Joseph AW, et al. JAMA Facial Plast Surg. 2017;doi:10.1001/jamafacial.2017.0793.

November 21, 2017
1 min read

Considerations for patients with Ehlers-Danlos syndromes undergoing surgery


Joseph AW, et al. JAMA Facial Plast Surg. 2017;doi:10.1001/jamafacial.2017.0793.

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Plastic surgeons should consider a number of complications in patients with Ehlers-Danlos syndromes undergoing surgery.

Researchers investigating PubMed and Medline databases found 37 publications, including reviews and case series, that involved Ehlers-Danlos syndromes. They noted that patients with these syndromes may seek treatment from a plastic surgeon for excessive facial rhytids, nasal deformities and facial scarring. While serious surgical complications may occur and should be identified before surgical intervention, guidelines do not yet exist.

Results showed that these syndromes are “more common than appreciated,” according to the researchers. The overall point prevalence observed ranged from one in 2,500 to one in 5,000.

Patients with the six most commonly reported subtypes of Ehlers-Danlos syndromes may demonstrate heterogeneity in terms of phenotype. The two most commonly observed subtypes include classic and hypermobile, which were previously associated with low rates of major complications in an elective surgery setting.

However, the researchers encouraged surgeons to consider preoperative, intraoperative and postoperative complications that may occur in these patients. For preoperative counseling, an echocardiogram is recommended, and patients with mitral valve regurgitation should be treated with prophylactic antibiotics. Bleeding may also occur.

For intraoperative complications, it is recommended that tissue is handled with care. Vascular fragility should also be considered, along with wound dehiscence. Anesthetic complications include cervical spine, craniocervical and mandibular instability, the researchers wrote. Skin trauma may occur during intubation, while joint dislocation in the positioning phase should also be on the radar of clinicians.

Infections and hematomas may occur in the postoperative setting. Anticoagulation should also be considered due to bleeding risk.

“Because of their skin laxity, patients with [Ehlers-Danlos syndromes] EDSs may present more frequently to facial plastic surgeons’ offices,” the researchers wrote. “Simple screening questions can help the surgeon identify potential patients with EDSs. Treatment should be individualized and performed with extreme care to ensure optimal outcomes.” – by Rob Volansky


Disclosures: The authors report no relevant financial disclosures.