Spontaneous neonatal pneumomediastinum: the "spinnaker sail" sign.

Spontaneous pneumomediastinum is a rare condition in the newborn, not associated with identifiable trauma or mechanical ventilation. It is diagnosed by a combination of physical examination and confirmatory chest radiograph, with various recognized signs identifiable in this condition. We report the case of a male neonate, who had pneumomediastinum confirmed by the presence of a wind blown spinnaker sail sign and was managed conservatively. We also reviewed the literature.

Therapeutic lung puncture for diffuse unilateral pulmonary interstitial emphysema in preterm infants.

BACKGROUND: Pulmonary interstitial emphysema (PIE) represents a severe complication of respiratory distress syndrome and can dramatically impair the ventilation of premature infants. OBJECTIVES: We report three mechanically ventilated premature infants with severe diffuse, unilateral PIE not responding to conventional treatment, whose clinical condition improved dramatically following an ipsilateral pneumothorax. RESULTS: In the first two patients, the pneumothoraces occurred spontaneously. In the third patient, the ipsilateral lung was punctured with a pigtail catheter to create and - subsequently drain - a pneumothorax. Thereafter, mechanical ventilation could be discontinued within 3 days in all infants. CONCLUSIONS: Lung puncture with consecutive tension release of the overinflated lung by drainage is a therapeutic option for premature infants suffering from diffuse PIE in whom other conservative measures fail. It may be considered before proceeding to surgical measures in order to preserve potentially functional lung tissue.

[Congenital absence of the portal vein (CAPV). Two cases of Abernethy malformation type 1 and review of the literature]. / Der kongenitale portosystemische Shunt. Zwei Fallvorstellungen einer Abernethy-Malformation Typ 1 und Uberblick über die Literatur.

The Abernethy malformation is a rare congenital portosystemic shunt in which the blood directly drains into the systemic vein bypassing the liver either through a complete (type 1) or a partial shunt (type 2). The diagnosis is most frequently established primarily with ultrasound. CT and MRI are used for further classification of the shunt and assessment of accompanying liver tumors and malformations. There is a wide spectrum of therapeutic options ranging from noninvasive conservative treatment to liver transplantation. The main prognostic factors are the occurrence of concomitant hepatic neoplasms and hepatic encephalopathy. We report two cases diagnosed with a type 1 shunt, hepatic encephalopathy, and associated liver tumors who underwent successful liver transplantation after having considered all therapeutic options.

Post transplant lymphoproliferative disease in pediatric solid organ transplant patients: a possible role for [18F]-FDG-PET(/CT) in initial staging and therapy monitoring.

Post transplant lymphoproliferative disease (PTLD) is a severe complication after solid organ or bone marrow transplantation. In pediatric transplant recipients PTLD is the most common malignancy. The aim of this study was to evaluate a possible role for positron emission tomography with [18F]-2-fluoro-2-desoxy-glucose (FDG) in the initial staging and in therapy monitoring of pediatric patients suffering from biopsy-proven CD20-positive PTLD after solid organ transplantation. Seven pediatric patients were included. All available imaging studies - CT (n=15), MRI (n=16) and PET/PETCT (n=16) - were reviewed on a lesion by lesion base. The performance of FDG-PET in the initial staging and during therapy with a chimeric anti-CD20 antibody was compared to conventional cross sectional imaging and correlated with the clinical outcome. FDG-PET identified all sites of disease as shown by CT/MRI and helped to clarify the significance of equivocal findings. The initial stage of disease was correctly identified by FDG-PET alone when compared to CT/MRI. During therapy, FDG-PET was superior to conventional cross-sectional imaging in the early evaluation of response.

[Imaging characteristics of childhood lipoblastoma]. / Charakteristika in der Bildgebung kindlicher Lipoblastome.

