Learning objectives
Highlight the ultrasound findings of cloacal exstrophy (OEIS Complex) including the major and minor criteria.
Outline the common and uncommon associated anomalies and how do they look in ultrasound.
Knowing how to differentiate cloacal exstrophy (OEIS Complex) from other forms of abdominal wall defects.
Background
Cloacal exstrophy is major birth defect characterized by omphalocele, bladder exstrophy, imperforate anus and spinal defects (also referred to as the OEIS complex), and often associated with other malformations.
Cloacal exstrophy (OEIS Complex) is rare but serious congenital abnormality with prevalence of around 1/10,000 births.
Due to the complexity of the Cloacal exstrophy, prenatal diagnosis is difficult, challenging and requires high level of radiologic expertise. Increasing awareness of the full spectrum of cloacal exstrophy should improve prenatal diagnostic accuracy. Early and accurate prenatal diagnosis is...
Findings and procedure details
In cloacal exstrophy (OEIS Complex), omphalocele is often the initial observation at its one of the most important major findings. Other major findings include failure of visualization of the urinary bladder between the two umbilical arteries, large midline infra-umbilical anterior abdominal wall defect, low insertion of the umbilical cord, and lumbosacral meningomyelocele which usually skin-covered.
The associated minor anomalies may include lower extremity defects, renal anomalies, ambiguous genitalia, ascites, widened pubic arches, narrow thorax, hydrocephalus, cardiac defects, and single umbilical artery.
During early prenatal ultrasound...
Conclusion
Cloacal exstrophy (OEIS complex) is rare congenital and complicated multisystem defect. Prenatal diagnosis is dependent on sonographic visualization of these defects as antenatal second trimester ultrasound plays extremely important role in the diagnosis; however, the diagnosis remains challenging. Because of the severity of this anomaly and subsequent poor quality of life for individuals born with Cloacal exstrophy, patients may choose pregnancy termination. Infants born with this condition will require significant long-term medical care and surgical intervention
Personal information and conflict of interest
N. M. Saloum:
Nothing to disclose
R. R. Hussein Yousef:
Nothing to disclose
A. A. A. M. Al-Rashid:
Nothing to disclose
M. A. M. Qasem:
Nothing to disclose
References
Reza Pakdaman, et al. “Complex Abdominal Wall Defects: Appearances at Prenatal Imaging”. RadioGraphics (2015): 636-649.
Nehal Saloum, et al. Role of Ultrasound in Diagnosis and Management of OEIS Syndrome, case series. EC Gynecology (2019).
Austin PF, et al. The prenatal diagnosis of cloacal exstrophy. J Urol (1998):1179–1181.
Ben-Neriah., et al. “OEIS complex: prenatal ultrasound and autopsy findings. OEIS complex: prenatal ultrasound and autopsy findings”. Ultrasound in Obstetrics and Gynecology (2007):170-177.
Barisic I, et al. Evaluation of prenatal ultrasound diagnosis of fetal abdominal wall defects by...