Negative extrathoracic pressure ventilation in central hypoventilation syndrome.

Nine patients with central hypoventilation syndrome (CHS) were treated with negative extrathoracic pressure ventilation (VNEP). Treatment with VNEP was started between 20 days and 57 months of age, which was two days to 47 months after diagnosis. The equipment to provide VNEP utilised a new system with a latex neck seal and Perspex chamber allowing easy access to the child. Seven patients are managed with VNEP at home by their parents. They did not have a tracheostomy when VNEP was started at ages of 22, 24, 31, 38, and 75 days, 5 and 57 months. They have continued to be successfully managed with VNEP and without tracheostomy. Short periods of intubation and positive pressure ventilation were required on 10 occasions (median duration 7 days, range 4 to 21 days) in four subjects during respiratory tract infections. Three patients required periods of continuous positive airway pressure (CPAP) via a nasal mask or a nasopharyngeal airway during sleep to overcome upper airway obstruction. In three patients the hypoventilation improved and two of these do not require regular ventilatory support at 1.3 and 3.4 years of age. Six of these seven patients are developing normally. In two patients with long term tracheostomies, VNEP could not be established at an age of 29 and 52 months because of tracheal obstruction after temporary removal of their tracheostomy cannula. VNEP is an effective, non-invasive, treatment in infants with CHS if initiated before tracheostomy. It may improve the children's quality of life during the daytime. If upper airway obstruction is a problem in the first year of life, it may be combined with nasal mask CPAP.     

Alternative treatment to tracheostomy in obstructive sleep apnea syndrome: nasal continuous positive airway pressure in young children

Two groups of prepubertal children with severe obstructive sleep apnea syndrome received continuous positive airway pressure (CPAP) treatment using a nasal mask. The five children in group A had nasal CPAP only in a hospital setting; the five children in group B received the treatment at home as well. Four of these five children, who have now been followed for as long as 10 months, have adapted well to the treatment at home. The problems encountered with nasal CPAP as a home treatment, such as poor cooperation from parents, allergic rashes, eye irritation resulting from air leaks, are discussed. Despite the problems, nasal CPAP is a valid alternative to tracheostomy in children as young as 3 years of age.     

Paediatric home ventilatory support: the Auckland experience

OBJECTIVES: To examine the trend over time, describe the disease categories treated, intervention success and outcomes of the children treated at home with continuous positive airway pressure (CPAP), non-invasive ventilation (NIV) and ventilation via tracheostomy (invasive ventilatory support, IVS) by the Respiratory Service at the Starship Children's Hospital in Auckland. METHODS: A retrospective review was undertaken of the Respiratory Service records from November 1991 to February 2004. RESULTS: Home CPAP, NIV or IVS was initiated in 160 children (89 boys, median age 6 years) in the study period. Sixty-nine (46 boys) remain on support and are still actively managed by the Starship Respiratory Service, of whom 46% live outside the Greater Auckland Region. Despite 42% of children being less than 5 years of age at initiation of therapy, institution of support failed in only 11%. The majority received treatment by non-invasive mask interface (68% (n = 108) CPAP, 29% (n = 47) NIV), with only 3% (n = 5) supported via tracheostomy. The numbers and complexity of support rose over the 12 years. Respiratory support was discontinued in 57% of cases, after a median of 12.5 months (range 3-52 months); in two-thirds, support was no longer required due to an improvement in the medical condition. The most common indication for support in current patients is respiratory parenchymal or airway disease followed by neuromuscular disease. Obesity is not a common indication. CONCLUSION: This review documents the increasing trend in children receiving respiratory support at home. Future planning and resources are needed to address this growing need.     

