1p36 deletion syndrome is characterized by a genetic deletion that frequently causes central nervous system, craniofacial, cardiac, and musculoskeletal anomalies. Perioperative management of patients with 1p36 deletion syndrome presents unique challenges due to multiple anomalies and potential complications. We present the successful anesthetic management of a 16-year-old patient diagnosed with 1p36 deletion syndrome who underwent general anesthesia for multiple dental procedures, including dental fillings, extractions, and tartar removal. The patient had micrognathia, hypotonia, flat eyebrows, a short neck, inability to sit without support, absence of speech and self-care skills, limited ability to follow simple commands, and poor cooperation. These factors increase the risk of difficult mask ventilation and intubation. To minimize aspiration risk, we successfully intubated the patient using rapid-sequence induction and intubation (RSII) method with cricoid pressure. A perioperative multidisciplinary team approach and comprehensive preoperative evaluation are crucial due to the existing anomalies, developmental and motor delays, and potential airway complications associated with this syndrome.

Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive neurometabolic synthesis disease with sympathetic autoregulation deficiency. Perioperative management of AADCD presents unique challenges due to autonomic dysfunction, impaired sympathetic regulation of heart rate, and potential airway complications. Herein, we report the successful anesthetic management of a 14-year-old male patient with AADCD under total intravenous anesthesia during extensive dental procedures. We applied the rapid sequence induction and intubation method with cricoid pressure to minimize the risk of pulmonary aspiration due to the potential difficulty in mask ventilation and intubation, limited mouth opening, microcephaly, micrognathia, and poor cooperation. Intubation was performed without any complications. Due to the autonomic dysfunction, potential airway complications, and developmental, mental, and motor delays in this disorder, perioperative management requires a multidisciplinary approach with comprehensive preoperative evaluation.

1p36 deletion syndrome is characterized by a genetic deletion that frequently causes central nervous system, craniofacial, cardiac, and musculoskeletal anomalies. Perioperative management of patients with 1p36 deletion syndrome presents unique challenges due to multiple anomalies and potential complications. We present the successful anesthetic management of a 16-year-old patient diagnosed with 1p36 deletion syndrome who underwent general anesthesia for multiple dental procedures, including dental fillings, extractions, and tartar removal. The patient had micrognathia, hypotonia, flat eyebrows, a short neck, inability to sit without support, absence of speech and self-care skills, limited ability to follow simple commands, and poor cooperation. These factors increase the risk of difficult mask ventilation and intubation. To minimize aspiration risk, we successfully intubated the patient using rapid-sequence induction and intubation (RSII) method with cricoid pressure. A perioperative multidisciplinary team approach and comprehensive preoperative evaluation are crucial due to the existing anomalies, developmental and motor delays, and potential airway complications associated with this syndrome.

Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive neurometabolic synthesis disease with sympathetic autoregulation deficiency. Perioperative management of AADCD presents unique challenges due to autonomic dysfunction, impaired sympathetic regulation of heart rate, and potential airway complications. Herein, we report the successful anesthetic management of a 14-year-old male patient with AADCD under total intravenous anesthesia during extensive dental procedures. We applied the rapid sequence induction and intubation method with cricoid pressure to minimize the risk of pulmonary aspiration due to the potential difficulty in mask ventilation and intubation, limited mouth opening, microcephaly, micrognathia, and poor cooperation. Intubation was performed without any complications. Due to the autonomic dysfunction, potential airway complications, and developmental, mental, and motor delays in this disorder, perioperative management requires a multidisciplinary approach with comprehensive preoperative evaluation.

1p36 deletion syndrome is characterized by a genetic deletion that frequently causes central nervous system, craniofacial, cardiac, and musculoskeletal anomalies. Perioperative management of patients with 1p36 deletion syndrome presents unique challenges due to multiple anomalies and potential complications. We present the successful anesthetic management of a 16-year-old patient diagnosed with 1p36 deletion syndrome who underwent general anesthesia for multiple dental procedures, including dental fillings, extractions, and tartar removal. The patient had micrognathia, hypotonia, flat eyebrows, a short neck, inability to sit without support, absence of speech and self-care skills, limited ability to follow simple commands, and poor cooperation. These factors increase the risk of difficult mask ventilation and intubation. To minimize aspiration risk, we successfully intubated the patient using rapid-sequence induction and intubation (RSII) method with cricoid pressure. A perioperative multidisciplinary team approach and comprehensive preoperative evaluation are crucial due to the existing anomalies, developmental and motor delays, and potential airway complications associated with this syndrome.

Aromatic L-amino acid decarboxylase deficiency (AADCD) is a rare autosomal recessive neurometabolic synthesis disease with sympathetic autoregulation deficiency. Perioperative management of AADCD presents unique challenges due to autonomic dysfunction, impaired sympathetic regulation of heart rate, and potential airway complications. Herein, we report the successful anesthetic management of a 14-year-old male patient with AADCD under total intravenous anesthesia during extensive dental procedures. We applied the rapid sequence induction and intubation method with cricoid pressure to minimize the risk of pulmonary aspiration due to the potential difficulty in mask ventilation and intubation, limited mouth opening, microcephaly, micrognathia, and poor cooperation. Intubation was performed without any complications. Due to the autonomic dysfunction, potential airway complications, and developmental, mental, and motor delays in this disorder, perioperative management requires a multidisciplinary approach with comprehensive preoperative evaluation.

Glycogen storage disease (GSD) is a group of inherited disorders, which result in the deficiency of enzymes involved in glycogen metabolism, leading to an accumulation of glycogen in various organs. Deficiency of amylo-1-6-glicosidase (debranching enzyme) causes glycogen storage disease type III (GSD III). The main problems that anesthesiologists face in patients with GSD III include hypoglycemia, muscle weakness, delayed awakening due to abnormal liver function, possible difficulty in airway, and cardiomyopathy. In the face of these difficulties, airway preparation and appropriate glucose monitoring and support during the fasting period are important. The doses of the drugs to be used should be calculated considering the increased volume of distribution and decreased metabolic activity of the liver. We present the case of a child with GSD IIIa who underwent dental prosedation under general anesthesia. She was also being prepared for liver transplantation. This case was additionally complicated by the patient’s serious allergic reaction to eggs and milk.