RESUMO
64-Year-old male patient with hepatocellular carcinoma (HCC), liver cirrhosis, chronic hepatitis C infection, and glottic cancer presented with acute progressive abdominal pain and palpable mass in right upper quadrant of the abdomen. Despite treatment with hyoscine and tramadol, the symptoms persisted. He had received three sessions of Transcatheter arterial chemoembolization (TACE) and two sessions of microwave ablation (MWA) for HCC treatment, with the last session being TACE 11 months prior. Plain film abdomen showed bowel gas pattern in the right hemithorax compatible with bowel obstruction. CT imaging revealed a right diaphragmatic hernia containing closed loop small bowel obstruction. An emergency exploratory laparotomy was performed. The patient improved and was discharged. There was no recurrence of diaphragmatic hernia or abdominal mass or pain at the 6-month follow-up visit. We herein demonstrate a catastrophic complication of TACE by using an IPA and MWA which leads to right diaphragmatic hernia.
Assuntos
Carcinoma Hepatocelular , Quimioembolização Terapêutica , Hérnia Diafragmática , Neoplasias Hepáticas , Humanos , Masculino , Carcinoma Hepatocelular/cirurgia , Quimioembolização Terapêutica/efeitos adversos , Quimioembolização Terapêutica/métodos , Hérnia Diafragmática/diagnóstico por imagem , Hérnia Diafragmática/etiologia , Hérnia Diafragmática/terapia , Neoplasias Hepáticas/patologia , Micro-Ondas/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento , Pessoa de Meia-IdadeAssuntos
Humanos , Masculino , Idoso , Hérnia Diafragmática/terapia , Hérnia Diafragmática/diagnóstico por imagem , Período Pós-Operatório , Esofagostomia/efeitos adversos , Colostomia/efeitos adversos , Tomografia Computadorizada por Raios X , Procedimentos de Cirurgia Plástica , Gastrectomia/efeitos adversosRESUMO
Spondylothoracic dysostosis is a rare congenital disorder characterised by multiple vertebral malformations, shortening of the spine and fusion of the ribs at the costovertebral junction. These abnormalities create anaesthetic challenges due to difficult airway, severe restrictive lung disease and spine deformity necessitating a multidisciplinary approach and careful perioperative planning. We present the perianaesthetic management of a parturient with spondylothoracic dysostosis who successfully underwent preterm caesarean delivery under general anaesthesia with awake videolaryngoscopy-assisted tracheal intubation.
Assuntos
Anormalidades Múltiplas/terapia , Anestesia Obstétrica , Hérnia Diafragmática/terapia , Complicações na Gravidez/terapia , Costelas/anormalidades , Coluna Vertebral/anormalidades , Adulto , Cesárea , Feminino , Humanos , Intubação Intratraqueal , Laringoscópios , GravidezRESUMO
A 24-year-old woman presented to neurosurgical consultation for chronic back pain. The patient was long term in wheelchair for vertebral deformity. She was the third child of first-degree consanguineous parents. The 2 older brothers had also vertebral malformations. The radiological images showed butterfly vertebra, vertebral fusion, hemivertebrae, scoliosis, and rib malformation. The patient was in follow-up for restrictive lung disease. Motor evoked potentials and lower limb electromyography were normal. We recommended conservative treatment for the back pain with antalgic and physical therapy. Diagnosis of spondylocostal dysostosis, or Jarcho-Levin syndrome, was made based on radiological features. Radiological mages are pathognomonic. Spondylocostal dysostosis is a rare hereditary disorder associated with multiple vertebral and rib anomalies. The entity is distinct from spondylothoracic dysostosis, which has a higher mortality due to respiratory complications. The patient was not compliant for genetic familiar counseling. At 12-year follow-up, the patient was in periodic respiratory and motor rehabilitation therapy.
Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Hérnia Diafragmática/diagnóstico por imagem , Coluna Vertebral/diagnóstico por imagem , Anormalidades Múltiplas/terapia , Dor nas Costas/diagnóstico por imagem , Dor nas Costas/terapia , Dor Crônica/diagnóstico por imagem , Dor Crônica/terapia , Consanguinidade , Feminino , Hérnia Diafragmática/terapia , Humanos , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
BACKGROUND: ADH is a rare and potentially fatal complication following LT. In this study, a systematic review was completed to identify risk factors which may contribute to ADH. METHODS: Transplant databases at three LT programs were reviewed. Four pediatric and zero adult cases were identified. Next, a systematic review was completed. Fourteen studies describing 41 patients with ADH were identified. Patient demographics, transplant characteristics, and features of ADH diagnosis were examined. RESULTS: The majority (90.2%) of ADH were in children. In pediatric LT, 95.1% received a segmental allograft. ADH occurred in the right P diaphragm 92.7% of the time, and 87.8% were repaired primarily. Patient demographics, post-transplant complications, and immunosuppression regimens were broad and failed to predict ADH. Most patients presented with either respiratory or gastrointestinal symptoms. There were two pediatric deaths related to undiagnosed ADH. The combined worldwide incidence of ADH in pediatric LT is 1.5% (34/2319 patients). CONCLUSION: ADH is a rare complication post-LT that primarily occurs in pediatric recipients. When diagnosed early, ADH can be repaired primarily with good outcomes.
Assuntos
Hérnia Diafragmática/diagnóstico , Hérnia Diafragmática/terapia , Falência Hepática/cirurgia , Transplante de Fígado/efeitos adversos , Adolescente , Adulto , Aloenxertos , Criança , Pré-Escolar , Feminino , Hérnia Diafragmática/complicações , Humanos , Incidência , Pulmão/patologia , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico , Fatores de Risco , Adulto JovemRESUMO
BACKGROUND: Maternal diaphragmatic hernias identified during pregnancy are rare and pose significant management challenges with regards to timing and mode of both delivery and hernia repair. CASE PRESENTATION: We describe a case of a maternal diaphragmatic hernia diagnosed at 31 weeks gestation in the setting of acute upper abdominal pain. Due to no evidence of visceral compromise and a stable maternal condition, the patient was conservatively managed, allowing for further foetal maturation. Delivery by caesarean section occurred following concerns of malnutrition and partial bowel obstruction. This was followed by immediate surgical repair of the hernia. The patient had an uncomplicated recovery. CONCLUSION: Maternal diaphragmatic hernias in pregnancy require multidisciplinary care and individualised management in order to allow for the optimal outcome for mother and foetus.
Assuntos
Tratamento Conservador , Hérnia Diafragmática/terapia , Complicações na Gravidez/terapia , Dor Abdominal/etiologia , Adulto , Cesárea , Feminino , Hérnia Diafragmática/cirurgia , Herniorrafia , Humanos , Gravidez , Complicações na Gravidez/cirurgiaRESUMO
INTRODUCTION: Lung hypoplasia and pulmonary arterial hypertension in congenital diaphragmatic hernia lead to a high perinatal mortality. Although sustained fetoscopic tracheal occlusion (TO) improves lung development, a major side effect is abnormal pneumocyte differentiation. This study evaluated the potential ability of intratracheal retinoic acid (RA) administration to reduce adverse effects of sustained TO in a rabbit model of diaphragmatic hernia. METHODS: A left diaphragmatic defect was created on day 23 in time-dated pregnant rabbits. On day 28, the same rabbits underwent sham surgery or TO, with an injection of empty or RA-loaded liposomes. On day 30, the fetuses were harvested, and the lungs were processed for histology, immunohistochemistry, and gene expression quantification. RESULTS: A tracheal RA injection at the time of TO had no effect on the lung-to-body-weight ratio, radial alveolar count or lung connective tissue composition. Retinoic acid plus TO had synergic effects on vascular measurements, proportional medial thickness, and endothelin-1 receptor type-A gene expression. The most noticeable effect was recovery of normal pneumocyte differentiation. CONCLUSION: Retinoic acid plus TO prevented abnormal pneumocyte differentiation and seemed to have a beneficial effect on pulmonary vascularization.
Assuntos
Antineoplásicos/administração & dosagem , Doenças Fetais/cirurgia , Hérnia Diafragmática/terapia , Pulmão/efeitos dos fármacos , Traqueia/cirurgia , Tretinoína/administração & dosagem , Animais , Apoptose/efeitos dos fármacos , Colágeno/metabolismo , Elastina/metabolismo , Feminino , Fetoscopia , Pulmão/embriologia , Pulmão/metabolismo , Gravidez , Surfactantes Pulmonares/metabolismo , CoelhosRESUMO
Extracorporeal membrane oxygenation (ECMO), a life-saving therapy for respiratory and cardiac failure, was first used in neonates in the 1970s. The indications and criteria for ECMO have changed over the years, but it continues to be an important option for those who have failed other medical therapies. Since the Extracorporeal Life Support Organization (ELSO) Registry was established in 1989, more than 29,900 neonates have been placed on ECMO for respiratory failure, with 84% surviving their ECMO course, and 73% surviving to discharge or transfer. In this chapter, we will review the basics of ECMO, patient characteristics and criteria, patient management, ECMO complications, special uses of neonatal ECMO, and patient outcomes.
