the diagnosis of cloaca involves repairing only the rectal component of the anomaly, leaving the patient with a persistent urogenital sinus.
Associated spinal anomalies
The sacrum is the most frequently affected bony structure. Traditionally, to evaluate the degree of sacral deficiency, the number of sacral vertebral bodies were 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. The lateral film is more accurate than the anterior posterior view because its calculation is not affected by the tilt of the pelvis. A hemisacrum is always associated with a presacral mass, which is commonly formed of dermoids, teratomas, or anterior meningoceles. Hemivertebrae may also affect the lumbar and thoracic spine, leading to scoliosis.
Spinal anomalies including a tethered spinal cord can occur [11, 12]. This anomaly, which refers to the intravertebral fixation of the phylum terminale is known to occur in approximately 25% of patients. The prevalence of tethered spinal cord rises with increasing height and complexity of the anorectal anomaly. In addition, patients with a hypodeveloped sacrum and with associated urologic problems have a higher likelihood of having tethered cord. Motor and sensory disturbances of the lower extremities may result. Concerning bowel and urinary function, patients with anorectal malformations and tethered cord have a worse functional prognosis but they also have higher anorectal defects, less developed sacrums, associated spinal problems, and less developed perineal musculature. So, the actual impact of tethered cord itself on their functional prognosis is unclear. Untethering of the cord is indicated in the neurosurgical literature to avoid motor and sensory problems. There does not appear to be evidence that this operation will impact on the functional prognosis of a patient with anorectal malformation. Spinal ultrasound in the first 3 months of life and magnetic resonance imaging thereafter are useful radiologic modalities to establish the diagnosis. Furthermore, patients may have other spinal anomalies besides tethered cord such as syringomyelia and myelomeningocele.
Perineal fistulas in both male and female have traditionally been called "low" defects. In these cases the rectum opens in a small orifice, usually stenotic and located anterior to the center of the sphincter (Figure 3). Most of these patients have excellent sphincter mechanisms and a normal sacrum. In males, the perineum may exhibit other features that help in recognition of this defect, such as a prominent midline skin bridge (known as 'bucket handle') or a subepithelial midline raphe fistula that looks like a black ribbon because it is full of meconium. These features are externally visible and help diagnose a perineal fistula. A simple anoplasty enlarges the stenotic orifice and relocates the rectal orifice posteriorly within the limits of the sphincter complex. The operation is called a "minimal posterior sagittal anoplasty". It is performed with the patient positioned prone with the pelvis elevated; multiple fine silk sutures are places at the mucocutaneous junction of the bowel orifice for traction. A short (1–2 cm) midsagittal incision is made posterior to the fistula site, dividing the entire external sphincter complex. The fistula and lower part of the rectum are carefully dissected to permit mobilization of the rectum for backward placement within the limits of the sphincter complex. The perineal body, that area where the fistula was located, is repaired with a few long-term absorbable sutures .
Anorectal malformations (ARM) represent a spectrum of abnormalities ranging from mild anal anomalies to complex cloacal malformations. The etiology of such malformations remains unclear and is likely multifactorial. There are however reasons to believe there is a genetic componenet. As early as the 1950s, it was recognized that there was an increased risk for a sibling of a patient with ARM to be born with a 