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Management and successful use of vascular access are critical issues in pediatric patients affected by malignancies. Prolonged course of disease, complex and various treatment protocols require long-lasting vascular access providing adequate tools to administrate those therapies and to collect routine blood sampling without painful and repeated venipuncture. For these reasons, central venous catheters are currently an important component in pediatric onco-hematological care, with a direct influence on outcome. Indeed, there are peculiar issues (techniques of insertion, management, complications etc.) which must be well-known in order to improve the outcome and the quality of life of children with cancer.

We describe treatments of acute appendicitis at "Bambino Gesù" Children's Hospital during the peak of the Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) pandemic in Italy.From March 1st to May 31st, all suspected appendicitis admitted to hospital entered this study. Following Institutional COVID19-protocol, between March 1st-21st, only patients with respiratory symptoms and/or history of recent travel to risk areas received nasopharyngeal swab. From March 22nd to May 31st, protocol was adapted to worsening epidemic conditions and a pre-triage area has been arranged to accommodate all patients undergoing the swab.14 out of 53 patients were hospitalized between march 1st-21st, 39 from march 22nd to may 31st. swab was performed in 2 patient of first group and in all of second.During the study period, no covid19-contagion occurred in hospital staff by covid19-patients.Our covid19-protocol protected staff and patients allowing the maintenance of our standard of treatment.Appendicitis, Children, Covid19, Sars-cov-2.L’obiettivo del nostro studio è stato quello di descrivere il trattamento dei pazienti giunti presso presso l’Ospedale Pediatrico Bambino Gesù, affetti da appendicite acuta, durante il picco della pandemia da Severe Acute Respiratory Syndrome- Coronavirus 2 (SARS-CoV-2) in Italia.Sono stati presi in considerazioni tutti i pazienti giunti presso il nostro Ospedale affetti da appendicite acuta tra il 1° marzo ed il 31 maggio. Seguendo il protocollo del nostro Ospedale per il COVID19, tra il 1° e il 21 marzo, solo i pazienti con sintomi respiratori e/o storia di viaggi recenti in aree a rischio sono stati sottoposti a tampone nasofaringeo. Dal 22 marzo al 31 maggio, il protocollo è stato adattato al peggioramento delle condizioni epidemiche ed è stata predisposta un’area pre-triage per accogliere tutti i pazienti sottoposti al tampone in attesa del risultato.14 pazienti su 53 sono stati ricoverati tra l’1 e il 21 marzo, 39 dal 22 marzo al 31 maggio. Il tampone è stato eseguito in 2 pazienti del primo gruppo ed in tutti quelli del secondo. DISCUSSIONE: durante il periodo preso in considerazione per il nostro studio, nessun contagio COVID19 si è verificato nel personale ospedaliero da parte dei pazienti COVID19.Questo studio conferma come il nostro protocollo per il COVID19 ha protetto sia il personale che i pazienti consentendo il mantenimento del nostro standard di trattamento.

Current pectus excavatum management includes a Computed Tomography scan to evaluate the Correction Index, whose superiority to the Haller Index in terms of specificity and sensibility is still under debate. Furthermore, several studies report interchangeability between the Haller Index as measured by Computed Tomography and as measured by X-radiograph; however it is not clear whether this correlation also exists for the Correction Index. The aim of our study was to evaluate the correlation between measurements of the Haller Index and the Correction Index obtained by Computed Tomography and by X-radiograph.This prospective study included 31 patients with Pectus Excavatum (who underwent preoperative chest Computed Tomography and X-radiograph) and a control group of 31 patients (who presented no chest deformity and underwent the same exams for other unrelated causes). We measured Haller Index and Correction Index on both exams for both groups. To demonstrate any correlation between Computed Tomography scans and X-radiographs for the two indexes, the Pearson R correlation test, Bland-Altman analysis and Anova Nested test were performed.Pearson's coefficient (0.829 with p0.0001) and Anova Nested test showed a significant correlation and similar results between the Haller Index and the Correction Index on Computed Tomography and on X-radiograph.Significant correlation and similar results are shown in our study in the measurement of CI and HI on Computed Tomography and X-radiograph. Further studies including a larger number of patients may be warranted.

