SciELO - Scientific Electronic Library Online

 
vol.99 número1Hipertensión intraabdominal y síndrome compartimental abdominal en el paciente grave índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Servicios Personalizados

Revista

Articulo

Indicadores

  • No hay articulos citadosCitado por SciELO

Links relacionados

  • No hay articulos similaresSimilares en SciELO

Compartir


Revista Información Científica

versión On-line ISSN 1028-9933

Resumen

CREME-LAMBERT, Limnet; DIAZ-ESTEVEZ, Harasay  y  LAMAS-AVILA, Mireya. Sturge-Weber syndrome. Literature review about a case. Rev. inf. cient. [online]. 2020, vol.99, n.1, pp.89-101.  Epub 30-Ene-2020. ISSN 1028-9933.

Introduction:

Sturge-Weber syndrome (SSW) is recognized among hamartoblastosis, whose diagnosis is unusual and complex treatment.

Objective:

To socialize the experience in the care of a pediatric patient with Sturge-Weber syndrome at the "General Pedro Agustín Pérez" Pediatric Teaching Hospital in Guantanamo.

Method:

A preschool patient was presented, with no family history of interest. One month after birth, he underwent congenital glaucoma surgery. During the first year of life he presented retardation of psychomotor development and involuntary movements considered tonic-clonic seizures. Literature was reviewed to transmit to the medical community, in particular, to medical students and general practitioners, information to achieve a diagnosis and adequate follow-up of this condition.

Results:

According to the clinical manifestations, physical examination by means of the clinical method and the results of the complementary examinations, the diagnosis of neurocutaneous syndrome was proposed, in particular a SSW. Treatment with diazepam was applied and when the crisis persisted, phenytoin. The first classification of neurocutaneous syndromes was carried out by Jan Van der Hoeve, who coined the term phacomatosis. It is a congenital neurological disorder, not inherited although there have been family cases described, uncommon but frequent compared to other neurocutaneous syndromes. An incidence of 5 x 100,000 live births is estimated. It affects all ethnic groups and both sexes.

Conclusions:

There is not always a relationship between the severity of cutaneous, neurological and ocular manifestations of SSW with brain disorders. The physical examination is important to establish the timely diagnosis and avoid future sequelae and complications.

Palabras clave : Sturge-Weber syndrome; angioma; hamartoblastosis.

        · resumen en Español | Portugués     · texto en Español     · Español ( pdf )