Sudha Nallasamy¹, Stefanie L. Davidson¹, Lori J. Howell², Holly Hedrick², Alan W. Flake², Timothy M. Crombleholme², N. Scott Adzick² and Terri L. Young¹

¹Division of Ophthalmology, Children’s Hospital of Philadelphia, Philadelphia, PA. ²The Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, Philadelphia, PA.

Abstract

Background: Fetal surgery is selectively offered for severe or life-threatening fetal malformations. These infants are often born prematurely and are thus at risk for retinopathy of prematurity (ROP). It is not known whether fetal surgery confers an increased risk of developing severe ROP relative to published rates in standard premature populations ≤37 weeks of age grouped by birth weight (<1500 grams or ≥1500 grams).

Design: This is a retrospective chart review.

Methods: We reviewed the charts of 137 patients who underwent open fetal/fetoscopic surgery from 1996–2004. Surgical indications included twin-twin transfusion syndrome (TTTS), myelomeningocele (MMC), congenital diaphragmatic hernia (CDH), sacrococcygeal teratoma (SCT), cystic adenomatoid malformation of the lung (CCAM), and twin reversed arterial perfusion sequence (TRAP). Of these, 17 patients had local ROP examination data. Binomial tests were performed to assess whether rates of ROP in our fetal/fetoscopic surgery cohort were significantly different from published rates.

Results: There were 5 patients each with an underlying diagnosis of TTTS and MMC, 2 patients each with CDH and TRAP, and 1 patient each with SCT, CCAM, and mediastinal teratoma. The mean gestational age at surgery was 23⁴/₇  ± 2³/₇ weeks, mean gestational age at birth was 30 ± 2⁵/₇ weeks, and mean birth weight was 1449 ± 510 grams (610–2485). Compared to published rates of ROP and threshold ROP, our fetal surgery patients had significantly higher rates of ROP and threshold ROP in both the <1500 grams and the ≥1500 grams group (all p-values < 0.05).

Conclusions: Fetal/fetoscopic surgery appears to significantly increase the rate of ROP and threshold ROP development. Greater numbers are needed to confirm these observations.