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Cloaca, Posterior Cloaca and Absent Penis Spectrum

  • Alberto Peña
  • Andrea Bischoff
Chapter
  • 1.7k Downloads

Abstract

Cloaca

This is one of the most extensive chapters of the book because of its relevance. Since 1982, when the first cloaca was repaired via posterior sagittal approach, over 500 cases have been operated by the senior author, and many lessons have been learned. Based on this experience, it is that now cloacas are described as a spectrum of defects with multiple anatomic variations. For each anatomic variation, a different surgical maneuver is indicated. This experience is presented in a systematic way, describing what is called the decision-making algorithm in the management of cloacas, beginning with a simple type of defects with excellent functional prognosis and going into more complex defects, describing different surgical alternatives. Special emphasis is placed on the surgical treatment of cloacas with good functional prognosis with a common channel shorter than 3 cm, describing in detail a very valuable surgical maneuver called “total urogenital mobilization.” This is illustrated with photographic intraoperative material, artwork, and special animations. Also, this type of material is presented to describe more complex maneuvers like “vaginal switch” and vaginal replacement. Space is dedicated to the neonatal management of patients with cloacas because of its relevance for the preservation of renal function. Subsequently, the authors present a detailed description of the best way to protect the kidneys after the repair, considering the fact that most of these patients have very important urologic issues. Further discussion is focused on the obstetric and gynecologic implications of cloacas based on the long-term follow-up of patients born with these defects.

Posterior Cloaca and Absent Penis Spectrum

A special type of malformation called by the authors “posterior cloaca” is presented. A single perineal orifice is located in the same location as the anus, and the urogenital sinus is posteriorly deviated and connected to the anterior rectal wall, the anus, or to the perineum. The surgical treatment of this particular malformation includes very specific details which make it completely different from the surgical approach of a typical cloaca. Most of these patients are born with potential for bowel control, and therefore, special emphasis is placed on surgical details to preserve bowel control in these patients. This is illustrated with elegant artwork and intraoperative pictures of patients with posterior cloacas.

In this same section is presented another malformation called absent penis. Even when this particular unusual defect occurs in male patients, it is presented here because of the striking similarities with the posterior cloaca. Both types of malformations in males and females have a very enlarged pubic bone or cartilage. The urogenital sinus in the case of females or the posterior urethra in males is posteriorly deviated and connected to the anterior aspect of the rectum or to the perineum, and to separate that urethra from the rectum or the anus requires a trans-anorectal operation in both cases. The authors propose that both malformations are part of the same spectrum in different genders.

Keywords

Bladder Neck Common Channel Anorectal Malformation Intermittent Catheterization Urogenital Sinus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Animation 16.1

Total urogenital mobilization in a case with a common channel shorter than 3 cm. (sagittal view). (a) In a case with hydrocolpos (WMV 30592 kb)

Animation 16.2

Total urogenital mobilization posterior sagittal exposure (WMV 30592 kb)

Animation 16.3

Separation of the bladder and urethra from the genital tract. (a) In a case with hydrocolpos (WMV 30592 kb)

Animation 16.4

Vaginal switch (WMV 30592 kb)

Animation 16.5

Vaginal replacement with descending colon. (WMV 28608 kb)

Animation 16.4

Vaginal switch (WMV 30592 kb)

Animation 16.5

Vaginal replacement with descending colon. (WMV 28608 kb)

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Alberto Peña
    • 1
  • Andrea Bischoff
    • 1
  1. 1.Pediatric SurgeryColorectal Center for Children Cincinnati Children’s HospitalCincinnatiUSA

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