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Previous reports have demonstrated that tumor necrosis factor alpha (TNF-alpha) plays an important role in the pathogenesis of fulminant hepatic necrosis. The purpose of this experimental study was to measure TNF-alpha blood activity in paracetamol-induced liver necrosis and in coronavirus (MHV3)-induced fulminant hepatitis in mice. No elevation of TNF-alpha activity was found in hepatic failure complicating paracetamol poisoning. In contrast, TNF-alpha activity significantly increased in response to MHV3, reaching 16.3 +/- 5.5 U/ml from 24 h post infection (P less than 0.01). This augmentation was observed even though the virus was not detectable in the liver. Serum alanine aminotransferase levels were low and no histological lesion was observed. In conclusion, our study further supports the implication of TNF-alpha in virus-induced hepatitis failure and confirms that paracetamol poisoning does not cause increased TNF-alpha activity in the circulation.

A paediatric intensive care unit is a complex environment with many hazards for potential medical errors. Due to the high acuity of illness and the complexity of medical care with multiple disciplines and individuals involved, medical errors occur due to breakdowns in communication and teamwork. Medication errors are the most frequent errors in paediatrics. This chapter proposes some strategies to prevent medical errors in paediatric intensive care units.

ResumeObjectif L'objectif de ce travail non controle etait d'evaluer la place de la ventilation non invasive (VNI) en mode « nasal continuous positive airway pressure » (NCPAP) dans la prise en charge des formes severes de bronchiolites saisonnieres. Population et methode Sur 2 periodes epidemiques successives (2003–2004, 2004–2005), 145 nourrissons admis en reanimation pediatrique ont ete inclus dont 121 ont necessite en premiere intention une assistance ventilatoire en mode VNI (n = 53) ou par voie endotracheale (VI) (n = 68). Les donnees ont ete recueillies de facon prospective. Resultats Les caracteristiques generales des patients etaient similaires entre les 2 periodes a l'exception de l'âge moyen au moment de l'admission, plus faible dans le groupe VNI (p = 0,003). Le pourcentage d'echec de la VNI, defini par la necessite d'une intubation tracheale, a ete stable sur les 2 periodes (25 %) alors que le pourcentage des enfants ventiles en mode non invasif a double. Les facteurs predictifs identifies d'echec etaient un score de PRISM a h24 plus eleve (9,2 ± 4,1 vs 5,1 ± 3,2, p = 0,02) et une reduction plus faible de la PaCO2 a H2 de NCPAP (p = 0,003). Quelle que soit l'evolution en VNI, une reduction significative de la frequence respiratoire (60 ± 16 vs 47,5 ± 13,7 cycles/minute, p

Summary Objectives The authors describe 28 pediatric cases of foreign body inhalation requiring treatment in intensive care units between 1987 and 1999. The purpose of this study was: (1) to analyze the circumstances, diagnostic difficulties and initial treatment of serious foreign bodies and (2) to compare our series with other literature descriptions and define principles for optimal prevention and initial treatment. Methods Twenty-one children presented a penetration syndrome, which was responsible in 13 cases for asphyxia with cardiorespiratory arrest. All these children died, regardless of the initial treatment. Seven children were hospitalized for apparent asthmatic symptoms that did not respond to traditional treatment. Results The inefficiency of external extraction methods like the Heimlich maneuver and the mean delay between clinical signs and initial treatment lead us to propose a new strategy for the emergency treatment of foreign bodies with asphyxia. Conclusions We recommend that emergency teams promote the use of a laryngoscope and Magill forceps. Flexible endoscopy is still recommended as the appropriate diagnostic tool to eliminate doubt in the case of a first severe asthma attack.

