Thoraco-abdominal aortic aneurysm rupture in a patient with Shprintzen-Goldberg syndrome

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Abstract

Shprintzen-Goldberg syndrome is a rare systemic connective tissue disorder characterized by craniosynostosis, skeletal abnormalities, infantile hypotonia, mild-to-moderate intellectual disability and cardiovascular anomalies. To our knowledge, this is the first report of a Shprintzen-Goldberg syndrome patient who developed a thoraco-abdominal aortic aneurysm. The aneurysm grew rapidly necessitating emergent thoraco-abdominal aortic replacement. The postoperative course was uneventful, and a careful lifetime follow-up was planned.

Original languageEnglish
Pages (from-to)1039-1040
Number of pages2
JournalInteractive Cardiovascular and Thoracic Surgery
Volume26
Issue number6
DOIs
Publication statusPublished - 2018 Jun 1

Fingerprint

Aortic Rupture
Abdominal Aortic Aneurysm
Craniosynostoses
Muscle Hypotonia
Intellectual Disability
Connective Tissue
Aneurysm
Shprintzen Golberg craniosynostosis

Keywords

  • Adult congenital surgery
  • Shprintzen-Goldberg syndrome
  • Vascular surgery

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

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abstract = "Shprintzen-Goldberg syndrome is a rare systemic connective tissue disorder characterized by craniosynostosis, skeletal abnormalities, infantile hypotonia, mild-to-moderate intellectual disability and cardiovascular anomalies. To our knowledge, this is the first report of a Shprintzen-Goldberg syndrome patient who developed a thoraco-abdominal aortic aneurysm. The aneurysm grew rapidly necessitating emergent thoraco-abdominal aortic replacement. The postoperative course was uneventful, and a careful lifetime follow-up was planned.",
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author = "Naritaka Kimura and Yu Inaba and Kaori Kameyama and Hideyuki Shimizu",
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AB - Shprintzen-Goldberg syndrome is a rare systemic connective tissue disorder characterized by craniosynostosis, skeletal abnormalities, infantile hypotonia, mild-to-moderate intellectual disability and cardiovascular anomalies. To our knowledge, this is the first report of a Shprintzen-Goldberg syndrome patient who developed a thoraco-abdominal aortic aneurysm. The aneurysm grew rapidly necessitating emergent thoraco-abdominal aortic replacement. The postoperative course was uneventful, and a careful lifetime follow-up was planned.

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