[Extracorporeal assistance in paediatric intensive care]. / Assistance extracorporelle en réanimation pédiatrique.

OBJECTIVE: For 3 years we have used extracorporeal assistance in intensive care frequently. This study evaluates our results. METHOD: We studied the patient records for those treated between January 2002 and January 2005. The method used, indications and morbidity/mortality were analysed. RESULTS: We performed 24 circulatory assistance procedures in 20 patients (median age: 5 months), arterio-venous with oxygenation (n=18), veino-venous with oxygenation (n=3) or biventricular (n=3). The indications were post cardiotomy cardio-respiratory failure (Group I; n=20, 16 patients), pure respiratory failure (Group II: n=1), or pre-transplant/recovery (Group III: n=3). Five procedures (4 from group I and 1 from group III) required cardiac massage (no fatalities). The average duration of assistance was 7 +/- 6 days (2 to 20 days). Treatment was successfully discontinued in sixteen patients 80%), one of them thanks to heart transplant. Four (20%) died during assistance. The morbidity essentially consisted of further surgery for haemostasis, multiple transfusions, and infections. Three patients (15%) died later (1 at 17 months after discontinuation) from complications unrelated to the assistance. No neurological sequelae were noted in the survivors. CONCLUSION: These results confirm the usefulness of circulatory assistance when medical treatment has failed, particularly in the post-operative period of paediatric cardiac surgery or while awaiting transplantation.

Kidney function in cyclosporine-treated paediatric pulmonary transplant recipients.

BACKGROUND: Lung or heart-lung transplantation is a useful therapy in life-threatening pulmonary disorders during childhood. Cyclosporine A is a major immunosuppressive treatment but has a number of adverse effects including nephrotoxicity. There have been no reports on the long-term evolution of renal function in a large series of paediatric pulmonary transplantation recipients. METHODS: We examined 19 patients followed up for at least 3 years after pulmonary transplantation. The mean time of follow-up was 5.36 years. Kidney function was evaluated by calculation of glomerular filtration rate (GFR) according the Schwartz formula. RESULTS: The GFR was normal before transplantation in all patients. The short-term evolution of GFR was marked by a significant drop during the first and until the 6th month. Then, regardless of the level reached at the end of the 6th month, the GFR remained stable in all patients except one until the end of follow-up. At the end of follow-up, 31% had normal GFR, 57% had mild chronic renal failure, and 5% had advanced renal failure. Hypertension was frequent and associated with renal failure. CONCLUSIONS: Paediatric pulmonary recipients showed evidence of long-term cyclosporine A-associated nephrotoxicity. Most of this toxicity occurred during the first 6 months.

Evaluating the prognosis of multiple, severely traumatized children in the intensive care unit.

OBJECTIVE: To assess the Pediatric Risk of Mortality (PRISM) score and to identify other prognosis factors in severe, multiple trauma in children. DESIGN: Retrospective study over a 9-year period. SETTING: A Pediatric Intensive Care Unit (PICU) in a University Hospital. PATIENTS AND PARTICIPANTS: One hundred and thirty-three traumatized children, 8.6 years (8 months-16 years), were reviewed. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Pediatric Trauma Score (PTS), Injury Severity Score (ISS), New ISS (NISS), Glasgow Coma Scale (GCS) score, and PRISM were calculated. The areas under the Receiver Operating Characteristic (ROC Az) curves, were compared. Univariate and multivariate analyses were performed. The mortality rate was 25.6%. PRISM performed well for discrimination between survivors and non-survivors. Az PRISM 0.9387 (0.029) was not different from Az GCS score 0.9451 (0.027) (P=0.568), but was significantly different from Az ISS 0.756 (0.052) (P<0.001), Az NISS 0.7606 (0.051) (P<0.001), and Az PTS 0.8244 (0.047) (P=0.016). Death was significantly associated with head trauma (P=0.014), PRISM >35, PTS <5, GCS <7, and ISS or NISS >32 (P<0.00001). PRISM >35 (P=0.001) and GCS <7 (P=0.003) were independent risk factors of death. CONCLUSIONS: PRISM is a reliable tool for evaluating the prognosis of multiple, severely traumatized children. Its relative simplicity and the fact that it is extremely widespread as a general prognosis score in PICUs represent other arguments for its use. Due to the leading influence of head trauma on mortality, GCS, a score even simpler than PRISM, showed identical accuracy regarding survival prediction.

