Abstract:Pulmonary artery sling (PAS) and tracheal agenesis (TA) are rare diseases, and most cases of PAS are associated with tracheal bronchial malformations. However, PAS associated with TA is yet to be reported. We report a case of PAS with TA diagnosed prenatally. Due to the extremely low incidence, physicians do not have sufficient understanding of these diseases and it is challenging to diagnose these diseases by prenatal ultrasound, with high rates of misdiagnosis. Prenatal examination of the pulmonary artery br… Show more
“…In most cases, the associated anomalies are cardiovascular, including septal defects, valvular defects, transposition of great arteries, or tetralogy of Fallot. Genitourinary and gastrointestinal anomalies and umbilical vessel alterations are also frequent in these patients ( 1 , 2 , 11 , 12 ). In addition, it can be associated with syndromes such as CHARGE association ( 13 ) (coloboma, heart anomaly, choanal atresia, retardation of growth, and genital and ear anomalies), VACTERL (vertebral defects, anal atresia, radial dysplasia, renal defects, and cardiovascular defects) ( 4 , 9 ), TACRD (tracheal agenesis/atresia, congenital heart disease, radial defects, and duodenal atresia), or FRASER (cryptophthalmia, syndactyly, laryngeal and tracheal anomalies, and urogenital malformations) ( 1 , 2 ).…”
IntroductionWe present the case of a patient with an unexpected postnatal diagnosis of tracheal agenesis, a severe and rare pathology with fewer than 200 cases documented in the literature, typically diagnosed postmortem. In our instance, early diagnosis was achieved through collaborative efforts and teamwork among various professionals. We provide illustrative images and videos to assist colleagues in identifying this congenital anomaly.Case presentationThe patient is a term newborn with prenatal indicators of polyhydramnios and a single umbilical artery. Upon birth, the infant exhibited severe respiratory distress, and orotracheal intubation via direct laryngoscopy was unfeasible. Consequently, an urgent fibrobronchoscopy, conducted by pediatric surgeons, led to the diagnosis of tracheal agenesis with tracheoesophageal fistula and the placement of a directed endotracheal tube. This intervention facilitated temporary ventilation until parental consensus on management was achieved. Following a multidisciplinary consultation, the decision was made to proceed with extracorporeal membrane oxygenation. Unfortunately, the patient experienced a prolonged refractory cardiorespiratory arrest and died after 7 h of life in his mother’s arms.ConclusionTeamwork in neonatology is indispensable when addressing emergent pathologies. In our experience, multidisciplinary management, including anesthesiologists and pediatric surgeons, should be contemplated in complex scenarios.
“…In most cases, the associated anomalies are cardiovascular, including septal defects, valvular defects, transposition of great arteries, or tetralogy of Fallot. Genitourinary and gastrointestinal anomalies and umbilical vessel alterations are also frequent in these patients ( 1 , 2 , 11 , 12 ). In addition, it can be associated with syndromes such as CHARGE association ( 13 ) (coloboma, heart anomaly, choanal atresia, retardation of growth, and genital and ear anomalies), VACTERL (vertebral defects, anal atresia, radial dysplasia, renal defects, and cardiovascular defects) ( 4 , 9 ), TACRD (tracheal agenesis/atresia, congenital heart disease, radial defects, and duodenal atresia), or FRASER (cryptophthalmia, syndactyly, laryngeal and tracheal anomalies, and urogenital malformations) ( 1 , 2 ).…”
IntroductionWe present the case of a patient with an unexpected postnatal diagnosis of tracheal agenesis, a severe and rare pathology with fewer than 200 cases documented in the literature, typically diagnosed postmortem. In our instance, early diagnosis was achieved through collaborative efforts and teamwork among various professionals. We provide illustrative images and videos to assist colleagues in identifying this congenital anomaly.Case presentationThe patient is a term newborn with prenatal indicators of polyhydramnios and a single umbilical artery. Upon birth, the infant exhibited severe respiratory distress, and orotracheal intubation via direct laryngoscopy was unfeasible. Consequently, an urgent fibrobronchoscopy, conducted by pediatric surgeons, led to the diagnosis of tracheal agenesis with tracheoesophageal fistula and the placement of a directed endotracheal tube. This intervention facilitated temporary ventilation until parental consensus on management was achieved. Following a multidisciplinary consultation, the decision was made to proceed with extracorporeal membrane oxygenation. Unfortunately, the patient experienced a prolonged refractory cardiorespiratory arrest and died after 7 h of life in his mother’s arms.ConclusionTeamwork in neonatology is indispensable when addressing emergent pathologies. In our experience, multidisciplinary management, including anesthesiologists and pediatric surgeons, should be contemplated in complex scenarios.
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