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112
Pedro Moreira
Propranolol in the treatment of an extensive facial and orbital
infantile hemangioma
Figure 2. Coronal T2-weighted FSE craniofacial-orbital MRI images,
before treatment.
Figure 3. Axial T2-weighted FSE craniofacial-orbital MRI images,
before treatment.
Figure 4. Gadolinium-enhanced T1-weighted SPGR craniofacial-
orbital MRI, before treatment.
The assessment by magnetic resonance imaging
(MRI) revealed a major deep intraorbital component, and a
subcutaneous inferior periorbital component. It showed a
voluminous intra and extraconal mass replacing the orbital fat,
occupying almost the entire orbital cavity with higher expression
on the medial and inferior aspect. The mass enwrapped the
ocular globe posteriorly
, the extraocular muscles and the optic
nerve. It was found to spread to the middle cranial fossa through
the superior orbital fissure; to the pterygopalatine fossa through
the inferior orbital fissure; and to the eyelids anteriorly, with
inferior predominance (figures 2-4).
A partial limitation of opening the right eye was noted.
No relative afferent pupillary defect was detected. On ocular
examination performed under general anesthesia, the anterior
segment was normal, a mean corneal diameter of 10,5 mm was
measured in both eyes; intraocular pressure were 20 mm Hg
in the right eye and 18 mm Hg in the left eye, measured by the
Perkins tonometer; and dilated fundus examination was normal
in both eyes.
A
detailed and extensive study was conducted, initially
and during the frequent follow-up visits as required, including
blood, urinary, imaging and others investigations, following
a multidisciplinary approach, concerning evaluation and
monitoring by several specialties (Departments of Pediatrics
and Neonatology, Ophthalmology, Dermatology – Pedro
Hispano Hospital, Porto; Departments of Pediatrics/Section of
Haemato-Oncology, Pediatric Cardiology, Pediatric Surgery –
São João Hospital, Porto).
Along with the craniofacial and orbital MRI assessment,
others exams were managed. They were performed initially and
repeated as required. A blood biochemistry and haematological
profile, including a reticulocyte count and iron profile; a
coagulation study; a urinary biochemistry and sediment;
serologic and viral markers; a chest radiograph; abdominal,
renal and pelvic ultrasounds; an electrocardiogram; an
echocardiogram; an electroencephalogram; and visual evoked
potentials; all were evaluated and no relevant findings were
found.
No relevant systemic findings were found, aside from a
right inguinal hernia corrected with a surgical intervention at
the age of 6 months.
No record of relevant family or prenatal history.
At this time, with 2-months-old, he began treatment with
systemic corticosteroids (prednisolone 2,5 mg/Kg/day).
At 5-months-old, the persistence of a major intraorbital
component was found in a MRI reassessment,
despite the
relative improvement of the subcutaneous component. A
fter
the initial course of 3 months of systemic corticosteroids,
this fact was considered an unsatisfactory clinical outcome,
therefore oral propranolol treatment was started (2 mg/Kg/day,
3id) by gradual dose escalation. Systemic corticosteroids were
gradually tapered, with complete suspension at 7 months of
age.
Oral propranolol treatment was instituted initially on an
inpatient setting, taking into account a rigid protocol established
by the Departments of Pediatrics/Section of Haemato-Oncology
and Pediatric Cardiology, at São João Hospital, Porto. Posteriorly,
it was continued on an outpatient basis, also considering strict
guidelines given to the parents. The outpatient follow-up visits
were performed every 2-4 weeks by the pediatricians and every
4-8 weeks by the ophthalmologists, both of them initially more
frequent.
At the 9-week follow-up visit after initiation of
propranolol treatment and after the complete suspension of
the corticosteroid therapy, the superficial component was
significantly reduced in size and the proptosis less apparent.
At 24 months of age, 19 months after the initiation of
propranolol treatment, a successful clinical and imaging outcome
was registered. The hemangioma progressively regressed and
disappeared, documented through MRI evaluation (figures 5-7).
The
ophthalmological examination was considered normal. The
Hirschberg and Brückner tests were normal. A normal fixation
behavior was noted through the
CSM
method (Central, Steady,
Maintain).
The cover tests and the assessment of eyemovements were
normal. Another ocular
examination performed under general
anesthesia was carried out: the anterior segment was normal;
intraocular pressure was 12 mm Hg in both eyes, measured by
the Perkins tonometer; and dilated fundus examination was
normal in both eyes.
Propranolol was discontinued through gradual tapering
over a 2-month period.
During these 19 months of propranolol treatment, there
was an attempt to lower the oral dosage, but the failure to
show regression was noticed on an imaging reassessment at the
thirteenth month of treatment, so the total oral dosage (2 mg/
kg/day) was re-instituted.
There were no adverse events related to the treatment.
Currently, at the age of 3 years, he is on clinical surveillance
every 4-6 months,
and ten months after the discontinuation
of propranolol treatment, no rebound growth was registered
and the ophthalmological examination was considered normal.
Coronal
Sagital
Axial