Bilateral Choanal Atresia in Premature Monozygotic Twins

Abstract

Bilateral congenital choanal atresia is a relatively rare anomaly of the upper airway, which may cause life-threatening respiratory emergency and require rapid diagnosis and treatment. This condition usually occurs sporadically, but has also been rarely described in siblings. We present monozygotic premature twin infants with identical findings of bilateral choanal atresia and no other associated anomalies. To our knowledge, this is the first report of such an occurrence.

Preoperative airway management in a preterm with bilateral choanal atresia

“…3 CA has been reported in both monozygotic and dizygotic twins, but there is no increased incidence of CA in twin pregnancy. [4][5][6] Corrective surgery is the only recognized treatment for CA. Providing a secure airway is usually a short-term solution.…”

Diagnostic and Therapeutic Sinonasal Endoscopy in Pediatric Patients

“…There is a female predominance with a F/M ratio of 5/1 among Caucasians. The lesion may be unilateral (60%) or bilateral (40%) and can subdivided into bony (90%) or membranous (10%) types (Vatansever et al, 2005). The genetic aspect of CA remains unclear and is likely multifactorial.…”

Treatment of bilateral choanal atresia in the premature infant

“…Bilateral choanal atresia is a rare and challenging disease occurring in 1 every 10,000 births [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]. The anatomic classification of choanal atresia was classically described as 90% bony and 10% membranous; however, more recent studies using high-resolution CT scan revealed that 30% of the atresia were purely bony and 70% were a mixed bony-membranous anomaly [2][3][4][5][6][7][8][11][12][13][14][15].…”

“…The anatomic classification of choanal atresia was classically described as 90% bony and 10% membranous; however, more recent studies using high-resolution CT scan revealed that 30% of the atresia were purely bony and 70% were a mixed bony-membranous anomaly [2][3][4][5][6][7][8][11][12][13][14][15]. Theories proposed to explain this congenital malformation include failure of the bucconasal membrane to rupture, persistence of the buccopharyngeal membranes, medial outgrowth of vertical and horizontal processes of the palatine bone and possible defects in neural crest migration [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15].…”

“…The diagnosis should also be raised when failed extubation of a newborn occurs. Associated congenital anomalies occur in 20-50% of patients [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15].…”