Successful treatment of fungal right atrial thrombosis complicating central venous catheterization in a critically ill child.

A 9-year-old boy was admitted to our pediatric intensive care unit after multiple trauma. On the 17th day post trauma, he developed catheter-related sepsis with candidemia. After removal of the catheter and 6 days of unsuccessful intravenous antifungal therapy, conventional and transesophageal two-dimensional echocardiography was performed revealing a large right atrial thrombus. Surgical thrombectomy under cardiopulmonary bypass was performed and the patient recovered within a few days. Fungal right atrial thrombus is a rare, life-threatening complication of central venous catheterization. Two-dimensional echocardiography is a simple and effective diagnostic technique that should be performed when candidemia is detected. The proper therapeutic response depends on the findings of this examination. For a symptomatic patient with a large, mobile thrombus, we strongly recommend thrombectomy. Surgery not only allows removal of the mass and thus elimination of the mechanical complication but is also a key to management of infection.

Double-lung transplantation in children: a report of 20 cases. The Joint Marseille-Montréal Lung Transplant Program.

In the last 3 1/2 years, we have performed 20 double-lung transplantations in children between 7 and 16 years old (mean age, 13 years). One patient had primitive bronchiolitis obliterans and the other 19, cystic fibrosis. Eight patients were operated on in an emergency situation, 7 of them requiring ventilator support before transplantation. The procedures were en bloc double-lung transplantation in the first 11 patients with separate bronchial anastomoses in 10, and sequential bilateral lung transplantation in the later 9 patients. There were no operative deaths. Two patients died in the hospital on postoperative days 37 and 73, and there were four late deaths, which were due to infection, rejection, and bronchiolitis obliterans. The acceptable incidence of airway complications, the improvement in lung function of survivors, and the acceptable midterm survival make double-lung transplantation an acceptable alternative to heart-lung transplantation in children. However, in very small children, heart-lung transplantation may be preferable because of the size of the airway anastomoses at risk.

Double-lung transplantation in mechanically ventilated patients with cystic fibrosis.

Many lung transplant programs consider ventilator dependence as a contraindication for transplantation. Among 54 patients in whom bilateral lung transplantations for cystic fibrosis were performed by the Joint Marseille-Montreal Lung Transplant Program, 10 were ventilator dependent. Three of them died in the early postoperative period (30%): 2 as a result of cerebral anoxia and sepsis, 1 of Pseudomonas cepacia pneumonia. Two patients died at 15 and 19 months after transplantation of obliterative bronchiolitis and secondary bacterial pneumonitis. Another 2 patients in whom obliterative bronchiolitis developed underwent retransplantation with a heart-lung block; 1 of those was operated on at 12 months and is well at 29 months after his initial transplantation; the second was operated on at 34 months and died of primary graft failure. Three other patients are alive and well at 3, 11, and 14 months after transplantation. Actuarial survival at 1 year was 70%. The postoperative course and the infectious and rejection complications were no different from those in patients who underwent transplantation while spontaneously breathing. Obliterative bronchiolitis developed in 66% of patients at risk (2 of 6 patients surviving more than 6 months). We conclude that transplantation in mechanically ventilated patients with cystic fibrosis is not associated with an increase in morbidity or mortality after bilateral lung transplantation. Long-term survival, as in patients who undergo transplantation while spontaneously breathing, is limited by the development of obliterative bronchiolitis.

Delayed cure of an omphalocele requiring abdominosternoplasty, right hepatectomy and partial splenectomy.

