CLOVES syndrome with thoracic and central phlebectasia: Increased risk of pulmonary embolism

Alomari, Ahmad I.; Burrows, Patricia E.; Lee, Edward Y.; Hedequist, Daniel J.; Mulliken, John B.; Fishman, Steven J.

doi:10.1016/j.jtcvs.2010.04.023

« Previous Next »The Journal of Thoracic and Cardiovascular Surgery
Volume 140, Issue 2 , Pages 459-463, August 2010

CLOVES syndrome with thoracic and central phlebectasia: Increased risk of pulmonary embolism

Ahmad I. Alomari, MD
- Division of Vascular and Interventional Radiology, Children's Hospital Boston and Harvard Medical School, Boston, Mass
- Address for reprints: Ahmad I. Alomari, MD, Division of Vascular and Interventional Radiology, Children's Hospital Boston and Harvard Medical School, 300 Longwood Ave, Boston, MA 02115.
Patricia E. Burrows, MD
- Division of Vascular and Interventional Radiology, Texas Children's Hospital, Houston, Tex
Edward Y. Lee, MD, MPH
- Department of Radiology, Children's Hospital Boston and Harvard Medical School, Boston, Mass
Daniel J. Hedequist, MD
- Department of Orthopedic Surgery, Children's Hospital Boston and Harvard Medical School, Boston, Mass
John B. Mulliken, MD
- Department of Plastic Surgery, Children's Hospital Boston and Harvard Medical School, Boston, Mass
Steven J. Fishman, MD
- Department of Surgery, Children's Hospital Boston and Harvard Medical School, Boston, Mass

Received 20 October 2009; received in revised form 7 April 2010; accepted 25 April 2010. published online 31 May 2010.

Objective

CLOVES syndrome (congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal/scoliosis and spinal abnormalities) is a rare, complex overgrowth syndrome with serious morbidity. In this communication we studied the presence of central and thoracic phlebectasia in patients with CLOVES syndrome and its clinical implications.

Methods

We conducted a comprehensive search of our databases at Children's Hospital Boston over the last 10 years (1999–2008) for patients with CLOVES syndrome and central and thoracic phlebectasia. Medical records, clinical photographs, and imaging studies of varying modalities were reviewed.

Results

Review of the clinical data and imaging studies of 12 patients with CLOVES syndrome documented the presence of central and thoracic phlebectasia in 11 patients. Two patients had serious perioperative pulmonary embolism, and 1 died.

Conclusions

Central and thoracic phlebectasia in patients with CLOVES syndrome is common and increases the risk of pulmonary embolism. Aggressive prophylactic measures should be considered before major interventions.

CTSNet classification: 11, 36

Abbreviations and Acronyms: CLOVES, congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal/scoliosis and spinal abnormalities, CT, computed tomography, IVC, inferior vena cava, SVC, superior vena cava