Outcome of congenital diaphragmatic hernia

Beresford, Michael W.; Shaw, Nicholas

doi:10.1002/1099-0496(200009)30:3<249::aid-ppul9>3.0.co;2-9

Cited by 81 publications

(22 citation statements)

References 64 publications

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“…3,4 Although competition for space by the abdominal viscera during fetal life affects the bronchial growth and the vascular elements in the lung, postnatal pulmonary hypertension and the degree of lung hypoplasia greatly determine survival. 5 Before, during, and after surgery, ECMO may be required to provide respiratory and circulatory support.…”

Section: Discussionmentioning

confidence: 99%

Congenital Diaphragmatic Hernia and Situs Inversus Totalis

et al. 2004

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ABBREVIATIONS. ECMO, extracorporeal membrane oxygenation; VA-ECMO, venoarterial ECMO. C ongenital diaphragmatic hernia is a relatively rare disorder (1:3000 newborns) that frequently presents with respiratory distress in the immediate neonatal period due to severe pulmonary hypertension and lung hypoplasia. Extracorporeal membrane oxygenation (ECMO) can be used as a last resort when artificial ventilation and/or modulation of the pulmonary vascular tone fail to improve the clinical condition.Situs inversus totalis is a rare condition in which orientation of all asymmetrical organs in the body is a mirror image of the normal morphology. 1 Diaphragmatic hernia may be caused by predisposing genes that are involved in left-right axis determination.A few cases of an eventration of the diaphragm combined with situs inversus totalis have been described in the literature. We report for the first time a patient with a right-sided posterolateral diaphragmatic hernia, type Bochdalek, and a situs inversus totalis for which contralateral cannulation for the institution of venoarterial ECMO (VA-ECMO) was warranted. CASE REPORTA girl with a birth weight of 3810 g, born at the postmenstrual age of 39 ϩ 6 weeks, developed acute respiratory distress, cyanosis, and lethargy within 30 minutes after birth. Physical examination revealed a scaphoid abdomen and decreased breathing sounds at the right side of the thorax. A chest radiograph revealed that the heart and mediastinum were displaced to the left and that the right pleural cavity was filled with intestines (Fig 1). Ultrasound confirmed that the right hemithorax was almost completely filled with loops of intestine, mesenterial fat, and the spleen due to a large defect in the right hemidiaphragm. An abdominal situs inversus was documented; ie, a normally formed liver positioned left and the spleen on the right side. Echocardiography confirmed a left descending aorta and a mirror image dextrocardia; ie, a structurally normal heart mirrored and unusually positioned in the right chest. At first, there were no signs of clinical, relevant pulmonary hypertension on ultrasonography.Karyotyping revealed a normal female (46XX). Family history showed no parental consanguinity and no congenital anomalies or minor laterality defects. Initial management consisted of emergency endotracheal intubation. Next, different ventilatory settings were used to optimize oxygenation, ranging from conventional mechanical ventilation to high-frequency oscillation with these following maximum settings: frequency, 10 Hz; mean airway pressure, 17; ␦ P, 50; fractional inspired oxygen concentration, 100%. Supportive therapy consisted of exogenous surfactant (Alvofact, 50 mg/kg, Boehringer Inc, Ingelheim, Germany), cardiovascular support with inotropic agents (maximum dosages: dopamine, 20 g/kg per min; dobutamine, 20 g/kg per min; noradrenaline, 0.3 mg/kg per min), and pulmonary vasodilators such as inhaled nitric oxide (maximum dosage: 20 ppm).Because the girl showed progressive respiratory failure complicated ...

