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.

Respiratory and Musculoskeletal Long-Term Outcomes after Surgical Resection of Congenital Cystic Adenomatoid Malformation of the Lung in Newborns, Infants, and Toddlers.

INTRODUCTION: The long-term outcomes of children who underwent surgery for congenital cystic adenomatoid malformation of the lung (CCAML) are not well documented, particularly regarding orthopaedic and respiratory follow-up (FU). The aim of this study was to assess the long-term pulmonary and orthopaedic outcomes of surgically treated CCAML in newborns, infants, and toddlers. MATERIALS AND METHODS: Retrospective examination of prospectively recorded data of consecutive patients with CCAML who underwent surgery at our tertiary referral institution from January 2000 to December 2015 (newborns, infants, and toddlers). Clinical, radiological, and surgical data, as well as FU data were revised. A multidisciplinary team followed the patients after discharge at scheduled time points. RESULTS: Seventy-seven patients were included. After surgery, patients were followed for a median of 8 years (range: 1-19 years) until they reached a median age of 8 years (range: 2-19 years). Thirty patients (39%) developed wheezing and 21 (27%) had lower respiratory tract infections (LRTIs) within 4 years of age. However, more than 50% of patients with respiratory symptoms underwent complete remission in the following 4 years. Thirty-one patients (40%) developed at least one minimal musculoskeletal deformity. Eighteen (23%) had scoliosis, 17 (22%) thoracic asymmetry, 10 (12%) pectus excavatum, and 5 (6%) winged scapula. CONCLUSIONS: Patients operated for CCAML had good overall outcomes despite pulmonary symptoms and musculoskeletal sequelae. Even though these issues are frequently paucisymptomatic, trying to use less-invasive procedures (such as minimally axillary open "muscle-sparing" thoracotomy or thoracoscopy) may reduce this burden. A structured multidisciplinary FU is required.

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.

Where is the testis? The role of ultrasound and diagnostic laparoscopy for Crossed Testicular Ectopia (CTE): Case report and review of literature.

Crossed Testicular Ectopia (CTE) or transverse testicular ectopia is an anecdotic urogenital anomaly in which both testes are located on the same side, generally associated with a patent processus vaginalis (PPV). The condition can be detected by ultrasound. Nevertheless, the diagnosis is often missed preoperatively and CTE is recognized intraoperatively. Controversy exists regarding management and the role of diagnostic laparoscopy. The surgical technique depends on the anatomy of vas, vessels and testis found on surgical exploration. Diagnostic laparoscopy can be useful to rule out a vanishing testis and detect Müllerian remnants. We present the case of 8-months infant with no palpable testis on the right side and no signs of inguinal hernia, reporting the management and reviewing the scarce existing literature in this regarding. KEY WORDS: Crossed Testicular Ectopia, Laparoscopy, Ectopia, Testis, Transverse Testicular Ectopia, Urogenital Abnormalities.

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.

Preoperative Spinal Angiography for Thoracic Neuroblastoma: Impact of Identification of the Adamkiewicz Artery on Gross Total Resection and Neurological Sequelae.

BACKGROUND: Patients with thoracic neuroblastoma (TNB) are at high risk of postoperative neurologic complications due to iatrogenic lesions of the artery of Adamkiewicz (AKA). The role of performing a preoperative spinal angiography (POSA) in these patients must be clarified. The present study sought to further understand the relationship between POSA and TNB, as well as the effects of identifying the AKA on surgical excision and neurological consequences. METHODS: Data from patients with TNB who underwent POSA between November 2015 and February 2022 at our tertiary pediatric center were retrospectively analyzed. RESULTS: Six patients were identified, five of whom (83%) were considered eligible for surgical excision. Gross total resection (GTR) was achieved in three patients (60%), which included two patients with an AKA contralateral to the tumor, and one with an homolateral AKAl. After a median follow-up of 4.1 years from diagnosis, no patients developed neurological complications; five (83%) were alive and well, and one died from refractory recurrence. CONCLUSIONS: Among patients with TNB, POSA was useful for identifying the AKA and defining the optimal surgical strategy. POSA should be considered in the preoperative evaluation of TNB to increase the likelihood of GTR and reduce the threats of iatrogenic neurologic sequelae.

