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.

The Intraperitoneal Use of Cephazolin: A Novelty in the Prevention of Intra-abdominal Abscess after Laparoscopic Appendectomy in Children.

BACKGROUND: Laparoscopic appendectomy followed by postoperative intravenous (IV) antibiotics is the standard of care for acute appendicitis and postoperative prevention of intra-abdominal abscesses. The aim of or study was to determine if intraperitoneal irrigation with antibiotics could help prevent intra-abdominal abscess formation after laparoscopic appendectomy for complicated appendicitis in pediatric patients. METHODS: A retrospective study was conducted on consecutive pediatric patients with acute appendicitis who had appendectomy in our Pediatric Surgery Department between August 2020 and February 2022. We compared two groups with similar age and symptoms. The first group (A) was treated with the normal standard of care, i.e., laparoscopic appendectomy and postoperative IV antibiotic therapy. For the second group (B) intraperitoneal cefazoline irrigation was added at the end of the laparoscopic procedure. Postoperative intra-abdominal abscess was diagnosed with ultrasound examination, performed after clinical suspicion/abnormal blood test results. RESULTS: One hundred sixty patients (males:females 109:51; median age 10.5 years [range 3-17 years]) who had laparosopic appendectomy for complicated appendicitis were included, 82 in group A and 78 in group B. In the first 7 days after surgery, 18 patients in group and 5 in group B developed an intra-abdominal abscess (p < 0.005). Drains were positioned in 38 patients in group A vs. 9 in group B. One patient in group A had a different complication which was infection of the surgical incision. CONCLUSIONS: Intraperitoneal cefazoline irrigation at the end of the laparoscopic appendectomy in pediatric patients significantly reduces the formation of intra-abdominal abscesses.

Case Report: An unusual case of wide ileoileal intussusception associated with intestinal volvulus in a 8-months-old infant.

Introduction: Midgut volvulus and intussusception are prevalent paediatric abdominal emergencies. To the best of our knowledge, this is the first reported case of a connection between intestinal volvulus and a massive intussusception. Case report: An 8-month-old male infant was brought to the emergency room with a history of abdominal pain and vomiting for <24 h. On physical examination, the child appeared restless and was found to have a circumferential hard mass of approximately 4 cm in diameter in the epigastric region. Upon admission, laboratory results showed a C-reactive protein level of 0.4 mg/dl, LDH level of 351 U/L, mild leukocytosis with a white blood cell count of 12 × 103 /µl, and 67% neutrophils. A physical exam was significant for abdominal distention, hyperresonance in percussion, and a palpable, painful epigastric mass. The findings of the operation included a dilated and ischemic intestinal loop, approximately 25 cm from the ileocecal valve, twisted upon itself for three turns. After de-rotation, an extensive occluding ileo-ileal invagination with an ischemic intestinal loop was identified, and a length of approximately 55-60 cm of the distal ileum, including the ischemic segment, was resected. Discussion: This is the first reported case of a connection between intestinal volvulus and a massive intussusception. Currently, only two reported cases describe the connection between volvulus and intussusception, which are insufficient to establish a direct link between the two clinical conditions.

Case Report: The acute appendicitis and incarcerated umbilical hernia: a rare association.

Introduction: One of the most prevalent congenital wall abnormalities in children, umbilical hernias are often linked to premature or small-for-gestational-age babies. In cases of intestinal malrotation or if the cecum is very movable, generalized peritonitis may facilitate the imprisonment of these hernias. Case report: We described a case of a 4-month-old baby who had a prior reducible umbilical hernia with a history of fever, vomiting, poor appetite, and constipation for around 48 h. The patient experienced significant intestinal bloating, vomiting, irreducibility of the umbilical hernia, skin pigmentation, and erythema at the umbilical site within 2 days after hospitalization. When there was no free abdominal air, a direct abdominal x-ray revealed evidence of hydro-gas stasis and various hydro-aerial levels that were pertinent to the ileum. In order to reduce the hernia, the patient had an emergency surgical treatment where the hernia sac was isolated and released from the ileal loop, which was securely attached to a fibrin plate. When the herniary sac was opened, a gangrenous and perforated appendix was found inside. On the seventh postoperative day, the patient was released from the hospital after an uncomplicated postoperative stay. Conclusion: Our patient's clinical presentation is similar to that of only one other case report involving a 25-day-old male patient. Our case presented with a variant of the clinical symptoms of the previously described umbilical hernia, which became unfixable and strangulated as a result of appendix inflammation. The appendix was discovered inside the hernia sac during surgery.

Femoral hernia in pediatric population: a diagnostic and surgical challenge.

