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Cloacal Malformations

  • Author: Marc A Levitt, MD; Chief Editor: Eugene S Kim, MD, FACS, FAAP  more...
 
Updated: Dec 09, 2015
 

Background

A persistent cloaca is defined as a confluence of the rectum, vagina, and urethra into a single common channel. This defect is one of the most formidable technical challenges in pediatric surgery. The etiology of persistent cloaca is unknown.

Cloacal anomalies occur in 1 per 20,000 live births. They occur exclusively in girls and comprise the most complex defect in the spectrum of anorectal malformations. Families and physicians dealing with a newborn baby girl who has a cloacal malformation have three main concerns, as follows:

  • Urinary control
  • Bowel control
  • Sexual function (menstruation, intercourse, obstetric issues)

The goals of treatment include an anatomic reconstruction and achieving bowel and urinary control, as well as normal sexual function.

Diverse treatments for the management of persistent cloaca have been proposed in the past. The most common approach consisted of repairing the rectal component of the malformation without repairing the urogenital sinus or planning its repair in a second stage. A combined abdominal, perineal, vaginal, and simultaneous rectal pull-through was also used.

Some treatments were adequate for certain cloacae but not for others. The perineal approach to urogenital sinus repair may be useful in patients with low defects, but not in those with higher defects. Similarly, the abdominal approach is required for some anomalies but not for others. Traditional approaches were limited in their exposure and, thus, could not clearly define the complex anatomy of the defect, and the urinary sphincter and anorectal sphincter were matters of speculation.

In 1982, the posterior sagittal approach, which was used to repair an imperforate anus, was first performed for a cloacal malformation.[1] It was ideally suited for the more complex repair of cloacae and led to the operation described as the posterior sagittal anorectovaginourethroplasty (PSARVUP). This approach allowed for direct exposure to the complex anatomy and an excellent visualization of the voluntary muscles of urinary and fecal continence.

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Pathophysiology

Genitourinary defects

More than 80% of all patients with a cloaca experience an associated urogenital anomaly.

  • Persistent cloaca - 90%
  • Rectobladderneck fistula - 84%
  • Rectoprostatic urethral fistula - 63%
  • Rectovestibular fistula - 47%
  • Rectobulbar urethral fistula - 46%
  • Rectoperineal fistula - 26%
  • Anorectal malformation with no fistula - 31%

All patients must be evaluated at birth for an associated urologic problems; the most valuable screening test is abdominal and pelvic ultrasonography.

Urologic evaluation, either by a pediatric surgeon experienced with pediatric urology or in consultation with a pediatric urologist, is necessary before proceeding with colostomy. This provides the surgeon the necessary information needed to address the urologic problem at the time of the colostomy. In addition, drainage of a distended vagina (hydrocolpos) may be required.

Tethered cord

A tethered spinal cord refers to an intravertebral fixation of the phylum terminale.

Tethered cord has a known association with anorectal malformations and is particularly common in patients with persistent cloaca.

Motor and sensory disturbances of the lower extremities may result.

Patients with anorectal malformations and tethered cord have a worse functional prognosis regarding bowel and urinary function. However, they also have higher anorectal defects, less-developed sacra, associated spinal problems, and less-developed perineal musculature; thus, the actual impact of tethered cord alone on the functional prognosis is unclear.

Untethering of the cord to avoid motor and sensory problems is indicated in the neurosurgical literature. No definitive evidence suggests that this operation affects the functional prognosis of a patient with anorectal malformation. Some evidence in the urologic literature indicates that this operation may improve urodynamics.

Anomalies of the sacrum, particularly hypodevelopment, sacral hemivertebrae, and hemisacra, are associated with tethered cord and most likely comprise the spectrum of caudal regression, of which spinal anomalies are a part.

Spinal ultrasonography in the first 3 months of life and MRI thereafter are useful in diagnosis.

Sacrum and spine anomalies

The sacrum is the most frequently affected bony structure. Traditionally, to evaluate the degree of sacral deficiency, the number of sacral vertebral bodies was counted. A more objective assessment of the sacrum can be obtained by calculating a sacral ratio. The sacrum is measured, and its length is compared with bony parameters of the pelvis (see the image below).

Calculation of sacral ratio. Calculation of sacral ratio.

The distance from the coccyx to the sacroiliac joint divided by the distance from the sacroiliac joint to the top of the pelvis provides the sacral ratio. Lateral radiography is more accurate than the anteroposterior view because its calculation is not affected by the tilt of the pelvis.

The assessment of the hypodevelopment of the sacrum correlates with the patient's functional prognosis. Normal sacra have a ratio of greater than 0.7. Bowel control has rarely been observed in patients with ratios less than 0.3.

A hemisacrum is almost always associated with a presacral mass, commonly teratomas, or anterior meningoceles. The Currarino triad, which includes an anorectal malformation, a hemisacrum, and a presacral mass, has been described.

Hemivertebrae may also affect the lumbar and thoracic spine, leading to scoliosis.

