Osteosarcoma in an Adolescent with Kniest Dysplasia: A Case Report.

CASE: A 19-year-old adolescent with Kniest dysplasia was incidentally found to have osteosarcoma of the proximal aspect of the humerus after having a chest radiograph for evaluation of an upper respiratory infection. He underwent chemotherapy and resection of the tumor, and there was no evidence of metastasis at the 16-month follow-up. CONCLUSION: Patients with osteochondrodysplasias often have multiple orthopaedic symptoms. Changes in chronic pain symptoms should be investigated to rule out insidious secondary causes of musculoskeletal pain, such as osteosarcoma.

Postoperative complications following neonatal and infant surgery: Common events and predictive factors.

BACKGROUND: Data on major non-surgical postoperative complications following neonatal and infant surgery is lacking. The goal of the present study was to describe common major complications and their predictive factors. MATERIAL AND METHODS: The study consisted of a retrospective review of medical charts of patients less than 6months of age operated in our institution over one calendar year, excluding herniorraphy surgery. The data collected included demographics, preoperative ICU bed status, ASA status, a history of cardiac malformation, hyaline membrane disease (HMD) or necrotizing enterocolitis (NEC), preoperative haemoglobin, emergent surgery status, surgery type and duration, duration of anaesthesia and the need for intraoperative fluid boluses. Complications were analysed until the 30th postoperative day. Analyses included descriptive statistics and the determination of factors associated with non-surgical complications using univariate and multivariate statistics. RESULTS: The study included 168 patients. Their postnatal age was 48±48days. Overall, 37 patients experienced major postoperative non-surgical complications. The most common major complications were haemodynamic compromise (n=19, 11.3%), multiple organ dysfunction syndrome (MODS, n=8, 4.8%) and respiratory failure requiring ventilation (n=3, 1.8%). Surgical complications occurred in 8 cases (4.8%). Four factors were identified as being predictive of non-surgical complications: PCA<40 weeks, a history of cardiac malformation, HMD or NEC, preoperative ICU status and intraoperative fluid bolus administration. CONCLUSION: This study describes common non-surgical postoperative complications in neonates and infants, and their risk factors. They were much more common than surgical complications. Further studies should focus on preventive strategies addressing these complications.

Re-alignment-procedures for skeletal dysplasia in three patients with genetically diverse syndromes.

OBJECTIVE: Disruption to endochondral ossification leads to delayed and irregular bone formation and can result in a heterogeneous group of genetic disorders known as osteochondrodysplasias. These genetic disorders arise through disturbances in the complex processes of skeletal growth causing development of unsightly skeletal deformities. METHODS : Each syndrome was diagnosed on the basis of detailed clinical and radiographic assessment. Lower limb deformities were the prime presenting feature. RESULTS: Here are presented three patients with diverse genetic syndromes, namely Wolcott-Rallison syndrome (WRS), Kniest dysplasia (KD) and Desbuquois dysplasia (DS). Genetic testing was performed in the patients with WRS and DS. The diagnosis of KD was made purely on a clinical and radiographic basis. Variable orthopaedic interventions to realign these patients' lower limbs were implemented with the aim of improving their balance and gait. CONCLUSIONS: The aim of this paper is twofold. The first part is to outline the importance of diagnosing the causes of various skeletal abnormalities in patients with osteochondrodysplasias by phenotypic and genotypic characterization. The second part is to demonstrate our techniques for surgical corrections in patients with joint laxity and malalignment and show how far techniques for growth modulation, re-alignment and ligament reconstruction have advanced.

