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NR 7-9/2008
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Pupillary block
glaucoma in child with persistent hyperplastic primary vitreus –
case report
Blok źreniczny u dziecka z
pierwotnie przetrwałym hiperplastycznym ciałem szklistym – opis
przypadku
Krystyna Kanigowska1,
Mirosława Grałek1, Wiesława Grajkowska2,
Maciej Pronicki2
1 Department of Ophthalmology of Children’s Memorial
Health Institute, Warsaw
Head: Professor Mirosława Grałek MD, PhD
2 Department of Pathology of Children’s Memorial
Health Institute, Warsaw
Head: Maciej Pronicki MD, PhD |
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| Summary: |
Purpose: The
purpose of this paper is to present the case of a
patient with PHPV, in whom the complication in a form of
pupillary block glaucoma was observed as a result of
idiopathic and complete lens dislocation into the
anterior chamber.
Material and methods: Examination was performed
on 3 months old boy with grey pupilla reflex, noted from
the birth, in the right eye. Microphthalmia, subcapsular
cataract and anterior-posterior form of PHPV was found
in that eye. The patient was qualified to surgical
treatment. Before the appointed time of operation the
increased intraocular pressure and significant globe
enlargement were noted. The reason of that was pupillary
block caused by lens dislocation into the anterior
chamber. One-step surgical procedure: trabeculectomy,
lensectomy and cutting out the retrolenticular fibrous
membrane, was performed in urgent course.
Results: Performed complicated and difficult
surgical treatment resulted in normalizing IOP and
created good conditions for vision rehabilitation for
the child. Postoperatively the detachment of the choroid
was noted as a transient complication.
Conclusions: In this case luxated lens and
pupillary block was caused by constriction of
retrolenticular fibrous membrane. Early surgical
intervention is necessary to prevent progressive
pathologic changes in eyes with this developmental
disorder and to obtain the best possible visual results. |
| Słowa kluczowe: |
pierwotne hiperplastyczne
przetrwałe ciało szkliste (PHPV), podwichnięcie
soczewki, jaskra, lensektomia, trabekulektomia,
pozasoczewkowa błona włóknista. |
| Key words: |
persistent hyperplastic
primary vitreus (PHPV), lens luxation, glaucoma,
lensectomy, trabeculectomy, retrolental fibrous membrane. |
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The recommendations for surgical
treatment of persistent hyperplastic primary vitreous have
changed in the last years simultaneously with increasing
knowledge on the subject of PHPV and significant progress in
surgical instruments (1,2). According to early reports on PHPV,
it was recommended to perform surgery at as early an age as
possible in order to prevent later complications, which may lead
to eye pain and its atrophy (3). Particular stress is laid
nowadays on the possibilities of improvement of the infant
vision after their surgical treatment. Unfortunately many
controversies are still present among which, the most important,
surrounds the problem what kind of a patient should be treated.
In the opinion of many authors of presented reports only eyes
with anterior form of PHPV have good prognosis to obtain
satisfying visual acuity after surgical intervention (4). Good
functional results that might be expected are limited by changes
in posterior pole and anomalies of retina and optic nerve. Early
performed surgical treatment should be optimistically evaluated
since it may prevent pathological changes induced by PHPV, from
becoming worse and gives hope for positive visual results.
The purpose of this paper is to present the case of a child with
persistent hyperplastic primary vitreous and with rapidly
increasing buphthalmia caused by complete lens dislocation into
the anterior chamber.
Case description
Parents with 2 months old infant were seen in the Department of
Ophthalmology of children’s Memorial Health Institute, because
of white pupilla reflex noted from the birth in their child’s
right eye. Ophthalmological examination showed light reactions
of both pupillas as normal. Corneal diameter of the right eye
was 10.5 mm and left 11.5 mm. In the left eye no abnormalities
were noted, both in the anterior and posterior segment. Slit
lamp examination of the right eye revealed shallow anterior
chamber and central subcapsular lens opacification that was in
conjunction with fibrous vascularized membrane pulling ciliary
processes. In biometry measurements length of the right eye was
15.5 mm and the left 17.5 mm. Ultrasonography examination showed
the funiculus coming from retrolenticular membrane to optic
nerve disc. Clinical picture was consistent with the diagnosis
of developmental disorder, which was anterior-posterior form of
persistent hyperplastic primary vitreous. The child was
qualified to planned surgical treatment including: lens
extraction, cutting off fibrous cord and excision of
postlenticular membrane, in order to release ciliary processes
from being tense. The assumption of surgical treatment was to
restore the anatomy of eyeball and to make conditions for
infant’s visual rehabilitation. In ten days after the
examination and before the planned date of surgery the parents
came back to the clinic because of rapidly increasing buphtalmia
of the right eye. It was caused by complete lens dislocation
into the anterior chamber with pupillary block (Fig. 1).
