Abstract
Down's syndrome (DS) is associated with duodenal atresia (DA) in about 8-10% of cases. Transient Myeloproliferative Disorder (TMD)/Acute Myeloid Leukemia (AML) is also associated with the trisomy 21 mutation. The occurrence of the two conditions together complicates the diagnosis and surgical management of the DA. We discuss the technical aspects of management of the DA in this clinical setting.
Highlights
There is about 40% incidence of duodenal atresia (DA) in the setting of Down's syndrome (DS) [1]
We describe surgical management strategy of DA in a newborn with massive hepatomegaly secondary to congenital Transient Myeloproliferative Disorder (TMD)/Acute Myeloid Leukemia (AML)
While its morphological and phenotypic features are indistinguishable from AML all blasts in TMD carry trisomy 21 mutation
Summary
There is about 40% incidence of DA in the setting of DS [1]. DS is associated with a plethora of malformations involving multiple body systems that may affect the timing but not the technical aspects of surgical correction of DA [2]. We describe surgical management strategy of DA in a newborn with massive hepatomegaly secondary to congenital TMD/AML. The infant was started on Ara-C [Cytosine Arabinoside at a dose of 50mg/m2] for the treatment of TMD/AML on day 3 of life.
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