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We reviewed our experience with renal transplantation (RTx) in children weighing

We retrospectively reviewed the records of 24 consecutive patients undergoing treatment for ureteral complications after RTx in the period 2001-2012 to determine the timing of presentation of the complications, and their open or endoscopic management. Three patients (12%) had a necrosis of the transplanted ureter soon after RTx. All required open urinary diversion in a native ureter. Ten cases (42%) developed ureteral obstruction. Time of presentation was variable mainly in relation to the underlying cause. Endoscopic treatment was successful in two cases with urinary stones and open surgery in two with mid-ureteral obstruction. Six patients had VUJ stenosis, three underwent open reimplantation, whereas temporary double-J stent placement was successfully performed in the remainder. Eleven patients (46%) had VUR. It seldom presented in the first year after RTx. Endoscopic treatment was attempted in all and was successful in all the six cases without vs. only one of the five with lower urinary tract pathology (p = 0.01). Endoscopic treatment is an option in patients with VUR in the absence of lower urinary tract pathology. It is an option also for the treatment of stones and can be attempted in case of VUJ stenosis. Ureteral necrosis always requires open treatment.

Occlusion of traditional sites for central venous cannulation is a challenging problem in patients that require a permanent central venous line for chronic administration of nutrients or drugs. In rare cases, extensive central venous thrombosis of the superior and inferior vena cava may preclude catheterization, and uncommon routes should be used. We describe our approach for placement of chronic central venous lines in two pediatric patients with short bowel syndrome and extensive caval occlusion.

PURPOSE The umbilical venous catheter (UVC) is routinely used in neonatal intensive care. Incorrect placement may expose the patient to some dangerous complications (i.e. thrombosis, abscesses, etc). We report a case of an hepatic abscess due to incorrect positioning of this device. We describe how the abscess was treated and we emphasize that one cannot be cavalier about proper positioning of the UVC.

Wandering spleen is a clinical entity which rarely affects children and adolescents. this condition can be asymptomatic or responsible of chronic pain, but it appears as a surgical emergency when an acute twisting occurs. the risk of post-splenectomy sepsis in the pediatric population suggests a conservative approach whenever possible, and also in case of acute torsion, most authors prefer to preserve the spleen and perform a splenopexy. the Authors describe a case of a child with acute splenic torsion, in whom a conservative surgical approach was initially adopted. the conservative option has to be balanced with the risk of prolonged thrombocytopenia, multiple transfusions and a possible second procedure to remove the spleen.

Heterozygous humans for PAX2 mutations show autosomal dominant papillorenal syndrome (PRS), consisting of ocular colobomas, renal hypo/dysplasia and progressive renal failure in childhood. PAX2 mutations have also been identified in patients with isolated renal hypo/dysplasia. Twenty unrelated children and young adults with kidney and urinary tract malformations and no ocular abnormalities were retrospectively recruited for PAX2 mutational analysis. All patients had undergone renal transplantation after end-stage renal disease. We identified two new sequence variations: (i) a deletion causing a frameshift (c.69delC) and (ii) a nucleotide substitution determining a splice site mutation (c.410+5 G/A) by predictive analysis. Therefore, we suggest PAX2 molecular analysis to be extended to all patients with congenital malformations of kidney and urinary tract (CAKUT).

Animal bite lesions in the pediatric patient are mainly accounted for by dog bites in 80% to 90% of the cases. They often present a favorable prognosis but serious lesions do account for 5% to 20% of the total incidence. We will present three particular clinical cases which required urgent surgical treatment as well as a review of the current literature to include both medical and surgical treatment methods for this kind of lesion.

In pediatrie and adult, patients pelvic lymphocele represents a potential complication of renal transplantation. The appropriate treatment of symptomatic cases is still debated. External drainage risks possible recurrence, while laparotomy and intraperitoneal marsupialization seems to be too extensive a procedure. Recently, the laparoscopic approach has been proposed as a reasonable alternative. The authors report their experience with this technique during January 1988 through December 1997, when 147 renal transplantation procedures were performed, resulting in 11 cases of pelvic lymphocele. Three cases resolved spontaneously, and two were successfully treated by percutaneous drainage. The last six patients were laparoscopically treated. The laparoscopy was performed with an open technique using three trocars; a peritoneal window was created and secured open with titanium clips. The operative times varied between 30 and 45 minutes, and no conversion was required. Recurrence occurred in one case, which required laparotomy. At follow-up, ranging from 6 months to 3 years, all five patients treated laparoscopically were asymptomatic, and sonographic results were normal. Laparoscopic marsupialization of symptomatic lymphocele, unresolved by external drainage, is an easy and effective alternative to the open surgical approach.

