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\begin{document}

\title{Spontaneous bilateral intra-orbital hematoma: A particular form of
complications of sickle cell disease in children.}



\author[1]{Haritanjona  Andriamiarintsoa}%
\author[2]{Herveat RAMANANDAFY}%
\author[1]{Koloina Randriantiana }%
\author[1]{Prosper Harinarivo   Randrianarivelo }%
\author[1]{Emmylou Prisca Gabrielle   Andrianah}%
\author[3]{Lova Hasina Ny Ony Narindra  Rajaonarison}%
\author[4]{Joëlson Lovaniaina  Rakotoson }%
\author[5]{Hanta Marie Danielle Vololontiana}%
\author[3]{Ahmad  Ahmad }%
\affil[1]{Université d'Antananarivo}%
\affil[2]{Medecins Sans Frontieres}%
\affil[3]{University of Antananarivo}%
\affil[4]{University of Madagascar}%
\affil[5]{University of Antananarivo Faculty of Medicine}%


\vspace{-1em}



  
  \date{June 14, 2024}


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\selectlanguage{english}
\begin{abstract}
Spontaneous bilateral intra-orbital hematoma is a rare complication of
sickle cell disease in children. Imaging examinations are of paramount
importance in the diagnosis and condition the management in order to
avoid complications that can compromise the visual function prognosis.%
\end{abstract}%



\sloppy


\textbf{INTRODUCTION}

Sickle cell disease is an inherited hemoglobinopathy characterized by
vaso-oclusive crises. Intraorbital hematoma secondary to hematopoietic
medullary infarction is a rare complication of sickle cell disease
{[}1{]}. The visual prognosis is at stake in the absence of immediate
treatment. We report an atypical form of intraorbital hematoma revealed
by bilateral exophthalmos in a sickle cell child, then we will highlight
the role of imaging in the diagnosis and management of intraorbital
hematoma.

\textbf{CASE REPORT}

A 15 year old boy, known to have homozygous sickle cell disease, came
for a consultation with headache and bilateral eye pain which had been
evolving for two weeks in a context of apyrexia. In his background,
there was no notion of craniofacial traumatism or recent surgery. On
ophthalmological examination; bilateral diplopia, palpebral edema and
bilateral exophthalmos painful, non-pulsatile and non-reducible were
noted. The measured visual acuity was altered to 5/10 for the right eye
and 7/10 for the left eye. The fundus examination was very limited and
did not show any particularities. Other examinations, especially
neurological, were unremarkable. The formal blood count showed a
discrete anemia with a hemoglobin level of 9.3 g/dl; the neutrophilic
polynuclear and platelet counts were within the normal range. The
C-reactive protein level was discretely elevated at 7 mg/L. The
hemostasis assessment was also normal. Oculo-cerebral CT scan with
parenchymal window showed a bilateral intraorbital structure,
spontaneously hyperdense, of hematic density, in intra and extra-conical
situations, pushing forward and down the two ocular globes (figure 1).
In the bone window, it showed a bilateral exophthalmos of grade 3
without abnormal bone structure (Figure 2). Ocular B-mode ultrasound was
performed but was very limited because of the narrowing of the acoustic
window due to the exophthalmos. In front of the anamnestic, clinical and
scannographic context, the diagnosis of a spontaneous bilateral
intraorbital hematoma in a sickle cell patient was evoked. The patient
was put on oral corticosteroids for five days. And in front of the
ocular pain, a treatment containing analgesic was instituted. The
evolution was favorable after one week, marked by the regression of the
exophthalmos and a progressive recuperation of visual acuity and
oculomotricity. The Oculocerebral CT scan at one week showed regression
of the intraorbital hematoma \textbf{(Figure 3)} . The recuperation of
visual acuity and oculomotricity ad integrum was achieved after one
month with total disappearance of the exophthalmos \textbf{(Figure
4).}The patient was then readmitted to the sickle cell management
center.

