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12.2 Surgical Management ofROP
Fig. 12.5 Difference in the angles when the syringeneedles entering into the vitreous cavity
between adults and children (a) The safe range for intravitreal injection in adults is relatively large,
and the injection direction can be toward the center of the vitreous cavity. (b) The safe range for
intravitreal injection in children is relatively small. To avoid iatrogenic damage to the lens, the
injection direction must be slightly perpendicular to the ground
255
12.2.3 Retinal Laser Photocoagulation
It must be performed under an indirect ophthalmoscope, with a relatively long
learning curve. Beginners can rst master the use of indirect ophthalmoscopes in
daily outpatient work and adult external surgeries and then gradually transition to
laser treatment of ROP.The key points in the operation are (1) treating the entire
avascular zone to avoid skipped areas; (2) ensuring no lesions within the avascular
zone are missed—the laser spots should extend anteriorly to the ora serrata; and (3)
avoiding direct laser application to the ridge at the interface between the vascular
and avascular retina.
When operating, the operator should pay attention to the following issues:
1. Maximal pupil dilatation. The pupil dilates after 20 min if you drop the eye
extensively (every minute) with all available dilating eye drops.
2. Prolong the time when the refractive medium is sufciently transparent as much
as possible, in addition to adequate pupil dilatation before surgery, the strength
and magnitude of the extra-scleral parietal pressure should be paid attention to
during surgery to avoid the sharp changes of intraocular pressure, resulting in
corneal edema and pupil narrowing.
3. The amplitude of eyeball rotation should be as small as possible. Due to the
small corneal diameter and large curvature of newborns, if the angle between the
light of the indirect ophthalmoscopes and the visual axis is too large, the aberration in the peripheral part will increase signicantly. Therefore, the light of the
indirect ophthalmoscopes should be as close to the vertical direction as possible,
256
12 Vitreoretinal Surgery inChildren andAdolescents
and then cooperate with the scleral indentator to complete laser
photocoagulation.
4. Proceed from easy to difcult, generally extending from the base of the ridge to
the periphery, and gradually reducing the laser energy.
5. In the process of extending to the periphery, clearly identify the ora serrata
before performing laser. The position and shape of the ora serrata in newborns
are quite different from those in adults. It is located about 1mm behind the limbus, a white area with leathery or shiny texture, adjacent to the dark brown area
of the ciliary body.
6. The laser spots should not be too dense or too strong, with an interval of 1–1.5
laser spot distances (beginners often make the laser spots too dense), and the
color should be grayish white (lighter than the laser spot reaction in adults).
7. The laser area is close to but not exceeding the ora serrata, avoiding anterior
extension to damage the ciliary body, otherwise, complications such as hypotony
and cataracts may occur.
8. The laser device has three settings: power, duration and interval. The critical setting is duration. A too high duration results in vitreous bleeding and progress of
ROP. Always begin with a duration of 100 ms and maximize power before
increasing the duration to 200 ms. Remark: In new laser devices, 100ms duration is sufcient; in old laser devices 200ms duration is required. 300ms is
too high.
12.2.4 Vitreoretinal Surgery
For ROP stage 4A, surgical treatment is not generally required. The therapy of
choice is an anti-VEGF injection. We recommend to examine the eye in general
anesthesia. If the eye has been pretreated with laser, then try to nd skip lesions and
treat these skip lesions with laser.
For ROP stage 4B, a vitrectomy is generally required, which is quite different
from that of adults, and the focus should be on understanding its pathological anatomical features, access selection, surgical intent, and the corresponding surgical
instruments.
(i) Pathologic Anatomical Features
The main manifestation is retinal detachment by traction and comparing it with
severe PDR can help us better understand the pathological-anatomical features of
ROP (Table.12.2).
(ii) Choice of SurgicalApproach
The choice of approach is determined by the severity of retinal detachment and the
sparing vitrectomy because the removal of the lens results in severe amblyopia. In
advanced cases peripheral membranes develop and pull the retina toward the lens.
