Klippel-Feil Syndrome (KFS) is a complex congenital syndrome of osseous and visceral anomalies. It is mainly associated with multi-level cervical spine fusion with hypermobile normal segments. Therefore, a patient with KFS can be at risk of severe neurological symptoms even after a minor trauma. We report a patient with type III KFS who developed a hemiparesis after a minor trauma and was successfully managed with operation.
Humans ; Klippel-Feil Syndrome* ; Paresis* ; Spine

No abstract available.
Humans ; Intubation* ; Klippel-Feil Syndrome* ; Laryngoscopes*

Klippel-Feil Syndrome (KFS) continues to pose significant challenges for anesthesiologists. Beyond the expected complexities of managing difficult airways in these patients, they often present with systemic anomalies that can elevate the risk of morbidity during surgeries conducted under anesthesia. Furthermore, laparoscopic procedures bring about additional physiologic changes that must be taken into consideration when planning the anesthetic care for these individuals. This report details the anesthetic management of a 29-year-old female diagnosed with Klippel-Feil Syndrome (KFS) and concomitant Müllerian duct aplasia-Renal agenesis-Cervicothoracic Somite dysplasia (MURCS) as well as Chiari Type 1 Malformation, who underwent a successful pelvic laparoscopic surgery. The airway was secured through awake fiberoptic-guided intubation while general anesthesia was maintained with a combination of sevoflurane inhalation and remifentanil infusion. Intraoperatively, the team prioritized neuroprotection, lung-protective ventilation strategies, and renal preservation measures. The anesthetic management of patients with KFS necessitates a comprehensive assessment of their anomalies. Incorporating these considerations into the anesthetic management will help mitigate the procedure's adverse effects and lead to favorable patient outcomes.
Anesthesia ; Airway Management ; Klippel-Feil Syndrome ; Laparoscopy

Patients with Klippel-Feil syndrome (KFS) have an increased incidence of vascular anomalies as well as vertebral artery (VA) anomalies. In this article, we presented imaging findings of a 15-year-old female patient with KFS with a rare association of extraforaminal cranially ascending right VA that originated from the ipsilateral carotid bulb. Trifurcation of the carotid bulb with VA is a very unusual variation and to the best of our knowledge, right-sided one has not been reported in the literature.
Adolescent ; Female ; Humans ; Incidence ; Klippel-Feil Syndrome* ; Vertebral Artery*

Hand Deformities ; pathology ; Humans ; Klippel-Feil Syndrome ; pathology ; Thumb

No abstract available.
Child* ; Humans ; Klippel-Feil Syndrome* ; Magnetic Resonance Imaging*

Sprengel's deformity is characterized by the congenital migration of the scapula superiorly in relation to the thoracic cage. Other congenital anomalies, such as Klippel-Feil syndrome, may occur in combination with Sprengel's deformity. We report on a case of Sprengel's deformity with a huge bilateral omovertebrae, which was combined with the clinical features of Klippel-Feil syndrome, including cervical fusion, short neck, low posterior hairline, and limitation of neck motion. However, no other deformities or functional defects were observed.
Congenital Abnormalities ; Klippel-Feil Syndrome ; Neck ; Scapula ; Shoulder Joint

Klippel-Feil syndrome is characterized by congenital fusion of cervical vertebrae with a wide range of associated anomaly. The authors present a 50-year-old Klippel-Feil syndrome patient with a minor trauma followed progressive quadriparesis. He had typical radiologic findings of type II Klippel-Feil syndrome and presented progressive myelopathy due to cord compression at foramen magnum level with cervical instability. The patient underwent craniocervical decompression and fusion. The authors reviewed the pertinent literatures and discussed this rare syndrome.
Cervical Vertebrae ; Decompression ; Female ; Foramen Magnum ; Humans ; Klippel-Feil Syndrome* ; Middle Aged ; Quadriplegia* ; Spinal Cord Diseases

Mirror movements(MMs) are involuntary movements executed on one side of the body during voluntary movements of the contralateral homologous body parts. Motor evoked potentials(MEP) to focal transcranial magnetic stimulation(TMS) have postulated that abnormal ipsilateral corticospinal tract is active in the patient with congenital MMs. MEP and post-MEP SP following to focal TMS were investigated in a patient with the Klippel-Feil syndrome(KFS) showing MMs and in five normal volunteers. In the patient unilateral transcranial stimulation evoked bilateral motor responses of normal latencies and SP was observed bilaterally, which tended to be shortened in duration. In the condition of KFS, the mechanism of MMs has been attributed to the presence of abnormally activated ipsilateral corticospinal tract, but it does not suffice for explaining the shortened SP. We propose that concurrent activation of both motor cortices be responsible for the mechanism of MMs in this case of KFS, in addition to the abnormality of the corticospinal tracts.
Dyskinesias ; Evoked Potentials, Motor* ; Healthy Volunteers ; Human Body ; Humans ; Klippel-Feil Syndrome* ; Pyramidal Tracts

Klippel-Feil syndrome is characterized by congenital fusion of cervical vertebra which shows classic triad of short neck, low posterior hairline and limited range of motion of the neck. We report a case of Klippel-Feil syndrome not only associated with renal hypoplasia and sensorineural hearing defect but also with repetitive severe hypoglycemia with lactic acidosis.
Acidosis, Lactic ; Hearing ; Hypoglycemia* ; Klippel-Feil Syndrome* ; Neck ; Range of Motion, Articular ; Spine