[Domiciliary mechanical ventilation in children: a Spanish multicentre study]. / Ventilación mecánica domiciliaria en niños: estudio multicéntrico español.

INTRODUCTION: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. MATERIALS AND METHODS: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. RESULTS: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. CONCLUSIONS: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated.

[Endovascular treatment of pulmonary sequestration with Amplatzer® vascular plugs]. / Tratamiento endovascular del secuestro pulmonar con dispositivos tipo Amplatzer®

Pulmonary sequestration is a rare malformation characterised by non-functioning lung tissue, separated from the tracheobronchial tree and with arterial supply from the systemic circulation. The classical therapeutic approach is surgical resection. In recent years, the endovascular embolisation technique is increasingly being used for the treatment of this disease. The embolisation materials used are coils and vascular plugs. The Amplatzer® vascular plug is a self-expandable cylindrical mesh device, particularly useful for the embolisation of large vessels with high flow. Three cases of pulmonary sequestration were treated by embolisation with Amplatzer® vascular plugs. In all cases the procedure was performed without complications, and the aortopulmonary collateral vessels were completely occluded. Our study provides new data on the safety and efficacy of pulmonary embolisation with Amplatzer® vascular plugs, and its preference over coils in the embolisation of large vessels.

[Respiratory disease and diaphragm paralysis in Charcot-Marie-Tooth disease]. / Enfermedad respiratoria y parálisis diafragmática en la enfermedad de Charcot-Marie-Tooth.

Diaphragmatic bilateral palsy is uncommon in children. The most important etiologies are thoracic surgery and obstetric trauma. Respiratory muscle impairment is a rare phenomenon in patients with Charcot-Marie-Tooth disease (CMT). However, it can be associated with restrictive pulmonary impairment, phrenic nerve dysfunction or thoracic cage abnormalities. We report two paediatric cases of CMT disease with type 2 respiratory failure due to diaphragmatic dysfunction. In both cases treatment with non-invasive mechanical ventilation resulted in satisfactory clinical improvement. Evidence of phrenic damage was the main clue in one patient in order to obtain an accurate diagnostic of her disease.

[Non-invasive ventilation after cardiac surgery. A prospective study]. / Aplicación de ventilación no invasiva en pacientes postoperados cardíacos. Estudio retrospectivo.

AIM: To report our experience with non-invasive ventilation (NIV) after cardiac surgery. MATERIALS AND METHODS: Prospective study of cardiac surgery patients admitted to our PICU between 2004 and 2007 who required NIV after extubation. RESULTS: A total of 331 patients were admitted to the unit after cardiac surgery during this period. Of these, 159 were extubated in the operating room. NIV was introduced in 29 episodes on 26 patients. Fallot's tetralogy and AVD were the most common heart diseases, and 65% had type II respiratory failure. The respiratory problems usually involved were acute pulmonary oedema and atelectasis. Indication was elective in 70% of episodes. BiPAP Vision was the commonest ventilator used and in the S/T mode (56%).Average length of use was 64h. Average length of stay in PICU was 22 days. Nine patients required reintubation, none of them at an early stage (first 12h) which represents an efficiency of 66%. The effectiveness of NIV was related to the type of disease and inversely with the time NIV was needed. There were complications in 12 episodes. Four patients required tracheostomy, all of which were associated with previous lung disease. Survival was 100%. CONCLUSIONS: NIV is effective and safe after cardiac surgery. It has very good results in respiratory failure due to atelectasis or pulmonary oedema. NIV failure in these patients is strongly associated with preoperative pulmonary sequelae secondary to heart disease. NIV indication in these patients has to be carefully evaluated.

[Seat belt syndrome. Acute spinal cord injury due to incorrect use of two-point seat belts]. / Síndrome del cinturón de seguridad: lesión medular aguda por uso incorrecto del cinturón de seguridad de dos puntos.