Neumotórax en el recién nacido: experiencia de un servicio de cirugía de tórax / Pneumothorax in the newborn: experience of a thoracic surgery department

Objetivos: Evaluar la experiencia del Servicio de Cirugía de Tórax del Hospital Universitario de Maracaibo en el diagnóstico y tratamiento del neumotórax en el recién nacido. Métodos: 23 recién nacidos con neumotórax fueron evaluados por el Servicio de Cirugía de Tórax en la Emergencia Pediátrica, Unidad de Cuidados Intensivos Pediátricos y en el Servicio de Neonatología entre los años 2000–2004. Se recolectaron los datos relacionados con: la duración de la gestación, complicaciones maternas durante el embarazo, examen físico, estudios diagnósticos, tratamiento yevolución de los pacientes. Resultados: 13(56.52%) delos recién nacidos eran varones y la edad gestacional promedio fue de 35.25 +/- 2.49 semanas. 12(52.17%) nacieron por cesárea y 10(43.47%) tenían antecedentes de maniobras de reanimación al nacer. El neumotórax se localizó en el hemitórax izquierdo en 8 recién nacidos (34.78%), hemitórax derecho en 14 recién nacidos (60.86%) y en ambos hemitórax en 1 recién nacido (4.47%). 22 (95.65%) presentaron dificultad respiratoria El tratamiento incluyó una conducta expectante en 2 recién nacidos con neumotórax menor de 25%, y la colocación de tubo de toracostomía en 21 recién nacidos (91.30%) con neumotórax mayor de 25% Conclusión: el neumotórax es una complicación que se observa principalmente en el recién nacido con Apgar bajo al nacer o querequieren maniobras de reanimación y se presenta con dificultad respiratoria. Los neumotorax menores de 25% pueden manejarse con observación y radiología seriada, pero aquellos mayores de 25% requerirán colocación de tubo de toracostomía.

Objectives: To evaluate the experience of Thoracic Surgery Service in the diagnosis and treatment of pneumothorax in newborn at theHospital Universitario de Maracaibo.Methods: 23 newborn with pneumothorax were evaluated by the Thoracic Surgery Service in the Pediatric Emergency Unit, PediatricIntensive Care Unit and Neonatology Service between the years 2000-2004. Data was collected regarding gestational age, complications during the pregnancy, physical exam, diagnostic studies, treatment and evolution of the patients. Results: 13 (56.52%) were males, with mean gestational age of 35 +/- 2.5 weeks. 12 (52.17%) were born by caesarean section and 11(47.82%) by vaginal delivery. 10 newborns had history of resuscitation manoeuvres (43.47%). Pneumothorax was located in lefthemithorax in 8 newborn (34.78%), right hemithorax in 14 newborn (60.86%) and both hemithorax in 1 newborn (4.47%). 22 (95.65%)presented respiratory distress. Treatment included observation in 2 newborns with pneumothorax < 25%, and tube thoracostomy in 21 (91.30%) with pneumothorax > 25%. Conclusion: Pneumothorax is a complication seen mainly in newborns with low Apgar score and those who required resuscitation manoeuvres and almost always presents with respiratory distress. Pneumothorax < 25% can be treated with observation and serialradiology but those > 25%.will require tube thoracostomy.

Factores de riesgo del Síndrome Dificultad Respiratoria de origen pulmonar en el recién nacido / Risk factors of pulmonar respiratory difficulty in newborn

El Síndrome de Dificultad Respiratoria constituye una de las afecciones más frecuentes en el recién nacido y se comporta como uno de los principales indicadores de morbilidad y mortalidad. Con el objetivo de caracterizar las causas más frecuentes de dificultad respiratoria de origen pulmonar en los neonatos, se realizó un estudio retrospectivo de corte transversal, descriptivo, en un grupo de pacientes con este diagnóstico que ingresaron en la Unidad de Cuidados Especiales Neonatales (UCEN) del Hospital Ginecoosbstétrico Ramón González Coro, en el período del 1ro de enero al 31 de diciembre de 2006. Se registraron 133 neonatos con dificultades respiratorias de origen pulmonar, para una frecuencia de 4,7 por ciento. La mayor parte de los pacientes estudiados nacieron por cesárea (61,7 por ciento) y el factor de riesgo materno más frecuente fue la rotura prematura de membrana (18 por ciento). Los recién nacidos a término (55,6 por ciento) y con sexo masculino (65,4 por ciento) fueron los que mayor morbilidad presentaron. La taquipnea transitoria fue la afección respiratoria que más se diagnosticó, con un total de 92 casos para el 69,2 por ciento y la mayoría de los pacientes evolucionaron favorablemente. La enfermedad de la membrana hialina fue la entidad respiratoria que más complicaciones reportó. El índice de ventilación fue el 18,8 por ciento y la neumonía postnatal fue la complicación que más se observó con 8 casos (6,1 por ciento). La tasa de mortalidad en este grupo fue el 1,7 por cada 1000 nacidos vivos(AU)