PURPOSE: Lipoblastoma and lipoblastomatosis are very rare benign neoplasms, which almost exclusively occur in infants and young children. Despite their potential to local invasion and rapid growth, these tumors have an excellent prognosis, particularly if resected completely. Usually, the diagnosis is not taken into consideration preoperatively, and depends on histopathological evaluation. This study was done to determine imaging characteristics of lipoblastoma. MATERIALS AND METHODS: Eight cases of histopathologically proven lipoblastoma treated from 1988 to 2003 were reviewed, comprising four girls and four boys ranging in age from 17 months to nine years. The localization was chest wall (four times), abdominal wall (once), gluteal region (once), lower leg (once) and forearm (once). Ultrasound, MRI and CT scans were evaluated and correlated to clinical data of the patients. RESULTS: Ultrasound showed lipomatous echogenicity and echotexture. The tumors appeared signal intensive on T1-weighted MR images and had a mean intensive signal on T2-weighted MR images with fat suppression. They were markedly hypodense on CT. The growth pattern was invasive in all imaging methods, with extension into preformed spaces, such as intercostal spaces and neural foramina, but without infiltration into surrounding structures or metastases. CONCLUSION: Ultrasound and MRI are the methods of choice to diagnose lipoblastoma by revealing structures and local growth pattern that appear specific of this rare tumor entity. The imaging characteristics of the lipoblastoma have to be correlated with the age of patients to exclude other conditions in the differential diagnosis.

Impact of magnetic resonance urography on preoperative diagnostic workup in children affected by hydronephrosis: should IVU be replaced?

PURPOSE: The aim of this study was to determine the role of magnetic resonance urography (MRU) in preoperative diagnostic workup of children with hydronephrosis in a prospective clinical study with comparison of MRU, standard diagnostic investigations, and intraoperative findings. METHODS: Thirty-one children with hydronephrosis secondary to different causes underwent ultrasound scan (US), intravenous urography (IVU), micturation cysto-urethrography (MCU), isotope nephrography (ING) and MRU. For MRU the authors performed sagittal and coronal halve-Fourier SSFSE scans in a 1.5 Tesla MR system. T1- and T2-weighted sequences were used in axial orientation to improve morphologic information. In 24 patients, preoperative data were compared with intraoperative findings. RESULTS: Comparison of the different imaging modalities proved MRU to be able to provide more detailed information about the correct localization of stenoses along the urinary tract and the morphology of renal parenchyma. MRU showed the highest concordance of all imaging modalities with intraoperative findings. CONCLUSION: As a reliable investigation, MRU has the potentials to replace IVU in preoperative diagnostic workup of hydronephrosis in childhood.

Intensive ALL-type therapy without local radiotherapy provides a 90% event-free survival for children with T-cell lymphoblastic lymphoma: a BFM group report.

The purpose of our study was to investigate the efficacy of an acute lymphoblastic leukemia (ALL)-type treatment with moderate-dose, prophylactic cranial irradiation and without local radiotherapy for childhood T-cell lymphoblastic lymphoma (T-LBL). From April 1990 to March 1995, 105 evaluable patients, 1.1 to 16.4 years of age, with T-LBL were enrolled in study NHL-BFM 90 (non-Hodgkin's lymphoma-Berlin-Frankfurt-Munster 90). They received an 8-drug induction over 9 weeks followed by an 8-week consolidation including methotrexate (MTX) 5 g/m(2) x 4. Patients with stage I (n = 2) and II (n = 2) continued with maintenance therapy (6-mercaptopurine daily and MTX weekly, both orally) until a total therapy duration of 24 months. Patients with stage III (n = 82) and IV (n = 19) received an 8-drug intensification over 7 weeks and cranial radiotherapy (12 Gy for prophylaxis) after consolidation, followed by maintenance. Residual tumor after induction had to be resected. Patients received intensified chemotherapy if tumor regression on day 33 of induction was less than 70% or when vital residual tumor was present after the complete induction phase. With a median follow-up of 4.5 years, the estimated event-free survival at 5 years is 90% (95% confidence interval, 82%-100%). Events were 1 early death, 8 tumor failures, and 1 secondary acute myeloid leukemia. A total of 101 patients were evaluable for the speed of tumor response. Two patients received intensified therapy due to less than 70% tumor regression on day 33. Of 19 patients with tumor residues after induction, 2 relapsed as compared to 4 of 80 patients with complete tumor regression. We conclude that, with intensive ALL-type chemotherapy including moderate cumulative doses of anthracyclines 240 mg/m(2) and cyclophosphamide (3 g/m(2)) and moderate-dose prophylactic cranial irradiation but no local radiotherapy, an event-free survival rate of 90% can be achieved in childhood T-LBL. (Blood. 2000;95:416-421)

Tubular esophageal duplication associated with esophageal stenosis, pericardial aplasia, diaphragmatic hernia, ramification anomaly of lower lobe bronchus and partial pancreas anulare.

