Correction of congenital diaphragmatic hernia in utero, V. Initial clinical experience

Harrison, Michael R.; Langer, Jacob C.; Adzick, N. Scott; Golbus, Mitchell S.; Filly, Roy A.; Anderson, Robert L.; Rosen, Mark A.; Goldstein, Ruth B.; deLorimier, Alfred A.

doi:10.1016/s0022-3468(05)80163-0

Cited by 238 publications

(86 citation statements)

References 25 publications

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“…To avoid the postnatal consequences of fetal lung hypoplasia in humans, atte mpts have been made to surgically treat this condition in utero, before the lungs are required for gas exchange. However, these treatments have principally foc used on correcting the und erlying abnormality rather than attempting to acce lerate lung growth directly (8,9). As increased fe tal lung expansion, resulting from tracheal obstruction, is a potent and rapid way of accelerating fetal lung gro wth (2-4), we considered it possible tha t trac hea l obstruction may rapi dly reve rse an exi sting lung growth defici t in utero.…”

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confidence: 99%

Lung Hypoplasia Can Be Reversed by Short-Term Obstruction of the Trachea in Fetal Sheep

1995

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i\HSTR,A T Vol. 38, No.5, 1995 Primed ill U.S.A.Our aim was 10 determine whether an existing lung growth deficit could be reversed, in utero, by short-term (6 d) obstruction of the fetal trachea. Chronically catheterized fetal sheep (term -145 d) were divided into four groups: 1) no treatment (control); 2) continuous lung liquid drainage to induce lung hypoplasia Th roughout mos t of gestation the lungs are filled with a liquid that is secreted across the pulmonary epithelium and leaves the lungs via the trachea. It is well established that the vo lume of liqu id retained wit hin the future airways is critical for normal lung growth and deve lopment. Obstruct ion of the feta l trach ea, wh ich causes secreted lung liquid to accumulate in the future airways resulting in increased expansion of the lungs (1) , greatly increases lung growth (2, 3). Conversely, prolonged drainage of feta l lung liqu id to the exterior causes lung deflation and a near cessation of lung grow th (2, 3). We have recently show n that the increase in lung growth induced by tracheal obstruction is ver y rapi d and occurs within 7 d of blocking the feta l trachea (4). Such results have led to the suggestion that short-term per iods of tracheal obstruct ion ma y rapi dly reverse an existing lung growth deficit in utero (4, 5).Inadequ ate lung growth during feta l life res ults in pul monary hypoplasi a at birth and is a common cause of perinatal mor- mg/kg versus 94 :t 7 mg/kg). Reestablishing tracheal flow for 6 d (drain and reconnect) increased fetal lung wet weights (19.2 :t 1.6 g/kg), but not total DNA contents (106 :t 9 mg/kg), compared with lung liquid drained fetuses (drain). After 6 d of tracheal obstruction (drain and obstruct) lung liquid volumes, wet lung weights, and total protein contents (weight, 28.6 :t 2.8 g/kg; protein, 1376 :t 97 mg/kg) were similar to control values (weight, 34.3 :t 2.6 g/kg; protein, 1506 :t 123 mg/kg); lung DNA contents were less than control but greater than values from lung liquid drained fetuses (drain and obstruct, 140 :t 9 mg/kg versus drain, 94 :t 7 mg/kg). We conclude that obstruction of the trachea can reverse an existing fetal lung growth deficit in approximately 6 d, whereas merely restoring tracheal continuity does not increase fetal lung growth. (Pediatr Res 38: 690-69 6, 1995) bidity and mortality in new born infants (6, 7). To avoid the postnatal consequences of fetal lung hypoplasia in humans, atte mpts have been made to surgically treat this condition in utero, before the lungs are required for gas exchange. However, these treatments have principally foc used on correcting the und erlying abnormality rather than attempting to acce lerate lung growth directly (8, 9). As increased fe tal lung expansion, resulting from tracheal obstruction, is a potent and rapid way of accelerating fetal lung gro wth (2-4), we considered it possible tha t trac hea l obstruction may rapi dly reve rse an exi sting lung growth defici t in utero. The aim of this study was to determine whether fe tal lun...

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Lung Hypoplasia Can Be Reversed by Short-Term Obstruction of the Trachea in Fetal Sheep

1995

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“…[8][9][10][11][12][13] In addition, the newer forms of treatment, such as extracorporeal membrane oxygenation, high-frequency ventilation and the use of surfactant and nitric oxide, 5,6,8,13,14 are all attempts at decreasing the mortality, as congenital diaphragmatic hernia presenting within the first six hours of life is associated with a mortality of 40%-50%. [2][3][4] This high mortality is attributed to several factors. Although associated anomalies may contribute to mortality, this is mainly due to hypoxia secondary to lung hypoplasia.…”