Syndrome of Respiratory Difficulty is one of more frequent afections in newborn and behaves as one of the main indicators of morbidity and mortality. To characterize the more frequent causes of pulmonar respiratory difficulty in neonates, we made a retrospective, descriptive and cross-sectional study in a group of patients presenting with this diagnosis admitted in Unit of Neonatal Special Care of Ramn Gonzlez Coro Gynecologic and Obstetrics Hospital from January 1 to December 31 2006. There was 133 neonates with pulmonary respiratory difficulty, for a frequency of 4,7 percent. Most of study patients were born by cesarian section (61,7 percent), and the more frequent mother risk factor was premature rupture of membrane (18 per cent). Term newborns (55,6 percent) and of male sex (65,4 percent) presented the greatest morbidity. Transient tachypnea was the more diagnosed respiratory afection with a total of 92 cases (69,2 percent), and most of patients evolved favourably. Hyaline membrane disease was the entity with more complications. Ventilation rate was of 18,8 percent and postnatal pneumonia was the more observed condition with 8 cases (6,1 percent). Mortality rate in this group was of 1,7 for each 1000 live births(AU)

Repair of coarctation of the aorta in infants weighing less than 2 kilograms.

BACKGROUND: We retrospectively reviewed our experience in aortic coarctation repair on infants weighing less than 2 kg to evaluate the results and assess the rate of recoarctation in this group of patients. METHODS: Twenty-four consecutive babies weighing 2 kg or less were operated on over a period of 15 years. Median gestational age was 33 weeks (range, 30 to 36), and median weight was 1.6 kg (range, 1.0 to 2.0). Seventeen of them had associated cardiac anomalies. The methods of repair undertaken were resection with extended end-to-end anastomosis (n = 13), subclavian flap angioplasty (n = 9), carotid flap angioplasty (n = 1), and patch repair using pulmonary homograft tissue (n = 1). RESULTS: Mean follow-up was 52.5 months (range, 0.5 to 151). There were 3 in-hospital deaths and 2 late deaths. Recoarctation developed in 7 babies. Four underwent balloon dilatation; 1 of them required further surgery; 3 others have mild recoarctation, but have not required further intervention. Risk factor analyses revealed that the presence of preoperative congestive cardiac failure, and coexisting noncardiac lesions as well as the duration of descending aortic cross-clamp and postoperative ventilation had a significant influence on mortality after repair. CONCLUSIONS: Coarctation repair in infants less than 2 kg can be performed safely. The incidence of recoarctation is acceptable and comparable with that of other pediatric cohorts that have been reported. Preoperative cardiac function and associated noncardiac lesions may influence the incidence of mortality after repair. Delaying the timing of surgical repair to achieve growth is not necessary.

