Vertebral artery dissection aneurysm in a pediatric patient: A rare case with unusual clinical manifestations, diagnostic, and management challenges.

Vertebral artery aneurysm is a rare condition with diverse clinical manifestations in pediatric patients. We present the case of a 12-year-old male who presented with diplopia, vomiting, ataxia, and severe headache. Diagnostic evaluation revealed an extracranial vertebral artery dissection with an associated aneurysm at the C3-C4 level. Despite the absence of recurrent ischemic strokes, the aneurysm posed challenges in differentiating the symptoms from other inflammatory demyelinating disorders, particularly internuclear ophthalmoplegia. Diagnosis relied on a thorough history, physical examination, and imaging studies. Magnetic resonance imaging with magnetic resonance angiography confirmed the diagnosis and played a crucial role in assessing the size, location, and extent of the aneurysm. Additionally, the imaging findings helped guide treatment decisions and determine the need for anticoagulation therapy. Regular follow-up imaging was initiated to monitor for late complications and evaluate the effectiveness of the management approach. This case highlights the atypical presentation of vertebral artery aneurysm in a pediatric patient, underscoring the importance of clinical suspicion and the role of advanced imaging techniques in facilitating accurate diagnosis and guiding appropriate management. Prompt diagnosis and optimal utilization of imaging modalities are essential in preventing severe morbidity and mortality. Further research is warranted to enhance our understanding of this condition and refine imaging and management protocols in pediatric population.

Parotid gland masses: outcomes in the pediatric age group.

BACKGROUND: Childhood parotid neoplasms appear to have different characteristics from adults. This point, in addition to the rarity of these tumors, reflects the challenges faced in diagnosing and treating parotid neoplasms in children. PATIENTS AND METHODS: This retrospective study included all children who presented to the Children's Cancer Hospital Egypt (CCHE, 57357) with parotid masses from January 2008 to December 2020. RESULTS: Twenty-one patients were included. Malignant neoplasms were found in 12 (57.1%) of which mucoepidermoid carcinoma was the most common. Benign neoplasms were found in 6 (28.6%) all of them were pleomorphic adenoma, and non-neoplastic lesions were found in 3 (14.3%). Superficial, deep, or total parotidectomy was performed according to the involved lobes. The facial nerve was sacrificed in three cases because of frank invasion by the tumor. Neck dissection was considered in clinically positive lymph nodes and/or T3/4 masses. Complications occurred in 7 (33.3%) all were of the malignant cases. Adjuvant radiotherapy was restricted to high-risk cases (7 cases). Recurrence occurred in two cases, and one patient died of distant metastasis. Fine needle aspiration cytology (FNAC) showed 88.9% sensitivity and 100% specificity for diagnosing malignant neoplasms. The correlation of radiological and pathological staging was fair (66.74% for overall staging). CONCLUSIONS: Parotidectomy is the backbone treatment for benign and malignant pediatric parotid tumors. Neck nodal dissection should be considered after preoperative FNAC of suspicious nodes. Adjuvant radiotherapy is considered only in high-risk tumors. Preoperative FNAC of parotid masses and clinically suspicious lymph nodes is highly recommended.

Operative management and outcomes in children with pheochromocytoma.

OBJECTIVE: This study aimed to evaluate management and prognosis in children with pheochromocytoma who were treated at an Egyptian tertiary center. METHODS: The authors conducted an 8-year retrospective analysis for 17 patients who were presented from January 2013 to January 2021. Clinical criteria, operative details, and follow-up data were assessed. Overall (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier method. An event was assigned with the occurrence of recurrence or metachronous disease, or death. RESULTS: Median age at diagnosis was 14 years (range: 6-17.5 years). Ten patients (58.8%) were males and seven (41.2%) were females. Hypertension-related symptoms were the main presentations in 15 patients (88%). None of the included children underwent genetic testing. Sixteen patients (94%) had unilateral tumors (right side: 12), whereas only one was presented with bilateral masses. The median tumor size was 7 cm (range: 4-9 cm). Metastatic workup did not reveal any metastatic lesions. All patients underwent open adrenalectomy, and clinical manifestations were completely resolved after surgery. Adjuvant therapy was not administered to any patient. There were no deaths or relapses at a median follow-up time of 40 months, whilst two children had metachronous disease after primary resection. Both were managed by adrenal-sparing surgery, and they achieved a second complete remission thereafter. Five-year OS and EFS were 100% and 88%, respectively. CONCLUSIONS: Complete surgical resection achieves excellent clinical and survival outcomes for pheochromocytoma in children. Meticulous, long-term follow-up is imperative for early detection of metachronous disease to facilitate adrenal-sparing surgery. Genetic assessment for patients and their families is essential; however, it was not available at our institution.

