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Caudal Regression Syndrome

Bladder Autoaugmentation and Mitrofanoff Appendicovesicostomy on a patient with Caudal Regression Syndrome: A Case Report.

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Caudal Regression Syndrome

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  1. Bladder Autoaugmentation and MitrofanoffAppendicovesicostomy on a patient with Caudal Regression Syndrome: A Case Report Alonzo, Ivan; Amaro, Alejandra; Amolenda, Patricia; Andal, Charlotte; Ang, Jessy; Ang, Joanne; Ang, Kevin; Aningalan, Arvin; Antonio, Abigaille; Aramburo, Jan; Arcilla, Martin; Argana, Desiree Consultant-in-charge: Dr. Bolong Resident-in-charge: Dr. Arcinas University of Santo Tomas Department of Surgery February 9, 2011

  2. Caudal Regression Syndrome • Occurs in one in 25,000 live births • Characterized by a series of congenital anomalies, including complete or partial agenesis of the sacrum and lumbar vertebrae associated with pelvic deformity • Commonly seen are femoral hypoplasia, clubbed feet, and flexion contractures of the lower extremities • CRS is also associated with presence of two umbilical arteries, non-lethal renal anomalies, non-fused lower limbs, abdominal wall defects, and abnormalities of tracheoesophageal tree, neural tube and heart • Patients with CRS lack motor function below the level of the remaining normal spine but sensation tends to be present at much more caudal levels

  3. Caudal Regression Syndrome • The cause is little understood • CRS is strongly associated with maternal diabetes mellitus. Infants of diabetic mothers have two to three times the average incidence of congenital anomalies

  4. MitrofanoffAppendicovesicostomy • Provides an alternative means to access the bladder • It uses the appendix to create a conduit to the bladder, through which patients with a sensitive, absent, or traumatized urethra can perform clean intermittent catheterization easily • It is typically performed when there is blockage of the urethral canal or when there is need for frequent catheterization as in neurogenic bladder

  5. MitrofanoffAppendicovesicostomy • The procedure involves separating the appendix from the cecum • One end of the appendix is connected to the urinary bladder and the other end is connected to skin forming a stoma • An incision is made in the umbilicus to serve as the canal for the catheter

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