Estudio controlado del tratamiento de la enfermedad de membrana hialina del recién nacido pretérmino con surfactante pulmonar exógeno (porcino vs. bovino) / Treatment of hyaline membrane disease in the preterm newborn with exogenous lung surfanctant. A controlled study

Introducción: La enfermedad de membrana hialina (EMH) por deficiencia de surfactante pulmonar en el neonato prematuro es una causa importante de morbimortalidad. El surfactante pulmonar exógeno ha revolucionado el tratamiento de esta entidad en países desarrollados, aunque este beneficio ha sido menor en países en vías de desarrollo. El surfactante porcino de manufactura cubana es económico, y su uso comparado con otros surfactantes es desconocido. Material y métodos: Se llevó a cabo un estudio prospectivo, controlado, aleatorizado, abierto, en 44 recién nacidos prematuros con EMH. Un grupo recibió surfactante bovino (SB) (Survanta), y el otro surfactante porcino (SP) de fabricación cubana (Surfacen). Se evaluó la respuesta en variables de oxigenación y ventilación, días de oxígeno suplementario, ventilación mecánica, incidencia de complicaciones, tiempo de hospitalización y mortalidad. Resultados: 23 pacientes recibieron el surfactante bovino, y 21 el porcino. Los dos grupos fueron similares clínicamente y en sus patrones de respuesta de oxigenación y ventilación, con una tendencia a mayor incremento inicial en la oxigenación en el grupo tratado con SP. La incidencia de complicaciones fue similar en los dos grupos. Fallecieron 10 pacientes (47.6%) en el grupo SP, y 12 (52.2%) en el grupo SB (p>0.05). Conclusiones: El surfactante porcino tuvo efectos clínicos similares al bovino en las variables de oxigenación y ventilación estudiadas; no hubo diferencia significativa en complicaciones y mortalidad. El surfactante porcino es una alternativa efectiva y de menor costo que el surfactante bovino para el tratamiento de la EMH.

Background: Hyaline membrane disease (HMD) due to lung surfactant deficiency in the preterm newborn is an important cause of neonatal morbidity and mortality. Exogenous lung surfactant has transformed HMD therapy in developed countries, but an equivalent benefit has not been accomplished in developing countries due to a variety of factors. Porcine surfactant developed in Cuba is an inexpensive alternative to other surfactants, and its use has not been studied in our settings. Methods: A randomized, open, prospective and controlled trial was undertaken in 44 preterm newborns with HMD diagnosis. One group received bovine surfactant (BS) (Survanta) and the other Cuban porcine surfactant (PS) (Surfacen). The following clinical response variables were evaluated: oxygenation and ventilation indexes, days with supple mentary oxygen, days with mechanical ventilation, incidence of compli cations, time of hospitalization, and mortality. Results: 23 Patients received bovine surfactant and 21 the porcine type. The two groups were clinically similar, with patterns of oxygenation and ventilation response that were the same between groups, with a tendency to higher initial oxygenation increase in the PS group. The incidence of complications was similar between groups. Ten Patients (47.6%) died in the PS group, versus 12 (52.2%) in the BS group (p>0.05) Conclusions: Porcine surfactant had similar clinical effects than bovine surfactant in the oxygenation and ventilation variables, with no significant differences in complications or mortality. Porcine surfactant is an effective and lower cost alternative to bovine surfactant in the treatment of HMD.

Long versus short inspiratory times in neonates receiving mechanical ventilation.

