Exstrophia splanchnica (exstrophy of cloaca) (1부검증례)

Abstract

Exstrophia splanchnica is rare complex anomalies involving exstrophy of urinary bladder , absence of a major portion of colon and imperforate anus. This malformation complex is understood along the line 。f the primary cloacal membrane remaining as a thin endodermal structure due to failure of development of lower parietal mesoderm. After breakdown of this membrane, the urogenital portion of endodermal cloaca becomes exposed. In addition there is failure of development of the urorectal septum , so that the dorsal wall of the hindgut is exposed too. Recently we had a case of exstrophia splanchnica that was associated with central nervous system anomalies in a newborn infan t. This male baby was a 2.35kg product of 32 weeks gestation to a 29 year old primigravid mother. The pregnancy was complicated by a marked oligohydramnios. No history of drug intake or infections during pregnancy was obtained. He did poorly after birth; apgar score being 1 at one and 5 minutes , and died several hours later. Postmortem examination showed gastroschisis, exstrophy of the bladder associated ￦it h imperforate anus and ambiguous genitalia. The terminal ileum opened to the exstrophied bladder mucosa The larger bowel πas represented by a 10cm long blind sac. Two appendices were found opening into the terminal ileum. The testes were undescended The external genitalia were abnormally formed, the phallus being represented by short broad midline structure and scrotal folds located below the exstrophy. There were bilateral dysplastic kidneys with a marked hydroureter on the left side and the small atretic ureter on the righ t. Both ureters opened int。 the everted bladder mucosa. Associated malformation of central nervous system included Arnold-Chiari malformation Type II and sacral meningomyelocele The umbilical cord had single umbilical artey.