[Prenatal diagnosis of chylothorax]. / Le chylothorax de découverte anténatale.

Chylothorax is defined as the presence of lymph in the pleural space. Congenital chylothorax is one of the most frequent causes of fetal pleural effusion. It may be primary or secondary. Careful assessment of the etiology and of possible associated anomalies is required. Main complications are pulmonary hypoplasia, hydrops fetalis and the risk of premature delivery. Management is still a mater of controversy, the diagnosis of fetal pulmonary hypoplasia being difficult in utero. Factors such as gestational age, evolution of pleural effusion on two weeks, signs of seriousness (hydrops fetalis), and pulmonary expansion after pleural puncture may help the physician to choose between abstention, pleural tapping or long-term in utero drainage. Post natal treatment consists of pleural drainage and assisted ventilation in cases of respiratory distress, correction of metabolic and immune disorders and exclusive parenteral nutrition. Once chylothorax is resolved, formula feeding without long-chain triglycerides is allowed. If pleural effusion persists despite a well conducted treatment, albumin infusion and diuretics may be used before considering surgical treatment.

[A rare complication of multiple exostoses: hemothorax]. / Une complication rare de la maladie exostosante: l'hémothorax.

BACKGROUND: Hemothorax is a rare complication of hereditary multiple exostosis. CASE REPORT: A 12 year-old boy suffered from abrupt thoracic pain, firstly attributed to pleural effusion. He had hereditary multiple exostosis known since the age of 9 years. The patients was given anti-inflammatory drugs and erythromycin but the pleural effusion became more abundant 6 days later requiring thoracentesis which showed hemothorax. All bacteriological and cytologic investigations were negative. X rays, ultra-sonography and CT scan showed several costal exostoses developing into the thoracic cavity. The hemothorax disappeared within 12 days and the patient was well 4 months later, without pleural sequelae. CONCLUSIONS: Hemothorax may be due to internal costal exostosis. It may be cured with thoracentesis; more aggressive therapy should be performed in exceptional cases with severe and/or recurrent bleeding.

[Role of echography in the screening of congenital hip luxation at a maternity department]. / Place de l'échographie dans le dépistage de la luxation congénitale de hanche à la maternité.

Ultrasound was established as a remarkable means of recognizing dysplasia of hips or dislocations in the neonatal period following the work of R. Graf and later in France of H. Gomes and J. Schirrer et al. All the same, different techniques have been suggested and the role of the examination has been debated. Those who advocate routine screening of all pregnancies are in opposition to those who employ it selectively in cases that are known to be abnormal or at risk. The authors report a study carried out in their maternity department using ultrasound of the hip as an examination complementary to clinical screening. Echography has been shown to be able to give more information than radiography of the pelvis which cannot usefully be employed for screening until the age of 4 months. In 1781 newborns examined consecutively the indication for ultrasound of the hip was suggested in 201 cases (11%) and showed an abnormality in 42 patients (21% of the examinations carried out). Exenteration in 2, dysplasia in 8, failure to ossify correctly in 25, and endoscopic laxity without a morphological abnormality in 7 cases. The clinical signs that were used to predict an abnormality on ultrasound were: feminine sex. Clinical appearance of instability and the association of 2 risk factors. 3 cases of dislocation were found. Two of them occurred early and one at the age of 5 months who was a girl delivered by the breech and in whom a delay in ossification was noted at birth.(ABSTRACT TRUNCATED AT 250 WORDS)

[Idiopathic pulmonary hemosiderosis and celiac disease in a child. Case report]. / Hémosidérose pulmonaire idiopathique et maladie coeliaque chez l'enfant. Une observation.

An enteropathy with almost total atrophy of the villi was discovered in a 13-year old girl with idiopathic pulmonary haemosiderosis. Coeliac disease was strongly suspected but could not be proven. This case draws attention to the possible association of an idiopathic pulmonary haemosiderosis with a coeliac disease.

[Flexible bronchoscopy in children. Experience at French centers of pediatric pneumology]. / La fibroscopie bronchique chez l'enfant. Expertise des centres français de pneumologie pédiatrique.

