Surgical Parameters and Prognostic Factors in Persistent Fetal Vasculature: Insights from a Retrospective Cohort Study

Abstract

Introduction

Persistent fetal vasculature (PFV) is a congenital anomaly associated with significant surgical challenges, including a high risk of postoperative retinal detachment (RD). This study aimed to evaluate the impact of surgical approach and axial length (AL) on RD risk and visual outcomes in pediatric PFV management.

Methods

A retrospective cohort study was conducted involving 76 eyes of 74 patients who underwent cataract surgery for PFV between 2014 and 2022. Patients were categorized by RD status postoperatively (14 with RD, 62 without RD). Key predictors, including surgical approach (corneal vs. pars plicata), AL, and age at surgery, were analyzed. The primary outcomes were RD incidence and final best-corrected visual acuity (BCVA).

Results

Compared to the pars plicata approach, the corneal approach was associated with a significantly lower risk of RD, as indicated by a multivariate odds ratio of 0.08 (95% CI 0.01–0.6, P = 0.011). A shorter AL increased the risk of RD (median 17 vs. 20 mm, P = 0.002). The RD group showed poorer visual outcomes (P < 0.001), with a 71% loss of light perception. Surgery before 3 months improved outcomes, regardless of RD. Visible ciliary processes were strongly correlated with RD (P < 0.001).

Conclusions

Corneal surgical approach and longer AL are associated with a lower RD risk in PFV cataract surgery. Early intervention and thorough preoperative assessment of the AL and ciliary processes are crucial for optimal outcomes.

Key Summary Points

Why carry out this study?

Persistent fetal vasculature (PFV) is a challenging congenital anomaly associated with significant visual morbidity and surgical complexity, including a high risk of postoperative retinal detachment (RD).

Optimal surgical approaches for managing PFV remain uncertain, with limited evidence regarding their impact on RD risk and visual outcomes.

This study hypothesized that surgical approach (corneal vs. pars plicata) and anatomical parameters (e.g., axial length) significantly influence RD risk and final visual outcomes.

What was learned from the study?

The corneal approach reduced retinal detachment risk by 92%, and shorter axial length was strongly associated with higher RD risk and poorer outcomes.

Early surgery (before 3 months) improved visual outcomes, underscoring the importance of individualized surgical planning based on anatomy.

Findings support the corneal approach as the preferred technique when feasible and highlight the need for further research on risk stratification and the role of pars plicata surgery in complex cases.

Introduction

Persistent fetal vasculature (PFV), a congenital anomaly resulting from the failure of embryonic hyaloid vasculature regression, poses significant challenges in pediatric ophthalmology because of potential sight-threatening complications such as postoperative rhegmatogenous retinal detachment (RD) and ocular malformations of varying severity that can compromise retinal integrity [1].

Microsurgical techniques have improved PFV management; however, the best strategies for reducing postoperative RD risk remain debatable. Research has investigated surgical approaches, including lens-sparing vitrectomy and combined lensectomy-vitrectomy, emphasizing the complex interplay between surgical techniques and patient-specific factors in determining visual outcomes [2, 3].

Despite these advancements, the relationship between surgical procedures and visual outcomes in patients with PFV remains unclear. Limited data on surgical techniques and postoperative RD in patients with PFV have impeded consensus on optimal management, leaving clinicians with inadequate evidence-based guidance for surgical treatment [4].

We hypothesized that the surgical method for patients with PFV undergoing cataract surgery would significantly affect the final visual acuity and postoperative RD risk. Key predictors of final best-corrected visual acuity (BCVA) and RD risk include age at surgery and ocular biometric parameters, particularly axial length (AL) [5, 6]. Early intervention improves visual outcomes in PFV, and AL affects surgical success and postoperative complications [7, 8]. This study investigated the association between surgical approach, patient factors, and postoperative RD risk in pediatric PFV management. By systematically studying PFV surgery outcomes, we aimed to identify trends in clinical variables and surgical techniques, contributing valuable data to the literature and potentially influencing surgical decisions to enhance visual prognosis in children with this complex ocular condition.

Methods

Study Design and Setting

This retrospective cohort study, conducted at King Khaled Eye Specialist Hospital, a tertiary eye care facility, included two arms. By examining electronic medical records, we identified patients diagnosed with PFV and cataracts between June 1, 2014, and December 31, 2022.

