378

Kathleen A. Marinelli

Majid Rasoulpour

53 Peritoneal Dialysis

Acute Peritoneal Dialysis (1–5)

In neonates, acute peritoneal dialysis (PD) is frequently preferred over hemodialysis (HD), continuous arteriovenous

hemofiltration with or without dialysis (CAVH/D), and continuous venovenous hemofiltration with or without dialysis

(CVVH/D), because it is technically easier to perform.

Peritoneal surface area per kilogram of body weight is relatively larger in newborns and children than in adults.

Therefore, PD usually allows adequate clearance and

removal of excess fluid (6). In addition, PD avoids the need

for anticoagulation and maintenance of adequate vascular

access, which are required for the other methods (7).

A. Indications

1. Renal failure, when conservative management has

failed to adequately control any of the following conditions (8,9).

a. Hypervolemia

b. Hyperkalemia

c. Hyponatremia

d. Refractory metabolic acidosis

e. Hyperphosphatemia

f. Azotemia

g. Additional fluid space needed for delivering drugs

and/or nutrition

2. Inherited disorders of organic and amino acid metabolism when HD or CVVH/D is unavailable (10,11)

a. In hyperammonemic metabolic crisis, however, evidence suggests that ammonia is more efficiently

removed by extracorporeal techniques than by PD

(12,13).

b. In babies with imminent or current intracranial

hemorrhage, PD is considered the therapeutic

option of choice, especially in nonhyperammonemic disorders (12).

B. Relative Contraindications

1. Acute abdomen

2. Abdominal adhesions

3. Immediately after abdominal surgery (14)

4. Diaphragmatic or abdominal wall disruptions

C. Equipment (Figs. 53.1 through 53.3)

Sterile

1. Masks, drapes, gowns, and gloves

2. Povidone–iodine

3. 1% lidocaine without epinephrine

4. 3-mL syringe with 25-gauge needle

5. IV cutdown tray with no. 11 surgical blade

6. 3-0 Prolene sutures (either as part of cutdown tray or

separately)

 

7. 22-gauge angiocatheter or a femoral catheter with

guidewire

8. A temporary catheter such as a 14-gauge angiocatheter

or one of the commercially available temporary dialysis

catheters, (e.g., a Trocath [Trocath Peritoneal Dialysis

Center, Kendall McGaw Laboratories, Sabana Grande,

Puerto Rico])

9. Dialysis solution (1.5%, 2.5%, or 4.25%)

a. Other concentrations can be made by manual mixing of standard solutions

10. Heparin

11. Inline burette set

12. Ultra Set CAPD Disposable Disconnect Y-Set

13. MiniCap Extended Life PD Transfer Set with Twist

Cap

14. Medicap with povidone–iodine solution

15. FlexiCap Disconnect Cap with povidone–iodine solution

Nonsterile

1. Waterproof tape

2. Baby weigh scale with low resolution (e.g., Medela,

which has a resolution of 2 g from 0 to 6,000 g) (Fig.

53.2)

An alternative approach is to utilize a pediatric

cycler set. Experience in using this equipment is necessary. We recommend a commercially available cycler

that provides a minimum fill volume of 50 mL with

10-mL increments.

Chapter 53 ■ Peritoneal Dialysis 379

3. HomeChoice Automated PD System (Fig. 53.2) or any

other reliable fluid warmer

4. IV pole

D. Preprocedure Care

1. Obtain informed consent.

2. Check body weight and abdominal girth.

3. Check for infection at the insertion site.

4. Decompress the stomach.

5. Catheterize the bladder.

6. Place preweighed diaper under the patient.

Before assembly of system, wash hands and put on a

mask. All connections should be made using sterile

technique. Universal precautions should be observed

(Chapter 5). Keep all tubing clamped. See Fig. 53.3 for

connections.