Assuntos
Oxigenação por Membrana Extracorpórea , Hérnia Diafragmática/terapia , Síndrome de Aspiração de Mecônio/terapia , Síndrome do Desconforto Respiratório do Recém-Nascido/terapia , Sepse/terapia , Medicina Baseada em Evidências , Oxigenação por Membrana Extracorpórea/métodos , Oxigenação por Membrana Extracorpórea/mortalidade , Hérnia Diafragmática/mortalidade , Humanos , Recém-Nascido , Síndrome de Aspiração de Mecônio/mortalidade , Guias de Prática Clínica como Assunto , Ensaios Clínicos Controlados Aleatórios como Assunto , Síndrome do Desconforto Respiratório do Recém-Nascido/mortalidade , Sepse/mortalidade , Taxa de SobrevidaRESUMO
Jarcho-Levin syndrome (JLS) is a genetic disorder characterized by distinct malformations of the ribs and vertebrae, and/or other associated abnormalities such as neural tube defect, Arnold-Chiari malformation, renal and urinary abnormalities, hydrocephalus, congenital cardiac abnormalities, and extremity malformations. The study included 12 cases at 37-42 weeks of gestation and diagnosed to have had Jarcho-Levin syndrome, Arnold-Chiari malformation, and meningmyelocele. All cases of Jarcho-Levin syndrome had Arnold-Chiari type 2 malformation; there was corpus callosum dysgenesis in 6, lumbosacral meningmyelocele in 6, lumbal meningmyelocele in 3, thoracal meningmyelocele in 3, and holoprosencephaly in 1 of the cases. With this article, the authors underline the neurologic abnormalities accompanying Jarcho-Levin syndrome and that each of these abnormalities is a component of Jarcho-Levin syndrome.
Assuntos
Malformação de Arnold-Chiari/complicações , Hérnia Diafragmática/complicações , Defeitos do Tubo Neural/complicações , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/terapia , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Feminino , Hérnia Diafragmática/diagnóstico por imagem , Hérnia Diafragmática/terapia , Humanos , Recém-Nascido , Masculino , Defeitos do Tubo Neural/diagnóstico por imagem , Defeitos do Tubo Neural/cirurgia , Tomografia Computadorizada por Raios XRESUMO
INTRODUCTION: Patients with congenital diaphragmatic hernias (CDH), omphaloceles, and congenital lung malformations (CLM) may have pulmonary hypoplasia and experience respiratory insufficiency. We hypothesize that given equivalent lung volumes, the degree of respiratory insufficiency will be comparable regardless of the etiology. METHODS: Records of all fetuses with CDH, omphalocele, and CLM between January 2000 and June 2013 were reviewed. MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV) were calculated. An analysis of outcomes in patients with O/E-TFLV between 40% and 60%, the most inclusive range, was performed. RESULTS: 285 patients were evaluated (161, CDH; 24, omphalocele; 100, CLM). Fetuses with CDH had the smallest mean O/E-TFLV. CDH patients were intubated for longer and had a higher incidence of pulmonary hypertension. Fifty-six patients with the three diagnoses had an O/E-TFLV of 40%-60%. The need for ECMO, supplemental oxygen at 30days of life, and 6-month mortality were similar among groups. CDH patients had a significantly longer duration of intubation and higher incidence of pulmonary hypertension than the other two diagnoses. CONCLUSION: Given equivalent lung volumes (40%-60% of expected), CDH patients require more pulmonary support initially than omphalocele and CLM patients. In addition to lung volumes, disease-specific factors, such as pulmonary hypertension in CDH, also contribute to pulmonary morbidity and overall outcome.
Assuntos
Hérnia Diafragmática/diagnóstico , Hérnia Umbilical/diagnóstico , Hérnias Diafragmáticas Congênitas/diagnóstico , Medidas de Volume Pulmonar , Oxigenação por Membrana Extracorpórea , Hérnia Diafragmática/complicações , Hérnia Diafragmática/terapia , Hérnia Umbilical/complicações , Hérnia Umbilical/terapia , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Hipertensão Pulmonar/etiologia , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Prognóstico , Terapia Respiratória , Estudos RetrospectivosRESUMO
The chest is a dynamic structure. For normal movement it relies on a coordinated movement of the multiple bones, joints and muscles of the respiratory system. While muscle weakness can have clear impact on respiration by decreasing respiratory motion, so can conditions that cause chest wall hypoplasia and produce an immobile chest wall. These conditions, such as Jarcho-Levin and Jeune syndrome, present significantly different challenges than those faced with early onset scoliosis in which chest wall mechanics and thoracic volume may be much closer to normal. Because of this difference more aggressive approaches to clinical and surgical management are necessary.
