Can J Plast Surg Vol 14 No 1 Spring 200641
Constriction band syndrome occurring in the setting
of in vitro fertilization and advanced maternal age
Brian Rinker MD, Henry C Vasconez MD
Division of Plastic Surgery, University of Kentucky, Lexington, Kentucky, USA
Correspondence: Dr Brian Rinker, Kentucky Clinic, K454, Lexington, Kentucky 40536-0284 USA. Telephone 859-323-5887,
fax 859-323-3823, e-mail firstname.lastname@example.org
B Rinker, HC Vasconez. Constriction band syndrome
occurring in the setting of in vitro fertilization and advanced
maternal age. Can J Plast Surg 2006;14(1):41-44.
The debate as to the pathogenesis of constriction band syndrome
began with Hippocrates and continues today. The exogenous theory
attributes the condition to entanglement of the fetus in the amniotic
remnants following premature rupture of the amnion, which is in
contrast to the endogenous, or genetic, mechanism.
A case of constriction band syndrome in the setting of in vitro fertil-
ization, where the child was genetically unrelated to the birth mother,
is presented. Constriction band syndrome has been reported follow-
ing amniocentesis and chorionic villus sampling, but it has not
heretofore been presented in the setting of in vitro fertilization. In
addition, the present case presents an opportunity to separate mater-
nal from genetic factors and, possibly, shed some light on the etiology
of the condition.
Key Words: Amniotic band; Congenital; Constriction band; In vitro
Maladie des brides amniotiques dans le
contexte d’une fécondation in vitro chez une
femme d’un âge assez avancé
Le débat sur la pathogenèse de la maladie des brides amniotiques a com-
mencé avec Hippocrate et se poursuit encore aujourd’hui. D’après la
théorie des facteurs exogènes, la maladie s’explique par l’enchevêtrement
du fœtus dans les débris du sac amniotique à la suite de la rupture pré-
maturée de l’amnios, théorie qui s’oppose au mécanisme endogène, ou
Voici un cas de maladie des brides amniotiques dans le contexte d’une
fécondation in vitro, dans lequel le fœtus n’avait aucun lien génétique
avec la mère qui lui a donné naissance. La malformation a déjà été asso-
ciée, dans des rapports, à l’amniocentèse et à la biopsie de villosités choriales,
mais elle n’a jamais été présentée jusqu’à maintenant dans le contexte de
la fécondation in vitro. De plus, le cas permet de distinguer les facteurs
maternels des facteurs génétiques et de mieux comprendre l’étiologie de la
onstriction band syndrome is characterized by the pres-
ence of circumferential indentations of the limbs or digits
at birth, and it is often associated with distal edema, intrauter-
ine amputations or syndactyly. The condition occurs sporadi-
cally, and its prevalence is 7.7 in 10,000 live births (1).
The etiology of constriction band syndrome is unknown but
has been the subject of debate since the time of Hippocrates
(2). The exogenous theory, proposed by Torpin (2) and Kino
(3), attributes the condition to the entanglement of the fetus
in the amniotic remnants (bands) following premature rupture
of the amnion. This is in contrast to the endogenous theory
suggested by Streeter (4), which ascribes the condition to a
focal developmental error affecting the subcutaneous germ cell
layer. More recent experimental work by Lockwood et al (5,6)
has yielded evidence for a vascular endothelial injury as the
primary endogenous mechanism.
Little is known about the risk factors for constriction band
syndrome, but maternal age has been shown to affect risk in an
inverse fashion (7,8). In addition, there have been reports
associating amniotic band syndrome with maternal trauma,
oophorectomy during pregnancy (9), intrauterine contracep-
tive device (10), amniocentesis (11-15) and chorionic villus
The purpose of the present paper was to present a recent
case of constriction band syndrome occurring in the setting of
in vitro fertilization (IVF) and advanced maternal age.
The patient was a white male born at full-term gestation,
weighing 3.04 kg. The patient’s mother was 47 years of age,
G3P2, and her pregnancy was the result of IVF and embryonic
implantation. The embryo was the product of her husband’s
sperm and a donated ovum. The egg donor was a healthy, nul-
liparous, white woman in her early 20s. The pregnancy was
complicated by hypertension and abruptio placentae. The
labour was induced at 39 weeks, and the delivery was compli-
cated by fetal intolerance of labour, prompting an emergent
cesarian section. At birth, the infant was noted to have a
lipomyelomeningocele, as well as constriction bands affecting
both hands. Apgar scores at 1 min and 5 min were 8 and 9,
respectively. The child was transferred to the neonatal inten-
sive care unit where ventilator support was required for two days.
