Diagnostic Management

Endoscopy includes a detailed investigation of the length of the UGS and the situation of the vagina(s), rectal fistula, the bladder neck, and urethral orifices. In addition, a distal loopogram is essential later, before definitive surgery. Without endoscopic aid it is usually not possible to catheterize the bladder because the fusion of the proximal urethra with the UGS is almost always sharply angulated in the direction of the pubic bone. In this situation a Tieman catheter may be helpful. The endoscopic situation should be demonstrated to the parents to allow later intermittent catheterization by the mother until the final reconstruction is performed. In many cases, however, the urine is passed by the baby first into the vagina before being evacuated through the common channel. In these patients it is sufficient to perform intermittent catheterization of the vagina. Only very few children with a cloacal anomaly need a persistent vesicostomy or vaginostomy.

It is essential to perform the x-ray studies in a strict sagittal position, the legs elevated and the anal

Cloacal Anomaly
Fig. 10.5 Sagittal x-rays of cloacal malformations. A Low type: short UGS, one vagina, but higher confluence of the rectum. B High type: long UGS, one vagina visible. Note the feeding tubes in all cavities after endoscopy

dimple marked with contrast material. The complete lumbosacral spine should be visible (Figs. 10.5 and 10.6). There is a clear correlation between the degree

Vaginostomy
Fig. 10.6 Radiographic imaging of a child with a high-type cloaca: high UGS, double vagina (VAG), rectum (RECT) with fecal material A Anterior-posterior (ap) view. B Sagittal view. BLAD Bladder

of vertebral and sacral malformations and the occurrence of neurogenic voiding and defecation disorders. A complete absence of S2-S4 leads to a lower motor neuron lesion and presents clinically as continually dribbling bladder. A disruption or severe deformity of higher lumbar neurons with intact sacral reflex activity results in an autonomic or reflex bladder, leading to an upper motor neuron lesion [28,29]. However, the majority of the patients suffer from a mixed motor neuron lesion with a varying amount of residual urine and uninhibited detrusor contractions.

Preoperative ultrasound may reveal tethered cord syndrome, sometimes associated with lipoma of the spinal canal or diastematomyelia. However, a tethered cord should be treated only when neurological problems start to arise. Many of the patients who have had detethering have experienced a retethering postoperatively due to postoperative scarring. Therefore, repeated neurological reviews by an experienced neurologist are necessary before untethering. On the other hand, it is true that once a deficit is established, it is unlikely to be improved after detethering the cord. The best time to operate on a tethered cord therefore remains an open question.

During endoscopy fecal material should be washed out from the rectal pouch using physiologic saline solution. It is sometimes difficult to clean the rectum from the colostomy side even if a colostomy with two separate orifices has been established.

A detailed description of the definitive treatment of cloacal deformities is given in Chaps. 21 and 22 by Levitt and Peña.

Essentials of Human Physiology

Essentials of Human Physiology

This ebook provides an introductory explanation of the workings of the human body, with an effort to draw connections between the body systems and explain their interdependencies. A framework for the book is homeostasis and how the body maintains balance within each system. This is intended as a first introduction to physiology for a college-level course.

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