Keywords
cerebellar hemorrhage - facial palsy - infant
Erratum: Neonatal Cerebellar Hemorrhage and Facial Nerve Palsy: An Unusual Association
Cerebellar hemorrhage (CH) is a frequent complication in preterm infant, and its recognition
has increased in high-risk newborn requiring intensive care due to improved neuroimaging
techniques.[1]
[2] Conversely, CH is a rare pathology in late and term infants and its incidence at
birth or shortly, thereafter, has been reported to be approximately 2/1,000 live births.[3] However, the exact incidence is unknown, and probably underestimated because only
a fraction of newborns show neurological symptoms in the first postnatal days.[4]
[5] However, Limperopoulos et al reported a case series of 17 term infants with cerebellar
hemorrhagic injury, examined by magnetic resonance imaging (MRI), over a 5-year period[6]; more recently, in a retrospective study, through MRI, Hong and Lee identified CH
in 2 out of 42 term infants with symptomatic intracranial hemorrhage over an 11-year
period.[7]
The pathogenesis of CH is poorly understood, but its etiology has been ascribed to
the distortion and rupture of veins in the subdural space, laceration of the falx
cerebri or tentorium, direct cerebellar contusion, or, less commonly, to bleeding
disorders or congenital vascular malformations.[1]
[2] Moreover, prolonged labor, precipitous delivery, instrumental delivery, primiparity,
and extreme multiparity are recognized risk factors.[8]
[9]
[10]
Symptoms typically occur within the first 24 to 36 hours of life, and are generally
represented by recurrent crisis of apnea and/or bradycardia, seizures, and signs of
obstruction to cerebrospinal fluid flow with cerebral ventricle dilation.[11]
CH may severely impair infants' neurodevelopment affecting particularly motor functions
and expressive language, and inducing behavioral disturbances.[6] On the other hand, early diagnosis of CH is critical to allow for prompt medical
treatment, such as in the case of coagulation disorders, or a neurosurgical approach,
in case of intracranial hypertension, as well as to plan a proper neurodevelopmental
follow-up.[6]
To underscore these considerations, we report the unusual case of a late preterm infant
with a peripheral facial palsy as presenting sign of CH.
Case Report
A Caucasian male was born at 360/7 weeks of gestation by vaginal delivery after a regular pregnancy with an Apgar's
score of 9 and 10 at 1 and 5 minutes, respectively. His birth weight was 2,575 g (30th
percentile). The newborn had a regular postnatal adaptation and normal physical examination.
At 24 hours of life, he showed inconsolable crying episodes but physical and neurologic
examinations, blood gas analysis, complete blood count, glycemia, sepsis workup, toxicologic
data, chest, and abdominal X-ray were all normal. However, 4 hours later, he developed
an asymmetry of the face with mouth left side deviation and incomplete right eye closure
during crying which suggested the diagnosis of right facial nerve palsy. The clinical
picture was completed by the subsequent development of a progressive axial hypotonia
and abnormal Moro's reflex. We performed a cerebral ultrasound through the anterior
and mastoid fontanelles, which revealed the dilatation of both lateral ventricles
and a diffuse hyperechoic round lesion in the right cerebellar hemisphere ([Fig. 1]). Coagulation tests were normal. A computed tomography (CT) scan confirmed a hemorrhage
in the right cerebellar hemisphere with midline shift and intraventricular bleeding
([Fig. 2]). An Ommaya's reservoir was inserted and 15 mL per day of cerebrospinal fluid were
withdrawn for 2 weeks. Postintervention MRI performed at 10 days of life showed a
slightly reduction of lateral ventricle size ([Fig. 3]). Our patient was discharged at 25 days of life and the facial palsy gradually recovered
with complete remission at 6 weeks of life. MRI scans at 6 months showed loss of substance
in the cerebellar right hemisphere and vermis ([Fig. 4]). At 9 months, the Ommaya reservoir was removed, and the baby underwent an endoscopic
third ventriculocisternostomy for progressive ventriculomegaly.
Fig. 1 Cranial ultrasound (Sonos 7500, Philips, Amsterdam, The Netherlands) through the
anterior (A) or mastoid fontanelle (B) showing dilatation of both lateral ventricles and a diffuse hyperechoic round lesion
on the right cerebellar hemisphere (arrow).