7. Add 500 U of heparin to each 1 L of the dialysis solution. Start with 1.5% dialysate.

8. Warm a liter bag of dialysate (Dianeal or other), or a

larger bag if 1 L dialysate is not available, by resting it on

the heating surface of the HomeChoice Automated PD

System, or a reliable fluid warmer. The temperature can

be set between 35°C and 37°C. For a newborn, keep the

temperature at 37°C (in older pediatric patients, the

temperature is usually set to 36°C, and occasionally to

35°C if the environmental temperature is high).

9. Spike the inline burette set (Abbott Laboratories, North

Chicago, Illinois) into the dialysate (Dianeal or other)

 

Pediatric Ophthalmology and Strabismus. Screening examinations of premature infants for retinopathy of prematurity. Pediatrics.

2006;117:572. Erratum Pediatrics 2006;118:1324.

2. Wilkinson AR, Haines L, Head K, et al. UK retinopathy of prematurity guidelines. Early Hum Dev. 2008;84:71.

3. Jefferies AL, Canadian Pediatric Society; Fetus and Newborn

Committee. Retinopathy of prematurity: recommendations for

screening. Paediatr Child Health. 2010;15:667.

4. An International Committee for the Classification of Retinopathy

of Prematurity. The international classification of retinopathy of

prematurity revisited. Arch Ophthalmol. 2005;123:991.

5. Silva RA, Murakami Y, Lad EM, et al. Stanford University network for diagnosis of retinopathy of prematurity (SUNDROP): 36

month experience with telemedicine screening. Ophthalmic Surg

Lasers Imaging. 2011;42:12.

6. Kivlin JD, Biglan AW, Gordon RA, et al. For Cryotherapy for

Retinopathy of Prematurity Group. Early retinal vessel development and iris vessel dilatation as factors in retinopathy of prematurity. Arch Ophthalmol. 1996;114:150.

7. Marcus I, Salchow DJ, Stoessel KM, et al. An ROP screening

dilemma: hereditary cataracts developing in a premature infant

after birth. J Pediatr Ophthalmol Strabismus. 2012;14:49;e1.

8. Simpson JL, Melia M, Yang MB. A report by the American

Academy of Ophthalmology. Current role of cryotherapy in retinopathy of prematurity. Ophthalmology. 2012;119:873.

9. Houston SK, Wykoff CC, Berrocal AM, et al. Laser treatment for

retinopathy of prematurity. Lasers Med Sci. 2011 Dec 2. [Epub

ahead of print].

10. Early Treatment for Retinopathy of Prematurity Cooperative

Group. Revised indications for the treatment of retinopathy of

prematurity: results of the Early Treatment for retinopathy of

Prematurity randomized trial. Arch Ophthalmol. 2003;121:1684.

11. Early Treatment for Retinopathy of Prematurity Cooperative Group.

Final visual acuity results in the Early Treatment for Retinopathy of

Prematurity Study. Arch Ophthalmol. 2010;128: 663.

12. Hubbard GB. Surgical management of retinopathy of prematurity. Curr Opin Ophthalmol. 2008;19:384.

13. Mintz-Hittner HA, Kennedy KA, Chuang AZ, et al. Efficacy of

intravitreal bevacizumab for stage 3+ retinopathy of prematurity.

N Engl J Med. 2011;364:603.

14. Darlow BA, Ellis AL, Gilbert CE, et al. Are we there yet?

Bevacizumab therapy for retinopathy of prematurity. Arch Dis

Child Fetal Neonatal Ed. 2011 Dec 30. [Epub ahead of print].

15. Tolentino M. Systemic and ocular safety of intravitreal anti-VEGF therapies for ocular neovascular disease. Surv Ophthalmol. 2011;56:95.

16. Spandau U, Tomic Z, Ewald U, et al. Time to consider a new

treatment protocol for aggressive posterior retinopathy of prematurity? Acta Ophthalmol. 2012 Jan 23. [Epub ahead of print].

17. Lim LS, Mitchell P, Wong TY. Bevacizumab for retinopathy of

prematurity: letter. N Engl J Med. 2011;364:2359.

18. Day S, Menke AM, Abbott RL. Retinopathy of prematurity malpractice claims: the Ophthalmic Mutual Insurance Company

experience. Arch Ophthalmol. 2009;127:794.

