Ciliated hepatic foregut cysts in children

Abstract

Ciliated hepatic foregut cyst (CHFC) is a rare foregut developmental malformation usually diagnosed in adulthood; however, rare cases have been reported in the pediatric population. CHFC can transform into a squamous cell carcinoma resulting in death despite surgical resection of the isolated malignancy. We report the presentation, evaluation, and surgical management of a symptomatic 17-year-old girl found to have a 6.5 x 4.5 cm CHFC and suggest that all patients with suspected CHFC undergo prompt evaluation and complete cyst excision.

Figure 1

Triple-phase CT scan with axial (Panel A) and coronal (Panel B) images of a large CHFC arising in segments IV and VIII (large arrow). The gallbladder is visible on the coronal view (panel B) and is marked by small arrowhead.

Figure 2

Intra-operative photographs during laparoscopy showing an intact CHFC (Panel A), incised CHFC (Panel B) with portion of the wall sent for frozen section evaluation, and the inside view of the cyst (Panel C). gb=gallbladder; cy=CHFC.

Figure 3

Hematoxylin and eosin stained permanent sections of cyst wall (Panel A - 200x magnification) showing pseudostratified epithelium lining, connective tissue, smooth muscle layer, an outer fibrous capsule cyst wall and underlying liver parenchyma with the closer view (Panel B - 400x magnification) highlighting the ciliated, columnar, pseudostratified epithelial lining. Li=liver parenchyma.