A home respiratory support programme for children by parents and layperson carers

Aim:   To describe a respiratory support programme for children at home by parents and layperson carers.
Methods:   Analysis of records of children with long-term mechanical respiratory support at home.
Results:   From 1979 to 2008 the programme managed 168 children (median age 7 years, range 3 weeks–19 years) with obstructive sleep apnoea (55, 32%), neuromuscular conditions (42, 25%), tracheo-bronchomalacia (23, 14%), kyphoscoliosis-cerebral palsy (20, 12%), acquired central hypoventilation (8, 5%), congenital central hypoventilation (7, 4%), chronic lung disease or pulmonary hypoplasia (8, 5%), traumatic quadriplegia (3, 2%) and tumour-related quadriplegia (2, 1%). One hundred and sixty-one (96%) were discharged: 73 (46%) remain in the programme; 27 (16%) transferred to adult services, 25 (15%) recovered and 36 (23%) died. Principal modes of therapy were mask continuous positive airway pressure (CPAP) 35%, mask bilevel positive airway pressure 30%, tracheostomy CPAP 20%, tracheostomy mechanical ventilation 8%, phrenic nerve pacing 3%, negative pressure chamber ventilation 2% and nasal tube CPAP 2%. Two unexpected deaths occurred at home: one from accidental tracheostomy decannulation and another unrelated to respiratory support. Average time in the programme was 3.3 years. Parents of 69 children were provided with trained carers. Successful discharge resulted from early recognition of potential to discharge, parental training, recruitment and training of carers, purchase of equipment and secure funding. Seven children were not discharged, two of whom died in the hospital and five are subject to discharge planning.
Conclusion:   Respiratory support of children at home by trained parents and layperson carers is safe and efficient. All modes of respiratory support may be used.     

Phrenic nerve paralysis of obstetrical origin: favorable course using continuous positive airway pressure]

INTRODUCTION: Isolated diaphragmatic paralysis due to obstetrical factors is rare and therapeutic management modalities are not quite clear. CASE REPORT: A neonate born by breech delivery presented with respiratory distress due to isolated paralysis of the right hemidiaphragm. The clinical course was progressive, his condition worsening with oxygen supplementation. Continuous positive airway pressure (CPAP) delivered via a nasal cannula was started in the one-month-old child, inducing gradual improvement towards recovery at the age of two months and a half. CONCLUSION: Non-invasive nasal CPAP should be proposed for the treatment of phrenic nerve obstetrical palsy before introducing more invasive ventilation techniques. Surgical plication should be delayed until the child reaches the age of at least three months.     

Bilateral Diaphragmatic Paralysis After Cardiac Surgery: Ventilatory Assistance by Nasal Mask Continuous Positive Airway Pressure

The case of an 8-month-old boy with bilateral diaphragmatic paralysis after surgical reoperation for congenital heart disease is presented. In order to avoid repeated intubation and long-term mechanical ventilation or tracheotomy, we used nasal mask continuous positive airway pressure (CPAP) as an alternative method for assisted ventilation. Within 24 hours the boy accepted the nasal mask and symptoms such as dyspnea and sweating disappeared. Respiratory movements became regular and oxygen saturation increased. Nasal mask CPAP may serve as an alternative treatment of bilateral diaphragmatic paralysis in infants, thereby avoiding tracheotomy or long-term mechanical ventilation.     

Developmental outcomes at the age of two years for very premature babies managed with nasal prong continuous positive airway pressure

AIMS: To describe the developmental progress of very premature babies at the age of 2 years, who were managed in an era where nasal prong continuous positive airway pressure (CPAP) was the preferred method for the management of initial respiratory disease. METHODS: Two groups of very premature babies of <32 weeks gestation were compared, the first being managed with an intubation and mandatory ventilation approach, and the second being managed with a CPAP approach. The groups are compared with regard to the presence of brain injury, retinopathy and requiring discharge home on oxygen and then at 2 years, for language impairment, non-ambulatory cerebral palsy and significant developmental delay. The study is designed as an audit of data collected prospectively and longitudinally for babies born from 1998 to 2002. RESULTS: A significant number of babies were successfully managed on CPAP in the second era and significantly fewer received post-natal steroids. The number being discharged home on oxygen, brain injury and retinopathy were similar in the two groups. Developmental outcome assessed at 2 years of age was the same in both eras. CONCLUSIONS: A CPAP approach to the management of initial respiratory disease in premature babies of less than 32 weeks gestation at birth is associated with no measurable developmental advantage or disadvantage at 2 years of age.     