PURPOSE: Femoral hernia (FH) is a rare and often misdiagnosed pathology in pediatric population. The aim of our study was to describe the experience of a Tertiary Center in children with FH, underlying diagnostic and surgical details that could improve its management. METHODS: A retrospective study of pediatric patients who underwent FH repair from January 2010 to June 2023 at our Institution was performed. RESULTS: In the analyzed period, 31 patients underwent surgical procedure for FH at our institution, of whom 16 (51.6%) were female. The mean age at time of surgery was 5.8 years (range 0.5-17.1 years). The rate of pre-operative misdiagnosis was 35.5% and open approach was adopted in all cases (16.1% with mesh application). Only two patients (6.4%) experienced surgical complications: one recurrence (repaired six months later) and one post-operative hematoma (treated successfully with conservative method). CONCLUSION: Due to the high rate of misdiagnosis, the variety of surgical approaches proposed, and the potential for intraoperative complications, FH poses a challenge for pediatric surgeons and urologists, as confirmed by the literature. It is essential to underline the importance of a proper clinical examination in order to correctly diagnose FH, make the best surgical plan for the patient and prevent post-operative complications.

Conservative and operative management of spontaneous pneumothorax in children and adolescents: Are we abusing of CT?

BACKGROUND: No age-specific pediatric guidelines exist for the management of spontaneous pneumothorax (SP) in children and adolescents. Treatment remains heterogeneous and center dependent. The role of computed tomography (CT) has yet to be defined. AIMS: Review the management of SP in children and adolescents, with emphasis on conservative management and role of CT. METHODS: Retrospective analysis of 61 consecutive patients with SP at single tertiary center. Clinical, radiological, surgical data, follow-up, and outcomes were revised. RESULTS: First-line management was conservative for 32 (53%) patients and operative for 29 (47%). Asymptomatic/paucisymptomatic patients managed conservatively experienced less first-line treatment failure. Furthermore, the patients needing at least a chest drain or surgery during the follow-up were significantly lower in the conservative group. Conservative and operative patients showed no significant differences regarding ipsilateral recurrences or contralateral occurrences. Of the 61 overall CTs performed, 14 (23%) had an impact on management. Forty-three (70%) patients had at least a CT, in 22 (51%) the CT was positive for blebs. For 10 of these patients (45%) the presence of blebs had an impact on management. Patients with and without blebs showed no differences regarding ipsilateral recurrence, contralateral occurrences, or the need for at least a chest drain or surgery during the follow-up. CONCLUSIONS: First-line conservative management had a significantly shorter hospitalization and better outcome, with a similar incidence of recurrences. The presence of blebs at CT does not predict the risk of recurrence. The CT scan should be reserved for a small number of selected patients who have post-VATS refractory or recurrent pneumothorax.

Benefits of using digital thoracic drainage systems for post-operative treatment in pediatric populations: personal experience and review of literature.

Introduction: The digital chest drainage monitoring system (Medela Thopaz+), unlike analogical systems, reliably regulates the pressure applied to the patient's chest and digitally and silently monitors critical therapeutic indicators (volume of fluid and/or drained air). Its use in adulthood has been widely described, but there is still little experience in the pediatric field. The aim of this study is to test this new device in the pediatric population. Materials and methods: We conducted a retrospective study of 160 patients undergoing chest surgery at our Hospital. These patients were divided into 82 treated with the Thopaz system in the period from January 2021 to April 2023 and 78 in whom Pleurevac, had been used in the time period from January 2020 to April 2023. Results: The average age of patients was 10.45 years (range: 3.1-17.2) for the Thopaz Group and 10.71 years for Pleurevac Group. The groups were homogeneus also by weight and type of intervention. The device was held in place for 10.64 days (mean) for Thopaz Group, compared to 16.87 days (mean) for Pleurevac Group (p < 0.05). The median number of postoperative x-rays before the closure of the chest tube was 4.29 in the digital drainage group compared to 8.41 in the traditional draining group (p < 0.05). Conclusions: The digital chest monitoring device provides objective measurement, allows for rapid patient mobilization (with good pain control and increased compliance). In addition, the use of Thopaz in the paediatric population seems to be safe (there is no statistically significant difference in terms of complications such as prolonged air leaks and pneumothorax after the chest tube closure) and potentially beneficial.

Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques.

Background: Described for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device for progressive internal distraction of the sternum (PIDS) in patients with symptomatic JS. In addition, we reviewed the contemporary English literature on existing surgical techniques for treating children with congenital JS. Material and methods: A retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed. Results: We presented two patients with JS who underwent surgery using an internal sternal distractor, a Zurich II Micro Zurich Modular Distractor, placed at the corpus of the sternum among the divided halves. Conclusions: We obtained promising results regarding the safety and effectiveness of this less-invasive device for PIDS in patients with symptomatic JS. Further studies on long-term outcomes are needed to validate these findings.

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.

Aggressive approach for spontaneous pneumothorax treatment in children with Marfan syndrome?

