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.
Key words
Pascual-Castroviejo type II syndrome (P-CIIS) - PHACE - cutaneous hemangioma - hepatic
hemangioma - mediastinal hemangioma - neurocutaneous disorders
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