A French observational study of botulinum toxin use in the management of children with cerebral palsy: BOTULOSCOPE.

BACKGROUND: Dystonia and spasticity are common symptoms in children with Cerebral Palsy (CP), whose management is a challenge to overcome in order to enable the harmonized development of motor function during growth. AIM: To describe botulinum toxin A (BTX-A) use and efficacy as a treatment of focal spasticity in CP children in France. METHODS: This prospective observational study included 282 CP children mostly administered according to French standards with BTX-A in lower limbs. Realistic therapeutic objectives were set with parents and children together before treatment initiation and assessed using the Visual Analogue Scale (VAS). Child management was recorded and the efficacy of injections was assessed during a 12-month follow-up period by physicians (Modified Ashworth Scale, joint range of motion, Physician Rating Scale, Gillette Functional Assessment Questionnaire and Gross Motor Function Measure-66) and by patients/parents (Visual Analogue Scale). RESULTS: BTX-A treatment was administered in different muscle localizations at once and at doses higher than those recommended by the French Health Authorities. Children were treated in parallel by physiotherapy, casts and ortheses. Injections reduced spasticity and improved joint range of motion, gait pattern and movement capacity. Pain was reduced after injections. BTX-A administration was safe: no botulism-like case was reported. The log of injected children who were not included in the study suggested that a large population could benefit from BTX-A management. CONCLUSIONS: We showed here the major input of BTX-A injections in the management of spasticity in CP children. The results are in favor of the use of BTX-A as conservative safe and efficient treatment of spasticity in children, which enables functional improvement as well as pain relief.

[Heat stroke and topiramate]. / Coup de chaleur et topiramate.

Heatstroke, which is a major disorder related to environmental hyperthermia, is a rare event in children. The risk is increased with predisposing medical conditions and specific medications. We report the case of a 10-year-old epileptic patient, who received topiramate. Topiramate causes hypohydrosis and hyperthermia. We suggest that topiramate treatment may be a risk factor for heatstroke.

[Follow-up network for newborns at risk for handicap in a French region]. / Réseau de suivi des nouveau-nés à risque de développer un handicap. L'expérience du réseau de suivi régional << grandir ensemble en Pays de la Loire >>.

BACKGROUND AND OBJECTIVES: - Follow-up networks for newborns with a handicap risk need to be put into place downstream of the perinatal health networks. Such a network was implemented in the Pays de la Loire region in 2003. Our objective is to evaluate the feasibility and the capacity to detect infants with an incapacitating condition at nine months corrected age and the patents'satisfaction with such a network. MATERIAL AND METHODS: - A common tool based on the Amiel Tison assessment was set up. Infants included since 1(st) March 2003 and who were two years old corrected age on 1(st) September 2006 were taken into consideration. A satisfaction survey was conducted with the parents of infants showing normal development at two years old corrected age or with an abnormal neuromotor examination. RESULTS: - Amongst the 1339 infants included, 1185 (88.4 %) were seen at the age of two years: 7.3 % showed pathological development, 4.5 % infants showed suspect neuromotor examination. Cares were proposed for 11% of the infants followed, permitting cares from nine months old for 65 % of the infants considered as having a pathological development at the age of two years. DISCUSSION/CONCLUSION: - This follow-up network has fulfilled its initial goal: 2.2 % of newborn babies in the region were included, a low rate of lost for follow-up was observed. Moreover, this network has a relative good capacity for detection and early initiation of care. The application of the parents is important and their level of satisfaction appeared to be high. This experiment shows that regional follow-up networks can become reality, but their efficiency still needs to be improved.

[Dietary water intoxication: a preventable pathology]. / Erreur diététique et intoxication par l'eau: une pathologie évitable.

CASE REPORT: The authors report the case of an infant presenting with status epilepticus, apneas and hypothermia, related to hyponatremia. The cause was water intoxication, secondary to feeding a dilute formula and free water. The parents were notoriously challenged by the care of their children. Such disorder could have been prevented. CONCLUSION: Dietary history may be key to the diagnosis of hyponatremia in children. Water intoxication of nutritional origin can be prevented.

Prophylactic ibuprofen versus placebo in very premature infants: a randomised, double-blind, placebo-controlled trial.