Traumatic spinal cord injury (SCI) is a rare entity in the pediatric age group. These injuries are mainly caused by road traffic accidents (RTA), especially in children not wearing a seat belt. The use of child safety devices such as seat belt restraints has decreased morbidity and mortality in RTA but their incorrect use can also produce serious injuries that are grouped under the term "seat-belt syndrome". This syndrome associates vertebral and spinal cord injuries, intra-abdominal, cutaneous, and muscle-skeletal lesions. We present three patients with complete spinal cord and intra-abdominal injuries, requiring urgent surgery in two of them. On examination, all three patients had seat belt marks on the lower abdominal region. The three patients can be included in this syndrome and its main cause was the use of a two-point seat belt.

[Ondine syndrome: diagnosis and management]. / Síndrome de Ondine: diagnóstico y seguimiento.

Ondine's syndrome is a congenital central hypoventilation syndrome due to a disorder in the autonomic control of breathing in the absence of any primary disease that would explain it. Although the incidence of this entity is low, it may be underestimated due to the variable clinical expression of this syndrome, depending on its severity. Early diagnosis is of great importance to provide appropriate management to prevent the acute and chronic asphyxia that determines the long-term prognosis of this disease. This review aims to present practical management guidelines to provide doctors unfamiliar with this syndrome with basic knowledge of the diagnosis, treatment and follow-up of these patients.

[Non-invasive ventilation in neonates with Ondine syndrome: a real indication?]. / Ventilación no invasiva en lactantes afectados de síndrome de Ondine: 'una indicación real?

Ondine's syndrome is a congenital central hypoventilation syndrome due to a disorder in the autonomic control of breathing in the absence of any primary disease that would explain it. Noninvasive ventilation (NIV) has been reported to be effective in the management of these patients, thus avoiding the need for tracheotomy for prolonged mechanical ventilation. We describe our experience of NIV in infants with Ondine's syndrome. Two infants with Ondine's syndrome were transferred to our center for management and adjustment of therapy. On admission NIV (BiPAP VISION) was started with nasal interphase in S/T (spontaneous/timed) mode, which failed to provide suitable ventilation (PCO2 > 70 mmHg). Finally, tracheotomy for continuous mechanical ventilation was performed. Although NIV has been reported to be successful in some patients with Ondine's syndrome, its application in patients younger than 5 years does not seem to be the general norm of treatment.

[Factor V Leiden mutation as a cause of venous thrombosis]. / Mutación del factor V de Leiden como causa de trombosis venosa.

INTRODUCTION: Venous thrombosis is infrequent in paediatrics. A lot of prothrombotic risk factors have been described. Disturbances of coagulation are present in more than half of children with stroke. Leiden V factor mutation is emphasized as one of the most common genetic cause of deep venous thrombosis in Caucasian children and adults, which represents 20-25%, depending on series. CASE REPORT: Two years-old girl with hypoxic-ischemic disease and West syndrome, which presents a deep venous thrombosis in both legs. This evolved to gangrene, requiring surgical exceresis of scar and amputation of 2nd, 3rd and 4th fingers of left foot. She also presented a right frontoparietal intraparenchima haemorrhagic stroke with tetraventricular bleeding and hydrocephalus. It required an external ventricular derivation and later a Ventricular-peritoneal valve. CONCLUSIONS: The coagulation study confirmed the Leiden V factor mutation in our patient. Later studies showed the same mutation in the father. The risk of recurrence and the severity of venous thrombosis indicate life antiaggregant treatment, currently maintained with aspirin.

[Pulse oximetry and capnography]. / Pulsioximetría y capnografía.

Noninvasive methods of monitoring are crucial in the management of intensive care patients, especially in the pediatric field. Pulse oxymetry measures arterial oxygen saturation in severely ill patients, allows oxygen requirements to be adjusted to the patient, reduces invasive gasometric studies and achieves continuous monitoring of the critically ill child. Motion and deficient tissular perfusion reduce the accuracy of the measured values, but more sophisticated pulse oximeters are more effective in preventing these artifacts. Capnometers are an excellent method of measuring end-tidal CO2 values in real time in intubated patients. Capnography produces a graphic curve of end-tidal CO2 while capnometry provides a numerical representation of this concentration. This technique is highly useful in the continuous monitoring of various respiratory problems and situations such as weaning or checking the correct placement of endotracheal cannulas.

