Ocular Complications in PHACE Syndrome: A True Association or a Coincidence?
J Pediatr
; 204: 214-218.e2, 2019 01.
Article
em En
| MEDLINE
| ID: mdl-30270159
ABSTRACT
OBJECTIVE:
To characterize the risk for ocular complications in patients with PHACE syndrome. STUDYDESIGN:
This study included consecutive patients with PHACE syndrome who were seen at Lurie Children's Hospital of Chicago from January 2000 through May 2017. A complete ophthalmic examination was performed in all patients, with extra attention for findings typically associated with PHACE syndrome.RESULTS:
Thirty patients (67% female, median age of onset 0.08 months) were included 38 (93%) demonstrated a segmental infantile hemangioma distribution. Twenty-one (70%) cases had a periocular involvement, and 47% had an infantile hemangioma with a deep component. Among 21 patients with periocular distribution, 9 had ocular complications secondary to the periocular location (mainly ptosis, nasolacrimal duct obstruction, and refractive errors), and one had an ocular complication specifically associated with PHACE syndrome (Horner syndrome). None of the patients without periocular distribution had an ocular complication.CONCLUSIONS:
In patients with PHACE syndrome who have a periocular infantile hemangioma, a complete eye examination is recommended. Although specific ocular anomalies related to PHACE syndrome are rare, serious ocular complications secondary to the location of the hemangioma may be present. Eye examination in patients with PHACE syndrome without a periocular infantile hemangioma distribution is likely of low yield.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Coartação Aórtica
/
Anormalidades do Olho
/
Síndromes Neurocutâneas
Tipo de estudo:
Etiology_studies
/
Observational_studies
/
Risk_factors_studies
Limite:
Child, preschool
/
Female
/
Humans
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Infant
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Male
/
Newborn
País/Região como assunto:
America do norte
Idioma:
En
Revista:
J Pediatr
Ano de publicação:
2019
Tipo de documento:
Article