Pediatric long-term noninvasive respiratory support in children with central nervous system disorders.

RATIONALE: The use of long-term noninvasive respiratory support is increasing in children along with an extension of indications, in particular in children with central nervous system (CNS) disorders. OBJECTIVE: The aim of this study was to describe the characteristics of children with CNS disorders treated with long-term noninvasive respiratory support in France. METHODS: Data were collected from 27 French pediatric university centers through an anonymous questionnaire filled for every child treated with noninvasive ventilatory support ≥3 months on 1st June 2019. MAIN RESULTS: The data of 182 patients (55% boys, median age: 10.2 [5.4;14.8] years old [range: 0.3-25]) were collected: 35 (19%) patients had nontumoral spinal cord injury, 22 (12%) CNS tumors, 63 (35%) multiple disabilities, 26 (14%) central alveolar hypoventilation and 36 (20%) other CNS disorders. Seventy five percent of the patients were treated with noninvasive ventilation (NIV) and 25% with continuous positive airway pressure (CPAP). The main investigations performed before CPAP/NIV initiation were nocturnal gas exchange recordings, alone or coupled with poly(somno)graphy (in 29% and 34% of the patients, respectively). CPAP/NIV was started in an acute setting in 10% of the patients. Median adherence was 8 [6;10] hours/night, with 12% of patients using treatment <4 h/day. Nasal mask was the most common interface (70%). Airway clearance techniques were used by 31% of patients. CONCLUSION: CPAP/NIV may be a therapeutic option in children with CNS disorders. Future studies should assess treatment efficacy and patient reported outcome measures.

Long term noninvasive ventilation and continuous positive airway pressure in children with neuromuscular diseases in France.

The aim of the study was to describe the characteristics of children with neuromuscular diseases treated with long term noninvasive ventilation or continuous positive airway pressure in France. On June 1st 2019, 387 patients (63% boys, mean age 11.2 ± 5.5 years) were treated with long term noninvasive ventilation/continuous positive airway pressure. Thirty three percent of patients had spinal muscular atrophy, 30% congenital myopathy/dystrophy, 20% Duchenne muscular dystrophy, 7% Steinert myotonic dystrophy, and 9% other neuromuscular diseases. Ninety-four percent of patients were treated with long term noninvasive ventilation and 6% with continuous positive airway pressure. Treatment was initiated electively for 85% of patients, mainly on an abnormal overnight gas exchange recording (38% of patients). Noninvasive ventilation/continuous positive airway pressure was initiated during a respiratory exacerbation in 15% of patients. Mean duration of noninvasive ventilation/continuous positive airway pressure was 3.3 ± 3.1 years. Mean objective long term noninvasive ventilation/continuous positive airway pressure use was 8.0 ± 3.1 h/24. Spinal muscular atrophy, congenital myopathy/dystrophy, and Duchenne muscular dystrophy represented 83% of children with neuromuscular diseases treated with long term noninvasive ventilation in France. Screening for nocturnal hypoventilation was satisfactory as noninvasive ventilation /continuous positive airway pressure was predominantly initiated electively.

Paediatric long term continuous positive airway pressure and noninvasive ventilation in France: A cross-sectional study.

OBJECTIVE: To describe the characteristics of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in France. DESIGN: Cross-sectional national survey. SETTING: Paediatric CPAP/NIV teams of 28 tertiary university hospitals in France. PATIENTS: Children aged <20 years treated with CPAP/NIV since at least 3 months on June 1st, 2019. INTERVENTION: An anonymous questionnaire was filled in for every patient. RESULTS: The data of 1447 patients (60% boys), mean age 9.8 ± 5.8 years were analysed. The most frequent underlying disorders were: upper airway obstruction (46%), neuromuscular disease (28%), disorder of the central nervous system (13%), cardiorespiratory disorder (7%), and congenital bone disease (4%). Forty-five percent of the patients were treated with CPAP and 55% with NIV. Treatment was initiated electively for 92% of children, while 8% started during an acute illness. A poly(somno)graphy (P(S)G) was performed prior to treatment initiation in 26%, 36% had a P(S)G with transcutaneous carbon dioxide monitoring (PtcCO2), while 23% had only a pulse oximetry (SpO2) with PtcCO2 recording. The decision of CPAP/NIV initiation during an elective setting was based on the apnea-hypopnea index (AHI) in 41% of patients, SpO2 and PtcCO2 in 25% of patients, and AHI with PtcCO2 in 25% of patients. Objective adherence was excellent with a mean use of 7.6 ± 3.2 h/night. Duration of CPAP/NIV was 2.7 ± 2.9 years at the time of the survey. CONCLUSION: This survey shows the large number of children treated with long term CPAP/NIV in France with numerous children having disorders other than neuromuscular diseases.

Kinematic motion abnormalities and bimanual performance in children with unilateral cerebral palsy.

AIM: To evaluate the relationship between the movement abnormalities of the impaired upper limb in children with unilateral cerebral palsy (CP) and bimanual performance. METHOD: Twenty-three children with unilateral CP (mean age 11y 10mo [SD 2y 8mo]) underwent evaluation of bimanual performance (Assisting Hand Assessment [AHA]) and a three-dimensional movement analysis to measure deviations in the movement of their affected upper limb, and compared with 23 typically developing children (TDC) (mean age 11y 11mo [SD 2y 5mo]). Kinematic indices, such as the Global Arm Profile Score (APS), which summarizes the global movement deviation of the upper limb from the norm, and the Global Arm Variable Score (AVS), which represent movement deviations for a given joint, were calculated and correlated to AHA. RESULTS: Values of kinematic indices were significantly higher in children with unilateral CP than in TDC. A strong correlation between Global-APS and AHA score (r=-0.75) was found. Other significant correlations were found with Global-AVS, especially in distal joints. INTERPRETATION: Children with unilateral CP had more movement deviations than TDC. The global movement deviation of the impaired upper limb was strongly correlated with bimanual performance. The influence of distal abnormalities confirms the importance of considering these limitations in therapeutics. WHAT THIS PAPER ADDS: Children with unilateral cerebral palsy had more movement deviations than typically developing children in unimanual tasks. A strong relationship was found between movement deviations of the impaired upper limb and bimanual performance.

Intracranial epidural hematoma in newborn infants: clinical study of 15 cases.

OBJECTIVE: Epidural hematoma (EDH) in newborn infants is rare. We have described the history of 15 newborns with EDH to provide a better understanding of this pathology. METHODS: This is a descriptive case series study using a retrospective review of the medical records of newborns who were admitted to the Pediatric Intensive Care Unit and Neurosurgery Department with the diagnosis of birth EDH over a 24-year period (1979-2002). RESULTS: There was no sex predominance, and most of the mothers were young, nulliparous women. The time latency from birth to the first signs varied from 0 to 24 hours. Clinical presentation was nonspecific: seizures and hypotonia were the main symptoms. The parietal area was the most frequent location. Surgical drainage was required in nine patients, and no deaths were reported. CONCLUSION: This report highlights the clinicoradiological characteristics of newborn EDH, which occurs more frequently in newborns that experienced difficult delivery from a nulliparous mother. Surgery is not a rule; some patients can be managed with conservative treatment. The outcome is generally good.