Since the incidence of shaken baby syndrome in Switzerland was not known, we conducted a nationwide prospective follow-up study for a 5-year period (from 2002 to 2007). The data were collected through the Swiss Pediatric Surveillance Unit. Inclusion criteria were the presence, in a child under or equal to 6 years of age, more than or equal to two clinical symptoms (altered consciousness, convulsions, respiratory irregularities, and bulging fontanel), one eye finding (retinal hemorrhages, vitreous hemorrhages), one MRI/ CT finding (subdural hematoma, subarachnoid hematoma, and parenchymatous lesions), or history of shaking.
29
F o r t b i l d u n g / F o r m a t i o n c o n t i n u e Vol. 21 No. 4 2\b1\b
17/100 000 person years, compared with
1\b.3/100 000 person years for accidental
traumatic brain injury 16). The incidence is
higher in male children and in children aged
12 months and younger.
In Switzerland, there have been no previous
studies on SBS to date. The aim of the pres –
ent study was to evaluate the incidence of
SBS in Switzerland and to investigate the
main clinical symptoms present at admis –
sion and during the clinical course and to
ascertain the outcome of the victims.
Methods
Children’s hospitals in Switzerland were
invited to report all cases of confirmed
or suspected SBS to the Swiss Pediatric
Surveillance Unit (SPSU). The SPSU is an
established system for monitoring rare
pediatric conditions. All Swiss children’s
hospitals (n=36) receive a monthly card
asking them to report cases of the condi –
tions being surveyed. The hospitals’ replies
go to the SPSU, which notifies the relevant
investigator.
The study period for SBS was from 1 July
2002 to 30 June 2007. The study was ap-
proved by the Ethical Committee of the
University Children’s Hospital of Zurich.
A first questionnaire was sent to the report –
ing physician immediately after the report –
ing. This questionnaire requested details
pertaining to history, clinical presentation,
diagnosis, and management. Details about
perpetrators and crying habits were not
asked. A second questionnaire was sent
to the attending physician 9 months after
hospital admission. Clinical features, ra-
diological findings, legal proceedings, and
subsequent outcome were recorded.
The data were handled strictly anonymously.
Each child was identified by month and year
of birth and age at admission. We had no
communication with the reporting physi-
cian, and no investigations were requested.
Live birth rate was obtained from the Swiss
Federal Statistical Office.
The inclusion criteria were age <6 years,
two or more of the clinical symptoms, and/
or one eye finding, and/or one MRI/CT
finding, or shaking history (Table 1). The
exclusion criteria were age >6 years or
documented accident or disease explaining
the symptoms/findings.
Abstract
Since the incidence of shaken baby syndrome
in S\bitzerland \bas not kno\bn, \be conducted
a nation\bide prospective follo\b-up study for
a 5-year period (from 2002 to 2007). The data
\bere collected through the S\biss Pediatric
Surveillance Unit. Inclusion criteria \bere the
presence, in a child
6 years of age, of 1)
more than or equal to t\bo clinical symptoms
(altered consciousness, convulsions, respira –
tory irregularities, and bulging fontanel) 2),
one eye finding (retinal hemorrhages, vitre –
ous hemorrhages) 3), one MRI/ CT finding
(subdural hematoma, subarachnoid hema –
toma, and parenchymatous lesions), or 4)
history of shaking. Exclusion criteria \bere age
>6 years or documented accident/disease
explaining symptoms/findings. To describe
outcome, \be used the King’s Outcome Scale
for Childhood Head Injury (KOSCHI). 56
cases \bere reported from 13 of 26 S\biss
cantons, representing 80% of the S\biss popu –
lation; 49 cases met the inclusion criteria.
Preponderance of male infants \bas high (31
male and 18 female); median age at admis –
sion \bas 4 months
1)–5), 8). Clinical symptoms
\bere present in 42 infants, retinal/vitreous
hemorrhages in 39 infants, and pathological
brain/head imaging in 46 infants. In 13 cases,
the caregivers admitted shaking the child.
Outcomes (KOSCHI 1–5; n=47 patients) \bere
death (KOSCHI 1) 8 (17.7%), vegetative state
(KOSCHI 2) 0, severe disability (KOSCHI 3)
11 (22.2%), moderate disability (KOSCHI 4)
14 (31.1%), and good recovery (KOSCHI 5) 14
(28.8%). Based on these data, the incidence
of shaken baby syndrome in S\bitzerland is
14 per 100 000 live births, \bhich corresponds
to the incidence in other Western countries.