Factors Associated with Failure of Pneumatic Reduction in Children with Ileocolic Intussusception.

Intussusception is one of the most common causes of intestinal obstruction in children. Pneumatic reduction is the treatment of choice and has a high success rate. The most common cause of pneumatic reduction failure is the presence of a pathological leading point. We aimed to identify other factors that can lead to pneumatic reduction failure in children with ileocolic intussusception. This was a retrospective study conducted in two centers. Data were collected from January 2013 to December 2014. A total of 156 patients were diagnosed with intussusception and underwent pneumatic reduction, with the exception of one patient with peritonitis. We included patients with ileocolic-type intussusception without apparent pathological leading points. Logistic regression analysis of clinical parameters was performed to identify factors associated with pneumatic reduction failure. Of 156 patients diagnosed with intussusception in both hospitals, 145 were enrolled in the study. The overall efficacy of pneumatic reduction was 85.7%, and surgical reduction was performed in 21 patients. Univariate analysis showed that a high segmented neutrophil count, low hemoglobin level, high body temperature, and higher weight percentile were associated with pneumatic reduction failure. Multivariate analysis showed that a high segmented neutrophil count, low hemoglobin level, and higher weight percentile were significantly associated with pneumatic reduction failure. Pneumatic reduction is safe and effective as a first-line treatment for pediatric intussusception. However, a high segmented neutrophil count, low hemoglobin level, and higher weight percentile are associated with the failure of this treatment.

Surgical Resection of Hepatoblastoma: Factors Affecting Local Recurrence.

INTRODUCTION: This study aimed to investigate potential factors contributing to local recurrence after surgical resection of hepatoblastoma (HB). MATERIALS AND METHODS: This retrospective study involving all patients with HB who underwent nontransplant surgery at our tertiary center between July 2007 and July 2018. Data were analyzed regarding microscopic surgical resection margin, tumor multifocality and extracapsular tumor extension in correlation with local recurrence. These relations were assessed by logistic regression. RESULTS: The study included 133 patients with a median age of 1.3 years (range: 0.5-12.8 years). They were classified into 99 cases (74.44%) standard risk and 34 cases (25.56%) high risk. Delayed surgical resection was adopted in all patients. Follow-up to July 2019 revealed that 23 patients (23/133, 17.3%) developed local recurrence, whereas the remaining 110 were locally disease free. Microscopic positive margin (R1) was detected in 29 patients, 8 of them had local recurrence (p = 0.097). Regarding tumor multifocality, there were 12 patients who had multifocal lesions, 3 of them developed local recurrence (p = 0.459). Forty-four patients had extracapsular tumor extension in their pathological reports, 12 of them had local recurrence (p = 0.032). CONCLUSION: Extracapsular tumor extension was a significant prognostic factor of local recurrence after surgical resection of HB. R1 margin does not necessarily require a second resection, and it could achieve accepted results when combined with adjuvant platinum-based chemotherapy. However, patients who are not eligible for surgical resection must be transferred for primary transplantation to obtain favorable outcome.

Management and outcome of pediatric Wilms tumor with malignant inferior Vena cava thrombus: largest cohort of single-center experience.