19. Davitt BV, Quinn GE, Wallace DK, et al. Early Treatment for

Retinopathy of Prematurity Cooperative Group. Astigmatism progression in the early treatment for retinopathy study to 6 years of

age. Ophthalmology. 2011;118:2326.

 

d. Sterile cotton-tipped applicators (CTA)

e. Sterile gloves

f. Topical Betadine

g. Topical antibiotic drops (ciprofloxacin 0.3%) and

ointment

h. Sterile syringe of bevacizumab (0.625 mg in

0.025 mL) with 30-gauge needle (one per eye)

7. Complications (Table 52.3)

a. The most worrisome risk is postinjection infection

(endophthalmitis). Babies with active or recent ocular surface or lid infections (e.g., conjunctivitis)

should not have intravitreal injection

b. The risk of adverse systemic side effects (bradycardia, oxygen desaturation) is mitigated by the absence

of systemic sedation/anesthesia, and the rapid nature

of the procedure. However, it is reasonable to follow

those precautions listed for laser treatment in

Section F4.

8. Technique

a. The baby’s eyes are dilated according to the standard

dilation protocol.

b. Sterile towels are placed around the baby’s head.

c. Topical anesthetics are instilled.

d. The lids are prepped with Betadine.

e. Wire lid speculum is placed.

f. The caliper is used to mark a spot on the sclera

1.7 mm posterior to the limbus in the inferotemporal quadrant.

g. A Betadine-soaked CTA is gently pressed over the

mark and excess Betadine is allowed to collect in the

inferior fornix.

h. The injection is given.

i. A topical antibiotic drop is given.

j. The ophthalmologist performs binocular indirect

ophthalmoscopy.

k. Dexamethasone/polymyxin B/dexamethasone ointment may be instilled.

Chapter 52 ■ Treatment of Retinopathy of Prematurity 377

9. Postinjection Care/Concerns

a. Topical antibiotic drops should be instilled 3 to

4 times a day for 3 days.

b. Portable slit-lamp examination should be performed

48 to 72 hours postinjection

c. Any signs of infection (lid edema and erythema,

conjunctival injection, clouding of the cornea)

should be reported immediately to the treating ophthalmologist.

d. Examination by treating ophthalmologist in 1 week.

E. Postdischarge Care

A critical component of treatment of ROP is postdischarge

care.

1. No baby with any ROP, or who has regressed ROP after

treatment, should leave the neonatal intensive care

unit (NICU) without a scheduled follow-up examination (1,18).

2. It is imperative that infants who develop any stage of

ROP, especially those with prethreshold stage 3 or those

that have received treatment, are seen within 1 to

2 weeks of discharge, or as directed by the ophthalmologist involved in the baby’s care.

3. A careful, reproducible tracking system for arranging

follow-up should be established by every NICU. A member of the staff of each NICU should be responsible for

maintaining and periodically auditing this system.

4. Verbal and written instructions for follow-up should be

given to the parents. Parents should be given a discharge

form indicating their baby’s scheduled follow-up among

their discharge instructions. The importance of scheduled follow-up should be prominently stated on the form.

F. Outcome

1. Early treatment for type I high-risk prethreshold ROP

has been shown to improve retinal structural outcome

and visual acuity outcomes at 6 years of age (11).

2. Favorable outcome with vision of 20/40 or better was

noted in 35% of treated eyes.

3. However, 65% of eyes receiving early treatment develop

visual acuity worse than 20/40.

4. Unfavorable outcome despite treatment: Visual acuity

20/200 in 15%; blindness or low vision in 9%.

5. The outcome for eyes with Zone I disease, although

poor, has improved with laser and incisional surgery (vitrectomy). Specifically, laser treatment of the posterior

avascular retina can be accomplished easily and without

necessitating conjunctival incisions, as in cryotherapy.

6. Treated eyes carry a risk of retinal dystopia, myopia, and

subsequent strabismus and amblyopia (11,19). To minimize the effect of refractive errors and strabismus, careful

follow-up by a pediatric ophthalmologist is mandatory.