Background and objectives: Marfan syndrome (MS) is a systemic disease of connective tissues consisting of a variable combination of anomalies. These patients have an increased risk of spontaneous pneumothorax (SP). However, there is a scarcity of pediatric literature on management, and no specific guidelines exist. Our aim was to analyze the management of spontaneous pneumothorax in children and adolescents with Marfan syndrome, comparing syndromic and non-syndromic patients. Methods: Retrospective analysis of pediatric patients (18 years) with SP diagnosed at our tertiary pediatric hospital (January 10-June 22), with special emphasis on diagnosis, treatment, and follow-up (FU). Results: Sixty-six patients with SP were identified, with nine (13%) having MS. In terms of baseline, there were no significant differences between the groups (age, sex, asthma, symptoms, and side, first-line treatment and hospitalization length). Overall, Marfan patients had significantly more first-line treatment failures requiring additional surgery, as well as more contralateral occurrences and the need for surgery/chest drain during the follow-up. Instead, conservative management resulted in significantly more ipsilateral recurrences and the need for surgery/chest drain in Marfan patients than controls during the follow-up. Conclusions: Treatment failure, contralateral occurrence, ipsilateral recurrence, and the need for surgery/chest drain during follow-up make management of patients with Marfan syndrome and spontaneous pneumothorax more difficult. In patients with a diagnosed MS a more aggressive first-line management should be considered, bearing in mind the higher risks of this population.

Uterine leiomyoma in pediatric population: A case report and review of the literature.

Uterine leiomyomas are rare in the pediatric population with less than 20 cases in adolescences reported in the literature. Furthermore, these masses represent a common presentation of gynecologic tumors with increasing age. We report a case of a 14-year-old female who presented with abdominal pain and increasing abdominal girth. Workup with ultrasound, CT and MRI demonstrated a large pelvic mass. Complete resection by median laparotomy was performed. The mass weighed 5,596 g and was 29.5 cm × 27 cm × 19 cm; the pathological examination confirmed the hypothesis of leiomyoma. The patient remained asymptomatic at 3 months follow up.

Vascular Access in Pediatric Oncology and Hematology: State of the Art.

Management and successful use of vascular access are critical issues in pediatric patients affected by malignancies. Prolonged course of disease, complex and various treatment protocols require long-lasting vascular access providing adequate tools to administrate those therapies and to collect routine blood sampling without painful and repeated venipuncture. For these reasons, central venous catheters are currently an important component in pediatric onco-hematological care, with a direct influence on outcome. Indeed, there are peculiar issues (techniques of insertion, management, complications etc.) which must be well-known in order to improve the outcome and the quality of life of children with cancer.

Case Report: Massive Intestinal Pneumatosis and Pneumoretroperitoneum Following Hematopoietic Stem Cell Transplantation in a 2-Year-Old Child.

A 2-year-old boy with severe combined immunodeficiency (SCID) developed intestinal graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation (HSCT), associated with massive intestinal pneumatosis (IP), pneumoretroperitoneum (PRP), and pneumomediastinum. His fair clinical conditions allowed conservative management, with progressive normalization of imaging findings. The patient did not require surgery and is alive and in good clinical conditions at follow-up. In children with GVHD-related IP but good clinical conditions and no signs of peritonitis, IP is not a mandatory indication for surgery, despite its potentially striking imaging features. Conservative management, with intestinal rest, decompression, and antibiotics, often allows regression of the clinical picture.

Appendicitis in COVID19 Pandemic Era. Early experience of an International Referral Center of Pediatric Surgery.

AIM: We describe treatments of acute appendicitis at "Bambino Gesù" Children's Hospital during the peak of the Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) pandemic in Italy. MATERIAL AND METHODS: From March 1st to May 31st, all suspected appendicitis admitted to hospital entered this study. Following Institutional COVID19-protocol, between March 1st-21st, only patients with respiratory symptoms and/or history of recent travel to risk areas received nasopharyngeal swab. From March 22nd to May 31st, protocol was adapted to worsening epidemic conditions and a pre-triage area has been arranged to accommodate all patients undergoing the swab. RESULTS: 14 out of 53 patients were hospitalized between march 1st-21st, 39 from march 22nd to may 31st. swab was performed in 2 patient of first group and in all of second. DISCUSSION: During the study period, no covid19-contagion occurred in hospital staff by covid19-patients. CONCLUSION: Our covid19-protocol protected staff and patients allowing the maintenance of our standard of treatment. KEY WORDS: Appendicitis, Children, Covid19, Sars-cov-2.

Gastric cancer, inflammatory bowel disease and polyautoimmunity in a 17-year-old boy: CTLA-4 deficiency successfully treated with Abatacept.

