How can the surgeon choose preoperatively the most appropriate antibiotic prophylaxis vs therapy in pediatric acute appendicitis?

INTRODUCTION: The aim of this study was to find statistically valid criteria to preoperatively divide acute appendicitis into simple and complicated to enable surgeons to administer the most appropriate antibiotic prophylaxis/therapy before surgery. MATERIALS AND METHODS: We retrospectively reviewed a cohort of patients who underwent appendectomy from January 2022 to December 2023. Patients included were 0-14 years of age. Exclusion criteria included patients who underwent interval appendectomy or concurrent procedures at the same time of appendectomy. We divided patients into two groups: simple (group S) and complicated (group C) appendicitis according to intraoperative finding. Generalized linear model (GLM) with logit function was developed to identify the predictive variables of the type of appendicitis (S vs C) in terms of CRP value, neutrophils percentage and WBC count adjusted for age and sex of patients. Finally, principal component analysis (PCA) was carried out to identify the cutoff value of statistically significant variables found in the previous analysis. RESULTS: One hundred and twenty patients were eligible (N female = 49, N male = 71) for the study. 74 and 46 patients were included in groups S and C, respectively. In a preliminary analysis using univariate and multivariate GLM, only CRP (p value = < 0.001) and neutrophils percentage (p value = 0.02) were predictive variables for the type of appendicitis. The GLM shows a statistical lower value of CRP (adjusted odds ratio [OR] per unit, 0.17 [95% CI, 0.08-0.39]) and neutrophil percentage (adjusted OR per unit, 0.37 [95% CI, 0.16-0.86]) in the S group compared to C adjusted to age and sex. PCA analysis revealed a P-ROC cutoff of 4.2 mg/dl and 80.1 of CRP value (AUC = 84%) and neutrophil percentage (AUC = 70%), respectively. CONCLUSIONS: We will perform a prospective study giving preoperative prophylactic cefazolin to patients with a CRP value under 4.2 mg/dl and amoxicillin-clavulanate therapy to patient with CRP value over 4.2 mg/dl.

Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

INTRODUCTION: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. CONCLUSION: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.

The role of mini-PCNL as primary approach for the treatment of pediatric kidney stones in a high-income country. Ten-year single-center report.

PURPOSE: To describe our experience in the use of percutaneous nephrolithotomy (PCNL) as a primary treatment for paediatric kidney stones and to highlight its benefits. METHODS: The design was retrospective and observational. All the children treated for kidney stones from 2011 to 2021 were included. The population was divided into Group A (PCNL) and Group B (retrograde intrarenal surgery, RIRS). The outcomes were stone-free rate (SFR), the rate of procedures per patient, the rate of failure and the rate of complications. RESULTS: Twenty-eight patients with 33 kidney units were included. Eighteen of them (64%) were males. The median age was 10 (IQR 6.8-13) years. Forty-seven procedures were performed. Twenty-four of them (51%) were mini-PCNL. Group A included 17 patients (61%). Group A presented a higher SFR (p = 0.007) and a lower number of procedures (p < 0.001). RIRS failed in five cases (45%) because of non-compliant ureter. Two urinary tract infections (UTI) were reported after PCNL and four UTIs after RIRS (p = 0.121). No major complications were reported. CONCLUSION: Mini-PCNL should be suggested as a primary approach for pediatric kidney stones. This technique presented a better effectiveness with a reduced number of procedures when compared to RIRS.

Hypospadias: clinical approach, surgical technique and long-term outcome.