Participants

Patients diagnosed with PFV who underwent cataract surgery were divided into two groups: those who developed RD post-surgery and those who did not.

Inclusion criteria:

Patients diagnosed with cataract and anterior or combined PFV who underwent lens aspiration with or without primary intraocular lens (IOL) implantation prior to reaching 3 years of age. A minimum of 12 months of follow-up data was collected through their most recent evaluation, extending up to 21 years of age.

Exclusion criteria:

Subjects exhibiting retinal dysplasia, severe tractional RD at presentation, posterior PFV, signs of other ocular or systemic conditions causing similar abnormalities, a history of prior ocular surgery, incomplete medical records, or loss to follow-up were excluded from the study because of the inherent complexities of this condition that could potentially confound the results. Intraoperative findings, such as the presence of ciliary processes, anterior segment distortion, and posterior segment involvement, were documented but not systematically utilized to stratify the risk of postoperative complications.

Variables:

The primary outcome variable, postoperative RD, was a binary outcome (present or absent), determined by clinical examination and imaging during follow-up. Key predictors included surgical approach (cornea/pars plicata), age at surgery (months), and AL (mm via A-scan ultrasonography). Potential confounders included patient sex, PFV type (anterior, combined, or posterior), presence of glaucoma, lens status (aphakic or pseudophakic), horizontal corneal diameter (HCD), spherical equivalent (diopters), and BCVA (logarithm of the minimum angle of resolution [logMAR] units). This comprehensive variable set enabled a detailed analysis of the factors influencing RD occurrence, offering a nuanced understanding of the surgical outcomes and risk factors.

Outcomes

To identify key predictors of postoperative outcomes in patients with PFV, focusing on the role of surgical approach (corneal vs. pars plicata) alongside other prognostic factors, such as AL, age at surgery, and intraoperative findings (e.g., visibility of ciliary processes), and final BCVA to provide a comprehensive framework for optimizing surgical decision-making and patient management. Second, this study assessed the relationship between these variables and postoperative RD risk. We examined patient-specific factors (PFV type, glaucoma, and lens status) and their interaction with surgical methods to predict visual outcomes and PFV management success.

Sample size calculation:

The sample size of 45 eyes, comprising nine eyes with postoperative RD and 36 eyes without, was determined using Epi Info (Centers for Disease Control and Prevention [CDC], Atlanta, GA, USA), based on a 49% incidence, 95% confidence interval (CI), 80% power, 0.5 effect size, and a 4:1 ratio of non-RD to RD presence after surgery [9].

Diagnostic criteria and measurements:

PFV diagnosis relied on clinical and ultrasonographic findings, including leukocoria, microphthalmos, elongated ciliary processes, shallow anterior chamber, cataracts, and retrolental fibrovascular membranes. B-scan ultrasonography confirmed the diagnosis, revealing a hyperechoic linear or funnel-shaped structure from the optic disc to the posterior lens surface, retrolental masses or membranes, vitreous opacities or hemorrhage, and tractional RD or retinal folds in advanced stages. Ultrasonographic results and clinical observations were correlated to confirm diagnoses and classify cases as anterior, posterior, or combined PFV, based on the anteroposterior extent of the fibrovascular stalk [10].

Visual assessment:

LogMAR BCVA was evaluated at the final follow-up using age-appropriate tests, including Teller acuity cards and retinoscopy, with the prescribed eyeglasses or contact lenses. For patients unable to perform recognition visual acuity testing, visual acuity was inferred from fixation patterns using a modified technique [7, 11], where central-steady-unmaintained equated to 0.2–0.7 logMAR, uncentral-steady-unmaintained to ≤ 1.2 logMAR, and uncentral-unsteady-unmaintained to ≤ 1.6 logMAR.

Glaucoma diagnosis:

Secondary glaucoma was diagnosed if patients had an intraocular pressure (IOP) > 21 mmHg, along with one or more of the following: corneal abnormalities (edema, haziness, increased thickness, vascularization, scarring), buphthalmos, Haab’s striae, progressive AL elongation, or optic disc cupping. These criteria ensured consistent diagnosis across the study cohort for PFV-associated ocular pathology [12].

Surgical Procedures

The surgical approach was based on the preoperative clinical features. The pars plicata approach was chosen for cases with more extensive retrolental plaque, poor anterior segment visualization, limited anterior chamber space, or severe combined anterior and posterior segment pathology in the PFV. This technique improves visualization and access to ciliary processes, which are essential for managing complex ocular pathologies in pediatric patients.

