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Acta Dermatovenerologica Croatica, Vol. 27 No. 4, 2019.

Case report, case study

PHACES Syndrome with Intestinal Hemangiomatosis

Ivona Vrkić Boban orcid id orcid.org/0000-0002-0250-1904 ; Department of Pediatrics, University Hospital of Split, Split, Croatia
Bernarda Lozić ; Department of Pediatrics, University Hospital of Split, Split, Croatia; 2University of Split, School of Medicine, Split, Croatia
Luka Stričević ; Department of Pediatrics, University Hospital of Split, Split, Croatia; 2University of Split, School of Medicine, Split, Croatia
Ivana Čulo Čagalj ; Department of Pediatrics, University Hospital of Split, Split, Croatia; 2University of Split, School of Medicine, Split, Croatia
Ana Skelin Glavaš orcid id orcid.org/0000-0002-5496-9883 ; Department of Pediatrics, University Hospital of Split, Split, Croatia
Vjekoslav Krželj orcid id orcid.org/0000-0001-6086-4669 ; University of Split, Department of Health Studies, Split, Croatia

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Abstract

We present a rare case of a neonate with PHACES syndrome (posterior fossa malformations, large facial hemangiomas, cerebral arterial anomalies, cardiovascular anomalies, eye anomalies and sternal clefting or supraumbilical raphe) and diffuse hemangiomatosis of the ileum, presenting with multiple intestinal perforations and peritonitis. The infant was successfully treated with propranolol and methylprednisolone as well as octreotide, tranexamic acid, and supportive therapy for massive intestinal bleeding.

Keywords

PHACES syndrome; neonate; intestinal hemangiomatosis

Hrčak ID:

290106

URI

https://hrcak.srce.hr/290106

Publication date:

20.11.2019.

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