Patients may have spinal anomalies other than tethered cord, such as syringomyelia and myelomeningocele.

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Epidemiology

The literature reports that the incidence rate of cloacal malformations is approximately 1 per 20,000-25,000 live births. However, the literature often contains reports of rectovaginal fistula, which are most likely cloacal malformations in which the rectal problem was corrected but the urogenital sinus was not. Therefore, cloacal malformations are probably more common than previously thought.

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Prognosis

Functional prognosis with regard to achieving fecal continence depends on the complexity of the defect and the status of the spine and sacrum.

Fecal continence is more likely in patients with a benign cloaca and in those who have a normal sacrum. Approximately 70% of patients can achieve voluntary movements (for comparison with patients with other anorectal anomalies, see Imperforate Anus).

Postoperative monitoring of bowel function is similar to that in patients with imperforate anus (see Imperforate Anus: Surgical Perspective).

Urinary control varies based on the length of the common channel (see the image below).

Repair of persistent cloaca with 4-cm common chann Repair of persistent cloaca with 4-cm common channel.

Intermittent catheterization is required in 69% of patients with persistent cloaca who have a common channel longer than 3 cm, compared with 20% in the group with a common channel shorter than 3 cm.

In most patients, the bladderneck is competent, and the patients who require catheterization remain dry between voids. If catheterization is not performed, overflow incontinence occurs. Occasionally, the bladderneck is not competent or is nonexistent; in these patients, urinary diversion, such as a Mitrofanoff procedure, with bladder neck tightening may be needed.

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Contributor Information and Disclosures
Author

Marc A Levitt, MD Surgical Director, Center for Colorectal and Pelvic Reconstruction, Department of Pediatric Surgery, Nationwide Children’s Hospital

Marc A Levitt, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Society of American Gastrointestinal and Endoscopic Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Alberto Pena, MD Founding Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children’s Hospital Medical Center

Alberto Pena, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, American Society of Colon and Rectal Surgeons, Royal College of Surgeons of England, Pacific Association of Pediatric Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Gail E Besner, MD Chief, Department of Pediatric Surgery, Principal Investigator, Center for Perinatal Research, Director, Pediatric Surgery Training Program, Associate Burn Director, Nationwide Children’s Hospital; H William Clatworthy, Jr, Professor of Surgery, Department of Surgery, Ohio State University College of Medicine

Gail E Besner, MD is a member of the following medical societies: American Surgical Association, Alpha Omega Alpha, American Academy of Pediatrics, American Burn Association, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Medical Womens Association, American Pediatric Surgical Association, Association for Academic Surgery, Federation of American Societies for Experimental Biology, Society of Critical Care Medicine, Society of Surgical Oncology, Society of University Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Eugene S Kim, MD, FACS, FAAP Associate Professor of Surgery, Division of Pediatric Surgery, Keck School of Medicine of the University of Southern California; Attending Pediatric Surgeon, Children's Hospital Los Angeles

Eugene S Kim, MD, FACS, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, Association for Academic Surgery, Society of Laparoendoscopic Surgeons, Society of University Surgeons, Texas Medical Association, Children's Oncology Group

Disclosure: Nothing to disclose.

Additional Contributors

Aviva L Katz, MD Assistant Professor of Surgery, University of Pittsburgh School of Medicine; Consulting Staff, Division of General and Thoracic Surgery, Children's Hospital of Pittsburgh

Aviva L Katz, MD is a member of the following medical societies: American Academy of Pediatrics, Association of Women Surgeons, American College of Surgeons, American Pediatric Surgical Association, Physicians for Social Responsibility, Wilderness Medical Society

Disclosure: Nothing to disclose.

References
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Calculation of sacral ratio.
Newborn with persistent cloaca and massive hydrocolpos.
Ultrasonogram showing hydronephrosis before and after drainage of hydrocolpos, which was compressing trigone of bladder.
Hysterosalpingogram in baby who has persistent cloaca with symmetric hemiuteri.
Perineum of baby with persistent cloaca.
Perineum of baby with persistent cloaca.
Perineum of baby with persistent cloaca. Note single perineal orifice.
Perineum of baby with persistent cloaca showing common finding of small external genitalia.
Artist's drawing of persistent cloaca, lateral view.
Posterior sagittal approach to repair cloacal malformation, showing rectum, vagina, and urethra.
Total urogenital mobilization during repair of persistent cloaca.
Repair of persistent cloaca with 4-cm common channel.
MRI of teenage girl born with persistent cloaca, with pelvic masses caused by metrocolpos and metrometra.
Artist's drawing of retrograde menstruation due to left atretic hemicervix in patient with persistent cloaca and hemiuteri.
Artist's view of laparotomy in patient with menstrual blood–filled anomalous müllerian structures.
Cross-lateral radiographic fluoroscopic image of cloaca (cloacagram). Hydrosoluble contrast is injected via cloaca into orifices of the bladder (solid black arrow), vagina (white arrow), and rectum (dashed black arrow) to demonstrate anatomic relation of these organs.
 
 
 
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