Cloacal exstrophy (EC) is a severe
birth defect
A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities ca ...
wherein much of the abdominal organs (the
bladder
The urinary bladder, or simply bladder, is a hollow organ in humans and other vertebrates that stores urine from the kidneys before disposal by urination. In humans the bladder is a distensible organ that sits on the pelvic floor. Urine enters ...
and
intestines
The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The GI tract contains all the major organs of the digestive system, in humans ...
) are exposed. It often causes the splitting of the bladder,
genitalia
A sex organ (or reproductive organ) is any part of an animal or plant that is involved in sexual reproduction. The reproductive organs together constitute the reproductive system. In animals, the testis in the male, and the ovary in the female, a ...
, and the
anus. It is sometimes called OEIS complex.
Diagnostic tests can include ultrasound,
voiding cystourethrogram
In urology, voiding cystourethrography (VCUG) is a frequently performed technique for visualizing a person's urethra and urinary bladder while the person urinates (voids). It is used in the diagnosis of vesicoureteral reflux (kidney reflux), am ...
(VCUG),
intravenous pyelogram
Pyelogram (or pyelography or urography) is a form of imaging of the renal pelvis and ureter.
Types include:
* Intravenous pyelogram – In which a contrast solution is introduced through a vein into the circulatory system.
* Retrograde pyelogram ...
(IVP), nuclear renogram, computerized axial tomography (
CT scan), and
magnetic resonance imaging
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wave ...
(MRI). Cloacal exstrophy is a rare birth defect, present in 1/200,000 pregnancies and 1/400,000 live births. It is associated with a defect of the
ventral
Standard anatomical terms of location are used to unambiguously describe the anatomy of animals, including humans. The terms, typically derived from Latin or Greek language, Greek roots, describe something in its standard anatomical position. Th ...
body wall and can be caused by inhibited
mesoderm
The mesoderm is the middle layer of the three germ layers that develops during gastrulation in the very early development of the embryo of most animals. The outer layer is the ectoderm, and the inner layer is the endoderm.Langman's Medical E ...
al migration. The defect can often be comorbid with
spinal bifida
Spina bifida (Latin for 'split spine'; SB) is a birth defect in which there is incomplete closing of the spine and the membranes around the spinal cord during early development in pregnancy. There are three main types: spina bifida occulta, m ...
and kidney abnormalities.
See also
*
Bladder exstrophy
Bladder exstrophy is a congenital anomaly that exists along the spectrum of the exstrophy-epispadias complex, and most notably involves protrusion of the urinary bladder through a defect in the abdominal wall. Its presentation is variable, often ...
References
External links
{{Medical resources
, DiseasesDB =
, ICD10 = {{ICD10, Q, 64, 1
, ICD9 =
, ICDO =
, OMIM = 258040
, MedlinePlus =
, MeSH =
, GeneReviewsNBK =
, GeneReviewsName =
, Orphanet = 93929
Congenital disorders of urinary system
Rare diseases
Intersex variations