Resume La bronchiolite virale du nourrisson demeure plus que jamais un probleme de sante publique. Le devenir respiratoire des nourrissons qui necessitent un support ventilatoire est peu etudie. Methodes. – Etude retrospective, sur une periode de quatre ans (1994–1998), de tous les dossiers d'enfants ventiles dans l'unite de reanimation pediatrique de l'hopital Bicetre pour bronchiolite. Les renseignements sur le devenir respiratoire ont ete obtenus par contact avec les familles et le medecin traitant. Resultats. – Cent trente-cinq nourrissons ont ete inclus sur la periode d'etude. L'âge median au moment de l'admission etait de 45 jours (extremes 8–396), 33,3 % etant nes avant 37 SA (10 % avant 32nSA). La mortalite etait de 2,9 % (4/135), tous les deces concernant d'anciens prematures ayant ete ventiles en periode neonatale. En analyse univariee, les facteurs associes a la mortalite etaient la prematurite (p = 0,056) et le syndrome de detresse respiratoire aigue (p = 0,017). A deux ans, l'incidence d'asthme du nourrisson etait de 40,4 %. Aucun facteur etudie n'etait associe a l'evolution vers un asthme du nourrisson. Conclusion. – Les bronchiolites admises en unite de reanimation pediatrique concernent essentiellement le petit nourrisson. Les anciens prematures representent une population a risque. L'incidence d'asthme du nourrisson au decours ne semble pas etre augmentee chez les enfants ventiles. Le benefice des techniques de ventilation non invasive meriterait d'etre evalue.

Resume Le syndrome de Reye idiopathique associe classiquement et de facon inexpliquee une encephalopathie non inflammatoire et une steatose hepatique microvesiculaire. Un certain nombre d’etudes cliniques ou epidemiologiques, principalement en langue anglaise, tendent a nier la realite meme de ce syndrome car de nombreuses maladies metaboliques, dont certaines ne sont pas encore identifiees, peuvent comporter une telle association. L’objectif de ce travail etait d’apprecier actuellement la realite des formes graves de syndrome de Reye, diagnostic positif retenu au terme d’un bilan metabolique exhaustif. Methodes. – Etude retrospective, sur une periode de 11 ans (1991–2001) des dossiers d’enfants hospitalises dans une unite de reanimation medicochirurgicale pour un tableau clinique repondant aux criteres de syndrome de Reye probable (Center for Diseases and Control). Tous nos patients ont beneficie, pendant et au decours de l’episode neurologique aigu, d’une investigation metabolique extensive. Resultats. – Quatorze dossiers (l’âge median a l’admission etait de 52 mois) ont ete retenus. Tous les enfants presentaient initialement un tableau febrile justifiant un traitement par acide acetylsalicylique et paracetamol chez respectivement 12 et sept d’entre eux. A l’entree, le score de Glasgow median etait de 7. L’ammoniemie etait en moyenne de 320 μmol/L et le pic d’alanine-aminotransferase (ASAT) de 1 475 ± 1 387 UI/L. Dix enfants ont ete ventiles et six places en hemofiltration veino-veineuse continue. Trois enfants sont decedes precocement en situation d’hypertension intracrânienne et 11 ont gueri sans sequelles ni recidive avec un recul moyen de cinq ans. Les explorations metaboliques standardisees, effectuees dans deux centres de reference, se sont revelees negatives a l’exception d’un enfant chez lequel le diagnostic d’intolerance au fructose a ete pose. Conclusion. – Les formes graves de syndrome de Reye sans etiologie metabolique identifiee n’ont pas disparu dans notre pays, contrairement a une opinion repandue. Elles restent grevees d’une importante mortalite et relevent d’une prise en charge agressive et precoce, plus particulierement centree sur la lutte contre l’œdeme cerebral. Si le lien de causalite entre consommation de salicyles et survenue d’un syndrome de Reye n’a pas ete etabli en France, la prudence impose neanmoins de respecter les precautions d’emploi figurant dans l’AMM de l’aspirine en pediatrie.

Resume Observation – Nous rapportons le cas d’un enfant de deux mois hospitalise en urgence en etat de choc cardiogenique associe a une hypertension arterielle. L’echographie abdominale mettait en evidence une masse developpee dans la loge surrenalienne gauche. L’examen tomodensitometrique la mesurait a 7 × 6 × 8 cm. Cette masse refoulait le rein gauche et l’aorte. Un examen histologique a fait porter le diagnostic de neuroblastome, sans amplification de l’oncogene N-myc. La prise en charge en reanimation a permis une stabilisation hemodynamique. Une chimiotherapie antitumorale alors realisee a entraine une diminution de la tumeur, et l’a rendue operable. L’enfant restait en remission de sa maladie trois ans apres le diagnostic. Conclusion. – Les catecholamines secretees par la tumeur pourraient provoquer une augmentation de la post-charge du ventricule gauche avec une augmentation du travail myocardique. Ces mecanismes pourraient etre responsables du choc cardiogenique. Ce mode de decouverte d’un neuroblastome est rare.

