Branchio-oto-renal syndrome (BOR) is a rare autosomal dominant disorder. The features include branchial cysts, hearing loss, ear malformation, preauricular pits, retrognathia, congenital heart disease, and renal abnormalities. However, anesthetic management of these patients has seldom been reported. We report a case in which general anesthesia was performed for dental treatment in a patient with BOR. Airway management, renal function, and hemodynamic changes can be of critical concern during anesthetic management. A 13-year-old girl diagnosed with BOR had severe right hearing loss, right external ear malformation, renal abnormalities, and postoperative patent ductus arteriosus (PDA). Dental extraction under general anesthesia was scheduled for a supernumerary tooth. The procedure was completed with sufficient urine volume, adequate airway management, and stable hemodynamics.
Adolescent ; Airway Management ; Anesthesia, General ; Branchio-Oto-Renal Syndrome ; Branchioma ; Ductus Arteriosus, Patent ; Ear ; Ear, External ; Female ; Hearing Loss ; Heart Defects, Congenital ; Hemodynamics ; Humans ; Renal Insufficiency ; Retrognathia ; Tooth, Supernumerary

BACKGROUND: Some patients with special needs exhibit intellectual disability, including deficits in cognitive skills and decreased quality of life. The purpose of this study was to retrospectively compare changes in body composition and hemodynamics during general anesthesia in patients with and without special needs. METHODS: The backgrounds of patients who underwent oral maxillofacial surgery under general anesthesia were recorded from medical records. Intracellular water (ICW), extracellular water (ECW), stroke volume variation (SVV), and heart rate (HR) were recorded for 3 h after the start of anesthesia. Categorical data were compared using an unpaired t-test, and a P-value of less than 0.05 was regarded as significant. Numerical data were compared using the Bonferroni correction, and a P-value of less than 0.0125 was regarded as significant. RESULTS: A total of 21 patients were included in the study: 10 patients without special needs (non-S-group) and 11 patients with special needs (S-group). There were no significant differences in patients' backgrounds, except with regard to height (P = 0.03). In both groups, ICW and ECW were maintained, although they were lower in the S-group compared to the non-S-group. SVV was maintained in both groups, although it was higher in the S-group than the non-S-group. HR was significantly lower in the S-group 1 h after induction of anesthesia (P < 0.003). CONCLUSIONS: Changes in hemodynamics due to body fluid imbalance should be monitored during general anesthesia, especially for patients with special needs.
Anesthesia ; Anesthesia, General ; Body Composition* ; Body Fluids ; Heart Rate ; Hemodynamics* ; Humans ; Intellectual Disability ; Medical Records ; Quality of Life ; Retrospective Studies ; Stroke Volume ; Surgery, Oral ; Water

Airway obstruction in pediatric patients always poses a challenge for anesthesiologists. Beckwith-Wiedemann syndrome causes various abnormalities such as macroglossia and omphalocele. Patients with these abnormalities often need corrective surgeries. Management of difficult airway caused by conditions such as macroglossia in patients with this syndrome could be challenging. We encountered a case of difficult airway in an infant with Beckwith-Wiedemann syndrome. It was predicted that macroglossia might cause difficult ventilation, intubation, and extubation. Preoperative assessment and preparations for difficult airway should be considered.
Airway Management* ; Airway Obstruction ; Anesthesia, General ; Beckwith-Wiedemann Syndrome* ; Hernia, Umbilical ; Humans ; Infant ; Intubation ; Macroglossia ; Ventilation

Nasotracheal intubation is generally a useful maxillofacial surgery that provides good surgical access for intraoral procedures. When nasotracheal intubation is difficult, laryngeal mask airway (LMA) insertion can be performed, and the flexible LMA™ (FLMA) is also useful for anesthetic management. However, the FLMA provides limited access to the mouth, which restricts the insertion of instrumentation and confines the surgical field available. Here, we present our experience using the FLMA airway management for dental treatment cases involving difficulty with intubation.
Airway Management ; Anesthesia, General ; Dental Care ; Intubation* ; Laryngeal Masks* ; Mouth ; Surgery, Oral

Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn syndrome. The selection of an appropriately sized tracheal tube and maintaining intraoperatively stable hemodynamics might be critical problems for anesthetic management. In patients with short stature, the tracheal tube size may differ from what may be predicted based on age. The appropriate size ( internal diameter ) of tracheal tubes for children has been investigated. Congenital heart disease is frequently associated with Wolf-Hirschhorn syndrome. Depending on the degree and type of heart disease, careful monitoring of hemodynamics is important.
Anesthesia, General ; Child ; Child, Preschool ; Chromosome Deletion ; Female ; Genetic Diseases, Inborn ; Heart Defects, Congenital ; Heart Diseases ; Hemodynamics ; Humans ; Intellectual Disability ; Intubation, Intratracheal ; Seizures ; Wolf-Hirschhorn Syndrome*

Awake fiberoptic nasotracheal intubation is a useful technique, especially in patients with airway obstruction. It must not only provide sufficient anesthesia, but also maintain spontaneous breathing. We introduce a method to achieve this using a small dose of fentanyl and midazolam in combination with topical anesthesia. The cases of 2 patients (1 male, 1 female) who underwent oral maxillofacial surgery are reported. They received 50 µg of fentanyl 2–3 times (total 2.2–2.3 µg/kg) at intervals of approximately 2 min. Oxygen was administered via a mask at 6 L/min, and 0.5 mg of midazolam was administered 1–4 times (total 0.02–0.05 mg/kg) at intervals of approximately 2 min. A tracheal tube was inserted through the nasal cavity after topical anesthesia was applied to the epiglottis, vocal cords, and into the trachea through the fiberscope channel. All patients were successfully intubated. This is a useful and safe method for awake fiberoptic nasotracheal intubation.
Airway Obstruction ; Anesthesia ; Conscious Sedation ; Epiglottis ; Fentanyl ; Humans ; Intubation ; Male ; Masks ; Methods ; Midazolam ; Nasal Cavity ; Oxygen ; Respiration ; Surgery, Oral ; Trachea ; Vocal Cords

Chromosome 6p duplication is very rare and clinically characterized by short stature, mental retardation, and congenital heart diseases. Patients with mental retardation may present with poor oral health conditions. Dental treatment may need to be performed under general anesthesia in such patients. Our case report deals with induction of general anesthesia to a patient with chromosome 6p duplication, for dental treatment. The selection of a nasotracheal tube of an appropriate size, because of the patient's short stature, was especially important for airway management. In the present case, the patient with chromosome 6p duplication was intubated with a nasotracheal tube, which was not age-matched but adapted to the height and physique of the patient.
Airway Management ; Anesthesia, General ; Dental Care ; Heart Diseases ; Humans ; Intellectual Disability ; Oral Health