Orthopaedic aspects of prune belly syndrome

Prune belly syndrome is a relatively uncommon disorder that is characterized by intrauterine urinary obstruction associated with cryptorchisdism, oligohydramnios, and orthopaedic deformations. The oligohydramnios is believed to produce limited intrauterine space, which in turn leads to fetal compression and the resultant deformities. The deformities observed in our patients were developmental dislocation of the hip (DDH), clubfeet, metatarsus adductus, vertical talus, and congenital muscular torticollis. These deformations should be treated aggressively because children with prune belly syndrome may be expected to have a relatively normal life if their renal function is good. The one exception is that as infants these children are very susceptible to pulmonary infections because of their inability to cough. Therefore, treatment of the hip and other deformations should be delayed until the children are old enough to be able to clear pulmonary secretions easily.