Journal Information
Vol. 58. Issue 6.
Pages 599-603 (01 June 2003)
Share
Share
Download PDF
More article options
Vol. 58. Issue 6.
Pages 599-603 (01 June 2003)
Full text access
Heterogenicidad del síndrome de Gorham-Stout: asociación a malformaciones linfáticas y venosas
Heterogenicity of gorham-stout syndrome: association with lymphatic and venous malformations
Visits
16356
I. Somoza Argibay**
Corresponding author
isomoza@canalejo.org

Correspondencia: Servicio de Cirugía Pediátrica. Hospital Materno-Infantil Teresa Herrera.Complejo Hospitalario Juan Canalejo.As Xubias, 84. 15006 A Coruña. España.
, M. Díaz González, L. Martínez Martínez, Z. Ros Mar, J.C. López-Gutiérrez
Unidad de Cirugía Plástica Infantil. Servicio de Cirugía Pediátrica.Hospital Materno Infantil La Paz. Madrid. España.
This item has received
Article information
Introducci ón

El síndrome de Gorham-Stout constituye una rara enfermedad de etiología desconocida que se caracteriza por osteólisis rápidamente progresiva y proliferación microscópica de vasos anormales. Presentamos 2 casos clínicos asociados a malformaciones linfaticovenosas.

Casos clínicos

El primer caso es un varón de 5 años afecto de linfangiomatosis generalizada de evolución desfavorable, con importante afectación pleural y lesiones osteolíticas. El segundo caso se trata de una niña de 5 años diagnosticada de síndrome de Klippel-Trénaunay con importante afectación ósea en miembros inferiores y fractura patológica secundaria.

Conclusiones

El síndrome de Gorham-Stout puede presentarse ocasionalmente asociado a distintas malformaciones linfaticovenosas. La afectación linfática del hueso puede provocar osteólisis y resorción ósea.

Palabras clave:
Síndrome de Gorham-Stout
Malformaciones linfaticovenosas
Resorción ósea
Osteólisis
Interferón alfa 2b(IFN-a2b)
Introduction

Gorham-Stout syndrome is a rare disorder of unknown etiology characterized by osteolysis and microscopic proliferation of abnormal vessels. We report two cases of this syndrome associated with lymphatic and venous malformations.

Case reports

The first case is a 5-year-old boy with disseminated lymphangiomatosis of poor prognosis, with significant pleural involvement and osteolytic lesions. The second case is a 5-year-old girl with a diagnosis of Klippel-Trenaunay syndrome with significant skeletal involvement of the lower extremities and secondary pathological fracture.

Conclusions

Gorham-Stout syndrome may occasionally be associated with various lymphatic and venous malformations. Osteolysis and bone resorption may be induced by lymphatic bone involvement.

