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Osteonecrosis (ON) is a critical complication in the treatment of childhood leukemia and lymphoma. It particularly affects survivors of acute lymphoblastic leukemia and non-Hodgkin lymphoma reflecting the cumulative exposure to glucocorticosteroid therapy. ON is often multiarticular and bilateral, specially affecting weight-bearing joints. A conventional approach suggests a surgical intervention even if pharmacological options have also recently been investigated. We reported two cases of long time steroid-treated patients who underwent Bone Marrow Transplantation (BMT) for hematological disease. Both patients developed femoral head osteonecrosis (ON) that was diagnosed by magnetic resonance imaging (MRI) and the ON was also accompanied with pain and a limp. Despite of the conventional strategies of therapy, we successfully started a short-term treatment with bisphosphonates in order to decrease the pain and the risk of fracture.

We report a case of fused testes in a patient with transverse testicular ectopia and persistent Mullerian duct syndrome. A laparoscopy performed to confirm the diagnosis showed a single vas deferens on the left side. Treatment included excision of the Mullerian remnant through a left inguinal incision and orchidopexy of the fused testes via the left inguinal canal after sectioning of the right internal spermatic vessels. Follow-up ultrasound has so far confirmed viability of both testes., (Copyright 2010 Elsevier Inc. All rights reserved.)

Objective: We examined the diagnostic accuracy of routine imaging studies (ultrasonography and micturating cystography) for predicting long-term parenchymal renal damage after a first febrile urinary tract infection., Methods: This study addressed the secondary objective of a prospective trial evaluating different antibiotic regimens for the treatment of acute pyelonephritis. Data for 300 children < or =2 years of age, with normal prenatal ultrasound results, who completed the diagnostic follow-up evaluation (ultrasonography and technetium-99m-dimercaptosuccinic acid scanning within 10 days, cystography within 2 months, and repeat technetium-99m-dimercaptosuccinic acid scanning at 12 months to detect scarring) were analyzed. Outcome measures were sensitivity, specificity, and negative and positive predictive values for ultrasonography and cystography in predicting parenchymal renal damage on the 12-month technetium-99m-dimercaptosuccinic acid scans., Results: The kidneys and urinary tracts were mostly normal. The acute technetium-99m-dimercaptosuccinic acid scans showed pyelonephritis in 54% of cases. Renal scarring developed in 15% of cases. The ultrasonographic and cystographic findings were poor predictors of long-term damage, showing minor sonographic abnormalities for 12 and reflux for 23 of the 45 children who subsequently developed scarring., Conclusions: The benefit of performing ultrasonography and scintigraphy in the acute phase or cystourethrography is minimal. Our findings support (1) technetium-99m-dimercaptosuccinic acid scintigraphy 6 months after infection to detect scarring that may be related to long-term hypertension, proteinuria, and renal function impairment (although the degree of scarring was generally minor and did not impair renal function) and (2) continued surveillance to identify recurrent urinary tract infections that may warrant further investigation.

Ciliated hepatic foregut cyst is the only ciliated cystic lesion known to occur in the liver. It is an extremely rare, benign and solitary cyst that probably arises from remnants of the embryonic foregut in the liver. We report a 16-month-old girl who underwent surgical excision of a hepatic cyst discovered during antenatal ultrasonography. Surgical exploration and excision were performed because of the uncertain aetiology of the cyst and because on postnatal follow-up US the size of the mass had increased causing extrinsic biliary obstruction. Pathology revealed a ciliated hepatic foregut cyst. This is the fourth child affected by this lesion reported in the literature, the second undergoing surgical excision, and the second with antenatal diagnosis.

Background: Approximately 20% of patients who have hepatoblastoma (HB) still have unresectable disease after preoperative chemotherapy (POC). In these circumstances, orthotopic liver transplantation (OLT) should be performed 1 month after POC. The authors sought to identify presenting features that would predict unresectability in patients with HB and to provide suggestions for early referral and listing for OLT., Methods: Notes, radiology films, and histology from patients who were treated over the previous 20 years were reviewed. Unfeasible resection was defined by bilobar involvement, vascular extension, and metastatic disease after POC. Failed conservative treatment (FCT) was used to categorize patients who were not disease-free with their native liver > or =1 year after surgery., Results: Of 28 patients who were studied, 14 patients underwent resection, and 10 patients required OLT. Four patients did not undergo any type of surgery because of tumor progression. Overall, the 5-year survival rate was 76% (95% confidence interval, 54.8-89%). Predictors of FCT were multifocality (P = .006), a high pretreatment extent of tumor (PRETEXT) score (III or IV; P = .006), portal vein involvement (P = .02), hepatic vein involvement (P = .02), or vena cava involvement (P = .05). Patients who achieved a curative resection presented at a younger age (median, 0.7 years vs 4.2 years, P = .02). Patients who had multifocal lesions and those who had an alpha-fetoprotein (alphaFP) level <100 ng/mL survived only if they underwent transplantation., Conclusions: Patients with HB who were managed by combined chemotherapy and surgery has a high survival rate. Older patients who had multifocal tumors, high PRETEXT scores, involvement of major liver vessels, and alphaFP levels <100 ng/mL were less likely to achieve curative resection. These findings at presentation should lead the clinicians to liaise early with a transplantation center.

