Ophthalmic Surgery, Lasers and Imaging Retina

Case Report 

Persistent Plus Disease Subsequent to Panretinal Photocoagulation in an Infant With Retinopathy of Prematurity

Saumya M. Shah, MD; Natalia F. Callaway, MD, MS; Darius M. Moshfeghi, MD

Abstract

Plus disease, or the presence of vascular dilation and tortuosity, is the most reliable predictor of the progression of retinopathy of prematurity (ROP), making resolution of plus disease one of the earliest signs of ROP regression. Patients with certain comorbid conditions such as anemia and cardiovascular disease may have persistent plus-like disease following successful resolution of ROP. The authors present a case of a 24-week premature infant who was treated with panretinal photocoagulation for stage 3, zone II.

[Ophthalmic Surg Lasers Imaging Retina. 2019;50:520–521.]

Abstract

Plus disease, or the presence of vascular dilation and tortuosity, is the most reliable predictor of the progression of retinopathy of prematurity (ROP), making resolution of plus disease one of the earliest signs of ROP regression. Patients with certain comorbid conditions such as anemia and cardiovascular disease may have persistent plus-like disease following successful resolution of ROP. The authors present a case of a 24-week premature infant who was treated with panretinal photocoagulation for stage 3, zone II.

[Ophthalmic Surg Lasers Imaging Retina. 2019;50:520–521.]

Introduction

Advances in neonatal care and technology have increased the survival of premature infants and as a result, retinopathy of prematurity (ROP) has become the leading cause of preventable childhood blindness contributing to infantile morbidity.1 Intervention for ROP is triggered by posterior disease, advanced stage, and the presence of vascular dilatation and tortuosity, also known as plus disease.2 Of these, plus disease is the most reliable predictor of the progression of ROP, making resolution of plus disease one of the earliest signs of ROP regression.3 Patients with certain comorbid conditions such as anemia and cardiovascular disease may have persistent plus-like disease following successful resolution of ROP.4 We present a case of a premature infant who was treated with panretinal photocoagulation (PRP) for stage 3 ROP, and maintained the appearance of plus disease at the most recent 5-year follow-up visit.

Case Report

A premature male infant was born vaginally at 24 weeks and 2 days of gestational age with a birth weight of 595 grams to a 40-year-old woman with normal prenatal care. The patient was diagnosed with a patent ductus arteriosus and developed numerous respiratory sequelae including pulmonary hypertension requiring high frequency oscillatory ventilation and continuous positive airway pressure (CPAP), eventually leading to chronic lung disease. He also underwent multiple exploratory laparotomies and surgical repairs for intestinal perforations and hernias, as well as one hospitalization related to sepsis.

At 2 months of age, he was noted to have stage 3, zone II ROP bilaterally with no evidence of plus, persistent plus disease, retinal breaks, retinal detachments, hemorrhages, masses, or abscesses. The patient underwent PRP in all four quadrants of the retina in both eyes. The patient was followed biweekly until the age of 50 weeks gestational age and subsequently on a yearly basis. He was followed by pediatric ophthalmology and developed amblyopia in the left eye and progressive myopia bilaterally that were treated with patching and continuous polycarbonate safety lenses use, respectively. At age 5, the patient still has persistent vascular tortuosity in all four quadrants of the retina bilaterally (Figure 1). Fortunately, there was no evidence of sequential retinal breaks or detachments, and the patient maintained visual acuity of 20/40 in the right eye and 20/60 in the left eye. The patient continues to be evaluated annually with appropriate imaging.

Presence of persistent vascular tortuosity in all four quadrants of the retina at age 5 in the right (A) and left eyes (B).

Figure 1.

Presence of persistent vascular tortuosity in all four quadrants of the retina at age 5 in the right (A) and left eyes (B).