INTRODUCTION: Fibreoptic bronchoscopy (FB) is an important diagnostic examination in paediatric pulmonology. In 2002 the Paediatric Pulmonology and Allergy Club undertook a retrospective study to establish the current status of fibreoptic bronchoscopy among its members. METHODS: In 2001 sixty five paediatric pulmonologists carried out an average of 116 examinations (+/- 111) in 35 paediatric centres. FB was performed either in an operating theatre (15 centres), a dedicated bronchoscopy suite (6 centres) or an endoscopy suite shared with gastro-enterologists (7 centres). Other examinations were performed in areas dedicated to, or associated with intensive care. General anaesthesia was routinely used in 18 centres. The others used sedation including an equimolar mixture of oxygen and nitrous oxide in 14 centres. Ten centres performed less than 50 examinations, 12 between 51 and 100, 4 between 101 and 200 and 8 centres more than 200 in the year. Seventy two per cent of the children were less than 6 years old. The washing and disinfection procedures were manual in 20 centres and automatic in 15. RESULTS: Three principal indications were reported: persistent wheezing, suspicion of a foreign body and ventilatory difficulties. Cough, desaturation and fever were the most frequently reported side effects. CONCLUSIONS: This is the first survey in paediatric pulmonology in France. It shows a wide variation in the practice of fibreoptic bronchoscopy in children.

[Dissociation of asthmatic syndromes in children]. / Dissociation des syndromes asthmatiques de l'enfant.

Allergic sensitization is not always the only factor in bronchial asthma in the young child, so that conventional management is often poorly effective. The main event is airflow obstruction, caused by widespread airway narrowing due to bronchospasm, mucus secretion, hypersecretion and inflammatory mucosal edema. Airway inflammation seems to be the most important factor associated with release of chemical mediators and the cellular phase of inflammation. Origin of this activation may involve exposure to environmental stimuli such as allergens, infection or pollutants. Bronchial hyperreactivity probably develops at the same time although genetic factors are likely to predispose the children to develop hyperresponsiveness. Different processes can result in bronchial obstruction and explain the forms of asthma in young children: allergic, infectious or complex asthma. Since the bronchial inflammatory reaction appears to be the most important factor in the subsequent development of asthma in young children, steroid inhalation is advocated, associated with conventional therapy.

[Spinal intradural arachnoid cyst]. / Kyste arachnoïdien intra-dural rachidien.

The authors report the case of a 13 year-old boy who presented after minor cranial trauma with severe gastrointestinal symptoms (acute hemorrhage) followed by neurologic signs evoking a spinal compression. Gastro-intestinal hemorrhage was related to a duodenal lesion induced by acetylsalicylic acid. Neurologic symptoms were related to an intra dural cervical arachnoid cyst. After surgical resection of this congenital malformation outcome was favorable.

[Pulmonary surfactants. Generalities]. / Le surfactant pulmonaire. Généralités

The alveolar wall on contact with air can be compared with a biological air-liquide interface. As with all interfaces, there are therefore superficial forces which tend to reduce the surface to a minimum. In the case of a pulmonary alveolus with a spherical surface, these forces are at the origin of an internal pressure excess dependent on the radius of the alveolus and on the superficial tension related to the nature of the interface. Owing to the disparity in the alveolar radii, under these conditions the smaller alveoli would collapse to the benefit of a larger one, the pressure being lower and lower in the latter. In addition, at any time in the respiratory cycles, this surpression must be negligible in order to avoid rupture of the equilibrium of the forces exerted on the alveolar wall. Consequently, it is necessary that this air-alveolar wall interface should have a superficial tension on the one hand variable with the surface, and on the other hand always very low. Owing to the demonstration of large concentrations of phospholipid in this area, it can be thought that a superficial film is substituted at the air-biological liquid interface and owing to this fact effectively has superficial properties necessary for alveolar stability. The "surface-tensio-active" effect of this film lead to the giving of the name of "surfactant" to these constituents as a whole.

[Bronchial atresia of a segment (author's transl)]. / L'atrésie bronchique segmentaire.

Bronchial atresia of a segment in the lung is a very uncommon cause of pulmonary disorder. The diagnosis is very easy. The plain film is sufficient. Distension of a segment with air trapping during the inspiratory phase with one or more tumor in the hile due to the impaction of mucous secretion of the bronchi above the obstruction. Frequently asymptomatic the revelation is accidental during the evolution of a communal upper respiratory tract infection. The treatment is surgical.

[Congenital tracheal stenosis and supernumerary bronchi]. / Sténose trachéale congénitale et bronche surnuméraire.

A child is described with a segmental congenital tracheal stenosis who also had a supernumerary bronchus with an accessory lobe arising from the trachea. Because of progressive tracheal obstruction, surgery to the trachea and bronchi was undertaken. The stenosis was relieved successfully.

[Desmopressin and water intoxication. Apropos of a case treated for enuresis]. / Desmopressine et intoxication par l'eau. A propos d'un cas traité pour énurésie.

