Case Report: Double Pectus Up in severe pectus excavatum, the new frontier of modified taulinoplasty.

Introduction: Pectus excavatum, also known as "sunken chest" or "funnel chest," is a congenital condition where the sternum caves inward, creating a noticeable depression in the chest. This deformity can range from mild to severe cases, and can affect appearance and lung and heart function. Treatment options vary depending on the severity of the condition and associated symptoms. A case study was conducted on three patients suffering from severe forms of pectus excavatum using modified taulinoplasty with two Pectus Up bars. Case description: The patients were males, with an age of 15 years. Preoperatively, they underwent spirometry, an echocardiogram, and allergy tests. The procedure involved inserting two Pectus Up bars into the chest wall at the major sternal defect, allowing the implant to remain completely invisible. The procedure involved placing the sternal plate at the deepest point and anchored to the sternum with five self-tapping screws. The chest plate was then fixed to the bar using two screws. Discussion: The use of Pectus Up was first reported in 2016 and has been a subject of scientific discussion and research. The double Pectus Up technique offers improved correction, increased stability, and reduced complications. However, it also presents challenges such as increased technical complexity and potential for prolonged operative times. Patient outcomes showed promising results in terms of short-term correction and long-term stability. The use of a double bar technique in the modified Taulinoplasty procedure is a key area of ongoing clinical research and innovation in pectus excavatum repair. Further studies will be needed, including more institutions that use this technique to validate our initial experience.

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: 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.

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.

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.

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.

Invasive pulmonary aspergillosis in a haematopoietic stem cell transplant recipient with sickle cell disease: a successful treatment.

Sickle Cell Anaemia (SCA) is the most common inherited blood disorder and is associated with severe morbidity and decreased survival. Allogeneic Haematopoietic Stem Cell Transplantation (HSCT) is the only curative approach. Nevertheless the decision to perform a bone marrow transplant includes the risk of major complications and transplant-related mortality. Infections represent the leading cause of death in SCA patients undergoing HSCT. Invasive Pulmonary Aspergillosis (IPA) is a devastating opportunistic infection and remains a significant cause of morbidity and mortality in HSCT recipients. Data regarding IPA in the setting of SCA are lacking. In the present report, we describe a patient with SCA, who developed IPA after allogeneic bone marrow transplant. The fungal infection was treated by systemic antifungal therapy in addition to surgery, despite mild chronic graft versus host disease (GVHD) and continuing immunosuppressive therapy. This case shows that IPA occurring in bone marrow recipients with SCA can be successfully treated.

Penile repair in patients with epispadias-exstrophy complex-can we prevent resultant hypospadias?

PURPOSE: Male genitalia reconstruction in patients with exstrophy-epispadias complex often leads to secondary hypospadias, thus requiring additional surgery and resulting in possible complications. We describe a technique of male epispadias repair to prevent resultant hypospadias. MATERIALS AND METHODS: The complete penile disassembly technique is modified by multiple Z-plasties, which enable creation of a funnel-like bladder neck and urethral plate lengthening. A total of 29 patients 2 months to 12 years old presenting with pure epispadias and bladder exstrophy were treated with this technique between 2004 and 2011. These patients were compared to 19 patients 1 day to 11 years old with exstrophy-epispadias treated with the standard penile disassembly technique between 2000 and 2004. RESULTS: Outcome measures were urethral orifice position, penile length and complications. There was no statistically significant difference between the 2 groups regarding penile length or complications. In the study group the urethra reached the tip of the glans in all patients, whereas the creation of intentional hypospadias was necessary in 6 patients (31.5%) in the control group (p <0.05). CONCLUSIONS: The modified technique was effective in obtaining appropriate meatal location without decreasing the penile length. The complication rate was not changed. Creation of resultant hypospadias at genitalia reconstruction in male epispadias should strongly be prevented.