The natural history of the rare association "multicystic encephalopathy-arthrogryposis" was traced in a fetus carefully followed after artificial insemination. The fetus exhibited normal viability and brain morphology up to the 32nd week. At 36 weeks, active movements diminished and at 37 weeks, hydramnios and signs of fetal distress led to cesarean section. The infant presented with severe arthrogryposis of the limbs and spine, but not with the other elements of a long-lasting akinesia. US showed multicystic encephalopathy. Both the clinical and the neuropathological findings established that multicystic encephalopathy was neither the cause nor the sequential consequence of the fetal akinesia, but the result of a recent diffuse, acute malacic process that also involved the anterior horn cells. Acute fetal distress, responsible for major ischemic damage to CNS but compatible with fetal survival, remains an obscure condition which allows for the development of severe arthrogryposis in a few weeks.

Resume L'electroencephalogramme (EEG) d'urgence aide a apprecier l'etat de la vigilance aussi bien chez l'enfant que chez l'adulte. Afin de determiner la valeur diagnostique et pronostique de l'EEG d'urgence en cas de confusion mentale, de troubles du comportement ou de troubles de la vigilance chez l'enfant, nous avons fait une etude retrospective chez 397 enfants âges de 2 mois a 16 ans. Les EEG ont ete pratiques moins de 24 heures apres une consultation d'urgence pour confusion ou trouble aigu du comportement (n = 106) ou apres admission pour coma en unite de soins intensifs (n = 291). L'EEG a apporte des indications diagnostiques principalement en cas de pathologie convulsivante ou d'encephalopathie hepatique. Chez les enfants comateux, nous avons etabli une classification en 4 stades EEG de severite croissante. Cette classification a ete comparee aux echelles EEG deja publiees dans la litterature, elle est apparue tres proche de celle de Pampiglione et Harden [18] etablie dans une population de 150 enfants apres arret cardiaque. La valeur pronostique pejorative de l'EEG apparait surtout en cas de trace postanoxique dit ≪burst-suppression≫ ou dans les traces isoelectriques de mort cerebrale. Nous concluons que, en dehors de ces situations, la valeur diagnostique et pronostique d'une classification de l'EEG d'urgence en pediatrie est modulee par une surveillance clinique et EEG repetees.

We report two patients with severe haemophilia A, factor VIII < 2 u dL-1, diagnosed at birth, through isolated bleeding in the adrenal gland (case 1) and through a haematoma in the liver (case 2). In these two clinical cases, the vital emergency, with haemorrhagic shock, required early diagnosis of the hereditary coagulation defect. Generally at birth, any unexplained bleeding should prompt screening for haemophilia. In the newborn period, the PTT is inadequate for many reasons and the laboratory evaluation must include factor VIII and IX levels.

Background. — Agranulocytosis associated with the use of silver sulfadiazine has been described in burn patients. This agranulocytosis may be due to either an allergic reaction or a bone marrow toxicity by silver sulfadiazine. Case report. — A 1-month old baby was hospitalized in pediatric intensive care unit after a stage one repair of a tracheosophegeal fistula. Gastroesophageal reflux and tracheomalacia appeared in the post operative period. When she was 2-months old, she developed a perineal erythema. A topical therapy was begun, with daily applications of silver sulfadiazine and ketoconazole. Five days later, agranulocytosis (granulocytes = 0.21 × 109 L−1) occurred. Silver sulfadiazine therapy was immediately stopped. Granulocyte count returned to normal over a few days. No other etiology than silver sulfadiazine could be found to explain agranulocytosis. The small surface of administration, the chronology of the events and the rapid correction of the disorders after silver sulfadiazine interruption argued for an immuno-allergic reaction to this drug. Medical problems were easily controlled and the patient recovered well. Conclusion. — This event occurred when sulfadiazine was applied in a small cutaneous area in a young baby. This may be an argument for an allergic reaction; alternatively, it could be explained by an increased cutaneous absorption of a cytotoxic drug in younger people.

