Case Report
Safe ECT in a Patient With the
Ehlers–Danlos Syndrome
*†Pascal Sienaert, MD, †Marc De Hert, MD, PhD, †Martine Houben,
*Filip Bouckaert, MD, *Sabien Wyckaert, MD, *‡Bénédicte Hagon, MD,
*André Hagon, MD, and †Jozef Peuskens, MD, PhD
Abstract
The Ehlers–Danlos syndrome is an inheritable dis-
order caused by genetic defects of collagen. Hyper-
extensibility and fragility of the skin, joint hyper-
laxity, and generalized fragility of the connective
tissues, involving the hollow organs and arteries,
are the cardinal features of this syndrome. Low
bone density has also been reported. Theoretically,
these patients can have an increased risk of rup-
ture of blood vessels and of bone fractures. A pa-
tient with a schizo-affective disorder and Ehlers–
Danlos Type I who received bifrontal ECT for a
depressive episode is described. Even without spe-
cific measures to lower blood pressure, a course of
ECT was finished without any complication.
Key Words: electroconvulsive therapy, schizoaffec-
tive disorder, Ehlers–Danlos syndrome
( J ECT 2003;19:230–233)
E
hlers–Danlos syndrome (EDS) is the most
prevalent heritable disorder of connective
tissue
1
caused by genetic defects of type I,
III, or V collagen.
2
EDS is a relatively rare
disorder, with current estimates placing the
prevalence at about 1 in 20,000.
3
Six differ-
ent types of this disorder are recognized: the
classic type (EDS I and II), the hypermobility
type (EDS III), the vascular type (EDS IV), the
kyphoscoliosis type (EDS VI), the arthrocha-
lasia type (EDS VIIB), and the dermatospar-
axis type (EDS VIIC).
4
Individuals diagnosed
with EDS types I, II, and III comprise approxi-
mately 90% of those diagnosed with this syn-
drome.
3
The different types of this syndrome
share as cardinal features hyperextensibility
and fragility of the skin, joint hyperlaxity, and
generalized fragility of the connective tissues,
involving the hollow organs and arteries.
4
Musculoskeletal problems include joint pain,
swelling and instability, and spinal deformi-
ty.
1
The classic type of EDS is characterized
by abnormal scarring, fragility of skin and
blood vessels, frequent varicose veins, and
joint laxity.
2,5
In patients with the vascular
type, severe fragility of the blood vessels
leads to excessive bruising and spontaneous
ruptures of middle-sized and large arteries,
causing catastrophic bleeding.
2,6
Life expec-
tancy can be shortened in the vascular type,
whereas in the other types life expectancy is
normal.
4
Bone is an important part of the
connective tissue group, but very little is
known about bone involvement in EDS. EDS
and osteogenesis imperfecta are closely re-
lated and are known to co-exist. The available
data suggest that some people with EDS
could have osteoporosis.
7,8
A recent study
identified a tendency of EDS patients to frac-
ture, have low bone mass, and have abnormal
bone structure.
9
Coelho and colleagues
10
found low bone density in the lumbar spine
(but not in the hip) in 4 patients with type I
EDS.
Given these theoretical data, we had to
weigh the possible benefits of ECT in a se-
verely depressed schizoaffective patient and
the possible higher risk of rupture of blood
vessels and bone fractures as a result of his
comorbid EDS. Apart from some reports on
complications during anesthetic and somatic
procedures in patients with different types of
EDS,
11
we did not find any publications to
guide our decision.
Received June 18, 2003;
accepted August 29, 2003.
From the *ECT Department,
University Centre Sint-Jozef,
Leuvensesteenweg, Kortenberg;
†Department of Psychotic
Disorders, University Centre
Sint-Jozef, Leuvensesteenweg,
Kortenberg; and ‡Department of
Anaesthesiology, CHU
Brugmann, Brussels, Belgium.
Reprints: Dr. P. Sienaert,
University Centre Sint-Jozef,
Leuvensesteenweg 517, 3070
Kortenberg, Belgium (e-mail:
pascal.sienaert@uc-kortenberg.be).
Copyright © 2003 by
Lippincott Williams & Wilkins
230
The Journal of
ECT
Volume 19, Number 4
December 2003