Cloacal Exstrophy




(1)
Pediatric Surgery, AlSadik Hospital, Qatif, Saudi Arabia

 




18.1 Introduction






  • Cloacal exstrophy is an extremely rare and major birth defect representing the severe end of the spectrum of the exstrophy-epispadias complex (Fig. 18.1).

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    Fig. 18.1
    Clinical photograph showing the components of the cloacal exstrophy


  • It is also called vessico-intestinal fissure.


  • The exact incidence of cloacal exstrophy is not known but the estimated prevalence ranges from 1 in 50,000 to 250,000 live births.


  • It is more common in males than females (a male-female ratio of 2:1).


  • Cloacal exstrophy, is also called the OEIS Complex:



    • O: Omphalocele


    • E: Exstrophy of the cloaca


    • I: Imperforate Anus


    • S: Spinal Defects


  • Clinically, patients with cloacal exstrophy present at birth and it is characterized by the followings spectrum of anomalies (Figs. 18.2, 18.3, 18.4, 18.5, and 18.6):

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    Figs. 18.2 and 18.3
    Clinical photographs showing two patients with cloacal exstrophy. Note the difference in the size of omphalocele and also the extent of the exstrophied ileo-cecal region


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    Figs. 18.4 and 18.5
    Clinical photographs showing cloacal exstrophy in two patients. Note the absence of omphalocele in the first one and associated anorectal agenesis in the second one


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    Fig. 18.6
    A clinical photograph showing cloacal exstrophy. Note the associated anorectal agenesis




    • Two exstrophied hemibladders


    • These are separated by a foreshortened hindgut or cecum


    • The hindgut is often blind-ending resulting in an imperforate anus.


    • This extrophied ileo-cecal region presents between the two hemi bladders (the “elephant trunk” appearance).


    • Omphalocele


    • The associated omphalocele may be major or minor and sometimes no associated omphalocele.


    • Malrotation


    • The symphysis pubis is widely separated


    • The pelvis is often asymmetrically shaped


    • The genitalia (ambiguous genitalia):



      • In males, the penis is divided into two halves usually located separately on either side of the bladder plates with the adjacent scrotal halves.


      • In females, the clitoris is divided into two haves usually located separately on either side of the bladder plates with the adjacent part.


      • Duplication of the vagina and uterus


      • Vaginal agenesis


18.2 Etiology and Pathogenesis




Jul 10, 2017 | Posted by in UROLOGY | Comments Off on Cloacal Exstrophy
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