Nasal high-frequency ventilation for premature infants

Aim: To assess the use of nasal high‐frequency ventilation (HFV) to provide noninvasive ventilatory support for very low birthweight (VLBW) infants. Study Design: VLBW infants, >7 days of age on nasal continuous positive airway pressure (CPAP), were placed on nasal HFV for 2 h using the Infant Star high‐frequency ventilator (Mallinckrodt, Inc., St. Louis, MO, USA). Mean airway pressure was set to equal the previous level of CPAP, and amplitude was adjusted to obtain chest wall vibration. Capillary blood was sampled before starting HFV and after 2 h to determine change in pH and partial pressure of carbon dioxide (pCO₂). Results: Fourteen subjects were studied, 10 males and 4 females. Gestational age was 26–30 weeks (median 27). Age at study was 18–147 days (median 30). Median birth weight was 955 g; median weight at study was 1605 g. Nasal CPAP pressure was 4–7 cm H₂O (mean 5). Amplitude was 30–60 (median 50). After 2 h, PCO₂ (mean 45 torr) was significantly lower than initial PCO₂ (mean 50 torr) (p = 0.01), and pH had increased significantly (7.40 vs. 7.37, p = 0.04). Conclusions: Nasal HFV is effective in decreasing pCO₂ in stable premature infants requiring nasal CPAP support. Long‐term use of nasal HFV requires further study.     

Nasal Mask Bilevel Positive Airway Pressure Ventilation for Diaphragmatic Paralysis After Pediatric Open-Heart Surgery

A 2-year-old boy underwent surgical repair of tetralogy of Fallot. Topical cooling of the heart with ice slush was used during the operation. Diaphragmatic paralysis occurred after the operation, inducing severe respiratory distress. To avoid repeated intubation and tracheostomy, the patient was placed on nasal mask bilevel positive airway pressure (BiPAP) ventilation. After ventilatory support with BiPAP for 40 days, the patient recovered spontaneously from the paralysis. No sedation was required during this time. This report illustrates the usefulness of BiPAP for a pediatric patient with diaphragmatic paralysis after cardiac surgery.     

Effect of jaw-thrust and continuous positive airway pressure on tidal breathing in deeply sedated infants

OBJECTIVES: To examine the physiologic impact of the jaw-thrust maneuver or the administration of continuous positive airway pressure (CPAP) on tidal breathing in deeply sedated infants.Study design: Prospective, non-randomized study of infants undergoing elective fiberoptic bronchoscopy while sedated with intermittent doses of propofol. METHODS: Spontaneous tidal breathing was measured in the supine position by means of a spirometer attached to a bronchoscopy face mask. Tidal breaths were recorded under the following conditions: (1) neutral sniffing position, (2) jaw-thrust, (3) neutral sniffing position, and (4) CPAP of 5 cm H(2)O. Improvement was defined as a change of more than twice the coefficient of variation of repeated measurements of tidal volume and flows from baseline. RESULTS: Jaw-thrust increased tidal volume, minute ventilation, and peak tidal inspiratory and expiratory flows significantly in all 13 infants studied (mean +/- SEM age = 8 +/- 2 months). CPAP increased peak tidal inspiratory and expiratory flows by more than twice the coefficient of variation of baseline measurements in 6 patients and tidal volume and minute ventilation in 5 of 10 patients studied. CONCLUSION: Jaw-thrust and CPAP are effective techniques to improve ventilation of sedated infants undergoing interventions that compromise upper airway patency.     

