IMAGING FEATURES OF TUMOR EXOPHTALMOS IN CHILDREN

1
IMAGING FEATURES OF TUMOR EXOPHTALMOS IN
CHILDREN

• M. LIMEME, H. ZAGHOUANI BEN ALAYA, S. KRIAA, H.
  AMARA, D. BEKIR, CH. KRAIEM
• Imaging department, Farhat Hached Hospital,
  Sousse, Tunisia

PED16
2
Introduction

• Clinical signs are frequent in children
  exophtalmos.
• Etiology predominant tumor.
• Imaging diagnostic etiology.

3
Introduction

• The malignancy is rare (20) and includes
  primitives tumors (retinoblastoma,
  rhabdomyosarcoma) and metastasis.
• The benign pathology, the most frequent (80), is
  varied. The dermoid cyst represents almost half
  of the orbital masses.

4
Objectives

• Show the interest of the imaging methods,
  particularly computed tomography (CT) and
  magnetic resonance imaging (MRI), in the
  exploration of the tumor exophthalmos in
  children.

5
Materials and Methods

• 14 cases of tumor exophthalmos in children were
  retrospectively reviewed.
• They were collected in our department over a
  period of two years (2008-2010).
• 12 were explored by a cranio-orbital CT
• MRI was performed in 12 patients.

6
Results

• The average age of patients was 6 years.
• An unilateral involvement was noted in 93 of
  cases.
• 89 of cases of tumor exophthalmos due to
  malignant tumors. The most common pathologic type
  is the retinoblastoma (5 cases) followed by the
  rhabdomyosarcoma (3 cases).
• Others are less common pathological types
  capillary hemangioma (1 cases), optic nerve
  glioma (1 cases), lymphoma (1 cases), orbital
  metastasis of a neuroblastoma (1 cases), leukemia
  (1 case), histiocytosis X (1 case).
• Radiological features were various, depending on
  the etiology.

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Discussion
8
Retinoblastoma

• Malignant eye tumor of children
• Average age between 1 and 2 years.
• Clinical signs- Leukocoria - Strabismus-
  Ocular inflammatory signs
• Ultrasound- Small solid tumor attached to the
  retina, well limited.- Large tumor with
  multifocal hyperechoic areas (calcifications).-
  Retinal detachment or hemorrhage.

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Retinoblastoma

• CT- Small tumor against the wall with or
  withoutretinal detachment.- Disseminated tumor
  in the vitreous.- Intense enhancement.-
  Calcifications single or multiple.-
  Locoregional and distance extension.
• MRI- Iso or T1 hyperintense, T2 hypointense.-
  Heterogeneous enhancement.- Extending around the
  eye.

10
A four month old infant Left leukocoria and
exophtalmos
Axial contrast-enhanced CT scan with a
parenchymal window The left vitreous is
heterogeneous with calcifications and retinal
detachment (red arrow). Enhancement with
asymmetric enlargement of the left optic
nerve (blue arrow).
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A
B
C
MRI patterns. Axial T2 (A) and axial without (B)
and after gadolinium injection (C) weighted MR
images. An expansive retinal process into the
vitreous hypointense T2, hyperintense T1,
enhanced after gadolinium injection (red
arrow). Asignal areas corresponding to
calcifications, best seen on CT.
Sagittal T1 with Gadolinium weighted
images. Enlargement of the left optic nerve
enhanced after gadolinium injection (blue arrow).
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Rhabdomyosarcome

• Malignant mesenchymal tumor
• The most common in children (6 of malignant
  tumors).
• Orbital Location frequent.
• Older children mean agegt 6 years.
• Clinical signs- Rapidly progressive
  exophthalmos with ptosis.
• - Pain, eyelid edema.- Mass.

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• CT- Mass isodense to muscles, well limited and
  enhanced.- Lysis of bony walls.- Invasion of
  sinuses and nasal cavities and cavernous sinus.
• MRI- Hypointense on T1 weighted-images.-
  Hyperintense on T2 weighted patterns.- Moderate
  to intense enhancement.- Periorbital
  Extensions- Extension to sinuses and meninges
  Changes the stage of the tumor and thus the
  therapeutic management and the prognosis.

14
A one year old infant Exophtalmos and
Inflammatory signs
A
B
Axial contrast-enhanced CT scan with a
parenchymal (A) and a bone (B) window Mass
isodense to muscles, well limited and enhanced
(red arrow).Lysis of the external bony walls
(blue arrow).
15
B
C
A
MRI patterns. Axial T2 (A) and axial T1 before
(B) and after gadolinium injection (C) weighted
MR images. Mass hyperintense on T2 and isointense
on T1 to muscles, well limited and highly
enhanced (red arrow). Periorbital extension and
to the meninges (blue arrow).
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Capillary hemangioma

• Benign vascular tumor due to a proliferationof
  the vascular endothelium.
• The second most common benign tumor in children
  after the dermoid cyst
• Evolution usually to a spontaneous regression
• Treatment
• - Therapeutic abstention in the simple forms-
  Surgical indications in the large
  shapesinfiltrating the visual axis

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Capillary hemangioma

• CT- Isodense mass.
• - Intense and homogeneous contrast
  enhancement.- No bone abnormalities.
• MRI- Mass with punctate hyposignal
  corresponding to blood flow ("signal void") in
  the capillaries.- Intermediate signal on T1
  between the muscle andthe fat.
• - Hyperintense on T2 .- Marked enhancement.