Key words:
Gorham-Stout syndrome
Lymphatic venous malformations
Bone resorption
Osteolysis
Alpha-2b interferon(IFN-a2b)
Full text is only aviable in PDF
Bibliografía
[1.]
L.W. Gorham, A.W. Wright, H.H. Shultz, F.C. Maxon.
Disappearing bones:A rare form of massive osteolysis. Report of two cases, one with autopsy findings.
Am J Med, 17 (1954), pp. 674-682
[2.]
L.W. Gorham, A.P. Stout.
Massive osteolysis (acute spontaneous absortion of bone, phantom bone, dissapearing bone):Its relation to hemangiomatosis.
J Bone Joint Surg (Am), 37 (1955), pp. 985-1004
[3.]
H. Hagberg, K. Lamberg, G. Aström.
a-2b interferon and oral clodronate for Gorham's disease.
Lancet, 350 (1997), pp. 1822-1823
[4.]
N.D. Choma, C.V. Biscotti, T.W. Bauer, A.C. Mehta, A.A. Licata.
Gorham's syndrome:A case report and review of the literature.
Am J Med, 83 (1987), pp. 1151-1156
[5.]
T.C. Shives, J.W. Beabout, K.K. Unni.
Massive osteolysis.
Clin Orthop, 294 (1993), pp. 267-276
[6.]
P. Calzavar a Pinton, A. Carlino, A.M. Manganoni, C. Donzelli, F. Facc hetti.
Epid ermal nevus síndrome with multiple vascular hamartomas and malformations.
G Ital Dermatol Veneoreol, 125 (1990), pp. 251-254
[7.]
Lymphatic Malformation. Child Health A to Z. Children's Hospital Boston web, 2001 [date access January 3th 2003]. Disponible en: web1.tch.harvard.edu/cfapps/A2Z.cfm
[8.]
P.R. Carring ton, M.J. Rowley, M. Fowler, R.P. Megison, P. Meyers.
Kasabach-Merri tt syndrome with bone involvement:The pseudomalignant sign of Gorham.
J Am Acad Dermatol, 40 (2001), pp. 726-728
[9.]
S.Y. Yoo, J.M. Goo, J.G. Im.
Mediastinal lymphangioma and chylotorax:Thoracic involvement of Gorham's disease.
Korean J Radiol, 3 (2002), pp. 130-132
[10.]
J.B.S. Jacks on.
Abonele ss arm.
Boston Med Surg J, 18 (1838), pp. 368-369
[11.]
J.S. Torg, H.H. Steel.
Essential osteolysis with nephropathy:A review of the literature and case report of an unusual syndrome.
J Bone Joint Surg (Am), 50 (1968), pp. 1629-1639
[12.]
T.S. Hsu, L.T. Cooper, T.P. Maus, E.A. Miller, J.H. Barlow, M.D. Da vis.
Cutane ous and gastrointestinal tract hemangiomas associated with disappearing bones:Gorham syndrome.
Int J Dermatol, 40 (2001), pp. 726-728
[13.]
A.A. Mendez, D. Keret, W. Robertson, G.D. MacEween.
Massive osteolysis of the femur (Gorham's disease):A case report and review of the literature.
J Pediatr Orthop, 9 (1989), pp. 604-608
[14.]
C. Szabo, W. Habre.
Gorham syndrome:Anaesthetic management.
Anaesthesia, 55 (2000), pp. 155-157
[15.]
S.F. Dunbar, A. Rosenberg, H. Mankin, D. Rosenthal, H.D. Suit, D. Phil.
Go rham's Massive osteolysis:The role of radiation therapy and a review of the literature.
Int J Radiation Oncology Biol Phys, 26 (1993), pp. 491-497
[16.]
J.S. Thomps on, D.J. Schurman.
Massive osteolysis:Case report and review of the literature.
Clin Orthop, 103 (1974), pp. 206-211
[17.]
C.J. Fretz, W.F. Jungi, J. Neuweiler, M. Haertel.
The malignant degeneration of Gorham Stout diseaseα.
Rofo-Fortschr Geb Rontgenstr Neuen Bildgeb Verfahr, (1991), pp. 579-581
[18.]
H.A. Kulenk ampff, G.M. Richter, W.E. Hasse, C.P. Adler.
Massive osteolysis in the Gorham Stout syndrome.
Int Orthop, 14 (1990), pp. 361-366
[19.]
T. Ohya, S. Shibata, Y. Takeda.
Massive osteolysis of the maxillo-facial bones:Report of two cases.
Oral Surg Oral Med Oral Pathol, 70 (1990), pp. 698-703
[20.]
G. Mö ller, M. Priemel, M. Amling, M. Werner, A.S. Kuhlmey, G. Delling.
The Gorham-Stout Syndrome (Gorham massive osteolysis):A report of six cases with histopathological findings.
J Bone Joint Surg (Br), 81 (1999), pp. 501-506
[21.]
M. Amling, G. Delling.
Cell biology of osteoclasts and mechanisms of bone resortion.
Pathologe, 17 (1996), pp. 358-367
[22.]
R.S. Evely, A. Bonomo, H.G. Schneider.
Structural requirements for the action of parathyroid hormone-related protein (PTHrP) on bone resorption by isolated osteoclasts.
J Bone Miner Res, 6 (1991), pp. 85-93
[23.]
R.D. Devlin, H.G. Bone, G.D. Roodman.
Interleukin-6:A potential mediator of the massive osteolysis in patients with Gorham-Stout disease.
J Clin Endocrinol Metab, 81 (1996), pp. 1893-1897
[24.]
H.D. Green, A.J. Mollica, A.S. Karuza.
Gorham's disease:A literature review and cases reports.
J Foot AnKle Surg, 34 (1995), pp. 435-441
[25.]
J.R. Mawk, S.K. Obukhov, W.D. Nichols, T.D. Wynne, J.M. Odell, S.M. Urman.
Successful conservative management of Gorham disease of the skull base and cervical spine.
Child's Nervous System, 13 (1997), pp. 622-625
[26.]
H. Tilg.
New insights into the mechanisms ofinterferon alfa:An inmunoregulatory and anti-inflamatory cytokine.
Gastroenterology, 112 (1997), pp. 1017-1021
[27.]
M. Vikkula, L.M. Boon, J.B. Mulliken.
Mollecular genetics of vascular malformations.
Matrix Biol, 20 (2001), pp. 327-335
[28.]
L.M. Boon, P. Brouillard, A. Irrthum, L. Karttunen, L.M. Warman, M. Vikkula.
A gene for inherited cutaneous venous anomalies ("glomangiomas") localizes to chromosome 1p21-22.
Am J Hum Genet, 65 (1999), pp. 125-133
[29.]
C. Laverdi ère, M. David, J. Dubois, P. Russo, L. Hershon, J.G. Lapierre.
Improvement of disseminated lymphangiomatosis with recombinant Interferon theraphy.
Pediatr Pulmonol, 29 (2000), pp. 321-324
[30.]
B. Tauro.
Multicentri c Gorham's disease.
J Bone Joint Surg (Br), 74 (1992), pp. 928-929
[31.]
F. Hardegg er, L.A. Simpson, G. Segmueller.
The syndrome ofidiopathic osteolysis:Classification, review and case report.
J Bone Joint Surg (Br), 67 (1985), pp. 89-93
[32.]
O. Burkhar d, J. Beyer, J. Schrezenmeir, A. Brunier, G. Delling.
A case ofidiopathic multicentric osteolysis.
Med Klin, 84 (1989), pp. 364-368
[33.]
I. Holroyd, M. Dillon, G.J. Roberts.
Gorham's disease:A case (including dental presentation) of vanishing bone disease.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 89 (2000), pp. 125-129
[34.]
Internat ional Society for the Study of vascular anomalies Montreal Meeting Abstractsbook 2000; p. 19
Copyright © 2003. Asociación Española de Pediatría
Idiomas
Anales de Pediatría (English Edition)
Article options
Tools
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?