Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Two cases of congenital atretic encephalocele misdiagnosed as dermoid cyst

유피낭종으로 오인된 atretic encephalocele 2례

  • Kim, Jae-Hui (Department of Pediatrics, College of Medicine, Gyeongsang National University) ;
  • Cho, Jae-Min (Department of Radiology, College of Medicine, Gyeongsang National University) ;
  • Jung, Jin-Myung (Department of Neurosurgery, College of Medicine, Gyeongsang National University) ;
  • Park, Eun-Sil (Department of Pediatrics, College of Medicine, Gyeongsang National University) ;
  • Seo, Ji-Hyun (Department of Pediatrics, College of Medicine, Gyeongsang National University) ;
  • Lim, Jae-Young (Department of Pediatrics, College of Medicine, Gyeongsang National University) ;
  • Park, Chan-Hoo (Department of Pediatrics, College of Medicine, Gyeongsang National University) ;
  • Woo, Hyang-Ok (Department of Pediatrics, College of Medicine, Gyeongsang National University) ;
  • Youn, Hee-Shang (Department of Pediatrics, College of Medicine, Gyeongsang National University)
  • 김재희 (경상대학교 의과대학 소아과학교실) ;
  • 조재민 (경상대학교 의과대학 방사선과학교실) ;
  • 정진명 (경상대학교 의과대학 신경외과학교실) ;
  • 박은실 (경상대학교 의과대학 소아과학교실) ;
  • 서지현 (경상대학교 의과대학 소아과학교실) ;
  • 임재영 (경상대학교 의과대학 소아과학교실) ;
  • 박찬후 (경상대학교 의과대학 소아과학교실) ;
  • 우향옥 (경상대학교 의과대학 소아과학교실) ;
  • 윤희상 (경상대학교 의과대학 소아과학교실)
  • Received : 2006.04.05
  • Accepted : 2006.07.10
  • Published : 2006.09.15
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Abstract

Atretic cephalocele is a degenerative form of encephalocele, which is detected as a cystic mass in the head, primarily in infants. Its presentation and prognosis vary and depend on various factors, including the nature of the tissues within the cyst, other concomitant anomalies, the site of development, and the presence or absence of an embryonic straight sinus. We here report 2 cases of atretic encephalocele, that were transferred to our hospital because round tumors, misdiagnosed as dermoid cysts, were detected in their parietal lobes immediately after birth. On diagnostic and differential MRI, an embryonic straight sinus was detected while histochemical results indicated that the lesions contained cerebral tissues. Despite these structural anomalies, the two patients developed normally neurologically and no other anomalies were detected. We here discuss these two cases and present a review of the relevant literature.

Atretic encephalocele은 1972년 James와 Lassmann에 의해 meningocele manque로 처음 보고 되었고 뇌류의 퇴행성 형태로 정의하였다. 이후로 atretic, occult, abortive, rudimentary cephalocele 등의 다양한 명칭으로 명명되었으며 포함된 조직, 동반 기형, 발생 위치, 배아성 직정맥동의 유무 등에 따른 차이점들에 대해 다양하게 보고 되어왔다. 두정엽의 후부에 낭성 종물을 주소로 입원한 환아의 진단 과정에서 두부 초음파, CT, MRI를 시행하였으며 그 과정에서 MRI가 진단에 가장 효과적인 영상을 제공하였다. 환아 진단 후 수술적 방법으로 종물을 제거하였으며 수술 중 이상 소견 및 추후 관찰 기간 동안 이상 소견관찰되지 않았다. Atretic encephalcele의 희귀함과 진단 방법의 장단점 및 위치상 특성과는 다르게 동반 기형 및 발달 장애가 관찰되지 않은 증례를 치험 하였기에 보고하는 바이다.

Keywords

References

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