Cloacal Anomalies

Fig. 55.1
Contrast study showing a classic cloaca where the rectum, vagina, and urethra join together and open in a single perineal opening
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Fig. 55.2
Diagrammatic representation of a cloaca showing the urethra, vagina, and rectum joining together in a common channel
  • The length of the common channel varies from 1 to 10 cm, with an average of approximately 3 cm (Fig. 55.3).
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    Fig. 55.3
    a and b Diagrammatic representation of cloaca. Note the length of the common channel which is variable, ranging from 1 to 10 cm but commonly around 3 cm
  • It is important to correctly diagnose persistent cloaca in the neonatal period because 90 % of babies with this malformation have an associated urologic problem, and 40 % have hydrocolpos .
  • About 30 % of these patients have a hydrocolpos. The hydrocolpos may produce two important complications:
    • It may compress the trigone of the bladder, producing ureterovesical obstruction, megaureter, and hydronephrosis.
    • The hydrocolpos if left undrained may become infected, leading to a pyocolpos .
  • Approximately 40 % of these patients have a double Mullerian system consisting of two hemiuteri and two hemivaginas. This septation disorder may be partial or total and symmetric or asymmetric.
  • The urinary tract and the distended vagina may both need to be managed within the newborn period to avoid serious complications.
  • The goals of treatment include:
    • Early diagnosis
    • Immediate neonatal management
    • An anatomic reconstruction to achieve bowel and urinary control, as well as normal sexual function

      Clinical Features

      • Persistent cloaca is a clinical diagnosis.
      • The presence of a single perineal orifice provides clinical evidence of persistent cloaca (Fig. 55.4).
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        Fig. 55.4
        Clinical photograph showing a single perineal opening in a patient with cloaca
      • The external genitalia often appear small.
      • Examination of the abdomen may reveal an abdominal mass (hydrocolpos).
      • The distended vagina is a common cause of an obstructed urinary tract because of its pressure on the trigone; therefore, once the vagina is decompressed, the urinary tract may no longer be obstructed. If the hydrocolpos is not drained during the newborn period, it can become infected forming pyocolpos .
      • A hemisacrum is almost always associated with a presacral mass, commonly teratomas, or anterior meningoceles.
      • The Currarino triad includes an anorectal malformation, a hemisacrum, and a presacral mass.

      Associated Anomalies

      • Associated defects are common with cloaca.
      • Most vital to recognize are the urological abnormalities.
      • More than 80 % of all patients with a cloaca have an associated urogenital anomaly.
      • These include absent kidney, vesicoureteral reflux, horseshoe kidney, ectopic ureters, double ureters, hydronephrosis, and megaureters as a result of vesicoureteral reflux or ureterovesical obstruction.
      • A tethered spinal cord: an intravertebral fixation of the phylum terminale.
      • Patients with anorectal malformations and tethered cord have a worse functional prognosis regarding bowel and urinary function.
      • Sacrum and spine anomalies. The sacrum is the most frequently affected bony structure. Anomalies of the sacrum include hypodevelopment, sacral hemivertebrae, and hemisacra.
      • Hemivertebrae may also affect the lumbar and thoracic spine, leading to scoliosis.
      • Patients with cloaca may have spinal anomalies other than tethered cord, such as syringomyelia and myelomeningocele.

      Investigations

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      Mar 8, 2017 | Posted by in PEDIATRICS | Comments Off on Cloacal Anomalies

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