Demographic characteristics and outcomes
of S\biss patients \bere comparable to pub –
lished studies.
Introduction
In the late 1960s, Ommaya and col –
leagues 2\b), 26) reported that high-speed ro-
tational displacements of the head on the
neck at the sagittal level alone, without
direct head impact, could produce cere-
bral concussion and hemorrhages over the
surface of the brain of subhuman primates.
Attachments between the outer surface of
the brain and the inner surface of the skull
are subjected to powerful tensile and shear –
ing stresses. The cortical veins, particularly
where they enter the more fixed portions of
the dural sinuses, can tear under such con-
ditions and result in subdural hematoma.
In 1971, Guthkelch
8), a British neurosurgeon,
reported details on two infants with subdural
hematoma and no external marks of injury
on the head. Extrapolating from the previous
animal work of Ommaya et al.
2\b), 27) Guthkelch
suggested that in some cases, repeated ac –
celeration/deceleration rather than direct
violence was the cause of the hemorrhage.
Guthkelch was therefore the first to suggest
that babies were injured by shaking.
Shaken baby syndrome (SBS) is a common
and preventable cause of infant mortal –
ity and severe permanent disability
24). The
term SBS, or non-accidental head injury
(NAHI), is used for a constellation of clini –
cal symptoms and findings such as retinal
hemorrhages and subdural and/or sub –
arachnoid hematomas. Patients present
with a variety of symptoms and injuries that
may include poor feeding, vomiting, neuro –
logical findings such as irritability, lethargy,
and convulsions, posterior rib fractures,
and metaphyseal fractures. Other «red
flags» that may indicate SBS include par –
ents’ accounts of the incident that differ
over time or differ between the parents/
caregivers
7). When no history of head
trauma or only minor trauma is reported to
treating physicians, the positive predicative
value for abuse is 0.92
11).
In the USA, the incidence of NAHI in children
2 years old or younger is estimated to be
Shaken baby syndrome in Switzerland:
results of a prospe\btive follow-up study,
2002–2007*
Manuela Fanconi**, Ulrich Lips***
* This article was published in the Eur J Pediatr (2010)
169 (8): 1023–1028, ISSN 340-6199, copyright Sprin –
ger Science + Business Media, reprinted with kind
permission of Springer Science + Business Media.
** Fähnlibrunnenstrass\de \b, 8700 Küsnacht
*** University Children’s Hospital Zurich
F o r t b i l d u n g / F o r m a t i o n c o n t i n u e
30
Vol. 21 No. 4 2\b1\b
To characterize global neurobehavioral out-
come we used the King’s Outcome Scale for
Children Head Injury (KOSCHI), which is the
specific pediatric adaptation of the original
adult Glasgow Outcome Scale (GOS)
\b). The
KOSCHI «provides a practical scale for pe-
diatric head injury that will enable clinicians
to describe rate and extent of recovery
\b)».
In this five-category scale, good outcome
(KOSCHI \b) indicates to a return to age-
appropriate or preinjury level of functioning;
moderate disability (KOSCHI 4) is assigned
if the child 1) has a significant reduction in
cognitive functioning, 2) has motor deficien –
cies, or 3) was referred to outpatient reha-
bilitation therapy. Severe disability (KOSCHI
3) is assigned if 1) cognitive scores are in
the deficient range, 2) severe motor deficits
are present, or 3) the child was referred to
inpatient rehabilitation. The last two catego –
ries are KOSCHI 2, defined as vegetative
state, and KOSCHI 1, death. To
make sure not to miss victims of SBS
that died and were referred directly to insti –
tutes of pathology and forensic medicine,
we asked these institutes to report these
children to our study.
Results
Of \b6 reported cases, 49 cases of SBS met
the inclusion criteria. These 49 cases came
from 13 Swiss cantons, which is half the
total number of the cantons in Switzerland.
The total number of live births in Switzerland
during the study period was 436 970; the
Age <6years
Two or more
clinical
symptoms Bulging fontanel
Convulsions
Respiration irregularities
Altered consciousness
And/or one
eye finding Retinal hemorrhages
Vitreous hemorrhages
And/or MRI/
CT findings Subdural hematoma
Subarachnoidal hematoma
Parenchymatous lesions
Or shaking
history Person admitted shaking
child
Table 1: In\blusion \briteria
Fig. 1: Map of Switzerland with the 26 \bantons. The numbers indi\bate the number of
SBS \bases de\blared dur\ling the study period (n=49)
Fig. 2: Sex and age (n=49) 13
cantons from which there were no cases
reported represent only 20% of this total
number (Fig. 1).