BACKGROUND: Wilms tumor (WT) with an inferior Vena cava (IVC) malignant thrombus comprises 4-10% of all WT cases. METHODS: This retrospective analysis included 51 pediatric patients presenting at Children Cancer Hospital Egypt-57357 from July 2007 to December 2016 with the diagnosis of WT with malignant IVC thrombus. RESULTS: Median age at presentation = 4.4 years and 28 cases (55%) were females. Twenty-five patients (49%) were metastatic and 4 patients (7.8%) had bilateral disease. Forty-seven cases (92.2%) had favorable histology with no evidence of anaplasia. Level of thrombus extension at presentation was classified as infra-hepatic, retro-hepatic, supra-hepatic and intra-cardiac in 33, 9, 6 and 3 patients, respectively. Fifty patients started neoadjuvant chemotherapy (CTH) with 16 patients showing complete resolution of thrombus after 6 weeks of CTH. None of the patients developed thrombus progression after neoadjuvant CTH; one patient had stationary intra-cardiac thrombus, while remaining patients showed partial regression of their thrombus and had nephrectomy with en-bloc thrombectomy. The mean cranio-caudal dimension of IVC thrombi at initial presentation was 6.5 cm, and 3.6 cm post 6th week of CTH. The 5-year OS and EFS were 75.9% and 71.1%, respectively. There was no significant correlation of initial levels of thrombus extension with survival. CONCLUSION: Neoadjuvant chemotherapy followed by radical nephrectomy with en-bloc thrombectomy and radiotherapy seems a successful approach for management of patients with WT and IVC tumor thrombus. Measurement of the cranio-caudal dimension of thrombus and its response to treatment should be considered in the surgical planning.

Clinical features and outcome of hepatosplenic fungal infections in children with haematological malignancies.

Hepatosplenic fungal infection (HSFI) is a severe invasive fungal infection observed during neutrophil recovery in patients with acute leukaemia treated with intensive chemotherapy. Retrospective analysis including all paediatric haematological malignancies patients with HSC treated in Children Cancer Hospital Egypt (2013-2018). Twenty-five patients with acute leukaemia developed HSFI (19 patients diagnosed as hepatosplenic candidiasis). Most of the cases (92%) occurred during the induction phase. Organs affected were as follows: liver in 18 patients, renal in 13 patients, spleen in 12 patients, skin in four patients and retina in one patient. Five (20%) patients had proven HSC, 14 (56%) probable and six (24%) possible HSFI. Ten patients had a PET-CT for response assessment. Candida tropicalis was the most common isolated spp. from blood/tissue culture. Six (24%) patients developed HSFI on top of antifungal prophylaxis. Steroids were given in 12 (52%) patients with HSFI as immune reconstitution syndrome (IRS). Caspofungin was the first line of treatment in 14 (56%) patients, liposomal amphotericin B in six (24%) patients and azoles in five (20%) patients. HSFI was associated with delayed of intensification phase of chemotherapy (median 42 days). The success rate was reported in 24 patients with complete response (68%) and partial response in (28%) patients, while failure (death) seen in 1(4%) patient. HSC is still a major challenge in paediatric leukaemias patients with impact on treatment delay and survival outcome. PET scan, non-culture diagnostics and steroid role evidence in IRS are growing. Antifungal stewardship for screening, early detection for high-risk patients and better response assessment is challenging.

Added predictive value of 18F-FDG PET/CT for pediatric rhabdomyosarcoma.

OBJECTIVE: To determine the prognostic value of quantitative fluorine-18-fluorodeoxyglucose (F-FDG) standardized uptake value (SUV) in patients with pediatric rhabdomyosarcoma (RMS). PATIENTS AND METHODS: Consecutive 98 (50 males and 48 females) (age range: 4 months to 17.5 years, mean age: 5.8 ± 4.5) patients with pathologically proven RMS who underwent PET/computed tomography for initial staging were retrospectively assessed for whether primary SUVmax and the primary/liver SUVmax ratio could predict event-free survival (EFS) and overall survival (OS) for 36 months using receiver operating characteristic curve analysis. Univariate and multivariate analyses were used to determine the reliability of these metabolic parameters and various clinical factors. RESULTS: Higher SUVmax was significantly related to the presence of regional or distant metastasis with worse prognosis. With receiver operating characteristic curve marked cut-off values of 3.6 and 2.1 for primary SUVmax and the primary/liver SUVmax ratio, respectively, both EFS and OS proved to be higher in patients with SUVmax ranked below the determinate values. Patients with a primary/liver SUVmax ratio below the cut-off value of 2.1 had OS (60.8%) and EFS (48.1%) compared with 44.5 and 14.8% for patients with lesions exceeding the cut-off point of uptake; however, this failed to achieve statistical significance. In the evaluation of primary SUVmax, similar results were obtained with P values of 0.76 and 0.62, respectively. High SUVmax was more prevalent among patients with less favorable clinical and pathological features including unfavorable primary site, alveolar pathology, and high-risk group. CONCLUSION: F-FDG PET/computed tomography may be considered an additional prognostic predictor of outcome in RMS patients, where higher F-FDG uptake seems to be linked to lower survival and correlated to different unfavorable parameters.