age of the child, as well as the operator’s preference. The method of choice is a lens-
12.2 Surgical Management ofROP
257
Table 12.2
Vitreous state Basically no liquefaction,
Precipitating factor Presence of avascular areas in the
Typical change The ridge exists at the interface
Retinal detachment From light to heavy, it can be
Treatment time
window
Operating space Narrow Spacious
Intraoperative
complications that
are difcult to
manage
Table 12.3
Month/age 0 1~6month 6~12month 1~3 age 3~6 age 6~18 age
Distance from limbus(mm) 0.5~1.0 1.5 2.0 2.5 3.0 3.5
Pathoanatomical features of ROP (≥4 stages) and severe PDR
ROP
delamination-like changes, and
all-round adhesion to the retina
below, there is great difculty in
posterior detachment
peripheral and mid-peripheral
retina
between the vascular and avascular
areas of the retina
expressed as peripheral shallow
detachment, and full detachment
can be wide-funnel or
closed-funnel
Extremely short Longer
Iatrogenic break Bleeding
The distance between the posterior scleral incision and the corneal limbus
PDR
There may be partial or complete
liquefaction, and adhesions to the
underlying retina are well
separated at the posterior pole and
mid-periphery
Poor retinal perfusion, ischemic
changes affecting the entire retina
Fibrovascular membranes are
present at the posterior pole and
mid-periphery
After the detachment, the limited
detachment of the pole is mainly,
and it can also be expressed as a
wide bucket or a closed bucket
The anterior pulled retina prevents access to the vitreous. In this case a lens removal
is required.
The distance of the scleral incision from the corneal limbus is determined by the
age of the child, usually starting at 0.5mm, but the preoperative axis of the child and
the degree of retinal detachment should be understood and adjusted (Table. 12.3).
Pars plana approach for ROP stage 4: The greatest advantage is that the clear lens
is expected to be preserved, which is important for the visual development of
the child.
One of the main problems encountered by beginners is the deformation of the
eye during trocar insertion, mainly due to two major factors: (1) soft sclera and (2)
the toughness of the conjunctiva and Tenon’s capsule (associated with age). If both
factors are present, the puncture becomes signicantly more difcult, and the puncture step is like a “downward spiral” for the surgeon. We recommend therefore a
perpendicular and not diagonal insertion of trocar cannulas. An additional help
when inserting the trocar cannulas is to xate the sclera with a forceps. An alternative is the opening of conjunctiva before you insert the trocar cannulas.
The pars plana approach is generally chosen for infusion. The biggest problem
with pars plana infusion is that the trocar may swing toward the lens, injure the lens
258
or prolapse backward, which can cause hypotony and choroidal detachment if not
detected in time. To prevent this from happening, we recommend to tape the infusion cable onto the drape so that the infusion cannula is directed into the middle of
the eye.
(iii) Basic Surgical Steps
1. Insertion of trocar cannulas
2. Insertion of infusion
3. Removal of the central vitreous
4. Removal of the peripheral vitreous
5. Decide whether to laser treat the retina
6. Partial uid against air exchange
7. Inject anti-VEGF
8. Withdraw trocars
9. Check the scleral incision for tightness
In stage 5 ROP vitrectomy becomes very difcult. A pars plana (plicata) access
is not possible. The only possible access is through the anterior chamber. The instruments are inserted through the limbus, the lens is removed, and then the closed retina is cautiously opened. The difculty of this surgery is extremely high, and the
postoperative results are very low so that surgery of stage 5 ROP is questionable.
Anterior approach: Insert the puncture knife at the corneal limbus, through the
iris root, and let the puncture knife insert into the lens, with the angle of the two
trocar being large enough, near 3 and 9 o’clock, respectively, to ensure sufcient
space for both hands to operate; place the perfusion tube through the clear corneal
incision; and remove the vitreous humor after the lens is removed; a clear corneal
incision is generally chosen. The intraocular pressure is maintained by placing a
perfusion tube, and care should be taken to avoid damage to the corneal endothelium.
For Stage 5 ROP, in addition to the above basic steps, a two-handed approach is
often required, which may require a ceiling light, intraocular scissors, intraocular
forceps, and “blunt” separation with viscoelastic if the vitreous is tightly adherent
to the retina.
12 Vitreoretinal Surgery inChildren andAdolescents
12.3 Rhegmatogenous Retinal Detachment
Secondary toFEVR
12.3.1 Overview
Familial exudative vitreoretinopathy (FEVR) was rst proposed and named by
Criswick and Schepens in 1969. The lesion is similar to ROP without a history of
prematurity or oxygen uptake and is inherited in autosomal dominant, autosomal
recessive, and X-linked recessive forms. It is characterized by bilateral, slowly progressive vitreoretinal anomalies, dense distribution of peripheral retinal vessels,
retinal neovascularization or intraretinal or subretinal exudation, optic disc or
12.3 Rhegmatogenous Retinal Detachment Secondary toFEVR
259
macular displacement, and in some cases, retinal detachment of retinal origin by
traction or retinal break.
