Neuropediatrics 2011; 42(1): 24-27
DOI: 10.1055/s-0031-1273705
Short Communications

© Georg Thieme Verlag KG Stuttgart · New York

Cutaneous, Mediastinal and Hepatic Hemangiomas in a Girl Followed During 12 Years

I. Pascual-Castroviejo1 , M. Parrón Pajares2 , S.-I. Pascual-Pascual3 , P. Jara4 , R. Velázquez-Fragua3
  • 1Private Surgery, Pediatric Neurology, Madrid, Spain
  • 2Department of Radiology, University Hospital La Paz, Madrid, Spain
  • 3Department of Pediatric Neurology, University Hospital La Paz, Madrid, Spain
  • 4Department of Pediatric Hepatology, University Hospital La Paz, Madrid, Spain
Further Information

Publication History

received 10.11.2010

accepted 11.2.2011

Publication Date:
09 May 2011 (online)

Abstract

We present in this paper the case of a 12-year-old girl who had the clinical features of 2 different disorders: neurofibromatosis 1 (NF1) and 3 hemangiomas located in the skin, liver and mediastinum. The patient did not receive any specific treatment and showed a normal progressive evolution that lasted 1 / to 2 years and a very slow regression that lasted for a more prolonged time than expected (the 3 hemangiomas have not completely disappeared yet), although all 3 have been asymptomatic. MRI of the brain did not disclose a hemangioblastoma of the cerebellum or any other vascular lesion of the brain. Mental development of this girl was in the borderline range, as is commonly seen in Pascual-Castroviejo II syndrome (P-CIIS)/PHACE syndrome and in NF1, 2 syndromes which have not been reported to be associated in the same patient previously.

References

  • 1 Denoyelle F, Leboulanger N, Enjolras O. et al . Role of propanolol in the therapeutic strategy of infantile laryngotracheal hemangioma.  Int J Pediatr Otorhinolaryngol. 2009;  73 1168-1172
  • 2 Frieden IJ, Reese V, Cohen D. PHACE syndrome: the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities.  Arch Dermatol. 1996;  132 307-311
  • 3 Herman TE, McAlister WH, Dehner LP. Posterior mediastinal capillary hemangioma with extradural extension resembling neuroblastoma.  Pediatr Radiol. 1999;  29 517-519
  • 4 Metry DW, Hawrot A, Altman C. et al . Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis.  Arch Dermatol. 2004;  140 591-596
  • 5 Pascual-Castroviejo I. Vascular and non-vascular intracranial malformations associated with external capillary hemangiomas.  Neuroradiology. 1978;  16 82-84
  • 6 Pascual-Castroviejo I, Viaño J, Moreno F. et al . Hemangiomas of the head, neck and chest with associated vascular and brain anomalies: A complex neurocutaneous syndrome.  Am J Neuroradiology. 1996;  17 461-471
  • 7 Pascual-Castroviejo I, Fernandez-Cuadrado J, Cortés P. et al . Hemangioma cutáneo asociado a hemangioma hepático y a neurofibromatosis tipo 1 (NF1).  Rev Neurol (Barcelona). 2002;  34 652-654
  • 8 Pascual-Castroviejo I, Pascual-Pascual SI, Velázquez-Fragua R. et al . Hemangiomas y malformaciones vasculares cutáneas y patología asociada (síndrome de Pascual-Castroviejo tipo II). Presentación de 41 pacientes.  Rev Neurol (Barcelona). 2005;  41 223-236
  • 9 Pascual-Castroviejo I, Viaño J, Pascual-Pascual SI. et al . Congenital and evolving vascular disorders associated with cutaneous hemangiomas: case report.  Neuropediatrics. 2009;  40 148-151
  • 10 Pascual-Castroviejo I, Pascual-Pascual SI, Velázquez-Fragua R. et al . Association of cutaneous red-to-purple hemangiomas with leptomeningeal hemangioma. A clinical study of two patients.  Neuropediatrics. 2010;  41 7-11
  • 11 Truong MT, Chang KW, Berk DR. et al . Propanolol for the treatment of life-threatening subglottic and mediastinal infantile hemangioma.  J Pediatr. 2010;  156 335-338

Correspondence

Ignacio Pascual-Castroviejo

Private surgery

Pediatric Neurology

14 Orense Street

28020 Madrid

Spain

Phone: +34/91/555 0356

Fax: +34/91/623 6572

Email: i.pcastroviejo@neurologia.e.telefonica.net

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