[Pediatric intensive care after brain tumor surgery]. / Postoperatorio de tumores cerebrales en la unidad de cuidados intensivos pediátricos.

INTRODUCTION: Primary brain tumors are the most common solid tumors in children. Surgery is the basis of treatment for these patients, who require postoperative admission to the ICU-P. The aim of this study was to at the epidemiology of brain tumors of patients admitted to our ICU-P and to analyze the progress of these children in the postoperative period. PATIENTS AND METHODS: Retrospective-prospective study of children admitted to our unit after brain tumor surgery between January 1998 and January 2007. We collected information such as, personal details, clinical characteristics, type of intervention and postoperative period. RESULTS: We reviewed 161 postoperative periods, corresponding to 134 patients (54.5% male). The mean age was 7 years and 8 months +/- 5 months. The most common location was the posterior fossa (44.8%). The most common histological type was low grade/intermediate astrocytoma (44.7%). The most frequent complication was diabetes insipidus (9.9%). Resection was complete in 58.4% patients. The median stay in the ICU-P was 2 days (0-61 days). 3 patients died after surgery. CONCLUSIONS: The epidemiology of the group of patients admitted to our ICU-P is similar to the general population. The most common complication is diabetes insipidus.

[Glossary of bioethic terms and expressions frequently used in pediatric intensive care practice]. / Glosario de términos y expresiones frecuentes de bioética en la práctica de cuidados intensivos pediátricos.

Ethical issues are of increasing interest in current medicine, and pediatrics is no exception. In critical care, the relevance of these considerations becomes even greater. Commonly used expressions in bioethics, frequently lead to terminological confusion and misunderstandings, as reported by several publications, revealing a lack of clear concepts in many cases. As an attempt to clarify or facilitate the comprehension of the most relevant terms in this field, the Spanish Society of Pediatric Intensive Care has prepared a Glossary of the most commonly used terms and expressions.

[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.

[Supraventricular tachycardia in infants and children]. / Taquicardia paroxística supraventricular en el niño y el lactante.

INTRODUCTION: Supraventricular tachycardia (SVT) is the second most frequent form of arrhythmia in pediatrics after extrasystole. OBJECTIVES: 1. To determine the clinical characteristics and treatment of SVT in infants and children. 2. To determine treatment response and the drugs used. METHOD: A retrospective review of 61 cases of SVT requiring PICU admission (1999-2004) was performed. PICU admission was due to persistent SVT after vagal maneuvers. RESULTS: There were 61 patients and 39 were boys (63.9%). The mean age was 2.1 years (SD +/- 3.1). Twelve patients had congenital heart disease (19.7%); three (4.9%) were admitted after heart surgery, and the remaining patients had no antecedents (60.7%). The mean cardiac frequency was 238 beats/min (SD +/- 42.86). Heart failure (HF) was observed in 14 patients (23%). Statistically significant differences were found between the presence of HF and time since onset (p < 0.01) and younger age (p < 0.01). The most frequent diagnosis was SVT due to re-entry in 28 patients (45.9%). Medical treatment was required in 46 patients (75.4%) and response was achieved in 35 (57.4%). At crisis the first drug used was adenosine triphosphate (ATP) in 35 patients (61.4%) with good response in 21 (36.8%). As maintenance therapy digoxin was used in 29 patients (50.9%) without relapses in 22 (78.6%). Radiofrequency ablation was required in 17 patients (27.9%), and there were three relapses (17.6%). The ages of patients who underwent ablation ranged from 3.5 days to 13 years. CONCLUSIONS: 1. HF was observed mainly in infants. 2. Most of the patients had good response to ATP therapy. 3. Radiofrequency ablation was mainly required in patients aged more than 1 year.

[Management of pediatric multiple trauma patients. Perspective of the pediatric intensive care unit]. / Asistencia al paciente politraumatizado. Realidad actual desde la perspectiva de las unidades de cuidados intensivos.