18
A six month old infant. Right exophtalmos and
ptosis
A
C
B
MRI patterns. Axial T2 (A), sagittal T1 (B) and
coronal T1 after gadolinium injection (C)
weighted MR images. Orbital mass with punctate
hyposignal corresponding to blood flow ("signal
void") in the capillaries (red arrow), markedly
enhanced (blue arrow).
6 months later, MRI patterns. Axial T2 (A) and
axial T1 after gadolinium injection (B) weighted
MR images. The mass decreased.
A
B
19
Optic nerve glioma

• Glioma in children benign tumor oftenpart of a
  known neurofibromatosis (NF1).
• Between 5 and 10 years
• There is aggressive forms that givemetastases
• Clinical signs
• - Unilateral decrease in the visual acuity.-
  Strabismus or nystagmus.- Exophthalmos.

20
Optic nerve glioma

• CT- Large optic nerve isodense to gray
  matter.- Cystic areas.- Enlargement of the
  optic canal.- Enhancement various.
• MRI- Iso-intense on T1, hyperintense on T2-
  Marked enhancement- Extension chiasmal and
  retro-chiasmatic damage.
• - To link to an NF1 characteristics of the
  disease the basal ganglia are hyperintenses.

21
A three year old infant Left exophtalmos and a
decrease in the left visual acuity
CT enlargement of the optic nerve with cystic
areas
A
C
B
MRI patterns. Axial T1 (A), coronal T2 (B) and
axial T1 after gadolinium injection (C) weighted
MR images. Iso-intense on T1, hyperintense on T2
markedly enhanced.
22
Dermoid and epidermoid cysts

• Location Medial corner of the eye
• Frequent component fatty (dermoid cyst)
• CT
• Mass well circumscribed hypodense,
• may contain calcifications and fat (dermoid)
• liquid (epidermoid)
• MRI
• Hyperintense on T2
• On T1 hyperintense (dermoid) and hypointense
  (epidermoid)
• Unenhanced

23
B
C
A
D
MRI patterns. Axial T1 (A), T2 (B). Coronal (C)
and axial (D) T1 after gadolinium injection
weighted MR images. A dermoid cystic located at
the medial corner of the left orbit fading on
the T1 fat sat sequence, hyperintense on T2 and
unenhanced.
24
Histiocytosis X

• Due to a proliferation of Langerhans cells
• Before the age of 10
• Orbital damage 10 of histiocytosis
• Clinical signs- Asymptomatic- Unilateral
  Exophthalmos- Bilateral exophthalmos

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Histiocytosis X

• CT- Osteolytic mass with well defined outlines
  isodense to soft tissue.- No enhancement.-
  Sometimes, of bone defectwith regular outlines
  containing a sequestrum.
• MRI- Heterogeneous signal on T1.
• - Hyperintense on T2.- Indispensable if
  intracranial extension.

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C
A
B
Coronal (A) and axial (B) contrast-enhanced CT
scan with a parenchymal window. Coronal (C) CT
with a bone window. Osteolytic mass with well
defined outlines (red arrow). A bone defect with
regular outlines (blue arrow).
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B
A
MRI Axial T1 (A), coronal T2 (B). Axial T1 after
gadolinium injection (C) weighted MR
images. Hypointense signal on T1, hyperintense on
T2. High enhanced mass.
C
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Orbital metastasis

• Clinical signs nonspecific
• Preferential localization site oculomotor
  muscles, the optic nerve, choroid and greater
  wing of sphenoid.
• Neuroblastoma- 8 to 20 of cases of orbital
  metastases.- Between 2 and 5 years.- Bilateral
  40 of cases.
• Ewing sarcoma
• - Aged child or adolescent
• Nephroblastoma
• - Very rarely the cause of orbital metastasis

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Orbital metastasis

• CT- Mass at the oculomotor muscle, localized,
  homogeneous and enhanced.- Osteolysis
  spiculation or thickening of the roof or floor of
  the orbit.- Sometimes intratumoral
  calcifications.
• MRI- Signal may vary depending on the nature or
  cystic areas.
• - Rarely enhanced.

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B
D
A
C
Axial (A) and sagittal (B) CT scan with a bone
window. Sagittal (C) and coronal (D)
contrast-enhanced CT scan with a parenchymal
window Mass localized at the external orbital
wall homogeneous and enhanced (red
arrow).Osteolysis spiculation on the external
orbital wall (blue arrow).
Axial contrast-enhanced CT scan with a
parenchymal window. Abdominal mass Neuroblastoma
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Conclusion

• The tumor exophthalmos is a common clinical
  signs in children.
• Several causes are found.
• Imaging CT and MRI in our particular context,
  can lead to the diagnosis and help therapeutic
  management.
• MRI is a great help when it is available.