BACKGROUND: Hypospadias is one of the most common congenital abnormalities in male newborn. There is no universal approach to hypospadias surgical repair, with more than 300 corrective procedures described in current literature. The reoperation rate within 6-12 months of the initial surgery is most frequently used as an outcome measure. These short-term outcomes may not reflect those encountered in adolescence and adult life. This study aims to identify the long-term cosmetic, functional and psychosexual outcomes. METHODS: Medical records of boys who had undergone surgical repair of hypospadias by a single surgical team led by the same surgeon at a single centre between August 2001 and December 2017 were reviewed. Families were contacted by telephone and invited to participate. Surgical outcome was assessed by combination of clinical examination, a life-related interview and 3 validated questionnaires (the Penile Perception Score-PPS, the Hypospadias Objective Score Evaluation-HOSE, the International Index of Erectile Function-5-IIEF5). Outcomes were compared according to age, severity of hypospadias, and respondent (child, parent and surgeon). RESULTS: 187 children and their families agreed to participate in the study. 46 patients (24.6%) presented at least one complication after the repair, with a median elapsed time of 11.5 months (6.5-22.5). Longitudinal differences in surgical corrective procedures (p < 0.01), clinical approach (p < 0.01), hospitalisation after surgery (p < 0.01) were found. Cosmetic data from the PPS were similar among children and parents, with no significant differences in child's age or the type of hypospadias: 83% of children and 87% of parents were satisfied with the cosmetic result. A significant difference in functional outcome related to the type of hypospadias was reflected responses to HOSE amongst all groups of respondents: children (p < 0.001), parents (p=0.02) and surgeon (p < 0.01). The child's HOSE total score was consistently lower than the surgeon (p < 0.01). The HOSE satisfaction rate on functional outcome was 89% for child and 92% for parent respondents. CONCLUSION: Surgeons and clinicians should be cognizant of the long-term outcomes following hypospadias surgical repair and this should be reflected in a demand for a standardised approach to repair and follow-up.

Combined Double-breasted Full-thickness Abdominal Flap Plication and Acellular Dermal Matrix in Prune-belly Syndrome Reconstruction.

Background: Reconstruction of the abdominal wall in patients with prune-belly syndrome (PBS) following previous intra-abdominal procedures is a challenging problem with a high incidence of revision due to persistent bulging or herniation. The abdominal wall flaccidity not only produces a severe psychological and aesthetic discomfort for the patient but often determines functional disabilities, including inability to cough properly, impaired bowel and bladder function, and delay in posture and balance. Methods: The authors describe three cases of reconstruction of abdominal wall using a modified double-breasted abdominoplasty fascial plication with additional acellular dermal matrix interposition and review the literature for innovations in the use of abdominal repair for reconstruction of these difficult cases. Results: Three children with PBS at a mean age of 7.3 years achieved successful reconstruction of the abdominal wall, using the modified double-breasted abdominoplasty fascial plication with acellular dermal matrix interposition. Patients underwent previous procedures, including orchiopexy in two patients and bilateral nephrectomy in one patient. No postoperative complications have been found, apart from superficial skin dehiscence along the abdominal incision treated conservatively in one child. At mean follow-up of 42 months (range 28-56 months), no patient presented incisional hernia, persistent or recurrent fascial laxity with abdominal bulging. All patients achieved significant aesthetic and functional improvements, including children's ability to cough, spontaneous gain of abdominal tonus, balance, and ambulation. Conclusion: Modification of the original vertical, two-layer plications of the deficient abdominal interposing biological mesh has the purpose of improving strength, aesthetics, and function of the abdominal wall in pediatric patients with PBS.

Townes-Brocks syndrome with craniosynostosis in two siblings.

This report describes a novel truncating c.709C > T p.(Gln237*) SALL1 variant in two siblings exhibiting sagittal craniosynostosis as a unique feature of Townes-Brocks syndrome (TBS, OMIM #107480). TBS is a rare autosomal dominant syndrome with variable phenotypes, including anorectal, renal, limb, and ear abnormalities, which results from heterozygous variants in the SALL1 gene, predominantly located in the 802 bp "hot spot region" within exon 2. Recent studies have suggested that aberrations in primary cilia and sonic hedgehog signalling contribute to the TBS phenotypes. The presence of the novel c.709C > T p.(Gln237*) SALL1 variant was confirmed in both the siblings and their father, whereas no mutations currently associated with craniosynostosis were detected. We hypothesise that the truncating c.709C > T p.(Gln237*) SALL1 variant, which occurs outside the "hot spot region" and inside the glutamine-rich domain coding region, could interfere with ciliary signalling and mechanotransduction, contributing to premature fusion of calvarial sutures. This report broadens the genetic and phenotypic spectrum of TBS and provides the first clinical evidence of craniosynostosis as a novel feature of the syndrome.