Among the main causes of acute liver failure (ALF) in children, metabolic diseases (especially in infants), viral and toxin or drug induced hepatitis are the most frequent. The cause remains, however, undetermined in about 30% of the cases. Management must be conducted in a pediatric hepatology unit or intensive care unit in relation with a pediatric transplant team in order: 1) to perform urgent etiological diagnosis; 2) to initiate specific therapy and symptomatic treatment; 3) to evaluate the severity and prognosis of liver disease for selection of children for emergency liver transplantation; 4) to evaluate contraindications to liver transplantation. The overall survival of post-emergency liver transplantation for ALF in children averages 65%.

Le syndrome de detresse respiratoire aigue (SDRA) de l'enfant est un motif d'admission frequent dans les unites de soins intensifs pediatriques. La mortalite de ces 20 dernieres annees demeure elevee en depit des progres de la ventilation mecanique conventionnelle et des traitements etiologiques. Plusieurs etudes chez l'animal ont demontre les effets deleteres d'une ventilation a haut volume, et des travaux cliniques ont clairement montre l'heterogeneite de la ventilation pulmonaire et la surdistension des zones saines (theorie du baby lung) dans le SDRA conduisant a une agression pulmonaire secondaire. La place des supports ventilatoires extracorporels reste neanmoins debattue. D'autres strategies ont ete developpees visant a reduire le traumatisme ventilatoire et a ameliorer l'oxygenation systemique, mais seules des etudes multicentriques et prospectives conforteront le bien-fonde des differentes approches.

Diaphragmatic paralysis, a difficult diagnosis in the pediatric age group, has classically been made by fluoroscopy or B-mode ultrasound. We report our experience with TM-mode exploration. Twenty-seven patients suspected to have diaphragmatic paralysis were examined by means of inspiratory and expiratory chest radiography, fluoroscopy and B-mode ultrasound. The diaphragmatic echo was recorded on TM-tracing during spontaneous breathing using coronal oblique scans. Direction, excursion and the pattern of the transition between inspiration and expiration were analysed. In 7 patients examination was normal and TM mode demonstrated movement of normal direction and excursion with a sharp aspect of the transition zone. Diaphragmatic paralysis was present in 11 patients: unilateral in 9 and bilateral in 2 cases. TM mode demonstrated paradoxical movement, reduced excursion and a smooth transition zone. In 9 patients with diaphragmatic dysfunction TM mode demonstrated movement in the normal direction but with reduced excursion and a smooth transition zone. Compared to other imaging modalities, TM-mode records diaphragmatic movements more objectively. It can identify direction of the movement even if they are fast and of weak amplitude and in the case of bilateral paralysis. TM can differentiate paralysis from dysfunction. Moreover, this low-cost, non-irradiating made of imaging can be performed at the bedside and is available on all basic devices.

Resume Une enquete menee sur 2 annees au sein du Groupe Francophone de Reanimation Pediatrique a permis de recenser 459 cas de bronchiolites graves hospitalisees en reanimation :2/3 de ces enfants ont moins de 3 mois et 50 % des nourrrissons admis ont au moins un des classiques facteurs de risque (antecedents de prematurite, de bronchodysplasie, cardiopathie congenitale, immunodepression). Plus de 60 % de ces malades requierent une ventilation mecanique d'une duree moyenne de 8 jours. Les complications classiques (barotraumatisme, SIADH et convulsions) touchent moins de 5 % des enfants. Le taux de mortalite globale est de 2,2 %.et il n'y a pas de surmortalite dans les groupes a risque. L'utilisation de la ribavirine a nettement augmente d'une annee a l'autre. L'evaluation de son efficacite sera delicate en raison de la difficulte a trouver des criteres d'evaluation.

