case_images: PMC10601854_01
case_images: Findings at 3 weeks of age. a, b A female infant with IFH over the right periauricular area extends down the neck and cheek area (a) and lower lip (b, arrow). c A right small globe, pseudoptosis, and a narrow palpebral fissure. d A right microphthalmia and a clear microcornea. e Ocular B-scan showing hyperechoic lesions in the vitreous cavity and stalk extending from the ONH (white arrow). f MRI showing right microphthalmos with dysmorphic globe. g Right globe on MRI showing a dislocated spherophakic lens (arrowhead) and hypo-intense stalk extending from the ONH to the posterior lens capsule (arrow). ONH, optic nerve head.
case_images: cop-2023-0014-0001-533887_F01.jpg
case_images: PMC10601854_01_cop-2023-0014-0001-533887_F01.jpg
case_images: F1
case_images: She had periauricular hemangioma that extended slightly toward the cheeks on the right (Fig. 1a) and buccal hemangioma involving the lower lip and gingival area (Fig. 1b, arrow). On initial ophthalmological evaluation, she had pseudoptosis of the RE with microphthalmia, esotropia, and hypertropia (Fig. 1c). Both corneas were not cloudy with a diameter of 5 mm in the RE and 10 mm in the left eye (LE) (Fig. 1d), the pupils and lens were difficult to examine and the fundus was unclear. B-scan showed features of linear and punctate areas of hyperechogenicity with mild organization suggestive of vitreous hemorrhage (VH) and a hyperechoic stalk extending from the optic nerve head to the posterior capsule of a dislocated lens, suggestive of persistent fetal vasculature (PFV) (Fig. 1e, white arrow). The magnetic resonance imaging (MRI) also revealed a dysmorphic globe (Fig. 1f) with a hypo-intense stalk (Fig. 1g, arrow) extending from the optic nerve head to a dislocated spherophakic lens (Fig. 1g, arrowhead), confirming B-scan findings suggestive of PFV but no features of a staphyloma or optic nerve hypoplasia.
case_images: Findings on follow-up. a RE corneal opacity with neovascularization at 8 months of age. b At 1 year of age with an ocular prosthesis fitted in the RE, good development and symmetry of palpebral fissure. c At 1 year of age with an obvious reduction in periauricular IFH. d At 1 year with a reduction of IFH of the lower lip.
case_images: cop-2023-0014-0001-533887_F02.jpg
case_images: PMC10601854_01_cop-2023-0014-0001-533887_F02.jpg
case_images: F2
case_images: With monthly ophthalmological examinations, the RE was noticeably shrinking, phthisis bulbi was progressing, and at 8 months of age, the cornea became cloudy, and developed neovascularization (Fig. 2a). The size of the prosthesis was checked and adjusted as necessary (Fig. 2b). Interventions by pediatricians and plastic surgeons have resulted in regression of the IFH in both the periauricular and lower lip area (Fig. 2c, d).