Patients underwent detailed preoperative assessments, including imaging, under general anesthesia to evaluate the extent of PFV. HCD was measured with a surgical caliper after topical anesthesia. AL was measured using a calibrated A-scan ultrasonography device (Tomey AL-2000; Tomey, Nürnberg, Germany) by a certified ophthalmologist, averaging at least five consistent measurements per eye. A CineScan S B-scan device (Quantel Medical SA, Le Brézet, France) with a 10 MHz probe was used for additional examination and applied to the closed eyelid to obtain multiple cross-sectional ocular images of various meridians.

In this investigation, all surgical procedures were conducted by experienced pediatric anterior segment and vitreoretinal surgeons. The pars plicata approach was employed selectively by surgeons with subspecialty expertise in pediatric vitreoretinal surgery, whereas the corneal approach was implemented by pediatric anterior segment surgeons.

The surgery started positioning the patient supine and sterilizing the operative eye. The pars plicata approach was executed as follows. A limbal incision and two 23-gauge pars plicata incisions were made using a trocar cannula system with sclerotomies posterior to the limbus for bimanual instruments or lensectomy. A vitreous cutter and infusion cannula were inserted through the superior sclerotomies and inferotemporal quadrants. The lens was removed using a vitreous cutter and various instruments were used to manipulate the plaque tissue while minimizing retinal traction and averting retinal breaks or detachment. Core vitrectomy cleared the residual lens material and addressed the vitreoretinal adhesions. The study included eyes that underwent extensive vitrectomy, retinal laser, cryotherapy, or silicone oil tamponade due to intraoperative breaks or RD. These severe cases, which often necessitate the pars plicata approach, were not excluded. In multivariate modeling, we adjusted for the complexity of these high-risk cases by considering variables such as the surgical approach, AL, and ciliary process involvement. Nonetheless, the severity of these conditions may have influenced the observed outcomes.

In uncomplicated cases with smaller plaques, a less invasive anterior approach through the clear cornea was preferred using two 23-gauge incisions for direct anterior chamber access. Viscoelastic gel-maintained chamber depth and protected the corneal endothelium. Various instruments were used for anterior capsulorhexis, and lens material was removed using an automated irrigation-aspiration system. Meticulous dissection, cauterization, and extraction of retrolental fibrovascular membranes were performed. The anterior vitrectomy cleared the anterior chamber and persistent hyaloid vessels, ensuring that no vitreous strands remained attached to the incision site. Both approaches likely include pupillary membrane dissection and synechiolysis.

IOL implantation was based on the surgeon’s clinical judgment, considering the patient’s age (> 15 months), HCD ≥ 10 mm, and the absence of complex PFV and glaucoma. Polyglactin 910 sutures (10-0) were used to secure the corneal incision, eliminating the need for post-surgical removal. Sclerotomies and limbal incisions were checked for leaks and sutured, if necessary.

Follow-up and postoperative care:

Postoperative care consisted of prednisolone acetate drops tapered over 4 weeks and moxifloxacin drops for 7 days to prevent infection and inflammation. The patient was monitored for complications. The evaluations included refraction, anterior segment examination, IOP measurement, and fundus evaluation under sedation. B-scan ultrasonography frequently assesses the posterior pole, particularly in cases of medial opacity.

Statistical Analysis

Operative reports underwent comprehensive examination to categorize cases based on anatomical complexity and to document the rationale for selecting the surgical approach. This analysis encompassed descriptions of retrolental plaque extent, anterior chamber space, and segmental involvement. Data from the electronic medical records were coded using Microsoft Excel spreadsheets (Microsoft Corporation, Redmond, WA, USA). Strict inclusion and exclusion criteria minimized the selection bias, ensuring a representative sample of patients with PFV and cataracts. Two trained investigators, both ophthalmologists with subspecialty expertise, independently reviewed the medical and operative records utilizing a standardized protocol to ensure accurate classification and data collection. Discrepancies were resolved through discussion with a senior ophthalmologist.

