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Spontaneous Hemothorax in a Patient with Type IV Ehlers-Danlos Syndrome - A case report -

제4형 Ehlers-Danlos 증후군 환자에서 발생한 자발성 혈흉 - 1예 보고 -

  • Han, Kook-Nam (Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine) ;
  • Kim, Young-Tae (Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine) ;
  • Nam, Jin-Hae (Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine) ;
  • Choi, Jin-Ho (Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine) ;
  • Kang, Chang-Hyun (Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine) ;
  • Kim, Joo-Hyun (Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine)
  • 한국남 (서울대학교병원 흉부외과, 서울대학교 의과대학 흉부외과학교실) ;
  • 김영태 (서울대학교병원 흉부외과, 서울대학교 의과대학 흉부외과학교실) ;
  • 남진혜 (서울대학교병원 흉부외과, 서울대학교 의과대학 흉부외과학교실) ;
  • 최진호 (서울대학교병원 흉부외과, 서울대학교 의과대학 흉부외과학교실) ;
  • 강창현 (서울대학교병원 흉부외과, 서울대학교 의과대학 흉부외과학교실) ;
  • 김주현 (서울대학교병원 흉부외과, 서울대학교 의과대학 흉부외과학교실)
  • Received : 2009.11.06
  • Accepted : 2010.01.27
  • Published : 2010.06.05

Abstract

We successfully performed bleeding control using roll-gauze packing in a patient with Type IV Ehlers-Danlos syndrome and this patient was suffering from spontaneous hemothorax. Thoracotomy for controlling ongoing bleeding in a patient with Type IV Ehlers-Danlos syndrome should be performed as a last resort after due consideration.

제 4형 Ehlers-Danlos 증후군 혈관 침범형 환자에서 발생한 자발적 혈흉을 개흉술 후 거즈를 충전 압박하여 성공적으로 지혈하였다. 지혈이 어려울 것으로 예상되는 환자에서 지혈 목적의 개흉 수술은 최후의 방법으로 신중히 고려되어야 할 것으로 보인다.

Keywords

References

  1. Pepin M, Schwarze U, Superti FA, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 2000;342:673-80 https://doi.org/10.1056/NEJM200003093421001
  2. Paepe A. Ehlers-Danlos syndrome type IV. Clinical and molecular aspects and guidelines for diagnosis and management. Dermatology 1994;189(Suppl II):21-5 https://doi.org/10.1159/000246984
  3. Kuivaniemi H, Tromp G, Prockop DJ. Mutations in fibrillar collagens (types I, II, III, and XI), fibril-associated collagen (type IX), and network-forming collagen (type X) cause a spectrum of diseases of bone, cartilage, and blood vessels.Hum Mutat 1997;9:300-15 https://doi.org/10.1002/(SICI)1098-1004(1997)9:4<300::AID-HUMU2>3.0.CO;2-9
  4. Dominguez J, Sancho C, Escalante E, Morera JR, Moya JA, Bernat R. Percutaneous treatment of a ruptured intercostal aneurysm presenting as massive hemothorax in a patient with type I neurofibromatosis. J Thorac Cardiovasc Surg 2002;124:1230-2 https://doi.org/10.1067/mtc.2002.124264
  5. Ali HA, Lippmann M, Mundathaje U, Khaleeq G. Spontaneous hemothorax: a comprehensive review. Chest 2008;134:1056-65 https://doi.org/10.1378/chest.08-0725
  6. Faber P, Craig WL, Duncan JL, Holliday K. The successful use of recombinant factor VIIa in a patient with vaszculartype Ehlers-Danlos syndrome. Acta Anaesthesiol Scand 2007;51:1277-9
  7. Malfait F, Paepe A. Bleeding in the heritable connective tissue disorders: mechanisms, diagnosis and treatment. Blood Rev 2009;23:191-7 https://doi.org/10.1016/j.blre.2009.06.001
  8. Caceres M, Buechter KJ, Tillou A, Shih JA, Liu D, Steeb G. Thoracic packing for uncontrolled bleeding in penetrating thoracic injuries. South Med J 2004;97:637-41 https://doi.org/10.1097/00007611-200407000-00005