Alimentary tract duplications are rare anomalies, which are often associated with other congenital malformations. We report on a newborn with the combination of tubular esophageal duplication, congenital esophageal stenosis, ramification anomaly of lower left lobe bronchus, complete pericardial aplasia and malrotation of the bowel with a partial pancreas anulare. The diagnoses are based on esophagogram, bronchogram, bronchoscopy, gastroscopy, explorative thoracotomy and laparotomy. The treatment was a resection of the duplication per thoracotomy, esophageal myotomy with fundoplicatio.

High incidence of significant bone loss in patients with severe congenital neutropenia (Kostmann's syndrome).

OBJECTIVE: Clinical observation of bone pain, unusual fractures in two patients, and diffuse osteopenia/osteoporosis led us to assess bone mineral content and density in 30 patients with severe congenital neutropenia who were treated with recombinant-methionyl-human granulocyte colony-stimulating factor (r-metHuG-CSF). STUDY DESIGN: We reviewed roentgenograms in 29 of these 30 patients to evaluate bone loss before and during treatment. In addition, in 17 of the 30 patients, bone mineral status could be assessed by both quantitative computed tomography (Q-CT; n = 16) and dual energy x-ray absorptiometry (DXA; n = 1). In one patient, Q-CT was not possible because of severe vertebral fractures. RESULTS: Of the 30 patients investigated, 15 had evidence of osteopenia/osteoporosis observed on spine radiographs (n = 5), on Q-CT/DXA (n = 1/n = 1), or on radiographs and Q-CT (n = 8). In 13 of the 30 patients, only a lateral radiograph of the lumbar spine was available, 5 of 13 showing either increased kyphosis and wedging of the vertebrae or compression fractures of the vertebral bodies, indicating severe established osteoporosis. In eight patients, the findings of the spinal radiographs were normal. In nine patients, spinal radiographs were taken before r-metHuG-CSF treatment. Osteoporotic vertebral deformation (n = 3) or reduced bone mass (n = 3) was seen in six of these nine patients. The levels of serum biochemical markers of bone metabolism were all within normal ranges except for mild elevation of the serum alkaline phosphatase level. The degree of spinal bone mineral loss did not correlate with dose and duration of r-metHuG-CSF treatment or with the age or sex of the patients. CONCLUSIONS: These data indicate a high incidence of bone mineral loss in children with severe congenital neutropenia. The underlying pathogenesis of bone demineralization is not clear. It is more likely that the bone loss was caused by the pathophysiologic features of the underlying disease, but it is possible that r-metHuG-CSF accelerates bone mineral loss.

[Evaluation of congenital vena cava anomalies and acquired vena cava obstructions after atrial switch operation using spiral computerized tomography and 3-dimensional reconstruction]. / Beurteilung angeborener Hohlvenenanomalien und erworbener Hohlvenenobstruktionen nach Vorhofumkehroperation mittels Spiralcomputertomographie und dreidimensionaler Rekonstruktion.

We report spiral-CT findings in adult patients with congenital abnormalities of the vena cava and systemic venous obstructions after atrial switch operation. Especially systemic venous obstruction is a well-known complication following Mustard procedure for transposition of the great arteries. The results demonstrate that computed tomography, particularly with the use of 3-dimensional surface reconstruction, is very useful as a highly sensitive procedure for the detailed depiction of abnormalities of the vena cava or of residua and sequelae after inflow correction for complete transposition. Demonstration of such abnormalities or obstructions after atrial switch operation is important, since the recognition and quantitation of caval anomalies by clinical techniques is unreliable and indeed often impossible. Imaging procedures such as spiral computed tomography are important for this purpose. Advantages of spiral computed tomography, particularly with 3-dimensional reconstruction, are discussed.

[Spiral CT and 3-dimensional reconstruction in evaluation of systemic venous obstruction after atrial switch operation for complete transposition of great vessels]. / Spiral-CT und dreidimensionale Rekonstruktion zur Beurteilung systemvenöser Obstruktionen nach Vorhofumkehroperation bei kompletter Transposition der grossen Gefässe.

We report findings in spiral-CT from 11 adolescent or adult patients after atrial switch operation. The results demonstrate that computed tomography, particularly with the use of three-dimensional surface reconstruction, is very useful as a highly sensitive procedure for the detailed depiction of residua and sequelae after inflow correction for complete transposition. Especially systemic venous obstruction (SVO) is a well know complication following Mustard procedure for transposition of the great arteries. Demonstration of such obstruction is important, since the recognition and quantification of caval obstruction by clinical techniques is unreliable and indeed often impossible. Imaging procedures such as spiral computed tomography are important for this purpose. Advantages of spiral computed tomography, particularly with three-dimensional reconstruction, are discussed. The images were compared with findings in echocardiography and/or angiography affecting the site of operation.

