Hindawi Publishing Corporation
Case Reports in Pediatrics
Volume 2013, Article ID 278726, 3 pages
http://dx.doi.org/10.1155/2013/278726
Case Report
Fetal Limb Ischaemia in Twin-to-Twin Transfusion Syndrome
Mark Kilby,
1,2
Rachel Pounds,
1
and Paul Mannix
3
1
School of Clinical & Experimental Medicine, College of Medical & Dental Sciences, University of Birmingham,
Birmingham B15 2TT, UK
2
Fetal Medicine Centre, Birmingham Women’s Foundation Trust, Edgbaston, Birmingham B15 2TG, UK
3
Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, UK
Correspondence should be addressed to Mark Kilby; m.d.kilby@bham.ac.uk
Received 18 September 2013; Accepted 4 November 2013
Academic Editors: R. Broadbent and D. A. Cozzi
Copyright © 2013 Mark Kilby et al. his is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Objective. To describe the rare association between prenatal vascular limb occlusion and twin-to-twin transfusion syndrome. he
Case. A woman with severe twin-to-twin transfusion syndrome was treated with fetoscopic laser ablation at 19-week gestation. At
27 weeks, the twins were delivered by an emergency caesarean section. he right arm of twin 1, the recipient twin, was noted to be
“ischaemic” and was later amputated. Conclusion. his case is unusual in that it afected the upper limb and there was no evidence
of polycythaemia, which is a suggested pathological mechanism. It was initially thought that the limb damage was due to the laser
ablation, but ater discussion with the fetal medicine team vascular limb occlusion in association with twin-to-twin transfusion
syndrome was considered. Limb ischaemia is a serious complication of twin-to-twin transfusion syndrome and is unrelated to any
form of fetal therapy. Implications. Neonatologists and paediatricians need to be aware of this association as it has medicolegal
implications and parents should be counselled as to the possible, albeit rare, occurrence, especially when twin-to-twin transfusion
syndrome is of advanced stage at presentation.
1. Introduction
Twin-to-twin transfusion syndrome (TTTS) occurs in
approximately 10% of the monochorionic twin pregnancies.
Without treatment, the fetal mortality associated with this
morbid prenatal condition is 90%, with over 50% of the
survivors having signiicant morbidity, including neurologic
and cardiovascular complications [1].
Although numerous treatment strategies have been advo-
cated, selective fetoscopic laser ablation of placental anasto-
moses appears to signiicantly improve the outcome, with the
overall outcome increasing to 64% (and at least one survivor
to 82%) [2] whilst reducing morbidity.
Prenatal ischaemic limb injury is a rare complication of
twin-to-twin transfusion syndrome that appears to occur
irrespective of management. Ischaemic limb injury can also
occur postnatally in recipient twins, despite being caused by
the TTTS in utero. Again, this is unrelated to any interven-
tions [3]. A recent multicentre cohort study from ten centres
in North America indicated that limb ischaemia has an
incidence of 0.51% and, therefore, in TTTS has a tenfold
increasing risk over uncomplicated monochorionic twins [4].
We discuss a single case study of prenatal vascular limb
occlusion associated with severe TTTS treated by fetoscopic
laser ablation.
2. Case Presentation
A 20-year-old multiparous woman (gravida 4, para 3) was
referred to a tertiary referral Fetal Medicine Centre at 19
weeks and 3 days with severe twin-to-twin transfusion syn-
drome (Quintero stage III recipient; III donor) in the UK. he
recipient twin had a maximum amniotic pool depth of 10 cm,
with intracardiac Doppler evidence of cardiac dysfunction,
whilst the donor twin had signiicant oligohydramnios (max-
imum pool depth of less than one centimetre), no fetal urine
visible in the fetal bladder, and absent end-diastolic velocity
on Doppler insonation of the umbilical artery.
Ater informed consent and counselling, the patient
underwent fetoscopic laser ablation. A 2 mm fetoscope was
introduced into the recipient twin sac under local anaesthetic
and maternal remifentanil sedation. Visualization of the
intertwin membrane was achieved and the chorionic plate
vasculature was mapped from the recipient and donor cord