Laryngeal atresia: antenatal diagnosis in a twin pregnancy

Abstract

Congenital laryngeal atresia is a rare malformation almost always incompatible with life. Prenatal sonographic diagnosis of this condition has been described on five occasions and all occurred in singleton pregnancies. To our knowledge, this is the first reported case of prenatal diagnosis of laryngeal atresia in a twin pregnancy. A monochorionic diamniotic twin gestation was noted at a routine ultrasound examination at 18 weeks' gestation. One twin was found to have changes consistent with laryngeal atresia with bilateral enlarged hyperechogenic lungs and hydrops fetalis. The other twin appeared normal. A Cesarean section was performed for worsening pre-eclampsia at 29 weeks' gestation. The abnormal twin died, despite resuscitative measures and autopsy confirmed the diagnosis of laryngeal atresia. The presence of the anomaly in only one twin of a monozygotic gestation supports the theory of a developmental anomaly within the branchial arches. Prenatal diagnosis of laryngeal atresia allows prenatal planning for resuscitation at birth, but its presence in one twin of a twin gestation poses several management dilemmas.

Prenatal Diagnosis of Laryngeal Atresia in Two Cases of Congenital High Airway Obstruction Syndrome (Chaos)

“…In all of these cases, the diagnosis was inferred from secondary changes, such as enlarged echogenic lungs, flattened or inverted diaphragm, fetal ascites, and/or hydrops. Subsequent investigators have visualized a dilated trachea, which confirmed the diagnosis of upper airway obstruction (Furness et al, 1992;Richards et al, 1992;Lawrence et al, 1992;Meagher et al, 1993;Hedrick et al, 1994;Tang et al, 1996). Doubt was expressed that the level of obstruction (larynx or trachea) could be visualized by prenatal ultrasound.…”

Perinatal Management of Unanticipated Congenital Laryngeal Atresia

“…The anomaly most likely represents the final common pathway of a variety of developmental insults, rather than a specific genetic lesion. This theory is supported by the occurrence of laryngeal atresia in 1 monozygotic twin, 7 as well as by variations in the microscopic appearance of the obstructing tissue in different cases. 8 Smith and Bain type I atresia is thought to correspond to an arrest of development before the end of the seventh week of fetal life and results in the most severe deformity.…”

Prenatal sonographic diagnosis of laryngeal atresia in association with single umbilical artery

“…A search of the medical literature revealed only 6 cases of the prenatal sonographic diagnosis of CLA. [2][3][4][5][6] Two of those cases involved termination of the pregnancy. Changes caused by obstruction of the larynx include symmetrically enlarged lungs that appear hyperechoic on sonography, ascites, downward displacement of the diaphragm, compression of the heart, and polyhydramnios.…”

“…[2][3][4][5][6] The sonographic findings noted were secondary to the airway obstruction and were often sufficient to predict the outcome of the pregnancy. We present a case in which prenatal sonography led to the diagnosis of CLA and a single umbilical artery.…”