A randomised controlled trial of two methods of delivering nasal continuous positive airway pressure after extubation to infants weighing less than 1000 g: binasal (Hudson) versus single nasal prongs

OBJECTIVES: Primary: to determine whether nasal continuous positive airway pressure (CPAP) delivered through binasal prongs results in a greater proportion of extremely low birthweight infants being successfully extubated, after a period of intermittent positive pressure ventilation, than nasal CPAP delivered by a single nasal prong. Secondary: to evaluate the effect of mode of delivery of nasal CPAP after extubation on the need for endotracheal reintubation, weight gain, rates of feeding intolerance, sepsis, suspected sepsis, cranial ultrasound abnormalities, retinopathy of prematurity, chronic lung disease, and the duration of assisted ventilation and care in the tertiary neonatal unit. DESIGN AND SETTING: Randomised, controlled, clinical trial conducted at the neonatal intensive care unit of the Royal Women's Hospital, Melbourne, Australia. PATIENTS: Infants of birth weight less than 1000 g, ventilated, requiring < 50% oxygen and ventilator rate less than or equal to 20/minute, and considered by the clinical management team to be ready for extubation. INTERVENTION: Infants were randomly allocated to receive nasal CPAP delivered through binasal (Hudson) prongs or a single nasal prong. PRIMARY OUTCOME MEASURE: Failure of extubation as defined by the following criteria: (a) apnoea (more than one episode/hour over a six hour period or one episode requiring bag and mask ventilation); (b) absolute increase in oxygen requirement greater than 15% above that required before extubation; (c) respiratory acidosis (pH < 7.25 with PCO(2) > 6.67 kPa). RESULTS: Ten of the 41 (24%) infants randomised to binasal prongs reached predetermined failure criteria compared with 26 of the 46 (57%) infants randomised to a single nasal prong (p = 0.005). Four of 17 (24%) infants of birth weight less than 800 g extubated to binasal prongs reached failure criteria compared with 14 of 16 (88%) extubated to a single nasal prong (p < 0.001). There were no significant differences in any of the secondary outcomes. CONCLUSIONS: For extremely low birthweight infants ventilated using an endotracheal tube, nasal CPAP delivered through binasal (Hudson) prongs is more effective in preventing failure of extubation than that delivered through a single nasal prong.     

Continuous positive airway pressure: current controversies

PURPOSE OF REVIEW: Continuous positive airway pressure is increasingly being used in the care of premature infants. The purpose of this review is to highlight the current controversies in the use of neonatal continuous positive airway pressure. RECENT FINDINGS: This review explores information about the devices available for delivering continuous positive airway pressure and the pressures that can be used. It also investigates the controversial issues of using continuous positive airway pressure during resuscitation of premature infants and whether infants who are going to be managed on continuous positive airway pressure should be intubated and given surfactant before continuous positive airway pressure is started. It reviews the use of continuous positive airway pressure and the prevention of chronic lung disease and the use of nasal intermittent positive pressure ventilation and the difficult area of weaning from continuous positive airway pressure. SUMMARY: Existing evidence suggests that short binasal prongs are most effective, nasal intermittent positive pressure ventilation is a useful way of augmenting neonatal continuous positive airway pressure and that very premature infants can be managed with neonatal continuous positive airway pressure in the delivery room as part of the resuscitation. Further research is required to determine whether important outcomes are improved with the use of nasal continuous positive airway pressure rather than endotracheal intubation and, if so, whether surfactant should be given to infants so managed. Definition of optimal levels of continuous positive airway pressure for infants at varying stages of their disease also requires further research.     