We report six previously healthy children who several days after a prodromal illness had an acute encephalopathy that ran a biphasic course. It appears to constitute a recognizable syndrome with a good prognosis that can be differentiated from other encephalopathies of obscure origin as previously defined by Lyon et al. The active phase was dominated by coma or confusion and by abnormal movements, including disordered gesticulation and attacks of orofacial dyskinesia or limb dystonia associated with permanent rigidity and culminating in opisthotonic posturing. Repeated seizures were observed in only two patients. Permanent slow waves were recorded on the electroencephalogram in all patients, even during bursts of abnormal movements. Cerebrospinal fluid and results of serologic studies were normal throughout the course of the disease, and attempts at viral isolation and antiviral antibody detection yielded negative results. Brain imaging either showed no abnormalities or suggested a moderate degree of brain edema. The recovery phase, which extended for several weeks, was characterized by a rapid return of motor function and persistent behavioral and cognitive disturbances. Nonverbal reasoning recovered long before verbal expression returned to normal. Four patients eventually recovered fully, whereas two had mild sequelae.

Resume Les insuffisances hepato-cellulaires aigues graves (IHCAG) de l'enfant entrainent 60 a 90% de mortalite, suivant les etudes, sans influence de l'âge. Vingt-deux enfants atteints de cette pathologie ont eu des EEG quotidiens; 15 sont decedes, sept ont survecu. L'etude neurologique electroclinique a mis en evidence une evolution stereotypee des anomalies EEG sans rapport avec l'âge, correlees aux stades cliniques de coma et a des troubles severes de la coagulation; 5/22 enfants ayant eu des manifestations convulsives cliniques et/ou EEG sont morts. Chez un seul enfant decede d'intoxication par le paracetamol, on a observe des ondes lentes triphasiques. Une etude prospective sur une population plus importante permettrait de reconnaitre des facteurs de decision pour une transplantation hepatique.

Among a series of 90 pediatric liver transplantations, 9 cases of hepatic artery thrombosis (HAT) in 8 patients are reported. All cases were diagnosed in the first two weeks and confirmed angiographically and surgically. Clinical presentation was often unreliable with only 2 cases showing the typical pattern of massive hepatic necrosis. Five cases presented with biliary complications and in 2 cases, HAT was discovered fortuitously on duplex sonography. The role of imaging modalities is emphasized. Duplex sonography is the best non-invasive screening method and we recommend a routine daily examination in the first 2 weeks. CT is the most useful method to assess the extension of liver infarction. PTC remains necessary to evaluate biliary complications and to plant the best therapeutic approach in this devastating event. Two patients died, 2 had to be retransplanted (one patient twice) and are doing well, 2 are on a waiting list for re-transplantation and one underwent an emergency surgical arterial desobstruction and is completely asymptomatic.

ORGAN procurement network needs the implication of transplant team outside of the transplant center. A transplant surgery team seems most appropriate for organ procurement because of their training in the field of organ procurement and transplantation. The 5-year kidney graft survival rate decreases to 8% when a nontransplant-surgeon makes the organ procurement. Moreover, the rate of acute tubular necrosis increases to 54% (vs 35%). The outcome of renal transplantation depends on surgical training of the organ procurement team. The purpose of this retrospective work was to compare the results of organ procurements made by our team (inside and outside our center) to those by other organ procurement teams.

Emergency orthotopic liver transplantation (OLT) in adults and children with fulminant liver failure (FLF) is well-established. With this procedure, 1-year survival rates range from 55 to 80% [1, 2]. However, OLT still encounters serious problems especially in the pediatric population, including patient selection, organ availability, and the definition of optimum preoperative management of these patients.

Among a series of 140 liver transplantations in children, 21 biliary complications (BC) (15%) are reported. BC were identified from 2 days to 3 months after LT. Positive US findings were present in 20 cases. Cholangiography was obtained by opacification of a surgical drain in 3 cases, per-operatively in 3 and by PTC in 15. Drainage was placed in 9 and balloon dilatation performed in 2. Causes of BC include hepatic artery (HA) thrombosis in 7, HA stenosis in 1, anastomosis stricture in 7, anastomosis kink in 3, mucocele of cystic duct remnant in 2 and sludge in 1. Treatment was surgical in all, but 2 cases were treated percutaneously. There is a great difference in severity of prognosis between complications secondary to HA thrombosis and isolated BC. Role of US in diagnosis and of PTC and interventional radiology in treatment are emphasized.