Purpose: The aim of our study was to assess the usefulness of positioning metal wires under ultrasound guidance for localising soft tissue lesions in the preoperative phase., Materials and Methods: We studied superficial soft-tissue lesions in 12 patients, using hooked mammographic wires of different lengths. One patient had a multifocal growth of disease which required a double localisation procedure. Correct positioning of the wire was confirmed by ultrasonography. All patients underwent surgery within five hours of hook-wire positioning., Results: Correct wire position was confirmed at surgery in 12 out of 13 procedures. In one case the hook-wire reached the margin of the lesion. In all cases, the preoperative localisation procedure facilitated identification and resection of the masses., Conclusions: In our experience, the main indications for hook-wire positioning before surgery are: marking of small lesions, localisation of lesions in anatomic areas structurally subverted by previous surgery and consequently difficult to detect, guidance for surgical sectioning in order safeguard the noble structures close to the lesions.

Background and Objectives: Veno-occlusive disease (VOD) is one of the most frequent complications after stem cell transplantation. We conducted a prospective survey of 244 hematopoietic stem cell transplants in children to determine the incidence of VOD, its main risk factors, treatment and effect on the transplant., Design and Methods: Two hundred and forty-four hematopoietic stem cell transplants (HSCT) performed in 220 pediatric patients from 1993 to 2003 were evaluated. The series included 127 males and 93 females with a median age of 6.7 years at the time of transplantation., Results: VOD was diagnosed following 26 of the 244 transplants (cumulative incidence 11%), but a higher incidence was found in patients with at least one known risk factor for VOD (cumulative incidence 20%). In multivariate analysis, risk factors for VOD were age < 6.7 years; type of VOD prophylaxis, and busulphan-containing conditioning regimens. Routine treatment of VOD was based on supportive care and, starting from 1999, defibrotide was used. All patients were monitored with daily Doppler ultrasound-(US) for early diagnosis of inversion of portal blood flow. Twelve patients developed inversion of portal flow (9 had severe VOD; 3 had moderate VOD) and were promptly started on fibrinolytic and anticoagulant therapy with heparin and recombinant tissue plasminogen activator (rt-PA). Hepatic flow reverted to normal in all 12 patients; only 4 patients ultimately developed multiorgan failure and died. The transplant-related-mortality (TRM) rate in patients with or without inversion of portal flow was 33% vs 7%, (p=0.1). The TRM in patients with or without VOD was 19% vs 8% (p=0.001)., Interpretation and Conclusions: This study showed that younger age, type of VOD prophylaxis, and busulphan-based conditioning regimens are independent risk factors for VOD. Inversion of portal flow was found in 9 of 10 patients with severe VOD. Doppler US monitoring may be helpful in early identification of the patients with VOD-induced inversion of portal flow who might benefit from therapy with heparin and rt-PA.

Purpose: This study was performed to develop a standardized methodology for the sonographic assessment of clubfoot at birth and at the end of both conservative treatment and surgical correction., Methods: Forty-two congenital clubfeet and 42 normal feet were examined sonographically in the position of spontaneous alignment and during passive manual correction. Scans along 4 planes provided information relevant to the assessment: sagittal posterior, sagittal anterior, coronal lateral, and transverse., Results: Sagittal posterior sonograms demonstrated the progressive gain of dorsiflexion ability during the different steps of treatment for clubfoot. Sagittal anterior sonograms could not demonstrate the normal alignment of the navicular in clubfeet because of the bone's medial displacement. On transverse sonograms, the talar head and the medially displaced navicular may lie on the same plane, depending on the severity of the deformity. Coronal lateral sonograms provided for estimation of the relationships between the calcaneus and cuboid, which were described by the calcaneal-cuboid angle., Conclusions: Sonography is a promising technique for assessment and monitoring of clubfoot during treatment. The method described here yields accurate and reproducible information about the anatomy of the nonossified clubfoot, helping the orthopedic team decide on appropriate treatment steps., (Copyright 2004 Wiley Periodicals, Inc.)

We report a case of a neonate with tetralogy of Fallot with aneurysmal dilatation of the pulmonary artery, complicated by bilateral relapsing pneumothorax. The relapsing air leak made it necessary to place up to five chest drains and to switch from conventional ventilation to high frequency ventilation. In the course of 30 days, all drains were removed. Once other anatomical and functional malformations of the respiratory system had been appropriately excluded and reasonable haemodynamic stability had been achieved, the patient underwent successful radical corrective heart surgery in hypothermia and cardioplegia. We emphasize the advantage of resolving respiratory failure preoperatively to guarantee the success of corrective heart surgery and treatment of other surgically severe cases.

