Management of symptomatic mesenchymal hamartoma of the chest wall: surgical resection only in symptomatic cases.

Mesenchymal hamartoma of the chest wall (MHCW) is a rare neonatal benign tumor with an estimated incidence of 1 in 3 000 among primary bone tumors, and 1 in one million in the general population. Traditionally, the treatment of choice was an "en bloc" resection, but surgery limited to symptomatic cases, is now suggested by most authors due to the numerous cases of spontaneous regressions. We report 2 patients of symptomatic MHCW, characterized by progressive respiratory distress, who underwent surgical treatment with prompt resolution of symptoms. Surgeons and neonatologists should be aware of this rare condition and its possible fatal or nearly-fatal complications.

Acute splenic torsion in children: which is the best treatment? A case report.

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.

PAX2 gene mutations in pediatric and young adult transplant recipients: kidney and urinary tract malformations without ocular anomalies.

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).

[Serious dog bite lesions in childhood: presentation of 3 cases and literature review]. / Lesioni gravi da morso di cane in età pediatrica: descrizione di 3 casi e revisione della letteratura.

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.

Hematometra in uterus didelphis: a pitfall in teenagers with lower abdominal mass. Report of two cases.

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.

Polypoid lesion of the gallbladder in childhood: case report and literature review.

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?

Case for diagnosis: 4-month-old infant with increasing cough, hemoptysis, and anemia.

A 4-month-old caucasian infant presented non-productive cough, fever associated with hemoptysis, and increasing anemia. He had mild tachypnoea; routine lab tests were normal. The thoracic HRCT scan showed a very large mass in the right lung adherent to the thorax wall, well defined and limiting the medium and upper lobe; the mass was well vascularized, and with central hypodensic areas. Fiberoptic bronchoscopy and bronchoalveolar lavage (BAL) cytology were normal. The definitive histology of the mass showed the presence of inflammatory cells admixed with fibroblasts and rare Touton giant cells in the lesion suggestive of a juvenile xanthogranuloma (JXG) of the lung.

De novo cancers in paediatric renal transplant recipients: a multicentre analysis within the North Italy Transplant programme (NITp), Italy.

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 transplantation, but developed EBV primary infection after transplantation. 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.

Kidney transplantation in children with reconstructed bladder.

There is some controversy about the safety of renal transplantation in patients with an augmentation cystoplasty because of the possibility of urinary tract infection in immunosuppressed patients leading to pyelonephritis and graft loss. Nevertheless, it is now well known that in patients with a small volume and poorly compliant bladder, reconstructive bladder surgery (augmentation cystoplasty or continent reservoir) creates a low-pressure and compliant reservoir, which protects the upper urinary tract and restores a functional lower urinary tract. Graft survival is not adversely affected when a kidney transplant is drained into a reconstructed bladder. When bowel segments are used for augmentation, a voiding modality with clean intermittent self-catheterization does not increase the risk of urinary tract infections, even in immunosuppressed patients.

In vitro reconstructed dermis implanted in human wounds: degradation studies of the HA-based supporting scaffold.

The objective of the present study was to demonstrate the safety and efficacy of a dermal replacement for cutaneous wounds of diverse origin. Autologous fibroblasts were cultured in fleece scaffolds made from benzyl esters of hyaluronic acid and applied onto cutaneous lesions. The cases presented are (1) skin removal for multiple epithelioma and (2) chronic deep decubitus ulcer. Dermal-like tissue applied by the surgeon elicited no adverse reactions, and was fully integrated and well-vascularized by 1-3 weeks. In Case 1, the material was fully integrated after 1 week, and after 3 weeks an epidermal autograft was overlaid which showed good take with excellent integration observed after 4 weeks. At 12 months, skin demonstrated visual normo-elastic properties and no signs of excessive scarring. In Case 2, 2-3 weeks after the dermal implant was applied, the wound was invaded with granulation tissue and healing occurred by secondary intention. The ulcer was healed by 8 weeks, with the biomaterial completely resorbed and a complete re-epithelialization over the dermal-like tissue. These results suggest that autologous fibroblast culture in hyaluronan-derived scaffolds may be successfully grafted in diverse cutaneous pathologies and constitute a suitable bed for further epidermal implantation.

Continuous peritoneal dialysis in newborns.