Gallbladder polypoid lesions are rare in the pediatric patient and sometimes represent an incidental finding. A 13 year old male was referred to the Padua Hospital Pediatric Department for an obesity. A routine abdominal ultrasound (US) detected a gallbladder polypoid lesion 6 mm in diameter, initially considered a gallbladder adenoma. Investigation did not detect any other biliary tract abnormality. After seven months, the asymptomatic patient underwent a follow-up US which revealed the disappearance of the polypoid mass. The following concerns are raised: what is the size of the polypoid mass that should be considered for surgery? How does the presence of symptoms worsen the diagnosis and lead to preferring a surgical approach (cholecystectomy) over an echographic follow-up?

Müllerian malformations represent a wide spectrum of anomaly of the female reproductive tract, usually detected at birth or at puberty. The presence of uterus didelphys and obstructed hemivagina, associated with ipsilateral renal agenesis, is known as Herlyn-Werner-Wunderlich syndrome, a rare congenital anomaly. Two cases of uterus didelphic and hematometra caused by vaginal septum are reported; the girls had a delayed diagnosis due to absence of a specific clinical feature. Clinicians must be aware of the existence of these malformations. An adequate radiological study is mandatory to assess the anatomic conformation, and to optimize the therapeutic procedures.

to evaluate the efficacy of a right common carotid artery cutdown as alternative access in neonates and small infants requiring a balloon dilation of aortic valve stenosis. In infants, the femoral approach is limited by difficulties in advancing the catheter across the valve and by the risk of femoral artery injuries.from January 1997 to July 2000, 16 infants at our department underwent balloon dilation through a carotid artery cutdown. Infant weight ranged from 2670 to 6450 g; mean weight 3967 g, and age ranged from 1 to 157 days, mean age 42,8 days. Fifteen of 16 infants had aortic valve stenosis; the remaining infant presented with a aortic coartation relapse.In 15 infants an adequate dilation of the valve was obtained with no complications. In only one infant an arterial intimal disconnection was caused by inadequate choice of surgical instruments. At the end of the procedure, the carotid arteries were reconstructed with interrupted 7-0 prolene stitches. There were no neurological sequaelae observed. All infants were followed-up and examined by echocolordoppler ultrasound: all carotid arteries were open with no significant stenosis.Our experience confirms that the carotid access proposed in 1973 by Azzolina et al is a valid and safe alternative to the usual percutaneous femoral access. In particular it could be useful in neonates and infants were the size of femoral vessels could facilitate important and dangerous complications.

This study includes all the children treated with OK-432 for lymphangioma at our institute. Twenty-nine children treated between 1999 and 2003 are reported for the first time: twelve cases regressed completely, eight cases regressed more than 50% and seven remained unchanged; two cases were lost at follow-up. The outcome was related to the size of the cysts, the larger ones having a better prognosis. The adverse reactions are discussed and the methods of treatment are described in detail. Fifteen children, treated before 1999 and already reported, are reviewed after a long-term follow-up. Four had a recurrence: one regressed spontaneously and three needed further treatment. The other 11 had no complaints. Even considering the risk of recurrence, OK-432 therapy remains our first line therapy for lymphangiomas, avoiding surgery in most cases.

We report the case of a 14-month-old child with Kasabach-Merritt Syndrome, due to a giant liver hemangioma. The therapeutic approach consisted of peripheral transcatheter embolisation of the right hepatic artery with Ivalon microspheres without the addition of thrombogenic material. This procedure brought to a sensible permanent reduction of the size of the liver hemangioma with normalisation of the previous altered coagulation parameters after 6 years of follow-up.

Hypertrophic pyloric stenosis (HPS) is rare in premature infants. We report a case of HPS in an extremely low birth weight neonate (28 weeks of gestation, 622 gr), discovered on the 10th week of life. Although the diagnosis and treatment of pyloric stenosis has ben well established for many years, the presentation in preterm babies is atypical and the diagnosis often delayed. A brief review of literature is included.

