Lumbo-sacral agenesis. Clinical analysis and treatment relating to 4 observations.
Lumbo-sacral agenesis fall within the framework of numerical anomalies by default of the vertebral column. They are characterized by a more or less significant absence of vertebrae of the sacral, lumbar and, less frequently, thoracic regions. They are grouped in Foix and Hilleman's "crural-vesical-gluteal dystrophy syndrome". It was Ranshaw and Stanley who first proposed a classification of sacral and lumbo-sacral agenesis into 3 groups. Being difficult to refer strictly to such a classification, the Authors divide their series of 56 cases of sacral agenesis into 3 groups, putting total sacral agenesis with different degrees of lumbar and thoracic agenesis into the first group and describing the treatment of 4 cases included in this group. The follow-up of the mentioned cases leads to the following considerations: 1. From the embryonal point of view, this pathology comes within the "caudal regression syndrome". Such regression, as first referred by Duhamel, results excessive in these cases. 2. Maternal diabetes seems to be the dominant etiology. 3. Accurate clinical and X Ray examination must be carried out before establishing the therapeutic iter, as prognosis depends on the instability of the rachis, the mobility of the hips as well as on the flexibility of the knees. 4. In view of improving functional possibilities and giving the patient maximum autonomy, both in sitting and standing positions, two types of interventions are envisaged: fixation of the spine correction of the lower limbs.
Gregoire
A
A
Service de Chirurgie Orthopédique-Traumatologique, Chirurgie Pédiatrique, Hopital d'Aubagne, 13677 Aubagne, Marseille, France.
Zerdani
S
S
D000015:Abnormalities, Multiple / Q000601:surgery*; D002648:Child; D006801:Humans; D008159:Lumbar Vertebrae / Q000002:abnormalities* / Q000601:surgery*; D012447:Sacrum / Q000002:abnormalities* / Q000601:surgery*