[Lethal aortic dissection in a 13-year-old boy with a vascular Ehlers-Danlos syndrome]. / Dissection aortique fatale chez un garçon de 13 ans atteint d'un syndrome d'Ehlers-Danlos vasculaire.

UNLABELLED: Vascular Ehlers-Danlos syndrome(EDS) is at high risk of death by arterial rupture. CASE REPORT: A 13-year-old boy with vascular EDS and aortic dissection was admitted in pediatric emergency care unit. The children died after surgery by massive hemorrhage and prosthetic rupture. DISCUSSION: The precocity of this vascular accident EDS is uncommon. It occurred despite clinical and echocardiographic follow-up. The death of the children confirmed the difficulties in surgery of vascular manifestation, even if appropriate and rapid management was already done. CONCLUSION: Patients with vascular EDS must be identified because of its lethal complications. Friability of the vessels makes surgical treatment difficult. A trial using beta blockers therapy is ongoing.

[Prenatal diagnosis of a right thoracic congenital ectopic kidney with a diaphragmatic hernia: a combination with a good prognosis]. / Diagnostic anténatal d'un rein droit thoracique et d'une hernie de coupole diaphragmatique droite: une association de bon pronostic.

Ectopic intrathoracic kidney is a rare congenital anomaly, usually asymptomatic. This anomaly is sometimes associated with a diaphragmatic hernia. Few cases of this combination have been described, often in the absence of a prenatal diagnosis. We report on the case of a female newborn infant who was diagnosed with an ectopic intrathoracic right kidney and a diaphragmatic hernia upon 33 weeks of gestation. The patient underwent surgery on the first day of life and the respiratory and renal outcomes were simple. We review the literature and discuss the seemingly good prognosis of this combination.

Antenatal detection of subdiaphragmatic pulmonary sequestration: a case report.

Extralobar pulmonary sequestration is part of the spectrum of bronchopulmonary foregut malformations. Usually found in the thorax, it may be located in the retroperitoneum. We report one case of subdiaphragmatic pulmonary sequestration detected by prenatal ultrasound, and diagnosed at surgery after birth. This case illustrates the diagnostic and therapeutic problems occurring during pregnancy and the neonatal period.

Congenital pseudarthrosis of the clavicle and thoracic outlet syndrome in adolescence.

A 15-year-old girl with thoracic outlet syndrome associated with congenital pseudarthrosis of the clavicle was examined. The indication for treatment of congenital pseudarthrosis of the clavicle is discussed.

[Urination disorders revealing posterior urethral valve: radiological aspects]. / Troubles mictionnels révélateurs de valves de l'urètre postérieur: aspects radiologiques.

Posterior urethral valves represent the most common form of congenital urethral obstruction; they are due to the presence of membranous recesses within the posterior urethra. Their consequences on the lower and upper urinary tract depend on the importance of the obstacle. Moderate forms have a late clinical onset in children who present with voiding disorders and a normal upper urinary tract; in such cases, the diagnosis is difficult since typical features are absent on the urethrocystogram. Radiological signs of urethral valves should be differentiated from congenital urethral strictures which are not obstructive and from bladder dysfunctions which modify both the bladder and the urethra during micturition.

[Lung abscess due to Mycoplasma pneumoniae in an adolescent]. / Abcès du poumon à Mycoplasma pneumoniae chez un adolescent.

UNLABELLED: Pulmonary abscess is an uncommon complication of pneumonia in children. Pyogenes, in particular Staphylococcus aureus or Streptococcus pneumoniae are the principal responsible bacteria. Mycoplasma pneumoniae is rarely the cause. CASE REPORT: A 14-year-old child was hospitalized with right thoracic pain. The patient was non-febrile and had a recent history of moderate infection. He was receiving antibiotic (macrolide) and non-steroidal anti-inflammatory therapy. CT scan confirmed a mid-lobe abscess in the right lung. Interruption of therapy resulted in fever and increase in C-reactive level with hyperleucocytosis, suggesting that the abscess was caused by a bacterial infection. The child's general condition and the radiographic picture improved with combined antibiotic therapy with amoxycillin and clavulanic acid, aminoglycosides and macrolides. The suspected diagnostic of M. pneumoniae was confirmed by increased IgM antibodies for M. pneumoniae. Recovery was complete two months later without sequelae. COMMENT: Pulmonary abscess is a rare complication of M. pneumoniae infection in children. This complication should be considered when the general condition does not improve despite appropriate early treatment of a pneumonia, as in the case of our patient.

[Intermittent ureteropelvic junction obstruction and aberrant vessel to the lower pole of the kidney in children]. / Obstruction intermittente de la jonction pyélo-urétérale et vaisseaux polaires inférieurs chez l'enfant.

Intermittent ureteropelvic junction obstruction in children is a distinct clinical syndrome, it is often associated with an anteriorly crossing aberrant vessel to the lower pole of the kidney. The presence of these vessels increases the risk of complication and requires surgical management. CT scan is already the best exam to find these vessels in adults before treatment. It seems to be also efficient in children. This case report illustrates this indication.

[Autoimmune hemolytic anemia associated with a mature ovarian teratoma]. / Anémie hémolytique auto-immune associée à un tératome mature de l'ovaire.

