A case of infant with bilateral multicystic kidney and ureteropelvic junction obstruction; surgical treatment after percutaneous nephrostomy

Abstract

女児, 20週齢で重篤な嚢胞性疾患を診断された。帝王切開によって出生, 高窒素血症のため1日齢で最大の嚢胞に腎瘻を造設したがこれが効果なく1週後にその内側に再度腎瘻を造設した。これが9ヵ月間有効で高窒素血症は改善した。9ヵ月齢で腎盂形成術をおこなった。尿管には蠕動が見られ, 膀胱から初めて排尿があった

We report a case of bilateral multicystic kidney and left ureteropelvic junction (UPJ) obstruction in a female infant. She was diagnosed to have severe cystic disease from 20 weeks of gestation. After birth with cesarean section, she was transferred to the neonatal intensive care unit (NICU) of our hospital. One day after birth, she was referred to our department for progression of azotemia. We placed a nephrostomy catheter into the largest renal cyst, but it did not work. One week later, we placed another nephrostomy catheter into another cystic lesion inside of the first one. It worked well for nine months and azotemia was improved. At the age of nine months her upper urinary tract was reconstructed by pyeloplasty. We observed peristalsis of ureter and first urination from bladder.