[Myelomalacia: a cause of delayed respiratory failure. Apropos of two cases].
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Biomedical subjects
Publications and source records attributed to M Pons Odena.
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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.
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.
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.
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.
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.
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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.
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.
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.
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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.