Comparison between prone and supine nephrolithotomy in pediatric population: a double center experience.

PURPOSE: Stone disease in the pediatric age is an increasing issue. Percutaneous Nephrolithotomy (PNL) can be used for larger and complex stones. As in adults it can be performed in the supine or prone position. METHODS: We retrospectively reviewed two centers' experience in prone and supine PNL in children to analyze its results and complications. RESULTS: 33 patients underwent prone and 19 supine procedures. Patients in the prone group were younger than in the supine, while no significant differences were found in stone burden, access size, operative time or complications. Complications were: 8 and 4 Clavien 1 for the prone and supine group, respectively, one case of urosepsis (4b) in the prone and 2 cases of Clavien 3 in the supine group (double J stent placement for renal colic and ureteroscopy for steinstrasse). Tubeless procedures and mean nephrostomy time were in favor of the supine group, whereas fluoroscopy time and ureteral drainage stay were in support of the prone group. Stone free rate was better in the supine group (83.3 vs 66.6%), possibly reflecting the capability to perform a combined approach in 12 patients (allowing to reach all the calyx with simultaneous anterograde and retrograde access) or younger age in the prone group (13 vs 2 patients ≤5 years), with no differences in stone burden. CONCLUSIONS: Supine approach seems to guarantee higher stone-free rates. Larger series are necessary to determine what the best technique is in terms of X-ray exposure, operative time and complications.

Thoracoscopic treatment of a rare bilateral extralobar lung sequestration in a 3-years old girl.

Majority of sequestrations fall into two categories: Intra-Lobar (ILS) and Extra-Lobar (ELS). Rarely the abnormal lung could be attached to the gastrointestinal tract, Bronchopulmonary Foregut Malformation (BPFM). We described a case of a girl of 3-years-old with antenatal diagnosis of left intrathoracic mass of the inferior lobe. Postnatal Computed-Tomography (CT) revealed a bilateral ELS with an isthmic bridge crossing the vertebral spine. She follows a MRI follow-up at 18months/30months confirming the lesion. Before surgery, a three-dimensional-CT-angiography was performed to study the mass, its blood supply and to plan surgery. She underwent to thoracoscopic resection. Two aberrant blood vessels were dissected from the thoracic aorta and ligated. The postoperative course was uneventful. She was discharged after 3 days. The rarity of our case is due to the bilateral extension. An appropriate preoperatory imaging study is necessary for the success of surgery while thoracoscopy is particularly appropriate in surgical treatment.

Once-daily intrapleural urokinase treatment of complicated parapneumonic effusion in pediatric patients.

In this paper, we describe our experience in the treatment of childhood empyema using urokinase. Patients' ages ranged from 2 to 12 years. Urokinase (dosage: 3,100 IU/kg/day) was diluted in normal saline to produce 1000 IU/ml (maximum dosage 100,000 IU in 100 ml of normal saline). After 2 hours, the clamped catheters were released and connected to water-seal suction at a negative pressure of 10 cm H2O. Pleural irrigations were continued once a day until thoracostomy tube output decreased to less than 10 ml/day (urokinase treatment mean duration: 11.5 days). The complete resolution of the chest effusion was assessed on chest ultrasound scan and radiographs. None of the patients experienced any side effects due to urokinase. It would now seem reasonable to advocate small chest tube thoracostomy and intrapleural urokinase as first-line treatment of pleural empyema in children, with surgery indicated as a secondary intervention.

Cytoreductive surgery and HIPEC in a 14 years old patient with peritoneal recurrence of adenocarcinoma of the right colon.

