| Abstrakt: |
Background: Torg winchester syndrome; Numerous, painless, subcutaneous nodules are seen, usually limited to the hands and feet. Radiographically, it is accompanied by characteristic enlargement of the osteolyzed metacarpal and metatarsal bones. The resulting bone loss causes pain, pathological fractures and limitation of movement. Bone and joint manifestations characteristically begin in the hands and feet, then spread to larger joints. It is accompanied by hypertrichosis. It can develop a white or transparent appearance in the eyes that covers the cornea. It is stated that Winchester syndrome is inherited by autosomal recessive inheritance. The protein inactivation mutation is found in the matrix metalloproteinase 2 gene (mmp2). MM2 is responsible for bone remodeling. This mutation causes a multicentric syndrome of osteolysis and arthritis. A complete skeletal radiographic examination is mandatory for the diagnosis of Winchester syndrome, along with a detailed musculoskeletal examination and assessment of craniofacial morphology. It is more common in women than men. There are only a few people worldwide who are reported to have this disorder. Case: A 25-year-old male patient with Winchester syndrome was taken to the operating room for cholecystectomy. He had an ASD repair operation, motor dysfunction in his fingers and toes. His pulmonary, neurological and abdominal examinations were unremarkable. It was learned that his older brother also had the same genetic disorder. On physical examination: height 90 cm, weight 45 kg, pulse rate 120/min, and blood pressure 120/76 mmHg On airway examination, temporomandibular (TM) joint mobility was normal, a Mallampati II with a distance between incisors of 4 cm and a thyromental distance of 6 cm. The patient was intubated by administering 1 mg midazolam 50 mcg fentanyl 40 mg lidocaine 100 mg propofol 30 mg rocuronium. Anesthesia was maintained with sevoflurane with a MAC of 1.3. There was no intraoperative or postoperative problem in our patient. 1000 cc of saline was administered intraoperatively and the procedure lasted for 2 hours. Conclusion: When the literature is examined, the problems that may be encountered are as follows: Thick skin, vertebral anomalies, decreased lung capacities, cardiac disease, difficult airway, limitation of movement in the atlanto-axial joint. [ABSTRACT FROM AUTHOR] |
