Studija slučaja
PHACES Syndrome with Intestinal Hemangiomatosis
Ivona Vrkić Boban
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.org/0000-0002-5496-9883
; Department of Pediatrics, University Hospital of Split, Split, Croatia
Vjekoslav Krželj
orcid.org/0000-0001-6086-4669
; University of Split, Department of Health Studies, Split, Croatia
Sažetak
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.
Ključne riječi
PHACES syndrome; neonate; intestinal hemangiomatosis
Hrčak ID:
290106
URI
Datum izdavanja:
20.11.2019.
Posjeta: 677 *