Article:
We report the rare complication of spontaneous globe rupture secondary
to unilateral retinoblastoma in an infant with Group E retinoblastoma. A
5-month-old previously healthy infant presented with a 1 month history
of right leukocoria. On examination under general anaesthesia, the right
intraocular pressure was 50mmHg, left 26mmHg. The right eye had an
uninflamed conjunctiva, clear cornea and normal diameter. The anterior
chamber was shallow with iris that bowed anteriorly. There was no
rubeosis or anterior chamber cells. A white tumour with overlying
retinal detachment filled the entire vitreous cavity and posterior
chamber, moulding to the posterior curvature of the lens. B-scan
ultrasonography showed a solid, homogenous lesion with hyperechoic foci.
The left eye was normal.
MRI scan of her brain confirmed the tumour, but showed no scleral
invasion or extraocular tumour extension. Enhancement of the right
central retinal vessel was noted. No contralateral or intracranial
tumour was identified. Lumbar puncture showed no evidence of tumour
cells within the cerebrospinal fluid. Following a multidisciplinary
team discussion and counselling of parents, right enucleation was
scheduled for the following week.
Six days following the initial assessment, the patient attended the
emergency department with an injected right eye with creamy discharge.
It was noted that her crystalline lens had expulsed. No trauma was
reported prior. The patient was diagnosed with spontaneous right globe
rupture and proceeded to undergo an urgent enucleation that same evening
(Figure 1A). Peritomy exposed thin sclera at the limbus with a rupture
at 9 o’clock. Prior to enucleation, the site of rupture was carefully
exposed without opening any tissue planes and submerged in distilled
water for 3 minutes. The limbal defect was closed with 6-0 vicryl suture
(Figure 1B). The ruptured area was then resubmerged in distilled water
to mitigate further spillover during the enucleation process. No implant
was inserted due to concern this may hinder the detection of orbital
recurrence. The patient was commenced on emergent chemotherapy
postoperatively as per ARET0332 using vincristine, etoposide and
carboplatin for 6 cycles.
Histology showed a moderately differentiated retinoblastoma with
haemorrhage, mixed inflammation, necrosis and dystrophic calcification
within the lesion. The site of the rupture at the limbus showed
interstitial keratitis and scleritis but there was no tumour invasion of
the sclera, iris or choroid. No infectious organisms were identified.
Tumour genetics found non-heritable homozygous RB1 whole gene deletion.
3-monthly MRI scans, the latest at 16 months post-diagnosis, have shown
no recurrence.
Spontaneous globe rupture secondary to retinoblastoma is rare. The only
reports found in the literature are dated in the 1900s as a complication
of end-stage disease, whereby perforation would occur at the limbus,
often preceded by buphthalmos and rupture of Descemet’s membrane or
sclera (1). This case is noteworthy in that globe rupture occurred
without warning or clear cause. Whilst the intraocular pressure was
elevated, this in itself is not unusual for many cases of group E
disease (2, 3). Furthermore, static and dynamic studies of post-mortem
eyes have shown that the human eye has the capacity to maintain globe
integrity up to 900mmHg before globe rupture may occur (4). The presence
of kerato-scleritis on histology may have contributed to scleral
weakness.
Globe rupture carries a high risk of tumour contamination and metastatic
spread. To mitigate this risk, surgery was expedited, the surgical site
was irrigated with distilled water prior to enucleation, and patient
commenced on emergent chemotherapy. Submersion of retinoblastoma cells
in distilled water leads to cell lysis and may be useful to limit
surface tumour spread (5).
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