12.3.2 Clinical Staging ofFEVR
In 2014, Kashani etal. proposed a new clinical staging method for FEVR based on
the results of fundus wide-eld uorescence imaging, which differs from ROP in
that the clinical symptoms of FEVR vary widely among individuals.
(i) Stage 1: no peripheral vascularity or abnormal intraretinal vascularization (1A:
no exudation or uorescein leakage; 1B: presence of exudation or uorescein
leakage)
(ii) Stage 2: peripheral retinal avascular area with extraretinal vascularization (2A:
no exudation or uorescein leakage; 2B: presence of exudation or uorescein
leakage)
(iii) Stage 3: retinal detachment without macular involvement (3A: no exudation or
uorescein leakage; 3B: presence of exudation or uorescein leakage)
(iv) Stage 4: retinal detachment involving the macula, but not yet total detachment
(4A: no exudation or uorescein leakage; 4B: presence of exudation or uorescein leakage)
(v) Stage 5: total retinal detachment (5A: open bucket shape; 5B: closed
bucket shape)
In addition to retinal detachment requiring surgical treatment, related studies
have shown that the presence of uorescein leakage is a precursor to the occurrence
of retinal leakage. For stage IB and 2B lesions, laser treatment should be performed
promptly to close the leakage area and the avascular area to stop the progression of
the lesion.
12.3.3 FEVR andRRD
Rhegmatogenous retinal detachment (RRD) in adolescents is not uncommon, but
we often overlook the genetic factors behind it, the most typical example
being FEVR.
FEVR is an underestimated disease in our country. In fact, among the majority of
young patients with rhegmatogenous retinal detachment (RRD) who undergo scleral
buckling in our practice, the underlying cause is eventually revealed to be FEVR
after thorough fundus examination and screening of their family members.
Because retinal detachment due to FEVR often spreads gradually from the
peripheral to the posterior pole, patients generally have no obvious subjective symptoms until the macula is involved, which is why many patients with FEVR may have
been ill for a long time when they come to the clinic, and some may even have
multiple subretinal bands or membranes visible. In recent years, there are also
260
12 Vitreoretinal Surgery inChildren andAdolescents
patients who were discovered during dilated pupil for fundus examination when
seeking myopic laser surgery.
12.3.4 Principles ofSurgical Management
(i) Laser photocoagulation, cryopexy, and anti-VEGF: Laser photocoagulation
and anti-VEGF are the treatment of choice for FEVR.Perform a 360° laser
photocoagulation from the ora serrata to the posterior pole. In young patients a
treatment in general anesthesia is recommended. In the case of inferiorly
located vasoproliferative tumors, a cryopexy can be added. The disadvantage of
cryopexy is that an exudative detachment may develop which takes several
months to resorb. It is therefore advisable to avoid cryopexy and if necessary to
use it cautiously. Repeated anti-VEGF injections are recommended.
(ii) In the case of an exudative detachment, we recommend an external drainage of
subretinal uid and then treatment with laser photocoagulation or cryopexy.
The healing time after treatment is long and may take 1–2years.
(iii) Vitrectomy
For Stage 5 FEVR with obvious preretinal or subretinal proliferation or
exudation, vitrectomy is generally required.
If the patient is young, no retinal tear is found, and the lesion is mainly
exudate and traction; it can be treated basically according to the ROP internal
surgery method, and postoperative attention should be paid to the treatment of
residual avascular area, supplemented with laser or anti-VEGF intraocular
injection if necessary.
If the patient is older and has retinal tears and proliferation, it should be
treated according to RRD combined with severe PVR.The main difculties of
the operation are as follows:
1. Posterior vitreous detachment is difcult, and vitreous remains at the posterior pole.
2. Peripheral vitreous adhesions. It is very difcult to excise a sufcient
amount. During the process of excision and stripping of the proliferative
membrane, iatrogenic breaks are likely to occur.
3. If heavy water is used improperly, heavy water residues under the retina
are likely to occur. If it is decided to perform vitrectomy, in order to better
ensure the long-term success rate, episcleral cerclage can be performed at
the same time to relieve the possible concentric traction caused by the
residual vitreous in the peripheral area.
