Neonatal Medicine

The Korean Society of Neonatology (대한신생아학회)
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A Clinical Study of Congenital Intrahepatic Portosystemic Shunt Diagnosed in Neonatal Period

신생아기에 진단된 선천성 간내 문맥 전신성 단락의 임상적 고찰과 치료

  • Kim, Ji-Young (Department of Pediatrics, Cheil General Hospital, Kwandong University College of Medicine) ;
  • Kim, Kyung-A (Department of Pediatrics, Cheil General Hospital, Kwandong University College of Medicine) ;
  • Lee, Yeon-Kyung (Department of Pediatrics, Cheil General Hospital, Kwandong University College of Medicine) ;
  • Ko, Sun-Young (Department of Pediatrics, Cheil General Hospital, Kwandong University College of Medicine) ;
  • Shin, Son-Moon (Department of Pediatrics, Cheil General Hospital, Kwandong University College of Medicine) ;
  • Han, Byung-Hee (Department of Radiology, Cheil General Hospital, Kwandong University College of Medicine)
  • 김지영 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 김경아 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 이연경 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 고선영 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 신손문 (관동대학교 의과대학 제일병원 소아청소년과) ;
  • 한병희 (관동대학교 의과대학 제일병원 영상의학과)
  • Published : 2011.05.31
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Abstract

Purpose: Congenital intrahepatic portosystemic shunts are rare disease and clinically asymptomatic shunts may be detected by chance on ultrasonogram before and after birth. We studied clinical course, treatment and prognosis of congenital intrahepatic portosystemic shunt at prenatal or neonatal period. Methods: Medical records of 8 patients which were diagnosed in intrahepatic portosystemic shunt in Cheil General Hospital from 2006 through 2010 were reviewed retrospectively. Results: Eight patients with congenital intrahepatic portosystemic shunts were identified. Six patients were diagnosed at prenatal radiological screening, including three cases of intrauterine growth restriction and two cases of preterm baby. One case with increased serum ammonia underwent coil embolization. In four cases including one case that presented elevated direct bilirubin, shunts were closed spontaneously within 11th month after birth. Two patients were diagnosed on abdominal sonogram after birth because of elevated direct hyperbilirubinemia, all of whom presented intrauterine growth restriction. Closure of shunts was confirmed during 4th month to 6th month. Conclusion: Congenital intrahepatic portosystemic shunts are clinically asymptomatic mostly and spontaneous closure is expected within 2 years age. But occasionally they have severe complication, so clinical and radiological observation is needed. Specially in cases of intrauterine growth retardation without evident cause, the possible diagnosis of congenital intrahepatic portosystemic shunts should be considered and prenatal and postnatal examination should be performed. When prenatal diagnosis is made, fetal wellbeing should be monitored periodically until spontaneous closure of shunts.

목적: 선천성 문맥 전신성 단락은 매우 희귀한 질환으로, 대부분은 증상이 없어 우연히 시행된 영상 검사를 통해서 발견되고, 심각한 합병증을 일으킬 수 있어 진단과 치료에 신중을 기해야 하는 질환이나, 재태 상태 또는 신생아기에 단락의 진단을 위한 표준화된 방법이 제시되고 있지 않은 실정이다. 이에 선천성 간내 문맥 전신성 단락이 신생아기에 복부 초음파로 확인된 8예의 임상 양상과 치료 및 예후에 대해 알아보았다. 방법: 2006년 1월부터 2010년 12월까지 관동대학교 의과대학 제일병원에서 출생한 환아 36,572명 중 산전에 단락이 진단된 신생아 6예와, 출생 후 간내 문맥 전신성 단락이 진단된 신생아 2예를 대상으로 의무 기록 정보를 후향적으로 분석하였다. 결과: 선천성 간내 문맥 전신성 단락은 해당 기간의 출생아중 8명에서 발생하였으며, 이 중 6예는 산전검사에서 진단되었으며, 2예는 출생 후 복부 초음파 검사를 시행하여 진단되었다. 산전의 진단된 6예 중 3예는 자궁 내 성장 지연을 보였으며, 2예는 미숙아였다. 1예에서 암모니아의 상승을 보여, 코일 색전술을 시행하였으며, 4예에서 생후 11개월 내에 단락의 자연 소실을 확인하였다. 출생 후 단락이 확인된 경우는 2예로 모두 자궁 내성장 지연을 보였으며, 단락은 생후 4개월과 6개월에 자연 소실된 것을 확인하였다. 고찰: 선천성 간내 문맥 전신성 단락은 생후 2년 내에 자연 소실되는 것으로 알려져 있으나, 소실 시까지 경과 관찰을 요한다. 단락은 4,500명의 출생아 중 1명에서 발생하였으며, 자궁 내 성장지연을 보인 5예를 포함한 8예에서 경험하였다. 뚜렷한 원인이 동반되지 않은 자궁 내 성장 지연을 보이는 경우, 반드시 선천성 문맥 전신성 단락을 의심하여야 하며, 산전 초음파를 시행하여 검증하여야 한다. 산전 초음파 결과 단락이 의심될 경우, 태아의 상태를 지속적으로 관찰해야 하며, 생후에도 단락의 자연 소실까지 주기적인 감시가 필요하다.

Keywords

References

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