Statistical analyses were performed using Stata version 14 software (StataCorp LLC, College Station, TX, USA). Categorical variables were summarized as frequencies and percentages, while continuous variables were expressed as medians with interquartile ranges (IQR) for non-normal distributions and means with 95% confidence intervals (95% CI) for normal distributions. Fisher’s exact test was used to assess associations between binary variables, the t-test for normally distributed continuous variables, and the Mann–Whitney U test for non-parametric distributions. The significance level was set at P < 0.05. Logistic regression models, both unadjusted and adjusted, reported as odds ratios (ORs) with 95% CIs, were used to explore predictor–outcome relationships. The Hosmer–Lemeshow test evaluated the model fit. Stratified analyses examined effect modification and robustness to comprehensively address confounding factors.

Ethical Aspects

This study adhered to the ethical standards of the Declaration of Helsinki and was approved by the Institutional Review Board (IRB) of King Khaled Eye Specialist Hospital (approval number RP 24102-R). Owing to its retrospective design, the IRB waived the need for informed consent. Patient confidentiality and privacy were rigorously protected by anonymizing the data.

Results

This study investigated 76 eyes of 74 patients with PFV over a median 5-year follow-up period (IQR: 4–9). Postoperative RD occurred in 14 eyes (18%) at a median of 1 month post-surgery, ranging from immediately to 7 years after surgery. One patient with bilateral PFV developed postoperative bilateral RD. The median age at cataract surgery did not differ significantly between the RD and non-RD groups (2 and 4 months, respectively; P = 0.432).

Table 1 displays the demographic and clinical characteristics according to the RD status. The two groups showed similar sex distributions, with no significant differences between groups. Combined PFV (74%) was more common than anterior PFV (26%), with no notable intergroup variation. The prevalence of glaucoma and aphakic eyes at the final follow-up was comparable between the groups, with 25% of individuals and 65% of eyes affected in each group.

Table 1.

Demographic, clinical, and surgical characteristics associated with retinal detachment outcomes in patients with persistent fetal vasculature: a comparative analysis

	Frequency (%)				Total	P-value Fisher test
	Retinal detachment (present)		Retinal detachment (absent)
Gender
Male	7 (9)		38 (50)		45 (59)	0.550
Female	7 (9)		24 (32)		31 (41)
		14 (18)		62 (82)	76 (100)
Type of PFV
Anterior	2 (2)		18 (24)		20 (26)	0.331
Combined	12 (16)		44 (58)		56 (74)
		14 (18)		62 (82)	76 (100)
Surgical approach
Corneal	10 (13)		60 (79)		70 (92)	0.009
Pars plicata	4 (5)		2 (3)		6 (8)
		14 (18)		62 (82)	76 (100)
Glaucoma
Yes	3 (4)		16 (21)		19 (25)	0.718
No	11 (14)		46 (61)		57 (75)
		14 (18)		62 (82)	76 (100)
Lens status
Aphakia	9 (12)		40 (53)		49 (65)	0.999
Pseudophakia	5 (6)		22 (29)		27 (35)
		14 (18)		62 (82)	76 (100)

	Median	IQR	Median	IQR	P-value
					Mann–Whitney test
Age (years)	5	(5;7)	7	(5;9)	0.114
Age of surgery (months)	2	(2;7)	4	(2;8)	0.432
Horizontal corneal diameter (mm)	9	(9;10)	10	(9;10)	0.324
Time from surgery to RD (months)	1	(0;19)
Final BCVA (logMAR)	3	(2.7;3)	2	(1;2)	<0.001
Refraction (spherical equivalent)	13	(10;15)	9	(1;15)	0.267
Axial length (mm)	17	(15;19)	20	(18;21)	0.002
Follow-up (years)	5	(4;5)	6	(4;9)	0.220

PFV persistent fetal vasculature, BCVA best-corrected visual acuity, RD retinal detachment

Surgical approach was significantly correlated with the occurrence of postoperative RD (P = 0.009). Among the eyes studied, 70 (92%) used a corneal approach, while six (8%) used a pars plicata approach. The overrepresentation of the pars plicata approach in the RD group likely reflects the selection of more severe cases of PFV for this method, rather than an inherently higher risk. This distribution suggests a preference for a corneal approach in patients with PFV at our institution. Logistic regression analysis (Table 2) indicated that surgical approach significantly predicted the presence of postoperative RD. Univariate analysis showed that RD odds were approximately 90% lower with the corneal approach than with the pars plicata approach. Multivariate analysis revealed that RD odds were approximately 92% lower using the corneal approach. Both analyses yielded significant P-values (0.008 and 0.011, respectively).

Table 2.