Isoperistaltic gastric tube for long gap esophageal atresia (LGEA) in newborn, infants, and toddlers: a case-control study from a tertiary center.

Background: Limited evidence exists about outcomes after gastric tube formation as "rescue" technique to avoid esophageal replacement in long gap esophageal atresia (LGEA). The last ERNICA Consensus Conference on the Management of LGEA has placed the techniques of gastric tubulization among the priorities for future research. Aims: Evaluate personal experience with Isoperistaltic Gastric Tube (IGT) and compare its outcomes with other more popular techniques for LGEA. Methods: A case-control study has been conducted. A retrospective monocentric analysis of LGEA patients (period: 2010-19) has been conducted in all consecutive IGT patients and each of these has been type matched with two cases of LGEA treated with other techniques. The follow-up (FU) considered was 24-months. Results: IGT and controls showed no statistically significant differences regarding preoperative variables like sex, gestational age, birth weight, syndromes, and EA type. However, IGT patients had a significantly longer esophageal GAP under boost pressure (4.5 vertebral bodies vs. 3.6, p = 0.019) at time of surgery. The analysis showed no statistical difference among the two groups about perioperative outcomes, ICU, or overall postoperative stay. No differences have been shown between IGT and controls during the follow-up regarding GERD, esophagitis, fundoplication, dysphagia, vocal cord paralysis, stenosis, and dilatations, auxologic data, need for anastomosis revision, oral aversion, and death. Conclusions: Isoperistaltic Gastric Tube is safe and effective even in LGEA patients with longer gaps, with good perioperative, post-operative and middle-term outcomes. This procedure may be considered as an alternative to avoid esophageal substitution when a primary anastomosis seems impossible for a residual gap after traction and growth techniques.

Pyelo-ureteral junction obstruction in poorly functioning kidneys: Does conservative management play a role in pediatric patients?

Background: Management of Pyelo-ureteral Junction Obstruction (PUJO) in poorly functioning kidneys in pediatric patients is still controversial, particularly regarding the role of conservative treatment. Aim: To evaluate and present the outcomes of internal diversion and follow-up results of a small series of pediatric patients with UPJO in poorly functioning kidneys. Study design: Retrospective review of 17 consecutive patients with unilateral PUJO in kidneys with Differential Renal Function (DRF) <20% undergoing temporary internal urinary diversion between 2009 and 2021 at a single tertiary center. DRF was reassessed after 1-3 months of diversion and subsequent management was conservative or surgical (pyeloplasty or nephrectomy) based on surgeon's and family's preferences without randomization. Results: After a trial of internal urinary diversion, 4/17 patients (23%) showed a DRF increase ≥5% (9%-12%), up to a maximum DRF of 28%, 3 underwent pyeloplasty, while 1 was managed conservatively. The remaining 13 patients showed no differential renal function improvement after diversion, and 7 were managed expectantly while 6 surgically (4 pyeloplasty, 2 nephrectomy). Overall, nine patients (53%) were managed surgically and 8 (47%) expectantly After a median (range) follow-up of 3.1 (0.3-7.9) years, no significant difference was observed between groups regarding symptoms (p = 0.205), need for further surgery (p = 1.000), and renal function (p = 1.000). Discussion: Although fraught with the limitation of a small sample size, this is the first study reporting on the conservative management of this controversial group of patients. Conclusion: In present pediatric series of pyelo-ureteral Junction obstruction in poorly functioning kidneys with differential renal function <20%, function recovery after a trial of internal urinary diversion was quite exceptional, and no difference was observed in outcome between patients managed surgically and conservatively after stent removal.

Chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis: Active disease or scarring? A case report and literature review.

