Persistent Tunica Vasculosa Lentis Research Articles

A rare presentation of pupillary block glaucoma and persistent tunica vasculosa lentis in Klippel–Trenaunay syndrome

Phacomatoses are a group of neurocutaneous disorders characterized by vascular and pigmentary birthmarks, often involving multiple organ systems. Klippel–Trenaunay syndrome (KTS), a rare phacomatosis, is characterized by the classical triad of cutaneous capillary hemangiomas, venous malformations, and soft tissue overgrowth. Glaucoma secondary to angle dysgenesis, raised episcleral venous pressure, or excess angle pigmentation is observed. We report a case of KTS that presented with cutaneous capillary hemangiomas and limb hypertrophy along with unilateral buphthalmos. This eye was noted to have persistent tunica vasculosa lentis and persistent pupillary membrane with pupillary block glaucoma that was treated with pupilloplasty. To the best of our knowledge, this mechanism of glaucoma in KTS has not been reported before. The purpose of this article is to document this rare presentation as well as highlight the importance of detecting the underlying mechanism for appropriate management.

Combined intravitreal ranibizumab and zone I sparing laser ablation in infants with posterior zone I retinopathy of prematurity.

Purpose:To evaluate the efficacy of combined intravitreal ranibizumab (IVR) and zone I sparing laser ablation in infants with posterior zone I Retinopathy of Prematurity (ROP).Methods:This was a retrospective, interventional case series including premature infants diagnosed with posterior zone I ROP (n = 24) on ROP screening. Charts and RetCam images of preterm infants with posterior zone I ROP treated with immediate IVR and zone I sparing laser ablation at 4 weeks between April 2016 and September 2019 were reviewed. Data were analyzed and tabulated using frequency and descriptive statistics to describe the demography, morphology, and treatment outcomes. Primary outcome measure was structural outcome at 6 months. It was further categorized as favorable and unfavorable.Results:Twenty-four infants (48 eyes) with a mean gestational age of 28.54 ± 1.98 weeks and birth weight of 1180.33 ± 280.65 grams were analyzed. Thirty-six (75%) eyes had persistent tunica vasculosa lentis and twenty-six (54.1%) eyes had iris neovascularization. All eyes had features of aggressive posterior retinopathy of prematurity (APROP) limited to posterior zone I. The mean duration between IVR and zone I sparing laser ablation was 29.62 ± 6.36 (range: 24-34) days. One infant (2 eyes) received a second IVR treatment for recurrence of plus disease and persistent new vessels close to the fovea. Laser augmentation was done in 13 (27.1%) eyes. A favorable structural outcome was seen in 45 (93.7%) eyes.Conclusion:Posterior zone I ROP presents as APROP. Combined IVR and zone I sparing laser ablation appears effective treatment option in these eyes.

Persistent Tunica Vasculosa Lentis as an Independent Risk Factor for Treatment in Retinopathy of Prematurity

This was a 1:1 nested case-matched, consecutive, single physician, retrospective series assessing the risk of treatment for ROP in patients with persistence tunica vasculosa lentis (TVL) on neonatal screening examination. The rate of meeting the criteria for treatment and having zone 1 disease were increased in the TVL group (30.85% vs 10.64%, p<0.001, odds ratio of 4.80) and (15.96% vs 2.12% p= 0.002, odds ratio of 7.50) respectively. Persistence of TVL is a poor prognostic sign in ROP with higher rates of treatment requirement and zone 1 disease when compared to age and birthweight matched controls.

Ocular Findings in Trisomy 13: Nasolacrimal Duct Stenosis Case Series

Trisomy 13, also known as Patau Syndrome, is a congenital malformation that leads to several ocular anomalies, of which cataracts are the most common, as well as iris and retinal colobomas, persistent hyperplastic primary vitreous, persistent tunica vasculosa lentis, and microphthalmos. While most do not survive beyond the neonatal period, those that do survive past one year of age have an 84% chance of making it to age 5. Therefore, treatments for ocular problems should be used to improve the quality of life if the child can withstand surgery. The literature on this topic has described histopathologic findings in postmortem eyes. This case series supports those observations and presents a new discovery of nasolacrimal duct obstructions or stenosis. Deciding on surgical intervention in cases with Trisomy 13 can be challenging, and a variety of factors must be taken into account before surgery is considered. Treatment of these cases can be difficult, necessitating deliberate consideration and determination on a case-by-case basis; however, this case series gives additional evidence to help guide these decisions. Traditionally, trisomy 13 was regarded as being lethal, multiple organ malformations and severe intellectual disability.