Univariate and multivariable logistic regression analysis of risk factors for RD in patients with PFV following cataract surgery

	Postoperative retinal detachment
	Univariate			Multivariable
	Odds	P-value	95% [CI]	Odds	P-value	95% [CI]
Gender	0.6	0.440	[0.2, 2.0]	0.4	0.211	[0.1, 1.7]
Type of PFV	0.4	0.271	[0.1, 2.0]	0.8	0.803	[0.1, 4.5]
Surgical approach	0.1	0.008	[0.01, 0.5]	0.08	0.011	[0.01, 0.6]
Glaucoma	0.6	0.503	[0.1, 2.9]	0.4	0.381	[0.07, 2.8]
Intraocular lens	0.9	0.987	[0.3, 3.3]	0.3	0.167	[0.07, 1.5]
Age of surgery (months)	1.0	0.365	[0.9, 1.1]	1.04	0.442	[0.9, 1.2]
Axial length (mm)	0.7	0.003	[0.2, 0.9]	0.6	0.060	[0.5, 2.4]
Horizontal corneal diameter (mm)	1.9	0.225	[0.7, 5.2]	1.9	0.213	[0.7, 5.4]
Best-corrected visual acuity (logMAR)	0.03	0.001	[0.003, 0.2]	0.02	0.005	[0.01,0.3]
Spherical equivalent (diopters)	0.9	0.249	[0.8, 1.1]	1.02	0.833	[0.86, 1.2]

CI confidence interval, PFV persistent fetal vasculature, Surgical approach corneal or pars plicata, RD retinal detachment

AL measurements indicated a notable difference between the groups (P = 0.002), with RD-developing eyes having a median AL of 17 mm versus 20 mm in non-RD eyes. Figure 1 shows the connection between AL, surgical method, and RD status in patients with PFV. Those receiving the corneal approach had longer and more variable ALs than those undergoing pars plicata surgery, especially in non-RD eyes. Univariate analysis (Table 2) highlighted a significant association between AL and RD risk (P = 0.003), showing a 30% decrease in RD probability with each 1 mm AL increment. However, in the multivariable model, this link was not statistically significant (P = 0.060), with the OR changing from 0.7 to 0.6, indicating up to a 40% lower RD risk per 1 mm increase in AL when accounting for other factors.

Fig. 1.

Axial length (AL) distribution in relation to surgical approach and retinal detachment (RD) status in patients with persistent fetal vasculature (PFV). The boxplot shows the AL distribution across different surgical methods and RD status in patients with PFV. Data are presented as medians, interquartile ranges, and measurement ranges in millimeters. Red boxes denote cases with RD and blue boxes indicate cases without RD. Categories based on the surgical approach were on the horizontal axis, and the AL in millimeters was on the vertical axis

The final BCVA was significantly poorer in the RD group (P < 0.001), making it a strong predictor (multivariable OR, 0.02; 95% CI 0.01–0.3, P = 0.005). In the RD group, 10 eyes (71%) lost all light perception, and four needed prostheses due to phthisis bulbi. The other 29% had a median final BCVA of 2.6 logMAR, indicating that severe visual impairment was associated with postoperative RD in our series.

Age-related visual acuity in patients with PFV showed distinct patterns between the RD and non-RD groups (Fig. 2). Patients with RD consistently had lower visual acuity, with a steeper decline (slope = 0.145), indicating that delayed surgery reduced visual acuity (R-squared = 0.496, P = 0.06). In contrast, the non-RD group experienced a milder decline with surgery delays (slope = 0.051, R-squared = 0.045, P = 0.238) and greater variability, especially for surgeries before 10 months. Some RD patients consistently had poor visual acuity (logMAR 3.0), regardless of the timing of surgery. The difference in slope between the groups was not statistically significant (P = 0.209).

Fig. 2.

Relationship between age at surgery and final visual acuity in patients with and without retinal detachment (RD): a comparative analysis. A scatter plot depicts the correlation between age at surgery and the final visual acuity in patients with and without RD. The x-axis represents age at surgery in months, while the y-axis shows the final visual acuity on the logarithm of the minimum angle of resolution (logMAR) scale, with higher values indicating worse acuity. Red triangles indicate patients with RD and blue circles represent those without RD. The trend lines (red dotted for RD-present and blue dotted for RD-absent) imply a possible association between older age at surgery and poorer visual outcomes, particularly in the RD group

Patients who underwent surgery before three months old exhibited superior visual outcomes irrespective of the presence of RD. For the RD cohort, early surgery yielded a mean final BCVA of 2.9 logMAR (95% CI 2.9–3.1), versus 3.0 logMAR (95% CI 3.0–3.0) for surgeries at 12–24 months. In the non-RD cohort, outcomes were 1.7 logMAR (95% CI 1.3–2.1) and 1.8 logMAR (95% CI 1.1–2.5) respectively. Visible ciliary processes were noted in 25 patients (33%) during surgery, with all 14 RD patients and 11 non-RD patients displaying them, indicating a significant contrast (P < 0.001).

