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Extra-ocular retinoblastoma: about 12 cases followed at the Mohamed VI university hospital of Marrakech.

Abstract Retinoblastoma is the most frequent childhood intraocular tumor. The aim of our study is to evaluate the clinical features and management of extra-ocular retinoblastoma in the Mohamed VI university hospital of Marrakech. Retrospective case series, the patient's records were reviewed for patient and tumor features, ocular management, histopathological findings, and patient survival. Over a period of three years, 35 eyes were diagnosed with retinoblastoma; 12 children (16 eyes) (46%) had extra-ocular retinoblastoma. Mean age was 27 months, 60% were males. Six cases had unilateral tumor, five bilateral and one case of trilateral retinoblastoma. There was no positive family history, proptosis was the mean mode of presentation (41,6%) followed by staphyloma (25%) orbital cellulitis (25%) and hyphema(8,3%). The median lag period was 18 months. On imaging and histopathological analysis, there was extrascleral involvement in 41.6%, involvement of orbital part of optic nerve (75%), of orbital muscles (50%) and eyelids in 16.6%. the surgical treatment included according to the degree of extension enucleation (75%) or exenteration (25%) associated to chemotherapy in all cases and one case of external beam radiation. There were 2 cases of orbital recurrence, one death and no metastases at 30 months follow-up.Orbital retinoblastoma still stands as a tall challenge requiring multi-modal and multi-disciplinary approach. Although the survival has increased over the last few years, lack of access to medical facilities, lack of education about the need for early medical attention and cultural resistance to enucleation continue to contribute to an epidemic of extra ocular disease at diagnosis in the developing world.
PMID
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Authors

Mayor MeshTerms
Keywords

Retinoblastoma

enucleation

extra-ocular

extrascleral involvement

orbital cellulitis

proptosis

Journal Title the pan african medical journal
Publication Year Start




PMID- 28292093
OWN - NLM
STAT- MEDLINE
DA  - 20170315
DCOM- 20170329
LR  - 20170330
IS  - 1937-8688 (Electronic)
VI  - 25
DP  - 2016
TI  - Extra-ocular retinoblastoma: about 12 cases followed at the Mohamed VI university
      hospital of Marrakech.
PG  - 131
LID - 10.11604/pamj.2016.25.131.8599 [doi]
AB  - Retinoblastoma is the most frequent childhood intraocular tumor. The aim of our
      study is to evaluate the clinical features and management of extra-ocular
      retinoblastoma in the Mohamed VI university hospital of Marrakech. Retrospective 
      case series, the patient's records were reviewed for patient and tumor features, 
      ocular management, histopathological findings, and patient survival. Over a
      period of three years, 35 eyes were diagnosed with retinoblastoma; 12 children
      (16 eyes) (46%) had extra-ocular retinoblastoma. Mean age was 27 months, 60% were
      males. Six cases had unilateral tumor, five bilateral and one case of trilateral 
      retinoblastoma. There was no positive family history, proptosis was the mean mode
      of presentation (41,6%) followed by staphyloma (25%) orbital cellulitis (25%) and
      hyphema(8,3%). The median lag period was 18 months. On imaging and
      histopathological analysis, there was extrascleral involvement in 41.6%,
      involvement of orbital part of optic nerve (75%), of orbital muscles (50%) and
      eyelids in 16.6%. the surgical treatment included according to the degree of
      extension enucleation (75%) or exenteration (25%) associated to chemotherapy in
      all cases and one case of external beam radiation. There were 2 cases of orbital 
      recurrence, one death and no metastases at 30 months follow-up.Orbital
      retinoblastoma still stands as a tall challenge requiring multi-modal and
      multi-disciplinary approach. Although the survival has increased over the last
      few years, lack of access to medical facilities, lack of education about the need
      for early medical attention and cultural resistance to enucleation continue to
      contribute to an epidemic of extra ocular disease at diagnosis in the developing 
      world.
FAU - Leila, Soltani
AU  - Leila S
AD  - Ophthalmology Department, Mohamed VI University Hospital, Marrakech, Morroco.
FAU - Ibtissam, Hajji
AU  - Ibtissam H
AD  - Ophthalmology Department, Mohamed VI University Hospital, Marrakech, Morroco.
FAU - Hafsa, Essafi
AU  - Hafsa E
AD  - Ophthalmology Department, Mohamed VI University Hospital, Marrakech, Morroco.
FAU - Abdeljalil, Moutaouakil
AU  - Abdeljalil M
AD  - Ophthalmology Department, Mohamed VI University Hospital, Marrakech, Morroco.
LA  - eng
PT  - Journal Article
DEP - 20161102
PL  - Uganda
TA  - Pan Afr Med J
JT  - The Pan African medical journal
JID - 101517926
SB  - IM
MH  - Child, Preschool
MH  - Exophthalmos/*etiology
MH  - Female
MH  - Follow-Up Studies
MH  - Hospitals, University
MH  - Humans
MH  - Hyphema/etiology
MH  - Infant
MH  - Male
MH  - Morocco
MH  - Neoplasm Recurrence, Local
MH  - Orbital Cellulitis/etiology
MH  - Retinal Neoplasms/*diagnosis/pathology/therapy
MH  - Retinoblastoma/*diagnosis/pathology/therapy
MH  - Retrospective Studies
MH  - Time Factors
PMC - PMC5326070
OTO - NOTNLM
OT  - Retinoblastoma
OT  - enucleation
OT  - extra-ocular
OT  - extrascleral involvement
OT  - orbital cellulitis
OT  - proptosis
COI - The authors declare no competing interest.
EDAT- 2017/03/16 06:00
MHDA- 2017/03/31 06:00
CRDT- 2017/03/16 06:00
PHST- 2015/12/09 [received]
PHST- 2016/04/05 [accepted]
AID - 10.11604/pamj.2016.25.131.8599 [doi]
AID - PAMJ-25-131 [pii]
PST - epublish
SO  - Pan Afr Med J. 2016 Nov 2;25:131. doi: 10.11604/pamj.2016.25.131.8599.
      eCollection 2016.

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