INTRODUCTION: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is nowadays a feasible and effective treatment for peritoneal metastasis. We present a case of a 14 years old child with peritoneal metastasis from recurrent colorectal cancer. PRESENTATION OF CASE: Colonoscopy and CT-scan were performed leading to the diagnosis of a stenosing adenocarcinoma of the right colon in 2015. Two pelvic lesions were found at the total body PET scan, suspected for peritoneal metastasis. Neoadjuvant chemotherapy was administered, and restaging CT-scan and magnetic resonance (MRI) highlighted a partial response. The patient underwent right laparoscopic hemicolectomy. The postoperative staging was T4 N1 G3. Seven months after the last cycle of adjuvant chemotherapy, CT-scan revealed two huge abdominal masses. The patient underwent explorative laparotomy and bilateral oophorectomy, positive for metastasis from colorectal cancer and peritoneal washing cytology was positive for neoplastic cells. A CT-scan was performed on December 2017 showed a suspect lesion below the anterior abdominal wall. The case was discussed at the tumour board and the indication for CRS and HIPEC was given. In January 2018 the child underwent complete CRS and HIPEC with no complications. No adjuvant chemotherapy was administered. After 11 months the follow up is negative for the recurrent disease. DISCUSSION AND CONCLUSION: Cytoreduction and HIPEC can be performed even in children as a feasible and safe treatment with successful outcomes. As for adults, an appropriate multidisciplinary pre-operative work up and a correct cases selection is needed to have the best results even regarding the quality of life.

Drug-Induced Urolithiasis in Pediatric Patients.

Drug-induced nephrolithiasis is a rare condition in children. The involved drugs may be divided into two different categories according to the mechanism involved in calculi formation. The first one includes poorly soluble drugs that favor the crystallization and calculi formation. The second category includes drugs that enhance calculi formation through their metabolic effects. The diagnosis of these specific calculi depends on a detailed medical history, associated comorbidities and the patient's history of drug consumption. There are several risk factors associated with drug-induced stones, such as high dose of consumed drugs and long duration of treatment. Moreover, there are some specific risk factors, including urinary pH and the amount of fluid consumed by children. There are limited data regarding pediatric lithogenic drugs, and hence, our aim was to perform a comprehensive review of the literature to summarize these drugs and identify the possible mechanisms involved in calculi formation and discuss the management and preventive measures for these calculi.

First case of laparoscopic partial splenectomy in a child with hamartoma: Case report and review of the literature.

INTRODUCTION: To date, laparoscopic surgery has played a key role in the treatment of not only splenic hematologic pathologies but also solid ones. Hamartoma is a rare disease; only twenty percent of them are of pediatric relevance; it is a benign tumor, but radiological features never allow proper differentiation from malignant neoplasms. In children, hamartoma may be associated with other morbid conditions, such as sickle cell disease or other hematological alterations. PRESENTATION OF THE CASE: We report a case of hamartoma in a 7-year-old child treated with partial laparoscopic splenectomy. After a multidisciplinary evaluation, the indication of laparoscopic splenectomy was decided; upon evaluating the age of the patient and the affected spleen portion, a partial splenectomy was proposed. The histological examination during surgery was performed to exclude any form of malignancy. The intraoperative frozen section of the specimen was negative for malignancies, and a partial splenectomy was performed. DISCUSSION: Surgery remains the first choice in the definitive treatment of solid lesions of the spleen; minimally invasive technique, namely, laparoscopy, has set itself as the technique of choice for surgical treatment. In this case, the possibility of obtaining an intraoperative pathological diagnosis by frozen section of the specimen, confirming the benign nature of the lesion, allowed the surgeon to decide in favor of a laparoscopic partial splenectomy. CONCLUSION: Partial laparoscopic splenectomy can be considered a safe, effective and reproducible alternative in patients suffering from benign solid diseases, safeguarding the hematological functions of the organ itself in pediatric age.

Transperitoneal laparoscopic adrenalectomy in children.