Surgery in perforated pediatric intestinal lymphoma.

BACKGROUND: Perforation is the most common surgical complication in pediatric intestinal lymphoma. During operation, many surgical decisions are debatable. AIM: To assess the outcome of surgical management of perforated pediatric intestinal lymphoma. PATIENTS AND METHODS: This is a retrospective analysis of all pediatric patients (<18 years old) with intestinal lymphoma treated in our hospital between July 2007 and June 2017. Risk factors for perforation, type of management and outcome in cases of intestinal perforation were analyzed. RESULTS: The study included 240 patients with intestinal lymphoma. Perforation developed in 16 patients (6.7%) with a median age of 5.3 (range: 2.8-15.7) years. Most of the patients (92.5%) had Burkitt lymphoma. The ileum was the most common site of perforation (n = 10). Perforation occurred at presentation (n = 2), during induction (n = 10), during maintenance chemotherapy (n = 2), or at relapse (n = 2). Primary resection anastomosis was done in 12 patients. The resected specimen showed a viable tumor in ten patients. Wound infection (25%) and dehiscence (12.5%) were the most common postoperative complications. The 5-year overall and event-free survivals of patients with perforation were 78.6% and 71.4%, respectively, compared with 85.5% and 81.2% in non-perforated patients; the difference was not significant (p = 0.374 and p = 0.270, respectively). CONCLUSION: Perforation is not an adverse prognostic factor for survival in pediatric intestinal lymphoma patients. Primary resection anastomosis seems to be a safe option if complete tumor resection is feasible.

Effect of total contact cast in non-healing diabetic foot ulcers in Lebanese patients.

The aim of this study was to evaluate the effectiveness of total contact casting (TCC) in treating non-healing diabetic foot ulcers in Lebanese diabetic patients. Twenty-three diabetic patients were treated with TCC, and relevant data were collected retrospectively. Sixteen patients were analysed; the average duration of casting was 6 weeks, and 75% of the patients achieved complete ulcer closure without recurrence during one year of follow-up and without any complications. TCC appears to be an effective treatment of diabetic foot ulcers.

Thoracoscopic Approach for Repair of Diaphragmatic Hernia Occurring After Pediatric Liver Transplant.

Diaphragmatic hernias (DH) occurring after pediatric liver transplantation (LT) are rare. However, such complications have been previously reported in the literature and treatment has always been surgical repair via laparotomy. We report our experience of minimally invasive thoracoscopic approach for repair of DH occurring after LT in pediatric recipients.From April 2010 to December 2014, 7 cases of DH were identified in pediatric LT recipient in Samsung Medical Center. Thoracoscopic repair was attempted in 3 patients. Patients' medical records were retrospectively reviewed.Case 1 was a 12-month-old boy, having received deceased donor LT for biliary atresia (BA) 5 months ago. He presented with dyspnea and left-sided DH was detected. Thoracoscopic repair was successfully done and the boy was discharged at postoperative day 7. Case 2 was a 13-month-old boy, having received deceased donor LT for BA 2 months ago. He presented with vomiting and right-sided DH was detected. Thoracoscopic repair was done along with primary repair of herniated small bowel that was perforated while attempting reduction into the peritoneal cavity. The boy recovered from the surgery without complications and was discharged on the 10th postoperative day. Case 3 was a 43-month-old girl, having received deceased donor LT for Alagille syndrome 28 months ago. She was diagnosed with right-sided DH during steroid pulse therapy for acute rejection. Thoracoscopic repair was attempted but a segment of necrotic bowel was noticed along with bile colored pleural effusion and severe adhesion in the thoracic cavity. She received DH repair with small bowel resection and anastomosis via laparotomy.Thoracoscopic repair was attempted in 3 cases of DH occurring after LT in pediatric recipients. With experience and expertise in pediatric minimally invasive surgery, thoracoscopic approach is feasible in this rare population of patients.

