The present paper describes a 14-year-old female who presented with hyperchloremic metabolic acidosis, secondary to retained urine in a redundant ileal conduit, and symptoms of müllerian outflow tract obstruction. [ncbi.nlm.nih.gov]

Clinical description Patients present at birth with two exstrophied hemibladders separated by a foreshortened hindgut (often blind-ending resulting in an imperforate anus) or cecum. [orpha.net]

• Wound Infection

  One patient had a superficial wound infection, and one had premature suprapubic tube dislodgement requiring replacement in the operating room. [ncbi.nlm.nih.gov]

• Atrial Septal Defect

  Cardiological ultrasonography found an atrial septal defect (ASD) of 1.9 mm and persistant arterious ductus (3.3 mm). The cytogenetic study was normal: 46, XY. Chorionic gonadotropin stimulation test was reactive. [criticalcare.imedpub.com]

• Systolic Murmur

  Cardiovascular : systolic murmur III/VI. Abdomen and pelvis: absence of abdominal wall, exposed bowel loops partially covered by the omphalocele membrane. Cloacal exstrophy, rectum and anal atresia. [criticalcare.imedpub.com]

• Long Arm

  The karyotype showed a de novo unbalanced translocation between the long arm of chromosome 9 and the long arm of chromosome Y resulting in a 9q34.1-qter deletion. [ncbi.nlm.nih.gov]

• Hypertelorism

  Additional malformations included hydrocephalus, hypertelorism, aplasia of the gall bladder, heart defects and kidney malformations. All karyotyped fetuses (11/11) showed a normal karyotype. [karger.com]

• Psychomotor Retardation

  retardation Table 1: Abnormalities of the gastrointestinal tract, genitourinary, skeletal system, cardiovascular and neurological system. [criticalcare.imedpub.com]

It demonstrates also that only a minority (19%) of the European centres involved in the treatment of exstrophy can be considered "high volume" exstrophy centres. [ncbi.nlm.nih.gov]

Treatment may include: Staged reconstruction - This method of treatment addresses the condition through a series of surgeries over a period of time. The timing and outcome of these surgeries will vary depending on your child's particular condition. [cedars-sinai.edu]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

Patients with ARMs and tethered cord have a worse functional prognosis regarding bowel and urinary function. [emedicine.medscape.com]

While not usually lethal, cloacal exstrophy often carries a poor prognosis where a series of operations are often required. [radiopaedia.org]

Generally, if no complications arise and the treatment is successful, the prognosis can be good Preventative measures for the condition are not well understood and ongoing research may help understand the condition better. [dovemed.com]

[…] addition to the traditional prognostic factors for bowel and urinary control (sacral ratio, tethered cord, and level of the rectum) these patients have other anatomic defects (absent bladderneck and short colon) that negatively affect the functional prognosis [ncbi.nlm.nih.gov]

The etiology is unknown, but likely heterogeneous. [ncbi.nlm.nih.gov]

Etiology EC results from an anomaly during early embryologic development associated with rupture of the cloacal membrane before fusion with the urorectal septum. [orpha.net]

Cloacal Exstrophy: An Epidemiologic Study From the International Clearinghouse for Birth Defects Surveillance and Research. American Journal of Medical Genetics. [rug.nl]

Summary Epidemiology Prevalence at birth for EEC is reported at 1/10,000. [orpha.net]

Birth Defects Epidemiology and Surveillance Branch,. Texas, USA. Fundação Oswaldo Cruz. Instituto Oswaldo Cruz. Estudio Colaborativo Latino Americano de Malformaciones Congénitas. [arca.fiocruz.br]

Gearhart, Ranjiv Mathews Springer Science & Business Media, 31.07.1999 - 252 Seiten This book contains the most up-to-date scientific information on the anatomy, physiology, and pathophysiology of the bladder muscle, nerves, and collagen in the patient [books.google.de]

Risk Factors Bladder exstrophy: only known association is related to offspring of in vitro fertilization pregnancies Pathophysiology Embryology Normal development By week 2 of gestation, the cloacal membrane is located at the caudal end of the infraumbilical [unboundmedicine.com]

Epispadias is a variant that displays normal bladder formation but incomplete urethral tubularization from the bladder neck down. [1] Pathophysiology The true cause of bladder exstrophy has not been elucidated. [emedicine.medscape.com]

[…] for Disease Control and Prevention (CDC). [mblynchfirm.com]

Abdominal reconstruction was performed for a series of recurrent and primary (preventive) cases. [ncbi.nlm.nih.gov]

Generally, if no complications arise and the treatment is successful, the prognosis can be good Preventative measures for the condition are not well understood and ongoing research may help understand the condition better. [dovemed.com]

Key Points Arises from maldevelopment of the cloacal membrane, which prevents migration of mesenchymal tissue and impedes normal development of the lower abdominal wall. [obgyn.mhmedical.com]