2003 Klippel-Feil Syndrome Plus

Description: H. Foyaca-Sibat, L de F Ibañez-Valdés: Klippel-Feil Syndrome "Plus". The Internet Journal of Internal Medicine. 2003. Volume 4 Number 1.
We report a case with Klippel-Feil (KFS) syndrome associated with hypertelorism, microtia, Sprengel deformity, hand and feet deformities, scoliosis, clinical manifestations of Klippel-Trenaunay syndrome, other anomalies, and severe arterial hypertension secondary to renal artery stenosis. This patient underwent for surgical revascularization unsuccessfully, we have hypothesized that for patients with KFS and unilateral renal artery stenosis medical treatment with ACE inhibitors can provide more benefits than surgery or percutaneous transluminal angioplasty. In order to get better results in the management of this patients all underlying problems should be proper identified before send patient for any kind of surgery, it is important to bring this problems to the anesthesiologist's attention for a very careful manipulation of the neck and head during induction of anesthesia. The final results will be strongly related with the capacity of management of the underlying cardio-respiratory, renal, and nervous system problems but the family physician should decide in which order of priority surgical or medical intervention should be done. We propose the term of Klippel-Feil syndrome "Plus" for those patients with cervical vertebral fusion and many other associated deformities rather than to add another eponym to the long lists that already exist. From our knowledge this is novel combination no previously reported to the medical literature