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).

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?

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.

[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.

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.

Further experience with OK-432 for lymphangiomas.

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.

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.

A new indication for buccal mucosal graft: isolated congenital fistula of the penile urethra.

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.

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.

Hepatic abscess and umbilical venous catheter: one cannot be cavalier about proper position.

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.

Measures to prevent renal impairment after resection of retroperitoneal neuroblastoma.

Postoperative renal impairment has been reported after kidney-preserving resection of retroperitoneal neuroblastomas (NB). To avoid renal damage, intraoperative procedures include monitoring of central venous pressure (CVP) and diuresis, i.v. administration of electrolyte solutions, mannitol, and furosemide, and local application of lidocaine to the renal vessels. During the first 5 postoperative (p.o.) days, CVP, diuresis, body temperature (T), and lactate dehydrogenase (LDH) levels were monitored; color Doppler ultrasound (CDUS) was also performed on the 7th p.o. day. Simultaneous increases of T (>38.5 degrees ) and LDH (>1,500 IU/l) and/or reduction of urinary flow (< 1.5 ml/kg.h) were indicative of renal damage: in these cases dopamine was administered and CDUS performed. If renal blood flow was normal on CDUS, dopamine was administered for another 2 days; if a vascular thrombosis was detected, fibrinolytic agents (urokinase 4,000 IU/kg bolus and then 4,000 IU/kg.h for 24 h) were utilized. Between May 1997 and June 2000, 29 children (9 F, 20 M, aged) 6-72 months (median 39) affected by grade II inoperable, grade III, and grade IV NB underwent these preventive procedures at the time of surgical excision of the mass after chemotherapy 23 had an uneventful p.o. course. CDUS at 7 days was normal in 22; in 1 (polar resection at operation) it demonstrated a slight reduction of blood flow in the renal cortex. For all 23 patients, the CDUS at 3, 6, and 12 months was negative. Three patients showed a single altered parameter; the CDUS p.o. and during follow-up did not show any alteration. The other 3 patients had two or more altered parameters and were treated with dopamine: in 1 the initial CDUS was normal, but it later showed moderate hypotrophy; in 2 patients a vascular thrombosis was documented. Treatment with urokinase was successful in only 1 patient who had a normalized CDUS after 3 months. The 2nd child developed renal failure and needed hemodyalisis. In our experience, elevation of serum LDH and T and/or oliguria were good indicators of renal impairment. CDUS was also useful in detecting renal blood flow reduction and artery occlusion. These preliminary results show these intra- and postoperative measures to reliably avoid renal impairment in these children. Long-term follow-up could confirm the efficacy of these methods.

Treatment of Kasabach-Merritt syndrome by embolisation of a giant liver hemangioma.

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.

Successful renal transplantation in children under 6 years of age.

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 (P < 0.05). 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.

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.

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.

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.

[Pyloric hypertrophic stenosis in the premature child. A clinical case]. / Stenosi ipertrofica del piloro nel bambino prematuro. Caso clinico.

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.

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.