Assuntos
Anormalidades Múltiplas/diagnóstico , Síndrome de Ellis-Van Creveld/diagnóstico , Hérnia Diafragmática/diagnóstico , Transtornos Respiratórios/diagnóstico , Parede Torácica/anormalidades , Anormalidades Múltiplas/terapia , Síndrome de Ellis-Van Creveld/complicações , Síndrome de Ellis-Van Creveld/terapia , Hérnia Diafragmática/complicações , Hérnia Diafragmática/terapia , Humanos , Respiração , Transtornos Respiratórios/etiologiaRESUMO
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. METHODS: Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm. An integer-based clinical prediction rule was created. The performance of the model was validated by using the remaining data in terms of calibration and discrimination. RESULTS: The final model included the following predictors: very low birth weight, absent or low 5-minute Apgar score, presence of chromosomal or major cardiac anomaly, and suprasystemic pulmonary hypertension. This model discriminated between a population at high risk of death (â¼50%) intermediate risk (â¼20%), or low risk (<10%). The model performed well, with a C statistic of 0.806 in the derivation set and 0.769 in the validation set and good calibration (Hosmer-Lemeshow test, P = .2). CONCLUSIONS: A simple, generalizable scoring system was developed for CDH that can be calculated rapidly at the bedside. Using this model, intermediate- and high-risk infants could be selected for transfer to high-volume centers while infants at highest risk could be considered for advanced medical therapies.
Assuntos
Técnicas de Apoio para a Decisão , Hérnias Diafragmáticas Congênitas , Índice de Apgar , Comorbidade , Feminino , Cardiopatias Congênitas/epidemiologia , Hérnia Diafragmática/epidemiologia , Hérnia Diafragmática/mortalidade , Hérnia Diafragmática/terapia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/epidemiologia , Recém-Nascido , Masculino , Medição de Risco , Índice de Gravidade de Doença , UltrassonografiaRESUMO
OBJECTIVES: Congenital diaphragmatic hernia (CDH) survivors often have gastrointestinal disorders, and limited outcome data exist. This study reviewed risk factors for low weight and use of feeding tubes after CDH repair at 1 year of age. The hypothesis was that patients who required extracorporeal membrane oxygenation (ECMO) or with significant pulmonary arterial hypertension (PAH) would have lower weight percentiles and more often require tube feedings. METHODS: A retrospective chart review of 89 infants with CDH diagnosed in the neonatal period was conducted. Statistical analysis was performed using χ test, Fisher exact test, and logistic regression. RESULTS: Seventy-two patients (81%) survived to 1 year of age. Of these patients, 35% were <5th percentile for weight and 18% were receiving tube feedings. Both ECMO and PAH were associated with an increased risk for requiring tube feedings at 1 year of age, with respective odds ratios of 6.00 (P = 0.01) and 15.75 (P = 0.02); however, neither ECMO nor PAH was associated with low weight at 1 year of age. No statistical correlation was found between an abnormal pH probe and tube feedings at 1 year of age. CONCLUSIONS: Patients with CDH are at increased risk for having a weight <5th percentile at 1 year of age. A history of ECMO and PAH are independently associated with an increased requirement for tube feedings at 1 year of age. Close nutritional monitoring and counseling should be considered in all of the patients with CDH, particularly those with a history of ECMO or PAH.