BACKGROUND: When intermittent positive pressure ventilation (IPPV) was introduced in newborn infants with hypoxic respiratory failure from hyaline membrane disease (HMD), mortality was high and air leaks problematic. This barotrauma was caused by the high peak inspiratory pressures (PIP) required to oxygenate stiff lungs. The primary determinants of mean airway pressure (and thus oxygenation) on a conventional ventilator are the inspiratory time (IT), PIP, positive end expiratory pressure and gas flow rates. In the 1970s uncontrolled studies on a small number of infants demonstrated a benefit in reducing barotrauma using a long IT and slow rates. This strategy was subsequently widely adopted. Current neonatal ventilators have been designed to minimise lung injury but rates of bronchopulmonary dysplasia (BPD) remain high. It is therefore important that the inspiratory time causing least harm is used. OBJECTIVES: To determine in mechanically ventilated newborn infants whether the use of a long rather than a short IT reduces the rates of death, air leak and BPD. SEARCH STRATEGY: The standard search strategy of the Cochrane Neonatal Review Group (CNRG) was used. Searches of electronic and other databases were performed. These included MEDLINE (1966 - April 2004) and the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 4, 2003). In order to detect trials that may not have been published, the abstracts of the Society for Pediatric Research, and the European Society for Pediatric Research were searched from 1998 - 2003. SELECTION CRITERIA: All randomised and quasi-randomised controlled trials enrolling mechanically ventilated infants with or without respiratory pathology evaluating the use of long versus short IT (including randomised crossover studies with outcomes restricted to differences in oxygenation). DATA COLLECTION AND ANALYSIS: The standard method of the Cochrane Collaboration and its Neonatal Review Group were used. Two authors independently assessed eligibility, and the methodological quality of each trial, and extracted the data. The data were analysed using relative risk (RR) and risk difference (RD) and their 95% confidence intervals. A fixed effect model was used for meta-analyses. MAIN RESULTS: In five studies, recruiting a total of 694 infants, a long IT was associated with a significant increase in air leak [typical RR 1.56 (1.25, 1.94), RD 0.13 (0.07, 0.20), NNT 8 (5, 14)]. There was no significant difference in the incidence of BPD. Long IT was associated with an increase in mortality before hospital discharge that reached borderline statistical significance [typical RR 1.26 (1.00, 1.59), RD 0.07 (0.00, 0.13)]. REVIEWERS' CONCLUSIONS: Caution should be exercised in applying these results to modern neonatal intensive care, because the studies included in this review were conducted prior to the introduction of antenatal steroids, post natal surfactant and the use of synchronised modes of ventilatory support. Most of the participants had single pathology (HMD) and no studies examined the effects of IT on newborns ventilated for other reasons such as meconium aspiration and congenital heart disease (lungs with normal compliance). However, the increased rates of air leaks and deaths using long ITs are clinically important; thus, infants with poorly compliant lungs should be ventilated with a short IT.

Bronchoplasty for bronchial stenosis in a neonate: a case report.

Bronchial stenosis is an uncommon problem in children. Management can be difficult because of the small luminal diameter and proximity of the lung parenchyma to the bronchial lesion. Bronchoplasty procedures have obvious advantages in children because of their long life expectancy. Long-term functional results are superior compared with pneumonectomy because of preserved lung tissue. A premature neonate weighing only 779 g at birth was born with severe respiratory distress syndrome complicated with hyperbillirubinemia and bronchopulmonary dysplasia. The child underwent bronchoplasty using a costal cartilage graft for a right main bronchus stenosis after 2 failed attempts at bronchoscopic dilatations. In spite of all complications, the child improved sufficiently to be discharged without any oxygen dependency. Our case highlights the successful application of bronchoplasty in a very small baby. Preoperative and intraoperative bronchoscopy was helpful in localization of the endobronchial lesion. Understanding the principle, precise attention to technical detail and meticulous postoperative care contribute toward long-term benefit for pulmonary conservation.

The value of ultrasound examination of the lungs in predicting bronchopulmonary dysplasia.

BACKGROUND: Bronchopulmonary dysplasia (BPD) is one of the most serious complications of neonatal mechanical ventilation. Early diagnosis may influence treatment options such as early steroid administration. OBJECTIVE: To assess the role of US of the lungs in predicting the development of BPD in infants with hyaline membrane disease (HMD) and to determine the earliest possible age at which the diagnosis of BPD could be made with certainty. PATIENTS AND METHODS: Consecutive premature newborn infants requiring assisted ventilation during 1997-1998 who were admitted because of HMD were studied prospectively. US of the lungs was performed within 3 days of birth and three times a week thereafter until discharge from the neonatal intensive care unit. Each patient was also clinically and radiographically evaluated for the presence or absence of BPD at day 28 of life. Lung US scans were correlated with the development of BPD. RESULTS: Retrodiaphragmatic hyperechogenicity (RH) was initially observed in 28 of the 36 patients with HMD, but resolved completely in 24 (96%) of 25 infants who had an uncomplicated clinical course. In eight of the ten infants who developed BPD, RH persisted. Day 9 was the earliest day where persistence of abnormal RH was observed with the highest predictor values for the development of BPD. CONCLUSIONS: Lung US is a valuable technique for predicting the development of BPD.

