Vascular malformations are congenital anomalies that may affect any part of the body. These malformations may be distinguished as macrocystic (characterised by large cystic cavities) or microcystic (characterised by small cystic cavities). The treatment of vascular malformations using the combined approach of Bleomycin and reversible electropora- tion is called BEST (Bleomycin ElectroScleroTherapy). BEST with intralesional Bleomycin infusion may be performed for macrocystic malformations. For microcystic malformations, however, BEST is performed with intravenous Bleomycin infusion. The technique, in both approaches, may be considered a feasible and effective therapy for the treatment of this type of vascular malformations.
Background: Adnexal torsion is an uncommon cause of abdominal pain, and its clinical presentation in children is nonspecific. In some studies, serum D-dimer showed promise as a biochemical marker. The aim of this multicenter prospective observational study was to evaluate the sensitivity and specificity of preoperative serum D-dimer levels for diagnosing adnexal torsion in children and adolescents. Methods: Female patients aged <18 years presenting to the emergency departments of participating centers with symptoms suggestive of adnexal torsion between January 2022 and December 2024 were invited to participate in the study. Preoperative serum D-dimer levels were measured in all patients undergoing surgical exploration. Patients’ characteristics were examined using descriptive and inferential statistics, and the accuracy of preoperative serum D-dimer levels for diagnosing adnexal torsion was assessed using univariate logistic regression and a receiver operating characteristic curve. Results: Twenty-eight patients aged 4–17 years were enrolled. Adnexal torsion was found in 17 patients, on the left side in 4 (23.53%) and on the right side in 13 (76.47%). Almost all patients were treated laparoscopically, and no postoperative complications occurred. Preoperative serum D-dimer levels were higher among patients with adnexal torsion than among those without. The univariate model of serum D-dimer levels had an odds ratio of 1, a sensitivity of 0.77, and a specificity of 0.82 (p = 0.27). Conclusions: No direct association was observed between preoperative serum D-dimer levels and adnexal torsion. Nonetheless, the sensitivity and specificity suggest the possible utility of including serum D-dimer levels in multi-marker diagnostic models to complement rather than replace existing tools.
IntroductionGastroesophageal reflux disease (GERD) is a common pediatric condition that can significantly impair quality of life and lead to severe complications in medically fragile patients. Although conservative and pharmacological therapies are effective in most cases, some children require surgical intervention, which remains associated with non-negligible postoperative morbidity and the need for reoperation. This study aimed to evaluate the surgical management of pediatric gastrointestinal reflux in a tertiary referral center, focusing on surgical approaches, operative outcomes, postoperative complications, and the need for reintervention. In addition, factors associated with unfavorable surgical outcomes were investigated in order to identify potential areas for improvement in the management of medically fragile pediatric patients.MethodsA retrospective observational study was conducted on pediatric patients undergoing surgical treatment for GERD, dysphagia, or both conditions at a tertiary pediatric center between December 2016 and December 2025. Demographic characteristics, comorbidities, nutritional status, ASA score, surgical procedures, operative times, postoperative complications, and reinterventions were analyzed. Associations between baseline risk factors and postoperative outcomes were explored using Fisher's exact test. Statistical significance was set at p < 0.05.ResultsA total of 67 patients were included in this study. The study population was characterized by a high prevalence of neurological impairment (79.1%), severe malnutrition (59.7% with weight percentile <3rd percentile), and elevated anesthesiologic risk (78.8% classified as ASA 3–4). Postoperative complications occurred more commonly among patients with severe malnutrition and high ASA scores.ConclusionsPediatric patients undergoing surgical treatment for GERD represent a highly complex and medically fragile population. Although fundoplication remains an effective therapeutic option, postoperative complications and reinterventions continue to occur, particularly among patients with severe malnutrition and elevated anesthesiologic risk. The results of this study support the need for less invasive therapeutic strategies and further research into innovative approaches aimed at reducing the surgical burden in this high-risk population. Emerging evidence regarding autologous micro-fragmented adipose tissue therapies may offer a promising regenerative approach to reinforce lower esophageal sphincter function while minimizing surgical and anesthetic burden.
