Ocular Complications in PHACE Syndrome: A True Association or a Coincidence?
J Pediatr
; 204: 214-218.e2, 2019 01.
Article
en 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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Coartación Aórtica
/
Anomalías del Ojo
/
Síndromes Neurocutáneos
Tipo de estudio:
Etiology_studies
/
Observational_studies
/
Risk_factors_studies
Límite:
Child, preschool
/
Female
/
Humans
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Infant
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Male
/
Newborn
País/Región como asunto:
America do norte
Idioma:
En
Revista:
J Pediatr
Año:
2019
Tipo del documento:
Article