Cloacal Exstrophy

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).


    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):


    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


    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


    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

  • The exact etiology of cloacal exstrophy is not known.

  • Several theories have been proposed to explain the pathogenesis of cloacal exstrophy but none of them can fully explain the spectrum of anomalies seen in cloacal exstrophy.

  • The most accepted theory is that cloacal exstrophy results from premature rupture of the cloacal membrane prior to caudal migration of the urorectal septum, and failure of fusion of the genital tubercles.

  • Embryo logically:

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

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