OEIS complex with glomerulocystic kidney disease: a case report.
We present a case of OEIS complex (omphalocele, exstrophy of bladder, imperforated anus, spinal defect) combined with colonic agenesis and glomerulocystic kidney disease (GCKD). The baby was born at 35.2 weeks of gestational age, weighing 2.51 kg. A prenatal ultrasound examination showed spina bifida, hydroureter, and a unilateral polycystic kidney. The postdelivery examination, which included a physical examination, simple X-ray, and pelvic MRI, showed a lower abdominal wall defect through which a small pouch with a segment of bowel protruded, imperforated anus, ambiguous external genitalia, spina bifida with meningomyelocele at the lumbosacral junction, and nonunion of pubic symphysis. The baby underwent surgery, including nephrectomy, colostomy, and repair of the abdominal wall defect. In addition to the abnormalities mentioned, a tailgut as a result of colonic agenesis and 2 appendices were identified in the course of surgery. The result of histopathological examination confirmed the polycystic kidney identified as GCKD. These radiological, surgical, and histopathologic findings are consistent with the OEIS complex. The postoperative course was uneventful during a period of 4 months of follow up. We herein report a case of the very rare OEIS complex in a newborn male baby and review the available literature.
Hong
Ran
R
College of Medicine, Chosun University, Gwangju, Korea.
Lim
Sung-Chul
SC
Jang
Jung-Whan
JW
Suh
Chae-Hong
CH
Jeon
Ho-Jong
HJ
Lee
Mi-Ja
MJ
Kim
Youn-Shin
YS
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000473:pathology*; D000328:Adult; D001006:Anus, Imperforate / Q000000981:diagnostic imaging / Q000473:pathology*; D001746:Bladder Exstrophy / Q000000981:diagnostic imaging / Q000473:pathology*; D003106:Colon / Q000002:abnormalities / Q000000981:diagnostic imaging; D005260:Female; D005865:Gestational Age; D006554:Hernia, Umbilical / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D007690:Polycystic Kidney Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D011247:Pregnancy; D011263:Pregnancy Trimester, Third; D016135:Spinal Dysraphism / Q000000981:diagnostic imaging / Q000473:pathology*; D016216:Ultrasonography, Prenatal