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14 results

  • Chapter 7 - Fetal Endoscopic Tracheal Occlusion (FETO)

  • from Section 2
  • Edited by Olutoyin A. Olutoye
  • Book:
    Anesthesia for Maternal-Fetal Surgery
    Published online:
    19 November 2021
    Print publication:
    11 November 2021, pp 95-102

  • Summary

    Despite advances in neonatal and surgical care, the outcome of severe congenital diaphragmatic hernia (CDH) is still quite poor. Improvements in the ability to diagnose and risk stratify CDH prenatally have led to investigations into whether the more severe forms of CDH may benefit from in-utero intervention to ameliorate the accompanying pulmonary hypoplasia. Fetal endoscopic tracheal occlusion (FETO) is an example of a prenatal surgical intervention that was made possible by the technological advancements in endoscopic surgery. FETO is generally performed in fetuses with more severe forms of CDH to stimulate prenatal lung growth. While FETO is still considered investigational therapy and has not attained standard of care status, the results from published data to date have been promising. The complexity of FETO, coupled with the surgical and anesthetic risk to the mother and fetus requires a highly functional multidisciplinary fetal team, which includes obstetrics, pediatric surgery, and anesthesiology. This chapter will provide an in-depth understanding of the history of fetal endoscopic tracheal occlusion for CDH, the operative and anesthetic approach to FETO and associated perioperative considerations. Congenital diaphragmatic hernia continues to be a disease that perplexes obstetricians, neonatologists, surgeons, anesthesiologists, and the entire care team.

  • Chapter 34 - Extracorporeal Membrane Oxygenation

  • from Section 6 - Heart Failure, Mechanical Circulatory Support, and Transplantation
  • Edited by Laura K. Berenstain, James P. Spaeth
  • Book:
    Congenital Cardiac Anesthesia
    Published online:
    09 September 2021
    Print publication:
    16 September 2021, pp 261-267

  • Summary

    A congenital diaphragmatic hernia is a rare birth defect characterized by a diaphragmatic defect allowing herniation of abdominal contents into the chest, subsequently impeding normal lung development. This results in structural and functional changes to lung structure, pulmonary circulation, and the heart. The size of the defect can significantly affect the prognosis, as the severity of this condition is proportional to the severity of lung hypoplasia and pulmonary hypertension. Initiation of extracorporeal membrane oxygenation is considered a last resort, life-preserving option for neonates with congenital diaphragmatic hernia who have failed all other medical therapies. This chapter details the management of an infant with congenital diaphragmatic hernia, currently on extracorporeal membrane oxygenation support and requiring repair of his hernia.

  • Maternal dietary intake of vitamin A during pregnancy was inversely associated with congenital diaphragmatic hernia: the Japan Environment and Children’s Study

  • Takehiro Michikawa, Shin Yamazaki, Makiko Sekiyama, Tatsuo Kuroda, Shoji F. Nakayama, Tomohiko Isobe, Yayoi Kobayashi, Miyuki Iwai-Shimada, Eiko Suda, Toshihiro Kawamoto, Hiroshi Nitta, the Japan Environment and Children’s Study Group
  • Journal:
    British Journal of Nutrition / Volume 122 / Issue 11 / 14 December 2019
    Published online by Cambridge University Press:
    04 November 2019, pp. 1295-1302
    Print publication:
    14 December 2019
    • Article
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  • The pathogenesis of congenital diaphragmatic hernia (CDH) is largely unknown; however, vitamin A seems to play a role in diaphragmatic development. Previous case–control studies reported that maternal dietary vitamin A intake was inversely associated with the risk of CDH. To our knowledge, however, there is no prospective evidence regarding this association. Our aim was to examine whether maternal intake of vitamin A was associated with CDH occurrence. Baseline data, from the Japan nationwide birth cohort study (2011–2014) of 89 658 mothers (mean age at delivery = 31·2 years) who delivered singleton live births, were analysed. We assessed dietary habits using an FFQ focused on the first trimester and estimated the daily intake of total vitamin A (retinol activity equivalents), retinol, provitamin A carotenoids and vegetables. The occurrence of CDH was ascertained from medical records. A total of forty cases of CDH were documented. The adjusted OR of CDH occurrence for the high total vitamin A intake category (median = 468 μg/d) was 0·6 (95 % CI 0·3, 1·2) with reference to the low intake category (230 μg/d). When we restricted to mothers with a prepregnancy BMI of 18·5–24·9 kg/m2, vitamin A intake was inversely associated with the risk of their children being born with CDH (OR 0·5, 95 % CI 0·2, 1·0). Even given the limited number of cases in the study, our findings provide additional evidence to link vitamin A with CDH.

