Spinal Neurenteric Cyst of the Ventral Cervicothoracic Junction With Klippel-Feil Syndrome as a Symptom of Progressive Myelopathy: A Case Report

Abstract

Neurenteric cysts are rare and account for only 0.7%-1.3% of all spinal tumors. Spinal neurenteric cysts are associated with spina bifida, split-cord malformations, and Klippel-Feil syndrome, a rare congenital disorder characterized by fusion of two or more cervical vertebrae. Klippel-Feil syndrome is rarely accompanied by neurenteric cysts. In this case report, we describe a cervicothoracic junction neurenteric cyst associated with Klippel-Feil syndrome in a 30-year-old man who presented with a 2-month history of neck pain with radiation of pain into both arms and a 1-month history of weakness in the left arm. Magnetic resonance imaging (MRI) of the spine revealed an expansive intradural extramedullary cystic lesion anterior to the spinal cord at the cervicothoracic junction. The neurenteric cyst was removed using an anterior approach, accompanied by C5-C6 corpectomy. The patient's condition improved postoperatively, and he was discharged after postoperative MRI. Spinal neurenteric cysts should be considered in the differential diagnosis in cases of vertebral developmental abnormalities concurrent with intraspinal cysts.

Keywords: Klippel-Feil syndrome; Neurenteric cyst; Spinal neoplasm, Cervical vertebrae.

FIGURE 1. Preoperative sagittal (A) and axial (B) T2-weighted magnetic resonance images demonstrating expansive intradural-extramedullary cystic lesion anterior of the spinal cord, extending from C5 to C7. Preoperative cervical spine 3D computer tomography (C) and C-spine lateral radiograph of cervical spine (D) demonstrating fused and deformed cervical vertebrae spanning from C5 to T3.

FIGURE 2. Intraoperative photograph of the neurenteric cyst. After the C56 corpectomy, the dura is exposed (A); Cyst rupture and cyst removal (B and C); Intradura cyst wall removal (D)

FIGURE 3. Two months follow up T2-weighted magnetic resonance images (A) demonstrate total neurenteric cyst removal; C-spine anterior (B) and lateral (C) radiograph of cervical spine demonstrating C56 corpectomy.

FIGURE 4. Histopathological findings of the cyst. Hematoxylin-eosin stained slide show (1) endodermal and glial tissue, (2, 3) columnar epithelium on the layer combined with gastric epithelium, and (4) respiratory epithelium (hematoxylin-eosin, ×40).