갑상선 반쪽 무형성증 1례

A Case of Thyroid Hemiagenesis

  • 김준성 (울산대학교 의과대학 울산대학교병원 소아과학교실) ;
  • 이경연 (울산대학교 의과대학 울산대학교병원 소아과학교실) ;
  • 김자형 (울산대학교 의과대학 울산대학교병원 소아과학교실) ;
  • 박상규 (울산대학교 의과대학 울산대학교병원 소아과학교실) ;
  • 정진영 (울산대학교 의과대학 울산대학교병원 소아과학교실) ;
  • 오기원 (울산대학교 의과대학 울산대학교병원 소아과학교실)
  • Kim, Joon-Sung (Department of Pediatrics, Ulsan University Hospital, University fo Ulsan College of Medicine) ;
  • Lee, Kyung-Yeon (Department of Pediatrics, Ulsan University Hospital, University fo Ulsan College of Medicine) ;
  • Kim, Ja-Hyeong (Department of Pediatrics, Ulsan University Hospital, University fo Ulsan College of Medicine) ;
  • Park, Sang-Kyu (Department of Pediatrics, Ulsan University Hospital, University fo Ulsan College of Medicine) ;
  • Jeong, Jin-Young (Department of Pediatrics, Ulsan University Hospital, University fo Ulsan College of Medicine) ;
  • Oh, Ki-Won (Department of Pediatrics, Ulsan University Hospital, University fo Ulsan College of Medicine)
  • 발행 : 2009.11.30

초록

갑상선 반쪽 무형성증은 갑상선의 한쪽 엽이 형성되지 않는 드문 선천성 갑상선 질환이다. 갑상선 반쪽 무형성증 자체는 대개 아무런 임상 증상을 초래하지 않으므로 대부분은 동반된 갑상선 질환을 진단하는 과정이나 수술 중에 우연히 발견된다. 저자들은 선천성 갑상선기능저하증이 의심되어 내원한 1개월된 영아에서 원인을 찾던 중에 갑상선 초음파 및 스캔 검사를 통해 갑상선 반쪽 무형성증을 진단하였기에 보고하는 바이다.

Thyroid hemiagenesis is a rare congenital anomaly in which one thyroid lobe fails to develop. Thyroid hemiagenesis usually does not cause clinical symptoms by itself, therefore, this anomaly is detected incidentally during the evaluation of other thyroid disorders. We describe a rare case of thyroid hemiagenesis in a 1-month-old female infant who presented with prolonged jaundice and abnormal laboratory findings of congenital hypothyroidism. The patient showed the characteristic features of thyroid hemiagenesis of the left lobe in Tc-99m pertechnetate scintigraphy and ultrasonography of the thyroid gland. The patient has improved with supportive care, including thyroid hormone replacement. Further long-term follow-up is required for the investigation of recurrence of thyroid abnormalities.

키워드

참고문헌

  1. Shabana W, Delange F, Freson M, Osteaux M, De Schepper J. Prevalence of thyroid hemiagenesis: ultrasound screening in normal children. Eur J Pediatr 2000;159:456-8. https://doi.org/10.1007/s004310051307
  2. Maiorana R, Carta A, Floriddia G, Leonardi D, Buscema M, Sava L, et al. Thyroid hemiagenesis: prevalence in normal children and effect on thyroid function. J Clin Endocrinol Metab 2003;88:1534-6. https://doi.org/10.1210/jc.2002-021574
  3. Korpal-Szczyrska M, Kosiak W, Swieton D. Prevalence of thyroid hemiagenesis in an asymptomatic schoolchildren population. Thyroid 2008;18:637-9. https://doi.org/10.1089/thy.2007.0408
  4. Mikosch P, Gallowitsch HJ, Kresnik E, Molnar M, Gomez I, Lind P. Thyroid hemiagenesis in an endemic goiter area diagnosed by ultrasonography: report of sixteen patients. Thyroid 1999;9:1075-84. https://doi.org/10.1089/thy.1999.9.1075
  5. Melnick JC, Stemkowski PE. Thyroid hemiagenesis (hockey stick sign): a review of the world literature and a report of four cases. J Clin Endocrinol Metab 1981;52:247-51. https://doi.org/10.1210/jcem-52-2-247
  6. Lee YS, Yun JS, Jeong JJ, Nam KH, Chung WY, Park CS. Thyroid hemiagenesis associated with thyroid adenomatous hyperplasia and papillary thyroid carcinoma. Thyroid 2008;18:381-2. https://doi.org/10.1089/thy.2007.0281
  7. Park IH, Kwon SY, Jung KY, Woo JS. Thyroid hemiagenesis: clinical significance in the patient with thyroid nodule. J Laryngol Otol 2006;120:605-7.
  8. Kim KH, Chung HP, Kim JW. Koh YW. Three cases of thyroid hemiagenesis. Korean J Head Neck Oncol 2006;22:183-7.
  9. Lee SH, Yang YS, Hong KH, Oh JK. A case of lingual thyroid with unilateral thyroid agenesis and contralateral goiter.Korean J Otolaryngol- Head Neck Surg 2005;48:271-4.
  10. Abbassi V, Auth R. Hemiagenesis of the thyroid: clinical and radiological presentation in the pediatric patient. J Nucl Med 1983;24:972-3.
  11. Nebesio TD, Eugster EA. Unusual thyroid constellation in Down syndrome: congenital hypothyroidism, Graves' disease, and hemiagenesis in the same child. J Pediatr Endocrinol Metab 2009;22:263-8.
  12. Sari O, Ciftci I, Toru M, Erbas B. Thyroid hemiagenesis. Clin Nucl Med 2000;25:766-8. https://doi.org/10.1097/00003072-200010000-00002
  13. Yildiz M, Bozkurt MK. Thyroid hemiagenesis. J Adolesc Health 2003;33:291-2. https://doi.org/10.1016/S1054-139X(03)00132-0
  14. Calaciura F, Motta RM, Miscio G, Fichera G, Leonardi D, Carta A, et al. Subclinical hypothyroidism in early childhood: a frequent outcome of transient neonatal hyperthyrotropinemia. J Clin Endocrinol Metab 2002;87:3209-14. https://doi.org/10.1210/jc.87.7.3209
  15. Eugster EA, LeMay D, Zerin JM, Pescovitz OH. Definitive diagnosis in children with congenital hypothyroidism. J Pediatr 2004;144:643-7. https://doi.org/10.1016/j.jpeds.2004.02.020
  16. Bando Y, Nagai Y, Ushiogi Y, Toya D, Tanaka N, Fujisawa M. Development of Graves' hyperthyroidism from primary hypothyroidism in a case of thyroid hemiagenesis. Thyroid 1999;9:183-7. https://doi.org/10.1089/thy.1999.9.183