Parosteal osteosarcoma of the thumb: A case report and literature review.

Parosteal osteosarcoma (PO) is a rare malignant tumor arising from the surface of the bone. Locations in the hand are even more exceptional. This low-grade osteosarcoma shows non-specific clinical and radiological presentation, making diagnosis challenging. Moreover, histologic examination is extremely difficult and can easily lead to misdiagnosis. We report the case of a 21-year-old woman who presented PO of the right thumb, initially diagnosed as a "benign exostosis" 9 years previously. En-bloc resection followed by reconstruction using a free corticocancellous iliac crest autograft provided good esthetic and functional outcome. No recurrence occurred at 2 years' follow-up. Our literature review confirmed the rarity of PO of the hand, with only 8 cases reported in the past 60 years. Amputation was the main treatment, but some authors reported limb-sparing surgery. The present result and those in the literature review support conservative surgery when feasible, with little recurrence and better functional and esthetic results. These rare tumors should not be misdiagnosed, and should be treated in specialized centers to optimize outcome.

[Infusion of vasoactive drugs at low flow rate: effects of the syringe switch and syringe volume (experimental study)]. / Perfusion des médicaments vasoactifs à très faible débit : influence des modalités du relais et du volume des seringues (étude expérimentale).

OBJECTIVE: The flow rate of intravenous administration of vasoactive agents should be steady in order to prevent changes in hemodynamics. In the newborns, because the flow rate is often lower than 1mL/h, it is difficult to switch of the syringe. The aim of our study was to compare the variations of concentration of active substance delivered after a manual or an automatic switch off the syringe with three different volumes (10mL, 20mL and 50mL). MATERIALS AND METHODS: Glucose solution (10g/L) was used to simulate the administered substance. Saline was administered in "Y" simultaneously with the glucose solution through a catheter. The infused substance was collected at the tip of the catheter. The glucose concentration was measured at 15min-interval for 3hours. RESULTS: The manual switch of the syringe was associated with a significant alteration of the flow rate, lasting more than 15min. In contrast, the automatic switch of syringes was associated with no change of the flow rate, especially with small-volume syringes (10mL). CONCLUSION: In newborns, in order to prevent the change in flow rate of drugs after a switch of syringes, our results suggest the use of syringe-pump with built-in automatic switch and small-volume syringes.

[The long-term follow-up of patients with a congenital diaphragmatic hernia: review of the literature]. / Hernie de coupole diaphragmatique: devenir à long terme.

The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.

[Per and post-natal medical management of congenital diaphragmatic hernia]. / Prise en charge médicale per et post-natale de la hernie congénitale du diaphragme.

Congenital diaphragmatic hernia (CDH) is a 1 out of 3500 live-born malformation with persistent 30-40% mortality rate, related to severe pulmonary hypoplasia and hypertension. Better knowledge on the mechanisms inducing failure of adaptation at birth is a prerequisite for improving CDH prognosis. CDH is also associated with longterm morbidity, including prolonged respiratory failure, failure to growth, oral aversion, and scoliosis. Early prevention starting as soon as the first hours of life are required to reduced long term morbidity. The aims of the management are not only to reduce early mortality, related to persistent pulmonary hypertension, but also to prevent late morbidity.

Neurodevelopment of children born very preterm and free of severe disabilities: the Nord-Pas de Calais Epipage cohort study.

AIM: To describe the development of very preterm children free of cerebral palsy or severe sensory impairment in the domains of gross and fine motor functions, language and sociability at a corrected age of 2 years; to identify factors associated with performances in each domain. METHODS: A total of 347 children born in 1997 before 33 weeks of gestation, part of the EPIPAGE population-based cohort study, had their psychomotor development assessed with the Brunet-Lezine scale. RESULTS: The study population had a mean gestational age of 30.1 +/- 2.0 weeks. Lower developmental quotients (DQ) were observed in the study group compared to the reference sample (96 +/- 13 vs 104 +/- 8, p < 0.01). Fine motor function, language and sociability were all affected with a p value <0.01. Multivariate analysis showed that duration of intubation and parents' educational and occupational levels were the only variables significantly related to each developmental domain (p < 0.01). CONCLUSIONS: Children very preterm and free of severe disabilities had mild delays in multiple areas of development. The mechanisms by which neonatal factors played a role need further investigation. However socioeconomic status had a great impact on development and our results underline the need for improved support of socioeconomically disadvantaged parents after a preterm birth.