The left hand configuration consisted of acrosyndactyly
involving the thumb, index and long fingers. The thumb and
index fingers appeared foreshortened. The right hand was
found to have acrosyndactyly involving the thumb, long, ring
©2006 Pulsus Group Inc. All rights reserved
and small fingers, and the parts were of normal size (Figure 1).
At four months of age, the child underwent the first stage of
the reconstructive sequence, consisting of surgical release of
the thumbs of both hands with full-thickness skin grafts. At
seven months of age, the child underwent release of the left
second web space syndactyly, full-thickness skin grafting and
deepening of the first web space with a four-flap Z-plasty. At
nine months of age, surgical release of the right second web
space was performed, with skin grafting and deepening of the
first web space. At seven months follow-up from the last surgi-
cal procedure, the patient was meeting his growth and devel-
opmental milestones. He was using both hands with excellent
coordination and grasp, and the aesthetic result was good
(Figure 2). Additional procedures to further deepen the first
web space, correct the left long finger constriction ring and
improve thumb opposition are planned.
The pathogenesis of constriction band syndrome is unknown.
The exogenous theory was proposed by Torpin (2), who stud-
ied more than 400 cases of constriction band syndrome.
According to Torpin, the initiating event is rupture of the
amnion without rupture of the chorion. This leads to transient
oligohydramnios due to passage of amniotic fluid through the
relatively permeable chorion. Contact of the fetus with ‘sticky’
mesoderm on the chorionic surface of the amnion would lead
to entanglement of fetal parts and skin abrasions.
Entanglement of the fetal parts would cause constriction rings
and amputations, whereas skin abrasions would lead to disrup-
tion defects, such as omphaloceles. Swallowing of the bands
would cause facial clefting (17).
Some of the clinical features of constriction band syndrome
are not explained by the exogenous mechanism, such as the
association with internal anomalies, such as tracheoesophageal
fistulas, renal agenesis and cardiac defects. There are also many
cases of constriction bands occurring in the absence of amniotic
rupture. These clinical findings led Lockwood et al (3,6) to
search for an endogenous mechanism. Experiments involving
the introduction of vasoactive substances in rats have repro-
duced external and internal features of amniotic band syn-
drome without disruption of the amnion (3,18). The proposed
pathogenesis would involve damage of the mesenchymal and
endothelial cells of the superficial vessels of the embryo and
the amnion, with disruption of epiblastic cells. This would lead
to limb amputations, constriction bands and the other findings
of constriction band syndrome (19). Amniotic band formation
would be a late and secondary event, analogous to adhesion
Rinker and Vasconez
Can J Plast Surg Vol 14 No 1 Spring 200642
Figure 1) Appearance of the hands at four months of age. A Right hand, volar view, with acrosyndactyly involving the thumb, middle, ring and small
fingers. B Right hand, dorsal view. C Left hand, volar view, with acrosyndactyly involving the thumb, index and middle fingers, and a congenital
amputation of the thumb. D Left hand, dorsal view
Constriction band syndrome is a rare condition and is, there-
fore, difficult to study in an epidemiological fashion. However, a
few large retrospective studies of risk factors have been carried
out (7,8,20,21). In three of four series, maternal age younger
than 25 years and nulliparity were found to be significant risk
factors for constriction band syndrome (7,8,20). In the fourth
series, a trend toward an inverse relationship between age and
risk were seen, which was not statistically significant (21). The
egg donor in the present case was in her early 20s and nullipari-
ous, whereas the birth mother was 47 years of age and multi-
parous. If maternal youth played a role in this case, its effect was
exerted through an endogenous, genetic mechanism.
There have been isolated case reports of constriction band
syndrome occurring following intrauterine instrumentation,
Constriction band syndrome and IVF
Can J Plast Surg Vol 14 No 1 Spring 200643
Figure 2) Appearance of the hands at 16 months of age, seven months following the third stage of the reconstructive sequence. A Right hand, volar view.
B Right hand, dorsal view. C Functional grip of the right hand. D Left hand, volar view. E Left hand, dorsal view. F Functional grip of the left hand
such as amniocentesis (11-15), chorionic villus sampling (16)
or the placement of an intrauterine contraceptive device (10).
No clear causative link has been established, but it is suggested
that the instrumentation required in the IVF procedure may
have played a role.
The debate about the pathogenesis of constriction band
syndrome began with Hippocrates and continues today.
Further experimental and epidemiological work will be
required before either the endogenous or exogenous theories
can become acknowledged as fact, and further epidemiologi-
cal work is needed before we can conclude that IVF is a risk
factor for constriction band syndrome. For the present, moth-
ers to undergo invasive intrauterine procedures should proba-
bly be counselled about the potential risk of constriction band
Rinker and Vasconez
Can J Plast Surg Vol 14 No 1 Spring 200644
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