The second most common abdominal tumor in children is Wilms' tumor, and the lung is where it most often metastasizes. The typical metastases are multiple, peripherally located, round, and variable-sized nodules. Atypical patterns are also possible and may create diagnostic challenges, especially in patients treated with chemotherapy. Among these, cavitating metastases are an anecdotal type of atypical secondary lung lesions. Here, we report a case of a chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis discovered during the follow-up for an anaplastic nephroblastoma in a 6-year-old girl. Furthermore, we conducted a review of the existing literature on this exceedingly rare radiological pattern to establish its best management.

Imaging strategy for focal nodular hyperplasia in children: long-term experience from two specialist European centres.

BACKGROUND: Focal nodular hyperplasia (FNH) in children is a rare but benign tumour, which must be differentiated from malignant entities to avoid unnecessary treatment, leading to potential morbidity. OBJECTIVES: To provide data on imaging findings of these lesions with a suggested algorithm for diagnosis, sampling and follow-up. MATERIALS AND METHODS: This retrospective review evaluated imaging of all patients diagnosed with FNH in two tertiary referral centres in Europe between 1975 and 2018. RESULTS: One hundred and four patients with 137 tumours were reviewed. The mean age at presentation was 8.2 years. The median tumour size was 5 cm (range: 0.3-29 cm). Multiple lesions were seen in 16.3% of patients. The male-to-female ratio was 1:2. CONCLUSION: FNH with typical features on imaging can be safely followed up once the diagnosis has been established. The use of contrast-enhanced ultrasound and magnetic resonance imaging allows accurate characterisation in most cases. Histological sampling is only advised when there is diagnostic doubt. Atypical arterial enhancement of FNH should prompt the search for a congenital portosystemic shunt.

Anorectal malformations (ARM) and VACTERL association and severity of congenital heart diseases (CHD): Experience of 396 consecutive patients in a tertiary center.

OBJECTIVE: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. STUDY DESIGN: All consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed. Prevalence and distribution of CHD in ARM patients were assessed. Patients were divided into groups depending on ARM severity and presence of VACTERL association. Pairwise comparison for CHD prevalence, severity, and timing at first cardiac surgery was performed between groups. RESULTS: Of 396 ARM patients identified, those with severe ARM showed a higher number of overall CHD compared to patients with non-severe ARM (36.7 % vs. 25.2 %, p = 0.032). VACTERL + patients had a significantly higher prevalence of CHD (73.4 % vs. 16.4 %; p <0.001) and major CHD (51.7 % vs. 26.9 %; p = 0.008) when compared with VACTERL-patients. Furthermore, VACTERL + patients underwent first cardiac surgery at a significantly younger age than VACTERL-patients (5.2 ± 15.2 months vs. 11.9 ± 6.3, p = 0.039). CONCLUSIONS: Patients with severe ARM had a higher number of CHD compared to patients with non-severe ARM. VATERL + patients had significantly more CHD and more severe CHD than VACTERL-patients. Early screening for CHD is strongly recommended in all newborns diagnosed with ARM before surgery. LEVEL OF EVIDENCE: III retrospective comparative study.

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.

OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome): Is it time for age-specific management?