7. Premature infants are at risk for intracranial pathologies

that may limit visual function. Pediatric ophthalmologists, neurologists, and others involved in the care of

former preemies should be in frequent contact in order

to address the often complex and changing visual deficits present in these children.

References

1. Section on Ophthalmology, American Academy of Pediatrics,

American Academy of Ophthalmology, American Association for

 

374 Section IX ■ Miscellaneous Procedures

j. Portable argon or diode laser (9) with indirect (headlamp) delivery system

k. Appropriate laser safety goggles

5. Precautions and Complications (Table 52.2)

a. Ensure that laser is fully functional.

b. If the infant is at high risk for an adverse event that

would terminate treatment prematurely, treat the

more advanced eye first (assuming both have threshold ROP).

c. Discontinue feedings at least 4 hours before the procedure, or empty the stomach with an orogastric tube.

d. Establish IV access for infusions of medications and

IV fluids.

e. Observe oxygen saturation monitor carefully, and

adjust administered oxygen appropriately.

Table 52.2 Complications of Laser for Retinopathy

Complication Treatment/Action

Systemic: Intra- and immediately postop

Bradycardia Interrupt treatment.

Assess airway, oxygen delivery.

Atropine 0.1 mg IV

Hypoxia/cyanosis Evaluate airway.

Administer supplemental oxygen.

Apnea Evaluate airway.

Gentle stimulation.

Administer supplemental oxygen.

Hand-ventilate (self-inflating resuscitation bag, face mask).

Tachycardia Assess pain control.

Administer additional analgesic.

Monitor blood pressure and perfusion.

Hypertension Assess pain control.

Administer additional analgesic.

If moderate, observe.

If severe, consider hydralazine 0.1 mg/kg IV

Arrhythmia Manage as appropriate for arrhythmia.

Seizure (mechanism uncertain: ?

anticholinergic effect)

Supportive care.

Phenobarbital.

Ocular: Intraop

Closure of central artery Relieve pressure on globe (stop scleral depression).

Corneal clouding/abrasion Rinse with balanced salt solution/saline.

Interrupt treatment.

Retinal/vitreous/choroidal hemorrhage Gentle pressure on globe (until arterial pulsations visible).

Avoid lasering blood.

May have to terminate treatment if extensive.

Ocular: Postop

Conjunctival hemorrhage Observation.

Conjunctival laceration Antibiotic ointment t.i.d. for 3–4 d.

Corneal abrasion Antibiotic ointment t.i.d. for 3–4 d.

Follow with slit lamp exam with fluorescein.

Hyphema Topical cycloplegic and steroids.

Follow intraocular pressure closely.

Consider washout if high pressure, no resolution in 7–10 d.

Retinal/vitreous/choroidal hemorrhage Close follow-up.

Ocular: Late

Amblyopia, strabismus, myopia Pediatric ophthalmology assessment 3–4 mo after treatment(s).

Educate parents prior to discharge regarding need for regular

ophthalmology follow-up.

t.i.d., three times per day.

 

Chapter 52 ■ Treatment of Retinopathy of Prematurity 375

f. Stabilize the infant: Correct electrolyte imbalances,

platelet deficiency, etc.

g. Use only 1% phenylephrine if there is a history of

hypertension.

h. Wipe off any excess drops spilling onto the skin to

avoid transcutaneous absorption (skin vessel blanching occurs with phenylephrine).

6. Technique

a. General preparation

(1) Instill eyedrops (per orders from ophthalmologist) into both eyes in the hour prior to procedure. Maximal dilation is critical for optimum

laser; therefore, several (three or four) instillations of drops may be required, especially in eyes

with neovascularization/vascular engorgement

of the iris.

(2) Transport the patient to surgical suite or designated procedure room in the nursery.

(3) Ensure monitors are attached and functioning.

b. Immobilize infant: Swaddle in a clean towel or blanket to immobilize arms and legs.

c. Ensure that the IV tubing is accessible.

d. Administer IV sedation.

