Peters anomaly: A 5‐year experience

Abstract

Background Peters anomaly is a rare, congenital eye malformation characterized by an opaque cornea and blurred vision. Central corneal opacification can lead to delayed progression of visual development caused by defects in Descemet membrane and the posterior stroma. These children require several anesthetics for multiple eye examinations under anesthesia and corneal transplantation. Aims We sought to review the anesthetic management of patients with Peters anomaly for ophthalmologic procedures at Westchester Medical Center, a major referral center for Peters anomaly. Methods A retrospective chart review was completed which included pediatric patients who underwent ophthalmologic procedures related to Peters anomaly from 2013‐2018. Results The charts of 35 patients with Peters anomaly were reviewed: 14 patients with Peters anomaly Type I, 10 patients with Peters anomaly Type II, and 11 patients with Peters plus syndrome. Thirty patients required three procedures on average, two examinations under anesthesia pre‐ and post‐transplant, and anesthesia for the corneal transplant itself. The youngest patient encountered for examination under anesthesia was 39‐week postconceptual age. Anesthetic time for examination under anesthesia averaged 31 minutes using a laryngeal mask airway while corneal transplant averaged 104 minutes utilizing endotracheal intubation. Postanesthesia care unit stay averaged 51 minutes following examination under anesthesia and 65 minutes after corneal transplant. All examinations under anesthesia were successfully completed without adverse events with the use of a laryngeal mask airway. This case series includes two patients with Goldenhar syndrome and Al‐Gazali syndrome accompanying Peters anomaly. Conclusion Although limited by its retrospective nature, this case series describes the cardiac and systemic implications of patients undergoing anesthesia with Peters anomaly. Our experience indicates that general anesthesia and airway manipulation are tolerated with minor postoperative concerns in these infants. Pediatric patients with Peters anomaly require multiple anesthetics for repeated ophthalmologic interventions. The laryngeal mask airway can be routinely utilized in infants less than 3 months of age for an eye examination under anesthesia with no airway complications noted. Perioperative providers should be aware of the multisystemic implications in patients with Peters plus syndrome.

New Phenotypic Feature in a Patient With a Rare Triplication of the 22q11.2 Region Presenting With Peters Anomaly, Congenital Heart Disease, and Global Developmental Delay: A Case Report and Literature Review

“…Central corneal opacification can lead to delayed visual development progression caused by defects in the Descemet membrane and the posterior stroma [ 8 ]. Our patient has Peters anomaly, a rare, congenital eye malformation involving the anterior segment of the eye characterized by an opaque cornea and blurred vision.…”

Overview of Congenital Corneal Opacities: Clinical Diagnosis, Treatment, and Prognosis

“…Patients tolerated general anesthesia and airway manipulations with minor postoperative concerns even in patient diagnosed with Peters plus syndrome (11 patients). 32 Sclerocornea The term sclerocornea has often been used as a misnomer by many clinicians to describe total congenital corneal opacification; currently there is some disagreement as how to best define this disease process. Sclerocornea was first decribed in 1965 by Bloch when he reported a pedigree with isolated peripheral corneal opacity without total corneal opacification.…”

“…In a retrospective study of 35 patients with Peters anomaly undergoing general anesthesia, 30 of 35 patients were administered general anesthesia 3 times; once for the PKP and twice for examination under anesthesia (preoperative and postoperative). Patients tolerated general anesthesia and airway manipulations with minor postoperative concerns even in patient diagnosed with Peters plus syndrome (11 patients) 32…”

Descemet Stripping Automated Endothelial Keratoplasty in Pediatric Age Group: A Decade of Our Experience

“…2,[8][9][10] Furthermore, a lower reversal rate after an episode of endothelial rejection further complicates visual rehabilitation. 5,[13][14][15] Recently, selective corneal replacement procedures are gaining popularity in the pediatric age group. 5,6,[16][17][18][19] DSAEK is an evolving procedure and has shown excellent results in adults for isolated endothelial dysfunction.…”

“…2,8–10 Furthermore, a lower reversal rate after an episode of endothelial rejection further complicates visual rehabilitation. 5,13–15…”