tailieunhanh - Abernethy malformation: A case report

Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. | Pathak et al. BMC Pediatrics 2012 12 57 http 1471-2431 12 57 BMC Pediatrics CASE REPORT Open Access Abernethy malformation a case report Acbicb p-u In A lí 1 2i l iti n A Cl .ir1 2 I3 I AAI d i c b ft lmndlixo1 Prntoolí tolnlcit4 ncd mb Ai or A1 Asi nsh rdtl Idk INiLin Agdlwdl Jdguish IVIdl Idliyd rldteek Gel not dl 10 IVIdmtd DI Idl lend Abstract Background Abernethy mdlformdtion is d very rare congenitdl vdsculdr mdlformdtion defined by diversion of portdl blood dWdy from liver. It is commonly dssocidted with multiple congenitdl dnomdlies. We present d cdse of Abernethy mdlformdtion without dssocidted congenitdl dnomdlies from Indid. Case presentation A 5-yedr-old femdle child presented with short history of jdundice. A provisiondl didgnosis of dcute virdl hepdtitis Wds mdde in view of clinicdl presentdtion dnd locdl endemicity of virdl hepdtitis A. Persistence of jdundice on follow up dfter4 weeks led to detdiled investigdtions. Ultrasound dnd doppler study of dbdomen revedled drdindge of portdl vein into inferior vend cdvd. CT dngiogrdphy wds performed which confirmed the didgnosis of Type 1 b Abernethy mdlformdtion without dssocidted mdjor dnomdlies. We discuss the common clinicdl presentdtions dssocidted dnomdlies didgnostic workup dnd tredtment options of this disorder. Conclusion The tredtment of the pdtients with congenitdl porto-systemic shunts depends on the site of the shunt dssocidted congenitdl dnomdlies dnd the extent of liver ddmdge but the prognosis depends on the complicdtions irrespective of dndtomicdl type. However the extent of dssocidted dbnormdlities should not deter pdedidtricidns to refer pdtients for tredtment. Whenever possible closure of the shunt should be ddvised for cure or to prevent complicdtions. Only symptomdtic type I pdtients with dbsence of possibility to close the shunt mdy require liver trdnspldnt. Long-term follow-up is indicdted for dll pdtients. Keywords Abernethy mdlformdtion Ultrd-sonogrdphy .