[Mechanical ventilation in pediatrics (III). Weaning, complications and other types of ventilation. Noninvasive ventilation]. / Ventilación mecánica en pediatría (III). Retirada de la ventilación, complicaciones y otros tipos de ventilación. Ventilación no invasiva.

Noninvasive ventilation (NIV), i.e. without tracheal intubation, has been reintroduced for the treatment of respiratory failure to reduce the complications of mechanical ventilation. Nowadays, NIV with positive pressure is the preferred method, applied through a mask held in place by a harness. Several masks can be used (nasal, bucconasal facial) and a variety of means can be used to keep them in place. Many respirators can be selected, ranging from those traditionally used in the intensive care unit(ICU) to specific NV respirators and conventional ICU respirators with specific software for NIV. Many respiratory modalities can be used according to the respirator (biphasic positive airway pressure [BIPAP], proportional assist ventilation, pressure support, synchronized intermittent mandatory ventilation [SIMV], etc.). NIV is mainly indicated in exacerbations of chronic respiratory failure: neuromuscular diseases, pretransplantation cystic fibrosis, and obstructive sleep apnea syndrome. It is also indicated in acute respiratory failure: pneumonia, status asthmaticus, and acute lung edema. The main contraindications are a weakened airway protection reflex(absent cough reflex) and hemodynamic instabiity. The advantages of NIV derive mainly from avoiding the complications associated with invasive ventilation. NIV also presents some disadvantages, especially the greater workload involved to ensure good patient adaptation to the respirator. The most common sequelae of NIV are skin lesions due to pressure on the nasal bridge.

[Coronary ischemia secondary to congenital anomaly of the left coronary artery]. / Isquemia coronaria secundaria a anomalías congénitas de las arterias coronarias.

Coronary artery anomalies constitute 2.2 % of congenital malformations of the heart. The most common abnormality is anomalous origin of the left coronary artery from the pulmonary trunk, also known as Bland-White-Garland syndrome. Clinical manifestations are due to myocardial ischemia caused by the creation of an arteriovenous shunt. The childhood type of this anomaly presents high mortality from heart failure. The adult type develops myocardial infarction, arrhythmias, sudden cardiac death or signs of congestive heart failure. Surgical repair is essential. Various surgical approaches are available and the treatment of choice is direct left coronary artery reimplantation in the aorta. An alternative technique is to create an aortopulmonary tunnel (Takeuchi technique). Marked improvement is usually observed after surgical repair. We report two cases of myocardial ischemia due to coronary anomalies.

[Postraumatic lesion of the carotid artery]. / Lesión postraumática de arteria carótida.

Postraumatic lesions of the carotid artery are very unusual in the general population and are especially rare in children due to the elasticity of their vessels. Because clinical expression of these lesions is mild, diagnosis can be delayed until the development of neurological signs, which are frequently irreversible. Neurological signs can be those of Horner's syndrome, drop attack, headache, vertigo, visual disorders, aphasia or transitory ischemic accidents. Carotid arterial lesion should be ruled out when the patient shows injuries in the soft tissue of the neck, when the neurological examination is incompatible with the findings of computed tomography (CT), when late neurological deficits develop or when the patient has Horner's syndrome. The patient reported herein presented partial motor seizures and hemiplegia 3 days after trauma. The most sensitive diagnostic test is angiography. Because this technique is aggressive, it is performed when suspicion is based on the results of Doppler sonography, CT or angiomagnetic resonance imaging. Treatment must be individualized. Standard therapy is anticoagulation but when this is contraindicated or the patient is asymptomatic anti-aggregating drugs are used. Thrombolytic treatment is reserved for the first few hours after injury. Surgical repair is the treatment of choice in patients with pseudoaneurysm. Because inaccessibility is one of the major difficulties in this type of surgery, intravascular stents can be a good therapeutic alternative in lesions unresponsive to medical treatment.

[Nebulized prostacyclin treatment for pulmonary hypertension in a 2-year-old girl]. / Prostaciclina nebulizada como tratamiento de la hipertensión pulmonar primaria en una niña de 2 años.

