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      Transiliac lengthening with posterior lumbar-iliac percutaneous fusion in sacral hemiagenesis

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          Abstract

          Sacral agenesis is a term that applies to a wide range of developmental disorders of the lower portions of the spine and pelvis. Hemisacrum patients with all sacral segments present on one side of the spine, and decompensated lumbar rotoscoliosis, whit instability torac-pelvic that had transiliac lengthening of the lower extremity, accomplished by an innominate osteotomy with interposition of a rectangular iliac-bone graft in the osteotomy site, besides a posterior lumbar-iliac percutaneous fusion. We reported 5 adolescent patients, 2 men and 3 women, treated from 2000 to 2009, associated with average pelvic imbalance of 3.2 cm (2.5–4.5 cm) without other associated congenital anomalies. Patients classified as Vergara (Acta Ortop Mex 19:6–12, 2005) type IB unilateral partial agenesis of the sacrum, asymmetry of the pelvic ring there’s a torac-pelvic cifoscoliotic deformity. Mean age was 12.2 years-old (range from 8.2 to 13.7). The mean follow-up was 7.2 years (from 2 to 8). The consolidation process of the osteotomy site was in an average of 6.4 (5–8.7 weeks) ( P = 0.036). None of the patients presented family medical history of diabetes on their mothers. None residual femoral nerve palsy. The procedure offers postural correction at the level of the pelvis, low morbidity and no additional operations were required to achieve the surgical objective. Level of evidence Level IV, therapeutic study: Case series (no, or historical, control group), Prospective: The study was started before the first patient was enrolled.

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          Autosomal dominant sacral agenesis: Currarino syndrome.

          Autosomal dominant sacral agenesis is characterised by a partial agenesis of the sacrum typically involving sacral vertebrae S2-S5 only. Associated features include anorectal malformation, a presacral mass, and urogenital malformation. Together, these features have been defined as the Currarino syndrome. Recently, HLXB9 has been identified as the major causative gene in Currarino syndrome allowing identification of asymptomatic heterozygotes. In this review, we have performed an analysis of medical publications, and our own additional cases, to identify the range of malformations and complications that occur. We have also estimated risks of malformation in heterozygotes by using Weinburg's proband method on families personally known to us in order to provide accurate genetic counselling information.
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            Involvement of the HLXB9 homeobox gene in Currarino syndrome.

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              Congenital agenesis of pubic bones: a case report.

              Congenital absence of bilateral pubic bones is extremely uncommon. A 2-year-old boy presented with a history of intoeing at our outpatient clinic. The testes were found located at the inguinal canal on ultrasonography. A pelvic radiograph and a spiral computed tomography scan of the pelvis with three-dimensional reconstruction revealed the absence of both pubic bones. In conclusion, we report that the absence of the pubic bones, either as an isolated finding or associated with undescended testes, is peculiar.
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                Author and article information

                Contributors
                +57-314-2448344 , +57-300-2597226 , pedroantonio.sanchezmesa@gmail.com , pasm@cable.net.co , pasm70@hotmail.com , pasm@clubcadera.com , www.clubcaderayrodilla.com , www.unidadortopedia.com
                Journal
                Strategies Trauma Limb Reconstr
                Strategies in Trauma and Limb Reconstruction
                Springer Milan (Milan )
                1828-8936
                1828-8928
                22 July 2011
                22 July 2011
                August 2011
                : 6
                : 2
                : 77-81
                Affiliations
                Specialized Unit in Orthopedics and Traumatology S.A.S Club Hip & Knee, Bogotá, DC Colombia
                Article
                109
                10.1007/s11751-011-0109-0
                3150650
                21785911
                5d2dbd18-a061-4adf-8c6e-7d9f2cbd257f
                © The Author(s) 2011
                History
                : 9 November 2009
                : 4 July 2011
                Categories
                Original Article
                Custom metadata
                © The Author(s) 2011

                Emergency medicine & Trauma
                hemiagenesis,rotoscoliosis,hemisacrum,pelvis,postural imbalance
                Emergency medicine & Trauma
                hemiagenesis, rotoscoliosis, hemisacrum, pelvis, postural imbalance

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