[Maternal and neonatal factors affecting the incidence of bronchopulmonary dysplasia in very low birth weight newborns]. / Fatores maternos e neonatais na incidência de displasia broncopulmonar em recém-nascidos de muito baixo peso.

OBJECTIVE: To determine the incidence of bronchopulmonary dysplasia, to identify maternal and neonatal factors associated with the disease, and to determine the correlation between bronchopulmonary dysplasia and the progress of newborns. METHODS: Data were prospectively collected on 153 infants born in Campinas (state of São Paulo, Brazil) from September 2000 to April 2002 weighing less than 1,500 g and treated at the University Hospital. The ratio of incidence rates with 95% CI, Breslow-Cox regression, Student's t test, linear regression and the Fishers exact test were utilized. RESULTS: Among the 124 babies who survived until 28 days of age, 33 (26.6%) developed bronchopulmonary dysplasia. Birthweight < or = 1,000 g (5.6; 95% CI 3.0, 10.4) and gestational age < or = 30 weeks (4.0; 95% CI 2.1, 7.2) were correlated with increased incidence of bronchopulmonary dysplasia. Breslow-Cox regression showed that other factors including gender, Apgar score, hyaline membrane disease, antenatal steroid therapy, pregnancy-induced hypertension, delivery route and maternal age were not associated with bronchopulmonary dysplasia. Mean duration of hospitalization and ventilator therapy in newborns with and without bronchopulmonary dysplasia was 78.8 days (SD = 26.67) vs. 43.0 days (SD = 14.49) (p < 0.01) and 27.2 days (SD = 21.26) vs. 3.7 days (SD = 3.02) (p < 0.01), respectively. Mean weight gain per day was lower in newborns with bronchopulmonary dysplasia (p < 0.01). Mortality in newborns with bronchopulmonary dysplasia was 21% (p < 0.00005). CONCLUSION: Gestational age and birthweight were inversely proportional to incidence of bronchopulmonary dysplasia. After the onset of bronchopulmonary dysplasia, newborns with the disorder required longer periods of ventilator therapy and hospitalization, and presented inadequate weight gain and higher mortality rates than newborns without bronchopulmonary dysplasia.

Fatores maternos e neonatais na incidência de displasia broncopulmonar em recém-nascidos de muito baixo peso / Maternal and neonatal factors affecting the incidence of bronchopulmonary dysplasia in very low birth weight newborns