Extreme Prematurity and Fibroblastic Overgrowth of Persistent Vascular Sheath Behind Each Crystalline Lens : I. Preliminary report

A six-months-old infant, one of twins born about two months prematurely, was found to have a grayish-white, opaque membrane behind each crystalline lens (frontispiece A and B). The pupillary responses were normal and the eyes transilluminated clearly. A jerky, irregular, and somewhat searching nystagmus was present. The membrane did not cover the entire posterior surface of the lens. Below at the extreme periphery, a clear fundus reflex could be obtained when the pupils were fully dilated. In this region thin dentate processes extended from the membrane peripherally as if they were attached to the ciliary processes (frontispiece, C and D). The membrane apparently was what is commonly, but perhaps erroneously, called persistent tunica vasculosa lentis. One of the consultants, Dr. Paul A. Chandler, has a case almost indentical in appearance in an infant also born very prematurely. Both of these infants were under the care of Dr. Stewart Clifford who discovered the ocular pathology. Recently, three other cases have been seen in the clinic of the Massachusetts Eye and Ear Infirmary. The findings are quite similar to those mentioned, even to the dentate periphery. All of these infants were premature, all weighed three pounds or less at birth, and no evidence of hereditary factors has been found.

Posterior zone I retinopathy of prematurity: spectrum of disease and outcome after laser treatment

ObjectiveTo report the morphological spectrum and outcome after laser treatment in posterior zone I retinopathy of prematurity. DesignRetrospective, consecutive, interventional case series. MethodsReview of charts and RetCam images of preterm infants with posterior zone I retinopathy of prematurity (defined as area within a circle, the radius of which extends from the center of the optic disc to the center of the macula) treated with confluent laser at a tertiary care referral institute between January 2006 and December 2015. Data was analyzed and tabulated using frequency and descriptive statistics to describe the demography, morphology, and outcome after laser treatment. ResultsFifty-six eyes (28 infants) with a mean gestational age of 28.65 ± 2.18 weeks and birth weight of 1153.04 ± 251.51 g were analyzed. Forty-two (75%) eyes had persistent tunica vasculosa lentis and 25 (44.6%) had preretinal hemorrhages. All eyes had circumferential ROP and a poorly developed foveal vasculature. Four distinct morphological patterns were encountered: I − typical aggressive posterior retinopathy was seen in 36 (64.3%) eyes; II − atypical aggressive posterior retinopathy (14 eyes; 25%) had raised fibrovascular proliferation always on the nasal retina close to the optic disc; III − hybrid retinopathy of prematurity (4 eyes; 8.7%) had a mat-like fibrous proliferation on vascularized posterior retina; and IV − threshold retinopathy of prematurity seen in 2 (4.4%) eyes. An unfavourable outcome was seen in 44 (78.6%) eyes. ConclusionsRetinopathy of prematurity in posterior zone I presents with atypical morphology and poor outcomes despite confluent laser photocoagulation.

Bilateral ocular anomalies in a South African fur seal (Arctocephalus pusillus pusillus)

A female South African fur seal (Arctocephalus pusillus pusillus) began having obvious clinical ophthalmologic problems by 8 weeks of age. The initial clinical sign was diffuse corneal edema, which progressed to bullae formation and ulcers; the underlying cause of corneal edema and bullous keratopathy was not identified antemortem.An ophthalmological evaluation was performed when the fur seal was approximately 6 months of age, but due to the diffuse corneal edema, intraocular structures could not be easily evaluated. An underlying infectious etiology was suspected; therefore,appropriate diagnostics were pursued, but did not identify a cause. Initial improvement was noted, but the fur seal then became blind and eventually became very painful.Due to decreased quality of life and aggressive behavior, the fur seal was euthanized.Histopathological diagnoses were persistent tunica vasculosa lentis and persistent hyperplastic primary vitreous with bilateral hypermature resorbed cataracts and retinal detachments with rosette formation.

