Congenital pancreatoblastoma: a case report

The literature describes 15 cases of congenital pancreatoblastoma (PB): 5 had prenatal diagnosis, none had metastases at diagnosis, 7 were associated with BeckwitheWiedemann syndrome (BWS). In 13 cases resection was radical, while in 2 there were macroscopic residues. Only one patient underwent chemotherapy after distant recurrence. All children are alive except one who died because of problems related to BWS. Our goal is to describe the approach adopted in an infant with congenital PB treated in our center. After a prenatal third semester diagnosis of abdominal anechoic lesion, the radiological investigations (ultrasound, MRI) performed at birth described a cystic lesion of unclear nature. We proceeded to laparoscopic exploration, transformed into open approach after the detection of a lesion located in the body of the pancreas; this lesion was resected, preserving the head and tail of pancreas. The histological diagnosis showed a completely excised PB. After excluding metastatic lesions, we decided to perform only careful follow-up without chemotherapy. The follow-up at 12 months is negative. Although PB is a malignant tumor that requires a multidisciplinary treatment, the congenital cases seem to have a less aggressive biological behavior. The treatment, therefore, in case of complete resection, could be only surgical, followed by a careful follow-up. These forms are often associated with congenital BWS, but in our case the patient did not have the typical characteristics of the syndrome

Could a careful clinical examination distinguish physiologic phimosis from balanitis xerotica obliterans in children?

The diagnosis of balanitis xerotica obliterans (BXO) in children may be challenging, since clinical examination only could lead to an underestimation of its incidence. The aim of this retrospective and single-centre study is to assess the diagnostic performance of clinical examination, together with clinical history, in identifying BXO. Ninety-seven children underwent circumcision for phimosis from 2015 to 2019. Histology was routinely performed. Cohen\u2019s kappa coefficient, sensitivity, specificity, predictive values, likelihood ratios and accuracy of macroscopic appearance of the foreskin, steroid administration and past medical history were estimated. Forty-eight patients (50%) were affected by BXO; 31 of them (69%) presented with suggestive clinical signs. A strictured or whitish urethral meatus was detected during surgery in nine cases (19%); this was associated to allergic or immune diseases (p = 0.046). Foreskin appearance alone mildly correlated with histology (k = 0.494; p < 0.001) and it showed a diagnostic accuracy of 75%. The specificity and positive predictive value of abnormal macroscopic findings at examination, together with a positive clinical history for other allergic or immune diseases, and/or for balanitis, were 100% and the positive likelihood ratio was greater than 10. Conversely, sensitivity decreased to 4.5% (95% CI 0\u201311%). Conclusion: Foreskin appearance together with clinical history could predict BXO with certainty. However, since the absence of a positive medical history could not exclude the diagnosis, foreskin histology is still highly recommended

VY anoplasty for ectropion of anal mucosa in an adult with anorectal malformation

Ectropion of anal mucosa (AME) is a possible complication following anorectal surgery and it is often cause of symptoms such as soiling, pain and bleeding affecting the patients' quality of life. Two-flaps anoplasty has been previously described for the correction of AME. We herein report a case of an adult patient who underwent a successful double V/Y-flaps for mucosal ectropion as consequence of repair of anorectal malformation (ARM).A 25 year-old woman with history of ARM presented with an extensive AME causing bleeding, pain, soiling, and dyspareunia. This condition prevented her from an effective rectal nursing and a satisfactory social life.The patient underwent the resection of ectopic mucosa and the creation of two V-shaped skin flaps to cover the skin gap. A minor dehiscence of one of the flaps was treated conservatively. At two years follow up the patient is symptoms free, has a good quality of life and can perform an effective bowel management.AME is a possible complication after anorectal surgery for ARM and the multiple V-Y plasty is a viable procedure for its treatment. A multidisciplinary approach in these cases is recommended.Level of Evidence V. Keywords: Anorectal malformation, Anoplasty, V-Y flaps, Mucosal ectropio