PURPOSE: The use of a minimally invasive approach for adrenalectomy is poorly defined in pediatric patients, although laparoscopic adrenalectomy is considered a standard procedure in adults. The aim of our study was to describe the safety and feasibility of minimally invasive adrenalectomy in children on the basis of surgical skills and results. MATERIALS AND METHODS: This was a retrospective study of 4 pediatric laparoscopic adrenalectomies performed at our center between 2009 and 2012. All patients underwent transperitoneal lateral laparoscopic adrenalectomies (2 right and 2 left adrenalectomies). RESULTS: Four laparoscopic adrenalectomies were performed. Indications for surgery were neuroblastoma in 2 patients, secernent adrenocortical tumor in 1 patient, and adrenocortical nodular hyperplasia in 1 patient. Patients had a mean age of 87 months (range, 17-156 months) at diagnosis, and the average lesion size was 3.23 cm (range, 0.7-6.4 cm). All laparoscopic adrenalectomies were successful, no conversions to open surgery were required, and no postoperative complications or deaths occurred. The average operating time was 105 minutes (range, 80-130 minutes), blood loss during surgery was minimal, and the mean postoperative hospital stay was 3.75 days (range, 3-5 days). None of the patients showed signs of recurring disease at 15-month follow-up. CONCLUSIONS: Laparoscopic adrenalectomy is a safe, feasible, and reproducible technique offering numerous advantages, including shortening of operating times and postoperative hospital stays, as well as reduction of blood loss and complications. It also provides good visibility and easy access to other organs.

Bilateral xanthogranulomatous funiculitis and orchiepididymitis in a 13-year-old adolescent boy.

Xanthogranulomatous orchitis is an extremely rare inflammatory nonneoplastic lesion of the testis. We report a case of a 13-year-old adolescent boy who presented a painless left hemiscrotal swelling. The subsequent ultrasonography and magnetic resonance imaging revealed the presence of abnormal expanding tissue located in both testes and spermatic cord, reaching the internal inguinal ring. Testicular tumor markers were normal. The frozen section examination of the surgical specimen showed only inflammatory tissue and not neoplastic tissue. No orchiectomy was performed. Definitive histopathologic diagnosis was xanthogranulomatous inflammation. To our knowledge, this is the youngest case of xanthogranulomatous orchiepididymitis and funiculitis found in medical literature.

Jejunal obstruction as initial presentation of polyarteritis nodosa in a 13-month-old boy.

Systemic polyarteritis nodosa (PAN) is a rare disease in childhood affecting small and midsized arteries. The typical presentation in children is of isolated 1- or 2-organ involvement, and the diagnosis is often based on the histopathology. We report a case of pediatric PAN whose presenting symptom was jejunal obstruction owing to ischemic necrosis of the small bowel. The postoperative recovery was uneventful, and the patient responded well to steroid therapy. There are only a few cases of PAN with intestinal involvement described in the literature, some of them fatal. The diagnosis of PAN should be considered in children with acute intestinal obstruction with no obvious etiology, as early recognition and treatment may reduce its high mortality.

Non-invasive methods can predict oesophageal varices in patients with biliary atresia after a Kasai procedure.

BACKGROUND: After a Kasai procedure, 70% of patients with biliary atresia develop chronic liver disease with portal hypertension and oesophageal varices. AIMS: To investigate the role of new non-invasive parameters in predicting the presence of varices in patients with biliary atresia after a Kasai procedure and to identify the cut-off values of these parameters in predicting the presence of varices. METHODS: 31 patients with biliary atresia who had undergone a Kasai portoenterostomy were studied. Clinical, biochemical and abdominal ultrasound examination, liver stiffness measurement (LSM), LSM-spleen diameter to platelet ratio score (LSPS) and upper digestive endoscopy were performed. RESULTS: 15 (47%) patients had oesophageal varices (Group A) and 16 had no varices (Group B). Median values of LSM (kPa) and LSPS were significantly higher in Group A than in Group B (LSM: 17.0 vs. 7.5, respectively; p=0.0001; LSPS: 19.62 vs. 2.94, respectively; p=0.0001). The optimal cut-offs for predicting oesophageal varices were: LSM>10.6 kPa (sensitivity: 87%, specificity: 87.5%, PPV: 87%, NPV: 87.5%, and AUC: 0.92) and LSPS ≥9.2 (sensitivity: 91%, specificity: 92%, PPV: 91%, NPV: 92%, and AUC: 0.96). CONCLUSIONS: Non-invasive methods can predict the presence of oesophageal varices in patients with biliary atresia; the sequential use of two non-invasive methods improves accuracy.