How is WAGR syndrome treated?
WAGR syndrome treatment is determined by its symptoms.
Doctors in our Department of Pediatric Surgery and our Urologic Tumor Program are specially trained in treating Wilms tumors as well as other kidney tumors. In WAGR syndrome, we recommend having your child's kidneys checked every 3 to 4 months until about the age of 8. Using ultrasound imaging to scan the kidneys can help your child’s doctor catch a Wilms tumor early, which greatly increases the chances of being able to cure it.
We continue to monitor the kidneys for signs of nephropathy, which is a disease caused by damage to the blood vessels that help the kidneys clean the blood. About 40% of children with WAGR syndrome develop late-onset nephropathy over the age of 12.
If we detect a developing Wilms tumor, we usually start treatment with a low dosage of chemotherapy to slow the tumor’s growth. This treatment is followed by a partial nephrectomy in which we remove the affected part of the kidney, taking care to save as much of the kidney as possible.
Aniridia causes the absence of the iris and can lead to lowered visual acuity and photophobia. Other eye problems such as cataracts, glaucoma and nystagmus can also develop due to aniridia. Our Department of Ophthalmology has vast experience treating these conditions. Treatment can range from eye drops to medication and surgery, depending on the severity of each condition, which can vary widely.
We see abnormalities of the genitalia and urinary tract more frequently in boys with WAGR syndrome than in girls. The most common anomaly in boys with WAGR is undescended testicles (cryptorchidism). If a testicle can be felt within the groin, your child may undergo a surgical procedure called an orchiopexy. For the procedure, the surgeon first makes a small incision in the groin so the testicle can be located, then makes a second incision at the base of the scrotum to place the testicle to prevent it from twisting or moving upward.
Girls with WAGR syndrome can form streak gonads, which are underdeveloped ovaries that usually do not function. They can also have a heart-shaped uterus known as a bicornuate uterus, which makes it difficult to carry a pregnancy to term. Bicornuate uterus also falls under the category of a Müllerian anomaly, which our Department of Pediatric and Adolescent Genecology has extensive experience treating. Our pediatric gynecologists provide counseling for bicornuate uterus about future reproductive risks, including the risk for premature delivery, which requires additional monitoring by an obstetrician during pregnancy.
Range of mental disabilities
WAGR syndrome can lead to a range of intellectual disabilities that cause developmental delay. Psychiatric or behavioral issues can include depression, anxiety, attention-deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD) or a developmental disorder called autism spectrum disorder that affects communication and social interaction.
Our Pediatric Mental Health Institute treats these conditions on a case-by-case basis, taking into account the combination of complications your child may have. They also work with each department to understand what medical conditions your child is facing so they can provide psychological support as your child undergoes treatment.
Why choose us for treatment of WAGR syndrome?
At Children's Colorado, we have specialists that expertly address every aspect of WAGR syndrome. From our Surgical Oncology Program to diagnose, monitor and treat WAGR syndrome-related tumors, to our ophthalmology and psychiatry departments, we work in close collaboration to care for every one of your child’s needs. Having a multidisciplinary team in one location also enables us to treat this complex condition while easing the burden on you and your child.