The following features are observed with<unk> : * V - Vertebral anomalies * A - Anorectal malformations * C - Cardiovascular anomalies * T - Tracheoesophageal fistula * E - Esophageal atresia * R - Renal (Kidney) and/or radial anomalies * L - Limb defects Although it was not conclusive whether<unk>  should be defined by at least two or three component defects, it is typically defined by the presence of at least three of the above congenital malformations. Spine Vertebral anomalies, or defects of the spinal column, usually consist of small (hypoplastic) vertebrae or hemivertebra where only one half of the bone is formed. About 80 percent of patients with<unk>  will have vertebral anomalies. In early life these rarely cause any difficulties, although the presence of these defects on a chest x-ray may alert the physician to other defects associated with<unk> . Later in life these spinal column abnormalities may put the child at risk for developing scoliosis, or curvature of the spine. Anal defects Anal atresia or imperforate anus is seen in about 55 percent of patients with<unk> . These anomalies are usually noted at birth and often require surgery in the first days of life. Sometimes babies will require several surgeries to fully reconstruct the intestine and anal canal. Cardiac defects Up to 75 percent of patients with<unk>  have been reported to have congenital heart disease. The most common heart defects seen with<unk>  are ventricular septal defect (VSD), atrial septal defects and tetralogy of Fallot. Less common defects are truncus arteriosus and transposition of the great arteries. It is subsequently thought that cardiac defects should be considered an extension of<unk> . Trachea and esophagus Esophageal atresia with tracheoesophageal fistula (TE fistula) is seen in about 70 percent of patients with<unk> , although it can frequently occur as an isolated defect. 15 to 33 percent of patients with TE fistulas will also have congenital heart disease. However these babies usually have uncomplicated heart defects, like a ventricular septal defect, which may not require any surgery. Kidneys Kidney defects are seen in approximately 50 percent of patients with<unk> . In addition, up to 35 percent of patients with<unk>  have a single umbilical artery (there are usually two arteries and one vein) which is often associated with additional kidney or urologic problems. Renal abnormalities in<unk>  can be severe, with incomplete formation of one or both kidneys or urologic abnormalities such as obstruction of outflow of urine from the kidneys or severe reflux (backflow) of urine into the kidneys from the bladder. These problems can cause kidney failure early in life and may require kidney transplant. Many of these problems can be corrected surgically before any damage can occur. Limbs Limb defects occur in up to 70 percent of babies with<unk>  and include a displaced or hypoplastic thumb, extra digits (polydactyly), fusion of digits (syndactyly) and forearm defects such as radial aplasia. Babies with limb defects on both sides tend to have kidney or urologic defects on both sides, while babies with limb defects on only one side of the body tend to have kidney problems on that same side. Extension Features secondary to<unk>  components are frequent enough to be considered an extension of<unk> . These include: single umbilical artery, ambiguous genitalia, abdominal wall defects, diaphragmatic hernia, intestinal and respiratory anomalies, and oligohydramnios sequence defects. Cardiac defects are thought to fit in this category. Growth Many babies with<unk>  are born small and have difficulty with gaining weight. Babies with<unk> , however, do tend to have normal development and normal intelligence.
VACTERL association
DiGeorge syndrome
Independent SAGE
Head injury criterion
Disease cluster
MURCS association
Clichy criteria
Injured reserve list