Manifestation and grading of ocular involvement in patients with Tessier number 10 clefts

Abstract

PurposeThe objective of this study is to evaluate and grade the extent and severity of ocular involvement in Tessier number 10 cleft.Patients and methodsA retrospective, noncomparative, interventional case series was conducted between January 2006 and December 2015. Clinical data were reviewed from 59 patients (85 eyes) with Tessier number 10 clefts. Detailed medical history and ophthalmic examination of patients with a confirmed diagnosis of Tessier number 10 cleft were recorded on an itemized data collection form. Ocular manifestations were categorized as upper eyelid defect, symblepharon with cutaneous pterygium (skin growing onto the globe), corneal complications, and lower eyelid ectropion; components were evaluated and graded on a scale from 0 to 3, according to their severity.ResultsMore than half of the cases (43 eyes, 53.8%) had severe upper eyelid defect, and severe symblepharon with cutaneous pterygium were observed in 38 eyes (47.5%). Nearly half of the cases (40 eyes, 50.0%) have severe corneal complications, and lower eyelid ectropion was found in 34 eyes (42.5%). The severity of symblepharon, corneal complications, and lower eyelid ectropion were significantly correlated with the upper eyelid defect; the correlation coefficient (r) ranged from 0.844 to 0.629 (P<0.0001).ConclusionThis study presents the ocular manifestation of Tessier number 10 clefts with large-series cases, and establishes an effective grading system to evaluate Tessier number 10 clefts, which is useful for the diagnosis, treatment, and prediction of outcomes in patients with a Tessier number 10 cleft.

Tessier cranio-facial clefts presenting to a tertiary eye care center in Northern India: Ophthalmic features and a review of management

“…The ophthalmic features include eyebrow madarosis, abnormal hairline, upper eyelid coloboma, superior symblepharon, ocular surface keratinization, and anterior staphyloma. [ 14 15 ] In our study, we found that the madarosis was lateral in cleft 9 and medial in cleft 12.…”

Isolated Unilateral Tessier Cleft 10 with Anterior Staphyloma

“…Shao et al [4] had published a classification and grading system of ocular involvement for Tessier cleft 10 patients, wherein the eyelid coloboma was graded from 0 to 3 (0, no upper eyelid defect; 1, less than 1/3rd eyelid defect; 2, greater than 1/3rd but less than 1/2 eyelid defect; 3, more than half eyelid defect); symblepharon and cutaneous pterygium were scored between 0 to 3 (0, no symblepharon; 1, symblepharon and cutaneous pterygium confined to the corneal limbus; 2, symblepharon and cutaneous pterygium extending up to the pupil margin; 3, symblepharon and cu-…”

“…The ocular abnormalities which are associated mainly include congenital ocular surface problems like symblepharon, cutaneous pterygium and corneal abnormalities like corneal neovascularization and keratinization [ 4 ]. In few cases lower eyelid ectropion has also been reported.…”

“…Shao et al [ 4 ] had published a classification and grading system of ocular involvement for Tessier cleft 10 patients, wherein the eyelid coloboma was graded from 0 to 3 (0, no upper eyelid defect; 1, less than 1/3rd eyelid defect; 2, greater than 1/3rd but less than 1/2 eyelid defect; 3, more than half eyelid defect); symblepharon and cutaneous pterygium were scored between 0 to 3 (0, no symblepharon; 1, symblepharon and cutaneous pterygium confined to the corneal limbus; 2, symblepharon and cutaneous pterygium extending up to the pupil margin; 3, symblepharon and cutaneous pterygium extending beyond the pupil into the central cornea); the severity of corneal involvement was graded from 0 to 3 (0, clear cornea; 1, corneal opacity and keratinization localized at the periphery less than two continuous hours; 2, corneal opacity and cutaneous pterygium less than 4 clock hours; 3, extensive opacity, cutaneous pterygium, keratinization, or neovascularization with more than four continuous hours); and lower eyelid ectropion was scored from 0 to 3 (0, no ectropion appeared; 1, less than one-third of the lower eyelid exhibited ectropion; 2, greater than one-third but less than two-thirds of the eyelid exhibited ectropion; 3, more than two-thirds of the lower eyelid exhibit ectropion). Based on that our patient had a grade 3 upper eyelid defect with grade 3 corneal involvement with a grade 3 severe lower eyelid ectropion.…”

“…Based on that our patient had a grade 3 upper eyelid defect with grade 3 corneal involvement with a grade 3 severe lower eyelid ectropion. Limb anomalies like syndactyly and cardiac defects like patent foramen oval are also known to be associated with cleft number 10, and thus it is recommended to conduct a thorough systemic evaluation including cardiac echo on encountering a similar patient [ 4 ].…”