Surgical excision of hyoid bone is the mainstay of thyroglossal duct cyst removal, to prevent recurrences. Bone excision by cut - bone forceps may prove inaccurate and somewhat dangerous. Therefore, use of alternative methods has been advocated.The proposal of the study is to cut the body of the hyoid bone with the only electric scalpel. Surgical casenotes of 57 pediatric patients operated by our Institution with modified Sistrunk technique and complete dissection of the entire body of hyoid bone and its removal at the cartilage junction with lateral horn with monopolar cauterization were reviewed.The population of our study had a mean age of 59.86 ± 29.57 months; 6/57 patients (10.5%) were under 2 years of age. Mean follow up was 55,04 ± 29,08 months ranging 2 to 115 months. In any patients post-operative bleeding was observed. Mean surgical time was 57,02 ± 11,77 minutes ranging from 35 to 125 minutes; 6 cases required postoperative drain. In 3 patients an immediate local edema was observed and in another one a partial wound dehiscence occurred, no late complications were observed. Cyst-recurrence requiring re-do surgery occurred in 5 patients (8.8%).The complete hyoid bone section with electric scalpel only ensures the ideal access to the posterior hyoid space and allows a greater and more symmetrical access to the proximal portion of thyroglossal duct in children with still negligible complication and recurrence rate.Modified Sistrunk technique, Sistrunk procedure, Thyroglossal duct cyst.L’escissione chirurgica del corpo dell’osso ioide rappresenta un passaggio fondamentale in corso di intervento per cisti mediana del collo al fine di ridurre il tasso di recidiva. L’incisione dell’osso tramite pinza ossivora di Liston può risultare incompleta e, in alcuni casi, pericolosa; a tal fine, sono stati quindi messi a punto metodi alternativi. L’obiettivo dello studio è di valutare l’efficacia in termini di recidive e complicanze chirurgiche dell’ asportazione del corpo dell’osso ioide tramite il solo utilizzo della diatermia monopolare. Sono stati revisionati i dati relativi a 57 pazienti di età pediatrica operati consecutivamente presso il nostro Ospedale con tecnica di Sistrunk modificata: la dissezione del corpo dell’osso ioide è stata realizzata mediante diatermia monopolare anche a livello della cartilagine di accrescimento tra il corpo ed i corni laterali dell’osso ioide La popolazione in esame presentava un’età media di 59.86 +/- 29.57 mesi; 6 pazienti su 57 (10.5%) avevano un’età inferiore a 2 anni. Il follow-up medio è stato di 55.04 ± 29.08 mesi (range 2-115 mesi). In nessun paziente è stato evidenziato sanguinamento post-operatorio. Il tempo chirurgico medio è stato di 57.02 +/- 11.77 minuti (range 35-125 minuti); in 6 casi è stato posizionato drenaggio. Tre pazienti hanno mostrato immediato edema post-operatorio ed uno una parziale deiescenza della ferita chirurgica; non sono state osservate complicanze post-operatorie tardive. In 5 casi (8.8%) è stato necessario un re-intervento per recidiva. La completa escissione del corpo dell’osso ioide tramite diatermia monopolare assicura una visuale completa dello spazio retroioideo e consente un accesso migliore e più simmetrico alla porzione prossimale del dotto tireoglosso in età pediatrica, con un tasso trascurabile di complicanze e recidive.