OBJETIVO: Obter a incidência de displasia broncopulmonar (DBP); avaliar os fatores maternos e neonatais associados com a doença; determinar a correlaçäo entre DBP e a evoluçäo dos recém-nascidos. MÉTODOS: Os dados foram coletados prospectivamente de 153 recém-nascidos com peso de nascimento inferior a 1.500 g, nascidos em Campinas de setembro de 2000 a abril de 2002 e tratados no Hospital Universitário. Foram utilizados razäo de taxas de incidências com intervalo de confiança de 95 por cento (IC 95 por cento), regressäo Breslow-Cox, teste t de Student, regressäo linear e teste exato de Fisher. RESULTADOS: Entre os 124 recém-nascidos que sobreviveram aos 28 dias de vida, 33 (26,6 por cento) apresentavam DBP. Peso de nascimento < 1.000 g (5,6; IC 95 por cento 3,0; 10,4) e idade gestacional < 30 semanas (4,0; IC 95 por cento 2,1; 7,2) foram correlacionados com um aumento na incidência de DBP. A regressäo de Breslow-Cox mostrou que outros fatores, incluindo sexo, índice de Apgar, doença da membrana hialina (DMH), uso de corticóide pré-natal, doença hipertensiva específica da gravidez (DHEG), tipo de parto e idade materna näo foram associados com DBP. As médias dos dias de internaçäo e de ventilaçäo mecânica nos recém-nascidos com e sem DBP foram, respectivamente, 78,8 dias (DP = 26,67) contra 43,0 dias (DP = 14,49) (p < 0,01) e 27,2 dias (DP = 21,26) contra 3,7 dias (DP = 3,02) (p < 0,01). A média de ganho de peso por dia foi menor nos recém-nascidos com DBP (p < 0,01). A mortalidade para recém-nascidos com DBP foi de 21 por cento (p < 0,00005). CONCLUSÕES: A idade gestacional e o peso de nascimento foram fatores inversamente proporcionais à incidência de DBP. Uma vez desenvolvida a doença, os recém-nascidos necessitam maiores tempos de suporte ventilatório e de internaçäo, apresentando inadequado ganho de peso e maior mortalidade quando comparados aos recém-nascidos sem DBP

Life threatening unilateral pulmonary overinflation might be more successfully treated by contralateral selective intubation than by emergency pneumonectomy.

During a period of 3 years, three infants were admitted to our hospital for unilateral emergency pneumonectomy due to life threatening overinflation of one lung, preventing adequate ventilation of the unaffected contralateral side. All three patients were able to be stabilized by unilateral selective bronchial intubation of the unaffected lung after bronchoscopy, ruling out a flap valve mechanism. No emergency pneumonectomies were required. In one patient, lung function of the overinflated side (three lobes) recovered fully and, in the two remaining patients, one lobe of the overinflated side recovered. The overinflated lobes were removed later by elective surgery, thus not exposing the children to a potentially dangerous emergency operation. These results are in contrast with reports in the literature. Emergency pneumonectomy in neonates and infants due to overinflation of one lung may be avoided by selective unilateral intubation of the main stem bronchus of the compressed lung.

Potter sequence complicated by congenital cystic lesion of the bladder.

We report a case complicated by oligohydramnios, pulmonary hypoplasia, bilateral renal dysplasia, and cystic lesion of the bladder. He was clinically compatible with Potter sequence. Congenital cystic bladder is the rarest form of the bladder. We can find no report of Potter sequence complicated by cystic lesion of the bladder. This lesion was similar to multilocular bladder. The diagnosis was confirmed it by autopsy, magnetic resonance imaging, and urography after his death.

Effects of birthweight and oxygen supplementation on lung function in late childhood in children of very low birth weight.

Impaired respiratory function has been found frequently in ex-premature children, but it is unclear which specific factors influence this impairment the most. The aim of this study was to determine the importance of the contributions of birth weight, gestational age, neonatal respiratory disease, and its treatment on subsequent childhood lung function at age 11 years in a cohort of children of very low birth weight (VLBW; 2,000 g) of similar age. VLBW children were shorter and lighter than controls (P < 0.0001) at 11 years of age, and had reduced expiratory flows (P < 0.00001) and forced vital capacities (P < 0.001). The residual volume to total lung capacity ratio (RV/TLC ratio) was increased (P < 0.00001), while total lung capacity (TLC) remained unchanged. Those with bronchopulmonary dysplasia (BPD) had the lowest mean expiratory flows. Males had lower expiratory flows than females. On univariate analysis, gestational age by itself accounted for 8.8% of the explained variance in FEV(1) at 11 years of age, but birth weight accounted for 16% on its own; both together accounted for a further 0.2% (16.2%), suggesting that the latter was the dominant factor. On multivariate analysis, the contribution of birth weight and gestational age was small, and the best predictors at 11 years of age, which together explained 43.4% of the total variance in FEV(1), were log days of supplemental oxygen (9.6%) and a reported history of asthma (10.8%). For FEF(25-75), these predictors explained 7.2% and 13.4%, respectively, of the total explained variance of 40.6%. The relation between neonatal oxygen supplementation and childhood FEV(1) was such that up to 20 days of supplemental oxygen had little effect on subsequent FEV(1) at 11 years of age, but each additional week of supplemental oxygen after that time was associated with a progressive reduction in FEV(1) of 3%. These data confirm the significant role of supplemental oxygen in the neonatal period and a history of asthma on the subsequent reduction of expiratory flows in VLBW children. Birth weight was a more important prenatal factor than gestational age, but both were of lesser predictive significance than either supplemental oxygen or a reported history of asthma.

