A rare presentation of isolated congenital splenic hemangioma with Kasabach-Merritt syndrome

Fredrick Dapaah-Siakwan, Carleene Bryan, Lashawndra S. Walker, Teresa Delmoral

Research output: Contribution to journalArticle

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Abstract

Introduction: Splenic hemangiomas (SHs) are the most common benign neoplasms of the spleen. However, they are rare in the newborn period. We present an extremely rare case of congenital SH complicated by Kasabach-Merritt syndrome. Case Presentation: A 2.93 kg male infant was delivered at term with a prenatal diagnosis of a left infrarenal mass diagnosed by ultrasound at 35 weeks of gestation. Magnetic resonance imaging demonstrated a well-defined splenic mass with multiple flow voids and scattered areas of high intensity suggestive of hemorrhage. He developed anemia, thrombocytopenia, and coagulopathy which required transfusion with packed red cells, platelets, cryoprecipitate, and fresh frozen plasma. Excision biopsy of the spleen led to resolution of anemia, thrombocytopenia, and coagulopathy. The diagnosis of SH was confirmed by histopathology. At 2 months outpatient follow-up, the patient was growing well without any evidence of tumor recurrence. Conclusions: Congenital SH is a rare entity that can be fatal if the potential complication of Kasabach-Merritt syndrome is not anticipated, evaluated, and promptly treated. Our patient had a favorable outcome with early surgical excision of the SH.

Original languageEnglish (US)
Pages (from-to)e29-e32
JournalJournal of Pediatric Hematology/Oncology
Volume39
Issue number1
DOIs
StatePublished - 2017

Fingerprint

Kasabach-Merritt Syndrome
Hemangioma
Thrombocytopenia
Anemia
Spleen
Prenatal Diagnosis
Neoplasms
Outpatients
Blood Platelets
Magnetic Resonance Imaging
Newborn Infant
Hemorrhage
Biopsy
Recurrence
Pregnancy

Keywords

  • Disseminated intravascular coagulation
  • Kasabach-Merritt syndrome
  • Neonate
  • Splenic hemangioma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

A rare presentation of isolated congenital splenic hemangioma with Kasabach-Merritt syndrome. / Dapaah-Siakwan, Fredrick; Bryan, Carleene; Walker, Lashawndra S.; Delmoral, Teresa.

In: Journal of Pediatric Hematology/Oncology, Vol. 39, No. 1, 2017, p. e29-e32.

Research output: Contribution to journalArticle

Dapaah-Siakwan, Fredrick ; Bryan, Carleene ; Walker, Lashawndra S. ; Delmoral, Teresa. / A rare presentation of isolated congenital splenic hemangioma with Kasabach-Merritt syndrome. In: Journal of Pediatric Hematology/Oncology. 2017 ; Vol. 39, No. 1. pp. e29-e32.
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