Section: Discussionmentioning

confidence: 99%

Congenital Diaphragmatic Hernia: The Impact of Preoperative Stabilization on Outcome

et al. 1999

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Despite the fact that it is diagnosed antenatally, and despite the availability of improved intensive care, congenital diaphragmatic hernia (CDH) is still associated with high mortality. [1][2][3] To overcome this problem, a variety of techniques have been proposed to decrease the pulmonary vascular resistance, and correct the hypoxia, acidosis and hypotension. These include in utero surgical repair, 4 introduction of extracorporeal membrane oxygenation, 5,6 and an extended period of preoperative stabilization. [7][8][9][10][11][12] Several investigators have proposed extended preoperative medical stabilization followed by delayed repair, but the results of such an approach in terms of overall outcome are controversial. [10][11][12] This report represents our experience with the management of 47 patients with congenital diaphragmatic hernia and analyzes the effect of preoperative stabilization on outcome. Patients and MethodsThis is a retrospective study of all patients admitted since 1982 to Tawam Hospital, Al-Ain, United Arab Emirates, with the diagnosis of congenital diaphragmatic hernia. The medical records of these patients were reviewed for age at presentation, sex, gestational age, birth weight, whether born at Tawam Hospital or referred from another hospital, associated anomalies, type and site of hernia, contents of the hernia, preoperative and postoperative management, blood gas parameters and outcome. The blood gases, as well as the alveolar arterial O 2 gradient (AaDO 2 =[173xFiO 2 ] -[PaCO 2 /0.8] -PaO 2 ) were analyzed in terms of outcome. The effect of preoperative stabilization, as well as changes in blood gas parameters, were also analyzed using Student's t-test. In the stabilized group, surgery was delayed in an attempt to achieve a pH of more than 7.2, PO 2 of greater than 50 torr, and PCO 2 of less than 50 torr.After evaluation at the Intensive Care Unit, endotracheal intubation was performed when indicated, and the patients were ventilated, using positive pressure ventilation (PPV) to start with, and positive end expiratory pressure (PEEP). Chest and abdominal x-rays were obtained, and respiratory support, as well as the ventilatory settings, were adjusted according to the blood gas estimates, which were obtained via umbilical and/or radial arterial catheters. Bowel decompression was achieved by nasogastric aspiration. All ventilated infants were paralyzed, and volume expanders as well as dopamine were used when indicated to maintain their blood pressure. Talozoline, surfactant, nitric oxide and dexamethasone were not used in any of our patients, and metabolic acidosis was corrected with bicarbonate therapy.All operations were performed by a transverse upper abdominal incision. After reducing the contents, primary closure of the diaphragmatic defect was performed and prosthetic graft was used whenever primary repair without tension could not be achieved. ResultsFrom 1982 to 1997, a total of 47 cases of congenital diaphragmatic hernia were admitted to our hospital. There were three cases of M...

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“…Essa compressão leva à hipoplasia pulmonar bilateral, pois há desvio do mediastino e também ocorre compressão do pulmão contralateral. Junto com a hipoplasia, há uma hipertrofia da camada média das arteríolas pulmonares e aumento da resistência vascular pulmonar, levando à hipertensão pulmonar (HP), que conforme sua gravidade pode levar à persistência do padrão fetal de circulação, acidose metabólica e respiratória, e morte neonatal 3,12 .…”

Section: Embriologia E Fisiopatologiaunclassified

“…Harrison et al 12 , na University of California, San Francisco (UCSF), começaram um ensaio clínico no qual foi empregada a correção cirúrgica fetal da HDC. Os resultados iniciais, de 1990, concluíram que quando havia herniação do fígado dentro do tórax, a correção fetal não deveria ser realizada, pois com a redução do fígado para a cavidade abdominal havia um acotovelamento da veia umbilical e consequente óbito fetal 12 . O mesmo grupo conduziu um novo ensaio para correção cirúrgica de fetos com HDC, no período de 1993 a 1996, mas sem evidência de componente hepático no tórax.…”

Section: História Da Intervenção Fetal E O Diagnóstico Pré-natalunclassified

Manejo pré-natal da hérnia diafragmática congênita: presente, passado e futuro

Gallindo

Gonçalves

Figueira

et al. 2015

Rev. Bras. Ginecol. Obstet.

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Manejo pré-natal da hérnia diafragmática congênita: presente, passado e futuro ResumoA hérnia diafragmática congênita é um defeito de formação do diafragma que acomete entre 1:2.000 e 1:4.000 nascidos vivos e constitui 8% das principais anomalias congênitas. Avanços médicos nos últimos 30 anos envolvendo diagnóstico pré-natal, intervenção fetal, manejo clinico e cirúrgico neonatal têm mudado a sobrevivência dos seus portadores. A evolução histórica desses avanços ajuda a compreender o esforço na busca de melhores resultados desse defeito muitas vezes fatal. Perspectivas na utilização de bioengenharia e terapia envolvendo células tronco podem trazer novas esperanças para os fetos com hérnia diafragmática congênita. AbstractThe congenital diaphragmatic hernia is a defect in the formation of the diaphragm, which affects between 1:2,000 and 1:4,000 live births and represents 8% of major congenital anomalies. Medical advances in the last 30 years involving prenatal diagnosis, fetal intervention, neonatal surgical and clinical management have changed the survival of these patients. The historical evolution of these advances helps us to understand the effort in pursuit of better results of this defect, which is often lethal. Perspectives on the use of bioengineering and therapy involving stem cells may bring new hope for fetuses with congenital diaphragmatic hernia.

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Correction of congenital diaphragmatic hernia in utero, V. Initial clinical experience

Cited by 238 publications

References 25 publications

Lung Hypoplasia Can Be Reversed by Short-Term Obstruction of the Trachea in Fetal Sheep

Lung Hypoplasia Can Be Reversed by Short-Term Obstruction of the Trachea in Fetal Sheep

Congenital Diaphragmatic Hernia: The Impact of Preoperative Stabilization on Outcome

Manejo pré-natal da hérnia diafragmática congênita: presente, passado e futuro

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