Intraocular pressure in the right eye increased to the level of
42 mmHg whereas in the left eye equals 15 mmHg. The right eye
length prolonged to 19.5 mm and the left did not change (17.5
mmHg). Antiglaucomatous drug treatment such as eye drops and
mannitol (i.v.), were given to a child as preparation to
surgical intervention. The child was qualified to
multiprocedural operation because of observed complications.
Trabeculectomy was performed under a scleral flap after
conjunctiva dissection and exposing the surgical limbus
(Fig. 2). Next the anterior chamber was inflated with
viscoelastic agent and the anterior capsule was separated from
endhothelium. After that the vitrectom was installed through
sclerectomy and the lens was removed from the anterior chamber
(Fig. 3). The retrolenticular fibrous membrane was cut off with
vitrectom and microscissors relasing the ciliary processes.
Fibrovascular cord connective with optic disc was severed after
being coagulated; its stump over optic disc was left (Fig. 4, 5,
6). Finally two sutures were placed on scleral flap and
conjunctiva was closed.
Postoperatively two serious, but temporary, complications were
noted despite the fact that we used the closed-chamber technique.
The first one, cornea decompensation, was found in 24 hours
after the surgery. It was caused by contact with dislocated lens
and surgical manipulation in the anterior chamber. The second
complication was hemorrhagic detachment of the choroid induced
by rapid decrease of intraocular pressure to 8 mmHg. During
hospitalization gradual improvement of clinical state was
observed. Postoperatively antiglaucomatous drops were given to
maintain IOP on the level of 16 mmHg, and after three months it
was still on this level without any drops.
Discussion
Most authors in their reports about coexistence of lens
opacification and anterior-posterior form of PHPV suggest the
necessity of surgical treatment (5). Decision of no treatment
may lead to many complications such as closure-angle glaucoma,
vitreous hemorrhage, retinal detachment and even eyeball atrophy.
Lens dislocation in the eye with no treated PHPV has been not
described in literature, so far.
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However, surgical
treatment of eyes with developmental abnormalities seem to be
quite a difficult procedure burdened with many possible
complications. It is demanded to be performed by an experienced
surgeon in a specialized center where additional studies (USG,
UBM) can be done to let the surgeon decide on the most
satisfying surgical technique. Our gain in those cases is to
minimize the risk of intra- and postoperative complications and
to choose the least invasive surgical technique. The reasons of
soft lens dislocation into anterior chamber in children are
different. The most common are posttraumatic and caused by
congenital predispositions. In our case we suspected that the
lens dislocation resulted from rentrolenticular membrane
contraction, what caused “pushing” the lens into anterior
chamber. Those assumptions were confirmed by histopathological
examination of excised membrane and the cord adjoining it to
optic disc (Fig. 7). Microscopical image showed contracting
fibroblasts and and hyalinizating membrane (Fig 8). Acute
angle-closure glaucoma induced by retrolenticular membrane
contraction in a 30 years old female is also presented by Sawada
et al in their report (6). In our case lens dislocation was the
reason of disturbances of aqueous circulation, high increase of
intraocular pressure and eye ball enlargement. Decision of
necessity of surgical intervention concerned not only removing
the reason of the complications – dislocated lens and
retrolenticular membrane, but also glaucoma. Taking into account
possible lesions in the filtration angle, which might have
appeared in microphthalmia with buphthalmus, we decided to
perform trabeculectomy in one-step surgery. Other activities to
decrease intraocular pressure are presented by Khan who
describes the case of 4 months child with glaucoma (40 mmHg),
PHPV and buphtalmus, but without lens dislocation (7). In that
case surgery included lensectomy and membranectomy. Intraocular
pressure was to be decreased by diode laser cyclodestruction of
the inferior hemisphere. Postoperatively no serious
complications were noted by the author. In one year after
surgery IOP was on the level of 15 mmHg on dorzolamid 2% and
timolol 0.5%. In our case at early postoperative stage we found
cornea haziness with Descemets’ folds and detachment of the
choroids in the second 24 hours. These complications were caused
by surgical removal of pupillar block with rapid IOP growth
followed by its sudden reduction. It appeared that despite
performing only one ora serrata 1.5 mm cut during surgery and
closed-chamber technique, which is recommended by many authors
for such cases, we noted a few complications. Our gain in
qualification the patient to surgical treatment was to give him
a chance for visual rehabilitation and to obtain useful visual
acuity. Later complications that occurred such as lens
dislocation followed by pupillary block glaucoma made the
surgical procedure much more difficult and induced serious
potential postoperative complications.
