CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 4, July/August 2014
e10
AFRICA
Case Report
Anesthetic management of a newborn with trisomy 18
undergoing closure of patent ductus arteriosus and
pulmonary artery banding
Oguzha Arun, Bahar Oc, Mehmet Oc, Ates Duman
Abstract
Background:
Peri-operative management of infants with
trisomy 18 syndrome is challenging due to various congenital
cardiac and facial anomalies.
Case report:
We report the anaesthetic management of a
13-day-old neonate with 1 540 g body weight, undergoing
closure of patent ductus arteriosus and pulmonary artery
banding. Anaesthesia was induced with sevoflurane, fentanyl
and rocuronium. Despite dysmorphic facial features, ventila-
tion and endotracheal intubation were achieved uneventfully.
Anaesthesia was maintained with sevoflurane and fentanyl
and was uneventful. The patient was transferred to the neona-
tal ICU intubated and with ventilatory support. The baby was
extubated on the second day postoperatively.
Conclusion:
Our knowledge of the proper anaesthetic tech-
nique for children undergoing palliative or corrective surgery
is limited. Further case reports will increase our experience in
peri-operative management of children with trisomy 18.
Keywords:
Edwards syndrome, trisomy 18, newborn, anaesthesia
Submitted 28/6/13, accepted 6/5/14
Cardiovasc J Afr
2014;
25
: e10–e12
DOI: 10.5830/CVJA-2014-024
Trisomy 18 syndrome, first described by Edwards in 1960, is
characterised by an autosomal chromosomal disorder with
multiple congenital anomalies.
1
The syndrome consists of major
and minor anomalies, prenatal and postnatal growth deficiency,
and an increased risk of neonatal and infant mortality. It is the
second most common autosomal trisomy syndrome after trisomy
21, and the live-birth prevalence of trisomy 18 is estimated at
between 1/3 600 and 1/10 000. The overall prevalence is higher
(1/2 500–1/2 600) due to the high frequency of foetal loss and
pregnancy termination after prenatal diagnosis.
2
Structural heart defects occur in over 90% of infants with
the syndrome, and the most common cardiac lesions are atrial
septal defect (ASD), ventricular septal defect (VSD), patent
ductus arteriosus (PDA) and polyvalvular disease.
3
There is
some controversy regarding offering cardiac surgery to patients
with trisomy 18 because of the elevated risk of mortality
during surgery and in the first month of life, and the presence
of significant developmental disability in surviving children. If
scheduled for surgery, the peri-operative management of these
children is challenging.
We report on the anaesthetic management of a newborn
with Edwards syndrome undergoing closure of patent ductus
arteriosus and pulmonary artery banding.
Case report
The patient was a 13-day-old girl born at 36 weeks and 4 days of
gestation with 1 540 g body weight. She had three healthy sisters
and there was not any consanguinity between her parents. Foetal
growth retardation, single umbilical artery, and polyhydramnios
were detected with ultrasonography during the second and
third trimester follow ups. After a successful caesarean section,
the first-, fifth-, and tenth-minute APGAR scores were 5,
7 and 10, respectively. At birth she had dysmorphic facial
features including microcephaly, malformed ears, micrognathia,
redundant skin at the neck, and hypotonia (Figs 1, 2). Karyotype
analysis revealed trisomy 18.
Structural heart defects such as atrial and ventricular septal
defects and patent ductus arteriosus; bilateral hydronephrosis
and hydro-ureter in the right kidney, and mega cisterna magna
variation in the central nervous system were also present. She
was transferred to the neonatal intensive care unit (NICU) and
given daily digitalis and antibiotic therapy. Despite two doses of
intravenous indomethacin treatment, the patient’s pulmonary
pressure increased and her general condition gradually worsened.
Echocardiography revealed a left atrium/aorta ratio (LA/Ao) of
1.4, PDA diameter of 3–4 mm, systolic pulmonary arterial
pressure of 55 mmHg and enlargement of the left ventricle.
Cardiovascular surgeons, paediatric cardiologists and the
patient’s parents were invited for consultation. Closure of
the PDA and pulmonary banding were expected to reduce
pulmonary blood flow and pulmonary artery pressure. The
parents were informed that closure of the PDA and pulmonary
Department of Anesthesiology and Reanimation, Faculty
of Medicine, Selcuk University, Konya, Turkey
Oguzhan Arun, MD,
Bahar Oc, MD
Ates Duman, MD
Department of Cardiovascular Surgery, Faculty of
Medicine, Selcuk University, Konya, Turkey
Mehmet Oc, MD