UNLABELLED: The association of autoimmune hemolytic anemia and mature teratoma of the ovary is rare, particularly in childhood, but must be known and looked for since the treatment of teratoma allows to cure anemia as well. CASE REPORT: A 9 year-old girl was admitted for hemolytic anemia. The etiologic work-up revealed an autoimmune mechanism (IgG autoantibodies with complement), as well as an ovarian tumor after ultrasound sonography of the abdomen and pelvis. Surgical excision of the tumor was complete and uncomplicated. Pathological examination concluded to a mature teratoma. Anemia, as well as the signs of autoimmunity, disappeared a few weeks later and the child is doing well with several months of follow-up. CONCLUSION: This second reported pediatric case shows that an ovarian teratoma should be searched for with ultrasound sonography in any girl presenting with autoimmune hemolytic anemia, since surgical excision is sufficient to cure both anemia and the tumor.

[Imaging of the hip in healthy children in MRI: evaluation of the acetabular coverage]. / Imagerie de la hanche de l'enfant sain en IRM: évaluation de la couverture cotyloïdienne.

PURPOSE: To define with MR imaging a reference value for acetabular coverage on coronal and sagittal images for a population of healthy children. MATERIALS AND METHODS: 36 children (72 hips) where prospectively studied over 1 year. T1-weighted spin-echo images in both coronal and sagittal planes were obtained. Slice thickness was 4 mm. Measurement of acetabular coverage (AHI: Acetabular-Head Index) was made in both planes with two different landmarks: bone and cartilage. Study of the evolution with age was made. RESULTS: The acetabular coverage is symmetrical and decreases with age. Its measurement is reproducible. CONCLUSION: This study shows that measurement of acetabular coverage (AHI) is easily obtained at MR imaging and is reproducible. We suggest 83 (mean 1SD) as the inferior limit for AHI based on cartilage measurements. We introduce sagittal values, never published. We believe that MR, by its ability to demonstrate the cartilaginous surfaces, will play a major role in the true understanding of the anatomical relationship between the femoral head and the acetabulum.

[Clinical course and treatment of pleural empyema in children]. / Evolution clinique et traitement de l'empyème pleural chez l'enfant.

BACKGROUND: Purulent pleurisy has become rare. It is often masked by previous antibiotic treatment so that functional prognosis may be poor. PATIENTS AND METHODS: Twenty children with purulent pleurisy of the large cavity admitted from 1987 to 1993 were included in the study: there were nine infants (age 5 to 18 months) with pleuro-pulmonary staphylococcal infection (group I) and 11 children (4-13 years) (group II). Clinical, biological, bacteriological and radiologic findings were analysed retrospectively as was the outcome. RESULTS: Patients of group I were admitted in poor general condition. X-ray showed moderate effusion and characteristic signs of staphylococcal infection. The bacteria identified in seven patients (77%) was S aureus. Recovery was rapid with antibiotics and simple local treatment. X-rays were normal two months after hospital discharge in seven patients (77%). One infant presented cicatricial bullous emphysema which required segmental resection. Patients of group II were admitted for moderate respiratory signs after a relatively long delay (14 days) since the onset of symptoms. X-rays showed considerable effusion in all and mediastinal shift in five patients (45%). Streptococcus pneumoniae was identified in one patient only. Local treatment of empyema was difficult; the effusion, already fibrinous, required repeated use of chest tubes in eight cases and surgical decortication in three. X-rays, performed 2 months after hospital discharge, were normal in only three patients. Long-term course was nevertheless favorable since chest X-rays at 5 months were normal in all children of both groups. CONCLUSIONS: Early recognition of purulent pleurisy is important in children aged over 3 years to ensure effective drainage before the effusion becomes fibrinous. All patients in whom the first tube was inserted after more than 10 days had a difficult follow-up requiring repeated chest drainages or surgery. Ultrasonography was a useful aid for diagnosis and local treatment. Computed tomography was useful for adapting treatment after several days of course.

Mesoblastic nephroma: prenatal ultrasonographic and MRI features.

Antenatal detection of mesoblastic nephroma by US is possible. Reviewing the literature, we found 19 previously reported cases, only 1 of which underwent prenatal MRI. We present a further case diagnosed by US and confirmed with MRI. The imaging findings and differential diagnoses are discussed. Early and correct detection of this rare entity is of great interest, as it may facilitate prevention and management of severe obstetric and neonatal complications such as polyhydramnios and prematurity. MRI can help to evaluate the origin and the morphological features of a fetal abdominal mass.

Percutaneous resection of osteoid osteoma under CT guidance in eight children.

In eight children with suspected osteoid osteoma, a percutaneous resection under CT guidance was performed. The specific drill resection system we currently use includes a 7-mm-diameter toothed drill. Osteoid osteomas were located in the appendicular skeleton in seven children and in the spine (second lumbar vertebral body) in one. All eight patients were successfully treated with complete relief of pain. There was no recurrence of symptoms during a follow-up period of 8-35 months. CT guidance was accurate enough to allow a focal bone excision, and no subsequent bone repair was needed. Histological confirmation was obtained in all cases. This simple and safe technique, when feasible, seems to be an effective means of treatment of osteoid osteoma in children.

[Management of a biliary cyst disclosed prenatally. Apropos of 2 cases]. / Conduite à tenir devant un kyste biliaire de découverte anténatale. A propos de 2 observations.

Two cases of prenatal detection of congenital biliary cysts are reported. Prenatal and postnatal ultrasonography demonstrate a solitary sonolucent cystic lesion. The differential diagnosis in fetus and infant includes any abdominal cystic lesions: ovarian cyst, duplication cyst, cystic lymphangioma, choledochal cyst and other congenital cysts of the liver. In opposite to the difficulty of diagnosis, the choice for treatment is easy: surgery is the only way to allow histologic diagnosis and to avoid any relapse or complications.