Background and Aim of the Study: Glutaraldehyde may promote calcification in xenograft tissue by the action of toxic aldehyde group residues involved in the cross-link process. Post-fixation treatment with homocysteic acid (HA) neutralizes this toxicity by bonding aldehyde groups, and enhances biocompatibility on the basis of strongly electronegative sulfonic groups. Previous studies in a rat subcutaneous model showed significant long-term mitigation of mineralization of glutaraldehyde-fixed pericardium treated with HA. This study aimed to assess the anticalcific efficacy of HA in a valvular implant in growing sheep, and establish if the tricuspid position is suitable for testing replacement bioprosthetic valves., Methods: Eleven stented 25 mm Pericarbon bioprostheses (seven HA-treated, four standard) were implanted in the tricuspid position of growing sheep. Infective endocarditis occurred in four prostheses. Among the remaining seven, three (two HA-treated, one standard) were explanted at 91 days (mid-term), and four (two HA-treated, two standard) at 140-141 days (long-term). All explants were studied by gross, X-ray, light, transmission and scanning electron microscopy, as well as by atomic absorption spectroscopy., Results: No histological and ultrastructural difference in tissue preservation were observed between HA-treated and standard Pericarbon bioprostheses, either in the mid or long term. The mean calcium content of mid-term HA-treated explants was 9.55 mg/g compared with 16.26 mg/g in mid-term standard explants. Only one late standard explant failed as a result of severe stenosis caused by massive dystrophic calcification. Among four late explants, two showed significant increase in mineralization (HA-treated, 87.45 mg/g; standard, 181.20 mg/g), while two showed calcium contents similar to those in mid-term explants (HA-treated, 11.96 mg/g; standard, 17.32 mg/g)., Conclusion: Post-fixation treatment with HA preserves structural properties after tricuspid implantation in growing sheep. The tricuspid implant in the sheep model failed to reproduce remarkable accelerated progressive calcification in all xenografts so as to demonstrate a significant difference between HA and standard explants. The tricuspid position for testing replacement bioprosthetic valves should be abandoned, and investigations repeated with the prosthesis in the mitral position.

Lipoblastoma is an uncommon, benign mesenchymal tumor with an excellent prognosis despite its potential to local invasion and rapid growth. However, in the literature, a spontaneous resolution has never been reported, and, consequently, the need for a complete surgical excision has never been questioned. The authors report a case of a 2-day-old boy with congenital diffuse lipoblastoma in the left thigh, which forced us to withhold from surgical treatment to avoid the risk of mutilation in a patient so young. The lesion was followed-up by imaging, and a complete spontaneous resolution of the diffuse lipoblastoma was shown by magnetic resonance imaging (MRI) at 1-year follow-up. In the literature, a complete surgical excision is recommended. The results of this case suggest that a "wait and see" approach is justified at least in infants with huge invasive lesions requiring a mutilating excision.

Purpose: We describe 3 cases of testicular cystic dysplasia that were diagnosed only by sonography to avoid an invasive approach., Materials and Methods: Three patients 5, 8 and 12 years old, respectively, had increased testicular volume and/or intermittent pain. Sonographic examination of the testis by high frequency (7.5 mHz.) probes showed the typical onset of testicular cystic dysplasia, characterized by several small focal or diffuse intraparenchymal cystic formations., Results: Biopsy or orchiectomy was not considered. At 16, 18 and 24 months of followup, respectively, testicular pain was absent in our 3 cases and sonographic findings were unchanged., Conclusions: Clinical and sonographic followup is considered sufficient to evaluate possible changes in the clinical course of this pathological condition which, although benign, still remains to be defined.

Fetal gallstones were visualized sonographically in two cases at 30 and 37 weeks' gestation; follow-up scans at 2 and 3 months, respectively, disclosed their spontaneous resolution. The hypotheses advanced to explain the formation of echogenic material in the fetal gallbladder are reviewed, and the clinical utility of postnatal long-term sonographic follow-up is stressed.