Background: The clinical presentation of adnexal torsion in children is nonspecific. In some studies, serum D-dimer showed promise as a biochemical marker. Thus, this multi-center prospective observational study aimed to evaluate the sensitivity and specificity of preoperative serum D-dimer levels for diagnosing adnexal torsion in children and adolescents. Methods: This study prospectively enrolled female patients aged < 18 years presenting to the emergency departments of participating centers with symptoms suggestive of adnexal torsion between January 2022 and December 2024. Preoperative serum D-dimer levels were measured for all patients undergoing surgical exploration. Patients’ characteristics were examined using descriptive and inferential statistics, and the accuracy of preoperative serum D-dimer levels for diagnosing adnexal torsion was assessed using univariate logistic regression and a receiver operating characteristic curve. Results: This study enrolled 28 patients aged 4–17 years. Adnexal torsion was found in 17 patients, on the left side in 4 (23.53%) and on the right side in 13 (76.47%). Almost all patients were treated laparoscopically, and no postoperative complications occurred. Pre-operative serum D-dimer levels were higher among patients with adnexal torsion than among those without. The univariate model of serum D-dimer levels had an odds ratio of 1, a sensitivity of 0.77, and a specificity of 0.82 (p = 0.27). Conclusions: No direct association was observed between preoperative serum D-dimer levels and adnexal torsion. Nonetheless, the sensitivity and specificity suggest the possible utility of including serum D-dimer levels in multi-marker diagnostic models to complement rather than replace existing tools.
Congenital pyloric atresia (CPA) is a rare cause of neonatal gastric outlet obstruction. Although usually isolated, CPA may coexist with additional gastrointestinal atresias. Its association with distal duodenal atresia (DA) is particularly relevant because the segment between the two obstructions may become a closed duodenal loop into which biliary and pancreatic secretions continue to drain. We report the case of a male newborn delivered at 35 + 3 weeks of gestation after prenatal detection of severe polyhydramnios, recurrent bowel dilatation, abdominal effusion, and a cystic abdominal lesion. Postnatal radiography did not show the classic double-bubble sign. Exploratory laparotomy revealed CPA, marked dilatation of the second duodenal portion, distal DA, extensive adhesions, and jejunal perforations. Surgical management included adhesiolysis, reduction duodenoplasty, gastrointestinal continuity reconstruction, and jejunal perforations repair. The postoperative course was complicated by hemorrhagic–hypovolemic shock, coagulopathy, liver dysfunction, and the need for a second surgery due to increasing abdominal free fluid and suspected pneumoperitoneum, followed by progressive recovery. Follow-up demonstrated adequate gastric emptying and duodenal transit, satisfactory growth, and age-appropriate neurodevelopment. A focused narrative review on concomitant CPA and DA was also conducted. Concomitant CPA and distal DA should be considered when prenatal imaging shows a cystic abdominal lesion associated with polyhydramnios and ascites. Recognizing closed duodenal loop syndrome may improve prenatal counseling, atypical imaging interpretation, and surgical planning.
Introduction:The diagnosis of adnexal torsion in children remains nonspecific. In some studies, D-dimer was shown to be a promising biochemical marker, especially in animals. This analysis aimed to preliminarily evaluate the specificity and sensitivity of D-dimer levels in predicting the diagnosis of adnexal torsion at our institution. Methods:All female patients who presented at the emergency department from January 2022 to December 2024 with symptoms suggestive of adnexal torsion were prospectively enrolled in the study. Preoperative D-dimer levels were obtained for all patients undergoing surgical exploration. Descriptive analysis was computed, and a receiver operating characteristic curve was constructed via univariate logistic regression. Results:Of the 27 eligible patients, 15 (aged 7-17 years) participated in the study. Torsion was found on the left side in six patients (40%) and on the right side in nine patients (60%). Almost all patients (80%) were treated laparoscopically. No postoperative complications were encountered. None of the examined variables were significantly correlated with adnexal torsion. Notably, D-dimer levels were higher in patients with adnexal torsion (odds ratio = 1, p = 0.29), with a sensitivity of 0.86 and a specificity of 0.75. Conclusion:No association was observed between D-dimer levels and ovarian torsion in our cohort. Nonetheless, the sensitivity and specificity results lead us to believe that preoperative D-dimer levels could help surgeons in more accurately predicting the possibility of an adnexal torsion in children and adolescents, but this should not replace imaging studies and diagnostic laparoscopy especially in doubtful cases.