(iv) Scleral buckling
In our clinical work, most of the adolescent FEVR patients are often seen
with mildor chronic retinal detachment. If RRD is not accompanied by obvious preretinal proliferation, regardless of whether the macula is involved or
not, or whether there are subretinal proliferation strips, scleral buckling is
12.4 Retinal Detachment Secondary toStickler Syndrome
generally recommended, which is what beginners need to focus on, and the
main reasons include the following.
(a) Better closure of the breaks: retinal breaks are mostly atrophic and in the
more peripheral area, which is less difcult to operate and easy to, especially when the primary break is located below, scleral buckling has a
strong advantage.
(b) The degree of retinal shortening is lower: it is generally not necessary to
perform subretinal uid drainage, and the effect of scleral buckling is better, which can more precisely apply cryotherapy on the breaks and does not
cause excessive eye wall and retinal damage.
(c) “Multiple benets in one stroke”: encircling and buckling can not only
effectively close the retinal break, but also when there are multiple peripheral small breaks in the neighboring areas, scleral buckling can easily
“close” all these breaks, so that the maximum effect can be obtained with
the minimum amount of surgery. At the same time, it is less difcult to
supplement retinal laser photocoagulation later because of the presence of
scleralridge.”
(d) “Addressing both the primary breaks and the underlying avascular pathol-
ogy”: since the pathology of FEVR is based on the presence of avascular
areas at the peripheral, scleral buckling not only closes the primary breaks
but also treats these avascular areas with extra-scleral indentation, which
largely relieves vitreous traction on the peripheral retina, and alsoprevents
the progression of FEVR(if intraoperative cryotherapy or postoperative
photocoagulation is applied).
261
It is worth noting that because the peripheral retina is relatively thin, the intensity
and scope of intraoperative cryotherapy should be strictly controlled, otherwise it
may cause retinal atrophy, which may lead to secondaryretinal redetachment, and
the risk of postoperative PVR may increase, making the second stage of treatment
signicantly more difcult.
We can applylaser photocoagulation aroundthe degenerative retina in the undetached area before surgery, and then moderately perform cryotherapy intraoperatively around the primary break. 1~2weeks after surgery, we can then perform laser
photocoagulation on the retina in all peripheral degenerative areas in ofce.
12.4 Retinal Detachment Secondary toStickler Syndrome
Stickler syndrome is a predominantly autosomal dominant disease of the connective
tissues of the body characterized by a defect in collagen production due to a genetic
mutation. It manifests as abnormalities in organs or systems such as the eyes, hearing, mouth and face, bones and joints, and heart.
262
12 Vitreoretinal Surgery inChildren andAdolescents
12.4.1 Typology ofStickler Syndrome
Stickler syndrome generally includes four subtypes (Table12.4), and the one closely
related to ophthalmology is type 1, accounting for about 80%–90%.
12.4.2 Clinical Features ofStickler Syndrome
Patients with Stickler syndrome may have a series of abnormalities in appearance
during childhood and adolescence, such as attened face, anterior nostril tilt, wide
or at nasal bridge, jaw deformity (small or receding), joint hyperextension, kyphosis, etc. These external features can help us make preliminary diagnosis, but the
gold standard for diagnosis requires genetic testing.
The abnormalities of Stickler syndrome in the eye are mainly manifested as follows: (1) congenital myopia with continuous increase; (2) premature liquefaction of
the vitreous, which may be accompanied by curtain-like or string-like degeneration;
(3) paravascular retinal degeneration and pigmentation; (4) focal lens cortical clouding; and (5) different types of retinal detachment.
Stickler Syndrome and Retinal Detachment
The lifetime probability of retinal detachment in patients with Stickler syndrome is
about 50%–65%, but in the past, retinal detachment in this group of patients was
often attributed to high myopia. With the advancement of our understanding of the
disease and the development of genetic testing technology in recent years, more and
more adolescents are diagnosed with the disease.
There are three common types of retinal detachment caused by Stickler syndrome: (1) giant retinal tear (GRT), (2) rhegmatogenous retinal detachment, and (3)
retinal detachment without a clearly visible ssure. The recommended surgery is
encircling band, vitrectomy and tamponadewithgas or silicone oil. A prophylactic
cryotherapy orlaser photocoagulation of the fellow eye is recommended.