If local anesthesia is to be used, a combination of

topical (e.g., tetracaine, proparacaine) and systemic

analgesic/sedative (e.g., IV morphine) medications

are administered prior to injection.

e. Distribute laser safety goggles and dim overhead

lights.

f. Retract lids.

g. Perform laser: Cover the avascular retina with confluent gray–white burns (Fig. 52.5).

h. Have an assistant count and record the number of

spots and the duration and power of each spot.

7. Postoperative Care

a. Instill 0.25% scopolamine hydrobromide in treated

eye(s) daily for 3 to 5 days.

b. Apply antibiotic–steroid preparation (e.g., tobramycin–

dexamethasone) to treated eye(s) three to four times

daily for 5 to 7 days.

c. Monitor the patient with a cardiorespiratory monitor

for 24 to 72 hours.

d. Perform a dilated retinal exam 1 to 2 weeks after

treatment.

e. If opaque media are present at the time of laser, or if

the pupil does not dilate adequately, complete treatment of the avascular retina may be impossible, and

“skip areas” may be visible in the weeks after treatment. Treatment of these areas should be considered if there is not marked resolution of the adjacent

plus disease and/or neovascularization.

f. Follow the infant every 1 to 2 weeks until the ROP

resolves completely. If at the time of discharge ROP

is still present, ensure that the parents and the physicians responsible for the care of the infant after discharge are aware of the extreme importance of

maintaining a regular schedule of outpatient examinations. Once the ROP has resolved completely, the

baby should be seen by a pediatric ophthalmologist

within 1 to 2 months to assess vision, ocular alignment and motility, refractive status, etc.

g. Long-term follow-up over several years is necessary.

See outcomes and postdischarge follow-up below.

D. Intravitreal Injection for ROP

1. Background

Recently, the efficacy of anti-VEGF drug bevacizumab for

use in ROP has been reported (13). The drug halts the

development of new vessels and halts disease progression.

Intravitreal injections of anti-VEGF agents have been used

to treat wet (neovascular) age-related macular degeneration (AMD), proliferative diabetic retinopathy, neovascular

glaucoma, etc. Although there is considerable debate and

no consensus on its use in ROP, this section is being

included in the Atlas for completeness, and to provide

additional treatment options if laser therapy is not possible

(14–16).

2. Precautions

a. The major concern with bevacizumab in premature

infants with ROP is systemic absorption and its

effect on the developing infant. Bevacizumab is

absorbed systemically after intravitreal injection.

The risks of systemic effects on developing neonates

have not been established.

b. The optimal and safe dose of bevacizumab in ROP

has not been determined; the current dose (0.625 mg)

Fig. 52.5. Freshly lasered avascular retina. is extrapolated from that used in adults with ocular

 

Chapter 52 ■ Treatment of Retinopathy of Prematurity 373

(2) Administers topical anesthetic

(3) Ensures that all personnel present at the treatment are wearing laser safety goggles

(4) Performs the laser

(5) Watches for and treats ocular complications that

may arise during and after the procedure

(6) Follows the baby postoperatively until ROP is

resolved

b. Neonatology fellow, attending neonatologist, or

pediatric anesthesiologist

(1) Administers systemic sedative agents (midazolam, fentanyl, ketamine, or a combination)

(2) Monitors patient for and treats any systemic

complications that develop during or after treatment

(3) Provides information to the ophthalmologist

regarding the patient’s overall condition throughout the procedure

c. Assistant to the ophthalmologist

(1) Helps with laser and instruments

(2) Records the treatment parameters used during

treatment

d. Neonatal nurse

(1) Instills dilating drops several times in the hour

preceding treatment

(2) Immobilizes the patient during treatment

4. Equipment

a. Cardiorespiratory, blood pressure, and pulse oximeter

b. Appropriate respiratory support (ventilator, laryngoscope and endotracheal tubes, face mask, self-inflating resuscitation bag, suction, and oxygen source)

c. Emergency medications (atropine, epinephrine,

bicarbonate, calcium, phenobarbital)

Note: Precalculation of weight-appropriate doses

is helpful.

