A report of two children with Gorham-Stout disease.

Autor: Tena-Sanabria ME; Unidad Médica de Alta Especialidad, Servicio de Ortopedia Pediátrica, Hospital de Pediatría 'Dr. Silvestre Frenk Freund', Centro Médico Nacional 'Siglo XXI', Instituto Mexicano del Seguro Social, Mexico City, Mexico., Jesús-Mejenes LY; Unidad Médica de Alta Especialidad, Servicio de Ortopedia Pediátrica, Hospital de Pediatría 'Dr. Silvestre Frenk Freund', Centro Médico Nacional 'Siglo XXI', Instituto Mexicano del Seguro Social, Mexico City, Mexico., Fuentes-Herrera G; Unidad Médica de Alta Especialidad, Servicio de Ortopedia Pediátrica, Hospital de Pediatría 'Dr. Silvestre Frenk Freund', Centro Médico Nacional 'Siglo XXI', Instituto Mexicano del Seguro Social, Mexico City, Mexico., Álvarez-Martínez FA; Unidad Médica de Alta Especialidad, Servicio de Ortopedia Pediátrica, Hospital de Pediatría 'Dr. Silvestre Frenk Freund', Centro Médico Nacional 'Siglo XXI', Instituto Mexicano del Seguro Social, Mexico City, Mexico., Victorio-García NP; Unidad de Investigación en Epidemiología Clínica, Hospital de Pediatría 'Dr. Silvestre Frenk Freund', Centro Médico Nacional 'Siglo XXI', Instituto Mexicano del Seguro Social, Avenida Cuauhtémoc 330. Colonia Doctores Delegación Cuauhtémoc C.P, 06720, Mexico City, Mexico., Núñez-Enríquez JC; Unidad de Investigación en Epidemiología Clínica, Hospital de Pediatría 'Dr. Silvestre Frenk Freund', Centro Médico Nacional 'Siglo XXI', Instituto Mexicano del Seguro Social, Avenida Cuauhtémoc 330. Colonia Doctores Delegación Cuauhtémoc C.P, 06720, Mexico City, Mexico. jcarlos_nu@hotmail.com.
Jazyk: angličtina
Zdroj: BMC pediatrics [BMC Pediatr] 2019 Jun 24; Vol. 19 (1), pp. 206. Date of Electronic Publication: 2019 Jun 24.
DOI: 10.1186/s12887-019-1561-0
Abstrakt: Background: Gorham-Stout disease is a rare condition characterized by unifocal and massive type IV osteolysis (variant of idiopathic nonhereditary osteolytic disease) with a slow progression, which is self-limiting for some years. It is characterized by recurrent vascular tumors with disruption of the anatomical architecture and intraosseous proliferation of vascular channels that leads to the destruction and resorption of the bone matrix. The aim of this study is to present the clinical features of this disease, as well as the importance of prompt diagnosis and treatment, with a review of the reported cases.
Case Reports: We describe two cases of Gorham-Stout disease between 2013 and 2017 with surgical interventions, follow-up and results. Case one involves an 11-year-old male with involvement of the left iliac bone, with adequate evolution after a surgical procedure with a lyophilized cadaveric tricortical bone allograft. Case two involves a 6-year-old male with cervical spine C1-C3 repercussion; in the protocol for surgical treatment, he presented with signs of spinal cord compression and died.
Conclusion: Diagnosis of Gorham-Stout disease is made by exclusion, and its clinical presentation varies widely, from spontaneous remission to a fatal outcome.
Databáze: MEDLINE
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