|
Year |
2002 |
|---|---|
|
Question Number |
201 |
|
Category |
Pediatric Orthopaedics |
Question
A 1 week old infant has prune belly syndrome. The spine shows no clinical deformity. The Barlow test and Ortolani maneuver are inconclusive. Both feet exhibit typical rigid clubfoot deformities. Further evaluation should include:
1- flexion-extension radiographs of the cervical spine
2- an AP radiograph of the pelvis
3- bilateral hip ultrasonography
4- MRI of the entire body
5- Simulaterd WB radiographs of both feet
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Answer (click for answer)
Preferred Answer 3
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 these patients are 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.
Brinker et al managed forty children for prune-belly syndrome between 1979 and 1989, and twenty-five of them had musculoskeletal abnormalities. The musculoskeletal abnormalities were primary in twenty-two children, secondary to renal osteodystrophy in one, and both primary and secondary to renal osteodystrophy in two. Thirteen children had marked abnormality of the hip, and congenital dislocation was typically resistant to conventional treatment. Scoliosis was seen in seven patients. Pectus excavatum (a chest-wall deformity) was seen in eleven patients, including five of the six who had an idiopathic-like curve. Although prune-belly syndrome is uncommon, the diagnosis necessitates a thorough orthopaedic evaluation because of the high prevalence of associated musculoskeletal abnormalities.
Reference: Green NE, Lowery ER, Thomas R: Orthopaedic aspects of prune belly syndrome. J Pediatr Orthop 1993;13:496-501
Brinker MR, Palutsis RS, Sarwark JF: The orthopaedic manifestations of prunw-belly(Eagle-Barret) syndrome. JBJS Am 1995;77:251-257