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Section: Discussionmentioning

confidence: 99%

Congenital Diaphragmatic Hernia and Situs Inversus Totalis

et al. 2004

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“…Unfortunately, detection is dependent on the quality of the study, technical expertise and skills available. In an earlier large multicenter study, done in 2000, up to 50% were not detected (15) and antenatal diagnosis was equated with a poor prognosis (58% mortality, compared with 48% if born alive and 33% after postnatal operative repair) (16).…”

Section: Antenatal Diagnosis Prognosis and Counsellingmentioning

confidence: 99%

“…Long‐term pulmonary outcome of survivors is generally good (16). Using age‐matched controls at Great Ormond Street, survivors 5–10 years postrepair were shown to have reasonable respiratory function (36).…”

Section: Long‐term Outcomementioning

confidence: 99%

Evolving management of congenital diaphragmatic hernia

Brown¹,

Bösenberg

2007

Pediatric Anesthesia

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“…82,83 Survival is associated with significant long-term morbidity, primarily from pulmonary, neurological and gastrointestinal disease. An incidence of approximately 1:2500 births 82 results in a new case every 5 hours in the European Union.…”

Section: Congenital Diaphragmatic Herniamentioning

confidence: 99%

Fetoscopic Surgery for in-Utero Management of Congenital Diaphragmatic Hernia

Nelson

Cameron

Deprest³

2006

Fet. Matern. Med. Rev.

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INTRODUCTIONUntil recently two possibilities were available to the expectant parents of a fetus diagnosed with a congenital diaphragmatic hernia: termination of pregnancy or continuation of the pregnancy until term with a potential change in the place of delivery. Open fetal surgery has been used to treat a growing number of congenital malformations with life-threatening or highly morbid consequences including congenital diaphragmatic hernia. 1 However, its effectiveness is limited by the occurrence of preterm labour, chorioamniotic membrane separation, preterm prelabour rupture of the membranes and altered fetal homeostasis. 2 These problems were the impetus for the development of minimal access fetal surgery. Developments in endoscopic surgical technology over the past three decades have provided the opportunity to develop techniques adapted for prenatal fetal intervention. 3 RATIONALE FOR FETOSCOPIC SURGERYExperimental studies on the effect of minimal access fetal surgery suggest that it reduces preterm labour and achieves better maintenance of fetal and maternal homeostasis. 4 With respect to preterm labour fetoscopic access in mid-trimester Rhesus monkeys did not result in any significant uterine contractions by 24 hours post-operatively. 5 Subsequent recordings of myometrial activity were repeated during the third trimester, and no premature contractions were noted. In contrast, Luks and colleagues studying third trimester sheep found that uterine contractions were present 52% of the time after maternal laparotomy irrespective of whether a hysterotomy or fetoscopic access using three ports was performed. 6 These myometrial contractions were noted in response to access only, as no fetal manipulation was undertaken. The primate uterus resembles the human uterus much more closely than the ovine and is generally accepted as a good model for studies into human parturition, questioning the application of ovine myometrial activity studies to the human situation.With respect to maternal-fetal homeostasis, Luks demonstrated in the same study a significant 27% reduction in uterine blood flow and utero-placental oxygen delivery with hysterotomy creation, whereas endoscopic access had no effect. 6 When uterine artery flow and thus oxygen delivery is reduced to 70% of control, there is a shift in fetal blood flow distribution, a decrease in fetal pH and an increase in serum lactate. 7 It is likely that oxygen delivery would be further impaired during open fetal surgery, by manipulation of the uterus, active pressure on uterine vessels and traction or compression of the umbilical cord. Therefore the endoscopic approach, which does not alter uteroplacental oxygen delivery, would be preferable. Clinical experience with human fetoscopic surgery reflects this experimental work. Fetoscopic intervention produces less preterm labour with less use of tocolytics, decreased maternal hospital stay and decreased maternal complications related to tocolysis. 8

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Outcome of congenital diaphragmatic hernia

Cited by 81 publications

References 64 publications

Congenital Diaphragmatic Hernia and Situs Inversus Totalis

Congenital Diaphragmatic Hernia and Situs Inversus Totalis

Evolving management of congenital diaphragmatic hernia

Fetoscopic Surgery for in-Utero Management of Congenital Diaphragmatic Hernia

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