INTRODUCTION: Primary pulmonary hypertension is an unusual disease of unknown aetiology in childhood. It is defined as a mean pulmonary artery pressure greater than 25 mmHg at rest with normal wedge pulmonary pressure and absence of secondary causes. Treatment is based on diuretics and calcium-channel blockers and, in non-responders, on continuous intravenous prostacyclin. We report the case of a patient who was treated with nebulized prostacyclin who showed clinical improvement for 3 months. CASE REPORT: A 2-year-old child with right heart failure was admitted and stabilized with oxygen, restriction of liquids, and diuretics. The patient was diagnosed with primary pulmonary hypertension after exclusion of secondary causes. Cardiac catheterization confirmed the diagnosis. Pulmonary vascular reactivity did not test positive. Because of clinical deterioration, treatment with nebulized prostacyclin was started. We observed clinical improvement with a decrease in oxygen and diuretics demand for 3 months. After this period, the patient deteriorated and continuous intravenous prostacyclin was started. The patient died of heart failure 6 months after diagnosis. DISCUSSION: The use of prostacyclin in the management of primary pulmonary hypertension is an alternative in non-responding patients. Clinical improvement and decrease in oxygen demand support the use of nebulized prostacyclin as a useful approach in pediatric patients.

[Kawasaki disease. A report of 50 cases]. / Enfermedad de Kawasaki. Presentación de cincuenta casos.

OBJECTIVE: We present 50 cases of Kawasaki's disease (KD), diagnosed and controlled in our hospital between January 1984 and December 1997. PATIENTS AND METHODS: Fifty patients were reviewed. In forty-six of these cases the onset was complete, according to the Research Committee of MLNS diagnosis criteria and four cases had incomplete onset. RESULTS: The ration male/female was 1.9/1. Both fever and oral cavity lesions were present in all cases. In 93.4% desquamation of fingers was observed. The most frequent skin lesion was maculopapular rash. Fourteen patients had atypical onset: adenophlegmon, aseptic meningitis, symptomatic hepatitis, parotiditis, queratopathia punctata and arthritis. Blood analysis showed alterations of high ESR (88% of cases), C-reactive protein (62%), leukocytosis (82%) and thrombocytosis (96%). Fifteen patients were treated with gamma globulin (IGIV), five received a single 2 g/Kg dose and ten received 400 mg/Kg per day during four days. Eight percent of our cases produced cardiovascular complications, none that included coronary aneurysms. CONCLUSIONS: After IGIV treatment we observed a shortening of the febrile period and amelioration of clinical symptoms. No deaths were reported.

[Epidural hematoma in children. Prognostic factors. Analysis of 70 cases]. / Hematoma epidural en niños. Factores pronósticos. Análisis de setenta casos.

OBJECTIVE: The aim of this study was to analyze the causes of epidural hematoma in order to know its incidence in craneoencephalic trauma and establish prognostic criteria. PATIENTS AND METHODS: A retrospective study of 70 children with the diagnosis of epidural hematoma between 1990 and 1995 were studied. Clinical, radiological, chronologic variables and evolution were analyzed. RESULTS: The diagnosis was made during the first 4 years in 63% of the cases. Neurologic impairment was present at admission in only 33% of the patients. Ages ranged between 7 days and 17 years (mean age: 8 years). Of these patients, 82% were admitted to the PICU, 53% were ventilated and 19% needed ICP monitorization. Radiological findings on the first CT were skull fracture (68%) and temporoparietal epidural hematoma (66%), right-sided (63%). Other kinds of lesions were also recorded in the first and subsequent CTs. Three patients died, 63% recovered fully, 10% had serious sequelae and 23% had some degree of neurodisability. CONCLUSIONS: The following data correlated with death or neurological impairment: Multiple cerebral contusion (p = 0.002), brain edema (p = 0.05), GCS less than 8 on admission (p = 0.002), and shock (p = 0.003). On the other hand, neither surgical drainage volume, age, location of the hematoma, nor ICP values correlated with a poor prognosis.