Background and Aim of the Study: Glutaraldehyde is considered a promoter of calcification by the action of toxic aldehyde group residuals from cross-linking. Post-fixation treatment with homocysteic acid (HA), besides bonding aldehyde groups and neutralizing toxicity, should enhance biocompatibility due to the strongly electronegative sulfonic group. The aim of this investigation was to evaluate HA efficacy on tissue preservation and dystrophic calcification mitigation in glutaraldehyde cross-linked bovine pericardium (BP) using a subcutaneous rat model., Methods: Four samples of BP, two with glutaraldehyde-HA and two with glutaraldehyde treatment, were implanted in each of 24 male Sprague-Dawley rats. Three rats were killed at 14 days, eight at 28 days, eight at 56 days and five at 84 days. Unimplanted glutaraldehyde-HA- and glutaraldehyde-treated samples served as controls. All samples were studied by gross examination, mammography, light transmission and scanning electron microscopy, and atomic absorption spectroscopy. The nature of mineralization was investigated by coupling techniques of scanning electron microscopy, electron microprobe analysis and X-ray powder diffraction., Results: No histological and ultrastructural differences were found between glutaraldehyde-HA- and glutaraldehyde-treated BP, whether implanted or unimplanted. In both groups, calcification progressed with time, but significantly less after glutaraldehyde-HA treatment than after glutaraldehyde alone and at all time intervals (14.63 +/- 21.34 versus 43.17 +/- 15.99 at 28 days, p = 0.003; 56.42 +/- 40.20 versus 90.59 +/- 32.90 at 56 days, p = 0.008; 91.68 +/- 67.68 versus 156.23 +/- 17.85 at 84 days, p = 0.01). Differences were evident by mammography and histology (von Kossa stain). Electron microprobe analysis in both groups showed the composition of calcified nuclei to be calcium phosphate, stoichiometrically close to apatite (Ca5(PO4)3(OH)). The occurrence of crystallized apatite was supported by X-ray powder diffraction findings, the amount of crystallized apatite being higher in glutaraldehyde-treated samples., Conclusions: Post-fixation treatment with HA preserves BP structural properties and significantly mitigates mineralization of long-term subcutaneous implants.

Background and Aims of the Study: Pericarbon is a new-generation bovine pericardial bioprosthesis designed to withstand mechanical wear. Following optimal in vitro testing and animal experiments, clinical trials were initiated in many European centers and explants sent to our department for pathological evaluation. This included gross, radiographic, histologic and ultrastructural investigations., Methods: Between 1986 and 1996, 24 bioprostheses (eight aortic, 11 mitral, two mitro-aortic, one tricuspid) were collected from 22 patients (10 males and 12 females; mean age 57.0 +/- 18.9 years) either at autopsy (nine) or reoperation (15)., Results: Ten bioprostheses explanted < 2 months after surgery were either normal or failed because of surgical problems or non-structural causes. Among the other 14 bioprostheses (mean placement 41.9 +/- 23.6 months; range: 7 to 90 months), structural deterioration occurred in seven and was due to dystrophic calcification with stenosis in five (three aortic, two mitral), mixed lesion in one (mitral), and incompetence in one by calcium-related commissural tear (mitral). At the ultrastructural level, calcification was detected either on cell debris or upon collagen fibers. No bioprosthesis failed because of fibrous tissue overgrowth. Of the remaining seven bioprostheses, vegetative endocarditis occurred in two, thrombosis in one, and aseptic paravalvular leak in one; whereas three showed no signs of dysfunction., Conclusions: This pathologic experience with the Pericarbon valve showed calcification to be the main cause of late structural failure, causing mainly cusp stiffness and bioprosthesis stenosis. Tissue rupture or abrupt dysfunction never occurred. Thus, prevention of mineralization remains the main challenge.

Renal calcification is a known complication of long-term furosemide therapy in infants with bronchopulmonary dysplasia (BPD). In a prospective study the clinical course and long-term renal sequelae of renal calcifications of 19 consecutive premature neonates (birthweight < 1250 g) with bronchopulmonary dysplasia who did not receive furosemide were examined. Infants were divided into two different groups on the basis of ultrasound evidence of renal calcifications (RC group) or absence of renal calcifications (NRC group). Serial examinations, performed at the age of 1, 2, 3, 6, 9 and 12 months, showed that 12 infants at the mean age of 68.5 +/- 12.8 days of life had renal calcifications (63%), and 3 of them had nephrolithiasis; 8 had bilateral renal calcifications. Among the 9 survivors, 2 had chronic renal calcifications at the age of 9 months; however, all normalized at the age of 12 months. Twelve infants received hydrochlorothiazide and spironolactone (63%), 17 had prolonged courses of xanthines and dexamethasone (89.5%), while furosemide was not part of the routine pharmacological administration. Statistical analysis showed that birthweight, gestational age, Apgar score and length of parenteral nutrition were comparable in the RC and NRC group infants. Mean serum creatinine, creatinine clearance, fractional sodium excretion and urinary calcium excretion values during the 12-month study period were comparable in the RC and NRC groups. Mechanical ventilation and hospital stay length were instead associated with renal calcification occurrence. The strongest indicator of renal calcification risk for this high-risk population is the severity of the unresolved acute lung disease, where different facets of respiratory management, other than the addition of furosemide, represent sufficient stimuli and renal injury to potentiate stone formation.