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Key Points

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  1. Initial total repair versus staged repair has shown no difference in outcome.

  2. Preservation of the hindgut is critical to avoiding nutritional and metabolic issues.

  3. Gender reassignment is controversial and all phallic structures should be preserved if there is any possibility of raising the 46xy infant as a male.

  4. Posterior sagittal anorectoplasty is a viable option in selected patients with help of an ACE procedure for bowel management.

  5. Urinary reconstruction is preferable with a plan for a catheterizable stoma in a urinary reservoir.

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Introduction

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More than 50 years have elapsed since the report by Rickham describing the first successful repair of this complex problem. Today, treatment has evolved such that survival approaches 90 to 100%; however, what is now apparent is that a team approach to treatment is essential—pediatric surgeons, urologists, orthopedic surgeons, neurosurgeons, endocrinologists, geneticists, etc.—and the outcome must include a focus on the child's quality of life.

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Disease Pathophysiology

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Anatomy

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The anatomy of this complex problem is usually readily visible to the experienced surgeon. The usual case consists of a moderate-sized omphalocele, a prolapsed terminal ileum (elephant-trunk deformity), 2 exposed hemibladders, 1 or 2 appendiceal orifices, and 2 ureteral orifices (Figs. 68-1 and 68-2). The anus is usually imperforate. The hemibladders may join superior or inferior to the prolapsed intestine. The genitalia of the male infant is usually manifested as a bifid penis that is widely separated, intra-abdominal testes, and either an absent scrotum or widely separated scrotal halves. The genitalia of the female infant are most commonly manifested as a bifid clitoris, but like the penis in males, it may be absent. Duplication of the uterus and vagina is usually present with the ovaries being normal.

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Figure 68-1

An infant with classic cloacal extrophy characterized by a large omphalocele and prolapsing terminal ileum (elephant-trunk deformity).

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Figure 68-2

Major components of classic cloacal extrophy: omphalocele, exstrophied bilateral hemibladders with ureteric or Müllerian remnant orifices; central exstrophied ileocecal bowel plate with superior orifice of the terminal ileum, inferiorly, the colon, and centrally, the appendix; bifid rudimentary external genitalia; separated pubic rami.

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Not all cases of cloacal exstrophy fit into 1 classical category. Hurwitz et al developed a classification system, summarized in Table 68-1, which describes the variations of cloacal exstrophy by using the bladder and bowel surface patterns as the basis for this system. The coding system developed by Manzoni et al emphasizes the surface anatomy and is very useful for the comparison of cases between institutions. Table 68-2 illustrates the coding symbols and all of the potential differences that may exist in a particular patient.

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Table Graphic Jump Location
Table 68-1Cloacal ...

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