Staged bilateral far-lateral approach for bilateral cervicomedullary junction neurenteric cysts in a 10-year-old girl

Case report

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Neurenteric cysts are rare and benign lesions that consist of ectopic alimentary tissue residing in the central nervous system. They tend to occur most frequently in an intraspinal rather than intracranial location. Intracranial neurenteric cysts are a rare occurrence in the pediatric population. These lesions typically present as unilateral cystic structures in the lower cerebellopontine angle and craniocervical junction. To the authors' knowledge, there have been no reported cases of bilateral localization of intracranial neurenteric cysts. In this report, they present an unusual case of a 10-year-old girl who was found to have bilateral intracranial neurenteric cysts at the pontomedullary junction. The patient was successfully treated with staged, bilateral far-lateral transcondylar resection of the cysts. The authors also provide a brief overview of the literature describing intracranial neurenteric cysts in children.

Abbreviation used in this paper:CN = cranial nerve.

Article Information

Address correspondence to: James K. Liu, M.D., 90 Bergen St., Suite 8100, Department of Neurological Surgery, University of Medicine and Dentistry of New Jersey, New Jersey Medical School, Newark, NJ 07101. email: james.liu@umdnj.edu.

Please include this information when citing this paper: published online June 28, 2013; DOI: 10.3171/2013.5.PEDS13100.

© AANS, except where prohibited by US copyright law.

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Figures

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    Preoperative T-1 weighted MR images obtained before (A and B, sagittal; D, axial) and after (C, coronal; F, axial) Gd administration and an axial T-2 weighted MR image (E) demonstrating a nonenhancing cystic skull base mass located in the lower clivus, ventral to the medulla, occupying both cerebellopontine angles with mass effect on the pons, medulla, and lower cranial nerves. The mass appeared to have a median septation with 2 distinct-appearing compartments (D–F). The left side of the mass demonstrated increased T1 and decreased T2 signal, while the right side of the mass demonstrated increased T1 and T2 signal.

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    Intraoperative photographs obtained during 2 successive farlateral transcondylar skull base approaches for resection of the right-sided (A and B) and left-sided (C and D) cysts. The images obtained before removal of the cysts (A and C) show the lesions (indicated by T) and their relationships to the cerebellum (indicated by Cb) and cranial nerves. The right-sided cyst appeared to be whitish yellow, whereas the left-sided cyst appeared slightly more yellow. The images obtained after removal of the cyst contents and cyst wall on either side (B and D) show the excellent decompression and visualization of the lower cranial nerves (IX, X, and XI), posterior inferior cerebellar artery (PICA), and hypoglossal nerve rootlets (XII) that were obtained bilaterally.

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    A: Preoperative axial T2-weighted MR image demonstrating bilateral cysts at the cervicomedullary junction. B: Postoperative axial T2-weighted MR image obtained after removal of the right-sided cyst demonstrating decompression of cyst contents in the first of 2 staged procedures. C–F: Postoperative MR images obtained at 3-month follow-up visit after the second stage of removal of the bilateral cysts demonstrating excellent decompression of the brainstem and cranial nerves bilaterally with no radiographic evidence of residual tumor (C, axial T2-weighted; D, sagittal T1-weighted post-Gd; E, coronal T1-weighted post-Gd; F, axial T1-weighted).

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    Photomicrographs demonstrating histopathological features of the bilateral neurenteric cysts. A: The right cyst wall was lined with squamous epithelium composed of focal regions with elongated cells with cilia. In addition, evidence of old hemorrhage, cholesterol clefts, and areas of calcification were also seen. B: The left cyst wall consisted of ciliated columnar cells, with submucosal inflammation, mainly lymphocytic. C: In addition, the lining of the cyst wall appeared consistent with squamous metaplasia with some cells coming off the inner lining. D: Also identified was evidence of some heaping up of cells going toward squamous metaplasia in addition to submucosal inflammation, with a few lymphocytes. H & E; original magnifications ×100 (A–C) and ×250 (D).

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    Follow-up sagittal and coronal T2-weighted (A and B) and axial T2-weighted FIESTA (C and D) MR images obtained 1 year after the staged removal of the bilateral cysts demonstrating no radiographic evidence of residual tumor. Cranial nerves VI, VII, and VIII and the basilar artery are well visualized (D).

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