신생아에서 심한 안와골 결손을 동반한 교세포 분리종...and maxillofacial region:...

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- 307 - 접수 : 2014년 10월 6일, 수정 : 2014년 10월 16일 승인 : 2014년 10월 17일 주관책임자 : 이나미, 156-755 서울시 동작구 흑석로 102 중앙대학교병원 소아청소년과 전화 : 02)6299-3181, 전송 : 02)6264-2167 E-mail : [email protected] Copyrightⓒ By The Korean Society of Perinatology 제25권 제4호, 2014 Korean J Perinatol Vol.25, No.4, Dec., 2014 http://dx.doi.org/10.14734/kjp.2014.25.4.307 바이다. 환아 : 정◯◯ 아기, 남아 주소 : 호흡곤란, 입인두와 오른쪽 볼의 종괴 출생력 및 현병력 : 재태 연령 40주 1일, 출생 체중 2,850 g, 질식분만으로 출생한 환아로 출생 직후 호흡곤란과 오른 쪽 볼에 종괴(Fig. 1)를 주소로 타 병원에서 인공 환기기를 적용 받았고 수술 위해 생후 5일째 본원에 전원되었다. 산모력 : 산모는 29세로 첫 번째 임신이었으며, 그 외에 특이 병력은 없었다. 산전검사에서 신경관 결손증이 의심 되어 양수검사를 시행 받았으나 특이소견은 관찰되지 않았 다. 이학적 소견 : 환아는 기관삽관 없이 기도유지기만 유지 한 채로 전원되었다. 출생 당시부터 오른쪽 뺨이 크게 부풀 어져 있는 상태로 부드러운 양상을 보였으며 구강에는 약 4 교세포 이소종(glial heterotopia)으로 알려져 있는 교 세포 분리종(glial choristoma)은 정상적인 뇌 조직의 종 괴 같은 병변이 뇌 이외의 다른 장소에서 나타나는 것을 의 미한다. 1-3 주로 두개 외 정중 구조물(extracranial midline structures)을 침범하며 특히 코, 목, 비인두(nasopharynx) 입인두(oropharynx) 등에 호발하나, 두개 외 비정중 구조물(extracranical non-mildline structures)을 침범 하는 경우는 드물다. 4 성인에서는 외상, 수술과 만성염증 등 의 선행요인을 가진 증례들이 보고된 바 있으나 신생아에 서는 드물게 발생하고 국내에서도 보고된 바 있다. 5, 6 이에 저자들은 비인두, 입인두, 구강과 안와를 광범위하게 침범 한 신생아 1례를 경험하였기에 문헌고찰과 함께 보고하는 신생아에서 심한 안와골 결손을 동반한 교세포 분리종 중앙대학교 의과대학 중앙대학교병원 소아청소년과 1 , 영상의학과 2 , 이비인후과 3 한석환 1 ^김태희 1 ^이나미 1 ^김재균 2 ^이세영 3 Glial Choristoma Accompanied with Severe Bony Defect in Orbit in a Newborn Suk Hwan Han, M.D. 1 , Tae Hee Kim 1 , Na Mi Lee, M.D. 1 , Jae Kyun Kim, M.D., Ph.D. 2 , and She Young Lee, M.D., Ph.D. 3 1 Departments of Pediatrics, 2 Radiology, 3 Otorhinolaryngology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea Glial choristoma is a mass-like lesion composed of otherwise normal, mature brain tissue that is isolated from the spinal cord and cranial cavity. In generally, this choristoma involves extracranial midline structures, especi- ally the nose, as well as the nasopharynx, oropharynx and cleft. Glial choristomas rarely involve extracranial non-midline structures such as the scalp, orbit, middle ear or mastoid and account for approximately 10% of all glial choristomas. Moreover, such glial choristoma presentations are usually seen in adults, most of whom have predisposing factors such as trauma, surgery, or chronic inflammation. Here, we present a case of a glial choristoma that invaded a neonate’s the nasopharynx, oropharynx, oral cavity and orbit. Key Words : Brain, Choristoma, Heterotopia, Orbit, Newborn

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- 307 -
: 2014 10 6, : 2014 10 16 : 2014 10 17 : , 156-755 102 : 02)6299-3181, : 02)6264-2167 E-mail : [email protected]
Copyright By The Korean Society of Perinatology
25 4, 2014 Korean J Perinatol Vol.25, No.4, Dec., 2014 http://dx.doi.org/10.14734/kjp.2014.25.4.307

: 40 1, 2,850
g,
(Fig. 1)
5 .
: 29 ,
.