Based on the total of reported cases, the in -
cidence of SBS in Switzerland is 14/100 000
live births (0.14‰).
In Switzerland 21.1% of the population do
not have Swiss citizenship (Swiss Federal
Statistical Office). Of the 49 children with
SBS here, 32 (6\b%) were Swiss citizens.
Five (10%) were from Portugal, and four (8%)
were from countries in the Balkan region.
Three (6%) were immigrants from other EU
countries, two (4%) were from Sri Lanka
and India, one (2%) child was from Iraq, one
(2%) was from Romania, and one (2%) was
from Ethiopia.
Boys were more frequently affected than
girls (31 boys and 18 girls, resp. 62% and
38%). Median age at admission was 4
months. Boys were younger (4 months;
range, 1–1\b months) than girls (\b months;
range, 1–\b8 months) (Fig. 2).
One or more clinical symptoms were found
in 42 of 49 (86%) children with SBS (Table 2) .
The Glasgow Coma Scale (GCS)
9) score on
admission was documented and available
for 38 of 49 (82%) children (26 boys and
14 girls) (Table 3). Of 49 (98%) children, 48
had an ophthalmologic examination: 46 of
49 (94%) examinations were conducted by
an ophthalmologist, one by a pediatrician
and one by a neuropediatrician. In 39 of 49
(80%) patients, hemorrhages were detected:
28 retinal and eight vitreous hemorrhages;
31
F o r t b i l d u n g / F o r m a t i o n c o n t i n u e Vol. 21 No. 4 2\b1\b
children), 17 had bruises (mostly cranial and
facial), and 16 had one or more fractures
(Table 5).
Injury mechanism and perpetrator were
known in only 18 of 49 (37%) cases. In \b
(28%) of these 18 cases, a fall was given
as the reason for the injury. In 13 of the 18
(72%) cases, the person admitted shaking
the child: Seven of these 13 persons (\b4%)
did not give any reason for doing so, \b of 13
(3 8%) stated that they shook the child in
order to resuscitate it, and one of 13 (8%)
stated that he/she shook the child in order
to calm it. These confessions and state -
ments were made during admission to the
hospital and medical history taking and not
during a legal investigation.
In total, 8 of 49 children died within days
after the shaking (mortality, 16%): four girls
and four boys. No children who died were
referred directly to institutes of pathology
and forensic medicine.
12
were bilateral. Nine children had both
retinal and vitreous hemorrhages; two of
them were bilateral (Table 4).
In 46 (94%) of 49 children, MRI and/or
CT showed brain/head injuries. Subdural
hematomas were present in 41 of 46 (89%)
cases, subarachnoidal hematomas in 11 of
46 (24%) cases. In 1\b of 46 (37%) cases,
subdural hematomas were associated with
parenchymatous lesions. Of the 41 cases
with subdural hematomas, 30 (73%) were
bilateral and 11 (27%) unilateral. In one child
(2%), there were parenchymatous lesions
without hemorrhages. Five of 46 children
(11%) had hygromas, and \b of 46 children
(11%) had brain edema. Intracerebral hemor -
rhages were present in 2 of 46 children (4%)
and intraventricular hemorrhages in one of
46 (2%) (Table 4).
