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Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_II | Pages 132 - 132
1 Feb 2004
Martín RT Cimarra-Díaz A Fernández-Doral J Sardá-Gascòn J Jiménez-González ML García-Sorando R
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Introduction and Objectives: This rare disease (17 per million newborns) was described by Chatelaine in 1882 and is more prevalent in females compared to males (2.3:1). It is commonly accompanied by congenital or genetic malformations. Within the syndrome known as genu recurvatum, congenital knee dislocation (CKD) is the most extreme clinical form. In view of the scarcity of trials and reviews on the subject of CKD and results of therapy for the same, we determined to gather clinical case data from the hospital from 01–01-1964 to 01–01-2003 and evaluate therapeutic, orthopaedic, and surgical experience.

Materials and Methods: Of 28,753 patients treated in the aforementioned period, 55 were treated for genu recurvatum. We selected 22 with unilateral or bilateral CKD. We individualised the cases of 14 patients with 20 knees as patients diagnosed and treated since birth by this centre, excluding those with multi-malformation genetic syndromes (Larsen’s syndrome, arthrogryposis, etc.). In terms of treatment, 13 knees were treated orthopaedically by means of successive manipulations and splinting until 90° of flexion was achieved. At that point, physical therapy was initiated. There were 7 other knees that received at least one surgical treatment when results of the previously-described orthopaedic procedures were unsatisfactory. The clinical outcome for the treatment method used in each case was assessed by means of evaluation of range of mobility of the knee joint, stability upon examination, residual deviation from the axes, and ability of the patient to walk at time of release.

Results: Of the 65% of knees that were treated exclusively by orthopaedic methods, we observed ranges of flexion and stability such that splinting was not needed at an average of 55.2 days, with good to excellent clinical outcomes at an average of 42.4 days. Of the 35% of knees that required at least one surgical procedure, the procedure was performed between the ages of 30 and 176 days of life, with a mean of 90.8 days. As many as 75% of these knees required repeated surgical intervention during the treatment period, and good to excellent results were achieved in only 35% by 5 years of age.

Discussion and Conclusions: Orthopaedic treatment continues to be the foundation of managing these patients, and plays a vital role even in cases were surgical intervention is chosen. The existence of other genetic or non-genetic developmental anomalies concurrent with CKD will determine the therapeutic strategy that is indicated and when such action should take place. We do not recommend surgical treatment except in cases of a continued lack of response to orthopaedic treatment.