VACTERL
Information
VACTERL association is a “syndrome-like” association between many frequently occurring multi-system anomalies. VACTERL stands for Vertebral, Anal, Cardiac, Tracheal, Esophageal, Renal and Limb. It occurs in 1 in 10,000 to 1 in 40,000 live births.
Many patients have at least three of the above system associations.
Vertebral anomalies predominate, (involving 60-80% of patients), and may include fusion of vertebrae, missing (atresia) vertebrae, or even extra vertebral bodies. Surgery may be required to correct severe angular spinal deformities and/or severe spinal stenosis. Similarly, Anal atresia is frequently present, requiring immediate surgical intervention shortly after birth; this is also frequently associated with other genito-urinary tract anomalies.
The Most commonly associated cardiac defects include atrial-septal and ventriculo-septal defects (ASD/VSD), Tetralogy of Fallot; less likely but reported are Truncus Arteriosus and Transposition of the Great Vessels (TGV). Tracheal-esophageal fistulas are frequently present, requiring surgical attention early in life to correct.
Renal abnormalities include missing or mis-shaped kidneys and/or severe vesiculo-urethral reflux.
Treatment Strategies
The most frequently associated orthopedic abnormalities involve the upper limbs and/or thumbs; Radial club hand/Radial agenesis are the conditions Dr.Paley frequently addresses in the VACTERL patient. Treatment strategies may incorporate re-orienting the wrist-hand angle (“ulnarization”), repositioning a finger to the position of a functioning thumb (“pollicization”), and, in the future, the possibility of forearm lengthening. For more information on these procedures, see Radial Club Hand.
Occasionally, lower limb deformities are also present, such as congenitally flexed knees (bony or soft tissue) and club foot (talipes). These issues are addressed on a case-to-case basis and may include correction with open surgeries and/or application of external fixators. Serial casting utilizing the techniques of Ignasio Ponseti are offered by our highly trained physical therapy team to address club foot pathology, if present. For more information on the Ponseti Method, see Clubfoot.
During the orthopedic treatment phase, we also keep a close watch on the child’s additional body system issues, and with the multi-disciplinary specialist available on-site at St. Mary’s hospital, can assure a high degree of safety. On site there is available pediatric cardiac surgery, intervention cardiac catheterization, a dedicated Pediatric Intensive Care Unit (PICU) team staffed with board-certified intensivists, pediatric ear, nose throat specialists, pediatric endocrinologists and general surgeons, and pulmonologists, amongst others. In the operating room, board-certified pediatric anesthesiologists attend our surgeries.
