Fire in the Operating Room During Hypospadias Repair

Fire in the operating room (OR) is a very distressful and shocking occurrence with potential dramatic consequences. Despite safety rules and rigorous recommendations, such unintentional events do occur every so often. Notably, the vast majority of cases have been reported in the adult population, with very few pediatric cases described to date. Herein, we report on a 16-month-old boy undergoing reconstructive surgery for penoscrotal hypospadias, who experienced an OR fire most likely related to the use of alcohol-based solution ignited by monopolar electrocautery

Videothoracoscopic management of hemoptysis due to anomalous bronchial vessel treated with multiple bronchial artery embolizations: a case report

IntroductionHemoptysis is an alarming clinical presentation caused by a vast number of primitive conditions (infectious, malignancies, malformations, vasculitis). However, at the root of hemoptysis, there is always a “noxa patogena” altering vessel structure, usually bronchial arteries, which are characterized by high pressure. Bronchial artery embolization (BAE) is the first-line treatment for hemoptysis for its technical and clinical success, although the long-term overall outcome is not equally adequate.Case reportA 12-year-old boy was referred to our hospital for massive hemoptysis after a history of recurrent episodes since the age of 3. The patient had been diagnosed with bilateral and widespread bronchial artery hypertrophy at another hospital and treated with several BAE procedures. We performed BAE to stabilize the child as well as an angio-CT scan, which confirmed the presence of the recently placed coil to embolize a hypertrophic bronchial arteriosus branch originating from the left thyrocervical trunk and directed to the right lower lobe. Results of previous embolization (metal coils) were found at the origin of the right inferior thyroid artery and the right costo-cervical trunk. After 21 months since his first admission to our hospital, the patient was transferred by air ambulance for a massive hemoptysis recurrence. Further BAE of the previously coiled vessel coming from the right succlavia (and right inferior thyroid artery) was impossible to perform due to the presence of the coils positioned in the past. A thoracoscopic approach was chosen: the previously identified anomalous vessel was isolated and ligated using double metal clips, two on both the proximal and distal sides. Accurate exploration of the thoracic cavity was accomplished, verifying the absence of collateral vessels coming from the diaphragmatic side. The patient was discharged in four days in good clinical.DiscussionAlthough bare-minimum invasive embolism (BAE) is still the gold standard for treatment, there are situations when it may not produce the desired clinical outcome and increase the risk of rebleeding. In these situations, minimally invasive surgical procedures using a videothoracoscopic approach can be beneficial if there is a suspicion of an aberrant vessel on a DSA or CT scan

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

IntroductionMidgut 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 reportAn 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.DiscussionThis 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

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

Background and objectivesMarfan 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.MethodsRetrospective 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).ResultsSixty-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.ConclusionsTreatment 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

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

BackgroundDescribed 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 methodsA retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed.ResultsWe 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.ConclusionsWe 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

Rheumatoid Arthritis from Easy to Complex Disease: From the "2022 GISEA International Symposium"

: Rheumatoid Arthritis (RA) is a systemic disease with many different clinical phenotypes. RA could be classified according to disease duration, seropositivity for rheumatoid factor (RF) and/or anti-citrullinated protein antibodies (ACPA), joint subtype, clinical behaviourbehavior and many other subgroups. In this review, we summarize and discuss the multifaceted aspects of RA, focusing on the relationship between autoimmunity status and clinical outcome, achievement of remission and influence on treatment response, from the 2022 International GISEA/OEG Symposium

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

IntroductionInternational 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 methodThe 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.ResultsThe 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).ConclusionIn 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