Assuntos
Nutrição Enteral , Transtornos do Crescimento/etiologia , Hérnia Diafragmática/complicações , Hérnias Diafragmáticas Congênitas/complicações , Magreza/etiologia , Oxigenação por Membrana Extracorpórea , Feminino , Hérnia Diafragmática/terapia , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Lactente , Modelos Logísticos , Masculino , Razão de Chances , Prevalência , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Magreza/epidemiologiaRESUMO
BACKGROUND: Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns and, despite improved outcomes with multimodality therapies, optimal timing of repair remains undefined. We sought to evaluate the influence of surgical timing on patient outcomes and hypothesized that delayed repair does not improve survival in CDH. METHODS: Prospectively collected data from 1,385 CDH Registry infants without preoperative extracorporeal membrane oxygen therapy (ECMO) were evaluated. Patients were stratified by timing of repair: Day of life (DOL) 0-3 (group 1), 4-7 (group 2), or >8 (group 3), and the effect of surgical timing on mortality was determined by logistic regression and risk-adjusted for severity of illness. RESULTS: The unadjusted odds ratio (OR) for mortality increased significantly with delayed repair (group 2, 1.73 [95% CI, 1.00-2.98; group 3, 3.42 [95% CI, 1.97-5.96]). However, when adjusted for severity of illness, delay in repair did not predict increased mortality (group 2, 1.2 [95% CI, 0.7-2.2]; group 3, 1.4 [95% CI, 0.8-2.6]), nor did it portend an increased need for postoperative ECMO (group 2, 1.1 [95% CI, 0.5-2.4]; group 3, 0.5 [95% CI, 0.2-1.4]). CONCLUSION: After adjustment for known risk factors, the timing of CDH repair in low-risk infants does not seem to influence mortality. However, specific clinical parameters guiding timing of elective CDH repair remain unknown.
Assuntos
Hérnias Diafragmáticas Congênitas , Herniorrafia/métodos , Oxigenação por Membrana Extracorpórea , Hérnia Diafragmática/patologia , Hérnia Diafragmática/cirurgia , Hérnia Diafragmática/terapia , Herniorrafia/efeitos adversos , Herniorrafia/mortalidade , Humanos , Mortalidade Infantil , Recém-Nascido , Estudos Prospectivos , Sistema de Registros , Fatores de Risco , Índice de Gravidade de Doença , Texas/epidemiologia , Fatores de Tempo , Resultado do TratamentoAssuntos
Constrição Patológica/diagnóstico por imagem , Constrição Patológica/terapia , Hérnia Diafragmática/diagnóstico por imagem , Hérnia Diafragmática/terapia , Intubação Intratraqueal/instrumentação , Intubação Intratraqueal/métodos , Traqueia/anormalidades , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Radiografia , Traqueia/diagnóstico por imagemRESUMO
OBJECTIVE: To measure and evaluate the effects of tracheal dimensions on survival and ventilation in a large series of infants with congenital diaphragmatic hernia (CDH) treated antenatally with fetal endoluminal tracheal occlusion (FETO). STUDY DESIGN: Tracheal dimensions on chest radiograph (CR) were measured by 2 blinded radiologists. Survival, day 1 best oxygenation index and duration of ventilation, continuous positive airway pressure, and hospital stay were recorded. Survivors with a minimum 12-month follow-up were longitudinally compared for incidence of gastroesophageal reflux, chest infections, chest deformities, and hernia recurrence. RESULTS: Seventy infants with CDH (41 who underwent FETO) were treated between 2004 and 2010. Hernia repair was performed in 26 infants without FETO (8 with patch repair) and 35 infants with FETO (26 with patch repair; P = .0015). Infants with FETO had a wider trachea than those without FETO at T1 (P < .0001) and between T1 and the carina (P < .0001). Tracheal diameter was similar in survivors and nonsurvivors in the FETO group. Tracheal size was not correlated with day 1 best oxygenation index in the FETO group (R2 = 0.17) or the non-FETO group (R2 = 0.07). There were no between-group differences in duration of mechanical ventilation (P = .30), continuous positive airway pressure (P = .20), or hospital stay (P = .30). In the longitudinal study, tracheal widths were larger on the last CR than on preoperative CR in patients without FETO (T1, P = .02; widest point, P = .001; carina, P = .0001), and for patients with FETO at the widest point (P < .0001) and at the carina (P < .0001), but not at T1 (P = .12). There were no differences in clinical variables between the FETO and non-FETO groups. CONCLUSION: FETO has a significant impact on tracheal size of infants with CDH; however, tracheal size does not affect survival or the requirement for early respiratory support.