BACKGROUND: Patent ductus arteriosus is a common complication of prematurity that frequently requires surgical or medical treatment. The benefit of prophylactic treatment by indometacin, a cyclo-oxygenase inhibitor, remains uncertain compared with curative treatment. This benefit could be improved with ibuprofen, another cyclo-oxygenase inhibitor with fewer adverse effects than indometacin on renal, mesenteric, and cerebral perfusion. We aimed to compare prophylactic and curative ibuprofen in the treatment of this abnormality in very premature infants. METHODS: We did a randomised controlled trial in infants younger than 28 weeks of gestation, who were randomly assigned to receive either three doses of ibuprofen or placebo within 6 h of birth. After day 3, symptomatic patent ductus arteriosus was treated first by open curative ibuprofen, then back-up indometacin, surgery, or both. The primary endpoint was need for surgical ligation. Analysis was per protocol. FINDINGS: The study was stopped prematurely after 135 enrollments because of three cases of severe pulmonary hypertension in the prophylactic group. 65 infants received prophylactic ibuprofen, and 66 received placebo. Prophylaxis reduced the need for surgical ligation from six (9%) to zero (p=0.03), and decreased the rate of severe intraventricular haemorrhage from 15 (23%) to seven (11%) (p=0.10). However, survival was not improved (47 [71%] placebo vs 47 [72%] treatment, p=1.00), because of high frequency of adverse respiratory, renal, and digestive events. INTERPRETATION: In premature infants, prophylactic ibuprofen reduces the need for surgical ligation of patent ductus arteriosus, but does not reduce mortality or morbidity. Therefore, it should not be preferred to early curative ibuprofen.

Caecal volvulus in the Marden-Walker syndrome: an uncommon association of two rare diseases in a 4-year old child. Case report.

The authors report a case of caecal volvulus associated with Marden-Walker syndrome in a 4-year old girl. They point out the diagnostic difficulties of this devastating disease specially in debilitated children.

[Botulinum toxin in the management of spastic hip adductors in non-ambulatory cerebral palsy children]. / Toxine botulinique dans la spasticité des adducteurs de hanche chez les enfants IMC et IMOC non marchants.

PURPOSE OF THE STUDY: Spasticity of the hip adductors is a challenging problem for children with severe motor impairment due to cerebral palsy. It inhibits motor development and is also a risk factor for hip dislocation. Botulinum toxin has been found to be an effective means of treating spastic pes equinus in walking cerebral palsy patients and could have other indications. We conducted a prospective study to determine the functional and orthopedic contribution of botulinum toxin in the treatment of spastic hip adductors in non-ambulatory cerebral palsy children. MATERIAL AND METHODS: The study included 11 quadriplegic children with cerebral palsy (mean age 5 years 9 months). Seven of the children had unilateral migration of the hip at study onset (> 40% radiographically). The children were given a single injection of botulinum toxin (Dysport: 20 units/kg/hip) in the adductor muscles (21 treated hips). The children were seen again at months 1, 3, 6 and 12 after treatment (with the exception of one patient not seen after the 6(th) month at the request of the parents). Spasticity was measured with the modified Ashworth scale. The motor level was determined with 8 position and motor items and with the GMFCS classification. Hip x-rays were obtained at study onset and once or twice during the follow-up. RESULTS: There were no adverse effects of the treatment. Spasticity decreased by one point or more on the Ashworth scale in 20 hips at month 1 and remained low at month 3 in 14, and at month 6 in 12 of the 21 hips treated. The effect of the anti-spasticity treatment faded out from the 6(th) to the 12(th) month. Three children who experienced pain in the lower limbs were definitively relieved after treatment. Nine children achieved functional improvement (progress in at least one of the motor items). Three children were able to walk with a walker and two of them improved from level IV to level III on the GMFCS. The best functional responses appeared to occur in the younger children and in those who had good results at months 3 and 6. Among the 7 children whose hip was displaced by more than 40%, 5 had an unfavorable radiological progression and underwent surgery. DISCUSSION: This study demonstrates that the botulinum toxin can be effective against spasticity of the hip adductors and that its effect is still significant 6 months after the injection in more than half the hips treated. It has an analgesic effect. This treatment has a functional impact even in children with severe motor impairment. The benefit has been modest but three children were able to progress to walking with a walker. The best functional results were observed in the younger children and in those whose spasticity had declined at month 3 and 6. It could thus be favored either by innate potential for motor development or by the treatment itself. The botulinum toxin did not improve the orthopedic prognosis of the children: 5 of the 7 with a risk of luxation worsened. Nevertheless, our study suggests that the botulinum toxin is a well-tolerated anti-spasticity treatment that is effective for the hip adductors providing an important contribution to the management of non-ambulatory cerebral palsy children.