No additional symptoms such as bone
fractures or bruises were found in 4\b% (22)
of the children. Of the remaining \b\b% (27
Boys
(n=31)
n Girls
(n=18)
n
Altered consciousness
2214
Respiratory
irregularities 19
8
Convulsions 179
Bulging fontanel 12\b
No symptoms \b2
Table 2: Clini\bal symptoms (n=49)
Table 3: Glasgow Coma S\bale at admission
and sex (n=40)
GCS
3
n GCS
4–7
nGSC
8–15
n
Median
value
Boys
\b \b16 10
Girls 3 477.\b
n
No additional symptoms 22
Additional symptoms 27
Bruises 17
Cranial/facial/nec\dk 1\b
Extremity 6
Chest/abdomen 6
Fractures 16
Cranium 8
Rib \b
Tibia \b
Radius 3
Femur 2
Table 5: Additional symptoms\l (n=49)
Table 8: GCS a) and KOSCHI b) (n=38) a) GCS = Glasgow Coma Scale, b) KOSCHI = King’s Out-
come Scale for Childhood Head Injury \b)
Table 7: KOSCHI a) s\bore and symptoms\l (n=39) a) KOSCHI = King’s Outcome Scale for Childhood Head Injury \b)
n Bilateral
nUnilateral
n
No hemorrhages
9
No examination 1
Hemorrhages 39
Retinal hemorrhages only 281117
Vitreous hemorrhages only 817
Combination of retinal and vitreous hemorrhages 92 7
No brain/head injuries detected 3
Brain/head injuries detected 46
Subdural hematomas 4130 11
Subarachnoid hematomas 1174
Parenchymatous lesions with hemorrhages 1714 3
Parenchymatous lesions without hemorrhages 1
Hygroma \b
Brain edema \b
Intracerebral hemorrhages 2\b
Intraventricular hemorrhages 14
Table 4: O\bular and brain im\laging findings
GCS a
3
n GCS a
4–7
n GCS a
8–15
n
KOSCHI b \b 0 19
KOSCHI
b 4 0 38
KOSCHI
b 3 2 34
KOSCHI
b 2 0 00
KOSCHI
b 1 6 20
n (%)
KOSCHI a 3 (severely disabled) 11(28)
KOSCHI
a 4 (moderately disabled) 14 (36)
KOSCHI
a \b (good outcome) 14 (36)
Table 6: KOSCHI a s\bore (n=39) a) KOSCHI =
King’s Outcome Scale for Childhood Head Injury \b)
KOSCHI a 3
n KOSCHI a 4
n KOSCHI a 5
n
Visual problems 742
Epilepsy 62 0
Pathological tonicity 7\b0
Hemiparesis 730
Pathological psychomotor development 1160
Pathological development of head circumference 420
F o r t b i l d u n g / F o r m a t i o n c o n t i n u e
32
Vol. 21 No. 4 2\b1\b
limitations that should be mentioned. Un-
derreporting and misdiagnosis remain a
major problem, since the clinical setting
and the symptoms are often non-specific.
Vomiting, fever, irritability, and lethargy
are common symptoms of a variety of con -
ditions seen in children, including head
trauma
14). Another limitation of our study is
the unexpected lack of reporting from half
of the Swiss cantons, although they repre -
sent only 20% of total Swiss population and
the children under 1 year of age living in
these thirteen cantons represent only 21%
of all children under 1 year of age living in
Switzerland. Of the 13 cantons, which did
not report, eight do not have a children’s
hospital. It is to presume that children living
in these cantons were referred to a general
practitioner or an adult clinic where symp-
toms of SBS may not be known. Lack of re-
porting may also be due to poor knowledge
of SBS symptoms, making specific informa -
tion for pediatricians important. The short
follow-up period and the small number of
victims limit our detailed knowledge of the
survivors, particularly in the less severely
injured children. Finally, knowledge about
injury mechanism and perpetrators in our
study is limited.
Prognostic factors relating to poor out-
comes emerging from our data are a lower
score on the Glasgow Coma Scale at pre -
sentation, a higher number of lesions on
imaging studies, and respiratory problems
at presentation. This agrees with findings
reported in a 2002 publication by Prasad
et al
28).
SBS is a frequent and serious public health
problem in Switzerland. Between 1 in 3000
and 1 in 4 000 children younger than 1 year
of age are victims of significant or fatal
NAHI, making SBS almost as common as
cystic fibrosis and more common than acute
lymphocytic leukemia
1), 19) . The outcome of
SBS is devastating to the child. More work
is needed to develop effective methods of
prevention, for these are potentially healthy
children prior to the injury. In a paper on
infant crying as a trigger to shaking, Barr et
al.
2) showed that the age-specific incidence
curve in SBS cases has a similar starting
point and a similar shape to the normal cry-
ing curve. The duration of crying at a given
moment seems to be less relevant than the
parents’ perception of the crying in the long
term
29). Prevention should focus especially
Detailed
follow-up was available for 39 of
the surviving 41 babies; the mean follow-up
period was 13 months (range 3–\b9 months).
Of the 39 survivors, 2\b (64%) were disabled:
11 severely (KOSCHI 3) and 14 moderately
(KOSCHI 4). 14 of 39 (36%) children had a
good outcome (KOSCHI \b) (Table 6).
The symptoms of the disabled children as-
signed KOSCHI scores 3, 4, and \b are listed
in Table 7.
In 38 of 49 cases, we had information on
the GCS score at hospital admittance.
Comparison of initial GCS scores with
KOSCHI scores shows that lower GCS
scores result in lower KOSCHI scores
(Table 8) .
