Comprehensive genomic profiling of pediatric peritoneal mesothelioma: case report with a literature review.

Malignant peritoneal mesothelioma is an extremely rare and poorly recognized neoplasm in children. A 5-year-old boy presented with a 1-year history of progressive painless abdominal distension. A CT revealed a 19 × 19 × 11 cm3 cystic mass in the right hemiabdomen, without infiltrating the surrounding structures. The tumor was completely removed by surgery. The microscopic and immunohistochemical analyses confirmed peritoneal mesothelioma. Comprehensive genomic profiling revealed no major driving mutations including BAP1, no fusions, but with amplifications of AURKA, AURKC, HLA-1B, ZNF-217, OR5F1 and MEN1 genes. Imaging follow-up 3 months after surgery revealed metastatic disease. The patient died of pneumonia at another hospital shortly after the last follow-up examination at our institution. Pediatric peritoneal mesothelioma is an extremely rare malignancy with limited targeted options and a poor prognosis. Some of the identified molecular genomic biomarkers require further exploration and validation in this cancer.

The Frequency, Severity, and Risk Factors of Hickman CatheterRelated Complications in Pediatric Cancer Patients: A Single-Center Experience from Bosnia and Herzegovina.

OBJECTIVE: This study aimed to identify the frequency, severity, and risk factors associated with Hickman catheter-related complications in children with hemato-oncological malignancies at the largest pediatric tertiary care unit in Bosnia and Herzegovina. MATERIALS AND METHODS: A cross-sectional study was conducted on a cohort of pediatric cancer patients who underwent Hickman central venous catheters (CVCs) between January 2019 and December 2022. Mechanical, infectious, and thrombotic Hickman catheter-related complications were evaluated and analyzed. We also investigated possible risk factors associated with these complications. RESULTS: Seventy-one Hickman CVCs were inserted in 68 children (44 boys and 24 girls) at a mean age of 6.9 ± 4.6. Forty (58.8%) children had hematological malignancies and 28 (41.2%) solid cancers. The median follow-up after Hickman CVC insertion was 190 days (95% CI [160-212]) for 12 644 catheter days. During follow-up, 10 (14.1%) mechanical, 7 (9.9%) infectious, and 1 (1.4%) thrombotic complications were recorded (0.8, 0.48, and 0.08 for mechanical, infectious, and thrombotic complications per 1000 catheter days, respectively). A slightly higher incidence of complications was recorded in children with hematological malignancies (1.59 per 1000 catheter days) compared with children with solid cancers (1.22 complications per 1000 catheter days). CONCLUSION: Using Hickman CVCs for long-term venous access in infusional chemotherapy for pediatric cancer patients is safe but is associated with significant morbidity.

Lipofibromatosis arising in a right labiocrural fold in a 14-month-old female: a case report study with a 3-year follow-up.

Lipofibromatosis (LF) is a rare benign fibrofatty tumor of infancy and childhood with a predilection for distal extremities, poor margination, and a high local recurrence rate. We report a toddler who presented with an LF involving her right labiocrural fold. Imaging showed a soft tissue mass extending through the right labiocrural fold with possible infiltration into the underlying muscles. The mass was excised entirely, preserving adjacent structures. The histopathologic report revealed the mass to be LF. A 3-year follow-up revealed no disease recurrence. No other cases of LF in this localization have been presented in the literature. Despite its rarity, LF should be considered in diagnosing soft tissue neoplasms in children. Accurate diagnosis and proper surgical management with complete resection are essential to reduce the postoperative recurrence risk.

Epidemiology and early bacterial colonization of minor and moderate pediatric burns: A retrospective study from a developing country.