OBJECTIVE: To study the epidemiology and management of pediatric trauma patients as well as the organizational, human and technical resources dedicated to these children from the perspective of the pediatric intensive care unit (PICU). MATERIAL AND METHODS: A standardized data collection form was sent to 43 PICUs in Spain. Items inquired about the existence of training courses, trauma clinical practice guidelines and trauma registers, and which physician was in charge of trauma patients. Data on casuistics, the age of trauma patients, and the availability of human and technical resources, were also recorded. RESULTS: Twenty-four PICUs completed the questionnaire. The PICU physician was responsible for trauma patient care in 66% of the hospitals. No training courses were available in 59% of the hospitals. No trauma register was available in 62% of the hospitals. Trauma patients represented 11% of PICU admissions, and most patients were aged up to 14 years old. An anesthetist was always at the hospital in 100% of the hospitals. A radiologist and traumatologist were always at the hospital in 91%, a neurosurgeon in 66% and a pediatric surgeon in 50%. The remaining surgical and medical specialties were on call. Continuous intracranial pressure monitoring was available in 87% of the PICUs, jugular venous saturation monitoring in 54% and continuous electroencephalogram and transcranial Doppler ultrasound in 50%. Computed tomography and ultrasound were available at all times in all hospitals. Magnetic nuclear resonance and echocardiography were available at all times in 44% of the hospitals, and arteriography in 42%. CONCLUSION: In Spain, the organization of pediatric trauma management is based on pediatric teams under the supervision of a PICU physician. Some hospitals show a lack of technical and human resources. Therefore, the minimum criteria required to consider a hospital as a pediatric trauma center should be established. Trauma training courses are required.

[Fluid and electrolyte disorders following surgery for brain tumors]. / Trastornos hidroelectrolíticos en postoperados de tumores cerebrales.

INTRODUCTION: Fluid and electrolyte disorders such as diabetes insipidus, salt wasting syndrome (SWS) and syndrome of inappropriate antidiuretic hormone secretion (SIADH) can appear in the immediate postoperative period after surgery for brain tumors. Early diagnosis and treatment are important to prevent the potential adverse effects of these disorders on the central nervous system (CNS). OBJECTIVES: To determine the incidence and characteristics of fluid and electrolyte disorders in the immediate postoperative period after surgery for CNS tumors in children treated in our hospital. MATERIAL AND METHODS: We retrospectively analyzed clinical and laboratory data in all infants and children who underwent surgery for CNS tumors in our hospital from January 1998 to June 2005 and who met the laboratory criteria for diabetes insipidus, SWS or SIADH. RESULTS: Twenty-three electrolyte disorders were identified in 149 surgical patients (an incidence of 15.4%). The median age was 5 years and 3 months (from 6 months to 17 years) and 48.7% of the patients were male. The most frequent electrolyte disturbance was diabetes insipidus (65.2% of all electrolyte disorders). On average, onset of diabetes insipidus occurred 19 hours after surgery. Treatment with desmopressin was administrated in all patients. On average, diabetes insipidus was resolved 73 hours after diagnosis, except in one patient with permanent diabetes insipidus due to a surgical lesion of the hypothalamic-pituitary axis. The second most frequent electrolyte disturbance was SWS (26.1%) with a mean time of onset of 50.4 hours after surgery. On average, SWS was resolved 57.6 hours after administration of saline solutions. Only two patients developed SIADH, which was treated with water restriction and adequate sodium supply. Both cases of SIADH resolved spontaneously in the first 36 hours after diagnosis. At discharge, none of the patients showed neurological disturbances due to an electrolytic disorder. CONCLUSIONS: In our series, the most frequent electrolyte disorder after surgery for CNS tumors was diabetes insipidus. Early treatment with desmopressin almost always prevents hypernatremia. Unless there is a surgical lesion of the hypothalamic-pituitary axis, spontaneous resolution will take place in 3 days on average. The management of SWS and SIADH requires close monitoring of plasma sodium due to the risk of hyponatremia.

[Ethical attitudes in Spanish pediatric critical care units]. / Encuesta de ética en las unidades de cuidados intensivos pediátricos españolas.