Discussion
According to Kochanek et al. 19) non-acciden -
tal head injury (NAHI) is the leading cause
of death from traumatic brain/head injury
in children younger than 2 years of age. Jay-
awant et al.
13) conducted a three-year retro-
spective study of all children under the age
of 2 years with subdural hematoma in South
Wales and south west England and found a
subdural hematoma rate of 12.8/100 000
per year in children under 2 years of age; the
incidence of subdural hematoma in children
under 1 year of age was 21/100 000 per
year. The researchers estimated that NAHI
accounted for 82% of these cases.
Barlow and Minns
1) conducted an 1 8-month
prospective study of NAHI in Scotland;
they surveyed pediatric wards, pediatric
intensive care units, neurosurgical units
admitting children, the Death Register, and
the Scottish Health Information System.
There were no cases of NAHI older than 12
months. Shaken impact syndrome occurred
with an annual incidence of 24.6/100 000
children younger than 12 months.
In Estonia, Talvik et al.
30) conducted a 6-year
retrospective and prospective study survey -
ing two tertiary centers for pediatric inten-
sive care. The overall incidence of SBS was
28.7/100 000 infants up to 1 year of age.
Looking at the prospective and retrospec -
tive groups separately, the incidence was
40.\b and 13.\b/100 000 children under 1
year of age, respectively. Finally, the study
by Hobbs et al.
12) found a rate of NAHI of
7.1/100 000 under age 2 and 14.2/ 100 000
under age 1. According
to our data, the incidence in
Switzerland is 14/100 000 live births, which
is similar to the incidence obtained by the
Jayawant study and the Talvik study, higher
than that found by Hobbs, and lower than
that found in the study by Barlow and Minns.
SBS is largely restricted to children under 3
years of age, with the majority of cases oc-
curring during the first year of life
6), 9), 21) . Our
findings are consistent with previously pub-
lished data on SBS
12), 1\b), 30) that highlighted
the young age of the victims and the prepon -
derance of boys. This suggests that SBS is a
form of child abuse triggered by children’s
crying. In a study by Lee et al.
20) it emerges
that crying is an important stimulus for SBS,
especially in the first 4 months of age.
In 80% of the children in our study, ocular
hemorrhages were found, 70% of which
were related to the retina. This is close
to numbers (63.4–91%) previously report-
ed
12), 1\b), 30) .
Although non-ophthalmologists have a sur-
prisingly good success rate in detecting the
presence of these hemorrhages (up to 87%),
they often fail to describe the hemorrhages
in further detail
23). For this reason, ophthal-
mology consultation remains mandatory in
order to specify extent and details of retinal
hemorrhages
3), 4) .
The most frequent neuroradiological finding
in our study was subdural hematoma in 89%
of cases, which is similar to the rate found
in other studies: 81% of 22 SBS victims
30),
93% of 7\b SBS victims 22), and 86% of SBS
victims 17). Similar results were reported in
a postmortem study by Kivlin et al. 18) in a
series of 111 patients (92.\b%).
A study on outcomes showed that children
with SBS have a significant mortality rate,
and children who survive have serious mor-
bidity and special education needs
1\b).
In our study, we report a mortality rate of
16%, moderate to significant disability in
28–36%, and good recovery at the time
of follow-up in 36% of children with SBS.
Our data correspond with other published
stu dies that show a mortality rate ranging
from 1\b to 38%
16), moderate to significant
disability in 30–\b0%, and full recovery in
30% of children with SBS
10). However, there
is a lack of longterm outcome data in sur-
vivors of SBS, particularly in less severely
injured children.
Although the present study highlights the
devastating effects of SBS, there are several
33
F o r t b i l d u n g / F o r m a t i o n c o n t i n u e Vol. 21 No. 4 2\b1\b
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Corresponding author
Dr. Ulrich Lips
Child Protection Group, University Children’s
Hospital Zurich
Steinwiesstrasse 7\b
8032 Zurich, Switzerland
Tel. +41 44 266 73 18
ulrich.lips@kispi.uzh.ch
on
the effect of crying on parents and on
teaching parents how to cope adequately
with crying.
Acknowledgements
We thank the S\biss Pediatric Surveillance Unit and the
children’s hospitals in S\bitzerland that contributed to
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Informations complémentaires
Auteurs
Dr. med. Manuela Fanconi , Fähnlibrunnenstrasse 5, Küsnacht KD Dr. med. Ulrich Lips , ehemaliger Leiter Kinderschutzgruppe und Opferberatungsstelle, Universitäts-Kinderspital Zürich