Dramatic Regression of Persistent Tunica Vasculosa Lentis Associated With Retinopathy of Prematurity Following Treatment With Intravitreal Bevacizumab

The authors describe a preterm infant who developed advanced retinopathy of prematurity bilaterally with a prominent tunica vasculosa lentis. Treatment with intravitreal bevacizumab resulted in regression of the tunica vasculosa lentis and posterior manifestations of the retinopathy of prematurity. RetCam imaging (Clarity Medical Systems, Pleasanton, CA) of the anterior segment was used to document the dramatic tunica vasculosa lentis resolution. [J Pediatr Ophthalmol Strabismus 2013;50:e27–e29.]

Neonatal Candidemia Exacerbated the Progression of Retinopathy of Prematurity in an Extremely-Low-Birth-Weight Infant with Persistent Tunica Vasculosa Lentis.

Eyes with threshold retinopathy of prematurity (ROP) often show dilation of the existing tunica vasculosa lentis (TVL), which is a prognostic factor in ROP screening and treatment, but can be frequently misdiagnosed as true iris neovascularization (NV). Besides, candidemia in preterm infants can aggravate the severity of ROP, possibly to the threshold stage requiring laser treatment. A case of prolonged candidemia without ophthalmic infection in an extremely-low-birth-weight (ELBW) (birth weight: 800 g), pre-term (gestation: 26 weeks) infant with persistent TVL and severe ROP are reported. The threshold ROP was successfully treated with argon laser therapy. Candidemia without eye infection in this case was associated with early use of a systemic antifungal drug. To our knowledge, no similar cases have been reported in the literature.

Atypical Persistence of Tunica Vasculosa Lentis

The authors report an unusual case of persistent tunica vasculosa lentis in a patient with minimal retinopathy of prematurity (ROP). A 3-month-old male infant who had been born at 31 weeks' gestation presented with a significant amount of persistent tunica vasculosa lentis and arteriolar tortuosity with minimal ROP and no plus disease. After weekly observation and no surgical intervention, the arteriolar tortuosity lessened but the tunica vasculosa lentis persisted. Persistent tunica vasculosa lentis can be mistaken for iris vascular engorgement, suggesting plus disease and high-risk prethreshold ROP. Differentiation between tunica vasculosa lentis and iris vascular engorgement, as well as correct diagnosis of plus disease, is critical when considering laser treatment for high-risk prethreshold ROP.

Congenital pupillary-iris-lens membrane with goniodysgenesis

Background A unilateral congenital pupil-iris-lens membrane with goniodysgenesis syndrome, not benign tunica vasculosa lentis, was first described by Cibis et al. One of three cases developed angle closure. Robb described catastrophic vision loss from angle closure in one of his seven cases. Methods We did a retrospective review of previously unreported cases of pupil-iris-lens membrane with goniodysgenesis seen in our practices. Results We report the clinical spectrum of a further nine cases, three of which needed surgery for angle closure, two of which needed surgery for clearing the visual axis. Conclusion Congenital pupil-iris-lens membrane with goniodysgenesis is a unilateral membrane clearly differentiated from benign persistent tunica vasculosa lentis tissue. The membrane represents ectopic iris on the lens with abnormal iris stroma and chamber angle from aberrant induction, migration, or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. Surgery may be needed to open the visual axis even when glaucoma is not present and may prevent angle closure.

Persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous (PHPV) is a common congenital developmental anomaly of the eye that results following failure of the embryological, primary vitreous and hyaloid vasculature to regress. It typically presents unilaterally without associated systemic findings. Although the etiology is assumed to be identical in each of its three variants, PHPV is still subclassified into three presentations. The purely anterior presentation of PHPV is also known as persistent tunica vasculosa lentis and persistent posterior fetal fibrovascular sheath of the lens. It occurs in eyes with pathology of the anterior segment. This form typically involves cataract, glaucoma and a retrolenticular membrane. The purely posterior presentation of PHPV is termed falciform retinal septum and ablatio falcicormis congentia. It occurs in eyes with abnormalities confined to the posterior segment such as retinal folds, vitreal stalk, vitreal membranes, macular abnormalities and optic disc abnormalities. A combination of anterior and posterior presentations is the most commonly seen clinical presentation. Case report. We present a case in which an 11-year old male was referred to our office for reevaluation of a large angle esotropia, strabismic and deprivational amblyopia and previously diagnosed PHPV, OD. Conclusion. Without treatment, PHPV can produce recurrent intraocular hemorrhage, secondary glaucoma and eventually require enucleation. Early surgical intervention is necessary to prevent progressive pathologic changes and to obtain the best visual results. Finally, while PHPV is a documented source of leukocoria, clinicians should be aware of differential diagnoses which involve the white pupil (congenital cataract, retinoblastoma, Norrie's disease, retinopathy of prematurity, retinal detachment and Coat's disease).

Acquired cataracts after argon laser photocoagulation for retinopathy of prematurity

Purpose : Our purpose was to determine the incidence of cataract after argon laser photocoagulation of the retina in infants with retinopathy of prematurity (ROP). Methods : We reviewed medical records of 189 consecutive infants undergoing argon laser photocoagulation for acute ROP between 1993 and 1996. Birth weight, estimated gestational age at birth, chronologic and postconceptional ages at the time of treatment, ROP outcome, and the postoperative occurrence of cataract were recorded. Results : A total of 374 eyes in 189 infants were treated for threshold ROP. Mean birth weight was 916 gm (range 480 to 2500 gm), mean postconception age at birth was 26.4 weeks (range 23.5 to 34 weeks), and mean postconception age at surgery was 36.2 weeks (range 33 to 47 weeks). A favorable anatomic outcome occurred in 90% of eyes. However, severe macular traction, macular fold, or retinal detachment developed in 10% of eyes. A total of four eyes (1%) had cataracts. Laser-induced cataracts were diagnosed in only two eyes. A third eye had a posterior subcapsular cataract that may or may not have resulted from the laser treatment. In a fourth patient a unilateral punctate opacity judged to be visually insignificant was noted at surgery but it was not progressive. All patients who had cataracts had a persistent tunica vasculosa lentis; however, there was no statistically significant difference in the incidence of cataract formation in eyes with persistent tunica vasculosa lentis compared with eyes without it ( p = 0.057). Conclusion : Argon laser photocoagulation remains an effective alternative to transscleral cryotherapy in the treatment of threshold ROP. The incidence of cataract formation is approximately 1% and may be more likely to occur when persistent hyaloidal vessels are present on the lens.

Persistent tunica vasculosa lentis as a sign of congenital myotonic dystrophy.

We describe the ocular findings in eight patients with congenital myotonic dystrophy in the newborn period. While three infants had normal findings, five infants had evidence of persistent tunica vasculosa lentis (TVL) at gestational ages ranging from 34 to 40 weeks. Atrophy of vessels on the anterior lens capsule is normally complete by 34 weeks gestation and its persistence should suggest congenital infection or congenital myotonic dystrophy. Diagnosis of myotonic dystrophy has serious implications for the baby and the mother, in whom the condition may have not been recognized earlier.

Holoprosencephaly in neonates

A single nostril associated with alobar holoprosencephaly is a rare congenital lesion. This paper reports two female term neonates with holoprosencephaly. The first neonate asphyxiated at birth had a single nostril, hypotelorism, posterior cleft palate, inferonasal coloboma of the iris and disc and persistent tunica vasculosa lentis. The other neonate had cleft lip and palate and recurrent convulsions. Both neonates had gross motor and developmental delay. Cranial sonography and CT scan showed features consistent with alobar holoprosencephaly. Karyotyping for the first neonate and her family members was normal. Both of them were treated conservatively and supervised.

