대 한 소 아 신 경 학 회 지
Vol. 20, No. 1, March, 2012
□ 증 례 □Intracranial Neurenteric Cyst of the Anterior Brain Stem in a Girl
Chang Woo Lee, M.D., Soo Ho Lee, M.D. and Seung Taek Yu, M.D.
Department of Pediatrics, Wonkwang University School of Medicine, Iksan, Korea
= Abstract =
Neurenteric cyst is a rare, congenital, and benign cystic lesion of the central nervous sys- tem, which is generally thought to result from failure of separation of the neuro-ectodermal and neuro-endodermal elements during week 3 of embryogenesis. Neurenteric cysts in the intracranial area are very rare lesions that typically occur in the spinal canal or even more seldom in the posterior cranial fossa. A girl presented to the outpatient clinic with complaints of moderate to severe episodes of headache with associated vomiting for 2 weeks. There was a positive sign of severe neck stiffness. Her brain MRI showed a neurenteric cyst located within the anterior intradural space of the foramen magnum. We report our experience with an in- tracranial neurenteric cyst located in the anterior brain stem area.
Key Words : Neurenteric Cyst, Headache, Brain Stem
1)
Introduction
Neurenteric cysts of the central nervous sys- tem are rare cystic lesions lined by columnar epithelium of the presumed endodermal deriva- tion. Neurenteric cysts of the central nervous system have been given a number of names, in- cluding enterogenous cysts, gastrocytomas, and intestinomas. The etiology of these cysts is un- certain, but they are generally felt to be develop- mental anomalies resulting from ectodermal- endodermal adhesions in the third week of em- bryonic life, possibly due to abnormal splitting of the notochord, with contact resulting between the endodermal and ectodermal tissues.
*
This paper was supported by Wonkwang University in 2010.
접수 : 2011년 10월 24일, 수정 : 2012년 3월 22일 승인 : 2012년 3월 30일
책임저자 : 유승택, 원광대학교 의과대학 소아과학교실 Tel : 063)859-1516, Fax : 063)853-3670 E-mail: [email protected]
Neurenteric cysts are well defined on magnetic resonance imaging (MRI). Enteric cysts are usu- ally single, smooth, and unilocular. Neurenteric cysts are usually found in the spinal canal in the lower cervical and thoracic regions, and are commonly seen in intradural and extramedullary locations. These cysts rarely, occur in the lum- bar spine or intracranially in the pons, prepontine cistern, or cerebellopontine angle, and with ex- tremely rare intracranial occurrence.
Nearly all documented cases were in the pos- terior fossa, often in the midline, and sometimes more laterally
1). Most intracranial neurenteric cysts were found in the posterior fossa of brain.
Here, we describe the case of a 10-year-old
girl who presented with severe headaches. Brain
MRI showed a neurenteric cyst located within
the anterior intradural space of the foramen
magnum. We also discuss the incidence, histo-
genesis, and classification of neurenteric cysts
and the related literature.
Fig. 1. T1, T2 sagittal images (A, B) and T1, T2 transverse images (C, D) show a well defined 1.6×1.8 cm cystic mass in the anterior foramen magnum. Note that the cyst compresses the spinal cord at the cervicomedullary junction.
Case Report
A 10-year-old girl presented to the outdoor department of pediatric neurology of Wonkwang University Hospital with complaints of moderate to severe episodes of headache and associated vomiting for 2 weeks. Headache was persistent and, pulsatile in the occipital and temporal areas with nausea and dizziness.
She did not have any significant medical his- tory of head trauma, sinus problems, seizures, or fever. Cranial nerve examination revealed no abnormalities. Cerebellar signs were absent.
There were no motor or sensory deficits but she showed severe neck stiffness. Her mother suffered from migraine. The hematological profile and endocrine workup was within normal limits.
Chest and spine radiographs did not show any
lesions. Her brain magnetic resonance imaging
(MRI) showed a 1.6×1.8 cm well-defined cystic
mass in the anterior intradural space of the
foramen magnum that was, suggestive of a neu-
renteric cyst or neurocysticercosis, a dermoid
or epidermoid cyst. This lesion did not enhance
after contrast administration. The mass com-
pressed the spinal cord at the cranio-cervical
junction of the brain stem (Fig. 1).
Fig. 2. Histopathology of a cyst showing pseu- dostratified, ciliated, columnar epithelium, which is poor in mucin-producing cells. No mesoder- mal elements such as smooth muscle, fat and ependymal or glial tissue (Type 1).
Excision of the cyst wall was performed, and her headache subsided. The cyst wall was com- pletely dissected off from the surrounding struc- tures. Histological examination revealed that the mass was a neurenteric cyst. Microscopic exa- mination revealed that the cyst wall was lined with pseudostratified cuboidal and columnar epithelium (Fig. 2). On gross inspection, the cyst wall was thin, almost avascular, and contained a small amount of pale fluid.
Discussion
Neurenteric cyst is a rare congenital lesion, which is, also known as an epithelial, entero-
genous, endodermal, bronchogenic, foregut, tera- tomatous, or respiratory cyst
1-4). In 1934, Puu- sepp reported the first case of a neurenteric cyst in a 27-year-old woman with quadriparesis
5). The cyst was found at C3-C4 level, ventral to the spinal cord
6).