Discussion

This retrospective cohort study of 76 eyes of 74 patients with PFV showed that the surgical approach significantly influenced the postoperative RD risk. The postoperative RD rate in cases of PFV following cataract surgery was higher (18%) than the previously reported rates, which ranged from 5.3 to 9% in studies by Tartarella et al. and Kuhli-Hattenbach et al., respectively [8, 13]. However, Liu et al. reported RD rates of up to 29% in eyes undergoing small-gauge pars plicata vitrectomy for PFV [14].

While this study indicates a lower incidence of postoperative RD with the corneal approach, this finding likely reflects the inherent anatomical complexity and severity of cases of PFV necessitating a pars plicata approach. Factors such as visible ciliary processes, shorter AL, and anterior segment distortion significantly contribute to surgical outcomes. A comprehensive analysis must consider these variables, as the surgical approach often reflects case-specific anatomical challenges rather than intrinsic procedural superiority.

These comparisons highlight the variability in RD rates across different studies, which is influenced by surgical techniques, patient selection, and PFV characteristics [15]. The higher RD rate in our study may stem from the surgical approach, sampling model, patient demographics, or PFV severity. With a limited number of cases and the likelihood that the pars plicata approach was used for more severe cases of PFV, the increased RD incidence may reflect the underlying pathology rather than the surgical technique.

At our institution, 92% of cases used the corneal approach, significantly reducing RD risk compared to the pars plicata approach (OR, 0.08; 95% CI 0.01–0.6, P = 0.011), contributing to the debate on optimal PFV surgery methods. The exclusion of posterior cases of PFV, a subtype linked to poorer surgical outcomes due to its complexity requiring early intervention and often resulting in inoperability, suggests that our study likely focuses on more manageable anterior and combined cases of PFV [16, 17].

Lensectomy and vitrectomy using corneal or pars plicata/plana methods yield similar visual and anatomical results [15, 18]. Bata et al. and Ozdek et al. observed better visual acuity and reduced complications such as glaucoma and RD using the corneal technique compared with pars plana/plicata incisions in PFV surgery [6, 19, 20].

Despite conflicting evidence, some studies have found no significant difference in outcomes between limbal and pars plicata/plana approaches for PFV management [21]. However, Koch et al. reported lower complication rates and fewer additional surgeries with pars plicata/pars plana than with the corneal approach, potentially attributable to variations in patient populations, surgical techniques, or postoperative protocols [22]. Small gauge pars plicata vitrectomy has shown favorable anatomic outcomes, and acceptable complication rates in posterior or combined PFV with macular involvement, with suboptimal functional outcomes [14, 17].

In many cases of PFV, especially those with significant anterior segment involvement, the pars plana is absent and the retina is directly adjacent to the dragged ciliary processes. The decision between the limbal and pars plicata approaches in this study was determined by the anatomical complexity of each case, rather than randomization. This anatomical detail was carefully considered in surgical planning, with limbal lensectomy being preferred when cataracts obscured the pars plana. Our study aimed to assess the outcomes of different surgical approaches given these anatomical challenges and not to imply that surgeons were unaware of the typical PFV anatomy.

By analyzing the surgical outcomes of the limbal and pars plicata approaches, our findings highlight the need to customize surgical methods based on each patient’s anatomical features. The limbal approach showed a lower incidence of RD than the pars plicata approach, consistent with previous studies [6, 20]. The pars plicata approach was mainly used in cases of more severe PFV, possibly affecting the observed differences.

Randomized or stratified prospective studies are needed to assess the independent effects of surgical techniques on PFV management outcomes. Early intervention before three months of age significantly improved visual acuity, consistent with prior research (3)]. Combining corneal and pars plicata access or using endoscopic techniques could potentially improve outcomes and minimize complications [18, 23]. These discrepancies underscore the intricacy of PFV treatment and the need for additional investigations.

