Congenital High Airway Obstruction Research Articles

Indications, Resource Allocation, and Outcomes Associated with Ex-Utero Intrapartum Treatment Procedures: A North American Fetal Therapy Network Survey

Introduction: Neonates with cardiorespiratory compromise at delivery are at substantial risk of hypoxic neurologic injury and death. Though mitigation strategies such as ex-utero intrapartum treatment (EXIT) exist, the competing interests of neonatal beneficence, maternal non-maleficence, and just distribution of resources require consideration. Due to the rarity of these entities, there are few systematic data to guide evidence-based standards. This multi-institutional, interdisciplinary approach aims to elucidate the current scope of diagnoses that might be considered for such treatments and examine if treatment allocation and/or outcomes could be improved. Methods: After IRB approval, a survey investigating diagnoses appropriate for EXIT consultation and procedure, variables within each diagnosis, occurrence of maternal and neonatal adverse outcomes, and instances of suboptimal resource allocation in the last decade was sent to all North American Fetal Treatment Network center representatives. One response was recorded per center. Results: We received a 91% response rate and all but one center offer EXIT. Most centers (34/40, 85%) performed 1–5 EXIT consultations per year and 17/40 (42.5%) centers performed 1–5 EXIT procedures in the last 10 years. The diagnoses with the highest degree of agreement between centers surveyed to justify consultation for EXIT are head and neck mass (100%), congenital high airway obstruction (90%), and craniofacial skeletal conditions (82.5%). Maternal adverse outcomes were noted in 7.5% of centers while neonatal adverse outcomes in 27.5%. A large percentage of centers report cases of suboptimal selection for risk mitigation procedures and several centers experienced adverse neonatal and maternal outcomes. Conclusion: This study captures the scope of EXIT indications and is the first to demonstrate the mismatch in resource allocation for this population. Further, it reports on attributable adverse outcomes. Given suboptimal allocation and adverse outcomes, further examination of indications, outcomes, and resource use is justified to drive evidence-based protocols.

P638: Identification of a pathogenic variant in NSDHL in a patient with congenital high airway obstruction (CHAOS) syndrome

P638: Identification of a pathogenic variant in NSDHL in a patient with congenital high airway obstruction (CHAOS) syndrome

Pathology of chest organs in the fetus: review of literature and own observations

The article presents various nomological forms of malformations of the chest organs and their population frequency. The ultrasound and differential diagnostic features are described, such as cystic adenomatoid malformations of the lung, pulmonary sequestration, bronchogenic cyst and such rare diseases as congenital high-airway obstruction syndrome and bronchial atresia, pulmonary hypoplasia and pulmonary agenesis, mediastinal cystic lymphangioma. The timing of detection and accuracy of diagnosis of various malformations of the chest is reported. Data on the nature of concomitant abnormalities and chromosomal pathology are given. Considerable attention has been paid to resolving the issue of the advisability of maintaining or terminating a pregnancy, the most rational tactics of managing patients with this pathology, the timing and methods of delivery, as well as the selection of optimal methods for treating newborns in the postnatal period and the prognosis of their further development.

Prenatal ultrasonography findings of fetus with congenital high airway obstruction (chaos): A case report and review of literature

Prenatal ultrasonography findings of fetus with congenital high airway obstruction (chaos): A case report and review of literature

Congenital High Airway Obstruction – Case Report with Imaging Features

Congenital High Airway Obstruction – Case Report with Imaging Features

Congenital lung lesions: Prenatal diagnosis and intervention

Congenital lung lesions are common sonographic findings in pregnancy, usually detected at the routine 20 weeks scan. The most common is cystic adenomatous malformation of the lung (CCAM). This usually causes few prenatal problems; however, fetal hydrops occurs in about 5%. Prenatal intervention for these is possible in many to allow survival to birth. Bronchoplumonary sequestration (BPS), with an aberrant “feeder” vessel arising from the aorta may co-exist but is detectable as a separate entity by visualization of this vessel. Symptomatic or curative prenatal intervention is again possible in the few severe cases where hydrops or pleural effusions develop. Pleural effusions may be due to a primary leak usually of chylous fluid: prenatal thoracoamniotic shunting may prevent pulmonary hyoplasia or cure the consequent fetal hydrops. More often, however, effusions are a consequence of an underlying abnormality, including many structural or chromosomal abnormalities that may also cause co-existing fetal hydrops. Congenital high airway obstruction (CHAOS) is commonly fatal but cases potentially amenable to prenatal intervention or to immediate perinatal management may be identified using ultrasound or MRI.

Ex-utero intrapartum therapy

The ex-utero intrapartum therapy (EXIT) procedure was designed to secure the airway at delivery in fetuses who had undergone tracheal occlusion for severe congenital diaphragmatic hernia. The EXIT was then adapted for deliveries where the airway may be difficult to secure, such as large neck masses or congenital high airway obstruction. Subsequently, use of EXIT has been extended to fetal anomalies where resuscitation may be compromised, including large thoracic masses, severe congenital diaphragmatic hernia, or pulmonary agenesis. The key to EXIT is preservation of uteroplacental blood flow and gas exchange, using inhalational agents to provide uterine relaxation, and maintenance of uterine volume by amnioinfusion and only partial exposure of the fetus. This provides time for procedures such as laryngoscopy, bronchoscopy, vascular access, resection of neck or lung masses, or cannulation for extracorporeal membrane circulation, in order to convert an emergent crisis to a controlled situation.

Recurrent short-rib polydactyly syndrome: prenatal three-dimensional ultrasound findings and associations with congenital high airway obstruction and pyelectasia

Recurrent short-rib polydactyly syndrome: prenatal three-dimensional ultrasound findings and associations with congenital high airway obstruction and pyelectasia