Laryngeal mask airway used as a delivery conduit for the administration of surfactant to preterm infants with respiratory distress syndrome

BACKGROUND: The laryngeal mask airway (LMA(TM), Laryngeal Mask Co. Ltd, Jersey, UK) is a supraglottic device used to administer positive pressure ventilation (PPV) in adults, pediatric and neonatal patients. OBJECTIVES: To avoid endotracheal intubation, we evaluated the feasibility and practicality of administering surfactant via the LMA(TM) in preterm infants with respiratory distress syndrome (RDS). METHODS: Infants less than 72 h old with a gestational age of < or =35 weeks and a birth weight of >800 g, treated with nasal continuous positive airway pressure (CPAP, 5 cm H2O) for RDS were eligible for inclusion in the study if the arterial-to-alveolar oxygen tension ratio (a/APO2) was <0.20 over a period of >60 min. RESULTS: Eight preterm infants, median gestational age 31 (range 28-35) weeks; birth weight 1,700 (880-2,520) g, treated with nasal CPAP for RDS were enrolled. Three hours after surfactant instillation, the mean a/APO2 was significantly increased (0.13 +/- 0.04 to 0.34 +/- 0.11; p < 0.01) without complications. CONCLUSIONS: The LMA may be a useful and noninvasive conduit for the administration of surfactant therapy. A large randomized comparative clinical trial will be required to confirm the efficacy of this technique.     

影响新生儿鼻塞持续气道正压通气压力不稳定因素分析

目的 观察影响新生儿鼻塞持续气道正压通气(n-CPAP)过程中导致压力不稳定的因素.方法 对2006年10月 - 2009年8月新生儿科148例使用n-CPAP辅助呼吸的新生儿,记录导致n-CPAP压力不稳定的因素,包括鼻塞滑脱、鼻塞堵塞、管路冷凝水过多、患儿躁动不安、固定不妥、鼻塞过小、管路漏气等,并进行Logistics回归分析.结果 鼻塞滑脱、鼻塞堵塞、管路冷凝水过多、患儿躁动不安、固定不妥与n-CPAP压力异常显著相关.结论 鼻塞脱落、鼻塞堵塞、管路冷凝水过多、患儿躁动不安、固定不妥为引起压力异常的主要因素,应予以及时、及早排除.     

Hyaline membrane disease. Comparison of continuous negative pressure and nasal positive airway pressure in its treatment.

The effectiveness of continuous negative pressure (CNP) and nasal continuous positive airway pressure (CPAP) in the treatment of hyaline membrane disease and the incidence of complications were compared in 36 preterm infants randomly treated with CNP or nasal CPAP. Both methods of treatment were effective in increasing PaO2 and allowing a decrease in inspired O2 concentration. The time required with mechanical assistance and with an O2 concentration of more than 40% was equal in both groups. In the CNP group, four infants required mechanical ventilation because of clinical deterioration whereas in the nasal CPAP group, seven needed this type of therapy. Three infants in each group had extraalveolar air, and two patients in each group died. The results suggest that both methods of applying continuous distending airway pressure are effective. Nasal CPAP has the advantages of easier application and better access to the infant.     

Infant botulism: a review of 12 years' experience at the Children's Hospital of Philadelphia

Fifty-seven patients with infant botulism were cared for at The Children's Hospital of Philadelphia between 1976 and 1987. The ages of the children ranged from 18 days to slightly more than 7 months. The average duration of hospitalization was 44 +/- 34 days, with the average intensive care unit stay lasting 29 +/- 25 days (54 of 57 patients). The majority (77%) of the patients were ultimately intubated and mechanically ventilated (68%). The principal indication for intubation was loss of protective airway reflexes and not hypercarbia or hypoxemia. In those patients who required mechanical ventilation the average duration was 23 +/- 22 days, with the 10 most severely affected patients (greater than or equal to 28 days of mechanical ventilation) averaging 53 +/- 25 days. Excluding patients ventilated for more than a month, those who underwent tracheostomy were hospitalized nearly twice as long as those who were managed by nasotracheal intubation only (33.5 days vs 63.2 days). The use of continuous nasogastric feedings has supplied most infants with sufficient enteral feedings to avoid weight loss and the need for central intravenous alimentation.     

Controlled trial of continuous positive airway pressure given by face mask for hyaline membrane disease.