Since 1984 we have been involved in the management of 30 children who had cardiac manifestations secondary to cerebrocranial arteriovenous shunts. Aneurysm malformation of the vein of Galen was the most common vascular lesion observed (73% of cases). In 77% of the patients the cardiac symptoms were the main presenting complaint. Medical treatment and/or endovascular therapy were indicated, depending on the age of the patients and the severity of the cardiac manifestations. Following embolization, the cardiac symptoms resolved (73%) or improved (18%) in 1 or 2 sessions. Mortality in the embolized group was 9%, and transient nonneurologic morbidity occurred in one case. Overall mortality, including four patients rejected for embolization, was 20%. These results compare favorably with medical and/or surgical management, alone or combined. The technique, challenges, indications and contraindications of endovascular therapy are discussed. Embolization represents an effective adjunct treatment to control, improve or cure the congestive cardiac manifestations caused by cerebrocranial arteriovenous shunts.

ENAL surgery injuries, which sometimes occur during organ procurement, may contribute to morbidity and renal function impairment as well as increased hospital stay and cost. 1 In France, renal procurements are customarily performed by urologists directly involved in renal transplantation, who also have to deal with transplant complications. Prevention is the best way to manage renal surgery injuries, but this requires identifying the manner in which they may occur. The purpose of the present study was to clarify the causes of renal injuries during organ harvests over the past 15 years at our institution. PATIENTS AND METHODS Between 1985 and 2000 we performed 627 renal procurements (191 female and 436 male donors) including 558 from heart-beating and 69 from non– heart-beating donors. We noted the surgical injuries to the kidneys and the causes cited by physicians for not using organs. Statistical analysis was performed according to initial injury assignments based on our database. The chi-square test was used as appropriate; differences were considered statistically significantly if P .05. RESULTS A total of 1239 kidneys were harvested. Fifteen kidneys were not harvested. There were 106 kidneys not transplanted. The presence of surgical lesions was the reason for transplant refusal in four cases (1 renal decapsulation, 1 arterial injury, and 2 venous injuries, which could not be repaired properly). Twenty kidneys were refused for transplantation because they were macroscopically abnormal, namely a renal tumor or renal rupture, or because they displayed microscopic abnormalities, namely nephroangiosclerosis, glomerulosclerosis, or arteriolar injuries. Macroscopic surgical arterial injuries were observed in 2.3% (29 of 1254) of cases (26 polar artery transections and 3 hilar artery transsections). Venous lesions were observed in 0.5% (7 of 1254) of cases. Surgical injuries were more frequently observed during single-organ procurements than during multiorgan procurements: 8.2% vs 3.7%, respectively (P .05). Similarly, injuries were more frequent during non– heart-beating donor procurement: 9.3% vs 4.6% (P .01), or when the surgeon had less training (30 organ vs 30 procurements): 12% vs 3%, respectively (P .01).

Editor—The diagnosis of spinal cord injury is often delayed and/or missed in neonates.1 Non-invasive imaging, such as ultrasonography and magnetic resonance imaging, is important to confirm the diagnosis. From 1990 to 1997, we observed four spinal cord injuries in neonates. Three of these infants had a cephalic presentation and needed instrumental delivery using forceps. The other infant was a breech presentation and was born vaginally without instrumentation. The infants were depressed at birth, with no spontaneous breathing, and they all needed tracheal intubation. Two infants remained totally unreactive throughout their course, whereas a suck reflex and opening of the eyes were noted 24 hours after birth …

Summary We present an electroclinical study of 4 severe pneumococcal meningitis cases with intracranial hypertension. In addition to classical anticerebral edema therapy thiopental infusion was used. The plasma thiopental level seems to be only an incomplete indicator of cerebral drug effect, but must be measured to avoid accumulation. EEG monitoring appears to be better in evaluating the clinical status and barbituric impregnation. The burst suppression in EEG recording is noted at variable periods (9–48 h) after the start of the treatment and disappears on discontinuation of infusion while the plasma drug levels remain high. Further, in two cases EEG changes preceded clinical deficits. Evolution of clinical and EEG status was good in the 4 children.