[Atresia of the left-sided atrioventricular connection with single ventricle and transposition of great vessels in a adult patients]. / Atresie der linksseitigen atrioventrikulären Verbindung mit singulärem Ventrikel und Transposition der grossen Arterien bei einer erwachsenen Patientin.

Mitral atresia is an extremely rare congenital anomaly in which survival into adulthood is an exception. Without treatment most patients with this anomaly will die in early childhood and only few reach adolescence or early adulthood. The here described patient with mitral atresia, atrial septal defect, single ventricle and transposition of the great arteries survived to age 24 years. She died as consequence of massive cerebral embolism of a thrombus in her left atrium.

Clinical course of hypophosphatemic rickets in 23 adults.

Twenty-three adult patients (19 females, 4 males) with x-linked hypophosphatemic rickets (HPR) underwent a retrospective evaluation of the clinical course and a clinical examination by a nephrologist, orthopedic surgeon and dentist. Blood and urine analysis, bone density measurements with QCT and DEXA, ultrasonic examination of the kidneys were performed and the patients were asked to fill in a standardized questionnaire on pain and psychosocial rehabilitation. Mean final height was 152.4 cm +/- 8.5 SD in females and 157.3 cm +/- 8.9 SD in males. Decreased joint mobility was seen in all patients, deviations of the normal leg axis in 18/23 patients in spite of 69 correcting osteotomies in the past. Dental (n = 14) and psychosocial problems were associated with inability to work (n = 8). There was a trend that patients with a very low Tp/GFR had a more severe course of the disease. Early therapy with vitamin D metabolites and phosphate had a beneficial effect on growth, bone density and deformations. Eight patients had nephrocalcinosis due to vitamin D and phosphate therapy and had normal kidney function. Four patients had urinary tract abnormalities. We conclude that patients with HPR should receive continuous interdisciplinary care given by nephrologists, orthopedic surgeons, physiotherapists and dentists not only during childhood but also as adults.

Steroid resistant nephrotic syndrome associated with spondyloepiphyseal dysplasia, transient ischemic attacks and lymphopenia.

Focal segmental glomerulosclerosis, nephrotic syndrome and chronic renal failure were associated with spondyloepiphyseal dysplasia, growth failure, lymphopenia and transient ischemic attacks leading to severe neurological symptoms in three children. Two boys and one girl developed the full syndrome at the age of 5, 6 and 10 years. Positron emission tomography revealed perfusion defects of both cerebral and cerebellar arteries. A variant of the disease was found in two other children who had a nephrotic syndrome and terminal renal failure with only mild spondyloepiphyseal dysplasia, impaired growth and a normal cerebral function. It is concluded that there may be a close association between focal segmental glomerulosclerosis and spondyloepiphyseal dysplasias.

[Diagnostic imaging in follow-up of surgically treated stenosis of the aortic isthmus in adolescents and adults]. / Bildgebende Verfahren in der Verlaufskontrolle operierter Aortenisthmusstenosen bei Adoleszenten und Erwachsenen.

Local abnormalities of the ascending aorta, especially at the site of correction are well known complications after surgical correction of aortic coarctation. Regular follow-up is therefore necessary. Besides chest X-ray, transesophageal-echocardiography and substraction angiography, magnetic resonance imaging (MRI) and computertomography (CT) provide a complete and noninvasive examination of the great vessels. MRI and CT are ideally suited for this purpose.

[Unilateral left superior vena cava in absent right superior vena cava. Modern imaging diagnosis and clinical relevance]. / Unilaterale linke Vena cava superior bei fehlender rechter Vena cava superior. Moderne bildgebende Diagnostik und klinische Relevanz.

Cardiological and radiological findings in a 64-year-old patient with persistent left superior vena cava and absent right-sided superior vena cava are described. Presence of a persistent left superior vena cava (PLSVC) is considered to be one of the most frequent anomalies of the systemic venous return. It occurs most often in association with a right-sided superior vena cava and only in rare cases with an absent or atretic right-sided superior vena cava. This malformation is often associated with other congenital heart defects, but without major hemodynamic significance. The variation may cause difficulties in venous catheterization, pacemaker electrode insertion or during cardiopulmonary bypass. The diagnosis can be confirmed by angiography, computed tomography and nuclear magnetic resonance imaging.