A large ventral hernia resulting from the primary treatment of an omphalocele according to the Gross technique was repaired at age 16. The girl presented with extra-abdominal development of the liver and the spleen along with hypotrophy of the abdominal and thoracic cavities. The operation included enlargement sternoplasty, liver and spleen-size reduction and prosthetic abdominal closure. The cosmetic and functional results are good and stable on 7-year follow-up. Surgical issues and blood transfusion policy are discussed. A multi-disciplinary pediatric surgical approach is advocated.

[Causes of preoperative mortality in transposition of great vessels. 2 cases]. / Causes de mortalité préopératoire de la transposition des gros vaisseaux. A propos de 2 cas.

The prognosis of transposition of the great arteries improved tremendously with the development of an early medico-surgical strategy including balloon atrioseptostomy, prostaglandin infusion and the arterial switch operation within the first days of life. Nevertheless, some patients still die preoperatively. We report on two newborn infants whose fatal outcome was promoted by an inadequate intercirculatory mixing. Since the diagnosis was not immediately made, the restrictive foramen ovale resulted very quickly in deep metabolic acidosis and balloon atrioseptostomy performed yet in the first hours of life could not prevent death. We emphasize the importance of prenatal echographic detection of this defect, only way to plan a balloon septostomy immediately after delivery in those infants suffering from inadequate atrial mixing.

[Short and medium-term results of the Norwood intervention (first stage)]. / Résultats à court et moyen termes de l'intervention de Norwood (première étape).

We report the short and mid-term results of the Norwood procedure (Stage one) in 20 patients with hypoplastic left heart syndrome or univentricular heart with aortic obstruction. Seven patients were prenatally diagnosed. Preoperatively there was obstruction to pulmonary venous return in 6 cases, a mild to moderate tricuspid regurgitation in 6 cases, and 11 patients were supported by mechanical ventilation with multiorgan failure in 5 cases. The surgery was performed under cardiopulmonary bypass at a mean age and weight of 12.9 days and 3 kg, respectively. Nine patients (45%) died within 30 days postoperatively, whereas 5 had delayed sternal closure. The mean duration of mechanical ventilation and ICU stay were 5.7 and 11 days, respectively. Two patients were reoperated for bronchial compression and tracheotomy. Systemic venous thrombosis occurred in 5 patients. In multivariate analysis, an older age at surgery was correlated with postoperative hospital death (p = 0.03). Among the 11 patients discharged home after Stage one procedure, 5 patients underwent balloon dilation for recoarctation and one patient died at home. A bidirectional cavopulmonary anastomosis was performed in 8 patients at a mean age of 0.76 year, with one postoperative death. After a mean follow-up of 1 year (+/- 1.97 years), the 9 remaining patients are all in NYHA class I, at a mean age of 2.2 years. Their mean transcutaneous saturation is 81%. The Norwood procedure (Stage one) is associated with high hospital mortality. However, the functional status of the survivors is correct, like in patients with other type of univentricular hearts. Moreover, although the causes of death in our patients are often not clarified, other studies show that the leading causes of deaths in our patients are often not clarified, other studies show that the leading causes of death in those patients are correctable. In conclusion, the option of a Norwood procedure (Stage one) should be proposed in patients with hypoplastic left heart syndrome (or variant).

[Outcome of infants with hypoplastic left heart syndrome diagnosed in utero]. / Devenir des enfants atteints d'une hypoplasie du coeur gauche diagnostiquée in utero.

The authors report the results of prenatal diagnosis of the hypoplastic left heart syndrome since 1998 in the University Hospitals of Marseille and Montpellier. Twenty-four prenatal diagnoses of this condition were made in mothers with a mean age of 29 (18 to 40 years) and after a mean term of 22 (18.5 to 33) weeks of amenorrhea. Seventeen therapeutic abortions were carried out and 7 neonates born after a mean term of 39 (28 to 40) weeks, were admitted to the paediatric intensive care unit. Two patients required ventilatory assistance with one early death. The other patients were stable after surgery. A Norwood (first stage) procedure was carried out in 6 neonates at a mean age of 5 (1 to 6) days. There was only one survivor (17%). Prenatal diagnosis of the hypoplastic left heart syndrome allows cardiac and extracardiac evaluation of foetuses with this condition. Therapeutic abortions may be proposed and was the commonest choice of the parents in this study. On the other hand, despite better management of neonates with this prenatal diagnosis, the poor prognosis of the Norwood first stage procedure is unchanged. A systematic search for a restriction of the foramen ovale on foetal echocardiography could optimise neonatal management of this problem.