Discussion

We present a case of a 24-week premature infant with patent ductus arteriosus and cardiopulmonary instability who developed stage 3, zone II ROP treated with PRP that demonstrates persistent plus disease without other ROP complications 5 years after birth with regular follow-up. Plus disease is defined as arterial tortuosity and venous dilation of the central posterior retinal blood vessels.2 The timeline for the resolution of plus disease is distinct for laser photocoagulation (2 to 3 weeks)5 and vascular endothelial growth factor inhibitor (days to 1 week) treatments.6 However, resolution of plus is an absolute indication of disease improvement. Occurrence of persistent plus disease4 and persistent ROP7 has previously been reported in premature infants with tetralogy of Fallot. In these reports, the persistent plus disease is hypothesized to be due to the overall decreased systemic oxygen saturation of arterial blood. Active stage 3 ROP, usage of CPAP, and anemia have also been associated with delayed involution of ROP subsequent to treatment. Although our patient did not have tetralogy of Fallot, he was diagnosed with a patent ductus arteriosus and persistent pulmonary hypertension requiring CPAP, which can cause a state of systemic hypoperfusion and hypoxemia, altering the blood flow through the retinal vasculature and possibly contributing to the persistent plus disease.8,9 To our knowledge, there are limited reports that describe the occurrence of persistent plus disease in premature infants who received appropriate PRP treatment. Further studies need to be conducted in order to understand the pathophysiology behind this mechanism.

References

  1. Moraes Freitas A, Mörschbächer R, Thorell MR, Rhoden EL. Incidence and risk factors for retinopathy of prematurity: A retrospective cohort study. Int J Retin Vitr. 2018;4:20. doi:10.1186/s40942-018-0125-z [CrossRef]
  2. Campbell JP, Ataer-Cansizoglu E, Bolon-Canedo V, et al. Expert diagnosis of plus disease in retinopathy of prematurity from computer-based image analysis. JAMA Ophthalmol. 2016;134(6):651–657. doi:10.1001/jamaophthalmol.2016.0611 [CrossRef]
  3. Solarte CE, Awad AH, Wilson CM, Ells A. Plus disease: Why is it important in retinopathy of prematurity?Middle East Afr J Ophthalmol. 2010;17(2):148–155. doi:10.4103/0974-9233.63080 [CrossRef]
  4. Paulus YM, Moshfeghi DM. Persistent plus disease after laser in retinopathy of prematurity with tetralogy of Fallot. Eur J Ophthalmol. 2013;23(5):764–766. doi:10.5301/ejo.5000295 [CrossRef]
  5. Coats DK, Miller AM, Brady McCreery KM, Holz ER, Paysse EA. Involution of threshold retinopathy of prematurity after diode laser photocoagulation. Ophthalmology. 2004;111(10):1894–1898. doi:10.1016/j.ophtha.2004.02.005 [CrossRef]
  6. Isaac M, Tehrani N, Mireskandari KMedscape. Involution patterns of retinopathy of prematurity after treatment with intravitreal bevacizumab: implications for follow-up. Eye (Lond). 2016;30(3):333–341. doi:10.1038/eye.2015.289 [CrossRef]
  7. Gunay M, Yavuz T, Celik G, Uludag G. Persistence of retinopathy of prematurity in an infant with tetralogy of Fallot. Case Rep Pediatr. 2016;2016:7070316.
  8. Dice JE, Bhatia J. Patent ductus arteriosus: An overview. J Pediatr Pharmacol Ther. 2007;12(3):138–146.
  9. Steinhorn RH. Neonatal pulmonary hypertension. Pediatr Crit Care Med. 2010;11(2 Suppl):S79–S84. doi:10.1097/PCC.0b013e3181c76cdc [CrossRef]
Authors

From Mayo Clinic School of Medicine, Rochester, MN (SMS); and Byers Eye Institute, Horngren Family Vitreoretinal Center, Department of Ophthalmology, Stanford University School of Medicine, Palo Alto, CA (NFC, DMM).

Dr. Moshfeghi reports the following financial disclosures: 1-800 Contacts (Board of Directors, equity), Alcon (Data Monitoring Committee), dSentz (Founder, Board of Directors, equity), Iconic Therapeutics (Steering Committee), Grand Legend Technology (equity), Irenix (scientific advisory board), Novartis (CME consultant), Pr3vent (Founder, Board of Directors, equity), Promisight (Founder, Board of Directors, equity), Pykus (Scientifc advisory board, equity), Regeneron (CME consultant), Versl (Founder, equity), Vindico (CME consultant), Visunex Medical Systems Co. Ltd (Founder, equity, consultant). The remaining authors report no relevant financial disclosures.

Address correspondence to Darius M. Moshfeghi, MD, Byers Eye Institute, Horngren Family Vitreoretinal Center, Department of Ophthalmology, Stanford University School of Medicine, 2452 Watson Court, Room 2277, Palo Alto, CA 94303; email: darius.moshfeghi@stanford.edu.

Received: August 31, 2018
Accepted: February 01, 2019

10.3928/23258160-20190806-08

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