Impact of hard-copy size on observer performance in digital chest radiography.

To determine the impact of reduced hard-copy size on diagnostic performance of digital radiography, screen-film chest radiographs were compared with isodose digital storage phosphor radiographs in the detection of simulated nodules, fine pulmonary lines, and micronodular opacities superimposed on the chests of 10 healthy volunteers. Digital radiographs were laser-printed in a full-size conventional format and in image lengths of two-thirds, one-half, and five-elevenths of the conventional format. Eighteen thousand observations by eight radiologists were analyzed by use of receiver operating characteristics. The detectability of lines and micronodular opacities decreased with declining image format size. In the detection of micronodular opacities, only the nearly full-size digital images were equivalent to conventional images. In the detection of linear opacities, reduction of image length by one-half or more reduced performance (analysis of variance, P less than .05). Only for the detection of nodules was no major difference found.

[Assessment with magnetic resonance tomography of anatomy and ventricular function after Mustard correction of transposition of the great arteries]. / Magnetresonanztomographische Beurteilung von Anatomie und Ventrikelfunktion nach Mustard-Korrektur bei Transposition der grossen Arterien.

In order to evaluate postoperative sequelae and ventricular function after Mustard-operation in patients with transposition of the great arteries (TGA), 30 patients were assessed by magnetic resonance imaging in EKG-triggered spin-echo (SE) and gradient-echo (GE) technique. Twenty-three patients, aged 4.7 to 15.8 years, had transposition of the great arteries with intact ventricular septum with or without left-ventricular outflow tract obstruction (TGA+IVS +/- SPS). Seven patients aged 9.5 to 21.7 years had transposition of the great arteries with ventricular septal defect (TGA+VSD). Five patients showed a residual baffle leak, one had a pulmonary venous obstruction, five an obstruction at the caval veins, 13 a left-ventricular outflow tract obstruction, and 14 a tricuspid regurgitation. Right-ventricular enddiastolic volume in patients with TGA+VSD (77.0 +/- 25.5 ml/m2) was significantly higher than in patients with TGA+IVS +/- SPS (61.2 +/- 12.0 ml/m2). In TGA+VSD right-ventricular ejection fraction (47.6 +/- 13.0%) was significantly lower than in patients with TGA+IVS +/- SPS (56.7 +/- 10.7%). The ratio of muscle masses of right to left ventricle was 1.8:1 in patients with TGA+IVS +/- SPS and 2.5:1 in patients with TGA+VSD. In conclusion, after Mustard-operation in patients with transposition of the great arteries (TGA) magnetic resonance imaging provides a comprehensive and noninvasive assessment of postoperative sequelae, residuae, and ventricular function and will, therefore, become the method of choice for postoperative evaluation.

Adult height achieved in children after kidney transplantation.

We evaluated posttransplantation growth, bone maturation, and adult height in 20 adolescents who had received kidney transplants at the age of 10.5 to 17 years. Nine patients (five male, four female) were treated with cyclosporine and low-dose prednisolone, and 11 children (six male, five female) were treated with azathioprine and high-dose prednisolone. The cumulative dose of steroids after transplantation was significantly lower in the cyclosporine-treated group. Bone age, according to the radius-ulna-short bones method of Tanner and Whitehouse, was almost the same in both groups at the time of transplantation (15.0 and 14.6 years for male subjects, 13.3 and 13.1 years for female subjects). Predicted adult height (Tanner-Whitehouse Mark II-method of Tanner et al) and target height were estimated at transplantation. Adult height was defined as achieved when bone age in male subjects had reached 18 years and, in female subjects, 16 years. Bone maturation of the cyclosporine-treated patients occurred at a normal rate (0.92 bone-age years per chronologic year), whereas the azathioprine-treated group exhibited a significantly slower rate (0.56 bone-age years per chronologic year). The growth rate per year for the cyclosporine-treated group was more than double that of the azathioprine-treated group (3.0 cm vs 1.4 cm). The adult height of the cyclosporine-treated group exceeded the predicted adult height by a mean of 1.3 cm, but the azathioprine-treated group missed it by 3.9 cm. Target heights could not be achieved in any group. Kidney function was significantly lower in the cyclosporine- vs the azathioprine-treated group, but no patients suffered from severe renal insufficiency. We conclude that cyclosporine and low-dose prednisolone are associated with normal bone maturation and a better prognosis for final height in children with renal transplants.