[Double lung transplantation. Report of the 1st French case and comments on the 5 subsequent cases]. / Les transplantations bi-pulmonaires. Rapport de la première observation française et commentaires des cinq suivantes.

Using a modified version of the technique described by the Toronto group, the Marseille group has performed 6 double lung transplantation procedures. In 6 cases the underlying disease was cystic fibrosis. Four patients are currently alive. The technique was modified in two ways. First anastomosis was made on the two main stem bronchi in order to rule out the risk of ischemic complications. Second a special postoperative care technique in which the patient is regularly turned from one side to the other was applied to avoid lymph stasis during the first postoperative weeks.

[Perioperative fasting in children: current data]. / Le jeûne périopératoire de l'enfant: données actuelles.

Perioperative fasting aims at decreasing the incidence of gastric content inhalation during anesthesia. Current knowledge concerning gastric emptying and the epidemiology of pulmonary aspiration authorizes new perioperative fasting guidelines. If prolonged fasting does not guarantee gastric emptiness at the induction of anesthesia, shortening preoperative fasting by allowing clear fluids two hours before surgery does not modify gastric content and does not increase the risk of gastric content aspiration, while enhancing the patient comfort by reduction of the fasting period. On the other hand, after surgery, the mandatory intake of water significantly increases the incidence of postoperative vomiting. Therefore new guidelines may be applied for children operated in ambulatory surgery settings: 1) clear fluids may be allowed until 2-3 hours before operation, 10 ml.kg-1, or even ad libitum for some authors (by clear fluids one means water, tea, coffee, apple juice, syrup with water); 2) drinking is not absolutely necessary before discharge from day care surgery unit and should be left to the child's own assessment.

[Severe trauma in children]. / Les traumatismes sévères de l'enfant.

Trauma are responsible for approximately 50% of the deaths of the pediatric population between 1-15 years of age. This high mortality rate, associated with frequent sequelae, leading sometimes to severe handicaps, is a major problem of public health in the developed countries. Pediatric trauma have some particularities, due to anatomical and physiological differences, and to specific injury mechanisms. Management of a patient with severe trauma is best performed by trained physicians, working in a multidisciplinary team with a two steps approach: 1) emergency rapid clinical assessment and resuscitation. 2) a secondary complete clinical evaluation associated with medical imaging, mainly based on CT scan. Head injuries are frequent and represent the main prognosis factor, mass lesions being less frequent and cerebral oedema more frequent in children, than in adult; brain swelling appears to be less frequent than initially reported. Management of head trauma has evolved in recent years, and is now largely directed towards the prevention of secondary ischemic brain injury: new monitoring devices are proposed to pursue that goal: transcranial doppler and continuous jugular vein oxygen saturation monitoring. Spinal cord injuries are rare but may be severe: cervical and spinal cord injuries without radiological abnormality (SC/WORA) appear to be more frequent than in adult. Most often, abdominal plain viscera injuries are treated with a conservative non operative approach. Among chest injuries, pulmonary contusion is the most frequent, with a favorable outcome in most cases within 3-4 days. Child abuse must be suspected in any case where there is no clear injury mechanism or when there is a discrepancy between the severity of the injury and the alleged mechanism.

[Recent developments in the treatment of pediatric acute respiratory distress syndrome]. / Les développements récents dans le traitement du syndrome de détresse respiratoire aigu en pédiatrie.