Gut involvement is frequent in immunologic disorders, especially with inflammatory manifestations but also with cancer. In the last years, advances in functional and genetic testing have improved the diagnostic and therapeutic approach to immune dysregulation syndromes. CTLA-4 deficiency is a rare disease with variable phenotype, ranging from absence of symptoms to severe multisystem manifestations and complications. We describe a rare case of CTLA-4 deficiency in a boy with gastric cancer, very early onset inflammatory bowel disease and polyautoimmunity, the second-ever reported in the literature with the same characteristics. A 17-year-old boy was referred to Bambino Gesù Children's Hospital of Rome, a tertiary care center, for a gastric mass and a long-term history of very early onset inflammatory bowel disease, diabetes mellitus type 1, polyarthritis and psoriasis. Histology of gastric biopsies revealed the presence of neoplastic signet ring cells. Imaging staging showed localized cancer; therefore, the patient underwent subtotal gastrectomy with termino-lateral gastro-jejunal anastomosis. Immunological work up and genetic testing by next-generation sequencing panels for primary immunodeficiencies led to the diagnosis of CTLA-4 deficiency. Good disease control was obtained with the administration of Abatacept. The patient experienced an asymptomatic SARS-CoV-2 infection without any concern. Eighteen months after treatment initiation, the patient is alive and well. Immunologic and genetic testing, such as next-generation sequencing, should always be part of the diagnostic approach to patients with complex immune dysregulation syndrome, severe clinical course, poor response to treatments or cancer. The early recognition of the monogenic disease is the key for disease management and targeted therapy.

Electrocautery only for hyoid bone removal in pediatric thyroglossal duct cyst excision.

AIM: Surgical excision of hyoid bone is the mainstay of thyroglossal duct cyst removal, to prevent recurrences. Bone excision by cut - bone forceps may prove inaccurate and somewhat dangerous. Therefore, use of alternative methods has been advocated. MATERIAL AND METHODS: The proposal of the study is to cut the body of the hyoid bone with the only electric scalpel. Surgical casenotes of 57 pediatric patients operated by our Institution with modified Sistrunk technique and complete dissection of the entire body of hyoid bone and its removal at the cartilage junction with lateral horn with monopolar cauterization were reviewed. RESULTS: The population of our study had a mean age of 59.86 ± 29.57 months; 6/57 patients (10.5%) were under 2 years of age. Mean follow up was 55,04 ± 29,08 months ranging 2 to 115 months. In any patients post-operative bleeding was observed. Mean surgical time was 57,02 ± 11,77 minutes ranging from 35 to 125 minutes; 6 cases required postoperative drain. In 3 patients an immediate local edema was observed and in another one a partial wound dehiscence occurred, no late complications were observed. Cyst-recurrence requiring re-do surgery occurred in 5 patients (8.8%). CONCLUSIONS: The complete hyoid bone section with electric scalpel only ensures the ideal access to the posterior hyoid space and allows a greater and more symmetrical access to the proximal portion of thyroglossal duct in children with still negligible complication and recurrence rate. KEY WORDS: Modified Sistrunk technique, Sistrunk procedure, Thyroglossal duct cyst.

Caval replacement strategy in pediatric retroperitoneal tumors encasing the vena cava: a single-center experience and review of literature.

BACKGROUND: Complete encasement of the inferior vena cava by retroperitoneal tumors is rare. Although replacement of the vena cava has been considered for various conditions in adults, it is rarely used in children except for challenging resections and as a last chance approach - often aiming more at debulking than cure. MATERIALS AND METHODS: From January 2009 to February 2017, 4 patients (2 adrenal neuroblastomas, 1 renal cell carcinoma, 1 infantile fibrosarcoma) underwent elective en-bloc resection of tumor and of the infrahepatic portion of the inferior vena cava (IVC), with planned IVC prosthetic replacement. In three cases a portion of the left renal vein had to be resected as well, with the vein reanastomosed onto the prosthesis, and a concomitant auto-transplantation of the right kidney was associated in one neuroblastoma patient. RESULTS: All patients had an uncomplicated postoperative course. In one patient, the prosthetic conduit is patent at long-term (43 months), while the middle portion of the prosthesis did eventually thrombose at mid-term after surgery in the three others - with no related symptoms. Interestingly, all renal venous reconstructions remain patent. Three patients (2 neuroblastomas and 1 infantile fibrosarcoma) are alive and disease-free at 43, 74 and 108 months after surgery, respectively. One patient with renal cell carcinoma died of recurrence of the disease 21 months after surgery. CONCLUSION: Resection and reconstruction of the vena cava, including the renal vein portion, can be considered and planned electively in case of tumoral encasement. This strategy is associated with good tolerance of the operation, low morbidity and satisfactory long-term function, even in cases with progressive and/or secondary partial thrombosis. LEVEL OF EVIDENCE: IV.