OBJECTIVES: Infection is still the leading cause of morbidity and mortality among burn patients worldwide. Isolation and identification of pediatric burn wound bacterial colonizers can prevent infection and improve burn trauma treatment. In this study, we explored early microbial colonizers within the burn wounds and the susceptibility of those isolates to antibiotics among hospitalized pediatric patients with minor and moderate burns, clinically significant infections and outcomes. METHODS: A retrospective analysis of pediatric patients admitted to the inpatient pediatric surgical ward and treated for minor and moderate burns from 2009 to 2018 was performed. RESULTS: One hundred six patients met the inclusion criteria. The mean age was 3.6 ± three years (0.2-14.1 years). The most common type of burn was scald burns (82.1%). The mean TBSA of the hospitalized pediatric burn cases was 8.5% (IQR, 6-12%). Seventy-nine (74.5%) patients had positive wound cultures at admission, regardless of the hospital admission day. Fifty-eight (73.4%) had one bacterial growth (mono isolate), while 21 (26.6%) had mixed growth or poly isolates. Among patients with mixed growth or poly isolate, 16 had two bacteria, three had three bacteria, and one had four bacteria isolated, totaling 105 isolated microorganisms (14 different species, 70.5% Gram-positive bacteria and 29.5% Gram-negative bacteria). Twelve patients (11%) developed clinically significant infections (eleven got burn wound infection, and one had septicemia). All patients received prophylactic systemic antibiotics. Only 35.2% of the isolated bacteria from the wounds were sensitive to the prophylactic antibiotics, and only ∼17% in case of clinically significant infections. We found a statistically significant difference in the length of hospital stay between patients with initially colonized samples of burn wounds compared with patients with initial negative samples (p = 0.008). All patients in the cohort survived hospital discharge. CONCLUSION: Despite common bacterial colonization of acute burn wounds, only ∼10% of the patients developed clinically significant infections, a minority of which were sensitive to prophylactic antibiotics. Our findings indicate the need to refine the antibiotic approach in pediatric patients with minor/moderate burns in our local setting.

The Role of Demographic and Clinical Characteristics in Distinguishing Testicular Torsion from Torsion of the Appendix Testis: A Single-center Retrospective Study.

The acute scrotum (AS) in the pediatric population is a medical emergency. AS is usually caused by testicular torsion (TT) and torsion of the appendix testis (TAT). The current study explored which demographic and clinical characteristics can help distinguish between TT and TAT. We analyzed all children ≤16 years who underwent surgical exploration for AS. The patients were divided into Group 1/TT and Group 2/TAT. Ninety patients were included in the study (24 with TT and 66 with TAT). The peak incidence of TT was significantly higher than in the TAT group (p<0.001). Scrotal pain was more prevalent in the TAT group (p=0.02), whereas systemic signs (nausea/vomiting and abdominal pain) affected more frequently the TT patients (p=0.003 and p<0.001, respectively). The duration of symptoms was significantly longer in the TAT group (p<0.001). The duration of symptoms in the TT cohort significantly impacted the testicular salvage (p=0.008). Color Doppler ultrasound (CDUS) findings of absent/decreased testicular blood flow in the affected testis strongly favored the diagnosis of TT (p<0.001). The older age, shorter duration of symptoms, systemic signs, and CDUS findings can help distinguish between the two most common acute scrotum causes.

A Long-Term Outcome of the Patients with Sacrococcygeal Teratoma: A Bosnian Cohort.

OBJECTIVE: Sacrococcygeal teratoma is an extragonadal germ cell tumor that develops during fetal and neonatal periods and is associated with significant perinatal morbidity and even mortality. This study aimed to determine the clinicopathologic characteristics, the clinical outcomes, and postoperative complications of sacrococcygeal teratoma cases in the low-volume pediatric surgery service in a developing country. MATERIALS AND METHODS: The study included data from all sacrococcygeal teratoma cases diagnosed from 2011 to 2020. All the relevant clinicopathologic data were recorded and analyzed. RESULTS: A total of 7 pediatric (5 females and 2 males) patients with sacrococcygeal teratoma were identified, ranging in age from 3 to 222 days. A prenatal diagnosis was made in 57.1% of cases. The mean gestational age for all cases was 37.1 weeks (34-38 weeks), and the mean birth weight was 3285 g (range, 2300-4700 g). Preoperative alpha-fetoprotein levels had a mean value of 24.327 ng/mL (range, 649.7-110.600 ng/mL). The surgery involved resection of the primary tumor and coccygectomy in all cases. Three (42.9%) tumors were classified as Altman type II lesions, 2 (28.6%) tumors were type IV, and 2 remaining cases were types I and III, respectively. Histology was benign in 4 (57.1%) and immature in 3 patients (42.9%). The mean follow-up time was 101.4 months (30-146 months), with 2 recurrences of high-grade immature teratomas at 11 and 30 months following the surgery. Three patients had postoperative bladder and rectal dysfunctions. CONCLUSION: Sacrococcygeal teratomas are rare tumors associated with frequent postoperative dysfunctions. Recurrences may also be seen, particularly in immature, high-grade forms of sacrococcygeal teratomas.