To evaluate the efficacy of renal transplantation in small pediatric patients, we have reviewed 41 allografts performed in 39 children (28 M/11 F) less than 6 years of age between 1987 and 1998 in the North Italy Transplant Program. Of these patients, 39 had a cadaver donor and 2 a living-related donor, with ages ranging from 20 days to 35 years. The mean follow-up was 56 months. Graft survival was 74.5% and 70.5% at 1 and 5 years, respectively. The causes of graft lost were acute rejection (4), graft vascular thrombosis (4), and hemolytic uremic syndrome recurrence (1). Only 1 patient has died due to chickenpox. Double and triple immunosuppressive therapies were used in 63% and 37% of patients, respectively, on the basis of different center protocols, without differences in graft survival. Steroids were successfully administered on alternate days in 37% of patients, 6-12 months after transplantation. Thrombosis was reported in 2 of 6 kidneys from donors less than 1 year of age and in 2 of 35 donors older than 1 year (P0.05). Thirty rejections occurred in 23 patients: 7 episodes were steroid resistant and were treated with ATG/OKT3. Thirty-four infections were reported in 16 of 41 patients; of these 17 were viral, 14 bacterial, and 3 due to Mycoplasma. Four surgical complications were reported: 1 graft artery stenosis, 1 ureteral stenosis, 1 urinary leak, and 1 lymphocele. Mean height standard deviation score improved from -2.0 +/- 1.3 pre transplantation to -1.8 +/- 1.4, -1.5 +/- 1.3, and -1.5 +/- 1.5 at 1, 2, and 5 years post transplantation. Linear growth was significantly better in infants treated with alternate-day steroids. Hypertension was a frequent complication, since 19 of the 30 patients with a 5-year follow-up were still being treated with antihypertensive drugs. In conclusion, graft survival in patients less than 6 years old is satisfactory and similar to that obtained in children aged from 6 to 18 years (70.5% vs. 78.9% at 5 years, P = NS). Consequently, since there are many difficulties in managing infants on maintenance dialysis, an early transplant should be considered. Donors older than 24 months carry a low risk of vascular thrombosis and may be successfully grafted in infants.

The purpose of this study was to determine the frequency and the outcome of de novo malignancies in a cohort of renal transplant paediatric patients. The records of 493 kidney transplants, carried out in 454 paediatric recipients at the three paediatric transplant centres of the North Italy Transplant programme (NITp, Italy) were reviewed. 10 cases of malignancies (2.2%) comprising both PTLD (post-transplant lymphoproliferative disorders) (6 cases, 1.3%) and non-PTLD malignancies (4 cases, 0.88%) were reported. Non-PTLD included one urothelial carcinoma and one Wilms' tumour of the recipient's left native kidney, one abdominal dysgerminoma and one optic nerve glioma of the left eye. The PTLD consisted of localised or disseminated Epstein–Barr virus (EBV)—associated B-lymphocyte monoclonal (5 cases) and polyclonal (1 case) proliferations. All patients suffering from PTLD had been EBV-negative at the time of transplantion, but developed EBV primary infection after transplantion. All PTLD patient donors were EBV-positive. In addition, all but 1 patient received, before and/or after transplantation, a range of immunosuppressive drugs in addition to the baseline prophylactic immunosuppressive regimen. Moreover, 3 patients suffered from syndromes associated with a genetic predisposition to cancer. Finally, the malignancies reported here were associated with 20% graft failure and 20% mortality rates.

Extremely low birthweight neonates often require total parenteral nutrition by central venous catheterization. The technique of choice is the percutaneous cannulation via the basilica or cephalic vein; in particular cases, these peculiar patients need a cut down catheterization. This paper describes some unusual complications of this surgical approach.