Laser Indirect Ophthalmoscope Photocoagulation in an Incubator for the Treatment of Retinopathy of Prematurity

Argon blue-green or diode laser indirect ophthalmoscope photocoagulation, performed through the transparent wall of an incubator, was used to treat threshold retinopathy of prematurity in two premature infants who were in poor systemic condition and could not be treated out of the incubator. Despite technical difficulties posed in one or both cases by their lateral position, small pupils, persistent tunica vasculosa lentis, and hazy cornea, there were no systemic complications and the retinopathy regressed in both cases.

Retinal neovascularization in Potter's syndrome secondary to renal agenesis*

Abnormalities of retinal vascularization were observed in a male stillborn at 33 weeks gestation with Potter's syndrome secondary to renal agenesis. Each eye had a persistent tunica vasculosa lentis and hyaloid artery as well as enlarged intraretinal vessels and a sharply demarcated avascular peripheral retina. Light microscopy revealed marked proliferation of capillaries and mesenchymal spindle cells of the retina. Electron microscopy of the retinal vascular endothelial cells revealed intact tight junctions with no fenestrations. Spindle cells had gap and intermediate junctions that appeared normal in number and morphology.

APPEARANCE OF RETROLENTAL FIBROPLASIA

The first description of RLF as a disorder of extreme prematurity was published as a preliminary report by Terry in 1942. He stated that the condition was either "persistence of the entire vascular structure of the fetal vitreous" or a "fibroblastic overgrowth of the persistent tunica vasculosa lentis." His studies led him to conclude that an "overgrowth of embryonic connective tissue in the interstices of the persistent tunica vasculosa lentis behind the lens" accounted for the pathology observed. He stated: "It is really a retrolental fibroplasia." The terminology was used in his subsequent reports and became generally accepted, although later studies by Owens and Owens (1949) showed that RLF was not related to an embryonic abnormality but was caused by an abnormal blood vessel change in the retina. On the basis of clinical observation, Terry (1942) concluded that the disease in its classical form was not present at birth but developed between two and six months after birth. He considered many possible factors: "Of all the probable causes listed, precocious exposure to light is considered the most tenable, and preventive measures should be taken." The light theory was discarded after several investigators found that occluding the eyes after birth had no effect on development of the disease (Hepner et al., 1949; Crosse, 1950; Locke and Reese, 1954). After discovering a disease that had appeared as an isolated rarity in 1942, Terry collected 117 cases of RLF during the next three years. The condition appeared to be on the increase. In 1945 Terry found that the incidence at the Boston Lying-In Hospital was approximately 12% in infants weighing 1,400 gm or less (based on fewer than 50 cases).

Ocular manifestations of the Meckel syndrome.

The Meckel syndrome is a genetically determined (autosomal recessive) disease with major developmental abnormalities incompatible with life. The ocular manifestations include cryptophthalmos, clinical anophthalmos, microphthalmos, sclerocornea, microcornea, abnormal iridocorneal angle, partial aniridia, cataract, persistent tunica vasculosa lentis, retinal dysplasia, posterior staphyloma, and hypoplasia of the optic nerve. The ocular abnormalities are very similar to those of the 13-15 trisomy syndrome, but the chromosome karyotype is normal in the Meckel syndrome.

OCULAR MALDEVELOPMENT IN EXTREMELY PREMATURE INFANTS

In 1941 it was recognized that a massive growth of embryonic connective tissue occurring in the meshwork of a persistent tunica vasculosa lentis is linked with prematurity.1To my knowledge there are at least 162 cases at the present time. Dr. Stewart Clifford2has found that 12 per cent of the infants weighing 3 pounds (1,360 Gm.) or less at birth develop this disease. This percentage, based on less than 50 cases of prematurity, can be considered only a trend. If the true percentage of infants developing this disease is about 10, approximately 6003cases are to be expected in this country each year, which, if some prophylaxis is not found, will add materially to the 175,000 blind.4 Typical well developed cases show seven characteristic findings: There is opaque vascularized tissue behind the crystalline lens in which the vessels tend to radiate from a central