Epidemiologic and clinical characteristics of selected congenital anomalies at the largest Bosnian pediatric surgery tertiary center.

Congenital anomalies (CA) are any abnormality present at birth, either structural or functional, that may potentially affect an infant's health, development, and/or survival. There is a paucity of studies on clinical characteristics and outcomes of CA in Bosnia and Herzegovina, mainly due to the lack of a nationwide congenital malformations monitoring system. A 5-year hospital-based study was conducted to determine the prevalence at birth and clinical characteristics of selected major CA in Sarajevo Canton, Bosnia and Herzegovina. Ninety-one CA were observed from 2012 to 2016 (the overall prevalence was 39.6 cases/10,000 live births). The mean age of neonates at diagnosis was 3 days. The gastrointestinal tract was the most commonly affected system (76.9%), with esophageal atresia (EA) being the most frequent (17.6% of all CA). Major CA were more prevalent among preterm infants than term infants (P = .001), particularly in males (61.5% vs. 38.5%; P = .028; M:F ratio was 1.59). Multiple CA were seen in 37.4% of neonates. The overall mortality rate of neonates was 11%, and the median length of hospital stay was 19.8 days. Our study revealed the distribution and clinical patterns of common major CA in the largest tertiary care facility in Bosnia and Herzegovina. It also confirmed a relatively high mortality rate, which requires further efforts to improve the quality of neonatal care in the country.

Assessment of postoperative cosmetic outcomes of distal form hypospadias repair with the Hypospadias Objective Scoring Evaluation (HOSE).

Aim A standardized assessment for the optimal repair of hypospadias remains elusive. The aim of this study was to assess a postoperative cosmetic outcome of hypospadias repair using a validated questionnaire, Hypospadias Objective Scoring Evaluation (HOSE). Methods During the period between January 2016 and May 2019, 40 patients who underwent hypospadias repair were identified and they agreed to a follow-up using the HOSE. Distal hypospadias repairs underwent a cross-sectional assessment of the cosmetic outcome. Cosmetic assessment was performed by an independent physician using the HOSE scoring system. Results The native meatus was coronal in 10 (25%), subcoronal in eight (20%), and distal penile in 22 (55%) patients. Mean followup was 35.90 months (SD ±29.58) postoperatively (range 12-162 months). Complications occurred in one (2.5%) patient. Out of 40 uncomplicated repairs, 39 (97.5%) were satisfactory. A vertical slit-like meatus located at the distal glans was created in 33 (82.5%) boys, and at the proximal glans in seven (17.5%). The urinary stream was single and straight in 39 and spray in one patient. A straight erection was observed in 39 (97.5%) boys. The median HOSE score was 16 (range 12-16). One patient had a small, single coronal fistula. The technique used included tubularised incised plate urethroplasty. Conclusion The HOSE score is simple, easy, non-invasive and non-expensive tool for objective assessment of long-term outcomes of hypospadias repair.

Successful Pregnancy Outcome after Amnioreduction Treated Acute Polyhydramnios Caused by Duodenal Atresia.

The aim of our manuscript is to report of a successful perinatal outcome after treatment of acute polyhydramnios caused by duodenal atresia. A 34-year-old G3P1 was referred due to polyhydramnios in the 30th week of pregnancy. Ultrasound revealed polyhydramnios, amniotic fluid index (AFI) 28, and a double bubble sign that indicated duodenal atresia and dilatated oesophagus. In the 32nd week of gestation, the volume of amniotic fluid increases, AFI 35, along with symptoms of dyspnea and abdominal pain. Due to the clinical picture and the early gestational age, it was decided to perform an amnioreduction. In the 36th week of gestation cesarean section was performed. The baby was taken for exploratory laparotomy and found to have a simultaneous complete duodenal atresia and annular pancreas with associated dilated the first portion of the duodenum and the stomach. A side-to-side duodenoduodenostomy via single-layer hand-sewn anastomosis was performed over a transanastamotic feeding tube (TAFT). The postoperative course was uneventful. Amnioreduction is useful and safe in the treatment of acute polyhydramnios caused by duodenal atresia and thus has a significant role in prolonging gestation until fetal maturity.

