eMedicine Specialties > Dermatology > Pediatric Diseases

Ehlers-Danlos Syndrome: Follow-up

Author: Enrico Ceccolini, MD, Consulting Staff, Department of Dermatology, University of Bologna, Italy
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Feb 18, 2009

Follow-up

Further Inpatient Care

  • Type IV Ehlers-Danlos syndrome patients should be carefully monitored because they are at high risk for spontaneous rupture of a large artery (eg, splenic artery, aorta) or perforation of internal organs. These patients should be educated that surgery can pose life-threatening risks.
  • Fernandez-Alcantud reviewed management of anesthesia in vascular type IV Ehlers-Danlos syndrome patients.24
  • Jones reports the use of local anesthesia for elective cesarian delivery in a type III Ehlers-Danlos syndrome woman.25

Deterrence/Prevention

  • Patients should avoid trauma and participation in contact sports.
  • Pregnancy is dangerous for some patients.
  • Bleeding risk should be considered in surgical operations.

Prognosis

  • Ehlers-Danlos syndrome type IV is a severe form, and patients with this disease often have a shortened lifespan.
    • Arterial aneurysms, valvular prolapse, and spontaneous pneumothorax are common complications.
    • The prognosis with this type is poor.
    • Sudden death can occur after visceral perforation or after the rupture of a large vessel, most commonly an abdominal and splenic vessel.
  • The other types usually are not as dangerous, and affected individuals can live healthy if somewhat restricted lives.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • This diagnosis can be important, especially with regard to the severe form, type IV, in which skin also is fragile but not extensible.

Special Concerns

  • Pregnancy is dangerous for some patients.
 


More on Ehlers-Danlos Syndrome

Overview: Ehlers-Danlos Syndrome
Differential Diagnoses & Workup: Ehlers-Danlos Syndrome
Treatment & Medication: Ehlers-Danlos Syndrome
Follow-up: Ehlers-Danlos Syndrome
Multimedia: Ehlers-Danlos Syndrome
References
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References

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Further Reading

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Keywords

Ehlers-Danlos syndrome, EDS, tenascin-X deficiency syndrome, lysyl hydroxylase deficiency syndrome, inherited connective tissue disorders, inherited connective-tissue disorders, connective-tissue disorder, connective tissue disorder

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Contributor Information and Disclosures

Author

Enrico Ceccolini, MD, Consulting Staff, Department of Dermatology, University of Bologna, Italy
Enrico Ceccolini, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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