Schizencephaly: clinical and imaging features in 30 infantile cases.

Schizencephaly is an uncommon structural disorder of cerebral cortical development, characterized by congenital clefts spanning the cerebral hemispheres from the pial surface to the lateral ventricles and lined by cortical gray matter. Either an antenatal environmental incident or a genetic origin could be responsible for this lesion which occurs between the third and fourth month of gestation. We report the clinical and cranial imaging features of 30 children, of whom 15 had unilateral and 15 had bilateral lesions. Their ages at the time of the first presentation ranged from 1 month to 10 years. They were thoroughly studied from clinical, epileptical, imaging and electroencephalographic (EEG) viewpoints. Five patients were investigated by cranial computed tomography (CT), eight by cranial magnetic resonance (MR) imaging, and 17 by both methods. The clinical features consisted of mild hemiparesis in 17 cases (57%), 12/17 were related to a unilateral phenotype (80% of all unilateral forms) and 5/17 to a bilateral phenotype. A tetraparesis was present in nine cases, all of which were due to a bilateral cleft. Bilateral forms were significantly associated with tetraparesis, whereas unilateral forms were associated with hemiparesis. Mental retardation was observed in 17 cases (57%), and was observed significantly more often in bilateral clefts (80%). When both hemispheres are involved, an absence of reorganization of the brain function between the two hemispheres leads to severe mental deficits, in addition to the cerebral anomaly itself. Eleven patients had seizures (seven from unilateral and three from bilateral forms). The degree of malformation was not related to the severity of epilepsy. Migration disorders, such as dysplasia or heterotopia, were observed in 30% of cases and are also important etiopathogenetic factors. The septum pellucidum was absent in 13 cases (43%), with septo-optical dysplasia in two cases. Corpus callosum dysgenesis was noted in 30% of cases. Four cases of mega cisterna magna were noted. Although familial cases and environmental factors have been previously reported, schizencephaly appears to be, in the majority of cases, sporadic.

Internal drainage into an Onlay-Roux-en-Y jejunal loop in isolated pancreatic injury with ductal transection: short-term and long-term follow-up in two pediatric cases.

Two pediatric cases of isolated pancreatic injury treated by internal drainage into an Onlay-Roux-en-Y jejunal loop are reported. The discussion concerns the difficulties of early diagnosis, the importance of endoscopic retrograde cholangiopancreatography (ERCP) in surgery and the reasons for the choice of internal drainage by Onlay-Roux-en-Y jejunal loop in our two cases.

Unilocular hydatid cyst of the kidney in a child: a diagnostic challenge.

A case of an unilocular renal hydatid cyst in a 10-year-old French girl without any notion of travelling in a foreign country is reported. In children affected by renal echinococcosis, clinical symptoms and physical examination are nonspecific. Among the diagnostic procedures, the role of echography in the assessment of the diagnosis is pointed out.

[Lead blood levels in children under 6 years of age in the Mans region]. / Etude de l'imprégnation saturnine d'enfants de moins de 6 ans de la région du Mans.

OBJECTIVES: High lead levels in children can have a deleterious effect on intellectual development. We assessed blood lead levels in children in the Le Mans region. METHODS: Children aged between 6 months and 6 years were included in the study. Inclusion criteria were health status requiring a blood sample and amount of blood available after ordered tests sufficient for lead blood analysis. The study group included 365 children. RESULTS: Mean blood level in the 365 children was 37.2 +/- 20.6 micrograms/l. Six of the children had blood levels greater than 100 mu/l. None of the children had a level over 200 micrograms/l. Location of the home or date of construction of the home were not significantly correlated to blood lead levels, however blood lead levels were higher in children with neurological or behavioral disorders. This observation was made in a limited number of children. CONCLUSION: The risk of excessively high blood lead levels in children under 6 years of age is low in the Le Mans region. There is however a risk when old houses are renovated or in children with neurological or behavior disorders.