OBJECTIVES: To assess physicians' awareness and experience of ethical problems that arise when dealing with critically ill children in pediatric intensive care units (PICUs). MATERIAL AND METHODS: Questionnaires containing 20 questions about ethical dilemmas and attitudes related to the care of children admitted to PICUs were mailed to 43 PICUs in Spain. RESULTS: Ninety-five responses corresponding to 24 residents and 71 attending physicians were received from 21 PICUs. The occurrence of ethical dilemmas in the PICU was recognized by 96.8 % of the respondents. The most frequent method of solving these problems was through medical consensus (80 %), while family participation in the decision making process was highly variable. A total of 95.8 % of respondents stated that decisions to limit therapy were made in their PICU, although only one third of these decisions were written in the medical record. The most frequent form of therapeutic limitation was the do not resuscitate order. One third (32.6 %) of participants considered there were ethical differences between withdrawal and withholding of treatment. Attending physicians had greater experience of therapeutic limitation than did residents, but their opinions on the subject were similar. CONCLUSIONS: Ethical dilemmas are common in the PICU. In this setting, decisions about limitation of therapy are frequent, although many physicians admit to not being clear on this issue or on other aspects of clinical ethics. Family members' participation in the decision making process is insufficient in Spanish PICUs.

[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.

[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.

[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.

[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.

[Acute infantile bilateral thalamic necrosis]. / Necrosis talámica bilateral aguda infantil.

INTRODUCTION: Acute necrotizing encephalitis is a clinical entity recently described in previously healthy Japanese children. Following a respiratory or gastrointestinal infection of a viral nature, these patients present a sudden deterioration in the level of consciousness. Since neuroimaging shows a bilateral thalamic disorder in all cases, the name infantile bilateral thalamic necrosis has been put forward to refer to this affection. CASE REPORT: We report the case of an 11 month old Spanish patient who was brought into the hospital with disconnection from the surroundings and hypertonia in the upper extremities, within the context of a febrile gastrointestinal picture. Blood sample analysis revealed haemoconcentration and metabolic acidosis, although it did not suggest an infection. The computerised tomography scan of the brain showed a striking hypodensity of both thalami. The initial hypertonia was followed by a generalised hypotonia, accompanied by haemodynamic and respiratory deterioration. The patient died 40 hours after admission. CONCLUSIONS: Acute necrotizing encephalitis was initially described in Japanese children but in recent years isolated cases have also been reported in the Western world. In the case of the patient we have described, one of the most outstanding points is that evolution was unfavourable in spite of mechanical ventilation and the use of inotropic drugs, together with the clinical state of brain death that occurred on the second day after admission. Since neuroimaging showed a selective disorder of the thalami in all cases, and the fact it is an entity that affects small children, it seems more appropriate to use the term acute infantile thalamic necrosis

[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.

[Cerebral venous thrombosis in a girl carrier of the prothrombin gene mutation 20210G--> A treated by local fibrinolysis of the superior sagittal sinus]. / Trombosis venosa cerebral en niña portadora de la mutación 20210G--> A del gen de la protrombina, tratada mediante fibrinólisis local del seno sagital superior.

CASE REPORT: Girl, aged 4, without antecedents who was admitted to our hospital for drowsiness and progressive sensorial depression. Within 24 hours the clinical picture deteriorated with partial seizures of the right hand side of the body and right hemiparesis. A brain CAT scan showed a left temporoparietal parenchymatous haematoma with collapse of the left lateral ventricle and moderate obliteration of the basal cisterns. MR angiography and cerebral arteriography displayed images that were compatible with thrombosis of the superior sagittal, the left transverse and the sigmoid sinuses. The patient was heterozygotic for the G A mutation in position 20210 of the prothrombin gene, which is linked with a high risk of thrombosis. She was given heparin intravenously, but continued to display endocranial hypertension and tissue ischemia with partial response to hyperosmolar agents and barbituric coma. She was therefore submitted to selective catheterization of the superior sagittal sinus and continuous local fibrinolysis with urokinase for 72 hours. The outcome was satisfactory, with repermeabilisation of the thrombosed sinuses and a good clinical response with no complications. At present the patient has functional paresis of the right hand and receives treatment with oral anticoagulants. CONCLUSIONS: We advocate the use of early local fibrinolytic treatment with urokinase in children affected by thrombosis of the venous sinuses who do not respond to treatment with sodium heparin. We consider it necessary to include the molecular study of the G20210A mutation of the prothrombin gene in screening for prothrombotic risk factors in small children

[Neurofibromatosis type 2 as a result of a de novo mutation: a case report]. / Neurofibromatosis tipo 2 por mutación de novo. A propósito de un caso.