Acute respiratory distress syndrome (ARDS) is a severe condition with a high mortality rate, despite conventional treatment using mechanical ventilation. Better understanding of the pathophysiology and awareness of important iatrogenic lung injury secondary to mechanical ventilation has led to new therapeutic principles. Mechanical ventilation strategy during ARDS is characterized by positive end-expiratory pressure, increase in the inspiratory time, high inspiratory oxygen concentration and, more recently, use of permissive hypercapnia. High frequency ventilation allows optimal lung recruitment under small tidal volume. The effectiveness of extracorporeal oxygenation techniques is demonstrated, but because of their cost and morbidity these therapies are rational only in patients who seem likely to die. Partial liquid ventilation and inhaled nitric oxide have great potential but require further studies. Intratracheal exogenous surfactant might be beneficial but controlled trials are needed to confirm the usefulness of this expensive therapy. Finally, a number of adjuncts to mechanical ventilation are currently available to minimize iatrogenic lung injury and improve the outcome. The role of these new treatments must be defined with randomized and controlled clinical trials using homogenous inclusion criteria.

[Value of surfactant in the treatment of acute respiratory distress syndrome in a child]. / Intérêt du surfactant exogène dans le traitement du syndrome de détresse respiratoire aiguë de l'enfant.

BACKGROUND: Adult respiratory distress syndrome has a high mortality rate, despite treatment including mechanical ventilation with positive end-expiratory pressure, increase of the inspiratory time and high inspiratory oxygen concentration. Exogenous surfactant, a well established treatment in premature newborns with neonatal respiratory distress syndrome has only been occasionally evaluated in adult respiratory distress syndrome. CASE REPORT: A 3 year-old child suffered from adult respiratory distress secondary to respiratory syncytial virus infection. Both mechanical ventilation and inhaled nitric oxide failed to improve the respiratory distress. Two doses of intratracheal surfactant application immediately improved pulmonary functions, transiently after the first instillation on day 13 and definitely after the second one, on day 16. CONCLUSION: Exogenous surfactant appeared to be useful for the survival of our patient but prospective evaluation of this treatment in adult respiratory distress syndrome is needed.

[Severe upper airway obstruction in infectious mononucleosis: a life emergency]. / L'obstruction sévère des voies aériennes supérieures au cours de la mononucléose infectieuse: une urgence vitale.

BACKGROUND: Upper airway obstruction can represent a severe, life-threatening complication of infectious mononucleosis. We report a rare case of airway obstruction in a child with infectious mononucleosis associated with herpes virus infection, and we discuss management strategy that can be proposed in such cases. CASE REPORT: A 9-year-old girl was hospitalised in intensive care unit for obstructive dyspnea during infectious mononucleosis. Despite five days of corticosteroids and tracheal intubation, persistent pharyngo-tonsillar tumefaction led us to perform a surgical adenotonsillectomy. This latter treatment allowed immediate tracheal extubation and a rapid recovery. Histology showed a herpes virus infection associated with infectious mononucleosis. CONCLUSION: Maintaining airway opening in infectious mononucleosis needs sometimes to use instrumental interventions: nasal trumpet, endotracheal intubation, even tracheostomy. Early tonsilloadenoidectomy may relieve airway obstruction and allow a rapid recovery in the most severe cases. Airway obstruction in infectious mononucleosis may be aggravated by concomitant herpes virus infection that should be searched for in this situation, in order to adapt the treatment.

[Epstein-Barr virus. An unusual cause of acute myocarditis in children]. / Le virus d'Epstein-Barr. Une cause inhabituelle de myocardite aiguë sévère chez l'enfant.

UNLABELLED: Epstein-Barr virus does not belong to the principal causative agents of acute myocarditis, whose diagnosis and pathogenesis are often difficult to determine. Treatment is also controversial regarding the use of anti-inflammatory or immunosuppressive therapy. CASE REPORT: We describe a 13-month-old girl, admitted for acute heart failure, in whom cardiac catheterization with endomyocardial biopsy revealed an acute myocarditis. Acute viral titers indicated infectious mononucleosis caused by Epstein-Barr virus, and the virus genome was identified with a polymerase chain reaction in the patient's serum. The patient had clinical improvement after corticosteroid administration. CONCLUSION: The different diagnostic tools and the screening examinations to determine the causative agent of myocarditis are discussed. The frequency of Epstein-Barr virus in pathogenesis is also considered. The favorable outcome with immunosuppressive therapy suggests its administration in cases of acute myocarditis.