Spleen histology in children with sickle cell disease and hereditary spherocytosis: Hints on the disease pathophysiology

open2Hereditary spherocytosis (HS) and sickle cell disease (SCD) are associated with splenomegaly and spleen dysfunction in pediatric patients. Scant data exist on possible correlations between spleen morphology and function in HS and SCD. This study aimed to assess the histological and morphometric features of HS and SCD spleens, in order to get possible correlations with disease pathophysiology. In a large series of spleens from SCD, HS and control patients the following parameters were considered: (i) macroscopic features; (ii) lymphoid follicle (LF) density; (iii) presence of peri-follicular marginal zones (MZs); (iv) presence of Gamna-Gandy bodies; (v) density of CD8-positive sinusoids; (vi) density of CD34-positive microvessels; (vii) presence/distribution of fibrosis and SMA-positive myoid cells; (viii) density of CD68-positive macrophages. SCD and HS spleens have similar macroscopic features. SCD spleens had lower LF density and fewer MZs than HS spleens and controls. SCD also showed lower CD8-positive sinusoid density, increased CD34-positive microvessel density and SMA-positive myoid cells, and higher prevalence of fibrosis and Gamna-Gandy bodies. HS had lower LF and CD8-positive sinusoid density than controls. No significant differences were noted in red pulp macrophages. By multivariate analysis, the majority of HS spleens clustered with controls, while SCD grouped separately. A multi-parametric score could predict the degree of spleen changes irrespective of the underlying disease. In conclusion, SCD spleens display greater histologic effacement than HS and SCD-related changes suggest impaired function due to vascular damage. These observations may contribute to guide the clinical management of patients.embargoed_20161128Alaggio, RitaAlaggio, Rita; Gamba, Piergiorgi

Robot-assisted splenectomy in a teenager with chronic autoimmune thrombocytopenia

The use of the Da Vinci Xi system is gaining popularity among all surgical disciplines. A splenectomy is a treatment option for patients with hematological disorders and splenic lesions. The laparoscopic approach is nowadays the standard of care. Despite the initial controversy, recently it has been demonstrated the superiority of robotic splenectomy performed in ''difficult'' cases. We report, to our knowledge, the first case of robot-assisted splenectomy following embolization of the splenic artery in a 15-year-old patient with chronic immune thrombocytopenia, worsened by a severe cerebral sinus thrombosis, while being treated with eltrombopag and mycophenolate. Due to the need for a rapid rise in platelet counts and failure of several medical treatments, splenectomy was advocated. To raise the platelet count pre-operatively and minimize intraoperative bleeding, the embolization of the spleen artery was performed before the planned splenectomy. The intervention was carried on without any complication and at 1 year follow up the patient is in good clinical condition and has improved his neurological condition. We propose a robotic splenectomy following embolization of the splenic artery as a feasible and safe procedure. The advantages of the Da Vinci Xi system are highlighted especially in complex cases, requiring maximum precision

Decellularized diaphragmatic muscle drives a constructive angiogenic response in vivo

Skeletal muscle tissue engineering (TE) aims to efficiently repair large congenital and acquired defects. Biological acellular scaffolds are considered a good tool for TE, as decellularization allows structural preservation of tissue extracellular matrix (ECM) and conservation of its unique cytokine reservoir and the ability to support angiogenesis, cell viability, and proliferation. This represents a major advantage compared to synthetic scaffolds, which can acquire these features only after modification and show limited biocompatibility. In this work, we describe the ability of a skeletal muscle acellular scaffold to promote vascularization both ex vivo and in vivo. Specifically, chicken chorioallantoic membrane assay and protein array confirmed the presence of pro-angiogenic molecules in the decellularized tissue such as HGF, VEGF, and SDF-1\u3b1. The acellular muscle was implanted in BL6/J mice both subcutaneously and ortotopically. In the first condition, the ECM-derived scaffold appeared vascularized 7 days post-implantation. When the decellularized diaphragm was ortotopically applied, newly formed blood vessels containing CD31+, \u3b1SMA+, and vWF+ cells were visible inside the scaffold. Systemic injection of Evans Blue proved function and perfusion of the new vessels, underlying a tissue-regenerative activation. On the contrary, the implantation of a synthetic matrix made of polytetrafluoroethylene used as control was only surrounded by vWF+ cells, with no cell migration inside the scaffold and clear foreign body reaction (giant cells were visible). The molecular profile and the analysis of macrophages confirmed the tendency of the synthetic scaffold to enhance inflammation instead of regeneration. In conclusion, we identified the angiogenic potential of a skeletal muscle-derived acellular scaffold and the pro-regenerative environment activated in vivo, showing clear evidence that the decellularized diaphragm is a suitable candidate for skeletal muscle tissue engineering and regeneration