Bilateral clear cell sarcoma of the kidney.

Clear cell sarcoma of the kidney (CCSK) accounts for 2-5% of all pediatric renal malignancies, and is known for its propensity to metastasize to bone and other sites. We are reporting two cases with bilateral CCSK that were diagnosed at our institution. One patient initially presented with bilateral renal masses, as well as pulmonary, hepatic and bone metastasis; while other present only with bilateral masses with no evident distant metastasis. Both patients received aggressive neo-adjuvant chemotherapy to decrease tumor size. One patient completed his designated treatment and initially showed complete remission (CR); eventually suffering from relapse. The other patient's tumor progressed during the course of chemotherapy. Both cases manifested brain dissemination at the time of relapse or progression. This emphasizes the importance of staging stratification in CCSK. This also illustrates CCSK's ability to metastasize to bone and other sites including the brain (a primary relapse site in our cases).

Clinical significance of anaplasia in childhood rhabdomyosarcoma.

BACKGROUND: The presence of anaplastic features has been known to correlate with poor clinical outcome in various pediatric malignancies, including Wilms tumor and medulloblastoma but not in rhabdomyosarcoma. AIM: Aim was to study the frequency of anaplasia at presentation in childhood rhabdomyosarcoma and its relationship to clinical and pathological characteristics as well as to outcome. PATIENTS AND METHODS: Anaplasia was retrospectively assessed in 105 consecutive pediatric rhabdomyosarcoma patients who were registered at the Children's Cancer Hospital in Egypt (CCHE) during the period from July 2007 till the end of May 2010. RESULTS: Anaplasia was diagnosed in 18 patients (17.1%), focal in 10 (9.5%) and diffuse in 8 (7.6%). The distribution of anaplasia was found to be more common in older patients having age⩾10 years. Also it was more likely to occur in the high risk group and in tumors with unfavorable histology (alveolar subtype), and stage IV. The 3-year failure free survival rates for patients with and without anaplasia were 27.8±10.6% and 53.4±5.8%, respectively (p=0.014) and the 3-year overall survival rates were 35.3±11.6% and 61±6%, respectively (p=0.019). CONCLUSIONS: The frequency of anaplasia in pediatric patients with rhabdomyosarcoma in our study was 17.1%. The presence of anaplasia had statistically significant worse clinical outcome.

Clear cell sarcoma of the kidney: patients' characteristics and improved outcome in developing countries.

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor accounting for 5% of pediatric renal tumors with an incidence of 20 patients per year in the USA. It is bone metastasizing with poor prognosis. Our aim was to show characteristics of patients in relation to improved outcome in one of the developing countries. PROCEDURE: We included all patients diagnosed as CCSK in the period between July 2007 and March 2012 at Children's Cancer Hospital, Egypt. Patients' demographics, clinical presentation, pathology, and management were reviewed. Follow up was continued until April 2013. RESULTS: Twenty-five patients were identified in the defined time interval, accounting for 7% all renal tumors diagnosed at the hospital. Mean age was 36 months. Abdominal swelling and hematuria were the most common presentations. Stages I, II, III, IV, and V represented 9 (36%), 3 (12%), 8 (32%), 3 (12%), and 2 (8%), respectively. Twenty-four patients had radical nephrectomy either upfront or after neo-adjuvant chemotherapy. Surgery was followed by adjuvant chemotherapy. Abdominal radiotherapy was given for local stages II and III. Twenty-two patients reached complete remission, while one patient had stationary disease and two patients died due to progression and relapse. Overall survival was 88.5% and event-free survival was 87.8% at 45 months. CONCLUSION: Although previous studies indicate poor prognosis of CCSK, our experience shows that those patients can be treated using extensive chemotherapy combined with proper local control.