Assuntos
Oclusão com Balão/efeitos adversos , Fetoscopia/efeitos adversos , Hérnias Diafragmáticas Congênitas , Complicações na Gravidez/terapia , Resultado da Gravidez , Traqueia/fisiopatologia , Obstrução das Vias Respiratórias/diagnóstico , Obstrução das Vias Respiratórias/etiologia , Oclusão com Balão/métodos , Estudos Transversais , Feminino , Fetoscopia/métodos , Seguimentos , Hérnia Diafragmática/diagnóstico por imagem , Hérnia Diafragmática/terapia , Humanos , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Gravidez , Complicações na Gravidez/diagnóstico , Índice de Gravidade de Doença , Estatísticas não Paramétricas , Traqueia/diagnóstico por imagem , Resultado do Tratamento , Ultrassonografia Pré-Natal/métodosRESUMO
Congenital diaphragmatic hernia (CDH) is a congenital defect in the diaphragm that allows herniation of abdominal contents into the fetal chest and leads to varying degrees of pulmonary hypoplasia and pulmonary hypertension. Advances in prenatal diagnosis and the institution of standardized delivery and postnatal care protocols have led to improved survival. Fetal endoscopic tracheal occlusion shows early promise for patients with the most severe CDH, but prospective randomized data is still required. CDH survivors have a variety of associated morbidities that require long-term follow-up and early intervention strategies for optimal care.
Assuntos
Diafragma/cirurgia , Oxigenação por Membrana Extracorpórea/métodos , Fetoscopia/métodos , Hérnias Diafragmáticas Congênitas , Herniorrafia/métodos , Traqueia/cirurgia , Anormalidades Múltiplas/etiologia , Anormalidades Múltiplas/terapia , Feminino , Hérnia Diafragmática/complicações , Hérnia Diafragmática/diagnóstico por imagem , Hérnia Diafragmática/terapia , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Recém-Nascido , Pulmão/anormalidades , Pneumopatias/etiologia , Pneumopatias/terapia , Gravidez , Resultado do Tratamento , Ultrassonografia Pré-NatalRESUMO
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a frequently lethal birth defect and, despite advances, extracorporeal life support (ie, extracorporeal membrane oxygenation [ECMO]) is commonly required for severely affected patients. Published data suggest that CDH survival after 2 weeks on ECMO is poor. Many centers limit duration of ECMO support. STUDY DESIGN: We conducted a single-institution retrospective review of 19 years of CDH patients treated with ECMO, designed to evaluate which factors affect survival and duration of ECMO and define how long patients should be supported. RESULTS: Of two hundred and forty consecutive CDH patients without lethal associated anomalies, 96 were treated with ECMO and 72 (75%) survived. Eighty required a single run of ECMO and 65 survived (81%), 16 required a second ECMO run and 7 survived (44%). Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors. Apgar score at 1 minute, Apgar score at 5 minutes, and Congenital Diaphragmatic Hernia Study Group predicted survival all correlated with survival on ECMO, need for second ECMO, and duration of ECMO. Lung-to-head ratio also correlated with duration of ECMO. All survivors were discharged breathing spontaneously with no support other than nasal cannula oxygen if needed. CONCLUSIONS: In patients with severe CDH, improvement in pulmonary function sufficient to wean from ECMO can take 4 weeks or longer, and might require a second ECMO run. Pulmonary outcomes in these CDH patients can still be excellent, and the assignment of arbitrary ECMO treatment durations <4 weeks should be avoided.
Assuntos
Oxigenação por Membrana Extracorpórea , Hérnias Diafragmáticas Congênitas , Terapia Combinada , Técnicas de Apoio para a Decisão , Hérnia Diafragmática/mortalidade , Hérnia Diafragmática/cirurgia , Hérnia Diafragmática/terapia , Herniorrafia , Humanos , Recém-Nascido , Modelos Logísticos , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do TratamentoRESUMO
Extra corporeal membrane oxygenation (ECMO) is a form of life support, which facilitates gas exchange outside the body via an oxygenator and a centrifugal pumping system. A paediatric cardiac ECMO programme was established in 2005 at Our Lady's Children's Hospital, Crumlin (OLCHC) and to date 75 patients have received ECMO, the majority being post operative cardiac patients. The outcome data compares favourably with international figures. ECMO has been most successful in the treatment of newborn infants with life threatening respiratory failure from conditions such as meconium aspiration, respiratory distress syndrome and respiratory infections. There is no formal paediatric respiratory ECMO programme at OLCHC, or anywhere else in Ireland. Currently, neonates requiring respiratory ECMO are transferred to centres in Sweden or the UK at an average cost of 133,000 Euros/infant, funded by the Health Service Executive E112 treatment abroad scheme. There is considerable morbidity associated with the transfer of critically ill infants, as well as significant psycho-social impact on families. OLCHC is not funded to provide respiratory ECMO, although the equipment and expertise required are similar to cardiac ECMO and are currently in place. The average cost of an ECMO run at OLCHC is 65,000 Euros. There is now a strong argument for a fully funded single national cardiac and respiratory paediatric ECMO centre, similar to that for adult patients.