Congenital Dacryocystocele With a Massive Dumbbell-Shaped Intranasal Cyst: Open-Book Marsupialization and Histopathology
Congenital dacryocystocele (CDC) with a large intranasal cyst is not very common. Large intranasal cysts are managed by simple or cruciate marsupialization. A female neonate, aged 12 days, presented with a right-sided CDC (Fig. A) since birth. Endoscopic examination showed a massive, dumbbell-shaped (Fig. B, white arrows) intranasal cyst occupying the whole length of the inferior meatus and displacing the inferior turbinate superiorly (Fig. B). The cyst was marsupialized in an open-book fashion, and a small portion of the redundant mucosa excised (Fig. C). There was an immediate resolution of the CDC (Fig. D). The true posterior extent of the cyst could only be determined following marsupialization and intranasal drainage of the dacryocystocele contents (Fig. E). The cyst (Fig. E, star) extended posteriorly up to the root of the inferior turbinate (Fig. E, arrow), just short of the nasopharynx. The anterior and posterior flaps (Fig. F and G, arrows) of the marsupialized cyst were reflected similar to that of lacrimal sac flaps in an endoscopic DCR (Fig. F and G). The left-sided lacrimal drainage system was normal. Histopathological analysis shows the cyst wall to be lined by stratified columnar epithelium, and stroma shows patchy fibrosis and chronic inflammatory infiltrate (Fig. H). Postoperatively, the CDC completely resolved and the patient was asymptomatic.
Massive intranasal cysts reaching up to the nasopharynx are exceptionally rare in a case of CDC. The dumbbell shape could be explained by the folding of the redundant cyst wall up on itself in cases of very large cysts. Simple marsupialization may not be adequate in these cases to counteract the massive redundant mucosa and would ideally require additional superior and inferior relaxing cuts on the anterior and posterior flaps, so that they can be reflected like an open book.© 2020 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.