Infantile hypertrophic pyloric stenosis in Bosnia and Herzegovina: A retrospective cohort study from the largest tertiary care facility.

BACKGROUND: Infantile hypertrophic pyloric stenosis (IHPS) is the most common condition requiring surgery in infancy, but the etiology of IHPS is still unclear. The study aimed to analyze the epidemiological and clinical features of the infants with IHPS in our setting and determine the yearly trends in IHPS incidence in the Sarajevo Canton between 2007 and 2016. METHODS: We retrospectively analyzed epidemiologic, clinical, and operative data of all infants undergoing pyloromyotomy for IHPS over ten years in the largest tertiary care facility in Bosnia and Herzegovina. RESULTS: Fifty-three IHPS patients were diagnosed, yielding an overall incidence of 1.17 per 1000 live births (1.25 and 1.09 cases in 2007-2011 and 2012-2016, respectively). IHPS was more prevalent among male infants (ratio 6.6:1, p < 0.001). The mean age at onset of symptoms was 39.6 days (range, 17-107 days). The estimated median time from symptoms onset to hospitalization was 11 days (range, 1-17 days). The mean age at diagnosis was significantly longer in premature infants compared with term infants (p = 0.003). Both first-born rank and bottle-feeding were significantly associated with IHPS (p = 0.001 and p = 0.04, respectively). No seasonal variation associated with IHPS was detected (p = 0.25). No evidence was found of differences in the incidence of IHPS related to maternal age (p = 0.24) and smoking (p = 0.59). CONCLUSION: Our data indicate a declining trend and provide insights into the clinical characteristics of IHPS in Bosnia and Herzegovina. Most of the obtained results are in line with the published data and could improve the quality of local pediatric services.

Appendiceal carcinoid in a pediatric patient with Peutz-Jeghers syndrome: A case report and comprehensive literature review.

RATIONALE: Peutz-Jeghers syndrome (PJS), a rare autosomal dominant disorder, is characterized by mucocutaneous pigmentations, hamartomatous polyps in the gastrointestinal tract, and a high risk of developing various malignancies. To the best of our knowledge, only 1 case of appendiceal carcinoid associated with PJS has been previously reported in the pediatric population. PATIENT CONCERNS: We report a 7-year-old girl who was admitted for severe, intermittent abdominal pain and cramps, nausea, and vomiting. Multiple brown melanotic macules on the lips, buccal mucosa, and the tongue were noted. DIAGNOSIS: A plain abdominal X-ray in a standing position revealed dilated intestinal loops with multiple air-fluid levels. A computed tomography scan of the abdomen showing a "coffee bean" appearance of the jejunal loop with a transition point to the duodenal loop. Axial-contrast-enhanced computed tomography scan of the abdomen showing dilated jejunum loops, filled with fluid with the swirled appearance of mesentery typical for volvulus. The diagnosis of PJS was based on clinical findings along with the histopathologic confirmation of the hamartomatous polyps. INTERVENTIONS: An emergency laparotomy was performed, revealing a jejunojejunal intussusception starting 40 cm from the duodenojejunal flexure. Jejunotomy revealed that a lead-point intussusception was a necrotic hamartomatous polyp. After resecting the involved jejunal necrotic segment, including the polyp, end-to-end jejuno-jejunal anastomosis was performed. Further exploration revealed the presence of a jejunal mass 80 cm from the duodenojejunal flexure identified as another hamartomatous pedunculated polyp. The polyp was resected, and the enterotomy was then closed transversely. The grossly normal appendix was also removed. OUTCOMES: Clinical findings along with the histopathologically confirmed hamartomatous polyps were consistent with PJS. An appendiceal carcinoid (well-differentiated neuroendocrine tumor, European Neuroendocrine Tumor Society stage pT2) was incidentally detected during histological examination of the appendix. The patient and parents were counseled accordingly, focusing on active surveillance and control of symptoms. Two additional hamartomatous polyps (gastric and jejunal) were detected endoscopically and resected in the fourth postoperative week. A regular, 1-year follow-up and surveillance revealed no complications or recurrences. LESSONS: Unusual neoplasms can occasionally be encountered in well-defined syndromes such as PJS. Therefore, active follow-up and surveillance are mandatory for all patients with PJS.