A 4-year-old boy was treated with oxybutinine and desmopressine because of bladder instability associated with secondary enuresis. He was admitted with obnubilation, vomiting and experienced two seizure episodes concomitantly with hyponatremia and hypoosmolality. The child healed promptly under water restriction and intravenous administration of sodium chloride. This case report suggests that desmopressine may be responsible for severe side-effects. This drug should not be widely used and its indications should be restricted to patients with proven antidiuretic hormone secretion abnormalities.

[Duodenopancreatic contusions. Diagnostic difficulties in children]. / Contusions duodéno-pancréatiques. Difficultés diagnostiques chez l'enfant.

The authors describe a case of duodenopancreatic injury in a 6-year-old girl, with the clinical presentation of appendicitis. The findings point to the importance of a precise anamnesis, as in its mild form the traumatism is often poorly recognized, and may be incorrectly diagnosed. It is therefore recommended that a thorough clinical examination be carried out, with abdominal ultrasonography, X-ray barium and tomodensitometry if necessary. Frequently, as is the case with other abdominal, injuries, surgery may be avoided if the patient's condition is carefully followed.

[Pyloric stenosis with rare etiology]. / Sténose pylorique d'étiologie rare.

The authors report on a 30 month-old boy who experienced pyloric stenosis related to an hamartoma. This case shows the difficulties in such a clinical and histological diagnosis.

Coexistent hereditary coproporphyria and congenital erythropoietic porphyria (Günther disease).

We present data on one patient with an inheritance pattern for two porphyrias. From her mother she inherited the trait of hereditary coproporphyria; from both parents she inherited the trait of congenital erythropoietic porphyria (Günther disease). Enzyme studies confirmed this new type of dual porphyria.

[Pulmonary surfactant in neonatal pathology]. / Le surfactant pulmonaire dans la pathologie néonatale

Following some preliminary remarks on the role of the surfactant in pulmonary physiology and physiopathology, a physiochemical study of the lungs of 26 infants who died in the neonatal period is reported. Two techniques for measuring the decrease in surface tension are set out. The results in the first case (tensiometric method) allow an explanation of the physiopathological aspect of respiratory distress; in the second case (manometric method) the diagrams obtained are characteristic of different clinical states (normal subjects; subjects born prematurely without any hyaline membrane etc...). Furthermore, the second method gives rise to certain hypotheses as to the physiochemical structure of the substances responsible for the tensio-active properties of the surfactant.

[Unilateral small lung with translucent cystic appearance. Congenital or acquired origin? 2 case reports]. / Petit poumon unilatéral avec images claires d'aspect kystique. Origine congénitale ou acquise? Deux observations.

The distinction between congenital or acquired lung disease is often a difficult problem. This is illustrated by case reports on two boys aged 10 and 12. The clinical, pulmonary function and radiological details were very similar in the two cases, suggesting pulmonary hypoplasia. However, the histology seen in the second case, a type of bronchiolitis obliterans and follicular bronchiectasis were among several arguments in favour of acquired pulmonary disease of infective origin. The recorded literature as well as this observation show that certain respiratory viral infections may lead to bronchial and above all bronchiolar damage which can be severe and progressive.

[Pneumococcal pneumonia and septic shock in the newborn infant]. / Pneumopathie et choc septique à pneumocoque chez le nouveau-né.

Pneumococcal sepsis and pneumonia in the neonate are rarely reported. They appear either as an early-onset respiratory distress with a high mortality rate or as a delayed infection. The authors describe 3 term neonates with an early respiratory distress syndrome and recall the main points of this severe foeto-maternal infection. Neonatal pneumococcal sepsis is strikingly similar to early-onset group B streptococcal infection. The isolation of the germ in the mother's vaginal flora is hazardous. Such cases suggest that early respiratory support and intensive circulatory resuscitation lead only to a slight decrease in the mortality rate, and thus preventive antibiotherapy is a necessity.

Juvenile fibromatosis resembling aponeurotic fibroma and congenital multiple fibromatosis. One case with pleuropulmonary involvement.

This article deals with a young school boy born in 1971 with a tumor in the palm of his right hand. We have considered this a juvenile aponeurotic fibroma. This tumor has recurred several times, leading to unavoidable amputation of the right wrist and hand. Seven years after the initial diagnosis, the axillary area became involved, then the pleura and lung. Such an unusual process leads to reconsidering the diagnosis and to regard this case as an intermediate form between Keasbey's juvenile aponeurotic fibroma and generalized fibromatosis.