Although rarely observed, vascular diseases in children constitute a complex clinical problem. Until recently the diagnostic approach to infant vascular diseases was based on invasive methods such as arteriography, but now the use of ultrasonographic methods such as Doppler c.w. and Echo Doppler Color Flow (EDCF) in angiology can offer new and interesting application even in the pediatric age range. In the present study 15 children affected by vascular diseases (eight with arteriovenous fistulas (AVF), two with pseudoaneurysms, and five with angiomas or cysts) either of iatrogenic or congenital etiology, were examined by Doppler c.w. and EDCF. In three additional cases a suspected vascular disease was not confirmed. The data obtained by Doppler c.w. and EDCF proved useful to perform a preoperative diagnosis; however some diagnostic criteria differed from those described in adulthood, as in the AVF cases, for instance, we observed the prevalence of indirect signs such as alterations of venous flow, and the presence of "multicolored speckled mass" and of "the spreading of the color in extravasal space" rather than a direct connection between an artery and a vein. Also pseudoaneurysms were easily detected by EDCF. In two cases of congenital AVF, the diagnosis was confirmed by arteriography. Surgery was performed in 9 children, 7 with AVF and 2 with pseudoaneurysm; in all cases, the vascular defect detected by ultrasonography was visualized and corrected. In 7 cases EDCF was also used in the follow-up. In conclusion this study suggests a possible more extensive use of ultrasonographic methods in the diagnostic approach to vascular diseases in pediatric age groups, and points out their validity in screening very young patients and in their surgical follow-up.

Congenital diaphragmatic hernia (CDH) with severe respiratory failure is still associated with significant mortality. Modern treatment of CDH is now widely accepted to be delayed repair after stabilization. Availability of Extracorporeal Membrane Oxygenation (ECMO) led up to real improvement in survival. Several others modalities have been recently used in attempting to reduce the need for ECMO or, otherwise, to improve outcome. Multicenter controlled trial of high-frequency oscillatory ventilation (HFOV), exogenous surfactant replacement, nitric oxide (NO) inhalation and, more recently, liquid ventilation have been reported. We describe four cases of CDH treated in our ECMO-centre from 1993 to date, 25% surviving. One patient died by pulmonary hypertension and multiorgan failure while on ECMO; one by pulmonary hypertension and cardiac failure and one by sepsis, both ones far from effective ECMO weaning. All patients underwent extracorporeal bypass because of Oxygenation Index (OI) ranging 65-215. Venovenous has been always made but one patient needed early switching on venoarterial. Several trials with surfactant and nitric oxide were performed during extracorporeal bypass. In survived patient, diaphragmatic defect was repaired out of ECMO. Patients survived to the weaning underwent vascular reconstruction. Our ECMO data confirm worse prognosis for CDH rather than other ECMO requiring diseases (we report 66.7% surviving in overall ECMO application); we underline real improvement by using alternative therapies together with extracorporeal bypass and primary role of OI as predicting index for ECMO.

Isolated congenital urethrocutaneous fistula is uncommon, and its repair has been associated with high incidence of recurrence. However, the use of buccal mucosal graft offers a satisfactory closure after previous failures. We report a new case in whom we adopted the buccal mucosal urethral replacement to treat the recurrence.

177 EARLY ULTRASONOGRAPHIC DETECTION OF ATRIAL THROMBI IN NEONATES WITH CENTRAL VENOUS CATHETERS

The AA describe two particular complications of arteriovenous fistula for chronic hemodialysis in pediatric age: a king king and a stenosis. In these cases are necessary angiographic studies of the vessels and then, if it is possible, the AA prefer a surgical repair of the same arteriovenous fistula. The other alternatives in these cases are an extensive patch, a new angioaccess or a percutaneous transluminal angioplasty.

An acute volume expansion in water diuresis has been induced in 8 patients with liver cirrhosis and in 8 normal subjects, taken as controls. A two hour postinfusion urine sample has been utilized to obtain the urine fraction with natriuretic activity. This activity was assayed in 16 rats. Our results confirm a significantly lower natriuretic activity of cirrhotic urine fractions than those of controls.

The article reports an original technique of arterialisation of the portal vein in the rat, after porto-cava shunt, with termino-latreal anastomosis between the proximal stump of the portal vein and the aorta; compared with other techniques, this method has the advantage of avoiding right nephrectomy.

Reference is made to a personal series of vascular accesses for haemodialysis on 273 patients aged 6 months to 70 yr. During the course of 12 years, 533 operations were performed: 109 cannulations of the saphena, 152 by-pass, 251 arteriovenous fistulae (including 22 on patients under 10 yr of age), and 21 "difficult accesses". An assessment of the advantages and disadvantages of the techniques employed showed that the Cimino-Brescia fistula, combined, if necessary, with transient cannulation, is the soundest access owing to its longer life, lower incidence of complications, and better utilisation of the vascular material available.