Acute appendicitis is one of the most common surgical conditions in pediatric and general surgery. The purpose of this study was to retrospectively investigate the management strategies of the appendiceal stump during transumbilical laparoscopic-assisted appendectomy for acute appendicitis in terms of surgical time, hospital length of stay, and postoperative complications. The records of patients < 18 years of age who were treated for acute appendicitis at our Pediatric Surgery Unit from December 2018 to January 2024 were retrospectively reviewed. Data were extrapolated from the departmental database, and clinical notes were reviewed. The records of 278 patients who underwent appendectomy within a 5-year period at our institution were essentially used to compare appendiceal stump inversion and simple ligation. Appendiceal stump invagination was performed in 208 patients (74.8
Cholelithiasis is increasing in the pediatric population, and there are currently no guidelines for the management of asymptomatic patients with both cholelithiasis and a predisposing condition. This study seeks to highlight situations where prophylactic cholecystectomy may be desirable. We retrospectively reviewed the medical records of children who underwent elective laparoscopic cholecystectomy between October 2011 and September 2022. Thirty-two patients were included in the study. Five different groups of patients were identified based on associated pathologies. Twenty-six patients were symptomatic (81.25%), and six were asymptomatic (18.75%). All patients underwent a laparoscopic cholecystectomy. Hematologic and cystic fibrosis patients with asymptomatic cholelithiasis had a shorter length of hospital stay than patients with the same condition who progressed from asymptomatic to symptomatic gallstone disease. Consequently, patients with associated diseases (particularly hematologic diseases and cystic fibrosis) may benefit from early laparoscopic cholecystectomy, which could reduce the probability of surgical difficulties and shorten the length of hospital stay.
Introduction: Lipomas are among the most encountered neoplasms in clinical practice, occurring mainly in adults between the fourth and sixth decades of life. Deep-seated lipomas in children are found in the thorax, chest wall, mediastinum, pleura, pelvis, retroperitoneum, and paratesticular area. Herein, we present a case of a three-year-old child with a giant mesenteric lipoma, along with a review of the literature on mesenteric lipomas in childhood. Case presentation: A three-year-old male toddler was referred to our hospital for severe, intermittent abdominal pain. Imaging studies at admission revealed a fat lesion occupying most of the peritoneal cavity and dislocating adjacent structures. An urgent laparotomy was performed. A giant lipoma arising from the mesentery and leading to the torsion of the mesenteric radix was confirmed and completely excised alongside an adherent small tract of jejunum. The child recovered uneventfully and is still being followed-up with no signs of recurrence. Discussion: Lipomas of the mesentery in children are very rare, and they are reported to be more common among children younger than three years of age. Mesenteric lipomas appeared to be more frequent in males than females. Even though they might be asymptomatic, voluminous lipomas can also create a lead point for intermittent torsion of the mass causing ischemia and infarction. Abdominal pain was the most frequent symptom, and the ileum was the tract of bowel more frequently involved by the tumor. Laparotomy was reported to be the preferable approach to safely remove this abdominal mass, especially in case of huge dimensions.
An 8-month-old girl underwent urgent laparoscopy, revealing the presence of adnexal and concomitant uterine torsion. Uterine torsion in pediatric patients is extremely rare but can lead to impaired fertility or loss of reproductive function, highlighting the importance of early diagnosis and rapid surgical intervention when ovarian torsion is suspected.
Introduction: Acute appendicitis is one of the most common surgical conditions in pediatric and general surgery. To date, the management of the appendiceal stump during appendectomy remains controversial. The purpose of this study was to retrospectively investigate the management strategies of acute appendicitis in a tertiary children’s hospital in terms of surgical techniques and time, hospital length of stay, and postoperative complications. Methods: The records of patients < 18 years of age who were treated for acute appendicitis at our Pediatric Surgery Unit from December 2018 to January 2024 were retrospectively reviewed. Data were extrapolated from the departmental database, and clinical notes were reviewed. Results: The records of 278 patients who underwent appendectomy within a 5-year period at our institution were essentially used to compare appendiceal stump inversion and simple ligation. The two groups were similar in terms of sex (P = 0.05), age (P = 0.40), weight (P = 0.78), and associated pathologies (P = 0.12). Statistical analysis revealed no significant differences in length of hospital stay (P = 0.21) and postoperative complications (P = 0.15). Notably, 78.6% of appendicitis cases treated without stump invagination were uncomplicated, whereas 20% were complicated (4.3% abscess and 15.7% peritonitis cases). In contrast, AS invagination was performed in 87% of uncomplicated appendicitis and 13.1% of complicated appendicitis cases (6.8% abscess and 6.3% peritonitis cases). Therefore, simple ligation was also effective in complicated appendicitis cases (P=0.03), with no significant difference in postoperative complications (P = 0.15). Conclusions: Simple ligation does not compromise treatment efficacy nor increase postoperative complications, nor the length of hospital stay. Specifically, our data suggest that simple ligation is at least as effective as invaginating the appendiceal stump, and it might probably simplify the whole procedure.