Estudo comparativo entre recem-nascidos com doença da membrana hialina, tratados com doses altas e doses baixas de um surfactante natural bovino, e correlacionado com tempo de inicio da terapeutica / Comparative study between newborns with hyaline membrane disease treated with high doses and low doses of natural bovine surfactant and correlating time and beginning of therapy

O autor realizou estudo comparativo do uso de doses baixas (100mg/kg) e doses altas (200mg/kg) do surfactante natural bovino adicionado, correlacionando-o, com o tempo de inicio da terapeutica no tratamento da doenca da membrana hialina em 124 recem-nascidos. Desse total de recem-nascidos, 77 foram tratados com tres horas ou menos de vida (nascidos no proprio servico) e 47 tratados com mais de tres horas de vida (removidos de outros servicos); dos 77 recem-nascidos tratados com tres horas ou menos de vida, 59...

Comparison of four methods for measuring elevation of FRC in mechanically ventilated infants.

The aim of the study was to compare measurements of the elevation of functional residual capacity (FRC) above the relaxation volume obtained in 34 mechanically ventilated infants (median weight 2.6 kg, range 1.2-9) from four different methods: (1) direct measurement of the complete exhalation volume after brief disconnection from the ventilator, (2) calculated measurement from total positive end-expiratory pressure (PEEP) measured by end-expiratory occlusion of the breathing circuit, (3) extrapolated evaluation from the mathematical model of Brody, (4) extrapolated evaluation from the passive expiration method. We considered the direct measurement (1) as the "gold standard". Measurements obtained by total PEEP (2) and by the Brody's mathematical model (3) provided similar results than the direct measurement. Conversely, graphical extrapolation from the passive expiration method (4) underestimated the elevation of FRC. In conclusion, we suggest using the mathematical extrapolation from the Brody's model to evaluate the elevation of FRC in mechanically ventilated infants: this method is non-invasive, does not require disruption of gas flow, can be easily performed with all the neonatal ventilators, and allows continuous breath-by-breath measurements.

Discinesia coreiforme asociada a broncodisplasia pulmonar / Bronchopulmonary dysplasia associated chorea like motor disorder. A case report

Introducción. La broncodisplasia pulmonar es una complicación frecuente del recién nacido prematuro que desarrolla enfermedad de membrana hialina; su repercusión sobre el neurodesarrollo incluye: alteraciones motoras, retardo en la adquisición de habilidades lingüísticas y microcefalia; recientemente se ha relacionado con el desarrollo de movimientos involuntarios de tipo coreico y temblor de origen extrapiramidal durante el primer año de vida. Caso clínico. Se reporta un caso típico de discinesia con movimientos coreicos, temblor, postura distónica y dificultades en la succión y deglución asociadas a disquinesia oro-buco-lingual que se desarrolló en los primeros 3 meses de vida en un paciente con broncodisplasia pulmonar y dependencia de suplementos de oxígeno inhalado. Conclusión. Se hace hincapié en el diagnóstico diferencial de los trastornos de la succión y deglución, los hallazgos video-electroencefalográficos y la necesidad de reconocer correctamente el síndrome, así como su tratamiento farmacológico