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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Definitions
- Cardinal findings of cloacal exstrophy (CE) are hemibladder exstrophy, hindgut extrusion, imperforate anus
- Hemibladders contain ureter/vas deferens orifices in males, uterovaginal canal in females
- Hemibladders flank opening of small intestine/blind-ending large intestine
- Omphalocele present in 64% (i.e., it is not essential for diagnosis)
- Exstrophy epispadias complex (EEC)
- Spectrum of malformations ranging from epispadias to bladder exstrophy (BE) to CE
- Defects involve genitourinary/musculoskeletal systems, pelvis, pelvic floor, abdominal wall, spine, anus
- Term OEIS complex was proposed by Carey et al in 1978 as way to recall all defects present
- Omphalocele
- Exstrophy of bladder
- Imperforate anus
- Spinal deformities (includes vertebral anomalies, dysraphism, sacral defects)
- Birth defect registries now use other terms to describe other combinations of findings with CE
- OEI: Omphalocele, exstrophy, imperforate anus
- EIS: Exstrophy, imperforate anus, spine abnormalities
- Cardinal findings of cloacal exstrophy (CE) are hemibladder exstrophy, hindgut extrusion, imperforate anus
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Gondo K et al: Clues and pitfalls in prenatal diagnosis of classic cloacal exstrophy using ultrasonography and magnetic resonance imaging: a case with sequential observation from 17 to 30weeks' gestation and literature review. J Obstet Gynaecol Res. 46(8):1443-9, 2020
- Weiss DA et al: Key anatomic findings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy. J Pediatr Urol. 16(5):665-71, 2020
- Arteaga-Vázquez J et al: OEIS complex: prevalence, clinical, and epidemiologic findings in a multicenter Mexican birth defects surveillance program. Birth Defects Res. 111(11):666-71, 2019
- Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
- Coleman PW et al: Fetal MRI in the identification of a fetal ventral wall defect spectrum. AJP Rep. 8(4):e264-76, 2018
- Neel N et al: Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed. Urol Ann. 10(1):118-21, 2018
- Keppler-Noreuil KM et al: Clinical and risk factor analysis of cloacal defects in the National Birth Defects Prevention Study. Am J Med Genet A. 173(11):2873-85, 2017
- Mallmann MR et al: Omphalocele-exstrophy-imperforate anus-spinal defects complex: associated malformations in 12 new cases. Fetal Diagn Ther. 41(1):66-70, 2017
- Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
- Prefumo F et al: Fetal abdominal wall defects. Best Pract Res Clin Obstet Gynaecol. 28(3):391-402, 2014
- Calvo-Garcia MA et al: Fetal MRI of cloacal exstrophy. Pediatr Radiol. 43(5):593-604, 2013
- Suson KD et al: Intracranial anomalies and cloacal exstrophy--is there a role for screening? J Pediatr Surg. 48(11):2256-60, 2013
- Feldkamp ML et al: Cloacal exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research. Am J Med Genet C Semin Med Genet. 157C(4):333-43, 2011
- Sawaya D et al: Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience. J Pediatr Surg. 45(1):171-5; discussion 175-6, 2010
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Definitions
- Cardinal findings of cloacal exstrophy (CE) are hemibladder exstrophy, hindgut extrusion, imperforate anus
- Hemibladders contain ureter/vas deferens orifices in males, uterovaginal canal in females
- Hemibladders flank opening of small intestine/blind-ending large intestine
- Omphalocele present in 64% (i.e., it is not essential for diagnosis)
- Exstrophy epispadias complex (EEC)
- Spectrum of malformations ranging from epispadias to bladder exstrophy (BE) to CE
- Defects involve genitourinary/musculoskeletal systems, pelvis, pelvic floor, abdominal wall, spine, anus
- Term OEIS complex was proposed by Carey et al in 1978 as way to recall all defects present
- Omphalocele
- Exstrophy of bladder
- Imperforate anus
- Spinal deformities (includes vertebral anomalies, dysraphism, sacral defects)
- Birth defect registries now use other terms to describe other combinations of findings with CE
- OEI: Omphalocele, exstrophy, imperforate anus
- EIS: Exstrophy, imperforate anus, spine abnormalities
- Cardinal findings of cloacal exstrophy (CE) are hemibladder exstrophy, hindgut extrusion, imperforate anus
IMAGING
General Features
Ultrasonographic Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Gondo K et al: Clues and pitfalls in prenatal diagnosis of classic cloacal exstrophy using ultrasonography and magnetic resonance imaging: a case with sequential observation from 17 to 30weeks' gestation and literature review. J Obstet Gynaecol Res. 46(8):1443-9, 2020
- Weiss DA et al: Key anatomic findings on fetal ultrasound and MRI in the prenatal diagnosis of bladder and cloacal exstrophy. J Pediatr Urol. 16(5):665-71, 2020
- Arteaga-Vázquez J et al: OEIS complex: prevalence, clinical, and epidemiologic findings in a multicenter Mexican birth defects surveillance program. Birth Defects Res. 111(11):666-71, 2019
- Vilanova-Sanchez A et al: Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review. J Pediatr Adolesc Gynecol. 32(1):7-14, 2019
- Coleman PW et al: Fetal MRI in the identification of a fetal ventral wall defect spectrum. AJP Rep. 8(4):e264-76, 2018
- Neel N et al: Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed. Urol Ann. 10(1):118-21, 2018
- Keppler-Noreuil KM et al: Clinical and risk factor analysis of cloacal defects in the National Birth Defects Prevention Study. Am J Med Genet A. 173(11):2873-85, 2017
- Mallmann MR et al: Omphalocele-exstrophy-imperforate anus-spinal defects complex: associated malformations in 12 new cases. Fetal Diagn Ther. 41(1):66-70, 2017
- Pakdaman R et al: Complex abdominal wall defects: appearances at prenatal imaging. Radiographics. 35(2):636-49, 2015
- Prefumo F et al: Fetal abdominal wall defects. Best Pract Res Clin Obstet Gynaecol. 28(3):391-402, 2014
- Calvo-Garcia MA et al: Fetal MRI of cloacal exstrophy. Pediatr Radiol. 43(5):593-604, 2013
- Suson KD et al: Intracranial anomalies and cloacal exstrophy--is there a role for screening? J Pediatr Surg. 48(11):2256-60, 2013
- Feldkamp ML et al: Cloacal exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research. Am J Med Genet C Semin Med Genet. 157C(4):333-43, 2011
- Sawaya D et al: Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience. J Pediatr Surg. 45(1):171-5; discussion 175-6, 2010