A controlled trial of elective intervention with continuous positive airway pressure (CPAP) was performed on 24 infants with hyaline membrane disease whose arterial oxygen tension (Pao2) fell below 8kPa (60 mmHg) while they were breathing a fractional inspired oxygen concentration (F1O2) greater than 0.60. A face mask was used to apply the CPAP. The progress of the 12 infants who were treated on entry to the trial was compared with that of 12 infants who were treated later. All 12 infants in the early-intervention group and 8 infants in the late-intervention group survived. When CPAP was started, Pao2 increased and the early-treated infants breathed high concentrations of oxygen for a shorter period than the late-treated infants. The 4 infants in the early-intervention group who required mechanical ventilation needed lower mean airway pressures to achieve satisfactory gas exchange than the 7 ventilated infants in the late-intervention group. We conclude that a Pao2 less than 8 kPa while breathing an F1o2 greater than 0.60 is an adequate indication for giving CPAP in hyaline membrane disease, and that early intervention with CPAP allows infants who go on to require mechanical ventilation to be ventilated at lower pressures.     

Pulmonale Verkalkungen infolge einer Escherichia-coli-Pneumonie

We report a neonate (gestational age 24 5/7 weeks) who developed severe sepsis with Escherichia coli pneumonia on the 11th day of life. Nine weeks after the initial diagnosis of E. coli pneumonia, calcifications in the right lung were diagnosed. After intermittent antibiotic therapy for 98 days, invasive ventilation for 47 days, and nasal CPAP (continuous positive airway pressure) for 65 days, the infant was discharged on supplementary oxygen therapy.     

Pediatric tracheostomies: a recent experience from one academic center.

OBJECTIVES: To describe the indications, surgical timing, length of stay, hospital charges, and discharge disposition of pediatric tracheostomy patients. DESIGN: Retrospective case series. SETTING: Large urban academic pediatric hospital. PATIENTS: Seventy children and adolescents undergoing tracheostomy placement over a 24-month period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Hospital database records were used to determine demographics and readmission rates, tabulate charges, and confirm deaths. Indications for tracheostomies included airway obstruction, inadequate airway protection, chronic lung disease, neuromuscular weakness, and central hypoventilation. Surgical timing of the tracheostomy was grouped into three categories: prolonged mechanical ventilation, elective, or emergent. The overall median hospital stay was 46 days (range 14-254) with a median hospital charge of $136,718 (range $36,237-$913,934). The prolonged mechanical ventilation group underwent a tracheostomy after a median of 26 days (mean 37.5 days) on the ventilator. Eighty-one percent of children were discharged home; 63% of children were readmitted within 6 months, with 11% requiring four or more admissions. The six-month mortality rate was 13%; no deaths were related to the tracheostomy. CONCLUSIONS: Children with tracheostomies are a heterogeneous population. Children who require tracheostomy for long-term mechanical ventilation have longer hospital stays than children who receive a tracheotomy on an elective or emergent basis. Hospital readmissions should be anticipated in this complex group of patients.     

Using continuous nasal airway pressure in infants with craniofacial malformations

Obstructive sleep apnea (OSA) is common in infants and children with craniofacial malformations. Continuous positive airway pressure (CPAP) represents an effective noninvasive treatment for severe upper airway obstruction in these children, reducing the need of surgery or a tracheostomy. The decision to start CPAP should be discussed by a multidisciplinary team in order to decide the optimal individualized treatment strategy. CPAP initiation depends on patients’ clinical characteristics and local practices, with an increase tendency towards an outpatient program. Follow-up and monitoring strategy varies among centers but benefits from the analysis of built-in software data in order to assess objective adherence and breathing parameters, reducing the need of in-hospital sleep studies. The possibility to wean CPAP should be periodically checked after surgical treatment or when spontaneous resolution is suspected. Finally, these infants with craniofacial malformations should have a long term follow up because of the risk of OSA recurrence over time.