Our preference for the corneal approach stems from its potential for superior visual outcomes and reduced complication rates. This study included both anterior and combined anterior–posterior cases of PFV but did not clarify the differential risk between these forms, especially concerning intraoperative findings. Given that visible ciliary processes and anterior segment distortion may increase the risk of complications, future research should perform detailed risk stratification based on these observations to better identify eyes at a higher risk of postoperative RD and other complications.

Univariate analysis showed a significant link between shorter AL and increased postoperative RD risk in cases of PFV (median, 17 vs. 20 mm; P = 0.002), highlighting the importance of preoperative biometry for risk assessment. Although less significant in the multivariate model (OR: 0.6, 95% CI 0.5–2.4, P = 0.060), this observation remains clinically relevant, possibly indicating more severe PFV or microphthalmia. Karr et al. have shown that eyes with PFV often have reduced AL, especially in microphthalmia cases where AL ranges from 9 to 15 mm. This feature is linked to poorer visual outcomes and increased surgical complexity due to a limited surgical field and higher risk of complications like RD [4]. Our study supports this by quantifying the relationship between AL and postoperative RD, identifying shorter AL as a significant RD risk factor. These results highlight the importance of AL measurement in preoperative risk assessment, aiding in surgical planning, technique selection, and family counseling on visual outcomes and potential complications. Incorporating AL into preoperative evaluations allows clinicians to better address the anatomical challenges in the case of PFV.

While the multivariate analysis included high-risk cases, accounting for variables such as surgical approach and anatomical complexity, these cases may have affected the overall outcomes. Future studies with larger cohorts should further stratify these severe cases to clarify their impact on the surgical success rates.

Visual acuity outcomes were significantly worse in the RD group (P < 0.001), with patients often achieving LogMAR 2.6 vision or worse, consistent with previous studies [15]. Postoperative RD in cases of PFV leads to severe visual and anatomical deterioration, highlighting the need for preventive measures and surgical techniques that minimize RD risk given the difficulties in restoring normal ocular structures. Delayed surgery, especially in RD cases, results in poor visual acuity, emphasizing the importance of early intervention for optimal PFV outcomes [3, 20].

The age distribution in our cohort ranged from 1 to 18 months, with a median of 4 months, reflecting surgical timing guided by the clinical presentation and severity of PFV. In comparison to the study by Karacorlu et al., which reported a median surgical age of approximately 6 months, our findings indicate a trend toward earlier surgical intervention [18]. This discrepancy may be attributed to institutional practices, referral patterns, and case selection, with earlier surgeries in our cohort likely influenced by the identification of severe cases of PFV necessitating prompt management. The aforementioned study further elucidates the impact of anatomical challenges and referral timing on surgical decisions, factors that also informed the surgical approach in our study. These comparisons underscore the variability in surgical timing across cohorts and the necessity for individualized approaches based on patient anatomy, disease severity, and institutional protocols to optimize outcomes.

Surgery before 3 months yields better visual results, particularly in non-RD cases. However, postoperative RD remains a significant predictor of poor final visual acuity, regardless of surgical timing. The lack of an overall statistical significance underscores the complexity of PFV visual outcomes and the need for individualized surgical timing decisions.

Our findings indicated that visible ciliary processes significantly predicted postoperative RD and poor visual outcome (P < 0.001). Elongated ciliary processes increase surgical complexity, which is consistent with previous studies on the anatomical relationship between the ora serrata and ciliary body. These processes result in a pronounced anterior retinal elongation, extensive fibrovascular membranes, and peripheral retinal anomalies [9, 23]. This study highlights the need for thorough preoperative assessments and meticulous surgical techniques to mitigate risks and enhance outcomes in cases of PFV. Given the poor prognosis associated with visible ciliary processes, healthcare providers should inform guardians of the increased visual and structural risks and potential long-term adverse effects.

This study found no significant association between glaucoma or lens status and risk of postoperative RD in patients with PFV. However, their presence and management likely complicate PFV treatment, potentially affecting the long-term visual outcomes.

Limitations

This study’s retrospective design introduces limitations, including potential selection bias, reduced control over confounding variables such as surgical variability and patient selection, and limited generalizability despite efforts to mitigate these through stringent inclusion criteria.

However, selection bias may have affected our findings, as the pars plicata approach is often selected for cases with difficult anterior segment anatomy or extensive PFV involvement. This limitation warrants consideration when interpreting the results.