OBJECTIVES: To report the complications and outcome of 10 newborns affected by acute renal failure (ARF), treated by continuous peritoneal dialysis (CPD). DESIGN: All newborns admitted for tertiary treatment to the Neonatal Intensive Care Unit of the University of Padova, who underwent CPD between February 1986 and December 1990, were analyzed retrospectively. PATIENTS: Ten newborns (mean weight 2077 g, range 540-4930 g) received CPD, 6 of whom were preterm. All the survivors completed the study. INTERVENTIONS: A number 9, 5 French Tenckhoff catheter was used, and a closed circuit was created by means of a modified continuous ambulatory peritoneal dialysis (CAPD) technique. The mean duration of dialytic therapy was 7 days. RESULTS: At the end of the dialytic period, 7 of the 10 patients had normal serum potassium and sodium values. CPD produced two different types of complications: leakage of the dialytic fluid in very low weight newborns and one episode of peritonitis during a chronic dialysis treatment. Six died of severe respiratory failure (in no case, however, was this attributable to ARF or CPD procedure). All but one of the survivors regained normal renal function. The only exception necessitated a kidney transplant. CONCLUSION: We believe that this technique, although invasive, improves the outcome of both preterm and low birth weight newborns affected by ARF.

Complications linked to chronic peritoneal dialysis in children after kidney transplantation: experience of the Italian Registry of Pediatric Chronic Peritoneal Dialysis.

Our objective was to evaluate the infectious complications of the post-transplant period attributable to the persistence of catheter and other complications when chronic peritoneal dialysis (CPD) was performed post-transplantation. The design was a retrospective study, and the setting was an Italian registry of pediatric chronic peritoneal dialysis. There were 86 pediatric renal transplants (9/86 from living related donors, 2/86 simultaneous liver and kidney transplantation for oxalosis). Six of 86 transplants were lost at follow-up. Mean age of the children (n = 80) at transplantation was 9.3 years (range: 1.7-21 years). They had been on CPD for a mean period of 1.7 years (range: 0.2-4.6 years). During CPD, 67 peritonitis episodes (80% related to exit-site and/or tunnel infections) were observed, with an incidence of peritonitis of one episode per 16 months CPD. The mean safe interval of peritonitis and/or exit-site or tunnel infection was 208 days (range: 36-1897 days). The mean time of catheter removal was 80.3 days (range: 0-216 days) post-transplantation. During the first month post-transplantation, one episode of peritonitis secondary to a sepsis occurred in one child. No other episodes of peritonitis or exit-site and/or tunnel infections were observed. Two of 80 children returned to CPD (at four and at 12 months, respectively) because of persistent allograft failure. Furthermore, 12 patients were on CPD because of temporary graft failure. In all these patients the pretransplant peritoneal dialysis (PD) catheter was utilized, with no complications. These data show that the persistence of the PD catheter after kidney transplantation has produced no infections or other complications. What is more, the catheter was safely utilized during acute rejection or primary allograft nonfunction.

Echo Doppler color flow (EDCF) evaluation of vascular pathology in pediatric age groups.

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.

Arteriovenous fistula between descending aorta and hemiazygos vein.

The article describes a rare case of congenital A-V fistula between descending aorta and hemiazygos vein in a 9-year old girl. Differential diagnosis in these cases is difficult and only sonography and angiography can lead to a correct diagnosis. The recent literature of this subject is reviewed.

[Vascular access for hemodialysis]. / Gli accessi vascolari in emodialisi.

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.

[Experimental arterialization of the portal vein in the rat]. / L'arterializzazione sperimentale della vena porta nel ratto.

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.

Central venous access in pediatric patients.

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.

Vascular access in patients affected by short bowel syndrome.

The aim of this study was to evaluate the problems induced by the use of a central venous catheter (CVC) in a series of patients with short bowel syndrome observed at the university of Padua (Italy) between January 1981 and June 1997. During this period, 14 patients required central venous access for parenteral nutrition (PN); 55 catheters were inserted, 6 with percutaneous and 49 with surgical techniques. We divided the patients into two groups according to PN duration. The first group includes 11 children with short/medium-term PN 8 are now eating, and 3 died from respiratory failure) while the second group includes 3 patients on home long-term PN. Thirteen catheters were placed in the first group, and the mean PN duration was 173 days; the three patients on home PN required 42 catheters. It is our experience that the use of catehters in patients requiring short/medium- term PN is a safe procedure with few complications; patients on home long-term PN present an increasing number of complications, and the vascular access could become a serious problem as the number of PN-dependent increases.

Rare complications during surgical vein cannulation in extremely low birthweight neonates.

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.

Carotid artery approach as an alternative to femoral access for balloon dilation of aortic valve stenosis in neonates and infants.

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.