Summary Background Male patients treated for anorectal malformations (ARM) and recto-urethral fistula (RUF) tend to develop recurrent epididymo-orchitis (EO) which occurs approximately in 20% of all them. The optimal management of this condition is unclear because of the extreme its rarity and the unavailability of detailed analysis in literature. To date the majority of this patients benefits from medical treatment and symptoms reduce over time but few data have been published in literature about management of patients with intractable EO. Objective To describe the efficacy of unilateral vasectomy in patients operated on for anorectal malformations with RUF and affected by intractable EO. Study design We present five patients who met the criteria for intractable EO, and followed at our centre four of whom have undergone unilateral vasectomy. Results The first episode of EO presented at 42,00 mos ±29.39. Initially, patients were all managed with analgesics and antibiotics. For the failure of therapy, five patients were all offered unilateral vasectomy but only four families accepted procedure. Surgical treatment was performed as a day case without complications. Postoperative follow up was 88,50 mos ±68.36. Prompt and durable resolution of symptoms was observed. Discussion The long-term effects of recurrent EO in ARM are often underestimated. Prompt and appropriate intervention should prevent this undesirable sequela. Unfortunately, the optimal management of this complication is unclear, partly because of its extreme rarity. The established management needs to follow the route of correcting underlying anomalies and providing long-term analgesic and antibiotics but this may have undesired side effects. We therefore offered families vasectomy for complete symptom resolution and/or drug withdrawal. Vasectomy, as a form of treatment for, can be justified if it can prevent pain, infection and destruction of the testes. Early vasectomy may save enough functional testis tissue. Conclusion To date, the only available treatment to achieve definitive resolution of symptoms in intractable unilateral EO is vasectomy. Long-term effects of such procedure on fertility are unknown. The treatment of recurrent EO in cases without site predilection remains a matter of contention. Summary Table . Main features of case patients. Age at recruitment (mos) Age at first EO (mos) Medical Management UEDR at VCUG Unilateral Vasectomy Side of Vasectomy Age at UV (mos) Δ first EO-UV Symtoms Resolution PO FU (mos) A 24 24 12 mos “on demand” AB and AG Yes Yes Left 36 12 Yes 63 B 84 72 6 mos AB prophylaxis No Yes Left 92 20 Yes 60 C 3 72 12 mos “on demand” AB and AG Yes Yes Right 84 12 Yes 190 D 3 6 12 mos “on demand” AB and AG No Yes Left 20 14 Yes 41 E 3 36 12 mos “on demand" AB and AG + 4mos AB prophylaxis No No ∖ ∖ ∖ No ∖ Abbreviations: AB: antibiotics AG: analgesics; UEDR urethra-ejaculatory duct reflux; VCUG: voiding cystourethrography; UV unilateral vasectomy; Δ interval of time; PO postoperative; FU follow up.

Male genitalia reconstruction in patients with exstrophy-epispadias complex often leads to secondary hypospadias, thus requiring additional surgery and resulting in possible complications. We describe a technique of male epispadias repair to prevent resultant hypospadias.The complete penile disassembly technique is modified by multiple Z-plasties, which enable creation of a funnel-like bladder neck and urethral plate lengthening. A total of 29 patients 2 months to 12 years old presenting with pure epispadias and bladder exstrophy were treated with this technique between 2004 and 2011. These patients were compared to 19 patients 1 day to 11 years old with exstrophy-epispadias treated with the standard penile disassembly technique between 2000 and 2004.Outcome measures were urethral orifice position, penile length and complications. There was no statistically significant difference between the 2 groups regarding penile length or complications. In the study group the urethra reached the tip of the glans in all patients, whereas the creation of intentional hypospadias was necessary in 6 patients (31.5%) in the control group (p0.05).The modified technique was effective in obtaining appropriate meatal location without decreasing the penile length. The complication rate was not changed. Creation of resultant hypospadias at genitalia reconstruction in male epispadias should strongly be prevented.

Microscopic neoplastic thrombosis (MNT) is reported to occur frequently in Wilms tumour (WT). The aim of this study is to determine whether MNT influences prognosis in localised WT.Records and slides of 80 consecutive, unselected, localised WT patients were retrospectively reviewed. All patients received chemotherapy before surgery according to SIOP Protocol. The median follow-up was 9 years (range 0.5-25.8). The Kaplan-Meier method and the Cox proportional hazard model were applied.MNT was present in 14 (18%) cases. Out of 14 patients with MNT, 6 presented macroscopic thrombosis and 5 had either blastemal predominance or anaplastic histology. The 5-year overall survival (OS) and progression-free survival (PFS) for the whole population were 95% (95% confidence interval, CI, 87-98%) and 91% (95% CI 82-96%), respectively. The 5-year OS and PFS for MNT positive patients were 92% (95% CI 57-99%) and 77% (95% CI 44-92%), while the 5-year OS and PFS for MNT negative patients were 96% (95% CI 87-99%) and 94% (95% CI 85-98%), respectively; the difference was statistically significant (p0.05) for PFS. In multivariate analysis, only the presence of anaplasia retained significance with a hazard ratio (HR) of 14.8 and 12.9 (p0.05) for recurrence and death, respectively.These data suggest that the presence of MNT increases the risk of recurrence. MNT is associated with well-known prognostic factors, such as macroscopic thrombosis (possibly representing regression of macroscopic involvement) and anaplasia. Further prospective studies are needed to clarify the role of MNT as independent prognostic factor.