Background: Vascular malformations are relatively common in children. Current therapeutic strategies include observation, medical therapy, sclerotherapy or embolization, laser therapy, cryoablation, and surgery, depending on the type and anatomical location of the malformation. Surgery is commonly limited to small and/or circumscribed lesions, to debulking in case of large volumes, or in drug-resistant cases. Sclerotherapy is a minimally invasive treatment generally used to treat dysplastic vasculature and to significantly improve patients’ symptoms. Herein, we describe our preliminary experience with bleomycin electrosclerotherapy (BEST) in the treatment of peripheral low-flow venous and lymphatic malformations in the pediatric population. Methods: We prospectively collected and analyzed data from patients who underwent BEST for peripheral low-flow vascular malformations (venous and lymphatic) and were treated at our institution from May 2022 onward. Results: Twelve patients (4 boys and 8 girls) with peripheral low-flow vascular malformations who underwent BEST were enrolled in this preliminary study. The median patient age at the first procedure was 81 months (IQR = 46–128). The most frequent anomaly was peripheral low-flow venous malformation. No relevant postoperative complications were encountered in any of the patients. All patients underwent a clinical evaluation of the malformation 1 month after the procedure. A clinical and ultrasonographic evaluation of the malformation was performed 2 months after the procedure to determine whether to repeat BEST. In cases of clinical resolution, a second ultrasonographic evaluation was performed 6 months after the procedure. Conclusions: BEST appears to be a promising and safe option for treating peripheral low-flow vascular malformations in children. Further studies with a greater number of patients and longer follow-up periods are needed to confirm our preliminary experience.
An 8-month-old girl presented to the ER with abdominal pain and drowsiness. Ultrasound suggested ovarian torsion, which was later confirmed via laparoscopy, revealing left ovarian and tubal torsion with uterine torsion. Prompt intervention prevented hysterectomy, underscoring the importance of early diagnosis and rapid surgical action in suspected ovarian torsion cases.
A BSTRACT Granular cell tumors (GCTs) (Abrikossoff’s tumors) are rare neoplasms derived from Schwann cells. Immunohistochemistry remains the most useful instrument for diagnosing GCTs. Complete surgical excision has been demonstrated to be curative for benign lesions. However, long-term follow-up in these patients is strongly recommended.
Abstract Background Appendectomy is the most common surgical emergency that occurs in childhood and is typically performed to treat appendicitis. Nonetheless, postoperative complications remain common and the development of an intra-abdominal abscess is not an uncommon complication. Materials and Methods In this retrospective study, patient records of individuals under 18 years of age treated at our Pediatric Surgery Department for acute appendicitis and developed a post-operative abscess were examined. Examined data included demographic information and outcomes with particular attention to time at diagnosis of abscess after surgery, symptoms progression, biomarkers changes, abscess’ size, hospital length of stay, and treatment. Results Overall, thirteen children developed an abdominal post-appendectomy abscess as post-operative complication. Most patients had large-sized or multiple abscesses. The treatment was primarily performed with antibiotic therapy in the majority of cases, even though four patients required secondary surgical intervention. The success rate of the antibiotic treatment was about 70%, while that of the secondary surgical interventions was 100%. Median length of stay for patients experiencing post-operative complications was 7 days. Conclusions To improve the treatment of post-appendectomy abscesses in pediatric patients, implementing recent protocols for small and medium-sized abscesses should be considered. In addition, according to our experience, non-operative management can be initially attempted for large abscesses, followed by surgical drainage if no improvement is noted within 24–48 hours.