[Central alveolar hypoventilation syndrome and cerebral venous thrombosis: fortuitous association?]. / Syndrome d'hypoventilation alvéolaire centrale et thrombose veineuse cérébrale: association fortuite?

UNLABELLED: Central hypoventilation syndrome is defined as the failure of automatic control of breathing. Secondary central hypoventilation syndrome should distinguish from congenital central hypoventilation syndrome by brainstem abnormalities, place of respiratory control. CASE REPORTS: We report two clinical cases characterized by late onset central hypoventilation syndrome (three years--six months, and five years old): in the first case the diagnosis was made after general anesthesia and the second one presented with acute nocturnal comatose state. Neuroradiologic investigations showed bilateral cerebral sinus veinous thrombosis without any brainstem lesions. Moreover these children had severe behavior disorders: psychomotor instability, alterations of social relations, language dysfunction, and neurocognitive deficit. This symptomatology seems independent from central hypoventilation syndrome and cerebral venous thrombosis. CONCLUSION: Late onset central hypoventilation syndrome may be associated with cerebral venous thrombosis. Ischemia of central chemoreceptors or integration of their informations could be one of mechanism.

[Refractory pain in children with cancer: role of peridural analgesia]. / Douleurs réfractaires de l'enfant cancéreux: place de l'analgésie péridurale.

BACKGROUND: Adequate treatment of pain in children with cancer is a critical issue, and is of equal importance as discussions concerning chemotherapy, surgery and radiotherapy. OBJECTIVE: To evaluate the treatment of refractory pain by peridural analgesia. METHODS: Seven children (1-15 years) with solid tumors were treated with long term epidural analgesia for refractory pain. Catheters were inserted in epidural space (L1-L2) and infused with sufentanil, bupivacaine and clonidine. RESULTS: Three out of five children with good response to peridural therapy could be discharged. A 12-month-old infant had a poor response. Treatment was discontinued in a teenager boy because of patient refusal. The side effects were: early catheter displacement in two patients and a bacterial contamination in one. Serious adverse effects related to high doses of opiates were not observed. However, toxicity of bupivacaine was observed in three patients leading to treatment discontinuation in one. CONCLUSION: Long-term epidural analgesia looks promising in selected children with refractory pain.

[Severe potassium dichromate poisoning after accidental nasal introduction]. / Intoxication grave au dichromate de potassium après introduction nasale accidentelle.

BACKGROUND: Nasal foreign body (NFB) is a common situation in pediatrics. Poisoning is a rare complication of NFB insertion. We report a case of acute potassium dichromate poisoning secondary to NFB insertion. CASE REPORT: Six days after insertion of a NFB, progressive occurrence of diarrhea, vomiting, nasal obstruction, acute renal failure, pancreatitis, hepatitis and drowsiness justified hospitalization of a 3-year-old girl in the pediatric intensive care unit. Acute potassium dichromate poisoning was confirmed by high plasma chromium level and by the spectrophotometric analysis of the crystal. Recovery was satisfactory with supportive treatment. An official survey allowed to discover that the crystal was freely sold and that its toxicity was unknown by dealers, while no information was given to the customers. CONCLUSION: Transmucosal absorption of toxics is an unusual severe potential hazard that should be evoked to allow a rapid management. After the discovery and withdrawal of a NFB, occurrence of systemic symptoms, even trivial, must make one suspect a poisoning. In this circumstance, analysis of the foreign body should be done, associated with toxicologic dosages. This case report illustrates that potassium dichromate poisoning is a severe medical condition and that its clinical presentation assume a large widespread of symptoms due to multiple organ involvement.