Korean Journal of Head & Neck Oncology (대한두경부종양학회지)

The Korean Society for Head and Neck Oncology (대한두경부종양학회)
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A Clinical Report of 9 Cases of Congenital Thyroid Dysgenesis

선천성 갑상선 발육이상 9례(例)에 대한 보고

  • Lee Samuel (Department of Surgery, Presbyterian Medical Center) ;
  • Lee Seug-Zae (Department of Surgery, Presbyterian Medical Center) ;
  • Lee Hyouk-Jin (Department of Surgery, Presbyterian Medical Center) ;
  • Chon Seong-Eun (Department of Surgery, Presbyterian Medical Center) ;
  • Park Yoon-Kyu (Department of Surgery, Presbyterian Medical Center)
  • Published : 1994.11.01
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Abstract

Congenital thyroid dysgenesis including agenesis, hypoplasia and ectopia is the predominant cause of permanent hypothyroidism. Of these, two thirds are due to an ectopic thyroid and about one third to complete thyroid agensis. From Jan. 1981 to Dec. 1992, authors experienced the 9 cases of congenital thyroid dysgenesis. Aberrent thyroid was 4 cases (44.4%), thyroid hemiagenesis with aberrent thyroid was 3 cases(33.3%) and thyroid hemiagenesis was 2 cases(22.2%). The most predominant site of aberrent thyroid is the base of tongue(85.7%). 7 patients(77.8%) revealed euthyroidism and among them, 4 patients showed elevated TSH level. Hypothyroidism was 2 patients (22.2%). 7 cases responded to thyroid suppressive therapy. 2 cases of lingual thyroid which did not responed to thyroid suppressive therapy underwent surgery and they have placed on thyroid suppressive therapy postoperatively.

1) 선천성 갑상선 발육이상 환자 9명 모두, 경부종피를 호소하였으며, 이 중 1예는 연하통을 동반하였다. 2) 선천성 갑상선 발육이상을 세분하여보면, 이소성갑상선이 44.4%,편측발육부전 및 이소성갑상선을 동반한 경우가 33.3%였고, 편측발육부전을 보인 경우가 22.2%였다. 3) 이소성갑상선을 위치별로 보면 설기저부가 6예(85.7%-1예 중복됨), 갑상설관이 2예였다. 4) 정상 갑상선기능을 보인 경우가 77.8%였고, 갑상선기능저하증을 보인 경우가 22.2%였다. 정상기능을 보인 7명의 환자 중 4명에서 갑상선자극호르몬(TSH)이 증가되어 있었다. 5) 총 9명중 7명에서는 갑상선억제요법만 시행하였고, 2명에서는 갑상선억제요법에 반응하지 않아 절제술을 시행하였으며, 2명 모두 설갑상선이었다. 수술후 계속적으로 갑상선호르몬을 복용중이다.

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