PURPOSE:To study the biological relationship between congenital lung malformations (CLMs) and malignancy. METHODS:Biopsies of 12 CPAMs, 6 intralobar sequestrations and 2 extralobar sequestrations were analyzed through whole-genome sequencing. Blood samples from 10 patients were used to confirm or exclude somatic mosaicism. Putative somatic Single Nucleotide Variants (SNVs) were called for each malformed sample with a Panel of Normals built with control DNA samples extracted from blood. The variants were subsequently confirmed by Sanger sequencing and searched, whenever possible, in the blood samples of patients. RESULTS:All CLMs but one presented a signature of genomic instability by means of multiple clusters of cells with gene mutations. Seven tumor transformation-related SNVs were detected in 6/20 congenital lung malformations. Four very rare in the general population SNVs were found in a region previously linked to lung cancer in 5p15.33, upstream of TERT oncogene. Furthermore, we identified missense genetic variants, whose tumorigenic role is well known, in the RET, FANCA and MET genes. CONCLUSIONS:Genomic instability in 95% of CLMs and genetic variants linked to tumor development in 30% of them, regardless of histopathology, are predisposing factors to malignancy, that combined with exposure to carcinogens, might trigger the development of malignancy and explain the association between CLMs and lung cancer.
Schwannomas are benign, slow-growing tumors originating from the Schwann cells of nerve sheaths. Extracranial schwannomas are rare, particularly in pediatric populations. Here, we report the case of a hypoglossal schwannoma in a 15-year-old male who experienced tongue paresthesia and fasciculations and difficulty swallowing two years before hospital admission. Magnetic resonance imaging showed an oval mass with sharp and regular limits of approximately 45 × 29 × 25 mm in the cranial portion of the right carotid adipose space, caudal to the right carotid and lateral foramen. The patient underwent surgery, and a histological examination confirmed a schwannoma of the hypoglossal nerve. Six months after surgery, the patient was symptom-free. The literature on schwannomas of the hypoglossal nerve is scarce, with only a few previously reported cases in the adult population. Despite their rarity, schwannomas should be considered in the differential diagnosis of masses located in the neck that present with lingual and occasionally auditory symptoms, even in pediatric patients. Surgical resection is recommended and has a low risk of long-term recurrence.
Abstract Background: Cholelithiasis is increasing in the pediatric population. While its etiology is primarily idiopathic, it is often related to underlying diseases, and a significant number of patients may be asymptomatic. There are currently no guidelines for the management of asymptomatic patients with both cholelithiasis and a predisposing condition. Therefore, this study seeks to highlight situations where prophylactic cholecystectomy may be desirable based on pre, intra, and postoperative complications, length of hospital stay, and disease recurrence. Methods: We retrospectively reviewed the medical records of patients who underwent elective laparoscopic cholecystectomy between October 2011 and September 2022. Data, including clinical presentation at the time of diagnosis and admission, imaging, treatments, complications, and length of hospital stay, were collected. Results: Thirty-two patients (21 females and 11 males) were included in the study. The median age was 14 years (range <1–17). Five different groups of patients were identified based on associated pathologies. Ten patients had hematologic disease (31.25%), four had cystic fibrosis (12.5%), six were overweight or obese (18.75%), two had an autoimmune disease (6.25%), and ten had no underlying disease (31.25%). Twenty-six patients were symptomatic (81.25%), and six were asymptomatic (18.75%). The idiopathic etiology group was entirely symptomatic. All patients underwent a laparoscopic cholecystectomy. There was one patient (in the idiopathic etiology group) who required conversion from laparoscopy to open surgery and a reintervention (3.13%). Hematologic and cystic fibrosis patients with asymptomatic cholelithiasis had a shorter length of hospital stay than patients with the same condition who progressed from asymptomatic to symptomatic gallstone disease. Conclusions: Patients with cystic fibrosis and hematologic diseases who have concomitant asymptomatic cholelithiasis may benefit from early laparoscopic cholecystectomy, which could reduce the probability of surgical and postsurgical complications and shorten the length of hospital stays.
Wilkie syndrome (aortic-mesenteric compass) was eventually diagnosed in a 15 year old girl presenting with bilious vomiting and gastric distention.