Complex cases featuring smaller corneal diameters, shorter AL, and significant anterior segment distortions were more susceptible to RD, which is consistent with prior research. Although our multivariate analysis considered factors such as AL, age at surgery, and surgical technique, it did not fully control for other significant variables such as anterior segment distortion and disease severity.

The small sample size, especially in the RD group (n = 14), likely diminished the statistical power and hindered causal inferences, despite the low incidence of PFV. Nonetheless, it offers significant insights for the management of this rare condition. This study focused on the surgical management of PFV in pediatric patients.

Although the corneal approach is associated with a reduced risk of RD, this outcome is likely attributable to differences in anatomical challenges and case severity rather than an inherent advantage of the technique. Visible ciliary processes are significant risk factors for postoperative RD, highlighting the importance of meticulous preoperative evaluation. Prompt surgical action enhances the visual results, especially in patients without RD, although the complex anatomy of the PFV necessitates customized treatment approaches.

The lack of randomization in the surgical approach assignments was a major limitation of this study. It is acknowledged that the randomization of patients to different surgical approaches, such as the corneal versus pars plicata approach, is unlikely to be feasible in practice due to the requirement for equipoised surgeons possessing equal expertise in both techniques and comparable patient anatomy. Alternative designs, such as matched case–control studies, may be more practical.

The potential preferential use of the pars plicata technique in severe cases may have contributed to the higher incidence of RD observed with this method, necessitating cautious interpretation when comparing corneal and pars plicata approaches.

A limitation of our study was the lack of a detailed anatomical assessment during subsequent RD repair. Anatomical risk factors, such as anterior retinal insertion or abnormal vitreoretinal attachments, may predispose certain eyes to RD after cataract surgery in patients with PFV. Identifying these factors during repair would offer vital insights into the mechanisms underlying retinal complications, and aid in refining preventive and corrective surgical strategies.

To enhance our understanding of surgical outcomes in PFV, future research should prioritize prospective, multicenter studies with balanced case distributions. These studies should randomize patients to different surgical techniques or stratify them based on preoperative anatomical severity, such as AL, anterior segment distortion, and intraoperative findings like ciliary process visibility. Addressing these variables will enable more reliable comparisons of surgical outcomes across techniques, reducing the influence of confounding factors like case complexity. Such efforts are crucial for establishing evidence-based guidelines to optimize surgical decision-making and improve long-term visual outcomes in PFV management. Standardized surgical protocols are essential to understand the impact of anatomical factors on long-term outcomes and to improve the generalizability of the results.

Conclusion

This investigation demonstrates that in patients with PFV, the observed lower RD rates associated with the corneal approach compared to the pars plicata approach are likely attributable to the complexity and severity of the cases rather than indicative of the superiority of one surgical method over the other.

While shorter AL and visible ciliary processes correlate with less favorable outcomes, early intervention, particularly before 3 months of age, improves visual prognosis. The surgical approach was associated with variations in postoperative RD rates; however, these findings warrant cautious interpretation given the substantial disparity in case complexity and sample size between the two techniques. Although limited by its retrospective design, these findings provide valuable guidance for surgical approach selection in PFV management, warranting further validation through randomized, multicenter trials.

Author Contributions

Fatima AlGhazal and Gorka Sesma contributed to conceptualization, data collection, analysis, administration, supervision, and manuscript preparation. Valmore A. Semidey, Marcos J. Rubio-Caso, and Sulaiman M. AlSulaiman contributed to methodology, investigation, software, formal analysis, writing (original draft, review, and editing), and visualization.

Funding

No funding or sponsorship was received for this study or the publication of this article. The Rapid Service Fee was funded by the authors.

Data Availability

The deidentified data that support the findings of this study will be made available upon reasonable academic request to the corresponding author, subject to institutional review board approval and data sharing agreements to ensure patient privacy protection.

Declarations

Conflict of Interest

Fatima AlGhazal, Valmore A. Semidey, Marcos J. Rubio-Caso, Sulaiman M. AlSulaiman, and Gorka Sesma declare no competing interests.

Ethical Approval

This study adhered to the ethical standards of the Declaration of Helsinki and was approved by the Institutional Review Board (IRB) of King Khaled Eye Specialist Hospital (approval number RP 24102-R). Due to its retrospective design, the IRB waived the need for informed consent. Patient confidentiality and privacy were strictly protected by anonymizing all data.