborns. Methods: We retrospectively reviewed all infants consecutively diagnosed with BA who underwent a Kasai procedure at the Bambino Gesù Children's Hospital between January 1998 and December 2021. Prematurity was defined as a gestational age (GA) of <37 weeks. Demographic, laboratory, and histology data were recorded, and the main outcomes considered were clearance of jaundice (COJ), native liver survival, and mortality. Results: A total of 21 PBA were compared with 117 term BA controls (TBA). The median GA of PBA was 35.1 (32-36.1) weeks, with a mean birth weight of 2,100 (1,897-2,800) g. Age at first presentation was significantly lower in PBA patients: 46 (22-68) vs. 61 (44-72) days; p = 0.02. The median age at KPE was similar between the two groups: 70 days (33 corrected) for PBA vs. 67 in TBA; p = 0.8. At the time of surgery, median serum bilirubin was lower in the PBA group (7.7 vs. 8.6 mg/dl, p = 0.04). Similarly, the median APRi at the time of KPE was lower but not significant in the PBA group: 1.09 vs. 1.16; p = 0.8. No differences were found in terms of COJ between the PBA and TBA groups: n = 9 (43%) vs. 34 (35%); p = 0.2. Overall native liver survival was similar between the two groups: 8.6 (4.8-12.2) for the PBA group vs. 7.6 (5.6-9.5) years for the TBA group with no significant differences; p = 0.45. Post-KPE native liver survival was similar between the two groups: 38% vs. 52% at 5 years for the TBA and PBA groups, respectively; p = 0.54. Conclusion: The PBA and TBA groups appear to have similar outcomes in terms of COJ, overall native liver survival, and 5-year liver survival. Considering the corrected GA, early KPE is related to lower cholestatic damage. Further multicenter studies are required.

A new custom-made bivalve brace for pectus carinatum in children and adolescents: preliminary promising experience of 140 patients from a tertiary center.

Introduction: International research suggests that poor patient compliance is the main cause of tutor failures in the context of potential novel orthopedic bivalve braces for conservative treatment of pectus carinatum. Our entire experimental study is based on the hypothesis that a rigid bivalve brace that patients can accept could solve the main problem associated with the conservative approach-poor compliance. The hypothesis was to reduce the thickness and weight of the classic bivalve brace to ensure concealment and make it sustainable enough to be worn several hours a day without compromising its therapeutic efficacy. Materials and method: The research was conducted from January 2020 to December 2022 to ensure follow-up of all participants for at least 6 months. In 36 months, 140 patients with pectus carinatum were assessed and conservatively treated with the studied guardian to analyze the therapeutic efficacy of the bivalve brace and patient compliance. From the initial visit, the parents and patient were informed that this is a 2-year therapeutic course during which the bivalve brace should be worn at least 23 h a day (with 1 h of abstinence per day for routine personal hygiene practices). Compliance is the key to therapy success, and the duration of treatment depends on patient adherence. Results: The exceptional effectiveness of the experimental brace was confirmed by both the questionnaire from the patients (with an average satisfaction rate of 8.9/10) and an assessment of the therapy's results by a properly selected medical committee (with a VAS scale satisfaction of 7.2/10 for symmetric forms and 7.1/10 for asymmetric ones). Conclusion: In conclusion, the analyzed data confirmed the research hypotheses. First, none of the 140 patients had cardiovascular diseases directly related to their condition, confirming that pectus carinatum is a pathology of a purely aesthetic nature. Second, a cheap, lightweight, and easily obscured brace significantly improved patient compliance. Along with this, the social relevance of the aesthetic aspect today may be an important factor in motivating the study cohort to adhere to therapy. In the past, esthetics and appearance were less relevant at the social level, which may have contributed to the high abandonment and reduced compliance rates of the many studies in the literature.

Case report-Every thoracic surgeon's nightmare: cardiac and lung perforation during placement of Nuss bar for pectus excavatum.

Introduction: The prevalence of life-threatening complications (LTCs) related to the minimally invasive repair of pectus excavatum (MIRPE) is unknown and underreported. The aim of this study is to contribute to the real prevalence of these rare but dramatic complications and show what went wrong in order to prevent it in the future. Case presentation: A 15-year-old boy affected by pectus excavatum with severe asymmetric deformity of the chest wall was evaluated for elective corrective surgery. Preoperative computed tomography showed a Haller index of 5.7 and a correction index of 0.40. MIRPE was performed under right video-assisted thoracoscopy. Cardiac arrhythmias occurred after placement of the bar introducer. The introducer was removed, and massive bleeding was noted. Emergency Clamshell thoracotomy was performed, and cardiac surgeon was alerted immediately. A first pulmonary wound was found and controlled. Two cardiac lacerations were found: on the interventricular wall and on the right atrium. Under cardiopulmonary bypass, cardiac lacerations were sutured and other three pulmonary wounds were repaired. An urgent fasciotomy was performed for compartmental syndrome of the right lower art after femoral cannulation. Pulmonary distress occurred; the patient was admitted on ECMO (ExtraCorporeal Membrane Oxygenation) in intensive care unit. Right lower lobectomy was carried out on the fifth postoperative day due to massive pulmonary bleeding requiring temporary tracheostomy. The patient was discharged to rehabilitation after 3 months with no brain injuries, minor hearing loss, and tracheostomy. Conclusion: We want to maintain the high alertness required for this procedure. Reporting these scaring complications contributes to the real prevalence of LTCs. We suggest the use of bilateral thoracoscopy and crane elevator in severe sternal defects. We also suggest to have a cardiac surgeon available in the hospital owing to cardiac perforation.

