Bilateral congenital dacryocystocoele with intranasal extension is very rare and may lead to mild to severe respiratory distress, depending on the degree of obstruction, in an otherwise healthy newborn. If severe, an urgent surgical intervention may be life saving. Our aim is to alert paediatric surgeons to this rare condition. Early detection and early treatment with a minimally invasive surgical procedure may be life saving and prevent severe sequelae due to respiratory distress.
We present a healthy newborn girl who was admitted to neonatal intensive care with progressive respiratory distress. After a full work-up, she was diagnosed with bilateral dacryocystocoele with intranasal extension and complete obstruction of the anterior nasal cavity. Emergency bilateral endoscopic marsupialization of the cysts with probing of the nasolacrimal duct was performed. The girl recovered without sequelae.
In congenital dacryocystocoele, coexistent obstruction of the valve of Rosenmüller and the valve of Hasner is observed. Subsequent intranasal protrusion with obstruction of the inferior nasal cavity may occur. The incidence is unknown, but if bilateral, it is very rare. In most cases, a dacryocystocoele is uncomplicated and may be treated conservatively. However, if intranasal protrusion occurs, and especially if the involvement is bilateral, it is a surgical emergency.
Early diagnosis with subsequent minimally invasive surgical treatment of bilateral congenital dacryocystocoele with intranasal protrusion may prevent serious complications due to respiratory distress.

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