[Management of severe respiratory failure in the newborn: indication of inhaled NO]. / Prise en charge de la détresse respiratoire sévère du nouveau-né: place du NO inhalé.

Severe respiratory failure results from large right to left shunting. In the newborn, both intrapulmonary and/or extrapulmonary shunting may cause severe respiratory failure. Right to left extrapulmonary shunting is associated with pulmonary hypertension (PPHN). Management depends on the main mechanism of the respiratory failure. Intrapulmonary shunting requires optimizing lung recrutment, whereas vascular recrutment is required to decrease extra-pulmonary shunting. Analysis of anamnesis, clinical examination, chest X-ray, and echocardiography allows distinguishing the mechanism of the respiratory failure. In case of PPHN, favorizing factors such as acidosis, stress, lung over distension, and polyglobuly should be treated. Inhaled NO is recommended in the treatment of PPHN after optimizing lung volume.

[Umbilical and peripherally inserted venous central catheterization of the newborn]. / Le cathétérisme veineux ombilical et épicutanéocave chez le nouveau-né

Umbilical venous and peripherally inserted venous central catheters are widely used to perfuse low-weight preterm and term newborns in intensive care units. This catheter must be inserted carefully and monitored rigorously to prevent complications. This paper develops today's knowledge on the use and complications in the newborn population.

[Central venous catheters in pediatric patients]. / Le cathétérisme veineux central chez l'enfant.

Central venous catheterizations are often used in pediatric intensive care units or for long-term intravenous treatment. It consists in positioning the catheter extremity in the venous cava-right atrium junction. Adapted material and techniques are necessary for young children because of particularities in anatomy and the size of the different venous trunks. The aim of this paper is to present the different material and techniques and to show the indications, complications and follow-up in central venous catheterization for young children.

[Maternal and foetal anaesthesia for ex utero intrapartum treatment (EXIT) procedure]. / Anesthésie maternofoetale pour "ex utero intrapartum" traitement (EXIT) procédure: à propos de deux cas.

The ex utero intrapartum treatment (EXIT) procedure is a surgical procedure maintaining utero-placental circulation during caesarean section. Anaesthetic implications are described: foetal transplacental anaesthesia to avoid first breathing and to permit surgical procedure on obstructed foetal airway, deep maternal haemodynamically stable anaesthesia to relax uterine smooth muscle during a long caesarean procedure but avoiding post-partum haemorrhage. Volatile anaesthesia with sevoflurane seems to be adequate for these aims. Two cases are described.

[Gluthathion synthetase deficit in a newborn infant]. / Déficit en glutathion synthétase (à propos d'un cas).

CASE REPORT: We report an observation of a triplet newborn presenting with haemolysis, metabolic acidosis with no lactic acidosis revealing a glutathione synthetase deficiency. These biological signs were associated with multiple malformations (IUGR, toes hypoplasia and cerebral ventricular anomalies), not described in this disease. CONCLUSION: This rare diagnosis can be confirmed by elevation of urinary 5-oxoproline. Prognosis is linked to diagnosis and treatment precocity. We have no argument to think that the malformations we found are related to a glutathione synthetase deficiency. However, as the neurological evolution is often unfavourable, neuroradiological explorations could give information about the location and severity of potential cerebral lesions.

Neurological assessment at five years of age in infants born preterm.

AIM: To evaluate the agreement between Touwen's neurological examination and a derived simplified one, created to be applied at the age of 5 y to infants born preterm. METHODS: 185 children born at a gestational age (GA) of <33 wk and/or with a birthweight <1501 g, free of cerebral palsy, underwent Touwen's neurological examination at a mean age of 5 y and 8 mo (5-6.5 y). One-hundred and seventy had a full examination and were included into the study. They were born at a mean GA of 30 wk (range 24-35 wk) with a mean birthweight of 1250 g (range 600-2690 g). A simplified examination, based on the clinical experience of two of the authors, was created a priori. The data were reviewed retrospectively and the concordance between the two forms was assessed. The reviewers were blinded to the original categorization from the long form. RESULTS: On the basis of the original Touwen's neurological examination, the 170 children were classified into 122 with a normal neurological examination, 41 with grade 1 minor neurological dysfunction (MND) and 7 with grade 2 MND, giving 28% of the cohort with MND. The concordance between the two forms was excellent, with an agreement in 169 out of 170 subjects. None of the infants with the most complex form of MND was misclassified. CONCLUSION: The simplicity of this examination could allow its diffusion and its use in follow-up programmes. It could improve the quality of routinely collected follow-up data of preterm neonates.