Case Report: Modified Taulinoplasty: a new technique for minimally invasive repair of pectus excavatum.

Introduction: About 95% of congenital chest wall deformities are pectus abnormalities, with pectus excavatum (PE) being the most common. The purpose of this work is to offer a modified Taulinoplasty Technique based on 35 consecutive PE patients' 1-year single-center experience in 2022. Technique: One minimally invasive procedure for PE is taulinoplasty. In order to prevent invasion of the mediastinum or pleural cavity, it is considered that external traction can be used to raise the sternum. Our experience indicates that the most common surgical consequences of this procedure-which involves creating a submuscular and subcutaneous tunnel to install the metal device-are post-operative seroma and wound dehiscence. We modified the conventional method to achieve more aesthetically pleasing results. Discussion: Taulinoplasty seems to be a safe technique, easier and more feasible than standard Taulinoplasty, with better outcomes in terms of surgical complications, although further experience is necessary to confirm our preliminary data.

Non-invasive treatment of sigmoid volvulus in a child. The role of the endoscopist.

Sigmoid volvulus (SV) is a rare cause of intestinal obstruction in children. Its varied presentation and rapid progression engender a high risk of morbidity and mortality. We report two cases of SV in teenage boys. Patient 1 is 16 years old and patient 2 is 17. Both presented to our institution with recent-onset abdominal pain, constipation, and nausea and vomiting, and both had previous episodes of SV. Patient 1 had been surgically treated with manual derotation, and patient 2, who had redundant colon, had two past episodes of endoscopically-treated SV. Both patients were in poor condition and had distended but treatable abdomens, with no peritoneal signs. After computed tomography (CT) confirmation of the clinical impression of no ischemia or perforation, we decided to attempt detorsion of the SV and decompression of proximal dilated colon by flexible endoscopy, and planned for elective surgery shortly after the endoscopic procedure. Because sigmoid volvulus is so rare in children, operative and technical details of endoscopic management are gleaned from the larger adult experience. In patients without signs of complication, initial endoscopic reduction is the gold standard, and elective sigmoid resection with primary anastomosis is often required to prevent recurrence. KEY WORDS: Case report, Children, Endoscopy, Endoscopic treatment, Sigmoid volvulus, Volvulus.

Burned-Out Testicular Tumors in Adolescents: Clinical Aspects and Outcome.

Purpose: Testicular germ cell tumors are the fourth most common neoplasm in adolescents, accounting for 8% of all tumors in the age group 15-19 years. On rare instances, the primary testicular lesion is not clinically or radiologically evident while nodal or visceral metastases represent the clinical manifestations of the disease. This phenomenon is described as "burned-out testicular tumor." In this paper, the authors report a single-institution experience with burned-out testicular tumors in adolescents and discuss their clinical implications. Patients and Methods: All the patients diagnosed with metastatic testicular germ cell tumors at Bambino Gesù Children Hospital between January 1, 2010, and June 30, 2020, were included in the study. Patients were categorized into two groups: "primary testicular" and "burned out." All the patients were staged and treated according to the AIEOP-TCGM 2004 protocol. Results: Eleven patients were classified as "primary testicular," and five patients were classified as "burned out." "Burned-out" tumors were associated with the presence of systemic symptoms compared to "primary testicular" tumors (80 vs. 0%; p = 0.0027) and higher aFP, hCG, and LDH levels (p < 0.00001). The "burned-out" population had a statistically significant higher incidence of relevant toxicity than the "primary testicular" population (80 vs. 18%; p = 0.0357) and a worse outcome in terms of both mean overall survival (15 vs. 43 months; p = 0.0299) and mean event-free survival (12 vs. 38 months; p = 0.0164). Conclusion: "Burned-out" testicular tumors seem to be a well-distinct clinical entity with a high treatment-related toxicity and poor prognosis. Further studies are needed to clarify the "burned-out phenomenon" and to identify more effective therapeutic strategies for these patients.