Management of Anesthesia in Biotinidase Deficiency for Cataract Surgery
Ökkeş Hakan Mi̇ni̇ksar1, Erol Toy2, Ahmet Selim Özkan2
1Clinic of Anesthesiology, Malatya Training and Research Hospital, Malatya, Turkey
2Department of Anesthesiology, Inonu University Facultyl of Medicine, Malatya, Turkey
OBJECTIVE AND AIM: We present the anesthetic management in a 10 months-old boy with biotinidase deficiency (BD) for cataract surgery.
CASE REPORT: A 10 months -year-old boy weighing 6 kg was scheduled for a probing procedure due to cataract. BD was diagnosed ın the outer center and treated with biotin 5 mg/day. A severe respiratory tract infections occurred during the next two months. Physical examination was growth retardation, dermatitis, alopecia, skin turgor-tonus reduced, mucous membrane dry, lethargic, hypotonia and mental retardation. Neurological symptoms (mental retardation, hypotonia) are reviewed by paediatric neurology. In preoperative evaluation, laboratory findings were normal except anemia due to iron deficiency and AST: 167 U/l, ALT: 157 U/I. The patient was taken to the operating room without premedication. Following the routine monitoring of the patient, the vascular route was opened with 24 G branules. Patient's heart rate was 145/min, blood pressure was 115/65 mmHg and SpO2 was 90% before induction of anesthesia. Induction was facilitated with lidocaine 5 mg, propofol 2 mg/kg and remifentanyl 0.25 μg/kg iv and then, size one classic laryngeal mask airway (LMA) was inserted. Following successful LMA insertion, anaesthesia was maintained with 1 MAC (minimum alveolar concentration) of sevoflurane in N2O+O2. Ventilation was controlled manually such as to maintain end tidal carbondioxide concentration (EtCO2) between 35-45 mmHg. Hemodynamic stability was maintained during the surgery. A 10 mg kg-1 paracetamol suppository was applied rectally to provide post-surgical analgesia. The surgery lasted 1 hour, the patient was awake from anesthesia uneventfully and transported to the service.
DISCUSSION: İn our case had muscle weakness, we used a safe approach of low dose propofol and remifentanyl and inserted LMA without neuromuscular blocking agents because of hypotonia. İn patients with BD, anesthetic management should be planned carefully to avoid epileptic activity, acidosis, hemodynamic instability, respiratory problems and malignant hyperthermia.
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