.
.
4
(glial heterotopia)
(glial choristoma)

.1-3 (extracranial midline
structures) , , (nasopharynx)
(oropharynx) ,
(extracranical non-mildline structures)
.5, 6
, ,
1

1, 2, 3
1^1^1^2^3
Glial Choristoma Accompanied with Severe Bony Defect in Orbit in a Newborn
Suk Hwan Han, M.D.1, Tae Hee Kim1, Na Mi Lee, M.D.1, Jae Kyun Kim, M.D., Ph.D.2, and She Young Lee, M.D., Ph.D.3
1Departments of Pediatrics, 2Radiology, 3Otorhinolaryngology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea
Glial choristoma is a mass-like lesion composed of otherwise normal, mature brain tissue that is isolated from the spinal cord and cranial cavity. In generally, this choristoma involves extracranial midline structures, especi- ally the nose, as well as the nasopharynx, oropharynx and cleft. Glial choristomas rarely involve extracranial non-midline structures such as the scalp, orbit, middle ear or mastoid and account for approximately 10% of all glial choristomas. Moreover, such glial choristoma presentations are usually seen in adults, most of whom have predisposing factors such as trauma, surgery, or chronic inflammation. Here, we present a case of a glial choristoma that invaded a neonate’s the nasopharynx, oropharynx, oral cavity and orbit.
Key Words : Brain, Choristoma, Heterotopia, Orbit, Newborn
- 308 -
×4 cm
.
.
(stridor) .
.
: (magnetic reso-
nance imaging, MRI) 3.1×2.4 cm

(Fig. 2A). 2.5×2.2 cm
(multiloculated cystic mass)
(masticator space) (buccal space)
(foramen lacerum)
(Fig. 2B).
(computed tomography, CT)
(Fig. 2C).
.


(debulking surgery) (excisional biopsy)
(glial tissue)
(choroid plexus) (Fig. 3A).
(glial fibrillary acidic protein,
GFAP) (Fig. 3B).
(cystic fluid)
. (normal cellu-
larity) (atypia) (pleo-
morphism) ,
A B Fig. 1. Gross photographs taken prior to surgery. A mass at the right buccal area (A) and a mass in the oral cavity with mouth was opened (B).
A B B C Fig. 2. The patient’s radiologic findings. Magnetic resonance imagine (MRI) showing a mixed cystic and solid lesion approximately 3.1x 2.4 cm with a bony defect in the right inferior orbital wall (A). MRI showing a multiloculated cystic mass approximately 2.5x2.2 cm with peripheral enhancement in the right masticator and buccal space as well as connected with cystic lesion in right foramen lacerum on MRI (B) and a computed tomography scan showing a bony defect in the right orbit floor (C).
: - -
- 309 -


.


(marsupialization)
.
, 2-3 , ,
.

(teratoma), (glioma),
(neurofibroma), (myofibroma), (en-

.
1907 Wolbach ,
, (spinal cord)
- .8
6
(intraparenchymal lesions, dural and leptomeningeal
lesions, intracranial extracerebral lesions, midline
lesions, distal lesions of the lung and uterus, extra-
cranial non-midline lesions) .3, 4, 9

, .3, 6
,
.4, 6, 9, 10.
.5 , (middle ear),
(maxillofacial), (temporal bone)
,1, 3, 11

.
, ,


.

.
, .6, 9

,
.
(hypercellularity), (cellular pleo-
Fig. 3. Patient’s pathologic findings. The mass is composed of mature glial tissue and choroid plexus-like area with papillary formation (A, Hematoxylin-and-eosin stain, x100). Both the glial and choroid plexus-like area showing a strong positive reaction for glial fibrillary acidic protein (B, x100).
Suk Hwan Han, et al. : - Glial Choristoma Accompanied with Severe Bony Defect in Orbit in a Newborn -
- 310 -
in the middle ear and mastoid bone: a case report. J Korean Med Sci 2004;19:155-8.
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9) Shemanski KA, Voth SE, Patitucci LB, Ma Y, Popnikolov N, Katsetos CD, et al. Glial choristoma of the middle ear. Ear Nose Throat J 2013;92:555-7.
10) Wu L, Sun J, Zhang F. Glial heterotopia of the middle ear and Eustachian tube in children. Otolaryngol Head Neck Surg 2013;148:884-5.
11) Sun LS, Sun ZP, Ma XC, Li TJ. Glial choristoma in the oral and maxillofacial region: a clinicopathologic study of 6 cases. Arch Pathol Lab Med 2008;132:984-8.
12) Kallman JE, Loevner LA, Yousem DM, Chalian AA, Lanza DC, Jin L, et al. Heterotopic brain in the pterygopalatine fossa. AJNR Am J Neuroradiol 1997;18:176-9.
morphism)
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References
1) Heffner DK. Brain in the middle ear or nasal cavity: hetero- topia or encephalocele? Ann Diagn Pathol 2004;8:252-7.
2) Klein MV, Schwaighofer BW, Sobel DF, Fantozzi RD, Hes- selink JR. Heterotopic brain in the middle ear: CT findings. J Comput Assist Tomogr 1989;13:1058-60.
3) Lee JI, Kim KK, Park YK, Eah KY, Kim JR. Glial choristoma
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