Fig. 2 Axial views of CT scan showing hyperdense round lesion on the right cerebellar hemisphere
(A), ventricular dilatation, and intraventricular bleeding (B) (arrows). CT, computed tomography.
Fig. 3 Postintervention MRI at 10 days of life. T2-weighted axial (A, B) and midsagittal view (C), showing huge round hypointense bleeding causing shift of the midline with involvement
of the vermis and ventricular bleeding (arrows). MRI, magnetic resonance imaging.
Fig. 4 MRI T2-weighted axial (A–C) and midsagittal view (D) scans at 6 months showing an isolated substance defect of the right cerebellar hemisphere
and of the vermis, and ventricular dilatation (arrows). MRI, magnetic resonance imaging.
Nearly 12 and 18 months of follow-up examinations (with the Bayley-III scales and
Gross Motor Function Classification System) demonstrated infant's motor function and
speech delay associated with hyperreflexia and tremors.
Discussion
We report the unusual case of CH with inconsolable cry and right facial nerve palsy
as presenting sign. Unilateral facial palsy as the first symptom of CH is an extremely
rare finding,[12] and can therefore lead to a lack of or delayed recognition.
The early onset of symptoms may suggest that in our case cerebellar injury occurred
during the delivery inducing progressive bleeding. The imaging and the complete remission
of palsy in few weeks' time support the hypothesis that it was caused by a transient
compression of right facial nerve at the cerebellopontine angle due to the hemorrhage.
In addition, our patient exhibited another relevant, but nonspecific symptom: an inconsolable
cry probably due to the development of posthemorrhagic ventricular dilatation, as
previously described.[5]
[12]
[13] Interestingly, the most common symptoms of CH, such as seizures, apnea, bulging
fontanelle, and abnormal level of consciousness,[5]
[7]
[13] did not occur in this case.
Recently, Mühlbacher and coworkers described a late preterm newborn who showed peripheral
facial palsy at birth due to cerebellum infarction. Unlike our case, the baby developed
focal tonic seizures contralaterally to the stroke with secondary generalization.[14]
Our patient did not present two of the most frequently reported risk factors for the
development of intracranial hemorrhage, and in particular for CH, that is, bleeding
disorders[5] or thrombocytopenia.[15] Only primiparity could be identified as a classical risk factor. However, the increased
risk of CH in nulliparous women is due to a greater occurrence of instrumental delivery
(i.e., vacuum extraction)[9]
[12], which did not occur with our patient.
The differential diagnosis of neonatal facial nerve palsy includes several pathologies,
such as perinatal trauma, intrauterine malposition, intrapartum compression, and congenital
aplasia of cranial nerve nucleus.[3] However, perinatal injuries are the most frequent causes of facial nerve palsy and
are commonly associated with birth trauma due to the relatively superficial course
of the extracranial facial nerve, meaning it is susceptible to damage during labor
and delivery. Congenital facial nerve palsies include Moebius, cardiofacial, Poland's,
and Goldenhar's syndromes.[16] As expected, perinatal posttraumatic facial nerve palsy is a self-resolving condition,
while congenital facial nerve palsy is not reversible.[3]
[16]
Our case was complicated by the development of posthemorrhagic ventricular dilation,
which was initially treated by serially withdrawing cerebrospinal fluid through an
Ommaya reservoir, and later with a third ventriculo-cisternostomy. This complication
is frequent and neurosurgical intervention has been reported in almost 25% of cases
of CH.[2]
[7]
Follow-up examinations revealed a delay in infant's motor function and speech. CH
is associated in childhood with a wide spectrum of neurodevelopmental disabilities,
such as motor, cognitive, language, and behavioral deficit—particularly in infants
with large lesions (>1 cm).[7] Thus, early diagnosis is needed to allow for adequate neurodevelopmental follow-up
and intervention to limit possible deficits.[6]
In conclusion, our case suggests that the postnatal finding of an unusual symptom,
such as facial nerve palsy, may be rarely associated with the diagnosis of CH. The
rarity of this association might make its recognition difficult but, in any case,
it should be suspected and quickly diagnosed by cerebral ultrasound to allow for proper
in-hospital management and postdischarge neurodevelopmental follow-up.