  • The effect of congenital diaphragmatic hernia on the development of left-sided heart structures

  • Zachary J. Coffman, Eugene D. McGahren, Brooke D. Vergales, Christine H. Saunders, Jeffrey E. Vergales
  • Journal:
    Cardiology in the Young / Volume 29 / Issue 6 / June 2019
    Published online by Cambridge University Press:
    06 June 2019, pp. 813-818
  • Introduction:

    Patients with congenital diaphragmatic hernias often have concomitant congenital heart disease (CHD), with small left-sided cardiac structures as a frequent finding. The goal of this study is to evaluate which left-sided heart structures are affected in neonates with congenital diaphragmatic hernias.

    Methods:

    Retrospective review of neonates between May 2007 and April 2015 with a diagnosis of a congenital diaphragmatic hernia was performed. Clinical and echocardiographic data were extracted from the electronic medical record and indexed to body surface area and compared to normative values. Univariable regression models assessed for associations between different variables and length of stay.

    Results:

    Data of 52 patients showed decreased mean z scores for the LVIDd (–3.16), LVIDs (–3.05), aortic annulus (–1.68), aortic sinuses (–2.11), transverse arch (–3.11), and sinotubular junction (–1.47) with preservation of the aorta at the diaphragm compared to age-matched normative data with similar body surface areas. Regression analysis showed a percent reduction in length of stay per 1 mm size increase for LVIDd (8%), aortic annulus (27%), aortic sinuses (18%), sinotubular junctions (20%), and transverse arches (25%).

    Conclusions:

    Patients with congenital diaphragmatic hernias have significantly smaller left-sided heart structures compared to age-matched normative data. Aortic preservation at the diaphragm provides evidence for a mass effect aetiology with increased right-to-left shunting at the fetal ductus resulting in decreased size. Additionally, length of stay appears to be prolonged with decreasing size of several of these structures. These data provide quantitative evidence of smaller left-sided heart structures in patients with congenital diaphragmatic hernias.

  • Chapter 19.1 - Congenital diaphragmatic hernia

  • from Section 2 - Fetal disease
  • Edited by Mark D. Kilby, Anthony Johnson, Baylor College of Medicine, Texas, Dick Oepkes
  • Book:
    Fetal Therapy
    Published online:
    05 February 2013
    Print publication:
    13 December 2012, pp 370-375

  • Summary

    In congenital diaphragmatic hernia (CDH) pulmonary hypoplasia is variable depending on the size of the diaphragmatic defect and the amount and timing of visceral herniation. In CDH pulmonary hypoplasia may arise from loss of lung fluid, decreased fetal breathing secondary to diaphragmatic dysfunction, and decreased intrathoracic volume. The lungs in CDH appear to be arrested in the saccular phase of development with poorly developed airspaces and thickened interalveolar septums. Pulmonary hypoplasia and decreased lung compliance contribute to the increased susceptibility to ventilation-induced lung injury seen in CDH. Persistent pulmonary hypertension of the newborn is the failure of the normal circulatory transition after birth. Four major components of surfactant proteins have been identified: surfactant protein A and D are hydrophilic and protein B and C are hydrophobic. A surfactant deficiency is clearly seen in the experimental lamb model of CDH.

  • Section 1 - General principles

  • Edited by Mark D. Kilby, Anthony Johnson, Baylor College of Medicine, Texas, Dick Oepkes
  • Book:
    Fetal Therapy
    Published online:
    05 February 2013
    Print publication:
    13 December 2012, pp -

  • Summary

    Initial therapies such as intrauterine transfusion for severe hemolytic disease of the fetus were undertaken based on sound physiological principles and an understanding of the basic pathogenesis but were never subjected to randomized clinical trials. More recent targeted interventions for such conditions as treatment for severe twin-to-twin transfusion syndrome (TTTS), myelomeningocele (MMC) and congenital diaphragm hernia (CDH) have been the subject of well-designed randomized investigations. This chapter elucidates the history and the basis for the treatment of these fetal interventions. Routine induction of labor at a premature gestation was the only therapy that could be offered to attempt to curb the inevitable death due to hemolytic disease of the fetus and newborn (HDFN) that occurred in 30% of cases. The human fetus has indeed become a patient and development of treatments is a work in progress.

