KEGG   DISEASE: Cloacal exstrophy
Entry
H01856                      Disease                                
Name
Cloacal exstrophy;
Vesicointestinal fissure
Description
Cloacal exstrophy is an extremely rare congenital complex deformity that is associated with anterior abdominal wall defects, reflex and exposure of the cloaca (uninterrupted ureter and bowel), aproctia, a widely separated pubic bone, and defects or dysplasia of external genital organs. Cloacal exstrophy is thought to be related to abnormal development of the cloacal membrane, a transitory structure composed of endoderm and ectoderm that overlies the embryonic cloaca. The result is primarily an abdominal wall defect with failed closure of the lower urinary tract. Cloacal exstrophy has also been referred to as the OEIS complex (omphalocele, exstrophy, imperforate anus and spinal defect), and other malformations of the urogenital, gastrointestinal, skeletal and neurospinal axis frequently coexist.
Category
Congenital malformation
Brite
Human diseases in ICD-11 classification [BR:br08403]
 20 Developmental anomalies
  Structural developmental anomalies primarily affecting one body system
   Structural developmental anomalies of the digestive tract
    LB17  Structural developmental anomalies of anal canal
     H01856  Cloacal exstrophy
Other DBs
ICD-11: LB17.3
OMIM: 258040
Reference
  Authors
Makiguchi T, Yokoo S, Takaku Y, Morita H, Yasuda M
  Title
Squamous cell carcinoma arising from abdominal wall defect lesion complicated with cloacal exstrophy.
  Journal
Plast Reconstr Surg Glob Open 3:e315 (2015)
DOI:10.1097/GOX.0000000000000286
Reference
  Authors
Woo LL, Thomas JC, Brock JW
  Title
Cloacal exstrophy: a comprehensive review of an uncommon problem.
  Journal
J Pediatr Urol 6:102-11 (2010)
DOI:10.1016/j.jpurol.2009.09.011
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