The conclusions of summing up the analyzed case are the
following:
1. In the presented case of anterior-posterior form of PHPV lens
dislocation followed by pupillary block glaucoma was caused by
contraction of retrolenticular fibrous membrane.
2. Early surgical intervention is necessary to prevent
progressive pathologic changes in eyes with this congenital
disorder and to create conditions for rehabilitation and visual
development for a child.
References:
1. Kanigowska K, Grałek M, Klimczak-Ślączka D, Seroczyńska M:
Pierwotnie przetrwałe ciało szkliste – wada rozwojowa gałki
ocznej u dzieci. Klin Oczna 2006, 108, 225-227.
2. Dass AB, Trese MT: Surgical results of persistent
hyperplastic primary vitreus. Ophthalmology 1999, 106, 280-284.
3. Anteby I, Cohen E, Karshai I, BenEzra D: Unilateral
persistent hyperplastic primary vitreus: Course and Outcome. J
AA.POS 2002, 6, 92-99.
4. Pollard ZF: Results of treatment of persistent hyperplastic
primary vitreus. Ophthalmic Surg 1991, 22, 48-52.
5. Kanigowska K, Grałek M, Chipczyńska B, Hautz W: Problemy w
leczeniu chirurgicznym pierwotnie przetrwałego ciała szklistego
u dzieci. Klin Oczna 2006, 108, 51-55.
6. Sawada H, Fukuchi T, Ohta A, Suda K, Togano T, Nakatsue T,
Funaki S, Hara H, Shirakashi M, Abe H: Persistent hyperplastic
primary vitreous – a case report of adult onset acute
angle-closure glaucoma. Nippon Ganka Gakkai Zasshi 2001, 105,
711-715.
7. Khan A.O.: Buphthalmos in the setting of persistent
hyperplastic primary vitreous cataract. Am. J. Ophthalmol. 2003,
136, 945-947.
Praca wpłynęła do Redakcji 06.06.2008 r. (1057)
Zakwalifikowano do druku 12.08.2008 r.
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Ryc. 1. Soczewka zwichnięta do komory
przedniej.
Fig. 1. Dislocated lens into the anterior chamber.
Ryc. 2. Trabekulektomia.
Ryc. 2. Trabeculectomy
Ryc. 3. Lensektomia przez otwór
trabekulektomii.
Fig. 3. Lensectomy through trabeculectomy.
Ryc. 4. Wycięcie włóknistej błony
zasoczewkowej pociągającej wyrostki rzęskowe.
Fig. 4. Cutting off the retrolenticular fibrous membrane pulling
ciliary processes.
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Ryc. 5. Diatermokoagulacja
włóknistonaczyniowego powrózka połączonego z tarczą n.
wzrokowego.
Fig. 5. Diathermocoagulation of the fibrovascular cord connected
with optic disc.
Ryc. 6. Wycięcie błony włóknistonaczyniowej.
Fig. 6. Excisio of the fibrovascular membrane.
Ryc. 7. Pobieranie wyciętej błony w celu
badania histopatologicznego.
Fig. 7. Uptaking of the cut out membrane for histopathological
examination.
Ryc. 8. Obraz mikroskopowy błony hialinowej i
fibroblastów.
Fig. 8. Microscopical image of fibroblasts and hyaline membrane.
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