The predictors of perforated appendicitis in the pediatric emergency department: A retrospective observational cohort study.

OBJECTIVE: Appendiceal perforation has significant effects on perioperative morbidity and postoperative outcome. The present study aimed to identify possible predictive factors associated with perforated appendicitis (PA) in children at admission in the emergency department (ED). METHODS: In this retrospective observational cohort study, consecutive medical records of children <18 years old with surgically and histopathologically confirmed acute appendicitis (AA) over three years (2013-2015) were analyzed. Patients were divided into two groups: PA and non-perforated appendicitis (NPA). The differences between the two groups and potential predictors of PA were explored using univariate and multivariate analyses. RESULTS: During the study period, 295 patients underwent an appendectomy and had confirmatory AA diagnoses. Ninety-two patients had a PA (31.2%). In the univariate analysis, male gender, vomiting, diarrhea, fever, elevated white blood cell count (WBC) levels, and high C-reactive protein (CRP) were identified as predictors of PA. In the multivariate analysis, male gender (odds ratio [OR]: 3.133; 95% confidence interval [CI]: 1.610-6.096); vomiting (OR: 2.346; 95% CI: 1.141-4.822); diarrhea (OR: 4.549; 95% CI: 1.850-11.181); fever (OR: 3.429; 95% CI: 1.765-6.663); elevated WBC (OR: 2.962; 95% CI: 1.491-5.884) and elevated CRP (OR: 3.061; 95% CI: 1.267-7.396) were variables that predicted the PA in children. CONCLUSION: Our data indicate that several clinical and biochemical parameters can reliably distinguish between pediatric PA and NPA at admission in the emergency department.

The Effects of Ranitidine Treatment on the Risk of Necrotizing Enterocolitis in Preterm Infants: A Case-Control Study.

INTRODUCTION: Gastric acidity plays an important role in the protection of infants against various pathogens from the environment. The histamine-2 receptor blockers (H2-blockers) are off-labeled drugs that are frequently prescribed in preterm neonates to prevent stress ulcers. The impact of the H2-blockers on the development of the necrotizing enterocolitis (NEC) in preterm infants is still controversial, particularly in the developing world. MATERIALS AND METHODS: One hundred twenty-two preterm infants were enrolled in the study. The multivariate logistic regression model was used to identify potential postnatal risk factors associated with NEC. RESULTS: Preterm infants (n = 51) with total NEC, medical NEC, and surgical NEC had the highest rate of receiving ranitidine compared with controls (n = 71) (39.2%, 19.6%, and 47.6%, p < 0.05). Logistic regression analysis revealed that ranitidine use and nosocomial infections were significantly associated with NEC development (odds ratios 1.55 and 3.3). CONCLUSIONS: We confirm that ranitidine administration was associated with an increased risk of NEC in preterm infants. H2-blockers use should be only administered in very strictly selected cases after careful consideration of the risk-benefit ratio.

Duration of symptoms is the only predictor of testicular salvage following testicular torsion in children: A case-control study.

OBJECTIVE: Testicular torsion (TT) is an emergency requiring a prompt diagnosis and surgery to avoid irreversible changes and a complete loss of testis. The present study aimed to identify potential factors that may be predict a testicular salvage after TT in pediatric patients. METHODS: Consecutive medical records of all children ≤16 years old with surgically confirmed TT over a period of five years (2011-2016) were collected. Patients were divided into 2 groups according to testicular viability and the type of treatment: Orchidectomy and orchidopexy. The differences between the two groups and potential predictors of testicular salvage were analyzed. RESULTS: Thirty-one boys with TT met the inclusion criteria and were included in the study. The mean age was 13.6 years (range, 10 days - 15.8 years). Testicular salvage was possible in 18 (58.1%) patients. The duration of symptoms and a lesser degree of torsion indicated a testicular salvage in children and adolescents with testicular torsion, but in multivariate analysis only duration of symptoms (time to surgical detorsion) was significantly associated with the risk of non-salvage. At follow-up, testicular atrophy affected 73.3% of the patients treated with orchidopexy. CONCLUSION: Duration of symptoms is the only predictor of successful testicular salvage following testicular torsion in children. It is associated with a substantial risk of testicular loss and atrophy.