Objective The optimal management of congenital adenomatoid malformation of the lung remains controversial. Prenatal ultrasonographic analysis has increasingly discovered asymptomatic lesions, raising questions about the need for and timing of surgical treatment for asymptomatic congenital adenomatoid malformation. The aim of our study was to analyze the short-term postoperative outcome of symptomatic congenital adenomatoid malformations compared with asymptomatic malformations. Methods All the data of patients presenting with congenital adenomatoid malformations histologically diagnosed and operated on between 1998 and 2005 at our institution were retrospectively reviewed. Patients were divided into 2 groups: group A comprised asymptomatic infants, and group B comprised symptomatic infants. Major outcomes considered were the length of ventilation, pleural drainage, and hospital stay. Postoperative morbidity and mortality were also evaluated. Asymptomatic patients were further stratified for age at the time of the operation to evaluate whether age at surgical intervention affects the outcome. The Fisher's exact and Mann–Whitney tests were used as appropriate. Results Fifty-seven patients were consecutively treated. Thirty-five patients were given diagnoses of asymptomatic lesions and were enrolled into group A, whereas 22 patients presenting with symptoms were entered into group B. The lengths of ventilation, pleural drainage, and hospital stay were significantly longer in patients with symptomatic congenital adenomatoid malformations. Moreover, symptomatic patients presented with a higher postoperative complication rate. The age-based stratification of asymptomatic children did not show any difference on either postoperative mortality or major outcome considered. Conclusion Children with congenital adenomatoid malformations operated on when asymptomatic present a better short-term outcome than symptomatic children. In addition, age at the time of the operation does not negatively affect the outcome. Our findings support early surgical treatment for asymptomatic congenital adenomatoid malformation.

Sickle Cell Anaemia (SCA) is the most common inherited blood disorder and is associated with severe morbidity and decreased survival. Allogeneic Haematopoietic Stem Cell Transplantation (HSCT) is the only curative approach. Nevertheless the decision to perform a marrow transplant includes the risk of major complications and mortality transplant related. The infections represent the main cause of mortality for SCA patients undergoing transplant. Invasive Pulmonary Aspergillosis (IPA) is a devastating opportunistic infection and remains a significant cause of morbidity and mortality in HSCT recipients. Data regarding IPA in the setting of SCA are lacking. In the present report, we describe a patient with SCA who developed IPA after allogeneic bone marrow transplant. The fungal infection was treated by systemic antifungal therapy in addition to the surgery, despite mild chronic GVHD and with continuing immunosuppression therapy. This case shows that IPA occurring in bone marrow recipient with SCA can be successful treated

Background/Purpose Neurologically impaired (NI) children have an increased incidence of gastroesophageal reflux and many will require surgery. Methods The case notes of 50 NI children who underwent total oesophagogastric dissociation (TOGD) were reviewed. Thirty-four were done as a primary procedure, and 16 were rescues for failed fundoplications. Results There was no operative mortality. Morbidity consisted of 1 subphrenic collection, 1 oesophagojejunal dehiscence and 2 stenoses that responded to dilatation, and 2 bowel obstructions. In 1 case, partial gastric resection was needed because of transhiatal herniation of stomach. Gastrostomy feeding was established by 3 to 5 days. The mean hospital stay was 10.9 days. At 4 months to11 years of follow-up, there was no recurrence of reflux. Children who could swallow enjoyed oral feeds. Their general health and weight SD scores improved. Food aspiration, chest infections, and hospitalizations were reduced, with an improvement in quality of life. There were 5 late deaths in the "primary" and 7 in the "rescue" group from deterioration in their original condition. Conclusion Total oesophagogastric dissociation is a safe and versatile procedure without immediate mortality and limited surgery-related morbidity. Review of our practice suggests TOGD should be considered as a primary procedure in severely NI children with gastroesophageal reflux and significant oropharyngeal incoordination and dependence on enteral tube feeding. Rescue TOGD carries a greater morbidity because of previous surgery with consequent difficult dissection, poor oesophageal tissue, and higher incidence of vagal nerve injury.