INTRODUCTION: Neurofibromatosis type 2 is a dominant autosomic hereditary disease which courses with distinct tumours of the central nervous system and scant cutaneous manifestations. The increased knowledge of the natural history and the genetics of NF 2 acquired over the past few years has shown that clinical onset possibly occurs during the paediatric age and an early diagnosis of these patients can be decisive in the final outcome. CLINICAL CASE: A 12 year old girl who visited the clinic because of a month old presentation of cervical tumour, otalgia and dysphonia. Exploration revealed signs of cranial nerve disorder and the magnetic resonance (MR) showed bilateral schwannomas of the eighth cranial nerves. The extension study showed ocular, auditory, troncoencephalic and cervical spinal cord disorders. The patient died three months after hospital admission. The genetic study showed a de novo mutation in the NF 2 gene (chromosome 22q12). DISCUSSION: The identification of the various mutations that cause NF 2 has enabled the early diagnosis of the patient s relatives. However, there are still patients who have not been confirmed genetically. Furthermore, de novo mutations are not predictable. NF 2 diagnosis is still clinical. In the last few years, two disease phenotypes have been defined: mild and moderate/serious, which is associated with an early onset and de novo mutations. The high incidence rate of cataracts and other associated tumours, such as those affecting paraspinal and cutaneous areas together with meningiomas, which up until now could have gone unnoticed, has also been observed. Clinical onset in the paediatric age is more frequent than was expected and shows distinct and subtle symptoms.

[Knowledge of health care ethics in paediatric residents]. / Conocimientos sobre ética asistencial de los residentes de pediatría.

INTRODUCTION: Bioethics has been recently incorporated in to the educational programs of both medical students and medical residents as part of their curriculum. However, its training based on clinical practice is not well structured. OBJECTIVE: To evaluate the knowledge of bioethics in Spanish paediatric residents, and to analyse how this relates to the medical education during graduate and post-graduate training. MATERIAL AND METHODS: A questionnaire with 20 multiple choice questions was designed to evaluate the knowledge in basic ethics with potential implications in clinical practice. We evaluated the education received during graduate and post-graduate training, and the main ethical conflicts faced. RESULTS: A total of 210 completed questionnaires were received from medical residents in paediatrics from 20 different Spanish hospitals, of whom 47 of these were first year residents (R1), 49 were second year residents (R2), 57 were third year residents (R3), and the remaining 57 were final year residents (R4). The mean number of correct answers was 16.8 out of 20. No differences were found between residents in different years of training, nor were there any differences between the group that had received specific training in bioethics versus those who had not. Residents were more likely to give wrong answers related with informed consent, the law on the freedom of the patient, principles of quality of life, the case analysis system, and the dimension of distributive justice. CONCLUSIONS: Limitation of therapeutic efforts was identified as the main ethical problem faced in clinical practice by Spanish residents in paediatrics. Most of the knowledge of bioethics is acquired during graduate training, and improved very little throughout the period of medical residence. Our results suggest that efforts are required in organising and structuring the education in bioethics during the training of residents in paediatrics.

[Fever, asthenia, myalgia and murmur due to cardiac myxoma]. / Fiebre, astenia, mialgia y soplo por mixoma cardíaco.

Cardiac tumours are rare, especially in children, and most of them are benign. Myxomas are unusual in children, being more common among adults. They are usually located in the left atrium, with 25% appearing in the right. The clinical signs and symptoms depend mainly on where the tumour is located. A feature of these tumours is that they can be accompanied by constitutional symptoms and laboratory abnormalities. Echocardiography is the study of choice, and a prompt resection is required to prevent serious complications. We present a case of a 10 year-old girl diagnosed with right atrial myxoma who presented with a fever, myalgia, asthenia and laboratory abnormalities. Diagnosis was made by echocardiography, and the early surgical resection of the tumour ran smoothly and showed a good postoperative recovery.