Urinary neutrophil gelatinase-associated lipocalin level as a biomarker of acute kidney injury following extracorporeal shock wave lithotripsy.

INTRODUCTION: Although extracorporeal shock wave lithotripsy (ESWL) is minimally invasive and highly efficient for the management of kidney stones, adverse effects have been described. Available indicators of renal function exhibit insufficient sensitivity in acute renal injury (AKI). We aimed to evaluate the severity of the kidney tissue response to ESWL injury by measuring the urinary neutrophil gelatinase-associated lipocalin (uNGAL), which can indicate AKI in its early phase. MATERIAL AND METHODS: The prospective, controlled study included 62 patients with nephrolithiasis undergoing single ESWL treatment. uNGAL level was measured before the procedure, and 6 h and 12 h after. RESULTS: The median uNGAL level increased by 126.0%, 6 h after ESWL (p <0.001). The growth rate continued and 12 h after was higher by 583.7%, compared to the pre-treatment level (p <0.001). The median value of estimated glomerular filtration rate (eGFR) dropped by 15.3% 12 h after the treatment (p <0.001). It increased by 5.0% in the period 7 days to 3 months after (p <0.001) and after 3 months it was lower by 10.1% compared to pre-ESWL values (p <0.001). uNGAL level after 12 h was significantly negatively associated with eGFR, 12 h, 7 days and 3 months after the ESWL. The sensitivity of uNGAL 12 h after ESWL was 60.6%; its specificity was 75.0%, with a positive predictive value of 74.0% and negative predictive value of 61.7%. CONCLUSIONS: uNGAL appears to be a useful biomarker for the assessment and prediction of AKI. It was noticed that uNGAL had the highest predictive value 12 h after the ESWL treatment.

Clinical course and short-term outcome of postsplenectomy reactive thrombocytosis in children without myeloproliferative disorders: A single institutional experience from a developing country.

OBJECTIVES: To evaluate the clinical outcome and complications in the pediatric population who had splenectomy at our institution, emphasizing the incidence of postplenectomy reactive thrombocytosis (RT) and its clinical significance in children without underlying hematological malignancies. MATERIALS AND METHODS: The medical records of pediatric patients undergoing splenectomy were retrospectively reviewed for the period 1999-2018. The following variables were analyzed: Demographic parameters (age, sex), indications for surgery, operative procedures, preoperative and postoperative platelet count (postplenectomy RT), the use of anticoagulant therapy, and postoperative complications. The patients were divided into two groups according to indications for splenectomy: The non-neoplastic hematology group and the non-hematology group (splenectomy for trauma or other spleen non-hematological pathology). RESULTS: Fifty-two pediatric (37 male and 15 female) patients who underwent splenectomy at our institution were reviewed. Thirty-four patients (65%) were in the non-hematological group (splenic rupture, cysts, and abscess) and 18 patients (35%) in the non-neoplastic hematological group (hereditary spherocytosis and immune thrombocytopenia). The two groups did not differ significantly in regards to the patients' age, sex, and preoperative platelet count (P>0.05 for all variables). Forty-nine patients (94.2%) developed postplenectomy RT. The percentages of mild, moderate and extreme thrombocytosis were 48.9%, 30.7%, and 20.4%, respectively. The comparisons of RT patients between the non-neoplastic hematology and the non-hematology group revealed no significant differences in regards to the patients' age, sex, preoperative and postoperative platelet counts, preoperative and postoperative leukocyte counts, and the average length of hospital stay (P>0.05 for all variables). None of the patients from the cohort was affected by any thrombotic or hemorrhagic complications. CONCLUSIONS: We confirm that RT is a very common event following splenectomy, but in this study it was not associated with clinically evident thrombotic or hemorrhagic complications in children undergoing splenectomy for trauma, structural lesions or non-neoplastic hematological disorders.

Penile Degloving and Dorsal Dartos Flap Rotation Surgery in the Management of Severe Isolated Penile Torsion in a 6-Year-Old Boy.

Penile torsion is a rare congenital anomaly that is usually characterized by a counterclockwise rotation of the penile shaft or glans. Although several surgical techniques for its correction have been proposed, the consensus of choosing the most efficient technique remains controversial. Herein, we report our operational approach that successfully corrected a severe (>90 degrees) isolated penile torsion in the form of penile degloving and dorsal dartos flap rotation surgery.