Complete bilateral agenesis of the ilium in a 7-year-old ambulatory girl: case report.
METHODS
A 7-year-old ambulatory girl with complete bilateral agenesis of the ilium is reported.


OBJECTIVE
To document the clinical evolution and the spino-pelvic balance of a girl with bilateral agenesis of the ilium.


BACKGROUND
Scapuloiliac dysostosis is a rare focal skeletal dysplasia consisting in hypoplasia of ilium and scapula.


METHODS
A 7-year-old girl with a short stature was referred to our clinic at 35 months of age for an asymptomatic lumbar hyperlordosis. She presented a waddling gait, and normal range of motion of her upper and lower limbs. The neurologic examination was within normal limits.


RESULTS
Initial radiographs at 35 months of age showed complete bilateral agenesis of the ilium with preservation of the pubis, ischium, and sacrum, associated with a bilateral high hip dislocation. The lumbar lordosis and sacral slope were 89 degrees and 84 degrees, respectively. A radiographic skeletal survey also showed hypoplasia of both scapulae. At 7 years old, the girl was fully ambulatory, with a waddling gait and had no pain. The sacral slope and lumbar lordosis have increased to 97 degrees and 97 degrees, respectively. There was no evidence of spondylolisthesis. So far, the patient has required no treatment for her condition.


CONCLUSIONS
This article reports a unique case of scapuloiliac dysostosis with complete bilateral agenesis of the ilium in an ambulatory girl. This case indicates that a functional gait is possible with bilateral congenital absence of the ilium, provided that there is adequate muscular support and normal neurologic function.
Mac-Thiong
Jean-Marc
JM
Department of Surgery, Université de Montréal, Montréal, Québec, Canada. jmmt@justine.umontreal.ca
Leduc
Stéphane
S
Labelle
Hubert
H
D002648:Child; D004413:Dysostoses / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D005260:Female; D005684:Gait; D006621:Hip Joint / Q000002:abnormalities / Q000000981:diagnostic imaging; D006801:Humans; D007085:Ilium / Q000002:abnormalities* / Q000000981:diagnostic imaging; D008141:Lordosis / Q000000981:diagnostic imaging / Q000503:physiopathology; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D004856:Postural Balance; D011859:Radiography; D012540:Scapula / Q000002:abnormalities / Q000000981:diagnostic imaging; D016138:Walking