d. Topical ocular anesthetic (e.g., tetracaine, proparacaine)

e. Cycloplegic/mydriatic eye drops: Cyclomydril (Alcon

Laboratories, Fort Worth, Texas) (cyclopentolate

hydrochloride 0.2% and phenylephrine hydrochloride 1%) or 0.5% cyclopentolate and 1% or 2.5%

phenylephrine

f. Calcium alginate-tipped nasopharyngeal applicators

or Flynn depressor (Fig. 52.4), for scleral depression

g. Balanced salt solution for rewetting cornea during

procedure

h. Neonatal eyelid speculum (Fig. 52.4)

i. 28- and 20-diopter lenses

Fig. 52.3. Dilation and tortuosity of iris vessels may be seen in

severe threshold retinopathy of prematurity.

Fig. 52.2. Dilated, tortuous vessels end in vascular shunts at a

thickened ridge of fibrovascular tissue. Avascular retina lies anterior to the ridge.

Fig. 52.4. Lid speculae and Flynn depressor.

 

(1) Determines the need for treatment

Table 52.1 Follow-up Examination Schedule

Findings Follow-up

Stage 1–2 in Zone 1 1 wk or less

Stage 3 in Zone II

Immature retina (no ROP) in Zone I 1–2 wk

Stage 2 in Zone II

Regressing ROP in Zone I

Stage 1 in Zone II 2 wk

Regressing ROP in Zone I

Immature retina (no ROP) in Zone II 2–3 wk

Regressing or Stage 1–2 in Zone III

From Section on Ophthalmology, American Academy of Pediatrics, American

Academy of Ophthalmology, American Association for Pediatric Ophthalmology and

Strabismus. Screening examinations of premature infants for retinopathy of prematurity. Pediatrics. 2006;117:572. Erratum Pediatrics 2006;118:1324.

Fig. 52.1. Scheme of retina of right eye (RE)

and left eye (LE), showing zone borders and clock

hours employed to describe location and extent of

retinopathy of prematurity. (From Committee for

Classification of Retinopathy of Prematurity. An

international classification of retinopathy of prematurity. Arch Ophthalmol. 1984;102:1130, with

permission.)

 

370 Section IX ■ Miscellaneous Procedures

References

1. McComb JG, Ramos AD, Platzker AC, et al. Management of

hydrocephalus secondary to intraventricular hemorrhage in the

preterm infant with a subcutaneous ventricular catheter reservoir.

Neurosurgery. 1983;13:295.

2. Limbrick DD Jr, Mathur A, Johnston JM. Neurosurgical treatment of progressive posthemorrhagic ventricular dilation in preterm infants: a 10 year single institution study. J Neurosurg Pediatr.

2010;6:224.

3. Willis B, Javalkar V, Vannemreddy P, et al. Ventricular reservoir and

ventriculoperitoneal shunts for premature infants with posthemorrhagic hydrocephalus: an institutional experience. J Neurosurg

Pediatr. 2009;3:94.

4. Peretta P, Ragazzi P, Carlino CF, et al. The role of the Ommaya

reservoir and endoscopic third ventriculostomy in the management of post-hemorrhagic hydrocephalus of prematurity. Childs

Nerv Syst. 2007;23:765.

5. Brouwer AJ, Groenendaal F, Koopman C, et al. Intracranial hemorrhage in full term newborns: a hospital based cohort study.

Neuroradiology. 2010;52:567.

6. Wellons JC III, Shannon CN, Kulkarni AV, et al. A multicenter retrospective comparison of conversion from temporary to permanent

cerebrospinal fluid diversion in very low birth weight infants with

posthemorrhagic hydrocephalus. J Neurosurg Pediat.r 2009;4:50.

7. Whitelaw A, Evans D, Carter M, et al. Randomized clinical trial

of prevention of hydrocephalus after intraventricular hemorrhage

in preterm infants: brain-washing versus tapping fluid. Pediatrics.

2007;119:e1071.