Spondylodiscitis and mediastinitis after esophageal perforation owing to a swallowed radiolucent foreign body.

A 6-year-old boy with aphagia presented with a radiolucent foreign body, esophageal perforation, mediastinitis, and a C6-C7 spondylodiscitis. A rigid plastic gear wheel was removed via thoracotomy, and the mediastinal abscess was drained through the esophagomediastinal fistula. Treatment included antibiotics as well as nonsurgical orthopedic management of the spondylodiscitis. The recovery period was uneventful, and the patient has remained asymptomatic for 2 years. Physicians must be aware of radiolucent foreign bodies. Computed tomography is very helpful in establishing the diagnosis of radiolucent foreign body, mediastinal abscess, and spondylodiscitis.

[Vascular tracheal compression presenting as bronchiolitis in infants]. / Compression trachéale vasculaire se présentant comme une bronchiolite chez le nourrisson.

BACKGROUND: Anomalies of the aortic arch and pulmonary arteries may produce compression of the trachea with chronic stridor or wheezing aggravated by crying, feeding and flexion of the neck. CASE REPORTS: Three infants, 3, 5 and 2 months-old, respectively, were admitted suffering from acute bronchiolitis. The first patient had intermittent wheezing for one month; lateral chest X-rays showed an opaque area inserted between the trachea and oesophagus and computed tomography showed pulmonary artery sling with tracheal stenosis which was successfully operated. The second patient displayed pulmonary atelectasia which required bronchoscopy; this technique showed tracheal stenosis which was due to pulmonary artery sling. The third patient had a history of congenital stridor: he required endotracheal intubation; a bronchoscopy performed because persistent pulmonary atelectasia showed tracheal stenosis which was secondary to a double aortic arch. The condition was improved after surgical correction. CONCLUSIONS: A history of stridor and/or wheezing in patients admitted for bronchiolitis must lead to consider the possibility of vascular compression of the trachea and to perform specific investigations.

Inhaled nitric oxide for a severe respiratory syncytial virus infection in an infant with bronchopulmonary dysplasia.

OBJECTIVE: To report the first case of ARDS in children treated with nitric oxide (NO) inhalation. METHODS: A 13-months infant presented with BPD and severe hypoxemia related to RSV infection and ARDS. Inhaled NO was delivered in the ventilatory circuit of a continuous flow ventilator (Babylog 8000, Dräger) in a concentration of 20-80 ppm for 7 days. NO and NO2 were continuously monitored (Polyton Draeger). Respiratory mechanics were evaluated by using the method of passive inflation by the ventilator. RESULTS: NO inhalation improved oxygenation (tcSaO2) and reduced respiratory system resistance without affecting arterial pressure. NO2 level remained below 5 ppm, and methaemoglobin level below 1%. The child survived without neurologic sequela. CONCLUSIONS: Two mechanisms to explain oxygenation improvement can be suggested: selective improvement in perfusion of ventilated regions and bronchodilation.

[Treatment with intermittent high dosage corticotherapy in chronic interstitial pneumonia in an infant. A case report]. / Traitement par corticothérapie intermittente à forte dose de la pneumopathie interstitielle chronique du nourrisson. A propos d'une observation.

Chronic interstitial pneumonia (PIC) are rare in infants. We report a case of an infant who presented with a chronic interstitial pneumonia following an infection with a respiratory syncytial virus. The diagnosis was confirmed histologically (open lung biopsy). The failure of oral steroid therapy (Prednisolone 2 mg/kg/day) led to the use of boluses of methylprednisone (30 mg/kg/day, daily for three days in three series one month apart). The treatment led to a progressive improvement and thirty months after the last bolus the respiratory examination was normal as was the radiology. The efficacy of bolus steroid therapy observed by other authors should be confirmed in multicentre trials using surgical biopsy to precise the pulmonary lesions.

Human beta-mannosidase deficiency associated with peripheral neuropathy.

Human beta-mannosidosis is an inherited lysosomal storage disorder described in only seven families. We present a further case in a black African 14-year-old boy with severely deficient beta-mannosidase activity, bilateral thenar and hypothenar amyotrophy, electrophysiologically demonstrable demyelinating peripheral neuropathy, and cytoplasmic vacuolation of skin fibroblasts and lymphoid cells. The clinical and biochemical features of our patient are compared to those of previously reported patients.