BACKGROUND: OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome) is a rare Müllerian malformation. Usually, symptoms begin with worsening dysmenorrhea in post-menarche adolescents. The management in pre-menarche period is controversial and has only recently been subject of study. AIMS: To review the experience of a pediatric tertiary center and to propose an age-specific management protocol for patients diagnosed before menarche. METHODS: A retrospective cohort study (review of medical records - period 2009-2021). RESULTS: Twenty-eight patients were diagnosed (mean age 11.9 years), seven (25%) before menarche, one (3%) perinatally. One patient had Floating-Harbor syndrome. Twenty-three patients had ipsilateral renal agenesis, while five had a multicystic-dysplastic kidney. The contralateral kidney showed hypertrophy in 25 patients, pelvicalyceal ectasia in 8 and dysplasia in 1. Twenty-four patients were symptomatic. Three of the seven diagnosed prior to menarche had symptoms. All post-menarche diagnosed patients were symptomatic. Twenty-six patients underwent surgery (one-stage drainage, vaginal septal resection, and vaginoplasty). Asymptomatic pre-menarche patients were followed-up until surgery after menarche onset. No patient underwent surgery prior to menarche solely for OHVIRA diagnosis. At follow-up (median 3.5 years, 3 lost to follow-up), eighteen patients were asymptomatic, one developed endometriosis, one had impaired renal function, two needed reoperations. CONCLUSIONS: Pre-menarche OHVIRA patients, without symptoms, should undergo regular follow-up until the onset of menarche. Surgery must be considered in post-menarche or symptomatic patients. Post-operative, long-term follow-up is required, evaluating both renal and gynecological issues. LEVEL-OF-EVIDENCE: IV.

Conservative management of congenital bronchial atresia: The Bambino Gesù children's hospital experience.

INTRODUCTION: Congenital bronchial atresia (CBA) is a rare airway malformation. No management guidelines exist because of limited evidence: treatment, surgical or conservative, is based on consensus and opinion. OBJECTIVE: To review the experience of a pediatric tertiary center and provide additional data about nonsurgical management of CBA and its outcomes after a structured follow-up, and to help formulate appropriate evidence-based guidelines. METHODS: A retrospective analysis of clinical, radiological, surgical, and pathological data of all pediatric patients with suspicion of CBA referred to the surgical department of the Bambino Gesù children's hospital of Rome between December 2013 and 2019, along with a review of the literature. RESULTS: Among the 18 children initially included in the study, 2 were lost to follow-up after radiological diagnosis, 4 underwent surgery for radiological suspicion of other pulmonary malformations. The final population is composed of 12 conservatively managed patients. At the end of the follow-up (median: 29 months, range 3-61), 1 patient (8%) was symptomatic. CONCLUSION: Conservative management for CBA appears to be safe. Surgery should be reserved for patients with symptomatic or complicated cases.

Management of focal nodular hyperplasia of the liver: Experience of 50 pediatric patients in a tertiary center.

BACKGROUND: Focal nodular hyperplasia (FNH) is a rare benign hepatic lesion in children. No management guidelines for pediatric population exist because of limited evidence. OBJECTIVE: To review the experience of a large tertiary liver center, providing additional clinical data to help formulate management guidelines for FNH in the pediatric population. METHODS: We analyzed data of children <18 years diagnosed with FNH from 1996 to 2018 at our hospital, detailing management and long-term clinical outcome. RESULTS: 50 patients were identified. The median age was 10 years old (range 0.75-15.5 years old). The mean diameter of FNH was 5.9 cm (±3.1 cm). 10 patients had multiple lesions. First-line management: watchful waiting with serial checks (n = 37), surgery (n = 13). Of the watchful waiting patients, 10 required eventual second-line surgery. After a median follow-up of 4.7 years (range 0.5-20 years), 46 patients were asymptomatic, with no significant difference in clinical outcome (p = 0.962) between the two first-line management approaches. Lesions demonstrated growth in 13 cases: 5 of these required second-line surgery. In these patients, there was no significant difference in clinical outcome (p = 0.188) compared to nonoperative patients. Considering all surgically treated patients, there was no significant difference between first-line and second-line surgery for clinical outcome (p = 0.846), hospital stay (p = 0.410), complications (p = 0.510) and severe complications (p = 0.385). CONCLUSIONS: Our data support the hypothesis that watchful waiting is a safe initial approach to pediatric FNH management in patients with no major symptoms or complications. Surgery should be reserved for patients with diagnostic doubt, persistent symptoms and/or biological or significant anatomical abnormalities. FNH growth alone should not be considered as an indication for surgery. TYPE OF STUDY: Therapeutic study. LEVEL OF EVIDENCE: Level III.