Continence management in children with severe caudal regression syndrome: role of multidisciplinary team and long-term follow-up

Caudal Regression Syndrome—A Narrative Review: An Orthopedic Point of View

“…In less severe sacrococcygeal agenesis, the diagnosis may be made only in children who are a few years old [ 11 ]. Nevertheless, early diagnosis is crucial due to the risk of neuropathic bladder [ 3 , 11 ]. Delayed diagnosis may lead to an increased risk of recurrent urinary tract infections, incontinence and even renal impairment.…”

“…Esposito recommended a special diagnostic protocol for all newborns at increased risk of urologic anomalies. It consists of performing an ultrasound examination in all patients from this group and, if any abnormalities are detected, conducting a urodynamic or videourodynamic study [ 3 ]. Gastrointestinal anomalies can lead to problems with bowel movements and control (incontinence and encopresis).…”

“…Gastrointestinal anomalies can lead to problems with bowel movements and control (incontinence and encopresis). Fecal incontinence, according to some studies, concerns 1/3 of patients, and imperforate anus is a significant risk factor [ 3 , 51 ].…”

“…The extent of the defect may vary from partial to complete absence of the sacrum and/or distal lumbar vertebrae. The CRT is also known as caudal dysplasia sequence, sacral regression syndrome, and a large number of authors refer to it as sacral agenesis [ 3 ].…”

“…Caudal regression syndrome is a rare disorder, with an incidence of about 1–2:100,000 according to some authors, or 1:60,000 live births according to others [ 3 , 6 , 9 , 10 ]. This defect is more common in boys, with an M:F ratio of 2.7:1, but in a recent review of 83 cases, there was no gender predilection [ 3 , 10 ]. The most common CRS defect is sacral agenesis (partial or total), with an incidence of less than 0.5% [ 11 ].…”