Pediatric Surgery and Anesthesia in Low-Middle Income Countries: Current Situation and Ethical Challenges

Low-middle income countries (LMICs) are currently experiencing an important population growth, leading to a substantial raise in the number of children living in those areas. As a consequence, the existing gap between the need for surgical and anesthetic care and the available therapeutic options will increase. To overcome this, an improvement in the available expertise, infrastructures, and supplies will be mandatory. The implementation of educational and training programs for local healthcare providers should be a top priority. Alongside, the population's awareness on the necessity to seek for medical care should be deployed, together with an eased access to health facilities. Based on the existing literature and our 20-years' experience in humanitarian missions, our article aims to investigate the status of pediatric surgery in LMICs, and the role of western aids in the implementation of this ever-increasing field of expertise

3D extracellular matrix derived model of alveolar rhabdomyosarcoma

INTRODUCTION: Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood, among the subtypes the Alveolar (ARMS) is the more aggressive with a higher tendency to metastasize [1]. Integrins are a class of transmembrane adhesion molecules that mediate survival, differentiation, migration and differentiation [2]. Here we investigate the role of integrins in ARMS metastatic migration in an engineered 3D scaffold. METHODS: ARMS xenografts are obtained from subcutaneous injection of RH30 cells in immunodeficient mice. Composition of the ECM is determined by proteomic analysis. The main components of the ECM are used to enrich a 3D collagen scaffold cultured in a perfusion bioreactor. Cells are analyzed by qPCR for the expression of a panel of integrins. Presence of the protein is confirmed by flow cytometry immunofluorescence. MMPs expression is evaluated by zymography. RESULTS: Verified the expression of human and ARMS marker and typical tumor morphology in xenografts, they are processed for proteomic analysis. Proteomic data analysis is currently under investigation. Preliminary data culturing RH30 cells in 3D bioreactor show upregulation of ITG5 and CXCR4 receptor compared to 2D condition. Localization and quantification at protein level will be assessed respectively by immunofluorescence and cytofluorimetry. Expression of MMP-9 and MMP-2 has been assessed by zymography comparing the expression of these MMPs in 2D vs 3D bioreactor and RH30 isolated from the xenograft. DISCUSSION & CONCLUSIONS: Preliminary data on ITG expression show that in 3D scaffold the expression of ITG5 and CXCR4 is upregulated. In parallel the active form of MMP-2 is more present in 3D models compared to 2D. Other groups reported a mechanical interaction between ITG5 and MMP-2 [3]. This interaction will be studied in a more representative engineered 3D scaffold to shed light on the complex interaction between ECM and metastatic progression

Combination antifungal therapy and surgery for the treatment of invasive pulmonary aspergillosis after hematopoietic stem cell transplantation

An 8-year old boy, affected by severe aplastic anemia, developed a probable pulmonary invasive aspergillosis (IA) early after a second unrelated allogeneic hematopoietic stem cell transplant (HSCT). He was treated promptly with the combination of liposomal amphotericin B and caspofungin. Despite the initial stabilization, the patient deteriorated and the antifungal therapy was switched to voriconazole and caspofungin. The patient gradually improved and was discharged home on day +29 post-HSCT on oral voriconazole. On day +119, a sudden episode of hemoptysis occurred and a right superior lobectomy was decided to remove the residual aspergilloma. The patient is now alive and well more than 24 months from HSCT. This case demonstrated that antifungal combination therapy and surgery are valid options to cure pulmonary IA even in patients at high-risk and severely immunosuppressed