Cloacal Malformations: Technical Aspects of the Reconstruction and Factors Which Predict Surgical Complexity

Abstract

Cloacal malformations are rare anomalies which occur in one in 50,000 live births. Anatomically these anomalies are defined by the presence of a single perineal orifice. There is however a substantial range in their complexity. Defining these differences is key to surgical planning and timely referral of selected cases to centers with the capabilities to manage the most challenging cases. Traditionally the common channel length as measured during cysto-vaginoscopy has been used to differentiate between patients that can be repaired with a reproducible operation and those requiring a more complex repair. The quality and range of imaging available has advanced and thus a more detailed anatomic picture is now possible to help with pre-operative planning. Cross sectional imaging with 3D reconstruction has enhanced the understanding of the anatomic variations in these patients. In addition, the sacral ratio, previously thought to only have an influence on long term continence predictions, has been shown to not only forecast the presence of urological anomalies, but also the complexity of the malformation. In principle, cloacal malformations have two major components to the reconstruction. First, the rectum needs to be separated from the urogenital tract and second, the urogenital sinus needs to be managed to create a urethral orifice and vaginal introitus. The length of the urethra has been shown to be vital in deciding between the two main surgical maneuvers; a total urogenital mobilization (TUM) and a urogenital separation. The technical demands of a urogenital separation are significant and discussed here in detail. The need for vaginal replacement adds further complexity to the care of these patients and has also been shown to be related to the length of the urethra. Predicting complexity in an accurate and non-invasive way will facilitate the care of the most complex cloacal malformations and improve outcomes.

Cloacal Malformation in Female Children: Outcome of Initial Management

“…The other complications found in the current series were vaginostomy stenosis and bowel stoma retraction or prolapse. 22,23 Severe sepsis at the time of presentation was the major cause of mortality in our series. 24 The mortality was further increased if superimposed by the associated anomalies.…”

Reconsidering Diagnosis, Treatment, and Postoperative Care in Children with Cloacal Malformations

“…Similarly bringing the bladder neck below the pelvic floor has the potential to lead to poor outcome. 11 More recently it has been proposed to select between these two techniques, urogenital separation and TUM more carefully by using both common channel length and urethral length and making this selection prior to starting the operation in order to prevent function loss as far as possible. TUM is widely used and almost always successful if the patient has a common channel of less than 3 cm and a urethra of greater than 1.5 cm.…”

“…[8][9][10] Pain, inconvenience and the cost of treatment are therefore important real-world considerations. 11 If ARM form a continuous spectrum, then the same may be true for CM since there is wide variation in both types and degrees of severity. Appreciation of the key issues likely to affect an individual's long-term outcome, including quality of life, would allow personalized care.…”

Long-term urologic and gynecologic follow-up and the importance of collaboration for patients with anorectal malformations

“…In more complex cloacal malformations pre-operative vaginoscopy assessing the gynecologic anatomy is crucial to successful pre-operative planning. 43 For example, a long common channel length is associated with a higher likelihood of requiring a vaginal replacement. 44 Alternatively, identifying a longitudinal vaginal septum and two cervices during preoperative vaginoscopy provides insight into the anticipated uterine anatomy as well as the opportunity to remove the septum at the time of the primary reconstruction.…”