David W. Hollister M.D.
Reprinted from the Journal of Reproductive Medicine Vol. 36, No. 8, August 1991, pp. 622-24
Permission to reprint this article was granted by Dr. Elmar P. Sakala. A. Appearance
Ehlers-Danlos syndrome (EDS) is a heterogeneous connective tissue
disorder rarely complicating pregnancy. Some types are associated with
high maternal mortality, while others appear to be more benign. Two
term, vaginally delivered pregnancies with good outcomes from one
patient were managed with EDS type III. The patient experienced no
significant morbidity attributable to the EDS. The only other
previously reported case of EDS type III involved serious problems with
joint laxity and pain morbidity, requiring preterm cesarean delivery.
Ehlers-Danlos syndrome (EDS), a rare inherited connective tissue
disorder, has been associated with numerous pregnancy complications
ranging from increased bruising to maternal death. At least 10
varieties of EDS (types I-X) have been identified, based upon either
biochemical defects or clinical findings. The likelihood and severity
of pregnancy complications appear to vary according to the EDS type.
Not all types of EDS are necessarily associated with unacceptable
morbidity or mortality rates. However, much of the literature does not
identify the EDS type when reporting on complications of pregnancy.
There is a need for reporting type-specific outcomes in order to
provide accurate pre-pregnancy counseling for EDS patients as well as
reliable information for optimizing management of pregnancies when they
A 29-year-old, white woman, was diagnosed at age 19 as having EDS
type III during an evaluation for chronic temporomandibular joint
subluxation subsequently treated with temporal tendon scarification.
The patient described a history of excessive joint mobility noted first
at age 11 months, after dislocating her elbow in falling from her
playpen. The patient was able to dislocate her right knee while nearly
standing still and was able to dislocate both hips spontaneously. She
had undergone multiple knee operations because of injuries incurred
during gymnastic activities but had not had bleeding complications.
Excessive joint laxity was confirmed on the physical examination. The
skin was easily stretched, although the vasculature could not be seen
The patient's mother and maternal grandmother both had
dislocatable joints as well as a history of arthritis. Two siblings
gave histories of multiple joint dislocations and joint hypermobility.
Another sibling had a cerebral arteriovenous malformation with a bruit.
The patient's first pregnancy, at age 24, resulted in
an uncomplicated, spontaneous, first-trimester abortion without
The patient's second pregnancy occurred at age 26. With
a height of 67 inches and pre-pregnancy weight of 143 pounds, she
gained only 14 pounds. She received genetic counseling and was informed
that the risk of her child's being affected was 50:50. The patient was
hospitalized at 21 weeks gestation for severe right lower quadrant pain
that resolved spontaneously and was diagnosed subsequently as round
ligament syndrome. After gestational diabetes was diagnosed at 30 weeks
gestation, the blood glucose values were maintained within the desired
range with diet alone. Serial sonograms demonstrated normal fetal
growth, and weekly non-stress tests from 32 weeks gestation were
reassuring. The patient experienced no activity limitations and
ambulated up to the day of delivery.
Labor was induced at 40 weeks gestation after the
patient's membranes ruptured spontaneously. A 2-hour, 30-minute active
phase and a 1-hour second stage led to the spontaneous vaginal delivery
of a healthy, 2,838 gram, female infant with Apgar scores of 8 and 9. A
midline episiotomy was repaired without an excessive blood loss. The
wound healing was unremarkable.
The pregnancy weight gain for the patient's third
pregnancy, at age 28, was 23 pounds. A level II obstetric sonogram
showed no fetal anomalies. Genetic amniocentesis was offered, but the
Pre-term contractions with minimal cervical dilation
were noted at 30 weeks gestation, leading to hospitalization,
parenteral (intravenous) tocolytic (an agent that arrests uterine
contractions) administration and subsequent outpatient oral terbutaline
management. A diabetes screen was negative. Although ambulation was
restricted because of pre-term contractions, the patient's ability to
walk was not hampered by her joint problems. The tocolytic agents were
discontinued at 36 weeks. Spontaneous labor began at 38 weeks. After a
2-hour, 30-minute active phase and a 1-hour, 23-minute second stage,
the patient underwent spontaneous vaginal delivery of a healthy, 3,200
gram, male infant with Apgar scores of 9 and 9. A midline episiotomy
was repaired without excessive blood loss. The wound healed without
The incidence of EDS in pregnant women has been estimated at 1 in
150,000 (1981). Types I-III are the most common, with the percentage of
EDS cases approximately 30 for each (1987). Type IV is found in 10% of
cases, with the remaining 6 types exceptionally rare (1987).
It is important to identify the type and severity of
risks for pregnancies complicated by the four most common types of EDS.
"Gravis" type I EDS, is associated with tissue fragility, hemorrhage
and poor wound healing. Maternal mortality with "ecchymotic" type IV
EDS, is quoted to be as high as 25% because of arterial rupture. Since
types I and IV are the most severe forms of EDS, some authors have
suggested that perhaps pregnancy should be contraindicated in those
patients. Reports on "mitis" type II EDS patients, describe a
relatively benign obstetric course with complications primarily from
tissue laxity and limb mobility. Advice to avoid pregnancy does not
appear warranted because of type II complications.
The only previously reported case of "benign,
hypermobile" type III EDS in pregnancy, involved a patient who had
progressive knee instability and sacrolumbar nerve root pain from a
prolapsed intervertebral disk. She was hospitalized because of the
severe back pain at 27 weeks and managed with bed rest, analgesics,
pelvic traction and prophylactic heparin. She underwent a scheduled
cesarean delivery at 35 weeks gestation because of uncontrollable pain.
Her post-operative healing and recovery were without complications. The
authors suggested that type III EDS patients can expect worsening
symptoms, possibly requiring a hospital admission.
The experience of our patient, with type III EDS, was
far more benign. While she did experience pregnancy complications, she
had no significant problems secondary to the EDS. She was able to
ambulate throughout the pregnancy and experienced no more skeletal
discomfort than does the normal pregnant woman. In spite of pre-term
contractions, her cervix did not dilate significantly before term. It
is remarkable that her active phase of labor, even with the first
delivery, was only 2.5 hours. We did not encounter extensive
lacerations, delayed wound healing or postpartum hemorrhage.
While the low incidence and multiple subtypes of EDS
preclude the existence of a large series from which to develop
pregnancy management guidelines, we suggest that the obstetric course
of women with type III EDS may result in well-tolerated pregnancies,
successful vaginal delivery and a favorable outcome for both mother and
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