Summary During a 4 year period, 79 infants aged from 1 month to 2 years suffered a status epilepticus (SE). The seizures were most often either generalized of tonic-clonic or clonic type, or unilateral clonic; they lasted or were repeated with persistent unconsciousness between the seizures for periods ranging from 30 min to several days. All the cases of SE lasting over 6 h resulted from a recognizable acute brain injury, whereas 11 of 14 cases lasting 1 h or less were cryptogenic. In 2 cases only, the neurological status was worse after the status, apparently as a result of the seizures themselves. The authors discuss the hypothesis of purely febrile HH syndrome and point out the importance of detailed clinical ictal and interictal characteristics for the etiological approach.

The French Club of Pediatric Intensive Care has prospectively studied 90 cases of infectious purpura which were hospitalized in 1981; the purpose of this study was to determine prognostic factors. The statistical study (X2 test) of all these cases is in agreement with data in the literature and shows that the mortality is significantly higher when there is: shock (p less than 0.001), coma (p less than 0.05), ecchymotic or necrotic purpura (p less than 0.01), temperature less than 36 degrees C (p less than 0.05), no clinical meningism (p less than 0.001), white cell count less than 10,000/mm3 (p less than 0.05), thrombocytopenia less than 100,000 (p less than 0.01), fibrinogen less than 1.5 g/l (p less than 0.001), kalemia greater than 5 mEq/l (p less than 0.01), spinal fluid cell count less than 20/mm3 (p less than 0.01). Because shock is one of the main prognostic factors (23 deaths in 55 shocked patients, versus 2 in 35 non-shocked) we have performed another statistical study (with the Benzecri method) to determine a prognostic index for patients in shock. For its determination, five initial parameters are used: age, kalemia, white cell count, clinical meningism, platelet count. The predictive value for survival is 91%. The predictive value for death is 87%. The score was applied on the patients hospitalized in shock in 1982: the predictive value for survival is 75%, the predictive value for death is 61%.

The authors report a series of 36 vein of Galen aneurysmal malformations (VGM) diagnosed in the paediatric (78%) and adult (22%) populations that were referred to them for therapeutic management between 1982 and 1988. The clinical signs leading to the diagnosis were variable: 36% of systemic manifestations, 22% of neurological symptoms, 17% of hydrocephaly and 11% of intracranial haemorrhage. 30 angioarchitectural analyses could be obtained and allowed to classify these VGMs into 5 different types: 44% parenchymatous AVMs, 20% mural AVFs, 30% choroidal arteriovenous fistulas, 3% dural AVFs, 7% vein of Galen varices. This series demonstrates that the paediatric population is most sensitive to shunt effect whatever its type. Systemic manifestations and hydrocephaly are the most common signs encountered in the newborn and infants; whereas neurological signs and symptoms and haemorrhage belong mostly to the adult symptomatology. Because of the poor outcome of VGMs, all authors believe that these malformations have to be treated aggressively. However, we found contra-indications to be represented by pretherapeutic demonstration of cerebral tissue damage, or uncontrollable systemic failure, thus treatment is indicated to compensate for cardiac failure previously responding (even partially) to medical treatment. Secondly, appearance of sub-cortical calcifications, resistance to medication or clinical deterioration will also lead to urgent treatment. The endovascular method represents at present the best treatment with an overall low mortality (13%) and a 0% technical morbidity in children compared to the surgical one of (91% mortality in newborns and 38% in infants). The results achieved by embolization in this series were as follows: 27% satisfactory results with complete or almost complete occlusion of AV Shunt, 53% significant clinical improvement, 7% of patients were unchanged. The authors believe fundamentally that these patients (specially those belonging to the paediatric population) have to be treated in a centre where a paediatric intensive care unit, neurological, neurosurgical and surgical neuro-angiographic departments coexist, in order to assure the best possible management of these children.