  • Chapter 19.2 - Congenital diaphragmatic hernia

  • from Section 2 - Fetal disease
  • Edited by Mark D. Kilby, Anthony Johnson, Baylor College of Medicine, Texas, Dick Oepkes
  • Book:
    Fetal Therapy
    Published online:
    05 February 2013
    Print publication:
    13 December 2012, pp 376-388

  • Summary

    This chapter discusses congenital diaphragmatic hernia (CDH) from a perspective of antenatal management, including fetal intervention. It summarizes actual survival rates when this condition is managed after birth, essentially showing that there is no effective postnatal therapy in a subset of fetuses. Prediction methods are typically based on estimation of lung size by ultrasound and determination of liver herniation into the thorax. Three-dimensional (3D) ultrasound (US) and MRI both allow measurement of absolute lung volumetry. MRI allows better visualization of the ipsilateral lung than 3D US. Preliminary work on the use of diffusion-weighted imaging (DWI)-MRI as a tool to differentiate between normal and pathological lung development has shown a significant relationship between DWI-MRI parameters and gestational age in the normal fetus. The chapter also describes the current clinical experience with fetal surgery, including the design of trials that will have to determine the place of fetal surgery.

  • Sildenafil therapy for neonatal and childhood pulmonary hypertensive vascular disease

  • Tilman Humpl, Janette T. Reyes, Simon Erickson, Ruth Armano, Helen Holtby, Ian Adatia
  • Journal:
    Cardiology in the Young / Volume 21 / Issue 2 / April 2011
    Published online by Cambridge University Press:
    08 December 2010, pp. 187-193
  • Objectives

    We hypothesised that sildenafil would improve hemodynamics in children with pulmonary hypertension and attenuate rebound pulmonary hypertension after inhaled nitric oxide withdrawal.

    Patients and methods

    We undertook an open-label, single-drug study of sildenafil in patients under 5 years of age with either symptomatic or rebound pulmonary hypertension following inhaled nitric oxide withdrawal.

    Results

    We recruited 25 patients (median age 180 days, 10–1790) to receive sildenafil. The median right ventricular to systemic systolic blood pressure ratio before sildenafil therapy was 1.0 (0.5–1.4) and decreased to 0.5 (with a range from 0.3 to 1.3; p = 0.0002). In five patients the baseline pulmonary vascular resistance index was 10 (7.1–13.6) Wood units metre square and decreased to 5.8 (2.7–15.6) Wood units metre square (p = 0.04) at 6 months. Ten patients were treated with sildenafil for a median of 34 days (9–499) until resolution of pulmonary artery hypertension and continue to do well. Six patients continued sildenafil therapy for a median of 1002 days (384–1574) with improvement but without resolution of pulmonary hypertension. There was no change in serum creatinine, urea, liver function tests, or platelet count. In 15 patients sildenafil abolished rebound pulmonary artery hypertension following withdrawal of inhaled nitric oxide. Median right ventricular pressure to systemic systolic pressure ratio decreased from 1.0 (0.8–1.4) during nitric oxide withdrawal to 0.4 (0.3–0.8) p = 0.006 after pre-treatment with sildenafil.

    Conclusion

    In children under 5 years of age with severe pulmonary hypertension, sildenafil therapy resulted in prolonged hemodynamic improvements without adverse effects. Sildenafil attenuated rebound pulmonary hypertension after withdrawal of inhaled nitric oxide.

  • Chapter 20 - Fetal Surgery

  • from Part III - SURGICAL PROCEDURES
  • John Patrick O'Grady, Tufts University, Massachusetts, Martin L. Gimovsky, Lucy A. Bayer-Zwirello, Kevin Giordano
  • Book:
    Operative Obstetrics
    Published online:
    07 May 2010
    Print publication:
    04 August 2008, pp 638-662

  • Summary

    This chapter provides an overview of the principles of modern operative fetal intervention. In practice, maternal safety has remained the highest priority in fetal surgery. Some have suggested that pregnant women are a particularly vulnerable group of patients who might have a low threshold to consent to highly invasive fetal therapies, even if the benefits to their unborn children could be small. Preoperative preparation for fetoscopic surgery is done in a fashion similar to that used in open fetal surgery. Fetoscopy offers several distinct advantages when compared with open fetal surgery. The chapter talks about twin-twin transfusion syndrome, airway obstruction, thoracic anomalies, and sacrococcygeal teratoma, congenital diaphragmatic hernia, myelomeningocele, and aortic stenosis. Although most prenatally diagnosed anomalies are best managed after birth, several disorders have predictable, irreversible, and devastating consequences under expectant prenatal management.