8. Moghal NE, Quinn MW, Levene MI, et al. Intraventricular hemorrhage after aspiration of ventricular reservoirs. Arch Dis Child.

1992;67:448.

9. Kormanik K, Praca J, Gorton HJL, et al. Repeated tapping of ventricular reservoir in preterm infants with post-hemorrhagic ventricular dilatation does not increase the risk of reservoir infection.

J Perinatol. 2010;30:218.

 

371

William F. Deegan

Jayashree Ramasethu

Treatment of Retinopathy

of Prematurity

52

Retinopathy of prematurity (ROP), a disorder of developing

retinal blood vessels in the preterm infant, may lead to poor

visual acuity or blindness. Screening and timely treatment

improves visual outcomes.

A. Screening for ROP (1–3)

Guidelines for screening preterm infants for ROP are published and updated regularly (1–3). Recommendations for

screening in the United States are (1)

1. Infants with a birth weight of <1,500 g or a gestational

age of 30 weeks or less (as defined by the attending neonatologist).

2. Selected infants with a birth weight between 1,500 and

2,000 g or gestational age of more than 30 weeks with

an unstable clinical course, including those requiring

cardiopulmonary support and who are believed by their

attending pediatrician or neonatologist to be a high risk.

3. The timing of the first exam varies with gestational age.

The initial examination for infants born between 22

and 27 weeks’ gestational age is at 31 weeks’ postconceptional age (gestational age at birth plus chronological age). Infants born later than 27 weeks’ should be

screened initially 4 weeks after birth.

4. Follow-up exams depend on the retinal findings as classified by the International Classification of ROP (4).

Table 52.1 has been adapted from the joint policy statement of the American Academies of Pediatrics and

Ophthalmology, and the American Association for

Pediatric Ophthalmology and Strabismus (1).

5. Babies whose clinical condition deteriorates should be

followed closely (i.e., weekly), as late reactivation and

worsening are possible.

6. Binocular indirect ophthalmoscopy with scleral depression after pupillary dilation remains the gold standard

to accurately screen and monitor babies with ROP. The

exams are done at the bedside with the assistance of the

baby’s nurse.

7. Telemedicine screening with wide-field imaging has

been shown to have excellent sensitivity and specificity

in some centers (5).

B. Classification of ROP (4)

1. Location: Three zones based on concentric circles,

centered on the optic disc (Fig. 52.1)

a. Zone I: Circle whose center is the optic disc and

whose radius is twice the distance from the optic

disc to the center of the macula

b. Zone II: Circle whose radius extends from the optic

disc to the nasal ora serrata and is peripheral to

Zone I

c. Zone III: Temporal crescent of retina anterior to

Zone II

2. Extent of disease

The retina is divided into 12 equal segments, or

clock hours. The extent of retinopathy specifies the

number of clock hours involved.

3. Staging the disease (1,4) (Figs. 52.2 and 52.3)

a. Stage 1—Demarcation line: A flat white line in the

plane of the retina, separating avascular retina anteriorly from vascularized retina posteriorly

b. Stage 2—Ridge: Elevated fibrovascular tissue

extending out of the plane of the retina and separating the vascularized and avascular retina.

c. Stage 3—Extraretinal fibrovascular proliferation:

Neovascularization extending from the ridge into

the vitreous. This tissue may cause the ridge to

appear ragged or “fuzzy” (Fig. 52.2).

d. Stage 4—Partial retinal detachment: A separation of

the retina from the underlying choroid. Traction by

the vitreous, through the presence of neovascular

tissue, pulls the retina away from its underlying

attachments. The intervening (subretinal) space fills

with a proteinaceous fluid.

(1) Stage 4A: Detachment spares the macula.

(2) Stage 4B: Involves the macula

e. Stage 5—Total retinal detachment: Retinal tissue

becomes inextricably bound to reactive vitreous and

is pulled by the vitreous into the retrolental space

(hence the older term, retrolental fibroplasia).

4. Additional signs indicating severity of active ROP

a. “Plus” disease: Dilation and tortuosity of retinal vessels in at least two quadrants of the eye. This is seen