\textbf{DISCUSSION}

In the orbit; the hematopoietic medullary space is smaller, It is
therefore rare to find orbital bone infarcts in sickle cell patients
{[}1{]}. About thirty cases are described in the literature with an
average age of 14 years , half of which had an intraorbital hematoma
{[}2{]}. The singleness of our case in Madagascar would therefore allow
comparison with the literature and could provide additional data on the
diagnosis and management of intraorbital hematoma in children with
sickle cell disease. The bilateral form is rarely reported in the
literature. In a series conducted by Soko, the bilateral form represents
one third of reported cases {[}2{]}. This one makes our case a
particular form of intraorbital hematoma. Exophthalmos is a reflection
of the mass effect, it is the main mode of discovery of the
intra-orbital hematoma. The first functional manifestations are ocular
pain and localized edema. In the absence of urgent management, the
visual prognosis is at risk in case of optic nerve compression
{[}1,3{]}. Before the rapid onset of exophthalmos, especially since it
is bilateral; the differential diagnosis is vast. Among these, we can
cite orbital pseudotumor, orbital rhabdomyosarcoma, optic nerve glioma,
orbital extension of bilateral retinoblastoma, bilateral orbital
cellulitis. In all these possibilities, Imaging studies are of paramount
importance in characterizing the nature of the compressive mass. In our
case, the hematic density was spontaneously hyperdense on the CT scan,
suggesting the diagnosis of a bilateral intra-orbital hematoma. The
Magnetic resonance imaging, which would determine the hematic nature of
the collection {[}4{]} not being accessible, the CT scan therefore
remains an examination that retains its place although it is relatively
non-specific. However, the absence of sinus pathology and the location
of the collection on the lateral wall of the orbit are the arguments
against an abscess {[}2{]} especially that the clinical and biological
examination are sterile. Medical treatment seems to be sufficient in
most cases, including treatment of the crisis and the use by some teams
of corticosteroids to be used with caution in the context of sickle cell
disease {[}1,2{]}. Surgery is reserved for cases of persistent
compression of the optic nerve {[}2{]}. In our case, the treatment is
purely medical, making the exophthalmos disappear after one month with a
recovery of the visual acuity ad integrum.

\textbf{CONCLUSION}

Spontaneous bilateral intra-orbital hematoma secondary to bone
infarction is a rare but serious complication of sickle cell disease.
Imaging examinations hold an important place in the diagnosis and have
an implication in the management to preserve the visual function. A
regular follow-up of sickle cell disease with the implementation of a
background treatment and the precaution of factors favoring
vaso-oclusive attacks could avoid this complication which sometimes has
a poor prognosis.

\textbf{KEY CLINICAL MESSAGE}

The report of this case would allow researchers to compare with the
literature and could provide additional data on the diagnosis and
management of intraorbital hematoma in children with sickle cell
disease.

\textbf{ACKNOWLEDGEMENTS}

This work was carried out with the working group of SIMAD (Societ\selectlanguage{ngerman}é
d'Imagerie de Madagascar).The authors thank the patient who gave written
permission for this report.

\textbf{AUTHOR'S CONTRIBUTIONS}

All the authors have read and approved the final draft of the
manuscript. All authors contributed to this work and approved the final
version.

HA: followed up the patient, collected the clinical data.

HR: Collected the clinical data, and drafted the report

KR: followed up the patient, collected the clinical data.

PHR: followed up the patient

EPGA, LHNONR: designed and critically revised the report.

JLR, HMDV and AA: validated the report.

\textbf{CONFLICTS OF INTEREST}

None declared

\textbf{ETHICAL APPROVAL}

The article does not contain any personal information that could
identify the patient. The names and dates on the chest CT scan have been
hidden. The authors have included only information necessary for
scientific understanding.

\textbf{FINDING STATEMENT}

This research has not received any specific grant from any public,
commercial or for-profit.

\textbf{DATA AVAILABLE STATEMENT}

Data available on request from the corresponding author.

\textbf{CONSENTS STATEMENTS}

Written consent was obtained from the patient for publication of this
case report and accompanying images.

\textbf{REFERENCES}

1{]} Ganesh A, Al-Zuhaibi S, Pathare A, William R, Al-Senawi R,
AlMujaini A, et al. Orbital infarction in sickle cell disease. Am J
Ophthalmol 2008;146(4):595--601.

{[}2{]} Sokol JA, Baron E, Lantos G, Kazim M. Orbital compression
syndrome in sickle cell disease. Ophthalmic Plast Reconstr Surg
2008;24(3):181--4.

{[}3{]} Talbot M, Meunier B, Lavoipierre V. L'hématome sous-périoste,
une complication méconnue de la drépanocytose. Rev med int.\emph{Volume
40, Supplément 1}~; 2019~:
159-60.\emph{https://doi.org/10.1016/j.revmed.2019.03.207}

{[}4{]} Tostivint L, Pop-Jora D, Grimprel E, Quinet B, Lesprit E.
Orbital bone infarction in a child with homozygous sickle cell disease.
Arch Pediatr 2012;19(6):612--5.

\textbf{LISTS OF FIGURES}

\textbf{Figure 1:} Oculo-cerebral CT scan with parenchymal window:
Bilateral intraorbital formation, spontaneously hyperdense, intra and
extra-conical situation, hematic density.

\textbf{Figure 2~:} Oculo-cerebral CT scan with bone window:
Exophthalmia stage 3 , with absence of abnormalities in bone structure.

\textbf{Figure 3:} Oculo-cerebral CT scan with parenchymal window:
Regression of the intraorbital hematoma

\textbf{Figure 4~:} Oculo-cerebral CT scan with bone window: Total
disappearance of exophthalmos\selectlanguage{english}
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