Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment.

Scimitar syndrome is a variant of partial anomalous pulmonary venous return with an aberrant vein, the Scimitar vein, draining the right lung to the inferior vena cava instead of the left atrium, resulting in a left-to-right shunt. The classic frontal radiographic finding, designated as "the scimitar sign", is of a scimitar (a Turkish sword) shaped density along the right cardiac border. The diagnosis can be made by echocardiography, and cardiac catheterisation remains the gold standard to assess the left-to-right shunt. However, the place of multimodal cardiac imaging by computed tomography and magnetic resonance imaging is increasing. We report the case of a 26 year-old man presenting with chest pain during a brief panic attack, in whom scimitar syndrome was associated with a bicuspid aortic valve, a clinical association rarely reported in the literature. CT and MRI cardiac imaging was as accurate as echocardiography and hemodynamics, particularly for shunt quantification.

A rare case of ectopic intrathoracic kidney in a 1-year-old child.

Congenital thoracic ectopic kidney is a rare developmental anomaly, with the thorax being the rarest and higher location of ectopic kidneys. This anomaly is usually asymptomatic and discovered incidentally on routine chest radiography. At least 200 cases of thoracic kidney have been described, with the vast majority of cases documented in adults. To our knowledge, this is only the sixth case of congenital ectopic thoracic kidney described in a child. Herein, we report a rare case of a 1-year-old child exhibiting ectopic intrathoracic kidney. We further discuss the embryonic background and the current management of this pathology.

Impact of fetal growth restriction on body composition and hormonal status at birth in infants of small and appropriate weight for gestational age.

BACKGROUND: Fetal growth restriction (FGR) has been related to several health risks, which have been generally identified in small-for-gestational age (SGA) individuals. OBJECTIVE: To evaluate the impact of FGR on body composition and hormonal status in infants born either small- or appropriate-for-gestational age (AGA). METHODS: Fetal growth was assessed by ultrasound every 4 weeks from mid-gestation to birth in 248 high-risk pregnancies for SGA. Fetal growth velocity was calculated as change in the estimated fetal weight percentiles and FGR defined as its reduction by more than 20 percentiles from 22 gestational weeks to birth. Impact of FGR on body composition, cord insulin, IGF-I, IGF binding protein-3 (IGFBP-3), and cortisol concentrations was assessed in SGA and AGA newborns. RESULTS: Growth-retarded AGA infants showed significantly reduced birth weight, ponderal index, percentage of fat mass, and bone mineral density when compared with AGA newborns with stable intrauterine growth. Cord IGF-I and IGFBP-3 concentrations were significantly decreased in growth-retarded infants in both SGA and AGA groups. Cord insulin concentration was significantly lower and cord cortisol significantly higher in AGA infants with FGR versus AGA newborns with stable intrauterine growth. After adjustment for gestational age and gender, birth weight was directly related to fetal growth velocity and cord IGF-I concentration. The variation in infant's adiposity was best explained by fetal growth velocity and cord insulin concentration. CONCLUSIONS: FGR affects body composition and hormonal parameters in newborns with birth weight within the normal range, suggesting these individuals could be at similar metabolic risks as SGA. .

Open vs thorascopic surgical management of bronchogenic cysts.

BACKGROUND: The aim of this study was to compare the operative outcome in children undergoing open vs thoracoscopic resection of bronchogenic cysts. METHODS: The medical records of children who underwent the resection of bronchogenic cysts from 1990 through 2000 were reviewed. Four cyst resections were performed by the open technique and five using a thoracoscopic procedure. The age of the patients, length of hospital stay, duration of drainage, operating time, and outcome were investigated. RESULTS: The mean age of patients undergoing the open procedure was 3 years and 3 months; the mean age for thoracoscopy patients was 7 years and 10 months (p < 0.05). The operating time for the open procedure was 70 +/- 25 min; for the laparoscopic procedure, it was 78 +/- 6 min (p, NS), except in one case with a main bronchial tail that required conversion (320 min). Duration of surgical drainage was 6.5 +/- 3 days for the open procedure and 2.5 +/- 1 days for the thoracoscopic one (p < 0.05). Hospital stay for open patients was 12 days +/- 0 days; it was 6 +/- 1.6 days for thoracoscopic patients (p < 0.01). There were no deaths. The thoracoscopic procedure failed once due to a main bronchial tail and had to be converted to an open procedure. Other early complications included a bronchopulmonary infection after an open cyst excision and an atelectasis after a thoracoscopic cyst excision. Late complications included one reoperation for incomplete excision in each of the two groups. CONCLUSION: Bronchogenic cyst resection can be performed safely. For complete treatment of these patients, total excision of the wall cyst is needed. In selected patients, the thoracoscopic procedure may decrease the duration of surgical drainage and length of hospital stay without increasing the operating time or MSK for complications.

[Low bone mineral density in children with Crohn's disease]. / Déminéralisation osseuse au cours de la maladie de Chron de l'enfant.

UNLABELLED: Recent studies have reported low bone mineral density in children with Crohn's disease. The aims of this retrospective study were to quantify its frequency and to search for risk factors. POPULATION AND METHODS: Bone mineral density of 29 children with Crohn's disease was measured by dual-energy X-ray absorptiometry. All the children were taking calcium and vitamin D, during all the follow-up. RESULTS: Osteoporosis (Z-score < or = -2.5 S.D.) was found in 38% of the children, and osteopenia in 38% (Z-score between -1 and -2.5 S.D.). Low bone mineral density was correlated with age, suggesting it begins with puberty. Daily corticosteroid exposure was significantly higher for patients with osteoporosis. Disease severity measured with Harvey-Bradshaw index and exposure to immunosuppressive drugs were almost statistically significant. Sex, height, duration and site of disease, nutritional assistance exposure were not associated with low bone mineral density. CONCLUSION: This study confirms the high frequency of low bone mineral density in children with Crohn's disease, mainly during puberty. Corticosteroid exposure is a risk factor, and the disease severity, a probable one (non significant). New treatment strategy has to be defined to prevent and to treat this complication.

Isolated pericardial effusion in the human fetus: a report of three cases.

OBJECTIVE: Our objective was to determine the possible underlying etiologies and outcome in isolated fetal pericardial effusion. METHODS: Doppler fetal echocardiography allowed the diagnosis of pericardial effusion in three patients and revealed the etiology in two. RESULTS: We present the findings in three cases of isolated pericardial effusion. In the first, the pericardial effusion was a manifestation of trisomy 21 associated with a myeloproliferative disorder. In the second, the pericardial fluid collection was the first sign of an autosomal recessive disease, idiopathic infantile arterial calcification. The third case was remarkable because of the spontaneous resolution of a large pericardial fluid collection. CONCLUSION: Isolated fetal pericardial effusion covers a wide spectrum of etiologies from severe genetic and chromosomal diseases to transient forms.