Table 12.4 Four subtypes of Stickler syndrome
Mode of
Typing
Genes
COL2A1 1 Autosomal
COL11A1 2 Autosomal
COL11A2 3 Autosomal
COL9A1/
COL9A2
inheritance Clinical manifestations
dominant
dominant
dominant
4 Autosomal
recessive
Vitreous membrane like degeneration,
congenital macroglossia, arthropathy,
hearing impairment, cleft palate, etc
Fibrous or beaded vitreous degeneration 10%–20%
No general eye involvement Rarely
Vitreous membrane like degeneration,
congenital macroglossia, no systemic
involvement seen
Proportion
80%–90%
Rarely
12.4 Retinal Detachment Secondary toStickler Syndrome
263
(i) GRT
1. For fresh GRT without obvious PVR, because the vitreous liquefaction at
the posterior pole is obvious and the mobility of the detached retinais high
enough, vitrectomy is usually not so difcult, but the following details
should be noted: (1) adequate removal of the peripheral vitreous to relieve
the traction; (2) because mobility of theretina is high, iatrogenic damage
caused by the intravitreal instruments should be avoided; and (3) if uid/air
exchange is required, the eye position should be adjusted appropriately to
avoid rolling and slippage of the retinal ap.
If you choose to ll with silicone oil, you can directly choose the oil/
liquid exchange method to prevent slippage of the retinal ap; some patients
can also choose to short- or medium-term tamponade of heavy liquid, and
the patient can maintain the supine position after surgery, and then remove
the heavy liquid 7~10daysafter the initial surgery.
2. With obvious PVR mainly because of the longer duration, the surgery is
more difcultand easy to have iatrogenic damages.Encirling can be considered as a supplementary option to prevent slippage of the peripheral retina.
(ii) Rhegmatogenous Retinal Detachment
Unlike common rhegmatogenous retinal detachment, the primary break is
often multiple, and the crumpled and degenerated vitreous membrane-like tissue will adhere tightly to the retina, so attention should be paid to the strength
and scope when peeling off the proliferative membrane during surgery, otherwise it will cause new ssures or enlargement of ssures.
Some patients with long-standing occult disease may have different degrees
of subretinal proliferative tissues, which are often in the peripheral part, so the
retina can be attened with heavy water to determine the area where the subretinal retractive cords are located, and then the retinotomy site can be reasonably designed, and nally the proliferative cords or membranes can be pulled
out with intraocular forceps. The key to this step is the design of the retinal
incision site, which is generally close to the superior and mid-periphery.
In younger patients, simultaneous scleral episcleral ligation is generally
recommended.
(iii) Retinal Detachment Without Visualization of the Break
The main cause of detachment is diffuse vitreous crinkling degeneration
with extensive adhesions to the underlying retina, and the primary ssure is not
visible although the retina is extensively detached preoperatively.
The surgical focus is to remove the vitreous proliferative membrane in the
posterior pole and mid-periphery, inject TA and ICG if necessary to better label
the residual vitreous, and strive for adequate removal of the crinkled vitreous.
A retinotomy is often required, with the superior and mid-peripheral areas preferred. In younger patients, concomitant scleral episcleral ligation is generally
recommended to relieve the centripetal pull that may be associated with the
circumferential vitreous.
264
12 Vitreoretinal Surgery inChildren andAdolescents
12.5 Summary
“The surgeon who operates without understanding the nature of the disease is like a
traveler who journeys without a map.” In the demanding eld of pediatric retina,
adhering to basic surgical protocols is merely the baseline; true mastery requires a
profound comprehension of the underlying pathophysiologic mechanisms unique to
the developing eye. This deep understanding enables the surgeon to transition from
a generalized approach to a highly targeted surgical strategy, tailored to the specic
vascular and tractional dynamics of each case. Furthermore, in the delicate environment of a child’s eye, the prevention of iatrogenic trauma is paramount—precision
must be absolute to avoid complications of medical origin. When managed with this
level of expertise, modern vitreoretinal technology provides a powerful means to
effectively salvage and preserve vision that was once deemed lost.
Complex pathologies such as Retinopathy of Prematurity (ROP), Familial
Exudative Vitreoretinopathy (FEVR), and Stickler Syndrome represent some of the
most formidable challenges in ophthalmology. These conditions are not only rare
but technically exhausting to treat, characterized by aggressive vitreoretinal traction
and fragile anatomical structures. Mastery of these pathologies necessitates a tripartite foundation: a comprehensive intellectual grasp of the disease’s evolution, the
performance equipment. Only through this integration can the surgeon navigate
these difcult cases with the condence and precision required for success.
renement of advanced surgical skills, and the utilization of specialized, high-