Two-thirds of all neurenteric cysts occur in males
7). They are most frequently discovered in children, with 50% of patients less than 15 years, but have been reported as late as the seventh decade. However, 60% of the intracranial cysts occur in females and the average age at presentation is considerably greater.
Although the pathogenesis is unknown, an origin of endoderm that has fused with the de- veloping notochord during week 3 of gestation has been suggested
8). Normal development in the third week of embryonic life involves closure of the neurenteric canal and notochordal separa- tion from the primitive gut
9). A few hypotheses persist regarding neurenteric cyst embryoge- nesis. One of them assumes formation of an abnormal enteric diverticulum by splitting of the notochord around a prematurely fused endoderm and ectoderm, duplication of the notochord, or retention of the endodermal tissue during noto- chord formation
10-13). Neurenteric cysts in the brain originate from the most caudal aspect of the primitive intestine and contain epithelium that resembles the embryonic intestine. Intra- cranial neurenteric cysts ventral to the brainstem are rare lesions occurring in young adults. These lesions should be considered in the differential diagnosis for intracranial extra-axial cystic le- sions anterior to the brainstem
14). Bejjani et al.
identified 50 cases of endodermal cysts in the
literature
2), of which. 46 were cases of posterior
fossa endodermal cysts. In our case, the cyst
was located in the anterior intradural space of
the foramen magnum.
The walls of neurenteric cyst are lined by a combination of pseudostratified columnar epi- thelium, non-ciliated cells with microvilli and a surface granular glycocalyx coating, and mucin- containing goblet cells. Histologically, these cysts are characterized by epithelium of endodermal origin and have been classified into 3 types
15). The simpler forms (Type 1) are thin- walled, with a layer of stratified cuboidal or columnar epithelium on a basement membrane. The more complex varieties are less common and have additional mesodermal elements such as smooth muscle, fat (Type 2), and sometimes ependy- mal or glial tissue (Type 3).
Neurenteric cysts behave as space-occupying lesions that compress the nervous tissue; there- fore, clinical presentation depends on location.
Neurological deficits, in order of frequency, include motor weakness, sensory loss, and sph- incter disturbances. Symptoms are progressive in three-quarters of patients and, intermittent in the rest
16).
The diagnosis of neurenteric cysts is usually made based on preoperative radiological features and postoperative histological confirmation. Ap- pearance on MRI can vary, but MRI is the method of choice for visualizing these lesions. The lobu- lated extramedullary cysts appear iso-intense or slightly hyperintense on T1-weighted images and appear slightly hyperintense with T2-weighted sequencing
17). Computed tomography (CT) usu- ally shows a hypodense non-enhancing lesion that can be seen by angiography as avascular, MRI provides better resolution than CT
18).
Treatment should involve complete excision of the cyst. Partial excision has been reported to be useful in some cases, butneurenteric cyst are benign lesions, so surgical excision is widely
accepted as the optimal treatment
19). Total re- section of endodermal cysts in the medulla ob- longata is recommended, despite their location and adhesion to surrounding structures. Subtotal removal results in recurrence within a relatively short time period
20)- if performed before neu- rological damage. In cases where only partial excision, or puncture, is possible because of cyst position (for instance, cysts involving mul- tiple cranial nerves), the prognosis is much poorer. As recurrences may be delayed in the order of years, periodic clinical and MRI follow- up is advised for at least 10 years following sur- gery
21).
한 글 요 약
여아에서 진단된 대후두공 상부의 신경장관 낭종
원광대학교 의과대학 소아과학교실
이창우ㆍ이수호ㆍ유승택
신경장관 낭종은 원시 내배엽 배아 잔류물의 기형 으로 인해 발생한다. 대부분 저 경부와 상 흉부 분절 내 척추 관에 위치하며, 보통 경막 내부와 골수외부 에 위치한다. 두개 내 발생은 세계적으로 드물게 사 례가 보고 되고 있다. 두개 내 신경장관 낭종은 대부 분 후부오목에 발생하며 뇌 줄기의 두경부 경계 앞 부분에도 발생한다. 저자들은 대후두공 상부에 발생 한 신경장관 낭종 1례를 경험하여 이를 보고하는 바 이다.
10년 3개월 된 여아가 내원 2주전부터 후두부와
측두부에 일상생활에 지장을 줄 정도의 박동성 두통
이 지속되어 내원하였다. 두통은 아침과 오전 중에
심하였고 오심과 현기증을 동반하였다. 가족력상 어
머니가 편두통이 있었고 정상이었으며 경부강직 외
에 신경학적 검사상 이상소견은 없었다. 뇌자기공명
영상에서 1.6×1.8 cm 크기의 대후두공 상부 뇌 줄
기의 두경부 경계부위 신경장관 낭종 소견을 보였으
며 외과적인 절제 후에 증상 호전되었다. 저자들은 심한 두통을 주소로 내원하여 시행한 뇌자기공명 영 상에서 두개 내의 신경장관낭종 1례를 경험하여 이 를 보고하는 바이다.
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