Exstrophia splanchnica (exstrophy of cloaca) (1부검증례)
Exstrophia splanchnica (An autopsy case)

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서연림; 지제근
Issue Date
서울대학교 의과대학
Seoul J Med 1984;25(2):271-276
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.
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 25 No.2 (1984)
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